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Sample records for con sarcoidosis ocular

  1. Ocular Sarcoidosis

    PubMed Central

    Pasadhika, Sirichai; Rosenbaum, James T

    2015-01-01

    Sarcoidosis is one of the leading causes of inflammatory eye disease. Ocular sarcoidosis can involve any part of the eye and its adnexal tissues, and may cause uveitis, episcleritis/scleritis, eyelid abnormalities, conjunctival granuloma, optic neuropathy, lacrimal gland enlargement and orbital inflammation. Glaucoma and cataract can be complications from inflammation itself or adverse effects from therapy. Ophthalmic manifestations can be isolated, or associated with other organ involvement. Patients with ocular sarcoidosis can present with a wide range of clinical presentations and severity. Multi-disciplinary approaches are required to achieve the best treatment outcomes for both ocular and systemic manifestations. PMID:26593141

  2. [Ocular Manifestations in Sarcoidosis].

    PubMed

    Walscheid, K; Tappeiner, C; Heiligenhaus, A

    2016-05-01

    Sarcoidosis is an inflammatory multi-organ disease of unknown pathogenesis, characterised by non-necrotising granulomata. Sarcoidosis predominantly manifests in the lung, but any other organ may be affected. Ocular involvement is present in about 25 to 50 % of patients. The most common ocular manifestation is uveitis, especially of the anterior eye segment. If ocular sarcoidosis is suspected, interdisciplinary assessment of the patient is mandatory, including laboratory tests, chest X-ray, assessment by a specialist in internal medicine and, ideally, histological evidence of granuloma formation in a tissue specimen. Other (infectious) causes of granulomatous inflammation need to be excluded, especially tuberculosis or syphilis. For the ophthalmological assessment, detection of granulomatous lesions is of particular importance, especially by visualising chorioretinal granuloma by fluorescein and indocyanin green angiography. Cystoid macular oedema and glaucoma are the most frequent complications limiting visual acuity. Corticosteroids, which can be administered either locally or systemically, are the mainstay of therapy. Depending on the clinical course and the development of ocular complications, systemic steroid-sparing immunosuppressive medication may be indicated.

  3. [Clinical analyses of sarcoidosis with ocular involvement].

    PubMed

    Liu, Xiaofang; Sun, Yongchang; Dai, Honglei; Jin, Jianmin; Liu, Yong

    2014-11-04

    To explore the clinical characteristics and systemic manifestations of sarcoidosis with ocular involvement. The clinical data of 19 cases of sarcoidosis with ocular involvement confirmed by pathology at Beijing Tongren Hospital from March 2004 to February 2014 were analyzed retrospectively. The ocular manifestations, chest imaging findings, laboratory tests and pathological diagnosis were reviewed. And the clinical features of sarcoidosis with ocular involvement were summarized. Among them, there were 6 males and 13 females with an average age of (47 ± 14) (16-76) years. The ocular symptoms were the initial presenting manifestations of sarcoidosis in 12 cases while 2 cases presented ocular symptoms during the course of disease. And aother 5 cases without ocular symptoms were confirmed to have ocular involvement by eye examination. The main manifestations of ocular sarcoidosis were uveitis (n = 16), chorioretinitis (n = 3), retinal vasculitis (n = 2), optic neuritis (n = 1) and orbital mass (n = 3). The key feature of sarcoidosis was bilateral hilar lymphadenopathy (BHL) (14/16) on chest film. The diagnosis in 17 cases was confirmed by biopsy of extra-ocular organs. The positive diagnostic rate of bronchial biopsy was 8/9. Ocular involvement in sarcoidosis is relatively common due to a variety of ocular manifestations and serious vision impairment in some patients. Ophthalmologic examination is essential in the clinical management of sarcoidosis. Chest imaging and bronchial biopsy are important for the diagnosis of sarcoidosis with initial ocular manifestations.

  4. Ocular sarcoidosis: new diagnostic modalities and treatment.

    PubMed

    Yang, Sung J; Salek, Sherveen; Rosenbaum, James T

    2017-09-01

    Ocular involvement in sarcoidosis is present in up to 80% of patients and is frequently manifested before diagnosis of the underlying systemic disease. Considering the therapeutic consequences, early diagnosis of the underlying disease is advantageous in patients presenting with ocular inflammation. There are several ocular findings suggestive of underlying sarcoidosis, such as granulomatous keratic precipitates, iris nodules, cells in the vitreous humor known as snowballs and snowbanks, and retinal periphlebitis. High suspicion is crucial for the diagnosis of sarcoidosis. This review on ocular sarcoidosis will mainly focus on new diagnostic and treatment modalities. Recent studies found possible new diagnostic indicators for the diagnosis of ocular sarcoidosis which include not only serum profiles but also vitreous sample analysis. Ophthalmologic imaging techniques have improved to investigate the ocular structure in detail. Results from recent uveitis clinical trials have included sarcoidosis as an underlying cause and have reported positive results. The diagnosis of ocular sarcoidosis can be challenging in some cases. High suspicion is important to diagnose ocular sarcoidosis with various laboratory and ophthalmic tools. There are many possible options for the treatment of ocular sarcoidosis including various biologic agents.

  5. Resolution of chronic ocular sarcoidosis with antimycobacterial therapy

    PubMed Central

    Richmond, Bradley W.; Richter, Kyra; King, Lloyd E.; Drake, Wonder P.

    2014-01-01

    Ocular and cutaneous sarcoidosis is a chronic manifestation of sarcoidosis that remains difficult to treat. Recent investigations demonstrating efficacy with antimicrobial therapy in pulmonary and cutaneous sarcoidosis have been reported. Here, we report dual clinical improvement in cutaneous and ocular sarcoidosis following administration of oral antimycobacterial therapy. PMID:25580448

  6. [Clinical manifestations of ocular sarcoidosis in Japanese patients].

    PubMed

    Ohara, K

    1994-06-01

    Ocular sarcoidosis was found in 57.5 to 93.5% of Japanese patients with systemic sarcoidosis. Many studies reported a high female/male ratio with high percentage of patients at 20s and 50s in age in ocular sarcoidosis. A cardinal involvement was intraocular and manifested as uveitis. A combinations of granulomatous iritis, trabecular nodules, tent-like peripheral anterior synechia, snow-ball vitreous opacities, perivasculitis, and candle-wax or spotty chorioretinal exudates were characteristic and highly specific intraocular lesions. The lesions often lead ophthalmologists to systemic examinations. In suspects of ocular sarcoidosis who had no or sparse systemic findings, a diagnosis should remain tentative. Transbronchial lung biopsy showed non-caseating epithelioid granuloma in 37 of 60 patients with suspected ocular sarcoidosis who showed no bilateral hilar lymphadenopathy.

  7. Diagnostic value of bronchoalveolar lavage in ocular sarcoidosis.

    PubMed

    Bienfait, M F; Hoogsteden, H C; Baarsma, G S; Adriaansen, H J; Verheijen-Breemhaar, L

    1987-12-01

    Bronchoalveolar lavage (BAL) is an investigation which has already proven its value in the diagnosis and follow-up of patients with pulmonary sarcoidosis. It also appears to be a valuable contribution to the diagnosis of patients presenting with ocular sarcoidosis. We evaluated the findings in BAL in 16 cases with suspected ocular sarcoidosis (14 cases of uveitis, one with eyelid-swelling and one with an inflammatory process of the lacrimal gland). BAL was positive in 11 cases e.g. showed a lymphocytosis with predominantly T4+ helper lymphocytes. There was one patient with ocular signs very suspect for sarcoidosis (a perivasculitis with candle wax infiltrates) with a normal percentage T lymphocytes (2%) in BAL. In two cases BAL was positive and showed a subclinical alveolitis, whereas no changes were seen on the chest X-ray and in Angiotensin Converting Enzyme (ACE) level.

  8. Ocular Sarcoidosis Limited to Retinal Vascular Ischemia and Neovascularization

    PubMed Central

    Dyer, Gawain; Shaikh, Saad

    2016-01-01

    A 59-year-old Caucasian male experienced progressive vision loss secondary to retinal vascular ischemia and neovascularization. At no time did he present with uveitis or vasculitis, and his serology tests were all negative. He was soon after diagnosed with sarcoidosis by hilar lymph node lung biopsy. Our patient demonstrates an atypical presentation of ocular sarcoidosis, manifesting solely as neovascularization and retinal vascular ischemia. Ophthalmologists should consider proliferative sarcoid retinopathy in patients with neovascularization. PMID:27928517

  9. Overexpression of IL-17RC associated with ocular sarcoidosis

    PubMed Central

    2014-01-01

    Background Sarcoidosis is a chronic inflammatory disease with a systemic granulomatous disorder affecting multiple organs including the eye. Both CD4+ T cell and macrophage have been linked to the pathogenesis of the disease. Methods The expression of IL-17RC was measured using FACS,immunohistochemistry and real-time PCR. Serum level of IL-17 was detected using ELISA. Results An elevated expression of IL-17RC on CD8+ T cells in peripheral blood was found in patients with ocular sarcoidosis as compared to healthy controls. Interestingly, we found a significant increase in the serum level of IL-17 in patients with ocular sarcoidosis as compared to healthy controls, which may be responsible for the induction of IL-17RC on CD8+ cells. In addition, IL-17RC appeared only in the retinal tissue of the patient with clinically active sarcoidosis. Conclusions Our results suggested a potential involvement of IL-17RC+CD8+ T cells in pathogenesis of ocular sarcoidosis. PMID:24885153

  10. Distinguishing Features of Ocular Sarcoidosis in an International Cohort of Uveitis Patients.

    PubMed

    Acharya, Nisha R; Browne, Erica N; Rao, Narsing; Mochizuki, Manabu

    2017-08-16

    To determine which clinical features distinguish ocular sarcoidosis from other forms of uveitis in an international population and to estimate the sensitivity and specificity of the International Workshop on Ocular Sarcoidosis (IWOS) clinical signs and laboratory tests. Multicenter, retrospective medical record review. Eight hundred eighty-four patients with uveitis from 19 centers in 12 countries. Data collected included suspected cause of uveitis, clinical findings, and laboratory investigations within 6 months of presentation. The IWOS criteria were used to classify patients as having definite (biopsy-proven), presumed (evidence of bilateral hilar lymphadenopathy [BHL] on chest radiograph or CT scan), probable, or possible ocular sarcoidosis. Patients with biopsy positive results or BHL on chest radiograph or CT scan were considered sarcoidosis cases. Sensitivity and specificity of clinical signs and laboratory investigations for diagnosing ocular sarcoidosis. Of the 884 uveitis patients, 264 (30%) were suspected to have ocular sarcoidosis. One hundred eighty patients (20%) met the IWOS criteria; 98 were definite (biopsy-proven) disease, 69 presumed disease (BHL), 10 probable disease, and 3 possible disease. Among sarcoidosis cases, the most common clinical signs were bilaterality (86%); snowballs or string of pearls (50%); mutton-fat keratic precipitates, iris nodules, or both (46%); and multiple chorioretinal peripheral lesions (45%). Sixty-two percent of sarcoidosis cases had elevated angiotensin converting enzyme or lysozyme and 5% demonstrated abnormal liver enzyme test results. Of the patients suspected of having sarcoidosis, 97 (37%) did not meet the IWOS criteria. With the exception of BHL, IWOS clinical findings and investigational tests had low sensitivities for diagnosing ocular sarcoidosis. In particular, liver function tests seem to have little usefulness in diagnosing ocular sarcoidosis. Many patients suspected of having sarcoidosis did not fit

  11. A case series of ocular disease as the primary manifestation in sarcoidosis.

    PubMed

    Sivakumar, M; Chee, S P

    1998-07-01

    Sarcoidosis is an idiopathic, systemic, non-caseating, granulomatous disease with protean clinical manifestations. This disease is highly prevalent in the southern states of United States of America, especially among African-Americans, but uncommon among Asians. Though sarcoidosis concerns physicians of virtually all specialties, it is particularly important to ophthalmologists since a significant number of patients will seek initial medical examination because of ocular disease. Out of the 262 cases of uveitis examined at the Singapore National Eye Centre over a period of two years, 9 cases were diagnosed as sarcoidosis. We report here 4 patients with active sarcoidosis who presented to us with ocular symptoms as the first and primary manifestation of the disease. All 4 patients developed symptoms and signs of systemic sarcoidosis at a later date. The ocular manifestations included simultaneous bilateral chronic granulomatous iridocyclitis, intermediate uveitis, posterior uveitis characterised by vasculitis with candle wax drippings, and optic nerve head granuloma.

  12. High Resolution Chest Computerized Tomography in the Diagnosis of Ocular Sarcoidosis in a High TB Endemic Population.

    PubMed

    Babu, Kalpana; Shukla, Sai Bhakti; Philips, Mariamma

    2017-04-01

    To review the role of high resolution chest computed tomography (HRCT) in ocular sarcoidosis in a high TB endemic population. This was a retrospective study. Out of 140 cases, 54 had ocular sarcoidosis, while 86 cases had ocular tuberculosis. Abnormal HRCT findings was noted in 52 cases (96.3%) of ocular sarcoidosis compared with 55 cases (64.7%) of ocular tuberculosis (p = 0.001). Mediastinal lymphadenopathy was the most common finding in both groups (p = 0.544). Hilar lymphadenopathy and fissural nodules were significantly seen in ocular sarcoidosis (p = 0.001). Necrosis was seen in three cases of ocular sarcoidosis. In nearly half of the cases, it was not possible to differentiate between sarcoidosis and tuberculosis on HRCT. HRCT is a useful diagnostic tool in ocular sarcoidosis. Bilateral hilar lymphadenopathy and fissural nodules are significant findings in ocular sarcoidosis. A confident diagnosis of ocular sarcoidosis is made by the amalgamation of results of clinical, radiologic, and other laboratory investigations.

  13. Sarcoidosis

    MedlinePlus

    ... page from the NHLBI on Twitter. What Is Sarcoidosis? Español Sarcoidosis (sar-koy-DO-sis) is a ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  14. [Clinical profile of presumed and definitive ocular sarcoidosis].

    PubMed

    Jovanović, Svetlana; Zlatanović, Gordana; Jovanović, Zorica; Radotić, Filip

    2013-01-01

    Sarcoidosis is an antigen-mediated disease of unknown cause defined by granulomatous inflammation of different organs. The aim of this study was to analyze the clinical picture in 26 patients with a definitive and presumed ocular sarcoidosis. The following tests were conducted: angiotensin-converting enzyme, tuberculin skin test, liver enzymes and calcium in urine and serum. Enlarged hilar lymph glands were diagnosed using X-ray tomography or computed tomography of the mediastinum. A biopsy of lymph glands was performed either transbronchially or transmediastinally. Ophthalmic examination included biomicroscopic examination, intraocular pressure measurement, photofundus, fluorescein angiography, optical coherence tomography and computerized perimetry. The average follow-up period of the disease was 6.1 years. The average age was 52.0 years. There were 62.5% female patients, with bilateral incidence of 69.2%. The clinical picture included: panuveitis (in 30.8% of patients), anterior uveitis (26.9%), posterior uveitis (26.9%), intermediate uveitis (7.7%) and vitritis in (7.7%). The following complications were observed: cataract (in 34.6% of patients), cystoid macular edema (23.1%), glaucoma (15.4%), macroaneurysms (15.4%), neovascularization (7.7%) and band keratopathy (3.8%). There was a statistically significant difference in the visual acuity (p = 0.033) and severity of clinical symptoms (p = 0.02) between the groups of patients with retinal vasculitis associated with"candle-wax" phenomenon and the group of patients with multiple chorioretinal lesions--"white dot" syndrome. To our consideration, the differences observed between the two groups are the result of retinal vasculitis and subsequent macular edema, which is significantly more frequent in the group with"candle-wax" phenomenon.

  15. Clinical aspects of posterior uveitis in ocular sarcoidosis.

    PubMed

    Jovanović, Svetlana V; Jovanović, Zorica D; Radotić, Filip M; Srećković, Suncica B; Paunović, Svetlana S; Stojanović, Jasmina D

    2012-06-01

    Two clinical forms of the "white spot" syndrome in patients with posterior uveitis in definitive and presumable ocular sarcoidosis were analyzed. Group 1 was characterized by periphlebitis and discrete white spots around the vein of the retina, so-called "candle-wax", whereas group 2 showed yellow-orange solitary nodules located at the choroid, i.e. multifocal choroiditis. Visual acuity and the severity of clinical presentation were assessed in both groups. Visual acuity, Snellen equivalent was 0.52 +/- 0.36 in group 1 and 0.82 +/- 0.39 in group 2 with lesions at the level of choroid. One-way analysis of variance ANOVA showed a statistically significant between-group difference in visual acuity (p = 0.03). The mean severity of clinical presentation was 11.80 +/- 2.04 points in group 1 and 5.80 +/- 4.18 points in group 2. T-test for independent samples yielded a statistically significant difference between the groups (p = 0.02). A statistically significant difference in visual acuity was the result of vasculitis in the group with the "candle-wax" phenomenon, which is associated with retinal vasculitis and causes cystoid macular edema and reduction of visual acuity. Complications such as cataract, glaucoma and neovascularization, which also decrease visual acuity, were more frequent in group 1.

  16. Two cases of ocular sarcoidosis in which vitreous cytology was useful for supporting the diagnosis

    PubMed Central

    Matsuoka, Masato; Ogata, Nahoko; Takahashi, Kanji; Kinoshita, Yuichi; Nishimura, Tetsuya

    2012-01-01

    The purpose of this paper is to report two cases of sarcoidosis with conflicting signs in which vitreous cytology was useful for supporting the diagnosis. Case 1 was a 56-year-old man who was referred with blurred vision, and was found to have iridocyclitis, vitreous opacities, and optic neuritis bilaterally. He also had a swelling of both mandibular glands. He received pulsed corticosteroid therapy, but the vitreous opacities and papilledema did not resolve. The second case was a 77-year-old man referred with blurred vision, and both eyes had snowball-like vitreous opacities and optic neuritis. The ocular findings in both cases strongly suggested sarcoidosis, but the systemic findings did not meet the diagnostic criteria for sarcoidosis. We performed pars plana vitrectomy to remove the vitreous opacities, and the collected vitreous samples were sent for cytological analyses. Epithelioid and multinucleated giant cells pathognomonic of sarcoidosis were found in the vitreous samples which enabled us to make a diagnosis of sarcoidosis. Vitreous cytology can help in supporting a diagnosis of sarcoidosis, especially in cases of ocular inflammation suggesting sarcoidosis but systemic findings that do not satisfy the diagnostic criteria for this disease. PMID:22927729

  17. Sarcoidosis in the national veteran population: association of ocular inflammation and mortality.

    PubMed

    Birnbaum, Andrea D; French, Dustin D; Mirsaeidi, Mehdi; Wehrli, Sarah

    2015-05-01

    To describe the distribution of ocular sarcoidosis in the veteran population and to determine the association between ocular disease and all-cause mortality. Retrospective review. The Veterans Health Administration National Patient Care Database information on medical diagnoses, date of diagnosis, age, race, gender, and Veterans Administration medical center station number for site-specific calculations for fiscal years 2010 through 2012 was collected. Mortality data were obtained from the Beneficiary Identification Records Locator Subsystem. The patient cohort was identified with a primary diagnosis of sarcoidosis using International Classification of Disease, ninth edition, code of 135 in outpatient treatment files for the study period. The sarcoidosis patients were divided into those with uveitis or orbital inflammation (defined as ocular inflammation for this study) and those without uveitis or orbital inflammation. Survival analysis was performed using the Cox proportional hazard method. Association between ocular inflammation and 1-year mortality. Of 15 130 subjects with sarcoidosis, 3364 (22.2%) were evaluated in an eye clinic within a Veterans Administration Medical Center. Most patients were diagnosed with anterior uveitis (n = 1013; 80.7% of ocular inflammation), and the least common diagnosis was orbital granuloma (n = 28; 2.2% of ocular inflammation). Male gender was protective to the development of uveitis (estimate, 0.76; 95% confidence interval, 0.65-0.88; P = 0.0005). The overall 1-year all-cause mortality for all patients with a diagnosis of sarcoidosis was 2.0%. Ocular inflammation was associated with a decrease in 1-year all-cause mortality (simple model: hazard ratio, 0.36; P = 0.0015; complex model: hazard ratio, 0.35; P = 0.013). Veterans with ocular inflammation had significantly lower 1-year all-cause mortality than those without documented ocular inflammation. The reason for this finding remains to be established. Copyright © 2015 American

  18. Sarcoidosis

    MedlinePlus

    Sarcoidosis is a disease that leads to inflammation, usually in your lungs, skin, or lymph nodes. It starts as tiny, grain-like lumps, called granulomas. Sarcoidosis can affect any organ in your body. No ...

  19. Interleukin-2 Receptor and Angiotensin-Converting Enzyme as Markers for Ocular Sarcoidosis

    PubMed Central

    Gundlach, Enken; Hoffmann, Michael Marcus; Prasse, Antje; Heinzelmann, Sonja; Ness, Thomas

    2016-01-01

    Purpose To study the impact of soluble IL2 receptor (sIL2R), chest x-ray (CxR), and angiotensin-converting enzyme (ACE) as markers for sarcoidosis in uveitis patients. Design Retrospective study. Methods Serum concentrations of sIL2R and ACE were measured in patients with active uveitis. Those with elevated sIL2R and /or ACE values were examined for suspected systemic sarcoidosis. Main Outcome Measure Our main outcome parameters were the specificity and sensitivity of sIL2R, CxR and ACE in screening for ocular sarcoidosis. Results We measured 261 patients with uveitis for sarcoidosis using sIL2R and ACE between January 2008 and November 2011; sarcoidosis was been diagnosed using other tests (e.g. computer tomography, brochoalveolar lavage, biopsy) in 41 of 53 patients with elevated sIL2R values (>639 U/ml) and in one patient with normal sIL2R (582 U/ml). Their mean sIL2R value was 1310 U/ml, extending from 582 to 8659 U/ml. Only 9 patients, however, presented elevated ACE (>82 U/l). Their mean ACE value was 116.4 U/l, ranging from 84.1 to 175.5 U/l. IL2R specificity was 94% with 98% sensitivity. In contrast, ACE had a specificity of 99.5%, but a sensitivity of only 22%; the chest x-ray had a specificity of 100% with 50% sensitivity in detecting sarcoidosis. We observed the entire spectrum of uveitis: sixteen patients suffered from anterior, 8 from intermediate, 16 from posterior, and 2 from panuveitis. Conclusions An elevated level of soluble IL2R suggests sarcoidosis with uveitis more convincingly than ACE, making sIL2R a more effective marker parameter for sarcoidosis than ACE or chest x-ray in uveitis patients. PMID:26799486

  20. Sarcoidosis

    MedlinePlus

    ... the elbow Sarcoidosis on the nose and forehead Respiratory system References Judson MA, Morgenthau AS, Baughman RP. Sarcoidosis. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine . 6th ed. Philadelphia, PA: Elsevier Saunders; 2016: ...

  1. Diagnostic categorization according to the First International Workshop on Ocular Sarcoidosis (FIWOS) criteria in a series of 11 patients.

    PubMed

    Meneses, Carlos F; Egües, César A; Uriarte, Miren; Errazquin, Nerea; Valero Jaimes, Alejandro; Maíz, Olga; Belzunegui, Joaquín; Blanco, Ana

    Categorization of patients diagnosed with ocular sarcoidosis during the period 2009-2014. The medical records of patients with ocular sarcoidosis were reviewed and variables were collected to categorize the patients according to the criteria of the FIWOS. We found 11 patients, 7 women and 4 men, with sarcoid uveitis; the median age was 58 years. Bilateral panuveitis was the most common pattern (54.5%), followed by chronic anterior uveitis (27.2%). The diagnosis of sarcoidosis was definitive in 4 patients (36.3%), presumed in 5 (45.4%), probable in 1 (9%) and possible in 1 (9%). Ocular sarcoidosis was diagnosed in more than half of the patients who had no confirmatory biopsy. Bilateral panuveitis and chronic anterior uveitis were the patterns most frequently observed. Copyright © 2016. Publicado por Elsevier España, S.L.U.

  2. Cigarette smoking and male sex are independent and age concomitant risk factors for the development of ocular sarcoidosis in a new orleans sarcoidosis population

    PubMed Central

    Janot, Adam C.; Huscher, Dörte; Walker, McCall; Grewal, Harmanjot K.; Yu, Mary; Lammi, Matthew R.; Saketkoo, Lesley Ann

    2016-01-01

    Introduction Sarcoidosis is a multi-organ system granulomatous disease of unknown origin with an incidence of 1–40/100,000. Though pulmonary manifestations are predominant, ocular sarcoidosis (OS) affects 25–50% of patients with sarcoidosis and can lead to blindness. Methods A retrospective, single-center chart review of sarcoidosis cases investigated variables associated with the development of OS. Inclusion criteria were biopsy-proven sarcoidosis, disease duration greater than 1 year, documented smoking status on chart review and documentation of sarcoid-related eye disease. Multivariate analysis identified independent risk factors for OS. Results Of 269 charts reviewed, 109 patients met inclusion criteria. The OS group had a significantly higher proportion of smokers (71.4%) than without OS (42.0%, p=0.027) with no difference (p=0.61) in median number of pack years. Male sex was significantly higher in the OS group (57.1% versus 26.1%, p=0.009). Median duration of sarcoidosis was higher in the OS group (10 versus 4 years, p=0.031). Multivariate regression identified tobacco exposure (OR=5.25, p=0.007, 95% CI 1.58–17.41), male sex (OR=7.48, p=0.002, 95% CI 2.15–26.01), and age (OR=1.114, p=0.002, 95% CI 1.04–1.19) as concomitant risk factors for the development of OS. Conclusion To date, there are few dedicated investigations of risk factors for OS, especially smoking. This investigation identified male sex, age, and tobacco exposure as independent risk factors for OS. Though disease duration did not withstand regression analysis in this moderately sized group, age at chart review suggests screening for OS should not remit but rather intensify in aging patients with sarcoidosis. PMID:26278693

  3. Clinical Manifestations, Complications and Treatment of Ocular Sarcoidosis: Correlation between Visual Efficiency and Macular Edema as Seen on Optical Coherence Tomography.

    PubMed

    Paovic, Jelena; Paovic, Predrag; Sredovic, Vojislav; Jovanovic, Svetlana

    2016-09-14

    Sarcoidosis is a chronic systemic autoimmune disease which belongs to a group of systemic granulomatous diseases. It can be confirmed through characteristic systemic and ocular manifestations and histological findings. Biopsy is the golden standard for diagnosing sarcoidosis. Ocular sarcoidosis can be confirmed, probable, or possible. Over a two-year period, ocular manifestations were studied on a sample of 52 patients, each followed for four months and diagnosed with some form of systemic sarcoidosis. Most frequent systemic manifestations in patients with ocular sarcoidosis were pulmonary, skin, glandular, and systemic generalized sarcoidosis. The disease was diagnosed four times more frequently in females than males (42:10, respectively; p < 0.05). Most frequent, and statistically significant, manifestation of ocular sarcoidosis is anterior uveitis (64.61%; p < 0.01). Macular edema and periphlebitis associated with periarteritis were frequent, and statistically significant (43.90% and 29.26%, respectively; p < 0.05). Overall, with regards to gender and location (right eye; left eye), visual acuity was >0.5 and of statistical significance (76.92%; p < 0.01). The most common therapy consisted of systemic corticosteroids (26.67%) and/or a combination of corticosteroids and immunosuppressive drugs (23.33%). In 16 eyes treated with repeated doses of sub-Tenon's injections, both initial and control visual acuity correlated with average thickness. There was positive correlation between several optical coherence tomography findings before and after treatment.

  4. Association of High-Mobility Group Box-1 With Th Cell-Related Cytokines in the Vitreous of Ocular Sarcoidosis Patients.

    PubMed

    Takeuchi, Masaru; Taguchi, Manzo; Sato, Tomohito; Karasawa, Kyoko; Sakurai, Yutaka; Harimoto, Kohzou; Ito, Masataka

    2017-01-01

    High-mobility group box-1 (HMGB1) is a nonhistone DNA-binding nuclear protein released from necrotic cells, which is also secreted by activated leukocytes and acts as a primary proinflammatory cytokine. In this study, we compared vitreous HMGB1 levels in ocular sarcoidosis with those in noninflammatory vitreoretinal diseases and evaluated its association with Th cell-related and proinflammatory cytokines. The study group consisted of 24 patients with ocular sarcoidosis. The control group consisted of 27 patients with proliferative diabetic retinopathy (PDR) and 24 with idiopathic epiretinal membrane (ERM). Vitreous fluid samples were obtained at the beginning of vitrectomy. Vitreous levels of HMGB1 and IL-1β, IL-4, IL-6, IL-10, IL-17A, IL-17F, IL-21, IL-22, IL-23, IL-25, IL-31, IL-33, IFN-γ, soluble CD40 ligand (sCD40L), and TNFα were measured. High-mobility group box-1 was detected in the vitreous of 23 of 24 patients (95.8%) with ocular sarcoidosis. Mean vitreous level of HMGB1 was the highest in the sarcoidosis group, followed by the PDR and ERM groups, with significant differences between the three groups. In the sarcoidosis group, vitreous levels of IL-6, IL-10, IL-31, IFN-γ, sCD40L, and TNFα were significantly higher than those in the idiopathic ERM group, and IFN-γ and sCD40L were significantly higher than those in the PDR group. Vitreous HMGB-1 level correlated significantly with IL-10, IFN-γ, and sCD40L levels but not with IL-6, IL-17, IL-31, or TNFα levels. The vitreous level of HMGB1 is elevated in ocular sarcoidosis and is associated with vitreous levels of Th1- and regulatory T-related cytokines, but not with proinflammatory or Th17-related cytokines.

  5. Sarcoidosis presenting as orbital pseudotumor.

    PubMed

    Kobak, S; Topaloglu, A; Öncel, G; Karaarslan, A

    2015-09-16

    Sarcoidosis is a systemic multiorgan disorder of unknown etiology characterized by a non-caseating granuloma reaction. Ocular involvement has been reported in 25-60% of the patients. Seven percent of the patients with sarcoidosis may first see an ophthalmologist due to ocular complaints. This report aims to present our diagnostic and treatment approach to a female patient with significant unilateral lacrimal gland swelling and musculoskeletal involvement, who was diagnosed with sarcoidosis on the basis of tests and histological studies.

  6. Sarcoidosis Quiz

    MedlinePlus

    ... Share this page from the NHLBI on Twitter. Sarcoidosis Quiz Sarcoidosis is a disease of unknown cause that leads ... various organs in the body. The outcome of sarcoidosis varies. The disease leads to organ damage in ...

  7. Cardiac Sarcoidosis

    MedlinePlus

    ... sarcoidosis. Sarcoidosis is characterized by the presence of granulomas. These are ball-like collections of white blood ... to a foreign substance. The inflammation associated with granulomas can damage every part of the heart, including ...

  8. Learn About Sarcoidosis

    MedlinePlus

    ... and Diseases > Lung Disease Lookup > Sarcoidosis Learn About Sarcoidosis Sarcoidosis is a disease of unknown origin that ... Americans may experience more severe symptoms. What Is Sarcoidosis? While no one knows what causes sarcoidosis, it ...

  9. Neuro-ophthalmic sarcoidosis.

    PubMed

    Turner, R G; James, D G; Friedmann, A I; Vijendram, M; Davies, J P

    1975-11-01

    Sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. Fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed.

  10. Neuro-ophthalmic sarcoidosis.

    PubMed Central

    Turner, R. G.; James, D. G.; Friedmann, A. I.; Vijendram, M.; Davies, J. P.

    1975-01-01

    Sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. Fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed. Images PMID:1203222

  11. Subcutaneous sarcoidosis.

    PubMed

    Marcoval, Joaquim; Moreno, Abelardo; Mañá, Juan; Peyri, Jordi

    2008-10-01

    Subcutaneous sarcoidosis has been reported to occur in 1.4% to 6% of patients with systemic sarcoidosis. Most reported cases are in women, most often in their fifth and sixth decades, and appear as multiple, asymptomatic, hardly indurated subcutaneous nodules without changes in the overlying epidermis. The lesions are characteristically located in the upper extremities, mainly in the forearms, and usually are bilateral and asymmetric. In most cases the lesions appear at the beginning of systemic sarcoidosis and are not associated with chronic fibrotic disease. Histopathologically, sarcoidosis is characterized by noncaseating naked granulomas involving fat lobules, with minimal to no septal involvement.

  12. Cutaneous sarcoidosis.

    PubMed

    Noe, Megan H; Rosenbach, Misha

    2017-09-01

    Cutaneous sarcoidosis occurs in up to 30% of patients with sarcoidosis and skin findings are often the initial presenting symptom. Cutaneous sarcoidosis is a rare skin disease and many aspects of the disease presentation and treatment are not well understood. This review will highlight developments in the epidemiology, clinical presentation, diagnosis and treatment of cutaneous sarcoidosis over the past several years. Epidemiological studies from several different populations reaffirm that cutaneous sarcoidosis is more common in women and is often the presenting symptom of systemic sarcoidosis. Recently, more cases are being reported in association with oncologic immune modulators, which will be of great interest as use of those agents increases. Also, ultrasound has shown promise for the imaging of cutaneous granulomas for disease assessment and measuring response to treatment. Finally, the treatment of cutaneous sarcoidosis remains difficult and is based largely on retrospective data with a paucity of large, prospective trials. There have been recently introduced and validated cutaneous scoring tools which show promise and may lead to more high-quality studies going forward. The recent developments in cutaneous sarcoidosis have identified many new pharmacologic and physical triggers of disease, but the evidence for effective treatment is still lacking. Further research is necessary to improve the care of patients with cutaneous sarcoidosis.

  13. Specific (granulomatous) oral lesions of sarcoidosis: report of two cases.

    PubMed

    Marcoval, Joaquim; Mañá, Joan

    2010-05-01

    Sarcoidosis is a multisystemic granulomatous disease of unknown aetiology that usually presents with bilateral hiliar adenopathies, pulmonary infiltrations, and ocular and cutaneous lesions. The specific cutaneous lesions, or granulomatous, are produced in from 9% to 37% of patients with systemic sarcoidosis. Nevertheless, few patients have been described having specific lesions from sarcoidosis in the oral mucous membrane. We present 2 patients with systemic sarcoidosis who developed specific sarcoidosis lesions in the oral mucous membrane. The first patient presented a plaque on the tongue of a chronic nature associated with facial lupus pernio. The second patient presented a rapidly growing nodular lesion on the lower lip that was in fact the presentation sign of the disease. Although infrequent, oral lesions may constitute the first sign of systemic sarcoidosis. For this reason the oral mucous membrane needs to be explored when there is suspicion of sarcoidosis, and systemic sarcoidosis must be included in the differential diagnosis of oral granulomatous lesions.

  14. Cardiac Sarcoidosis.

    PubMed

    Birnie, David; Ha, Andrew C T; Gula, Lorne J; Chakrabarti, Santabhanu; Beanlands, Rob S B; Nery, Pablo

    2015-12-01

    Studies suggest clinically manifest cardiac involvement occurs in 5% of patients with pulmonary/systemic sarcoidosis. The principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. Data indicate that an 20% to 25% of patients with pulmonary/systemic sarcoidosis have asymptomatic (clinically silent) cardiac involvement. An international guideline for the diagnosis and management of CS recommends that patients be screened for cardiac involvement. Most studies suggest a benign prognosis for patients with clinically silent CS. Immunosuppression therapy is advocated for clinically manifest CS. Device therapy, with implantable cardioverter defibrillators, is recommended for some patients.

  15. Gastric sarcoidosis.

    PubMed Central

    Akinyemi, Emmanuel; Rohewal, Upinder; Tangorra, Matthew; Abdullah, Muhammad

    2006-01-01

    A 58-year-old Jamaican male presented with acute-onset, right-sided facial droop and slurred speech. He had an episode of upper gastrointestinal (GI) bleed on the second day of admission and endoscopy with biopsy of antral ulcer revealed gastric sarcoidosis. This case demonstrates the rare entity of gastric sarcoidosis presenting acutely with an upper GI bleed. Images Figure 1 Figure 2 PMID:16775918

  16. Biologic therapies in the treatment of sarcoidosis.

    PubMed

    Saketkoo, Lesley Ann; Baughman, Robert P

    2016-08-01

    Sarcoidosis is a disease of remarkable heterogeneity in organ manifestation, severity and natural history, characterized by the presence of non-caseating granulomas. The majority of cases are acute and self-limited or remit with short courses of glucocorticoids; however, a proportion progress to a life-threatening obliterative fibrotic type associated with significant disability related to pulmonary, cardiac, ocular or central nervous system involvement. Biologic agents have been demonstrated in the successful treatment of refractory organ-threatening sarcoidosis; and though sarcoidosis remains elusive in predictability of progression, strong evidence suggests an indisputably efficacious role for these agents in efforts to stave morbidity and mortality related to sarcoidosis. This paper provides a review of sarcoidosis mechanistic etiopathogenesis to highlight the hypothetical underpinnings of the utility and concerns of current biologic treatments in current use and the potential future applications of newer agents and those under development.

  17. Animal models of sarcoidosis.

    PubMed

    Hu, Yijie; Yibrehu, Betel; Zabini, Diana; Kuebler, Wolfgang M

    2017-03-01

    Sarcoidosis is a debilitating, inflammatory, multiorgan, granulomatous disease of unknown cause, commonly affecting the lung. In contrast to other chronic lung diseases such as interstitial pulmonary fibrosis or pulmonary arterial hypertension, there is so far no widely accepted or implemented animal model for this disease. This has hampered our insights into the etiology of sarcoidosis, the mechanisms of its pathogenesis, the identification of new biomarkers and diagnostic tools and, last not least, the development and implementation of novel treatment strategies. Over past years, however, a number of new animal models have been described that may provide useful tools to fill these critical knowledge gaps. In this review, we therefore outline the present status quo for animal models of sarcoidosis, comparing their pros and cons with respect to their ability to mimic the etiological, clinical and histological hallmarks of human disease and discuss their applicability for future research. Overall, the recent surge in animal models has markedly expanded our options for translational research; however, given the relative early stage of most animal models for sarcoidosis, appropriate replication of etiological and histological features of clinical disease, reproducibility and usefulness in terms of identification of new therapeutic targets and biomarkers, and testing of new treatments should be prioritized when considering the refinement of existing or the development of new models.

  18. [Optic disc granuloma secondary to sarcoidosis].

    PubMed

    Qu-Knafo, L; Auregan-Giocanti, A

    2017-02-01

    We report a case of optic disc granuloma due to sarcoidosis. A 64-year-old, caucasian female with a history of pulmonary sarcoidosis presented with a vision loss on her left eye. The ophthalmologic examination revealed a discrete optic disc infiltrate compatible with the diagnosis of optic disc granuloma. Fluorescein angiography showed diffusion and impregnation of the granuloma without vascularitis. The optical coherence tomography demonstrated a homogenous and isoreflective lesion at the optic disc. The patient recovered her visual acuity after systemic corticosteroid treatment. Isolated optic disc granuloma is a rare condition of ocular sarcoidosis.

  19. The epidemiology of sarcoidosis

    PubMed Central

    Hall, G.; Sharma, O. P.; Naish, P.; Doe, W.; James, D. Geraint

    1969-01-01

    The nationality, social factors, exposures, morbidity, mortality, and hospital discharge notes have been analysed in a series of patients with histologically proven sarcoidosis, and correlated with clinical and radiological features. Compared with the expected prevalence according to the Central London population obtained from the 1961 Census, Irish and West Indians attended the Sarcoidosis Clinic twice as frequently as British, whereas African Negroes are under-represented. Sarcoidosis is slightly commoner in women, particularly those in the childbearing years of life. Mass miniature radiography rates per 100,000 population reveal prevalence rates of twenty overall, forty-three in those aged 25-34 years, and ten in those aged over 45 years. Erythema nodosum, other skin lesions, and ocular involvement occurred twice as often in women. The death-rate of about 1·7/106 population is slightly higher in women and in those living in rural districts. Hospital discharge rates are about three per 100,000 people at risk each year. PMID:5821184

  20. [Livedo-like cutaneous sarcoidosis].

    PubMed

    Duboys, S; Cante, V; Monégier du Sorbier, C; Guillet, G

    2016-05-01

    The cutaneous signs of sarcoidosis are extremely polymorphous and may be classified under several different headings. Herein, we report the case of a 51-year-old female patient presenting bilateral livedo of the knees accompanied by systemic signs with polyarthralgia, impaired general state of health, weight loss, and a sensation of dyspnoea. Skin biopsy revealed giant-cell granuloma around the dermal vessels, with no caseous necrosis. Histopathological examination of the patient's blood vessels revealed no abnormalities. Laboratory tests showed high levels of angiotensin converting enzyme (1.5×ULN), bilateral mediastinal adenomegaly and incipient pulmonary fibrosis. Systemic sarcoidosis was diagnosed on the basis of the overall clinical and laboratory picture. The patient subsequently presented hepatic involvement and hypercalcaemia associated with the sarcoidosis; systemic corticosteroid therapy (prednisone) was initiated, with slow dose-reduction. The outcome was favourable with a return to normal laboratory values, regression of the adenomegaly and subsidence of the livedo. The literature contains reports of a number of cases of livedo heralding sarcoidosis. The majority of such cases involved young or middle-aged female patients of Asian origin presenting systemic sarcoidosis, with a high frequency of ocular and neural involvement. Livedo could be accounted for by the specific disposition of granulomas around the arterioles, resulting in disturbance of local blood flow, which was probably associated with the mechanical compression of vessels and with the micro-thrombi noted at histology. Livedo may be considered a clinical sign of cutaneous sarcoidosis. Laboratory tests to screen for sarcoidosis may be proposed in the exploration of atypical livedo. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  1. Living with Sarcoidosis

    MedlinePlus

    ... page from the NHLBI on Twitter. Living With Sarcoidosis Sarcoidosis has no cure, but you can take ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  2. What Causes Sarcoidosis?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Causes Sarcoidosis? The cause of sarcoidosis isn't known. More ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  3. How Is Sarcoidosis Treated?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Sarcoidosis Treated? Not everyone who has sarcoidosis needs treatment. ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  4. Foundation for Sarcoidosis Research

    MedlinePlus

    ... a Clinical Trial Our mission is to stop sarcoidosis — join us. The sarcoidosis community needs your help ... receive periodic emails from the Foundation. Foundation For Sarcoidosis Research 1820 W. Webster Ave., Ste 304 Chicago, ...

  5. Cutaneous sarcoidosis.

    PubMed

    Marchell, Richard M; Judson, Marc A

    2010-08-01

    Sarcoidosis is a systemic disease with skin manifestations. Skin manifestations are classified as nonspecific if they are not characterized by granulomatous inflammation and specific if the lesions have granulomas histologically. Erythema nodosum is the most common nonspecific skin manifestation, and it portends a good prognosis. Specific skin lesions have a varied clinical appearance, although often they can be distinguished by their yellow translucent character. Despite the potential variable appearance, there are common clinical presentations. Lupus pernio lesions are nodular violaceous specific skin lesions found predominantly on the face associated with scarring and a poor prognosis. Treatment of cutaneous sarcoidosis is primarily done to avoid scarring and cosmetic disfigurement. Local and systemic corticosteroids are the mainstay of treatment for the disease. Corticosteroid-sparing agents used to manage the disease include antimalarials, methotrexate, and tetracycline antibiotics. Tumor necrosis factor-alpha (TNF-alpha) antagonists such as infliximab may have a role in cutaneous sarcoidosis, especially in refractory cases that are resistant to the standard regimens.

  6. The pathology of sarcoidosis.

    PubMed

    Gal, Anthony A; Koss, Michael N

    2002-09-01

    Sarcoidosis is a multisystemic disease characterized by a nonnecrotizing granulomatous inflammatory process that can progress through various stages of activity. The distinctive histology of sarcoidosis is often associated with various morphologic findings in the lung and other organs. The rare variants, necrotizing sarcoidal granulomas and nodular sarcoidosis, share some of the histologic features of typical sarcoidosis, but may have different clinical and pathologic manifestations. The clinician and pathologist should be aware of the diverse pathologic appearances of sarcoidosis.

  7. Childhood sarcoidosis: Louisiana experience.

    PubMed

    Gedalia, Abraham; Khan, Tahir A; Shetty, Avinash K; Dimitriades, Victoria R; Espinoza, Luis R

    2016-07-01

    A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with

  8. Sarcoidosis Occurring After Lymphoma

    PubMed Central

    London, Jonathan; Grados, Aurélie; Fermé, Christophe; Charmillon, Alexandre; Maurier, François; Deau, Bénédicte; Crickx, Etienne; Brice, Pauline; Chapelon-Abric, Catherine; Haioun, Corinne; Burroni, Barbara; Alifano, Marco; Le Jeunne, Claire; Guillevin, Loïc; Costedoat-Chalumeau, Nathalie; Schleinitz, Nicolas; Mouthon, Luc; Terrier, Benjamin

    2014-01-01

    Abstract Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy. An association of sarcoidosis and lymphoproliferative disease has previously been reported as the sarcoidosis-lymphoma syndrome. Although this syndrome is characterized by sarcoidosis preceding lymphoma, very few cases of sarcoidosis following lymphoma have been reported. We describe the clinical, biological, and radiological characteristics and outcome of 39 patients presenting with sarcoidosis following lymphoproliferative disease, including 14 previously unreported cases and 25 additional patients, after performing a literature review. Hodgkin lymphoma and non-Hodgkin lymphoma were equally represented. The median delay between lymphoma and sarcoidosis was 18 months. Only 16 patients (41%) required treatment. Sarcoidosis was of mild intensity or self-healing in most cases, and overall clinical response to sarcoidosis was excellent with complete clinical response in 91% of patients. Sarcoidosis was identified after a follow-up computerized tomography scan (CT-scan) or 18fluorodeoxyglucose-positron emission tomography/computerized tomography (18FDG-PET/CT) evaluation in 18/34 patients (53%). Sarcoidosis is therefore a differential diagnosis to consider when lymphoma relapse is suspected on a CT-scan or 18FDG-PET/CT, emphasizing the necessity to rely on histological confirmation of lymphoma relapse. PMID:25380084

  9. Biomarkers in sarcoidosis.

    PubMed

    Chopra, Amit; Kalkanis, Alexandros; Judson, Marc A

    2016-11-01

    Numerous biomarkers have been evaluated for the diagnosis, assessment of disease activity, prognosis, and response to treatment in sarcoidosis. In this report, we discuss the clinical and research utility of several biomarkers used to evaluate sarcoidosis. Areas covered: The sarcoidosis biomarkers discussed include serologic tests, imaging studies, identification of inflammatory cells and genetic analyses. Literature was obtained from medical databases including PubMed and Web of Science. Expert commentary: Most of the biomarkers examined in sarcoidosis are not adequately specific or sensitive to be used in isolation to make clinical decisions. However, several sarcoidosis biomarkers have an important role in the clinical management of sarcoidosis when they are coupled with clinical data including the results of other biomarkers.

  10. Diagnosis of sarcoidosis

    SciTech Connect

    Sharma, O.P.

    1983-07-01

    During the last decade, many biochemical and immunologic advances have been made in the treatment and understanding of sarcoidosis. These studies have helped us to understand the basic mechanisms involved in granuloma formation, and many clinicians have used the information to diagnose and assess the activity of sarcoidosis. Further studies are needed to clearly establish the role of these advances in the everyday management of patients with sarcoidosis.

  11. Recent advances in sarcoidosis.

    PubMed

    Morgenthau, Adam S; Iannuzzi, Michael C

    2011-01-01

    Sarcoidosis, a systemic granulomatous disease of undetermined etiology, is characterized by a variable clinical presentation and course. During the past decade, advances have been made in the study of sarcoidosis. The multicenter ACCESS (A Case Control Etiologic Study of Sarcoidosis) trial recruited > 700 subjects with newly diagnosed sarcoidosis and matched control subjects. Investigators were unable to identify a single cause of sarcoidosis, but ACCESS paved the way for subsequent etiologic studies. The Mycobacterium tuberculosis catalase-peroxidase protein has been identified as a potential sarcoidosis antigen. Genetic aspects of the disease have been elucidated further. Genome-wide scans have identified candidate genes. Gene expression analyses have defined cytokine dysregulation in sarcoidosis more clearly. Although the criteria for diagnosis have not changed, sarcoidosis remains a diagnosis of exclusion best supported by a tissue biopsy specimen that demonstrates noncaseating granulomas in a patient with compatible clinical and radiologic features of the disease. Endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal lymph nodes has facilitated diagnosis, often eliminating the need for more invasive procedures, such as mediastinoscopy. PET scanning has proven valuable in locating occult sites of active disease. Currently, no reliable prognostic biomarkers have been identified. The tumor necrosis factor inhibitors, a relatively new class of agents, have been used in patients with refractory disease. It is unclear whether phosphodiesterase-5 inhibitors, prostaglandin analogs, or endothelin antagonists should be used for the treatment of sarcoidosis-associated pulmonary hypertension.

  12. Rheumatologic Manifestations of Sarcoidosis

    PubMed Central

    Sweiss, Nadera J.; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G.N.; Niewold, Timothy B.; Baughman, Robert P.

    2012-01-01

    Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration–approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients’ symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis. PMID:20665396

  13. Sarcoidosis: a Critical Review of History and Milestones.

    PubMed

    Spagnolo, Paolo

    2015-08-01

    Sarcoidosis is a chronic systemic disease of unknown origin and uncertain prognosis that most commonly affects young adults, and frequently presents with bilateral hilar lymphadenopathy, pulmonary infiltrates and ocular and skin lesions. The diagnosis is established when characteristic clinical-radiological features are supported by compatible histopathology of epithelioid cell granulomas, following exclusion of known causes of granulomatous inflammation. Indeed, sarcoidosis belongs to a large family of disorders that share granuloma formation as common denominator. Since its first description by Jonathan Hutchinson in 1869, sarcoidosis has generated enormous interest and considerable controversy. In Hutchinson's day, it was considered a dermatological condition, which gradually evolved into a multisystem disorder associated in the majority of cases with respiratory abnormalities. With time, it has also become clear that sarcoidosis occurs throughout the world, affecting individuals of both genders and all races, although its prevalence varies widely across ethnic and racial groups. In recent years, advances in different disciplines, particularly biochemistry, genetics, immunology and molecular biology, have improved dramatically our understanding of the disease. Yet, the critical questions regarding who gets sarcoidosis and whether it has an infectious origin remain unanswered. Sarcoidosis has a distinguished medical history that covers the last 150 years. Right from the time of seminal contributions by Hutchinson, Besnier and Boeck medical discussion on sarcoidosis has always been animated and to a certain extent emotional. Such discussions will inevitable continue until the true cause of the disease has been found, hopefully in the near future.

  14. Ventricular fibrillation via torsade des pointes of cardiac sarcoidosis with preserved left ventricular ejection fraction.

    PubMed

    Sekihara, Takayuki; Nakane, Eisaku; Nakasone, Kazutaka; Inoko, Moriaki

    2016-10-25

    Generally, low left ventricular ejection fraction (LVEF) is a risk for ventricular arrhythmia in patients with cardiac sarcoidosis. We present a case of cardiac sarcoidosis with preserved LVEF that evoked ventricular fibrillation (VF). A 73-year-old woman with VF presented to our emergency department. She had a history of ocular sarcoidosis, with gradual thinning of the basal intraventricular septum. LVEF was 62% on the most recent echocardiography. The electrocardiogram after defibrillation showed complete atrioventricular block (CAVB) with QT segment prolongation and frequent ventricular premature beats. VF via torsade des pointes (TdP) was suspected, and temporary intravenous ventricular pacing and magnesium sulfate infusion suppressed her VF. Cardiac sarcoidosis was diagnosed, and an implantable cardioverter defibrillator was implanted. Patients with cardiac sarcoidosis with CAVB are at risk of evoking VF via TdP regardless of LVEF. If cardiac sarcoidosis is suspected, early diagnosis and risk stratification of ventricular arrhythmia are important. 2016 BMJ Publishing Group Ltd.

  15. Ichthyosiform sarcoidosis: report of three cases from Japan and literature review.

    PubMed

    Miura, Takako; Kato, Yasunobu; Yamamoto, Toshiyuki

    2016-12-23

    Cutaneous lesions of sarcoidosis present with various manifestations including specific and non-specific cutaneous lesions. Ichthyosiform sarcoidosis is a rare form of cutaneous sarcoidosis, presenting with asymptomatic, adherent, polygonal scales, mainly appearing on the lower limbs.  Ichthyosiform sarcoidosis has a predilection for dark-skinned races, and cases affecting Japanese patients have rarely been reported in English literature.  We herein describe three Japanese cases of ichthyosiform sarcoidosis on the lower limbs. All of the patients were female, with an age range of 57-69 years old.  Histologically, sarcoidal granulomas were located in the mid- to lower dermis.  All cases had scar sarcoidosis on the knees.  Furthermore, Case 1 presented with papular sarcoidosis on the back, and Case 3 presented with subcutaneous nodules on the buttock as well as erythema nodosum-like lesions on the lower legs.  All patients had lung sarcoidosis, but ocular sarcoidosis was seen in only Case 2. Case 3 showed Heerfordt syndrome with facial nerve paralysis. Histological features showed that the granular layers were scarcely detected in the overlying epidermis; however, filaggrin expression was not decreased.  Sarcoidal granulomas accumulated around the sweat glands in one case, whereas those features were not detected in the other two cases. In conclusion, ichthyosiform cutaneous sarcoidosis may be overlooked or misdiagnosed as xerotic dry skin which is frequently found in elderly people, and ichthyosiform cutaneous lesions may be more prevalent than previously estimated.

  16. [Cutaneous manifestations of sarcoidosis].

    PubMed

    Amschler, K; Seitz, C S

    2017-03-17

    Skin manifestations of sarcoidosis occur in up to 30% of cases. This review summarizes and illustrates in detail the differences between specific and unspecific skin manifestations of sarcoidosis. Important differential diagnoses, such as tuberculosis, cutaneous lymphoma and syphilis have to be excluded. The indications for systemic treatment are primarily determined by the extent of organ involvement and also by the cosmetic impairment.

  17. Pathogenesis of Sarcoidosis

    PubMed Central

    Gundy, Karl Van; Sharma, Om P.

    1987-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology. The organs that are involved by sarcoidosis include the lungs in which the granuloma is seen in more than 90% of patients to the pituitary, which is only rarely affected. There are many hypotheses as to the cause of sarcoidosis. Some of them rely on the similarities seen between sarcoidosis and the other granuloma-forming diseases such as tuberculosis, berylliosis, pine pollen inhalation and acute and chronic bacterial and viral infections, while others find similarities between sarcoidosis and immune reactions observed in autoimmune disorders. Still other explanations implicate a genetic predisposition or a still-unknown agent as the underlying cause of the granuloma formation. Images PMID:3310401

  18. Musculoskeletal involvement in sarcoidosis*, **

    PubMed Central

    Nessrine, Akasbi; Zahra, Abourazzak Fatima; Taoufik, Harzy

    2014-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It most commonly affects the pulmonary system but can also affect the musculoskeletal system, albeit less frequently. In patients with sarcoidosis, rheumatic involvement is polymorphic. It can be the presenting symptom of the disease or can appear during its progression. Articular involvement is dominated by nonspecific arthralgia, polyarthritis, and Löfgren's syndrome, which is defined as the presence of lung adenopathy, arthralgia (or arthritis), and erythema nodosum. Skeletal manifestations, especially dactylitis, appear mainly as complications of chronic, multiorgan sarcoidosis. Muscle involvement in sarcoidosis is rare and usually asymptomatic. The diagnosis of rheumatic sarcoidosis is based on X-ray findings and magnetic resonance imaging findings, although the definitive diagnosis is made by anatomopathological study of biopsy samples. Musculoskeletal involvement in sarcoidosis is generally relieved with nonsteroidal anti-inflammatory drugs or corticosteroids. In corticosteroid-resistant or -dependent forms of the disease, immunosuppressive therapy, such as treatment with methotrexate or anti-TNF-α, is employed. The aim of this review was to present an overview of the various types of osteoarticular and muscle involvement in sarcoidosis, focusing on their diagnosis and management. PMID:24831403

  19. How Is Sarcoidosis Diagnosed?

    MedlinePlus

    ... can show whether sarcoidosis is affecting your lungs. Biopsy Your doctor may do a biopsy to confirm a diagnosis or rule out other causes of your symptoms. A biopsy involves taking a small sample of tissue from ...

  20. Sarcoidosis-lymphoma syndrome.

    PubMed

    Brandy-García, Anahy M; Caminal-Montero, Luis; Fernández-García, María Soledad; Saiz Ayala, Angel; Cabezas-Rodríguez, Ivan; Morante-Bolado, Isla

    A 65 year-old female with a history of sarcoidosis with pulmonary and joint involvement, who after 5 years of diagnosis begins with central nervous system involvement manifesting as diplopia. She presents normal analysis results. In imaging results, a mass is identified in the right intraconal space; it depends of right optic nerve, and shows multiple lymph node involvement. Biopsy was performed diagnosed with large B-cell lymphoma, an atypical form of tumor associated with sarcoidosis.

  1. Sinonasal and laryngeal sarcoidosis.

    PubMed

    Edriss, Hawa; Kelley, John; Demke, Joshua

    2017-10-01

    Sarcoidosis is a chronic granulomatous inflammation of uncertain etiology that can involve any organ system in the body. Sinonasal and laryngeal involvement is rare, poorly understood, and difficult to diagnose. Additionally, the extent of the disease is variable, and the response to systemic corticosteroids is often poor. We report a case of a 55-year-old woman with prior cutaneous sarcoidosis who presented with chronic nasal congestion, difficulty breathing, dysphonia, and stridor, and biopsy of the nasal vestibule revealed noncaseating granulomatous inflammation.

  2. Genetic influences on sarcoidosis.

    PubMed

    Ishihara, M; Ohno, S

    1997-01-01

    To investigate the genetic influences underlying the development of sarcoidosis, HLA class II genotyping was performed in Japanese patients with sarcoidosis and healthy controls using the PCR-RFLP method. The frequencies of both DR52 group antigen-associated alleles (HLA-DRB1*11, -DRB1*12 and -DRB1*14) and DRB1*08 alleles were higher in the patient group, suggesting that the common, specific amino acid residue on the DRB1 molecule of these alleles may determine susceptibility to sarcoidosis. Alternatively, it is possible that another susceptibility gene, linked to these DRB1 alleles, exists within the MHC region. We screened the TNFA, TNFB, HSP70-1 and Hum70t genes around the class III region, as well as the HLA-DMA and -DMB genes in the class II region, for genetic polymorphism in sarcoidosis. None of these genes suggested a susceptibility to sarcoidosis. These studies support the thesis that one of the major genetic factors controlling the development of sarcoidosis is located within the DRB1 locus in the HLA class II region.

  3. Occupational Causes of Sarcoidosis

    PubMed Central

    Newman, Kira L.; Newman, Lee S.

    2012-01-01

    Purpose of review Sarcoidosis, the multiorgan, granulomatous disease of unknown etiology, remains mysterious. Several important investigations in the past two years add to accumulating evidence for both occupational and environmental causes of granulomatous inflammation. Recent findings This review considers the most recent studies that contribute to the hypothesis that sarcoidosis occurs when individuals are exposed to foreign antigens and to inorganic particulates that promote inflammation. Major recent findings, such as those emerging from the study of World Trade Center responders, the study of nanoparticles, and cases of work-associated sarcoidosis support the probability that occupational, as well as environmental, exposures to inflammatory stimuli trigger sarcoidosis-like illness. Major recent studies of microbially-rich indoor environments, including moldy indoor workplaces and mycobacterially-contaminated settings, contribute to the evidence that a variety of microbial antigens serve as targets for the hypersensitivity immune response in an inflammatory milieu. Summary There is increasing evidence that sarcoidosis can occur in workplace settings in which there is exposure to both foreign antigens and inorganic triggers of inflammation that promote an exuberant granulomatous immune response. It is likely that sarcoidosis has more than one cause. PMID:22314258

  4. Pleural sarcoidosis: a rare presentation

    PubMed Central

    Loughney, E.; Higgins, B. G.

    1997-01-01

    Sarcoidosis is a chronic disorder of unknown aetiology which causes tissue injury and granuloma formation in many organs. Although over 80% of cases have intrathoracic disease at presentation, pleural sarcoidosis remains an unusual manifestation. A case of sarcoidosis presenting with a discrete pleural mass is reported. 




 PMID:9059488

  5. Fatigue syndrome in sarcoidosis.

    PubMed

    Górski, Witold; Piotrowski, Wojciech J

    2016-01-01

    Sarcoidosis is an inflammatory disease of unknown etiology. Most commonly it results in the formation of non-caseating granulomas in intrathoracic lymph nodes and lung parenchyma, but the clinical course and picture may be complicated by extrapulmonary involvement and many non-respiratory signs and symptoms which are directly related to the disease. In addition, sarcoidosis patients may suffer from a plethora of symptoms of uncertain or unknown origin. Fatigue is one of these symptoms, and according to some authors it is reported by the majority of patients with active sarcoidosis, but also by a smaller proportion of patients with inactive sarcoidosis, or even with complete clinical and radiological remission. Therefore the term fatigue syndrome is frequently used to name this clinical problem. The definition of fatigue syndrome in sarcoidosis is imprecise and the syndrome is usually recognized by use of validated questionnaires. In this review the uptodate knowledge in this field was presented and different challenges connected with this syndrome were described.

  6. Nonsteroidal therapy of sarcoidosis

    PubMed Central

    Korsten, Peter; Mirsaeidi, Mehdi; Sweiss, Nadera J.

    2014-01-01

    Purpose of review None of the medications used in clinical practice to treat sarcoidosis have been approved by the regulatory authorities. Understanding how to use disease-modifying antisarcoid drugs, however, is essential for physicians treating patients with sarcoidosis. This review summarizes the recent studies of medications used for sarcoidosis with a focus on nonsteroidal therapies. Studies from 2006 to 2013 were considered for review to update clinicians on the most relevant literature published over the last few years. Recent findings Several recently published pieces of evidence have helped expand our ability to more appropriately sequence second-line and third-line therapies for sarcoidosis. For instance, methotrexate and azathioprine may be useful and well tolerated medications as second-line treatment. Mycophenolate mofetil might have a role in neurosarcoidosis. TNF-α blockers and other biologics seem to be well tolerated medications for the most severely affected patients. Summary Corticosteroids remain the first-line therapy for sarcoidosis as many patients never require treatment or only necessitate a short treatment duration. Second-line and third-line therapies described in this article should be used in patients with progressive or refractory disease or when life-threatening complications are evident at the time of presentation. PMID:23884295

  7. Chronic cutaneous lesions of sarcoidosis.

    PubMed

    Marchell, Richard M; Judson, Marc A

    2007-01-01

    Sarcoidosis involvement of the skin is common. The skin lesions of sarcoidosis may be nonspecific, showing a nondiagnostic inflammatory reaction pattern on histologic evaluation. Nonspecific skin lesions are often associated with an acute presentation of sarcoidosis and, in general, portend a good prognosis. Specific sarcoidosis skin lesions reveal typical sarcoid granulomas on histologic examination. These lesions tend to be chronic and require therapy for resolution. This article will review the epidemiology, diagnostic evaluation, and description of the various chronic skin lesions of sarcoidosis. Various images of these skin lesions will be demonstrated.

  8. [Sarcoidosis : Renal manifestations].

    PubMed

    Löffler, C; Bergner, R

    2017-04-12

    Renal involvement in sarcoidosis is much more common than generally assumed from old epidemiological studies and is often only detected when actively searched for. Many patients with renal sarcoidosis present with no or only few symptoms. The diagnostic work-up of sarcoidosis should always include a possible renal involvement. In cases of impaired renal function, proteinuria or a pathological urine sediment, a renal biopsy specimen should be obtained to assess the type, severity and prognosis of the kidney disease. Treatment is primarily based on the use of corticosteroids. Steroid-sparing agents, such as disease-modifying antirheumatic drugs and infliximab can be applied; however, the evidence for efficacy of these therapies is mostly based on case series and expert opinions. Discontinuation of immunosuppression therapy bears a high risk of relapse.

  9. Sarcoidosis: Rheumatology perspective.

    PubMed

    Yee, Arthur M F

    2016-04-01

    Sarcoidosis is a systemic inflammatory granulomatous disease for which rheumatologists are uniquely trained and qualified to treat. Historically, sarcoidosis has been managed within silos of medical subspecialties, but with increased appreciation of the systemic nature of this disorder and the availability of more therapeutic options, it is clear that a multidisciplinary approach, with the rheumatologist as a key component, can offer more optimal care. This manuscript reviews clinically relevant immunology and pathophysiology, diagnostic issues, management decision-making, and therapeutics in the care of patients with sarcoidosis. Issues particularly relevant to rheumatologists are highlighted. These include aiding in establishing diagnosis; recognition of disease manifestations involving bone, joint, and muscle; management of calcium metabolism and metabolic bone disease; and formulation and implementation of anti-inflammatory and immunomodulatory therapies.

  10. Bronchoscopic modalities to diagnose sarcoidosis.

    PubMed

    Benzaquen, Sadia; Aragaki-Nakahodo, Alejandro Adolfo

    2017-09-01

    Several studies have investigated different bronchoscopic techniques to obtain tissue diagnosis in patients with suspected sarcoidosis when the diagnosis cannot be based on clinicoradiographic findings alone. In this review, we will describe the most recent and relevant evidence from different bronchoscopic modalities to diagnose sarcoidosis. Despite multiple available bronchoscopic modalities to procure tissue samples to diagnose sarcoidosis, the vast majority of evidence favors endobronchial ultrasound transbronchial needle aspiration to diagnose Scadding stages 1 and 2 sarcoidosis. Transbronchial lung cryobiopsy is a new technique that is mainly used to aid in the diagnosis of undifferentiated interstitial lung disease; however, we will discuss its potential use in sarcoidosis. This review illustrates the limited information about the different bronchoscopic techniques to aid in the diagnosis of pulmonary sarcoidosis. However, it demonstrates that the combination of available bronchoscopic techniques increases the diagnostic yield for suspected sarcoidosis.

  11. Cryptococcal meningitis complicating sarcoidosis

    PubMed Central

    Leonhard, Sonja E.; Fritz, Daan; van de Beek, Diederik; Brouwer, Matthijs C.

    2016-01-01

    Abstract Background: Cryptococcal meningitis is an uncommon but severe complication of sarcoidosis. Methods: We present 2 patients with cryptococcal meningitis complicating sarcoidosis and compared findings with 38 cases reported in the literature. Results: When analyzing our patients and 38 cases reported in the literature, we found that median age of sarcoidosis patients with cryptococcal meningitis was 39 years (range 30–48); 27 of 33 reported cases (82%) had a history of sarcoidosis. Only 16 of 40 patients (40%) received immunomodulating therapy at the time of diagnosis of cryptococcal meningitis. The diagnosis of cryptococcal meningitis was delayed in 17 of 40 patients (43%), mainly because of the initial suspicion of neurosarcoidosis. Cerebrospinal fluid (CSF) examination showed mildly elevated white blood cell count (range 23–129/mm3). Twenty-nine of 32 cases (91%) had a positive CSF culture for Cryptococcus neoformans and 25 of 27 cases (93%) had a positive CSF C neoformans antigen test. CD4 counts were low in all patients in whom counts were performed (84–228/mL). Twelve patients had an unfavorable outcome (32%), of which 7 died (19%) and 24 patients (65%) had a favorable outcome. The rate of unfavorable outcome in patients with a delayed diagnosis was 7 of 17 (41%) compared to 5 of 28 (21%) in patients in whom diagnosis was not delayed. Conclusion: Cryptococcal meningitis is a rare but life-threatening complication of sarcoidosis. Patients were often initially misdiagnosed as neurosarcoidosis, which resulted in considerable treatment delay and worse outcome. CSF cryptococcal antigen tests are advised in patients with sarcoidosis and meningitis. PMID:27583871

  12. Cryptococcal meningitis complicating sarcoidosis.

    PubMed

    Leonhard, Sonja E; Fritz, Daan; van de Beek, Diederik; Brouwer, Matthijs C

    2016-08-01

    Cryptococcal meningitis is an uncommon but severe complication of sarcoidosis. We present 2 patients with cryptococcal meningitis complicating sarcoidosis and compared findings with 38 cases reported in the literature. When analyzing our patients and 38 cases reported in the literature, we found that median age of sarcoidosis patients with cryptococcal meningitis was 39 years (range 30-48); 27 of 33 reported cases (82%) had a history of sarcoidosis. Only 16 of 40 patients (40%) received immunomodulating therapy at the time of diagnosis of cryptococcal meningitis. The diagnosis of cryptococcal meningitis was delayed in 17 of 40 patients (43%), mainly because of the initial suspicion of neurosarcoidosis. Cerebrospinal fluid (CSF) examination showed mildly elevated white blood cell count (range 23-129/mm). Twenty-nine of 32 cases (91%) had a positive CSF culture for Cryptococcus neoformans and 25 of 27 cases (93%) had a positive CSF C neoformans antigen test. CD4 counts were low in all patients in whom counts were performed (84-228/mL). Twelve patients had an unfavorable outcome (32%), of which 7 died (19%) and 24 patients (65%) had a favorable outcome. The rate of unfavorable outcome in patients with a delayed diagnosis was 7 of 17 (41%) compared to 5 of 28 (21%) in patients in whom diagnosis was not delayed. Cryptococcal meningitis is a rare but life-threatening complication of sarcoidosis. Patients were often initially misdiagnosed as neurosarcoidosis, which resulted in considerable treatment delay and worse outcome. CSF cryptococcal antigen tests are advised in patients with sarcoidosis and meningitis.

  13. [Physiopathology of sarcoidosis].

    PubMed

    Soler, Paul

    2008-05-31

    Sarcoidosis results from an uncontrolled cell-mediated immune reaction involving activated T cells and monocytes-macrophages, characterized by the formation of typical granulomas at the sites of the lesions. The interactions between both cell types induce the release of several mediators that play a central role in the development of granulomas, allowing to design new therapies. The aetiology of sarcoidosis remains unknown, but the disease is currently viewed as the consequence of a chronic immunological response associating a genetic susceptibility and specific environmental factors.

  14. Sarcoidosis Presenting Addison's Disease.

    PubMed

    Takahashi, Kentaro; Kagami, Shin-Ichiro; Kawashima, Hirotoshi; Kashiwakuma, Daisuke; Suzuki, Yoshio; Iwamoto, Itsuo

    2016-01-01

    We herein describe a second Japanese case of sarcoidosis presenting Addison's disease. A 52-year-old man was diagnosed with sarcoidosis based on clinical and laboratory findings, including bilateral hilar lymphadenopathy and elevated levels of serum angiotensin-converting enzyme and lysozyme, as well as the presence of noncaseating epithelioid granulomas. The patient also exhibited general fatigue, pigmentation, weight loss, hypotension and hyponatremia, suggestive of chronic adrenocortical insufficiency. An endocrine examination confirmed primary adrenocortical insufficiency. This case suggests the direct involvement of sarcoid granuloma in the adrenal glands.

  15. Pulmonary Hypertension in Sarcoidosis.

    PubMed

    Baughman, Robert P; Engel, Peter J; Nathan, Steven

    2015-12-01

    Pulmonary hypertension is a complication of sarcoidosis leading to dyspnea and associated with increased morbidity and mortality. Sarcoidosis-associated pulmonary hypertension (SAPH) can be due to several factors, including vascular involvement by the granulomatous inflammation, compression of the pulmonary arteries by adenopathy, fibrotic changes within the lung, and left ventricular diastolic dysfunction. Several case series have suggested that some patients with SAPH benefit from specific therapy for pulmonary hypertension. A randomized, placebo-controlled trial found 16 weeks' bosentan therapy to be associated with significant improvement in pulmonary artery pressure. Future studies may better define who would respond to treatment of pulmonary hypertension.

  16. Who Is at Risk for Sarcoidosis?

    MedlinePlus

    ... NHLBI on Twitter. Who Is at Risk for Sarcoidosis? Sarcoidosis affects people of all ages and races. ... Content: NEXT >> Featured Video Living With and Managing Sarcoidosis 05/18/2011 This video—presented by the ...

  17. Round atelectasis in sarcoidosis

    PubMed Central

    2011-01-01

    Round atelectasis is a benign inflammatory condition most frequently observed in patients with asbestos exposure but it can also result from a variety of chronic pleural diseases like infection. It has not previously been described in sarcoidosis. We report the occurrence of round atelectasis in four previously diagnosed sarcoidosis patients who were under follow up at our outpatient clinic. Three patients had symptoms consisting of thoracic pain, dry cough and sensation of fullness at the posterior thorax, respectively. Chest roentgenogram showed subpleural or pleural based opacity with diameters ranging from 2 to 3 cm in each of the patients. Chest computerized tomography (CT) revealed features of round atelectasis. Fiberoptic bronchoscopy with transbronchial lung biopsy was performed. Diagnosis was confirmed by the histopathologic examination of the biopsy samples. The mechanical influence of a prior pleural effusion due to sarcoidosis may be the predominant mechanism underlying the onset of round atelectasis in these patients. Clinicians should bear in mind the possibility of sarcoidosis as an etiologic factor for round atelectasis. PMID:22958304

  18. Round atelectasis in sarcoidosis.

    PubMed

    Tetikkurt, Cuneyt; Tetikkurt, Seza; Ozdemir, Imran; Bayar, Nihal

    2011-06-30

    Round atelectasis is a benign inflammatory condition most frequently observed in patients with asbestos exposure but it can also result from a variety of chronic pleural diseases like infection. It has not previously been described in sarcoidosis. We report the occurrence of round atelectasis in four previously diagnosed sarcoidosis patients who were under follow up at our outpatient clinic. Three patients had symptoms consisting of thoracic pain, dry cough and sensation of fullness at the posterior thorax, respectively. Chest roentgenogram showed subpleural or pleural based opacity with diameters ranging from 2 to 3 cm in each of the patients. Chest computerized tomography (CT) revealed features of round atelectasis. Fiberoptic bronchoscopy with transbronchial lung biopsy was performed. Diagnosis was confirmed by the histopathologic examination of the biopsy samples. The mechanical influence of a prior pleural effusion due to sarcoidosis may be the predominant mechanism underlying the onset of round atelectasis in these patients. Clinicians should bear in mind the possibility of sarcoidosis as an etiologic factor for round atelectasis.

  19. Evidence-based therapy for cutaneous sarcoidosis.

    PubMed

    Doherty, Christy B; Rosen, Ted

    2008-01-01

    Although healthcare providers have arrived at a relatively comfortable zone of accepted clinical practice in the management of cutaneous sarcoidosis, virtually every treatment is based on minimal evidence-based data and relies almost exclusively on anecdotal information. Although it would be convenient to blame this state of affairs on the lack of certainty about disease aetiology, the unavoidable fact is that little has been executed, even in the realm of well designed comparative trials. Nonetheless, worldwide accepted standard therapies for sarcoidosis include the administration of corticosteroids, antimalarials and methotrexate. A stepwise approach to patient care is appropriate, and potent topical corticosteroids (e.g. clobetasol) or repeated intralesional injections of triamcinolone (3-10 mg/mL) may be all that is needed in mild skin-limited disease. In patients requiring systemic therapy for recalcitrant or deforming skin lesions (or for widespread disease), corticosteroids (e.g. prednisone 40-80 mg/day, tapered accordingly) used alone or in combination with antimalarials or methotrexate may be indicated. Antimalarials and methotrexate are considered second-line interventions and may be used as monotherapy for steroid-resistant sarcoidosis or in patients unable to tolerate steroids. Given the concern regarding ocular toxicity, the maximum dosages of chloroquine and hydroxychloroquine should not exceed 3.5 and 6.5 mg/kg/day, respectively. Methotrexate is given in weekly doses of 10-30 mg, with the caveat that haematological, gastrointestinal, pulmonary and hepatic toxicities are possible. Despite universal acceptance as standard care, the aforementioned treatments often result in an incomplete clinical response or unacceptable adverse events. In such situations, more innovative treatment options may be used. Treatments that may well gain widespread future use include the tumour necrosis factor-alpha inhibitors infliximab and adalimumab. Experience is limited

  20. Sarcoidosis: Immunopathogenesis and Immunological Markers

    PubMed Central

    Loke, Wei Sheng Joshua; Herbert, Cristan; Thomas, Paul S.

    2013-01-01

    Sarcoidosis is a multisystem granulomatous disorder invariably affecting the lungs. It is a disease with noteworthy variations in clinical manifestation and disease outcome and has been described as an “immune paradox” with peripheral anergy despite exaggerated inflammation at disease sites. Despite extensive research, sarcoidosis remains a disease with undetermined aetiology. Current evidence supports the notion that the immune response in sarcoidosis is driven by a putative antigen in a genetically susceptible individual. Unfortunately, there currently exists no reliable biomarker to delineate the disease severity and prognosis. As such, the diagnosis of sarcoidosis remains a vexing clinical challenge. In this review, we outline the immunological features of sarcoidosis, discuss the evidence for and against various candidate etiological agents (infective and noninfective), describe the exhaled breath condensate, a novel method of identifying immunological biomarkers, and suggest other possible immunological biomarkers to better characterise the immunopathogenesis of sarcoidosis. PMID:26464848

  1. Infrequent cardiac manifestations of sarcoidosis.

    PubMed

    Ashraf, Said; Briasoulis, Alexandros; Afonso, Luis

    Cardiac sarcoidosis (CS) is found in 2-7% of patients with systemic sarcoidosis (SS). Its diagnosis and treatment is challenging, notwithstanding the poor prognosis and treatment. Hereby, we present a case of systemic sarcoidosis with rare cardiac manifestations of severe mitral incompetence and large coronary aneurysm in a previously healthy woman. She underwent successful mitral valve replacement and coronary artery bypass surgery and was maintained on low dose glucocorticoid therapy. Copyright © 2016. Published by Elsevier Inc.

  2. Extensive upper respiratory tract sarcoidosis.

    PubMed

    Soares, Mafalda Trindade; Sousa, Carolina; Garanito, Luísa; Freire, Filipe

    2016-04-18

    Sarcoidosis is a chronic granulomatous disease of unknown aetiology. It can affect any part of the organism, although the lung is the most frequently affected organ. Upper airway involvement is rare, particularly if isolated. Sarcoidosis is a diagnosis of exclusion, established by histological evidence of non-caseating granulomas and the absence of other granulomatous diseases. The authors report a case of a man with sarcoidosis manifesting as a chronic inflammatory stenotic condition of the upper respiratory tract and trachea.

  3. Ninth International Conference on Sarcoidosis.

    DTIC Science & Technology

    1981-10-15

    AC-AIOb 379 OFFICE OF NAVAL RESEARCH LONDON (ENGLAND) F/S 6/5 NINTH INTERNATIONAL CONFERENCE ON SARCOIDOSIS .(U) OCT 81 1 C ROSE UNCLASSIFIED ONRLC...INTERNATIONAL CONFERENCE ON SARCOIDOSIS S. PERFORMING ORG. REPORT NUMBER 7. AUTHOR(e) I. CONTRACT OR GRANT NUMBER(a) JOHN C. ROSE 9. PERFORMING...SUPPLEMENTARY NOTES 19. KEY WORDS (Continue on reverse side if necesery mnd identify by block nuatbor) ACE lymphocytes alveolitis sarcoidosis

  4. Coccidioidomycosis A Cause of Sarcoidosis

    PubMed Central

    Yourison, Isaac

    2017-01-01

    Abstract Background. Sarcoidosis is a granulomatous disease of unknown etiology and coccidioidomycosis is a granulomatous fungal infection endemic to the southwestern United States. Clinical observations on patients with sarcoidosis and coccidioidomycosis simultaneously led to the hypothesis that sarcoidosis can be caused by the fungus Coccidioides. Methods. Two patients with sarcoidosis and coccidioidomycosis were studied, one prospectively (ie, a patient with sarcoidosis was predicted to develop coccidioidomycosis) and the other retrospectively (ie, a patient with coccidioidomycosis develops sarcoidosis). In addition, a literature review found 5 patients with these 2 diseases, and they were analyzed to establish an archived relationship between the 2 entities. In addition, polymerase chain reaction (PCR) testing for Coccidioides deoxyribonucleic acid (DNA) was performed on tissue from 15 Arizona patients diagnosed with sarcoidosis. Results. Patient 1 was diagnosed with sarcoid in Arizona and followed prospectively. There was no evidence for coccidioidomycosis at diagnosis. This patient was observed for 8 years before he was documented to have disseminated coccidioidomycosis. Patient 2 was retrospectively studied. He was diagnosed with disseminated coccidioidomycosis, but after 3 years, while on antifungal therapy, he developed respiratory failure. A lung biopsy was consistent with sarcoidosis with no evidence of Coccidioides; cultures, histopathology, and serology were all negative. Paradoxically, PCR testing for Coccidioides DNA on tissue taken at the time of the sarcoidosis diagnosis were all negative, including Patient 2 who had proven coccidioidomycosis. Conclusions. The 2 study patients and 5 case reports from the literature support the hypothesis that Coccidioides can cause sarcoidosis in Arizona. There are similar reports in the literature that Histoplasma can be associated with sarcoidosis. Based on these observations, we propose that sarcoidosis can be

  5. [Thyroid involvement of sarcoidosis].

    PubMed

    Uto, Tomohiro; Inui, Naoki; Miyazaki, Hiroo; Matsushima, Sayomi; Kuroishi, Shigeki; Hashimoto, Dai; Naito, Tateaki; Nakamura, Yutaro; Sato, Jun; Suda, Takafumi; Chida, Kingo

    2008-08-01

    A 54-year-old woman was found to have abnormal shadows on her chest radiograph taken on an annual medical examination. The chest radiograph showed multiple nodules in the bilateral middle and lower lung fields accompanied with bilateral hilar lymphadenopathy. A computed tomography of the neck and chest revealed nodules in her right middle lobe and bilateral lower lobes with an enlarged thyroid. A metastatic malignant disease involving both thyroid and lungs was suspected, therefore thyroid and lung biopsies were performed. The histological examination of the thyroid and the lung specimens revealed non-caseating epithelioid cell granulomas which were compatible with sarcoidosis. Although the thyroid involvement of sarcoidosis is rare, it should be included in the differential diagnosis with patients with thyroid swelling.

  6. Cough in Sarcoidosis.

    PubMed

    Tully, Timothy; Birring, Surinder S

    2016-02-01

    Sarcoidosis is a granulomatous disorder that frequently affects the lungs. Cough is commonly reported by patients and can significantly reduce health-related quality of life. The mechanism of cough is unknown but airway inflammation, mechanical distortion from pulmonary fibrosis and disruption of the vagus nerve are possible. Recent evidence suggests cough reflex hypersensitivity may also be an important mechanism and predictor of the frequency of cough. The investigation of cough should evaluate common causes such as asthma, gastro-oesophageal reflux and rhinitis. In patients with suspected cough due to sarcoidosis, a trial of corticosteroids should be considered. The severity of cough should be evaluated with validated outcome measures such as visual analogue scales, cough severity diary, health-related quality of life questionnaires such as the Leicester Cough Questionnaire and objective cough monitors. Future studies are needed to identify targets for therapeutic development.

  7. Sarcoidosis: An unusual presentation.

    PubMed

    Madureira, Pedro; Pimenta, Sofia; Cardoso, Hélder; Guimarães Cunha, Rui; Costa, Lúcia

    A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis. Lab tests revealed increased liver enzymes. Liver MRI evidenced an enlarged liver and retroperitoneal lymphadenopathy. Histological analysis of the finger skin, lymph nodes and liver demonstrated the presence of granulomas, confirming the diagnosis of sarcoidosis. The patient started prednisolone with rapid improvement of the symptoms. Skin lesions are divided into two groups: specific for sarcoidosis (with granulomas, lupus pernio-like) and nonspecific (without granulomas, erythema nodosum-like). Specific cutaneous lesions usually cause no other symptoms beyond cosmetic changes. Lupus pernio stands out for having distinctive features but, to the best of our knowledge, the simultaneous involvement of both hands and feet has never been reported. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  8. Morbidity and Mortality in Sarcoidosis

    PubMed Central

    Gerke, Alicia K.

    2015-01-01

    Purpose of Review Chronic sarcoidosis is a complex disease with numerous comorbid conditions and can be fatal in some cases. Recognizing causes of morbidity and mortality is important to effectively select treatments, manage symptoms, and improve outcomes. The purpose of this review is to examine emerging knowledge on morbidity and mortality in sarcoidosis. Recent Findings Approximately one to five percent of patients with sarcoidosis die from complications of sarcoidosis. Recent population studies indicate that mortality may be increasing over the past decade. The reasons behind these trends are unclear, but could include increasing incidence, detection rates, severity of disease, or age of the population. Morbidity of sarcoidosis is reflected by a trend of increased hospitalizations over recent years and increased use of healthcare resources. Morbidity can be caused by organ damage from granulomatous inflammation, treatment complications, and psychosocial effects of the disease. Recent studies are focused on morbidity related to cardiopulmonary complications, bone health, and aging within the sarcoidosis population. Last, sarcoidosis is associated with autoimmune diseases, pulmonary embolism, and malignancy; however, the underlying mechanisms linking diseases continue to be debated. Summary Morbidity in sarcoidosis is significant and multifactorial. Mortality is infrequent, but may be increasing over the years. PMID:25029298

  9. [Cardiac sarcoidosis - clinical manifestation and diagnosis].

    PubMed

    Błaut-Jurkowska, Justyna; Podolec, Piotr; Olszowska, Maria

    2016-08-01

    Sarcoidosis is a multisystem inflammatory disease defined histologically by the formation of noncaseating granulomas. The etiology of sarcoidosis remains unknown. Heart involvement in the course of sarcoidosis concerns about 5% of patients. The most common manifestation of cardiac sarcoidosis are conduction abnormalities, arrhythmias and heart failure. The diagnostic algorithm includes performing a clinical history, a 12-lead electrocardiogram (ECG) and an echocardiogram. If any of the initial screening investigations yields an abnormality, diagnostics should be continue using advanced imaging techniques: cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Nowadays endomyocardial biopsy is not performed routinely.The clinical picture of cardiac sarcoidosis is highly variable. Screening for cardiac sarcoidosis should be performed in all patients diagnosed with extracardiac sarcoidosis. Cardiac sarcoidosis should also be suspected in young patients without a diagnosis of sarcoidosis who present with conduction abnormalities of unknown etiology, because cardiac sarcoidosis may be the first or the only manifestation of the disease. © 2016 MEDPRESS.

  10. Pharmacotherapeutic management of pulmonary sarcoidosis.

    PubMed

    Fazzi, Piera

    2003-01-01

    Corticosteroids are the mainstay of treatment for sarcoidosis. Although the indications for medical therapy of sarcoidosis are controversial, standard therapy for symptomatic, progressive disease consists of corticosteroids. The British Thoracic Society concluded, with respect to systemic corticosteroids for the treatment of sarcoidosis, that some patients required no treatment, some required prednisone for control of symptoms, and others, with persistent disease, appeared to benefit from long-term corticosteroid therapy. Inhaled budesonide can be an effective treatment for lung sarcoidosis, with few adverse effects, when used in combination with oral systemic corticosteroids such as deflazacort administered in a tapered regimen for 6 months. A randomized controlled trial has also demonstrated the efficacy of 3 months of treatment with oral prednisolone in a tapered regimen followed by inhaled budesonide for 15 months in patients with early stage pulmonary sarcoidosis.Alternative drugs are required in chronic resistant sarcoidosis and/or in conditions where systemic corticosteroids are contraindicated. Immunosuppressive agents (chlorambucil, cyclophosphamide, methotrexate, cyclosporine, azathioprine), anticytokine agents (thalidomide, pentoxifylline), antimalarials (chloroquine, hydroxychloroquine), melatonin and monoclonal antibody (infliximab) have been used in such situations. Chlorambucil and cyclophosphamide have been used in anecdotal cases of pulmonary sarcoidosis as corticosteroid-sparing agents. However, their toxicity and neoplastic potential recommend prudence in patient selection. A comparison between combination therapy with cyclosporine and prednisone and prednisone alone has shown an increased prevalence of serious adverse effects with combined therapy with no between-group differences in treatment efficacy. The cost and toxicity of cyclosporine limit its use to patients in whom its efficacy has been proven. In patients with chronic or refractory

  11. Evidence for Mycobacteria in Sarcoidosis

    PubMed Central

    Brownell, Isaac; Ramírez-Valle, Francisco; Sanchez, Miguel

    2011-01-01

    Despite its recognition as a distinct granulomatous disease for over a century, the etiology of sarcoidosis remains to be defined. Since the early 1900s, infectious agents have been suspected in causing sarcoidosis. For much of this time, mycobacteria were considered a likely culprit, yet until recently, the supporting evidence has been tenuous at best. In this review, we evaluate the reported association between mycobacteria and sarcoidosis. Historically, mycobacterial infection has been investigated using histologic stains, cultures of lesional tissue or blood, and identification of bacterial nucleic acids or bacterial antigens. More recently, advances in biochemical, molecular, and immunological methods have produced a more rigorous analysis of the antigenic drivers of sarcoidosis. The result of these efforts indicates that mycobacterial products likely play a role in at least a subset of sarcoidosis cases. This information, coupled with a better understanding of genetic susceptibility to this complex disease, has therapeutic implications. PMID:21659662

  12. Bone and bone marrow involvement in sarcoidosis.

    PubMed

    Yachoui, Ralph; Parker, Brian J; Nguyen, Thanhcuong T

    2015-11-01

    Bone and bone marrow involvement in sarcoidosis have been infrequently reported. We aimed to describe the clinical features, radiological descriptions, pathological examinations, and outcomes of three patients with osseous sarcoidosis and one patient with bone marrow sarcoidosis seen at our institution. Our case series included fluorodeoxyglucose positron emission tomography descriptions in assessing the whole-body extent of sarcoidosis. In the era of advanced imaging, large bone and axial skeleton sarcoidosis lesions are more common than previously reported.

  13. Digestive-tract sarcoidosis

    PubMed Central

    Ghrenassia, Etienne; Mekinian, Arsene; Chapelon-Albric, Catherine; Levy, Pierre; Cosnes, Jacques; Sève, Pascal; Lefèvre, Guillaume; Dhôte, Robin; Launay, David; Prendki, Virginie; Morell-Dubois, Sandrine; Sadoun, Danielle; Mehdaoui, Anas; Soussan, Michael; Bourrier, Anne; Ricard, Laure; Benamouzig, Robert; Valeyre, Dominique; Fain, Olivier

    2016-01-01

    Abstract Digestive tract sarcoidosis (DTS) is rare and case-series are lacking. In this retrospective case–control study, we aimed to compare the characteristics, outcome, and treatment of patients with DTS, nondigestive tract sarcoidosis (NDTS), and Crohn disease. We included cases of confirmed sarcoidosis, symptomatic digestive tract involvement, and noncaseating granuloma in any digestive tract. Each case was compared with 2 controls with sarcoidoisis without digestive tract involvement and 4 with Crohn disease. We compared 25 cases of DTS to 50 controls with NDTS and 100 controls with Crohn disease. The major digestive clinical features were abdominal pain (56%), weight loss (52%), nausea/vomiting (48%), diarrhea (32%), and digestive bleeding (28%). On endoscopy of DTS, macroscopic lesions were observed in the esophagus (9%), stomach (78%), duodenum (9%), colon, (25%) and rectum (19%). As compared with NDTS, DTS was associated with weight loss (odds ratio [OR] 5.8; 95% confidence interval [CI] 1.44–23.3) and the absence of thoracic adenopathy (OR 5.0; 95% CI 1.03–25). As compared with Crohn disease, DTS was associated with Afro-Caribbean origin (OR 27; 95% CI 3.6–204) and the absence of ileum or colon macroscopic lesions (OR 62.5; 95% CI 10.3–500). On the last follow-up, patients with DTS showed no need for surgery (versus 31% for patients with Crohn disease; P = 0.0013), and clinical digestive remission was frequent (76% vs. 35% for patients with Crohn disease; P = 0.0002). The differential diagnosis with Crohn disease could be an issue with DTS. Nevertheless, the 2 diseases often have different clinical presentation and outcome. PMID:27442665

  14. Acute pleurisy in sarcoidosis.

    PubMed Central

    Gardiner, I T; Uff, J S

    1978-01-01

    A 47-year-old white man with sarcoidosis presented with a six-week history of acute painful pleurisy. On auscultation a loud pleural rub was heard at the left base together with bilateral basal crepitations. The chest radiograph showed hilar enlargement as well as diffuse lung shadowing. A lung biopsy showed the presence of numerous epithelioid and giant-cell granulomata, particularly subpleurally. A patchy interstitial pneumonia was also present. He was given a six-month course of prednisolone, and lung function returned to normal. Images PMID:644534

  15. Calcium metabolism in sarcoidosis

    PubMed Central

    Dent, C. E.

    1970-01-01

    Fifteen patients with hypercalcaemia and sarcoidosis have been studied. Twelve were males of average age 34; the three females averaged 53. Cases included widely varying sarcoid manifestations, but five gave a clear history of rather excessive ingestion of low-dosage vitamin D preparations. The data confirm that in most cases there is an undue sensitivity to all the actions of vitamin D, the situation therefore mimicking vitamin D intoxication. Two patients volunteered to receive ultra-violet irradiation and became hypercalcaemic with corresponding clinical and biochemical changes. Steroids make normal the calcium abnormalities just as they do in straight vitamin D intoxication. However, in three further patients the hypercalcaemia did not respond to steroids and was shown to be due to the presence of an over-acting parathyroid gland, removal of which corrected the abnormality. There are a sufficient number of other similar cases in the literature to suggest that the development of parathyroid adenomas is another even rarer complication of sarcoidosis which must be carefully distinguished from vitamin D sensitivity. PMID:5481094

  16. Diagnostic criteria for sarcoidosis.

    PubMed

    Heinle, Robert; Chang, Christopher

    2014-01-01

    Sarcoidosis is a multiorgan system disease that often presents insidiously. The diagnosis is often made fortuitously upon routine chest radiography or that done for other reasons. Blacks are more commonly affected than whites and age of onset is typically adolescents to young adults. Lung involvement is common and symptoms may include cough, dyspnea and chest pain. Extrapulmonary symptoms may include the skin, joint and eye findings. Bilateral hilar adenopathy is the classic finding on chest radiograph. Anemia or other cell line deficiencies, elevated liver enzymes, hypercalciuria, and EKG abnormalities may also be present. Angiotensin converting enzyme levels may be elevated but are not diagnostic. Histopathological confirmation of noncaseating granulomas is essential for diagnosis. It is generally performed through a biopsy of the most peripheral site possible, although transbronchial biopsy is commonly required. Finally, other possible etiologies must be evaluated and differentiated with a particular emphasis on tuberculosis due to the multiple overlapping symptoms and findings. Newer techniques such as proteomics and transcriptional gene signatures may contribute to the understanding of the pathophysiology of sarcoidosis, and may even serve as diagnostic tools in the future. Copyright © 2014 Elsevier B.V. All rights reserved.

  17. Appendicular sarcoidosis mimicking acute appendicitis

    PubMed Central

    Hunjan, Tia; Chaudery, Muzzafer; Zaidi, Ahsan; Beggs, Andrew D

    2012-01-01

    Appendicular sarcoidosis is a very rare cause of acute abdominal pain, with only seven cases reported previously in the literature. A 45-year-old woman, known to have sarcoidosis, presented to the emergency department with a 1-week history of epigastric and right iliac fossa abdominal pain. At diagnostic laparoscopy, an acutely inflamed appendix was found and removed as well as an omental mass which was biopsied. Subsequent histopathological examination of the appendix demonstrated appendicular sarcoidosis without acute appendicitis and chronic inflammatory changes in the omental biopsy. The patients’ symptoms completely resolved postoperatively. It is important to undertake urgent operative intervention in patients with sarcoidosis who present with right iliac fossa pain, owing to the high risk of perforation. PMID:23162022

  18. Appendicular sarcoidosis mimicking acute appendicitis.

    PubMed

    Hunjan, Tia; Chaudery, Muzzafer; Zaidi, Ahsan; Beggs, Andrew D

    2012-11-15

    Appendicular sarcoidosis is a very rare cause of acute abdominal pain, with only seven cases reported previously in the literature. A 45-year-old woman, known to have sarcoidosis, presented to the emergency department with a 1-week history of epigastric and right iliac fossa abdominal pain. At diagnostic laparoscopy, an acutely inflamed appendix was found and removed as well as an omental mass which was biopsied. Subsequent histopathological examination of the appendix demonstrated appendicular sarcoidosis without acute appendicitis and chronic inflammatory changes in the omental biopsy. The patients' symptoms completely resolved postoperatively. It is important to undertake urgent operative intervention in patients with sarcoidosis who present with right iliac fossa pain, owing to the high risk of perforation.

  19. [Exercise capacity in patients with pulmonary sarcoidosis].

    PubMed

    Błaut-Jurkowska, Justyna; Knap, Klaudia; Kaźnica-Wiatr, Magdalena; Żygadło, Agnieszka; Tomkiewicz-Pająk, Lidia; Podolec, Piotr; Olszowska, Maria

    2017-08-21

    Exertional dyspnea is a common manifestation of sarcoidosis. Cardiopulmonary exercise testing (CPET) is a useful tool to evaluate exercise tolerance of sarcoid patients. The aim of the study was to evaluate of exercise capacity in patients with pulmonary sarcoidosis with regard to duration of the disease. Analysis of differences in physical tolerance between patients with cardiac sarcoidosis and without cardiac sarcoidosis. 39 patients diagnosed with pulmonary sarcoidosis were enrolled to our study. Cardiopulmonary exercise test was used to assess exercise capacity. According to time passed from diagnosis of sarcoidosis patients were grouped into 2 groups. Cardiac sarcoidosis was diagnosed in 9 subjects (23.07%). The control group consisted of 33 healthy volunteers. The results of the ergospirometry test in patients with lung sarcoidosis showed statistically significant differences in comparison to the control group. The age of patients with pulmonary sarcoidosis correlated negatively with maximal heart rate, oxygen consumption at peak exercise and at anaerobic threshold, breathing frequency at peak exercise, minute ventilation at peak exercise and metabolic equivalent. There was a negative, statistically significant correlation between the oxygen pulse at the peak of exercise and the treatment of steroids in the past. Exercise limitation in patients with pulmonary sarcoidosis is a consequence of both ventilatory and cardiocirculatory impairment. Patients with longer disease history of sarcoidosis achieved worse results at cardiopulmonary exercise test than patients with shorter disease history. Trend towards worse exercise tolerance in patients with cardiac sarcoidosis compared to patients without cardiac sarcoidosis was observed.

  20. Talc granulomatosis mimicking sarcoidosis.

    PubMed

    Iqbal, A; Aggarwal, B; Menon, B; Kulshreshtha, R

    2008-07-01

    Pulmonary disease due to talc, a group of hydrous magnesium silicates, is almost exclusively encountered secondary to occupational exposure or intravenous drug abuse. Talcosis or talc pneumoconiosis is one of the rarer forms of silicate-induced lung disease. It is seen in workers exposed during its production, and occasionally, in users of cosmetic talc and in intravenous drug addicts. Very often, the history of exposure is not recognised by the patient, and it is only the finding of granulomatous cellular interstitial lesions containing birefringent crystals which indicates considerable talc exposure. We report a 38-year-old woman who was initially diagnosed with sarcoidosis, until a bronchoscopic biopsy revealed the presence of numerous foreign body giant cells and birefringent particles forming non-caseating granulomas. There was no history of occupational exposure to talc or intravenous drug abuse. The patient responded to oral corticosteroid treatment. Talcosis is generally considered to be relatively benign.

  1. Sarcoidosis and Thyroid Autoimmunity.

    PubMed

    Fazzi, Piera; Fallahi, Poupak; Ferrari, Silvia Martina

    2017-01-01

    Most of the studies have shown a higher risk for subclinical and clinical hypothyroidism, antithyroid autoantibodies [overall antithyroid peroxidase antibodies (TPOAb)], and in general, thyroid autoimmunity, overall in the female gender in patients with sarcoidosis (S). A significantly higher prevalence of clinical hypothyroidism and Graves' disease was also described in female S patients with respect to controls. Gallium-67 (Ga-67) scyntigraphy in S patients, in the case of thyroid uptake, suggests the presence of aggressive autoimmune thyroiditis and hypothyroidism. For this reason, ultrasonography and thyroid function should be done in the case of Ga-67 thyroid uptake. In conclusion, thyroid function, TPOAb measurement, and ultrasonography should be done to assess the clinical profile in female S patients, and the ones at high risk (female individuals, with TPOAb positivity, and hypoechoic and small thyroid) should have periodically thyroid function evaluations and suitable treatments.

  2. Sarcoidosis presenting with multiple tattoo granulomata

    PubMed Central

    Iveson, J. M. I.; Cotterill, J. A.; Wright, V.

    1975-01-01

    A patient with sarcoidosis, multiple tattoo granulomata and arthritis is described. A combination of tattoo granulomata unrelated to any one colour in the tattoo and granulomatous inflammation elsewhere is indicative of sarcoidosis. ImagesFig. 1Fig. 2 PMID:1197172

  3. Development of minimal-change glomerular disease and Hashimoto's thyroiditis during the treatment of sarcoidosis.

    PubMed

    Ando, Fumiaki; Okado, Tomokazu; Sohara, Eisei; Rai, Tatemitsu; Uchida, Shinichi; Sasaki, Sei

    2013-11-01

    Minimal-change glomerular disease, sarcoidosis and autoimmune thyroid disease rarely occur in the same patient. We herein report a patient in which minimal-change glomerular disease and Hashimoto's thyroiditis developed during the treatment of sarcoidosis with steroids. A 66-year-old female was admitted to our hospital with symptoms of nephrotic syndrome. Nine months before admission, she was diagnosed as having ocular and pulmonary sarcoidosis, for which prednisolone at an initial dose of 40 mg/day was started. When the dose of prednisolone was tapered to 20 mg/day, she noticed swelling of the lower extremities. Examination confirmed the simultaneous occurrence of minimal-change glomerular disease and Hashimoto's thyroiditis, which were diagnosed based on kidney histology, ultrasonography of the thyroid gland and positive antithyroglobulin antibodies. We used intravenous methylprednisolone pulse therapy followed by 40 mg/day oral prednisolone. The patient achieved complete remission of nephrotic syndrome and steroids were tapered without relapse.

  4. [Posterior uveitis: sarcoidosis or tuberculosis].

    PubMed

    Ness, T; Virchow, J C

    2001-02-01

    To demonstrate the difficulties of the differential diagnosis between tuberculosis and sarcoidosis as the cause of posterior uveitis. A 56-year-old woman suffered from bilateral anterior uveitis, snow-ball like infiltrates in the vitreous, and peripheral retinochoroidal granulomas with marked exudation shown in fluorescein angiography. Angiotensin-converting enzyme, as a marker of sarcoidosis, was elevated; the tuberculin test, however, was negative. Chest X-ray revealed an infiltrate and numerous smaller granulomas. The presumptive diagnosis was sarcoidosis. Surprisingly, in the biopsy of the pulmonal lesion tubercle bacilli were detected by Ziehl-Neelsen staining. Thus, a diagnosis of pulmonal and also retinochoroidal tuberculosis was made. After tuberculostatic therapy the choroidal lesions healed off. In a second case, a 30-year-old man suffered from bilateral panuveitis with candle wax exudates near the retinal vessels. Chest X-ray revealed lymphomas in the hilus, and the lymph node biopsy showed granulomas with epitheloid cells, indicating sarcoidosis. Detection of mycobacterium tuberculosis by culture or histological criteria was negative. Only in the PCR was mycobacterium tuberculosis DNA detectable. Tuberculostatic therapy had no benefit. Under therapy with steroids, however, pulmonal and ophthalmologic findings rapidly disappeared. The difficult differential diagnosis between sarcoidosis and tuberculosis cannot always be made by laboratory tests or diagnostic imaging alone. Clinical manifestations, including response to therapy, are essential.

  5. Ultrasound findings in cutaneous sarcoidosis

    PubMed Central

    Dybiec, Ewa; Pietrzak, Aldona; Kieszko, Robert; Kanitakis, Jean

    2015-01-01

    The diagnosis of cutaneous sarcoidosis relies mainly on the patient's history, presence of characteristic skin lesions and histological examination that shows a granulomatous, non-necrotizing dermal infiltration. The aim of the study was to assess the ultrasonographic features of cutaneous lesions of sarcoidosis before and after treatment. A 38-year-old woman with systemic sarcoidosis and specific cutaneous lesions was treated with systemic steroids followed by hydroxychloroquine. Ultrasonographic examination of the cutaneous sarcoidosis lesions was performed with a Philips iU 22 and Siemens Acuson S 2000 device, with the use of linear 15 MHz and 17 MHz transducers. Histological examination of skin lesions showed characteristic, naked, non-necrotizing granulomas in the upper dermis. Ultrasound examination revealed well-demarcated, hypoechogenic changes. Power-Doppler scan revealed increased vascularity within the lesions and the surrounding tissue. Clinical improvement of the skin lesions was confirmed by ultrasound examination, which showed a decrease in their size and normalization of dermal echogenicity and vascularity. Ultrasound examination can show cutaneous sarcoidosis lesions and their regression after appropriate treatment. PMID:25821428

  6. Sarcoidosis Presenting with Cicatrizing Conjunctivitis.

    PubMed

    O'Donnell, John J; Karakus, Sezen; Doroslovacki, Pavle; Akpek, Esen K

    2015-08-01

    To report two patients with sarcoidosis initially presenting with cicatrizing conjunctivitis. Both patients with chronic conjunctivitis were referred for further management. The first patient had conjunctival granulomas, subepithelial fibrosis, and forniceal foreshortening. The second patient had extensive upper and lower conjunctival scarring with forniceal foreshortening and symblepharon formation of both eyes. Conjunctival biopsy specimens revealed noncaseating granulomas. Immunofluorescein studies were negative for immunoreactant deposition in the basement membrane. Because of further diagnostic evaluations, sarcoidosis was determined to be the final cause of the cicatrizing conjunctivitis. Sarcoidosis should be included in the differential diagnosis of cicatrizing conjunctivitis. Recognition of the characteristic noncaseating granulomas in the conjunctival biopsy and initiation of the appropriate evaluations are essential in establishing the diagnosis and determining the extent of systemic involvement.

  7. Systemic Sarcoidosis with Thyroid Involvement.

    PubMed

    Okuma, Hideyuki; Hashimoto, Koshi; Wang, Xin; Ohkiba, Noriaki; Murooka, Nozomi; Akizuki, Norikazu; Inazawa, Takeshi; Ogawa, Yoshihiro

    2017-08-15

    A 66-year-old woman, who was diagnosed with iritis, visited our hospital due to general malaise. A blood analysis revealed hypercalcemia. Computed tomography revealed mediastinal and hilar lymph node hyperplasia. Moreover, (67)Gallium scintigraphy demonstrated strong accumulation in the lesions, suggesting sarcoidosis. A core needle biopsy (CNB) of the hypoechoic areas of the thyroid was performed because the patient refused to undergo a bronchoscopic examination. The scattering of slightly acidophilic epithelioid cell granulomas was observed in the pathological examination of the biopsy specimen. Based on this finding, the patient was diagnosed with sarcoidosis. Although sarcoidosis rarely involves the thyroid gland, in the present case, thyroid CNB was an alternative diagnostic method that allowed a pathological diagnosis to be obtained.

  8. Hepatic sarcoidosis: a case series

    PubMed Central

    Ennaifer, Rym; Ayadi, Shema; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; Bougassas, Wassila; Hadj, Najet Bel

    2016-01-01

    Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven. PMID:27795804

  9. Hepatic sarcoidosis: a case series.

    PubMed

    Ennaifer, Rym; Ayadi, Shema; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; Bougassas, Wassila; Hadj, Najet Bel

    2016-01-01

    Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.

  10. Sarcoidosis associated with neuromyelitis optica.

    PubMed

    Sawaya, Raja; Radwan, Wael

    2013-08-01

    Neuromyelitis optica (NMO) is an autoimmune disorder diagnosed by an elongated spinal cord lesion associated with unilateral or bilateral optic neuritis and anti-aquaporin 4 (AQP4) antibodies in the serum. It is triggered by or associated with several autoimmune diseases, but not with sarcoidosis. It responds to immunomodulators better than to steroid treatment. Sarcoidosis is an autoimmune disorder which manifests as non-caseating granulomas, usually in the lung parenchyma, but also in other tissues, including the brain. The involvement of the central nervous system in neurosarcoidosis differs considerably from that in neuromyelitis optica and the association of these two diseases concurrently in the same patient is unusual.

  11. What Are the Signs and Symptoms of Sarcoidosis?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Sarcoidosis? Many people who have sarcoidosis have no signs ... symptom is more common in women than men. Sarcoidosis Signs and Symptoms The illustration shows the major ...

  12. [Hypercalcemia revealing sarcoidosis in a child].

    PubMed

    Kinné, M; Filleron, A; Salet, R; Saumet, L; Baron Joly, S; Tran, T A

    2016-05-01

    Sarcoidosis is a systemic granulomatosis disease with a classic triad of presentation: typical clinical and radiological signs, presence of tuberculoid granuloma without caseum in histopathology, and exclusion of other causes of granulomatosis, especially tuberculosis. Sarcoidosis is rare in the general population, and even more so in children. In the literature, few cases of sarcoidosis associated with hypercalcemia have been reported in children. We report here the case of a 14-year-old boy with bone marrow and lymph node sarcoidosis suspected, based on poor general condition with hypercalcemia. The patient was treated with hydration, diuretics, and bisphosphonates with good results. We also performed a literature review of published cases of hypercalcemia since 1990 with a diagnosis of sarcoidosis in children, comparing 23 cases (including ours) on clinical and epidemiological, biological, imaging, and histopathological diagnosis. When hypercalcemia is present in the initial clinical presentation, the diagnosis of sarcoidosis is usually made in younger children. Classical locations of the lesions, including lung, skin, and lymph nodes, were highly suggestive of sarcoidosis. Corticosteroids are commonly used to treat sarcoidosis lesions including hypercalcemia. In conclusion, sarcoidosis in children remains difficult to diagnose because the disease is rare and it is common to have nonspecific symptoms in the clinical picture (with diagnosis delayed between 3 months and several years). The classic triad is not always present. Sarcoidosis should be systematically considered and investigated in case of hypercalcemia of unknown cause in children. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  13. Cardiac sarcoidosis: diagnosis and management.

    PubMed

    Dubrey, S W; Sharma, R; Underwood, R; Mittal, T

    2015-07-01

    Cardiac sarcoidosis is one of the most serious and unpredictable aspects of this disease state. Heart involvement frequently presents with arrhythmias or conduction disease, although myocardial infiltration resulting in congestive heart failure may also occur. The prognosis in cardiac sarcoidosis is highly variable, which relates to the heterogeneous nature of heart involvement and marked differences between racial groups. Electrocardiography and echocardiography often provide the first clue to the diagnosis, but advanced imaging studies using positron emission tomography and MRI, in combination with nuclear isotope perfusion scanning are now essential to the diagnosis and management of this condition. The identification of clinically occult cardiac sarcoidosis and the management of isolated and/or asymptomatic heart involvement remain both challenging and contentious. Corticosteroids remain the first treatment choice with the later substitution of immunosuppressive and steroid-sparing therapies. Heart transplantation is an unusual outcome, but when performed, the results are comparable or better than heart transplantation for other disease states. We review the epidemiology, developments in diagnostic techniques and the management of cardiac sarcoidosis.

  14. Musculoskeletal sarcoidosis in an Ethiopian.

    PubMed

    Lemma, Tesfaye; Diro, Ermias; Aderaye, Getachew; Chaka, Tezera

    2007-01-01

    We report a patient with osseous lesions of his fingers and thumbs that eventually were proved to be a manifestation of systemic sarcoidosis. This had been mis-diagnosed as tuberculosis and the patient had previously been treated without effect with two full courses of anti-tuberculosis chemotherapy. Eventually the clinical and radiological suspicions were confirmed by biopsy.

  15. Gallium scan in intracerebral sarcoidosis

    SciTech Connect

    Makhija, M.C.; Anayiotos, C.P.

    1981-07-01

    Sarcoidosis involving the nervous system probably occurs in about 4% of patients. The usefulness of brain scintigraphy in these cases has been suggested. In this case of cerebral sarcoid granuloma, gallium imaging demonstrated the lesion before treatment and showed disappearance of the lesion after corticosteroid treatment, which correlated with the patient's clinical improvement.

  16. Multisystemic Sarcoidosis Presenting as Pretibial Leg Ulcers.

    PubMed

    Wollina, Uwe; Baunacke, Anja; Hansel, Gesina

    2016-09-01

    Sarcoidosis is a multisystemic disease of unknown etiology. Up to 30% of patients develop cutaneous manifestations, either specific or nonspecific. Ulcerating sarcoidosis leading to leg ulcers is a rare observation that may lead to confusions with other, more common types of chronic leg ulcers. We report the case of a 45-year-old female patient with chronic multisystemic sarcoidosis presenting with pretibial leg ulcers. Other etiology could be excluded. Histology revealed nonspecific findings. Therefore, the diagnosis of nonspecific leg ulcers in sarcoidosis was confirmed. Treatment consisted of oral prednisolone and good ulcer care. Complete healing was achieved within 6 months. Sarcoidosis is a rare cause of leg ulcers and usually sarcoid granulomas can be found. Our patient illustrates that even in the absence of sarcoid granulomas, leg ulcers can be due to sarcoidosis. © The Author(s) 2016.

  17. Sarcoidosis of the upper and lower airways.

    PubMed

    Morgenthau, Adam S; Teirstein, Alvin S

    2011-12-01

    Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Although clinical granulomatous inflammation may occur within any organ system, more than 90% of sarcoidosis patients have lung disease. Sarcoidosis is considered an interstitial lung disease that is frequently characterized by restrictive physiologic dysfunction on pulmonary function tests. However, sarcoidosis also involves the airways (large and small), causing obstructive airways disease. It is one of a few interstitial lung diseases that affects the entire length of the respiratory tract - from the nose to the terminal bronchioles - and causes a broad spectrum of airways dysfunction. This article examines airway dysfunction in sarcoidosis. The anatomical structure of the airways is the organizational framework for our discussion. We discuss sarcoidosis involving the nose, sinuses, nasal passages, larynx, trachea, bronchi and small airways. Common complications of airways disease, such as, atelectasis, fibrosis, bullous leions, bronchiectasis, cavitary lesions and mycetomas, are also reviewed.

  18. Sarcoidosis vs tuberculosis: Diagnostic mystery still unresolved.

    PubMed

    Jain, Rashi; Mohan, Anant; Guleria, Randeep

    2017-10-01

    Sarcoidosis and tuberculosis are chronic, multisystemic, granulomatous disease of alike clinical, radiological and histopathological manifestations. Idiopathic nature of the disease and a strong clinical similarity with tuberculosis make the effectiveness of various clinical examinations for the diagnosis of sarcoidosis difficult in a tuberculosis endemic area. Presently confirmation of a diagnosis of sarcoidosis in most cases requires a biopsy which is often not confirmatory. A variety of novel medical approaches is under research to replace invasive diagnostic procedures for a simple non-invasive investigation for the identification of sarcoidosis. Here we discussed the studies focussing on the features that can be useful for distinguishing sarcoidosis from tuberculosis. Multiple studies have found molecular, cellular, immunological and clinical biomarkers efficient to lead the way of clinicians for the exact diagnosis of sarcoidosis. Copyright © 2017. Published by Elsevier B.V.

  19. Sixteen cases of uveitis associated with sarcoidosis.

    PubMed

    Bienfait, M F; Baarsma, G S

    1986-12-01

    In 210 cases of uveitis sixteen were associated with sarcoidosis. All patients had bilateral involvement and thirteen patients had a panuveitis. Ophthalmic and systemic findings in these patients are described and compared with the literature. Nine patients were not known to have sarcoidosis previously; seven of these presented with the typical signs for sarcoidosis, such as periphlebitis with 'candle wax' exudates and/or small chorioretinal lesions.

  20. A rare clinical presentation of sarcoidosis; gingivitis.

    PubMed

    Güzel, Aygül; Köksal, Nurhan; Aydın, Davut; Aslan, Kerim; Gören, Fikret; Karagöz, Filiz

    2013-10-01

    Gingivitis due to sarcoidosis is a relatively rare condition. Gingivitis or isolated gingival involvement may be the first sign of systemic sarcoidosis. We report the case of a 37 year-old woman with isolated gingivitis due to sarcoidosis confirmed by biopsy. Following treatment with a systemic corticosteroid (prednisolone 40 mg/day), all clinical and radiologic findings were completely improved. In cases of chronic and intractable gingivitis, systemic sarcoidosis should be suspected. It should be confirmed with a biopsy, and the patient should be referred to a chest disease clinic to exclude other organ involvement.

  1. Musculoskeletal and cutaneous sarcoidosis: exuberant case report.

    PubMed

    Haddad, Natalie; Oliveira Filho, Jayme de; Nasser, Kassila da Rosa; Corbett, Ana Maria França; Tebet, Ana Carolina Franco; Reis, Mariana Lacerda Junqueira

    2014-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown cause. The osteoarticular involvement in sarcoidosis is rare and is often associated with cutaneous and long-standing chronic multisystem disease. More common in black women, osseous sarcoidosis is difficult to diagnose, with an incidence of 3 to 13%. The most characteristic radiological clinical picture evidences rounded, well-defined cysts, with no periosteal reaction and without peripheral sclerosis. The small bones of hands and feet are the most frequently involved sites. This report aims to demonstrate a rare case of osteoarticular sarcoidosis with characteristic clinical presentation, and highlight the importance of detecting osteoarticular involvement in this pathology.

  2. Sarcoidosis of the peripheral nervous system.

    PubMed

    Said, Gérard

    2013-01-01

    Neurological manifestations of sarcoidosis are relatively rare but constitute a treatable cause of central and peripheral neurological manifestations. Regarding the peripheral nervous system, cranial nerves are predominantly affected, and peripheral facial nerve palsy, often bilateral, is the most common neurological manifestation of sarcoidosis. Multifocal peripheral neuropathy is a rare event in sarcoidosis. In some cases, however, peripheral neuropathy is the presenting manifestation and seemingly the only organ affected. Definite diagnosis of sarcoidosis rests ideally on histological demonstration of sarcoid granulomas in tissue biopsy specimens.

  3. Musculoskeletal and cutaneous sarcoidosis: exuberant case report*

    PubMed Central

    Haddad, Natalie; de Oliveira, Jayme; Nasser, Kassila da Rosa; Corbett, Ana Maria França; Tebet, Ana Carolina Franco; Reis, Mariana Lacerda Junqueira

    2014-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown cause. The osteoarticular involvement in sarcoidosis is rare and is often associated with cutaneous and long-standing chronic multisystem disease. More common in black women, osseous sarcoidosis is difficult to diagnose, with an incidence of 3 to 13%. The most characteristic radiological clinical picture evidences rounded, well-defined cysts, with no periosteal reaction and without peripheral sclerosis. The small bones of hands and feet are the most frequently involved sites. This report aims to demonstrate a rare case of osteoarticular sarcoidosis with characteristic clinical presentation, and highlight the importance of detecting osteoarticular involvement in this pathology. PMID:25054759

  4. PSMA Uptake in Mediastinal Sarcoidosis.

    PubMed

    Ardies, Philip Junior; Gykiere, Pieterjan; Goethals, Lode; De Mey, Johan; De Geeter, Frank; Everaert, Hendrik

    2017-04-01

    Prostate-specific membrane antigen (PSMA) is a cell surface glycoprotein which is frequently overexpressed on prostate cancer cells. Ga-PSMA PET/CT plays an increasing role in prostate cancer management. However, growing evidence suggests increased PSMA uptake in a variety of other malignant tumor entities and in some benign lesions. This report describes PSMA uptake in numerous thoracic lymph nodes in a patient with known mediastinal sarcoidosis. Knowledge and recognition of these possibilities are important to avoid scan misinterpretation.

  5. Multimodality Imaging in Cardiac Sarcoidosis: Is There a Winner?

    PubMed Central

    Perez, Irving E.; Garcia, Mario J.; Taub, Cynthia C.

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown cause that can affect the heart. Cardiac sarcoidosis may be present in as many as 25% of patients with systemic sarcoidosis, and it is frequently underdiagnosed. The early and accurate diagnosis of myocardial involvement is challenging. Advanced imaging techniques play important roles in the diagnosis and management of patients with cardiac sarcoidosis. PMID:25784137

  6. FDG PET/CT in bone sarcoidosis.

    PubMed

    Grozdic Milojevic, Isidora; Sobic-Saranovic, Dragana; Videnovic-Ivanov, Jelica; Saranovic, Djordjije; Odalovic, Strahinja; Artiko, Vera

    2016-03-29

    Bone sarcoidosis is rare manifestation of disease usually accompanied with pulmonary involvement. Until today, exact prevalence of bone sarcoidosis is not known, since reported prevalence varies widely depending on the studied population and the used diagnostic techniques. To determine the prevalence of bone involvement and distribution pattern in active chronic sarcoidosis by using FDG PET/CT. Between January 2010 and December 2011, 98 patients with chronic sarcoidosis and presence of prolonged symptoms or other findings suggestive of active disease were referred to FDG PET/CT examination. Active disease was found in 82 patients, and they all were screened for presence of bone sarcoidosis on FDG PET/CT. All patients also underwent MDCT and assessment of serum ACE level. Bone sarcoidosis was present in 18/82 patients with active sarcoidosis. FDG uptake in bones was focal in 8 (44.4%), diffuse in 6 (33.3%) and both diffuse and focal in 4 (22.2%) patients. CT indicated bone abnormalities only in 5% patients. Osseous involvement was present in: pelvis (61.1%), vertebrae (44.4%), ribs (27.8%) and bone marrow (16.7%). Some patients had two or more locations of disease. Follow-up FDG PET/CT showed normal findings in two patients, same localization of active disease in four patients and progression of disease in one. In patients with active chronic sarcoidosis 22% of patients had osseous abnormalities on FDG PET/CT that mostly were not detected on CT.

  7. Cutaneous sarcoidosis simulating porokeratosis of Mibelli

    PubMed Central

    Elfatoiki, Fatima Zahra; Soussi, Wessal; Chiheb, Soumia; Jabri, Lamia; Benchikhi, Hakima

    2015-01-01

    We report a skin localization of systemic sarcoidosis, which presented with lesions that resemble porokeratosis of Mibelli. Skin biopsy showed non-caseating sarcoidal granuloma. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, our presentation is uncommon. Partial remission was obtained with hydroxychloroquine and prednisone PMID:26113926

  8. Cutaneous sarcoidosis simulating porokeratosis of Mibelli.

    PubMed

    Elfatoiki, Fatima Zahra; Soussi, Wessal; Chiheb, Soumia; Jabri, Lamia; Benchikhi, Hakima

    2015-01-01

    We report a skin localization of systemic sarcoidosis, which presented with lesions that resemble porokeratosis of Mibelli. Skin biopsy showed non-caseating sarcoidal granuloma. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, our presentation is uncommon. Partial remission was obtained with hydroxychloroquine and prednisone.

  9. Ocular Rosacea

    MedlinePlus

    ... Staff Ocular rosacea is inflammation that causes redness, burning and itching of the eyes. It often develops ... symptoms of ocular rosacea may include: Dry eyes Burning or stinging in the eyes Itchy eyes Grittiness ...

  10. Evaluation of Known or Suspected Cardiac Sarcoidosis.

    PubMed

    Blankstein, Ron; Waller, Alfonso H

    2016-03-01

    Sarcoidosis is a multisystem disorder of unknown cause, and cardiac sarcoidosis affects at least 25% of patients and accounts for substantial mortality and morbidity from this disease. Cardiac sarcoidosis may present with heart failure, left ventricular systolic dysfunction, AV block, atrial or ventricular arrhythmias, and sudden cardiac death. Cardiac involvement can be challenging to detect and diagnose because of the focal nature of the disease, as well as the fact that clinical criteria have limited diagnostic accuracy. Nevertheless, the diagnosis of cardiac sarcoidosis can be enhanced by integrating both clinical and imaging findings. This article reviews the various roles that different imaging modalities provide in the evaluation and management of patients with known or suspected cardiac sarcoidosis. © 2016 American Heart Association, Inc.

  11. [An unusual case of duodenal sarcoidosis].

    PubMed

    Leroy, C; Girard, C; Girard-Madoux, M-H; Coppéré, B; Desmurs-Clavel, H; Pérard, L; Hot, A; Ninet, J

    2015-11-01

    Sarcoidosis is a systemic granulomatous disorder of unknown aetiology. It may rarely affect the gastrointestinal tract. We reported a 54-year-old woman with a delayed diagnosis of duodenal sarcoidosis. She presented with gastric and right upper abdominal pain associated with vomiting and marked weight loss. Abdominal computed tomographic scan showed non-compressive retroperitoneal lymph nodes and histological examination revealed non-caseating epithelioid granulomas typical of sarcoidosis. Diagnosis of duodenal sarcoidosis was obtained at the third gastroscopy. The patient's condition improved quickly with corticosteroid therapy. Gastrointestinal sarcoidosis should be looked for in patients with digestive symptoms and another sarcoid localisation. Furthermore, it is important to repeat gastroscopy to confirm diagnosis because treatment improved most patients. Copyright © 2015. Published by Elsevier SAS.

  12. Papular sarcoidosis of the knees. A frequent form of presentation of systemic sarcoidosis.

    PubMed

    Marcoval, Joaquim; Mañá, Juan

    2016-03-29

    In recent years we have observed with increasing frequency granulomatous papular lesions involving the knees, for which we proposed the term papular sarcoidosis of knees. To evaluate the clinicopathological features of papular sarcoidosis of the knees. Patients with papular lesions of the knees and histopathologically sarcoid granulomas were included in the study. Systemic sarcoidosis was investigated in all cases. Clinical charts were retrospectively retrieved. Biopsy specimens were evaluated under polarized light to detect foreign bodies. Fifty-three patients fulfilled inclusion criteria. In 36 cases systemic sarcoidosis was diagnosed and these cases were considered as papular sarcoidosis of the knees. Foreign particles were observed in 21 of these 36 patients. In only 9/36 patients did the activity of systemic disease persist over two years. In 17 cases sarcoidosis could not be demonstrated during follow-up. Papular sarcoidosis of the knees can be considered a relatively frequent form of cutaneous sarcoidosis usually present at the beginning of the disease that can be useful for the diagnosis of sarcoidosis. It is mainly observed in acute forms of sarcoidosis and can be considered a sign of good prognosis.

  13. The three tiers of screening for sarcoidosis organ involvement.

    PubMed

    Judson, Marc A

    2016-04-01

    Sarcoidosis may involve any organ in the body. Organ involvement with sarcoidosis may go undetected. This manuscript addresses a suggested approach to screening for sarcoidosis organ involvement. As sarcoidosis organ involvement may never cause symptoms or clinical problems, it is not always necessary to expend the time or expense to detect all organs involved with sarcoidosis. On the other hand, some forms of sarcoidosis organ involvement may have potentially severe consequences such as permanent vision impairment from eye sarcoidosis and sudden death from cardiac sarcoidosis. We believe it useful to describe three tiers of screening for sarcoidosis organ involvement. The first tier of screening involves eliciting a medical history and performing a physical examination. This is a useful screen for all organs. The second tier of screening involves performing specific laboratory tests to search for specific sarcoidosis organ involvement, even if there is no clinical sign or symptom to suggest involvement of that organ. Such screening is only recommended for a limited number of specific organs. The third tier of screening is complex and involves the performance of multiple tests/algorithms or examinations by subspecialists to search for specific organ involvement. The third tier of screening is used to evaluate eye sarcoidosis, vitamin D dysregulation associated with sarcoidosis, and cardiac sarcoidosis. It is hoped that this approach to screening for sarcoidosis organ involvement will be a springboard for rigorous examination of this process that is likely to benefit sarcoidosis patients. Copyright © 2016 Elsevier Ltd. All rights reserved.

  14. Pulmonary mucosa-associated lymphoid tissue lymphoma associated with pulmonary sarcoidosis: a case report and literature review.

    PubMed

    Kokuho, Nariaki; Terasaki, Yasuhiro; Urushiyama, Hirokazu; Terasaki, Mika; Kunugi, Shinobu; Morimoto, Taisuke; Azuma, Arata; Usuda, Jitsuo; Gemma, Akihiko; Eishi, Yoshinobu; Shimizu, Akira

    2016-05-01

    Differentiating low-grade lymphoma from preexisting sarcoidosis is difficult because of their pathological similarity. This article describes a case of pulmonary mucosa-associated lymphoid tissue lymphoma associated with pulmonary sarcoidosis. The patient, a 45-year-old Japanese man, presented with a 10-year history of pulmonary sarcoidosis and 5-year history of ocular sarcoidosis with histologic findings. Because only the right S3 lung nodule had gradually enlarged, partial resection was performed. Pathological study revealed noncaseous epithelioid granulomas with lymphoplasmacytic proliferation but also marked lymphoid cell proliferation with lymphoepithelial lesion findings that differed from findings of typical sarcoid lesions. Our lymphoepithelial lesion evaluation via immunohistochemistry and analysis of Ig heavy-chain gene rearrangements with assessment of Propionibacterium acnes-specific antibody reactions allow us to report, for the first time, this case of pulmonary mucosa-associated lymphoid tissue lymphoma associated with pulmonary sarcoidosis in exactly the same location, which may be significant for differentiating these diseases and understanding their pathogenic association.

  15. Abdominal sarcoidosis: cross-sectional imaging findings

    PubMed Central

    Gezer, Naciye Sinem; Başara, Işıl; Altay, Canan; Harman, Mustafa; Rocher, Laurence; Karabulut, Nevzat; Seçil, Mustafa

    2015-01-01

    Sarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%–70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis. PMID:25512071

  16. Sarcoidosis Extent Relates to Molecular Variability.

    PubMed

    Monast, Calixte S; Li, Katherine; Judson, Marc A; Baughman, Robert P; Wadman, Eric; Watt, Rosemary; Silkoff, Philip E; Barnathan, Elliot S; Brodmerkel, Carrie

    2017-02-16

    The molecular basis of sarcoidosis phenotype heterogeneity and its relationship to effective treatment of sarcoidosis have not been elucidated. Peripheral samples from sarcoidosis subjects who participated in a phase 2 study of golimumab (anti-TNFα) and ustekinumab (anti-IL12p40) were used to measure the whole blood transcriptome and levels of serum proteins. Differential gene and protein expression analyses were used to explore the molecular differences between sarcoidosis phenotypes as defined by extent of organ involvement. The same data were also used in conjunction with an enrichment algorithm to identify gene expression changes associated with treatment with study drugs compared to placebo. Our analyses revealed marked heterogeneity among the three sarcoidosis phenotypes included in the study cohort, including striking differences in enrichment of the Interferon Pathway. Conversely, enrichments of multiple pathways, including T Cell Receptor Signaling, were similar among phenotypes. We also identify differences between treatment with golimumab and ustekinumab that may explain the differences in trends for clinical efficacy observed in the trial. We find that molecular heterogeneity is associated with sarcoidosis in a manner that may be related to the extent of organ involvement. These findings may help explain the difficulty in identifying clinically efficacious sarcoidosis treatments and suggest hypotheses for improved therapeutic strategies. This article is protected by copyright. All rights reserved.

  17. Pulmonary sarcoidosis with an alveolar radiographic pattern.

    PubMed Central

    Battesti, J P; Saumon, G; Valeyre, D; Amouroux, J; Pechnick, B; Sandron, D; Georges, R

    1982-01-01

    Thirty-three cases of sarcoidosis (4.4% of 746 patients) showed an alveolar radiological pattern. A study of pulmonary function was carried out in 25 patients and compared with that of 46 patients with the interstitial radiological type of sarcoidosis. Twenty-two cases have been followed up from one to six years after the initial examination. The radiographic lesions were most often bilateral and included nodules greater than 15 mm with ill-defined margins or diffuse, infiltrative, non-retractile opacities with fluffy margins. Bilateral mediastinal lymph nodes were present in 27 patients. In 20 patients an associated reticulation was found on radiography. In four patients an open lung biopsy was done. The granulomatous nodules were identical to those found in other forms of sarcoidosis, although they were more confluent in the affected areas. Clinical and functional findings did not differ from those in the more common forms of sarcoidosis. Alveolar sarcoidosis has a sudden course. The alveolar radiological patterns always disappeared, with or without steroid treatment, while reticular patterns persisted in four patients. Rapid radiological changes were observed. Some functional abnormalities persisted in cases that were followed. It is concluded that alveolar sarcoidosis is a distinct acute form of sarcoidosis. Images PMID:7135279

  18. Sarcoidosis of the cardio-pulmonary systems.

    PubMed

    Dubrey, Simon; Sharma, Rakesh; Underwood, Richard; Mittal, Tarun; Wells, Athol

    2016-02-01

    Sarcoidosis is a multi-system disease with a wide range of phenotypes. Pulmonary involvement is the most frequently identified target for sarcoidosis and is responsible for the majority of deaths. Cardiac sarcoid is less commonly identified, may be occult, is significantly influenced by race, and can portend an unpredictable and sometimes fatal outcome. Sarcoidosis remains an enigmatic disease spectrum of unknown aetiology, frequently difficult to diagnose and with a variable disease course. This article summarises current views on the diagnosis and management of cardiopulmonary involvement.

  19. Sarcoidosis, cancer and molecular mimicry.

    PubMed

    Tchernev, G; Wollina, U

    2013-01-01

    Molecular mimicry seems to be the most important factor for the heterogeneous clinical presentation and the immunopathogenesis of sarcoidosis. Molecular mimicry may occur as a result of altered activity of oncogenes. This can lead to crossed-type mediated body reactions targeting structurally similar sections or regions from the tissue homeostasis. Available data suggest that structural analogy between tissue and foreign or de novo-appearing peptides is not always reliable. Nevertheless, lack of amino acid identity between the tissue and the de novo-generated tumour antigens does not exclude the phenomenon of molecular mimicry as the major generator of sarcoidosis. There is growing evidence of the mimicry phenomena, caused not only by the similarity between the amino acids but also between the elements which connect segments in the immunological cascade and which may also be affected by external factors. Molecular mimicry may occur between two identified peptides having similar antigenic surfaces (transitory or not), in the absence of a primary homology in amino acid sequence. As far as tumour antigens are concerned, a structural analogy to the de novo-appearing tumour antigens is more likely than transitory imitation resulting from the additional interference of other physical forces. Further research should be performed to confirm, or reject, the transitory imitation thesis or hypothesis.

  20. [Hypercalcemia caused by generalized sarcoidosis].

    PubMed

    Hierl, T; Libicher, M; Nawroth, P; Ziegler, R; Kasperk, C

    2000-12-08

    A 70-year-old man with a 23-year history of recurrent nephrolithiasis was admitted for evaluation of hypercalcaemia (maximal calcium concentration 3.24 mmol/l). Physical examination on admission was unremarkable except for sensory neuropathy in the right foot. Chest radiogram, subsequent transbronchial lung biopsy, gallium scintigraphy and magnetic resonance imaging (MRI) of the vertebrae indicated generalized sarcoidosis, predominantly affecting the mediastinum, lumbar vertebrae and nerves of the distal limbs. Parathormone level was reduced, while the levels of vitamin D metabolites were normal. Serum calcium level became normal within 2 weeks of starting glucocorticoid treatment. Hypercalcaemia can occur in the course of sarcoidosis even in the absence of a elderly increased level of 1,25-dihydroxyvitamin D3. In this case the cause of the hypercalcaemia of a year's duration may have been due to lymphomacrophagocytic infiltration of the bone of several vertebrae as demonstrated by MRI of the vertebral columns but not by conventional radiography. In this case an increase in cytokine secretion by macrophages may have played an important role in addition to the level of 1,25-dihydroxyvitamin D3 at the upper limit of normal that, in view of the renal failure, could only have been caused by extrarenal production. Cytokines (e.g. interleukin-1 beta and tumour necrosis factor alpha) secreted by activated macrophages are among the most powerful stimulators of bone reabsorption.

  1. [Ocular allergies].

    PubMed

    Messmer, E M

    2005-05-01

    Recent developments indicate that ocular allergy is more than an IgE-mediated allergic conjunctivitis. Ocular allergy is a disease affecting the entire ocular surface including conjunctiva, lids, cornea, lacrimal gland and tear film. Besides an IgE-mediated reaction, a complex chronic inflammation is involved in the pathogenesis of many ocular allergies. According to their pathogenesis and clinical picture, ocular allergies are classified into mild forms, such as seasonal and perennial allergic conjunctivitis as well as giant papillary conjunctivitis, and chronic, potentially blinding forms such as atopic keratoconjunctivitis and vernal keratoconjunctivitis. New therapeutics act on the entire inflammatory process or try to modulate the allergic reaction early and specifically. The association with non-ocular allergic symptoms requires an interdisciplinary approach.

  2. Subcutaneous sarcoidosis in a rhinoplasty scar

    PubMed Central

    Dulguerov, Nicolas; Vankatova, Lenka; Landis, Basile Nicolas

    2015-01-01

    The presence of a subcutaneous hard bony-like lump at the lateral nasal wall after a septorhinoplasty procedure is an unfavourable result. The reported patient developed this complication 2 years after a revision surgery, in which percutaneous osteotomies were performed. An excision biopsy of the lump took place and the histopathological analysis revealed a granulomatous gigantocellular inflammation with absence of birefringent particles on polarised lamp and negative mycobacteria culture. After additional investigations, the final diagnosis was consistent with grade 2 pulmonary sarcoidosis associated with subcutaneous sarcoidosis. No treatment was initiated. The facial symptoms resolved without any additional treatment and the pulmonary function tests have not deteriorated after 1 year of follow-up. The polymorphism of cutaneous lesions in sarcoidosis, the absence of systemic symptoms and the unrecognised entity of subcutaneous sarcoidosis in a scar illustrate the diagnostic challenge with this patient. PMID:25819832

  3. Sarcoidosis complicated by cirrhosis and hepatopulmonary syndrome

    PubMed Central

    Gupta, Samir; Faughnan, Marie E; Prud’homme, Gerald J; Hwang, David M; Munoz, David G; Kopplin, Peter

    2008-01-01

    Sarcoidosis is a multisystem disorder commonly affecting the lungs, but also the liver, with cirrhosis and portal hypertension occurring in fewer than 1% of cases. Although hepatopulmonary syndrome (HPS) is seen in 15% to 20% of patients with cirrhosis of varying causes, it has rarely been associated with sarcoidosis. Also, although a brain abscess is not uncommon in patients with discrete pulmonary arteriovenous malformations, it is rarely seen in patients with the much smaller intrapulmonary vascular dilations that characterize HPS. A patient with an unusual series of uncommon sarcoidosis complications, including cirrhosis with HPS, brain abscess and finally Nocardia meningitis, is reported. The possibility of HPS should be considered in sarcoidosis patients with liver involvement, if gas-exchange abnormalities are out of proportion to the degree of lung involvement. These patients may also be susceptible to a cerebral abscess by paradoxical embolization, and to opportunistic infections due to cirrhosis. PMID:18437252

  4. Helicobacter pylori-related immunoglobulins in sarcoidosis.

    PubMed

    Herndon, Betty L; Vlach, Victoria; Dew, Michelle; Willsie, Sandra K

    2004-03-01

    The purpose of this study was to determine serum antibody titers against a common bacterial antigen, Helicobacter pylori (H. pylon), in subjects with sarcoidosis, comparing those titers to those present in a healthy population. With the approval of the Institutional Review Board of the University of Missouri-Kansas City, patients with sarcoidosis (pulmonary and extrapulmonary) who visited the Truman Medical Center-Hospital Hill pulmonary clinic were recruited to enter the study. A serum sample was frozen at -70 degrees C for later testing (n = 20). Specific information collected on subjects included corticosteroid use, use of histamine2 blockers and antacids, date of first diagnosis, and stage of sarcoidosis. Normal controls and demographically matched individuals who lacked pulmonary diseases, including sarcoidosis, were also recruited. Serum samples were processed as above. Antibody capture enzyme immunoassay was completed for H. pylori and urease antigens by serum dilution assay for each subject, from which titers for antigen-specific immunoglobulin (Ig)G and IgA were calculated. Nonspecific serum IgE was also measured. An increased incidence of high-titer IgG antibody directed against H. pylori antigens was found in subjects with sarcoidosis compared with controls. The sarcoidosis and control groups were significantly different with respect to IgG and IgA against H. pylori, both at p = .001. IgG directed against urease was also significantly different between sarcoidosis and control patients (p = .001), but IgA directed against urease was very low in all subjects and did not yield significant differences between groups. Specific H. pylori and urease IgG antibodies exceeded those expected in the population studied. The data suggest that in pulmonary sarcoidosis, the relationship of H. pylori and its products to sarcoid granuloma formation warrants further investigation.

  5. Subcutaneous sarcoidosis: a clinical analysis of nine patients.

    PubMed

    Ando, Masaru; Miyazaki, Eishi; Hatano, Yutaka; Nishio, Suehiro; Torigoe, Chihiro; Yamasue, Mari; Mukai, Yutaka; Nureki, Shin-Ichi; Kadota, Jun-Ichi

    2016-09-01

    Subcutaneous sarcoidosis is the specific subset of cutaneous sarcoidosis frequently associated with systemic disease. However, the disease activity, severity, and prognosis have not yet been elucidated due to the limited number of reported cases. The purpose of this study was to identify the clinical, laboratory, and prognostic differences between subcutaneous sarcoidosis and other type of cutaneous sarcoidosis. All patients with sarcoidosis diagnosed histopathologically from 2000 to 2012 at our institution were enrolled. The clinical, laboratory, chest X-ray, and pulmonary function test results were retrospectively evaluated in the patients with cutaneous sarcoidosis. In the 130 patients with sarcoidosis, cutaneous sarcoidosis was diagnosed in 37 patients (28.4 %), and 9 (6.9 %) of these patients had subcutaneous sarcoidosis. The serum levels of soluble interleukin-2 receptor (sIL-2R) were significantly elevated in the group of patients with subcutaneous sarcoidosis in comparison to the patients with other types of cutaneous sarcoidosis, whereas there was no significant difference in the severity score between the two groups. Following a 2-year observation period, three patients were in remission, five patients demonstrated stable disease, and one patient had progressive disease. Subcutaneous sarcoidosis may be associated with the disease activity, although it was not found to be associated with the disease severity and it was not a predictive factor for the prognosis. Furthermore, the prevalence of subcutaneous sarcoidosis may be higher than that in previously reported series.

  6. Sarcoidosis in Melanoma Patients: Case Report and Literature Review

    PubMed Central

    Beutler, Bryce D.; Cohen, Philip R.

    2015-01-01

    Sarcoidosis is a systemic inflammatory disease characterized by the development of noncaseating granulomas in multiple organ systems. Many hematologic malignancies and solid tumors, including melanoma, have been associated with sarcoidosis. We describe the clinical and pathologic findings of a 54-year-old man with melanoma-associated sarcoidosis. In addition, we not only review the literature describing characteristics of other melanoma patients with sarcoidosis, but also the features of melanoma patients with antineoplastic therapy-associated sarcoidosis. Sarcoidosis has been described in 80 melanoma patients; sufficient information for analysis was provided in 39 of these individuals. In 43.6% of individuals (17 out of 39), sarcoidosis was directly associated with melanoma; in 56.4% of oncologic patients (22 out of 39), sarcoidosis was induced by antineoplastic therapy that had been administered for the treatment of their metastatic melanoma. The discovery of melanoma preceded the development of sarcoidosis in 12 of the 17 (70.5%) individuals who did not receive systemic treatment. Pulmonary and/or cutaneous manifestations of sarcoidosis were common among both groups of patients. Most patients did not require treatment for sarcoidosis. Melanoma patients—either following antineoplastic therapy or without systemic treatment—may be at an increased risk to develop sarcoidosis. In antineoplastic therapy naive melanoma patients, a common etiologic factor—such as exposure to ultraviolet light—may play a role in their developing melanoma and sarcoidosis. PMID:26083934

  7. Prevalence and clinical picture of musculoskeletal sarcoidosis.

    PubMed

    Salari, Masoumeh; Rezaieyazdi, Zahra

    2014-07-01

    Sarcoidosis is a multisystem disease affecting different organs with different frequency rates depending on geographical location. Musculoskeletal abnormalities includes osseous lesions (small and large bone sarcoidosis), sarcoidal arthropathy, and sarcoidal myopathy. Musculoskeletal involvement is reported in a significant number of patients. This study aimed to determine the prevalence and clinical picture of musculoskeletal sarcoidosis in Iranian cohort of patients with sarcoidosis. We designed a descriptive cross-sectional study including 30 patients with sarcoidosis who had hospitalized in the Rheumatology Department at Ghaem Hospital, Mashhad, Iran. The patients were evaluated for musculoskeletal symptoms using history, physical examination, and paraclinical data. Of the 30 studied patients, 24 were female (80%) and six were male (20%). The mean age at diagnosis was 38 years. Sarcoidal arthropathy (arthritis and periarthritis) was observed in 26 patients (86.6%). Furthermore, the initial presentation was associated with joint symptoms in 19 cases (63.3%); acute arthritis developed in 17 (65%) while bone and muscle involvements each occurred in 2 (6.6%). Sarcoidosis is a common disease in women aged 20 to 40 years. The most common involved joint were consecutively ankles, knees, and wrists, reaching a accumulated frequency of 86.6%; however, bone and muscle involvements were uncommon.

  8. Cardiac sarcoidosis: challenges in clinical practice.

    PubMed

    Bakker, Anne L; Grutters, Jan C; Keijsers, Ruth G; Post, Martijn C

    2017-09-01

    To address the current recommendations for screening, diagnosis, and treatment of cardiac sarcoidosis and the difficulties to put these recommendations into clinical practice. The incidence of cardiac sarcoidosis appears to be higher than earlier reported, probably because of improved imaging techniques. Late gadolinium enhancement with cardiac MRI (LGE-CMR) and fluorodeoxyglucose positron emission tomography obtained a central role in the diagnostic algorithm and monitoring of disease activity. New techniques are being investigated: T1 and T2 mapping for early detection in CMR, a sarcoid-specific tracer in PET, integrated positron emission tomography/MRI scanners, and assessment of scar with LGE in cardiac computed tomography. Isolated cardiac sarcoidosis is an increasingly recognized phenotype, but still an enormous challenge in clinical practice. The prognostic value of (and extent of) LGE-CMR should be taken into account for risk assessment and internal cardiac defbrillator therapy, even in patients with preserved left ventricular function. Unfortunately, randomized controlled trials to guide immunosuppressive therapy are still lacking. A multidisciplinary approach to diagnose and treat cardiac sarcoidosis patients in specialized centers is strongly recommendable. Cardiac sarcoidosis is increasingly recognized because of improved imaging techniques; however, treatment of cardiac sarcoidosis is still mainly based on expert opinion.

  9. [Sarcoidosis - diagnosis and therapy today].

    PubMed

    Christ, R

    1980-12-15

    The etiology of sarcoidosis is still unclear. It belongs to the most frequent lung diseases; but also nearly all other tissues and organs may be established. One third of the diseases runs under the picture of the acute form of the course (Loefgren's syndrome). A histologic ascertainment is to be striven for. The tendency to spontaneous remission is great. This is still additionally supported by the application of cortison derivatives. In the individual case the indication to medicamentous therapy results from the tendency of the course of the disease, from the degree of the affection of organs and the clinico-functional disturbance. In 80 patients with Loefgren's syndrome the author adopts a definite attitude to diagnostics and therapy.

  10. Sarcoidosis presenting with pleurisy and bilateral pleural effusions.

    PubMed Central

    Johnson, N. M.; Martin, N. D.; McNicol, M. W.

    1980-01-01

    A patient with acute sarcoidosis is described who presented with pleurisy and bilateral pleural effusions, but no other abnormalities on examination or chest X-ray. This presentation of sarcoidosis is unusual and is discussed. Images Fig. 1 PMID:7433329

  11. [Sarcoidosis of the female genital tract].

    PubMed

    Šefčíková, A; Turková, M; Žurková, M

    To present the findings of sarcoidosis on female genital tract. Review. Department of Obstetric and Gynecology, Silesian Hospital Opava. Overview of published findings from case studies. Sarcoidosis is a multisystem granulomatous disorder of unclear cause. It typically involves the lymph nodes of mediastinum, predominantly billateral and/or pulmonary infiltrates. We find extrapulmonary involvement in 30-50% of cases. Sarcoidosis of the female reproductive system is a rare, it represent less than 1% cases of sarcoidosis. Lesions there may affect any organ, including the vulva, vagina, cervix, uterus, fallopian tube and ovary, but also for example placenta and breast. There is also recorded the incidence of multiple localization on female genitalia. Since sarcoidosis of this area is so rare, often proceeds asymptomatic and recognized only as an incidental finding, there are mention only the case histories in literature yet.Clinical symptoms may be non-specific, often imitating a tumor, or tend to be specific, depending on the localization of disability such as perineal pain, pain in the scar after the previous birth trauma, persistent pruritus, itching, irritation, dyspareunia, menstrual cycle disorders, menorrhagia, metrorrhagia, postmenopausal bleeding, amenorrhoe, abdominal pain, endometrial polypoid lesions, recurrent or persistent serometra or discharge. The diagnosis is made up of histologically - we are demonstrating noncaseating granulomas.The therapy is difficult, there are no available official guidelines. If the lesions are clinically silent, we can observed them because they may spontaneously disappear. If we are embarking on medical therapy, we start from a local application, and if this is unsuccessful then we approach the systemic administration. Corticosteroids are the drug of choice. If we diagnose the sarcoidosis of the female genital organs we must exclude systemic disease of sarcoidosis. The prognosis of disease is good.

  12. Sarcoidosis mimicking metastatic papillary thyroid cancer

    PubMed Central

    Salih, Abdulwahid M.; Fatih, Salah M.; Kakamad, F.H.

    2015-01-01

    Introduction Sarcoidosis is a multisystemic, idiopathic disease. It has a highly variable clinical course. It has been reported to present in association with malignancy. Coexistence of sarcoidosis and thyroid cancer is rarely reported in the literature. Presentatioin of the case We present a case with neck swelling for 3 months, and symmetrical painless thyroid enlargement without fixation to deep tissues of the neck. Multiple nodules on the both thyroid lobes, hard irregular, grade two goiter with lymphadenopathy all over anterior neck compartments. Fine needle aspiration cytology was done under ultrasound guide from right thyroid nodule and showed papillary thyroid carcinoma. Excisional biopsy of the neck lymphnode showed picture typical for sarcoidosis. Discussion Most researchers believe that patients with pulmonary sarcoidosis are predisposed to develop malignancies, less than a dozen of cases are reported in the literature to be associated with PTC with a very wide range of presentations and clincal coarses. An interesting finding of our case is that in contrast to what is reported, both diseases were not known by the physician until the time of presentation. Conclusion Cervical lymphadenopathy in association with goiter could be metastasis, sarcoidosis or mixed, therefore should be seperately biopsied. PMID:26432997

  13. EEG in Sarcoidosis Patients Without Neurological Findings.

    PubMed

    Bilgin Topçuoğlu, Özgür; Kavas, Murat; Öztaş, Selahattin; Arınç, Sibel; Afşar, Gülgün; Saraç, Sema; Midi, İpek

    2017-01-01

    Sarcoidosis is a multisystem granulomatous disease affecting nervous system in 5% to 10% of patients. Magnetic resonance imaging (MRI) is accepted as the most sensitive method for detecting neurosarcoidosis. However, the most common findings in MRI are the nonspecific white matter lesions, which may be unrelated to sarcoidosis and can occur because of hypertension, diabetes mellitus, smoking, and other inflammatory or infectious disorders, as well. Autopsy studies report more frequent neurological involvement than the ante mortem studies. The aim of this study is to assess electroencephalography (EEG) in sarcoidosis patients without neurological findings in order to display asymptomatic neurological dysfunction. We performed EEG on 30 sarcoidosis patients without diagnosis of neurosarcoidosis or prior neurological comorbidities. Fourteen patients (46.7%) showed intermittant focal and/or generalized slowings while awake and not mentally activated. Seven (50%) of these 14 patients with EEG slowings had nonspecific white matter changes while the other half showed EEG slowings in the absence of MRI changes. We conclude that EEG slowings, when normal variants (psychomotor variant, temporal theta of elderly, frontal theta waves) are eliminated, may be an indicator of dysfunction in brain activity even in the absence of MRI findings. Hence, EEG may contribute toward detecting asymptomatic neurological dysfunction or probable future neurological involvement in sarcoidosis patients.

  14. The Etiologic Role of Infectious Antigens in Sarcoidosis Pathogenesis.

    PubMed

    Celada, Lindsay J; Hawkins, Charlene; Drake, Wonder P

    2015-12-01

    Sarcoidosis is a granulomatous disease of unknown etiology, most commonly involving the lung, skin, lymph node, and eyes. Molecular and immunologic studies continue to strengthen the association of sarcoidosis with infectious antigens. Independent studies report the presence of microbial nucleic acids and proteins within sarcoidosis specimens. Complementary immunologic studies also support the role of infectious agents in sarcoidosis pathogenesis. Case reports and clinical trials have emerged regarding the efficacy of antimicrobials. They support increasing efforts to identify novel therapeutics, such as antimicrobials, that will have an impact on the observed increase in sarcoidosis morbidity and mortality. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. [2 cases of sarcoidosis in Yaounde (Cameroon)].

    PubMed

    Capdevielle, P; Vauterin, G; Mouden, J C; Darie, H

    1984-01-01

    Two new cases of sarcoidosis are reported from Cameroon. The first one concerned a man, 53 years old, who presented multiple dermal nodules of the face, polyadenopathy , splenomegaly, exertional dyspnea, reticular and micronodular aspect of lungs X-ray. Diagnosis was confirmed by histology of skin, ganglions and bronchus biopsies. Patient was treated by corticotherapy. The other one concerned a woman, 28 years old, who presented successively mediastinal adenopathies, then, 9 months later, reticular and micronodular aspect of lungs X-ray. During this period, a treatment against tuberculosis had been established. Bronchus biopsies confirmed the diagnosis. The patient was also treated by corticosteroids. The authors wonder whether sarcoidosis is actually or apparently rare in Negro-Africans, owing to the fact of frequent failure to recognize this affection, or confusion with tuberculosis. Correct incidence of sarcoidosis in Negro-Africans should be defined.

  16. Bidirectional ventricular tachycardia in cardiac sarcoidosis.

    PubMed

    Benjamin, Mina M; Hayes, Kevin; Field, Michael E; Scheinman, Melvin M; Hoffmayer, Kurt S

    2017-02-01

    A 73-year-old man with history of pulmonary sarcoidosis was found to have runs of non-sustained bidirectional ventricular tachycardia (BVT) with two different QRS morphologies on a Holter monitor. Cardiac magnetic resonance delayed gadolinium imaging revealed a region of patchy mid-myocardial enhancement within the left ventricular basal inferolateral myocardium. An 18-fluorodeoxyglucose positron emission tomography (FDG-PET) showed increased uptake in the same area, consistent with active sarcoid, with no septal involvement. Follow-up FDG-PET one year later showed disease progression with new septal involvement. Cardiac sarcoidosis, characterized by myocardial inflammation and interstitial fibrosis that can lead to conduction system disturbance and macro re-entrant arrhythmias, should be considered in differential diagnosis of BVT. BVT may indicate septal involvement with sarcoidosis before the lesions are large enough to be detected radiologically.

  17. Pleural effusion and sarcoidosis: an unusual combination.

    PubMed

    Ferreiro, Lucía; San José, Esther; González-Barcala, Francisco Javier; Suárez-Antelo, Juan; Toubes, M Elena; Valdés, Luis

    2014-12-01

    Pleural involvement in sarcoidosis is uncommon and appears in several forms. To document the incidence and characteristics of pleural effusion in sarcoidosis patients, a review of the cases diagnosed in our centre between January 2001 and December 2012 was carried out. One hundred and ninety-five patients with sarcoidosis were identified; three (two men and one woman) presented with unilateral pleural effusion (1.5%): one in the right side and two in the left. Two were in stageii and one was in stageiv. The pleural fluid of the two patients who underwent thoracocentesis was predominantly lymphocytic. One of these patients presented chylothorax and the other had high CA-125levels. In general, these effusions are lymphocyte-rich, paucicellular, serous exudates (sometimes chylothorax) and contain proportionally higher levels of protein than LDH. Most cases are treated with corticosteroids, although it may resolve spontaneously.

  18. Advances in radionuclide imaging of cardiac sarcoidosis.

    PubMed

    Kouranos, V; Wells, A U; Sharma, R; Underwood, S R; Wechalekar, K

    2015-09-01

    Radionuclide imaging for the diagnosis and monitoring of cardiac involvement in sarcoidosis has advanced significantly in recent years. This article is based on published clinical guidelines, literature review and our collective clinical experience. Gallium-67 scintigraphy is among the diagnostic criteria for cardiac involvement in systemic sarcoidosis, and it is strongly associated with response to treatment. However, fluorine-18, 2-fluoro-deoxyglucose (FDG) positron emission tomography (PET) is now preferred both for diagnosis and for assessing prognosis. Most data are from small observational studies that are potentially biased. Quantitative imaging to assess changes in disease activity in response to treatment may lead to FDG-PET having an important routine role in managing cardiac sarcoidosis. Larger prospective studies are required, particularly to assess the effectiveness of radionuclide imaging in improving clinical management and outcome. © The Author 2015. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  19. Sarcoidosis and the heart: A review of the literature

    PubMed Central

    Ipek, Emrah; Demirelli, Selami; Ermis, Emrah; Inci, Sinan

    2015-01-01

    Summary Sarcoidosis is a chronic multisystem disorder without any defined etiology. Cardiac sarcoidosis (CS) is detected in 2–7% of patients with sarcoidosis and more than 20% of the cases of sarcoidosis are clinically silent. Cardiac involvement in systemic sarcoidosis (SS) and isolated cardiac sarcoidosis (iCS) are associated with arrhythmia and severe heart failure (HF) and have a poor prognosis. Early diagnosis of CS and prompt initiation of corticosteroid therapy with or without other immunosuppressants is crucial. Electrocardiography, Holter monitoring, and Doppler echocardiography with speckle tracking imaging can serve as the initial steps to diagnosis of CS. Cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET) are promising techniques for both diagnosis and follow-up of CS. This review discusses the main aspects of cardiac involvement in sarcoidosis. PMID:26668777

  20. [Sarcoidosis flare after autologous stem cell transplantation: An immune paradox?

    PubMed

    Marchal, A; Charlotte, F; Maksud, P; Haroche, J; Lifferman, F; Miyara, M; Choquet, S; Amoura, Z; Cohen Aubart, F

    2017-09-01

    Sarcoidosis is a systemic granulomatous disorder of unknown cause. Apparition or flare of previously diagnosed sarcoidosis following hematopoietic stem cell transplantation (HSCT) has rarely been reported. We report a 62-year-old woman who presented a radiological flare of sarcoidosis post-autologous stem cell transplantation for a POEMS syndrome. Imaging findings and lymph node histology, which revealed non-caseating granuloma, were consistent with the sarcoidosis diagnosis. The patient was asymptomatic and was kept free of treatment. Sarcoidosis must be considered ahead of compatible clinicoradiological presentation occurring after HSCT. Sarcoidosis can mimic metastatic cancer or lymphatic relapse. Tissue biopsies and exclusion of differential diagnosis of granuloma diseases are warranted to confirm sarcoidosis diagnosis. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  1. Oral Antimycobacterial Therapy in Chronic Cutaneous Sarcoidosis

    PubMed Central

    Drake, Wonder P.; Oswald-Richter, Kyra; Richmond, Bradley W.; Isom, Joan; Burke, Victoria E.; Algood, Holly; Braun, Nicole; Taylor, Thyneice; Pandit, Kusum V.; Aboud, Caroline; Yu, Chang; Kaminski, Naftali; Boyd, Alan S.; King, Lloyd E.

    2014-01-01

    IMPORTANCE Sarcoidosis is a chronic granulomatous disease for which there are limited therapeutic options. This is the first randomized, placebo-controlled study to demonstrate that antimycobacterial therapy reduces lesion diameter and disease severity among patients with chronic cutaneous sarcoidosis. OBJECTIVE To evaluate the safety and efficacy of once-daily antimycobacterial therapy on the resolution of chronic cutaneous sarcoidosis lesions. DESIGN AND PARTICIPANTS A randomized, placebo-controlled, single-masked trial on 30 patients with symptomatic chronic cutaneous sarcoidosis lesions deemed to require therapeutic intervention. SETTING A tertiary referral dermatology center in Nashville, Tennessee. INTERVENTIONS Participants were randomized to receive either the oral concomitant levofloxacin, ethambutol, azithromycin, and rifampin (CLEAR) regimen or a comparative placebo regimen for 8 weeks with a 180-day follow-up. MAIN OUTCOMES AND MEASURES Participants were monitored for absolute change in lesion diameter and decrease in granuloma burden, if present, on completion of therapy. OBSERVATIONS In the intention-to-treat analysis, the CLEAR-treated group had a mean (SD) decrease in lesion diameter of −8.4 (14.0) mm compared with an increase of 0.07 (3.2) mm in the placebo-treated group (P = .05). The CLEAR group had a significant reduction in granuloma burden and experienced a mean (SD) decline of −2.9 (2.5) mm in lesion severity compared with a decline of −0.6 (2.1) mm in the placebo group (P = .02). CONCLUSIONS AND RELEVANCE Antimycobacterial therapy may result in significant reductions in chronic cutaneous sarcoidosis lesion diameter compared with placebo. These observed reductions, associated with a clinically significant improvement in symptoms, were present at the 180-day follow-up period. Transcriptome analysis of sarcoidosis CD4+ T cells revealed reversal of pathways associated with disease severity and enhanced T-cell function following T

  2. Right Heart Involvement in Patients with Sarcoidosis.

    PubMed

    Patel, Mita B; Mor-Avi, Victor; Murtagh, Gillian; Bonham, Catherine A; Laffin, Luke J; Hogarth, Douglas Kyle; Medvedofsky, Diego; Lang, Roberto M; Patel, Amit R

    2016-05-01

    The left ventricle (LV) is affected in 20-25% of patients with sarcoidosis and its involvement is associated with morbidity and mortality. However, effects of sarcoidosis on the right ventricle (RV) are not well documented. Our aims were to investigate the prevalence of RV dysfunction in patients with sarcoidosis and determine whether it is predominantly associated with direct cardiac involvement, severity of lung disease, or pulmonary hypertension (PH). We identified 50 patients with biopsy-proven extra-cardiac sarcoidosis and preserved LV function, who underwent echocardiography, pulmonary function (PF) testing, and cardiovascular magnetic resonance. RV function was quantified by free wall longitudinal strain. Tricuspid valve Doppler and estimated right atrial pressure were used to estimate systolic pulmonary artery pressure. Myocardial late gadolinium enhancement was considered diagnostic for cardiac sarcoidosis and assumed to involve both ventricles. Of the 50 patients, 28 (56%) had RV dysfunction, 4 with poorly defined PF status. Of the remaining 24 patients, 16 (67%) had lung disease, 8 (33%) had PH, and 10 (42%) had LV involvement. Ten patients had greater than one of these findings, and 4 had all 3. In contrast, in 4/24 patients (17%), RV dysfunction could not be explained by these mechanisms, despite severely reduced RV strain. In conclusion, RV dysfunction is common in patients with sarcoidosis and is usually associated with either direct LV involvement, lung disease, or PH, but may occur in the absence of these mechanisms, suggesting the possibility of isolated RV involvement and underscoring the need for imaging protocols that would include RV strain analysis. © 2016, Wiley Periodicals, Inc.

  3. [Osteo-articular manifestations of sarcoidosis].

    PubMed

    Alaoui, Fatima-Zohra; Talaoui, Maha; Benamour, Saida

    2005-01-15

    Osteo-articular sarcoidosis may be evoked in the presence of peripheral articular manifestations or bone lesions that are sometimes asymptomatic. The aim of this work is to describe clinical and progressive features of sarcoidosis with osteo-articular involvement. Our retrospective study concerned 18 patients presenting with osteo-articular sarcoidosis from 1985 to 1999. We included patients with clinical diagnosis suggestive of sarcoidosis and with at least one positive biopsy. Among 35 cases of sarcoidosis, 18 patients had an osteo-articular manifestation (51.42%), which revealed the disease in 2 patients. The female sex was predominant (sex ratio M/F of 0.12), the mean age was 47 years and the time before diagnosis was 3.6 years. Articular involvement was the most frequent. Inflammatory joint pains were present in 11 cases, a Lofgren syndrome in 2 cases, a chronic arthritis in 4 cases and acute monoarthritis of the elbow in 1 case. A female patient exhibited a probable association with a spondylarthropathy. The bone involvement, revealing the disease in 1 case, was also noted in 5 cases, located exclusively on hands; this sarcoidosal dactyly was represented in 2 cases in the form of phalangeal geodes, in wired form (2 cases) and in large bulla form (1 case). The bone biopsy when it was performed was positive in all 3 cases. The patients responded well to corticosteroids. The osteo-articular involvement of sarcoidosis is polymorphic and can reveal the disease or may appear during the course of its progression.

  4. Cardiac Involvement in Sarcoidosis: Evolving Concepts in Diagnosis and Treatment

    PubMed Central

    Lynch, Joseph P.; Hwang, Jennifer; Bradfield, Jason; Fishbein, Michael; Shivkumar, Kalyanam; Tung, Roderick

    2014-01-01

    Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy. PMID:25007089

  5. Coexistence of Sarcoidosis and Gouty Arthritis.

    PubMed

    Semiz, Hüseyin; Kobak, Senol

    2017-08-21

    Sarcoidosis is an inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It may present with bilateral hilar lymphadenopathy, skin lesions, the involvement of eye and symptoms on the locomotor system. Gouty arthritis is an autoinflammatory disease characterized by hyperuricemia, recurrent arthritis attacks and the deposition of monosodium urate crystals in the joints and the surrounding tissues. We reported the coexistence of sarcoidosis and gouty arthritis in this paper. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  6. Treatment of neuro-ophthalmic sarcoidosis.

    PubMed

    Frohman, Larry P

    2015-03-01

    Because of the rarity of neuro-ophthalmic sarcoidosis, there are no therapeutic guidelines based on evidence-based medicine for this disorder. Review of literature combined with personal experience. Corticosteroids are the preferred initial therapy for neuro-ophthalmic sarcoidosis. If patients cannot tolerate the requisite dose of corticosteroid needed to control their disease, or if corticosteroids fail to adequately control the disease process, the choices of a second agent are based on the consideration of rapidity of clinical response and the safety profile. Although methotrexate and mycophenolate mofetil are the medications that are often selected after corticosteroid failure, more rapidly acting agents that have been used are infliximab and intravenous cyclophosphamide.

  7. Sarcoidosis Blood Transcriptome Reflects Lung Inflammation and Overlaps with Tuberculosis

    PubMed Central

    Solberg, Owen D.; Peng, Jeffrey C.; Bhakta, Nirav R.; Nguyen, Christine P.; Woodruff, Prescott G.

    2011-01-01

    Rationale: Sarcoidosis is a granulomatous disease of unknown etiology, although M. tuberculosis may play a role in the pathogenesis. The traditional view holds that inflammation in sarcoidosis is compartmentalized to involved organs. Objectives: To determine whether whole blood gene expression signatures reflect inflammatory pathways in the lung in sarcoidosis and whether these signatures overlap with tuberculosis. Methods: We analyzed transcriptomic data from blood and lung biopsies in sarcoidosis and compared these profiles with blood transcriptomic data from tuberculosis and other diseases. Measurements and Main Results: Applying machine learning algorithms to blood gene expression data, we built a classifier that distinguished sarcoidosis from health in derivation and validation cohorts (92% sensitivity, 92% specificity). The most discriminative genes were confirmed by quantitative PCR and correlated with disease severity. Transcript profiles significantly induced in blood overlapped with those in lung biopsies and identified shared dominant inflammatory pathways (e.g., Type-I/II interferons). Sarcoidosis and tuberculosis shared more overlap in blood gene expression compared with other diseases using the 86-gene signature reported to be specific for tuberculosis and the sarcoidosis signature presented herein, although reapplication of machine learning algorithms could identify genes specific for sarcoidosis. Conclusions: These data indicate that blood transcriptome analysis provides a noninvasive method for identifying inflammatory pathways in sarcoidosis, that these pathways may be leveraged to complement more invasive procedures for diagnosis or assessment of disease severity, and that sarcoidosis and tuberculosis share overlap in gene regulation of specific inflammatory pathways. PMID:21852540

  8. [Sarcoidosis of the lacrimal sac as a cause of dacriostenosis].

    PubMed

    Burduk, Paweł K; Seredyka-Burduk, Małgorzata; Kaźmierczak, Wojciech; Dalke, Krzysztof

    2013-01-01

    Sarcoidosis is a systemic granulomatous disease of unknown aetiology. The course of sarcoidosis ranges from asymptomatic to severe. It could affect people of all ages. The disease affects predominantly the lungs and lymph nodes, skin, abdominal organs or eyes. Sarcoidosis of lacrimal sac is very uncommon. We describe a young man with lacrimal sac and nasal mucosa sarcoidosis present with epiphora. Few years before the onset of dacriocystitis the patient was treated because of lymph nodes sarcoidosis. The lacrimal sac sarcoidosis developed five years after systemic disease remission. An endoscopic dacriocystorhinostomy with intubation of nasolacrimal duct was performed. We used postoperatively systemic and local steroids for 3 months period. The patient is under observation for over four years with good health. The abnormal looking nasal or lacrimal sac mucosa could be associated with granulomatous disease as sarcoidosis and Wegener's or malignancies. If the sarcoidosis is established, it could be the first manifestation of sarcoidosis leading to future systemic sarcoidosis development or it may develop many years after the onset of systemic disease. Endoscopic dacriocystorhinostomy with intubation followed by intensive therapy with systemic and local corticosteroids may be helpful to maintain lacrimal drainage patency.

  9. Ocular toxocariasis.

    PubMed

    Arevalo, J Fernando; Espinoza, Juan V; Arevalo, Fernando A

    2013-01-01

    Ocular toxocariasis is an uncommon worldwide parasitic infection that affects mostly children and is found in both rural and metropolitan areas. In many parts of the world, parasitic infections of the eye are a major cause of blindness. The diagnosis of toxocariasis is essentially clinical, based on the lesion morphology and supportive laboratory data such as serum enzyme-linked immunosorbent assay (ELISA) titers and ELISA Toxocara titers on aqueous humor; other diagnostic methods are imaging studies including optical coherence tomography, fluorescein angiography, computed tomography, and ocular ultrasound. Treatment is directed at complications arising from intraocular inflammation and vitreous membrane traction. Early vitrectomy may be of value both diagnostically and therapeutically.

  10. Ocular Pathology.

    PubMed

    Bauer, Bianca S

    2015-08-01

    Although not comprehensive of all ocular conditions in the equine species, this article concentrates on various ophthalmic conditions observed in the horse where laboratory diagnostics are recommended. The importance of laboratory diagnostic testing cannot be underestimated with equine ophthalmic disease. In many cases, laboratory diagnostics can aid in obtaining an early diagnosis and determining appropriate therapy, which in turn, can provide a better prognosis. In unfortunate cases where ocular disease results in a blind, painful eye necessitating enucleation, light microscopic evaluation is imperative to determine or confirm the cause of the blindness and provide a prognosis for the contralateral eye.

  11. Parathyroid-hormone-related protein in sarcoidosis.

    PubMed Central

    Zeimer, H. J.; Greenaway, T. M.; Slavin, J.; Hards, D. K.; Zhou, H.; Doery, J. C.; Hunter, A. N.; Duffield, A.; Martin, T. J.; Grill, V.

    1998-01-01

    Parathyroid-hormone-related protein (PTHrP) is the main mediator of the humoral hypercalcemia of malignancy. It is also detected in many normal adult and fetal tissues. Altered calcium metabolism occurs in sarcoidosis, and two cases of sarcoidosis with hypercalcemia and elevated plasma PTHrP are described. An archival study of 20 lymph node biopsies with the pathological diagnosis of sarcoidosis was performed. Immunohistochemistry using a polyclonal antiserum to human PTHrP and in situ hybridization using a riboprobe to human PTHrP were performed on the lymph node biopsies. Immunohistochemistry for PTHrP was also performed on the biopsies from the two cases with elevated plasma levels. Immunohistochemical analysis detected PTHrP in macrophages within granulomata in 17 of the 20 (85%) biopsies. In situ hybridization detected a positive signal for messenger RNA in the granulomata of 11 of 19 (58%) biopsies. PTHrP immunoreactivity and PTHrP gene expression are present in sarcoid granulomata. PTHrP may contribute to the hypercalcemia of sarcoidosis. Images Figure 1 PMID:9422518

  12. Tissue Doppler imaging in cardiac sarcoidosis.

    PubMed

    Smedema, J P

    2008-07-01

    A middle-aged African lady, who presented with ventricular tachycardias, mitral valve regurgitation and congestive heart failure, was diagnosed with cardiac sarcoidosis. Tissue Doppler imaging demonstrated abnormalities suggestive of myocardial scar, which was confirmed by contrast-enhanced cardiac magnetic resonance.

  13. [Cardiac sarcoidosis: Diagnosis and therapeutic challenges].

    PubMed

    Cohen Aubart, F; Nunes, H; Mathian, A; Haroche, J; Hié, M; Le-Thi Huong Boutin, D; Cluzel, P; Soussan, M; Waintraub, X; Fouret, P; Valeyre, D; Amoura, Z

    2017-01-01

    Sarcoidosis is a granulomatous disorder of unknown cause characterized by non-caseating granuloma in young adults. Cardiac involvement is rare and range from 2 to 75% depending on diagnostic criteria. Cardiac involvement in sarcoidosis may be asymptomatic or may manifest as rhythm/conduction troubles or congestive heart failure. The diagnosis and treatment of cardiac sarcoidosis may be challenging. However, advances have come in recent years from the use of cardiac MRI and (18)FDG-TEP scanner, as well as from the stratification of the risk of ventricular tachycardia/fibrillation. Due to the rarity of the disease, there is no reliable prospective large study to guide therapeutic strategy for cardiac sarcoidosis. Corticosteroids are probably efficacious, in particular in case of atrio-ventricular block or moderate heart failure. Immunosuppressive drugs have not been largely studied but methotrexate could be helpful. In refractory forms, TNF-α antagonists have been used with success. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  14. Systemic sarcoidosis mimicking malignant metastatic disease

    PubMed Central

    Hammen, Irena; Sherson, David Lee; Davidsen, Jesper Roemhild

    2015-01-01

    We present a case of systemic sarcoidosis involving the liver, pancreas, lungs, mediastinal and intraabdominal lymph nodes and bones. Multiple organ system manifestations mimicked malignant metastatic disease. The diagnosis was established with clinical, radiological, and pathological findings after neoplasm was ruled out by pathological tests. The patient showed rapid symptom remission with systemic steroid treatment. PMID:26672956

  15. Sarcoidosis of the vagina treated with methotrexate.

    PubMed

    Şahin, N; Solak, A; Karaarslan, S; Doruk, S

    2016-06-01

    We describe the first case of mediolateral episiotomic sarcoidosis of the deep vaginal tissue, without involvement of the pulmonary parenchyma or pulmonary symptoms. A 68-year-old female was admitted with a vaginal mass that had developed about 1 month prior. On bimanual examination, we found a painful solid mass approximately 4 cm in diameter on the episiotomy line of the deep vaginal tissue. The patient underwent pelvic magnetic resonance imaging on suspicion of a malignancy, and a vaginal true-cut biopsy was performed. The biopsy specimen exhibited non-caseating, granulomatous inflammation and many multinucleated giant cells, strongly suggesting sarcoidosis. We had excluded other granulomatous diseases; a final diagnosis was made of stage-1 sarcoidosis in the deep vaginal mass. A 3-month course of methotrexate (2.5 mg/week) was commenced to treat a flare-up of rheumatoid arthritis. The vaginal mass resolved. To the best of our knowledge, this is the first case of sarcoidosis in a deep vaginal mass without pulmonary parenchymal or other solid-organ involvement.

  16. Sarcoidosis: assessment of disease severity using HRCT.

    PubMed

    Drent, Marjolein; De Vries, Jolanda; Lenters, Merinke; Lamers, Rob J S; Rothkranz-Kos, Snjezana; Wouters, Emiel F M; van Dieijen-Visser, Marja P; Verschakelen, Johny A

    2003-11-01

    The value of high-resolution computed tomography (HRCT) in diagnosing and assessing inflammatory activity in sarcoidosis is well established. The aim of the present study was to address the intra- and inter-observer agreements of the HRCT score by Oberstein et al. [8], and to evaluate the relationship between HRCT findings and disease severity expressed in respiratory functional impairment in sarcoidosis. The clinical records of 80 known sarcoidosis patients visiting the outpatient clinic between January 2000 and August 2001, who underwent a HRCT as well as lung function tests (including exercise testing), were reviewed. Two readers scored the first 60 HRCT images twice. Weighted kappa and intra-class correlation coefficient were used to assess the reliability of the HRCT scoring system. Spearman's rank correlation coefficients and multiple regression analyses were performed to evaluate the relationship between HRCT findings (first reading, reader A) and respiratory functional impairment. Intra- and inter-reader reliability demonstrated good agreement. All HRCT subscores, except enlargement of lymph nodes, were correlated to the FEV(1), FVC, DLco, Pao(2)max (all p<0.05) and A-aPo(2 )max ( p<0.001). Furthermore, HRCT abnormalities, but not the chest radiographic stage, were strongly associated with functional parameters. Abnormal changes of lung parenchyma, established by HRCT features, were associated with respiratory functional impairment in sarcoidosis. Moreover, compared with the radiographic stages, HRCT findings appeared to be much more sensitive in depicting respiratory disability, especially abnormal gas exchange.

  17. Cardiac manifestations of sarcoidosis: diagnosis and management.

    PubMed

    Birnie, David H; Kandolin, Riina; Nery, Pablo B; Kupari, Markku

    2017-09-14

    Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Cardiac symptoms are usually dominant over extra-cardiac as most patients with clinically manifest disease have minimal extra-cardiac disease and up to two-thirds have isolated cardiac sarcoidosis (CS). It is estimated that another 20-25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In addition, the extent of myocardial late gadolinium enhancement is emerging as an important prognostic factor. The literature shows some controversy regarding outcomes for patients with clinically silent CS and larger studies are needed. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest CS despite minimal data supporting it. Fluorodeoxyglucose Positron Emission Tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.

  18. Management of extrapulmonary sarcoidosis: challenges and solutions

    PubMed Central

    Al-Kofahi, Khalid; Korsten, Peter; Ascoli, Christian; Virupannavar, Shanti; Mirsaeidi, Mehdi; Chang, Ian; Qaqish, Naim; Saketkoo, Lesley A; Baughman, Robert P; Sweiss, Nadera J

    2016-01-01

    Background Sarcoidosis is a chronic multisystem disease of unknown etiology characterized by noncaseating granulomas that most often involves the lungs, but frequently has extrapulmonary manifestations, which might be difficult to treat in individual patients. Objective To review different disease manifestations, focusing on extrapulmonary organ systems, and to provide treatment options for refractory cases. Materials and methods We performed a literature search using Medline and Google Scholar for individual or combined keywords of “sarcoidosis, extrapulmonary, treatment, kidney, neurosarcoidosis, cardiovascular, gastrointestinal, transplantation, musculoskeletal, rheumatology, arthritis, and skin”. Peer-reviewed articles, including review articles, clinical trials, observational trials, and case reports that were published in English were included. References from retrieved articles were also manually searched for relevant articles. Results and conclusion Isolated involvement of a single organ or organ system is rare in sarcoidosis, and thus all patients must be thoroughly evaluated for additional disease manifestations. Cardiac sarcoidosis and neurosarcoidosis may be life-threatening. Clinicians need to assess patients comprehensively using clinical, laboratory, imaging, and histopathological data to recommend competently the best and least toxic treatment option for the individual patient. PMID:27853374

  19. Coronary flow reserve is reduced in sarcoidosis.

    PubMed

    Kul, Seref; Kutlu, Gonul Aciksari; Guvenc, Tolga Sinan; Kavas, Murat; Demircioglu, Kenan; Yilmaz, Yusuf; Yakar, Halil Ibrahim; Kanbay, Asiye; Boga, Sibel; Caliskan, Mustafa

    2017-09-01

    Sarcoidosis is a multisystem disease with frequent cardiac involvement, albeit manifest cardiac disease is rare. Though epicardial coronary arteries are not frequently involved, microvascular disease is rather common in both symptomatic and asymptomatic patients. The mechanism of microvascular involvement has not been elaborated yet. The aim of this study is to investigate coronary flow velocity reserve (CFVR) using transthoracic echocardiography in patients with sarcoidosis but without known atherosclerotic coronary artery disease or risk factors for atherosclerosis. A total of 40 patients with sarcoidosis and 42 healthy volunteers without any known medical conditions were enrolled prospectively. Diastolic peak coronary flow velocities were measured during rest and maximal hyperemia induced with adenosine. Patients within the sarcoidosis group had significantly higher diastolic peak velocity at rest (29.5 ± 5.8 vs. 22.8 ± 3.2, p < 0.01) but both the diastolic peak velocity during hyperemia (60.5 ± 18.2 vs. 68.9 ± 15.7, p = 0.03) and CFVR (2.08 ± 0.57 vs. 3.03 ± 0.60, p < 0.01) were lower compared to controls. Sarcoidosis was an independent predictor for low (≤2.0) CFVR (OR: 56.8, 95%CI: 6.1-531.7, p < 0.001), along with age and systolic blood pressure. For patients with sarcoidosis, age and systolic blood pressure were independent predictors for a low CFVR. Despite a lack of known risk factors for atherosclerosis, patients with sarcoidosis had lower CFVR compared to healthy controls, thus suggesting a dysfunction in the coronary microvasculature. A reduced response to vasodilators suggests possible structural alterations of the myocardial microvasculature, rather than being secondary to microvascular spasm as suggested previously. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Ocular phototherapy.

    PubMed

    Singh, A D

    2013-02-01

    Phototherapy can be translated to mean 'light or radiant energy-induced treatment.' Lasers have become the exclusive source of light or radiant energy for all applications of phototherapy. Depending on the wavelength, intensity, and duration of exposure, tissues can either absorb the energy (photocoagulation, thermotherapy, and photodynamic therapy (PDT)) or undergo ionization (photodisruption). For phototherapy to be effective, the energy has to be absorbed by tissues or more specifically by naturally occurring pigment (xanthophyll, haemoglobin, and melanin) within them. In tissues or tumours that lack natural pigment, dyes (verteporphin, Visudyne) with narrow absorption spectrum can be injected intravenously that act as focal absorbent of laser energy after they have preferentially localized within the tumour. Ocular phototherapy has broad applications in treatment of ocular tumours. Laser photocoagulation, thermotherapy, and PDT can be delivered with low rates of complications and with ease in the outpatient setting. Review of the current literature suggests excellent results when these treatments are applied for benign tumours, particularly for vascular tumours such as circumscribed choroidal haemangioma. For primary malignant tumours, such as choroidal melanoma, thermotherapy, and PDT do not offer local tumour control rates that are equivalent or higher than those achieved with plaque or proton radiation therapy. However, for secondary malignant tumours (choroidal metastases), thermotherapy and PDT can be applied as a palliative treatment. Greater experience is necessary to fully comprehend risks, comparative benefits, and complication of ocular phototherapy of ocular tumours.

  1. Old-scar mass and changing surgical perspective: Sarcoidosis

    PubMed Central

    Aktimur, Recep; Aktimur, Sude Hatun; Çolak, Elif; Alıcı, Ömer; Demirağ, Mehmet Derya

    2015-01-01

    Sarcoidosis is a systemic inflammatory disease of an unknown etiology. Skin is involved in 25% of all cases, and 29% of them present as a scar sarcoidosis. Asymptomatic old-scar masses are generally regarded as a foreign body reaction by surgeons and often result in excisional biopsy. We describe a case of a patient who developed sarcoidosis in a 34-year-old appendectomy scar and adjacent inguinal lymph nodes without any local or systemic symptom and radiologic finding. Surgeons should not underestimate the importance of such lesions as a simple condition. Scar sarcoidosis may resolve spontaneously, or the treatment with some topical agents is effective. Furthermore, scar sarcoidosis may be the initial manifestation of systemic sarcoidosis. PMID:25931946

  2. Etiology of Sarcoidosis: Does Infection Play a Role?

    PubMed Central

    Saidha, Shiv; Sotirchos, Elias S.; Eckstein, Christopher

    2012-01-01

    Sarcoidosis is a granulomatous inflammatory disorder of unclear etiology, which is known to affect multiple organ systems including the lungs, heart, skin, central nervous system, and eyes, among others. For this reason, sarcoidosis represents a systemic medical disorder that is clinically relevant to multiple medical sub-specialties. Despite extensive research, the etiology of sarcoidosis has yet to be elucidated, although most evidence supports that the pathogenetic mechanism of sarcoidosis is an aberrant immune response, driven by an unidentified antigen (or antigens) in genetically susceptible individuals. Multiple candidate etiologic agents, including microbial organisms and environmental agents, have been investigated, but study results are inconclusive. In this review, we describe the known histologic and immunologic features of sarcoidosis and discuss the evidence supporting a role for infectious processes in the pathogenesis of sarcoidosis. PMID:22461752

  3. Venous Leg Ulcer in a Sarcoidosis Patient: A Case Report

    PubMed Central

    Ohn, Jungyoon; Byun, Sang Young; Kim, In Su

    2015-01-01

    Venous leg ulcers, the most common form of leg ulcers, are relevant to the pathogenicity of pericapillary fibrin cuff. Sarcoidosis, a multiorgan granulomatous disease, causes fibrin deposition in tissues. We report a case of a 50-year-old man with venous leg ulcers coexisting with sarcoidosis. On the basis of the histologic findings, we propose the hypothesis that sarcoidosis patients are prone to the development of venous leg ulcers. PMID:26719645

  4. Sarcoidosis presenting as isolated gingival enlargement: a rare case entity.

    PubMed

    Tripathi, Pragya; Aggarwal, Jaihans; Chopra, Deepak; Bagga, Sukhchain; Sethi, Kanika

    2014-11-01

    Sarcoidosis is a non-caseating granulomatous disease . It is a multiorgan inflammatory disorder of unknown etiology. Conditions affecting skin or other organs frequently involve oral cavity and rarely manifest as gingival disease. Here we are reporting a rare case in which gingival hyperplasia was the initial symptom which finally led to the diagnosis of sarcoidosis. Oral mucous membrane needs to be examined carefully as it may constitute in presenting first sign of systemic sarcoidosis.

  5. Tenth international conference on sarcoidosis and other granulomatous disorder

    SciTech Connect

    Johns, C.J.

    1986-01-01

    This book contains papers divided among three sections: Basic Mechanisms of Sarcoidosis; Other Granulomatous Disorders; and Clinical Studies of Sarcoidosis. Some of the paper titles are: Radionuclides in Detecting Active Granuloma Formation: Gallium-67 Scintigraphy and Histopathology with Autoradiographic; a European Survey on the Usefulness of /sup 67/Ga Lung Scans in Assessing Sarcoidosis: Experience in 14 Research Centers in Seven Different Countries; and Reassessing the Standard Chest Radiograph for Intraparenchymal Activity.

  6. Sarcoidosis presenting as multiple pulmonary nodules and nephrotic syndrome.

    PubMed Central

    Vidal, F.; Oliver, J. A.; Campanya, E.; Baguer, A.; Carrera, M.; Richart, C.

    1986-01-01

    A 56 year old man presented with thrombophlebitis, nephrotic range proteinuria and multiple pulmonary nodules. A renal biopsy showed membranous glomerulonephritis, and after a thoracotomy a diagnosis of sarcoidosis was established. A pulmonary nodular pattern is unusual in sarcoidosis and is often mistaken for malignant disease. The association of this type of pulmonary involvement and membranous glomerulonephritis as the presenting form of sarcoidosis has not previously been described. Images Figure 1 PMID:3658854

  7. [Sarcoidosis presenting with Raynaud's syndrome: a case report].

    PubMed

    Nigro, A; Iannone, F; Grattagliano, V; Sanguedolce, F; Lapadula, G

    2004-01-01

    We report a case of association between sarcoidosis and Raynaud's syndrome. A 39 year old female presented fatigue, Raynaud's syndrome, IgG and erithrosedimentation rate (ESR) increase, polyarthralgy in which disseminated micronodular infiltration in the chest X-ray and histological demonstration of non-caseating epitheloid microgranulomas led to a diagnosis of concomitant sarcoidosis. Clues to the diagnosis of sarcoidosis coexisting with autoimmune disease are discussed.

  8. New molecular targets for the treatment of sarcoidosis.

    PubMed

    Chiarchiaro, Jared; Chen, Bill B; Gibson, Kevin F

    2016-09-01

    Sarcoidosis is a chronic granulomatous disease typically affecting the lung, lymph nodes, and other organ systems. Evidence suggests that the morbidity and mortality rates for sarcoidosis in the USA are rising, despite widespread use of anti-inflammatory therapies. In this review, we survey new therapies that target specific inflammatory pathways in other diseases (such as rheumatoid arthritis, Crohn's disease, and psoriasis) that are similar to pathways relevant to sarcoidosis immunopathogenesis, and therefore, represent potentially new sarcoidosis therapies. Immunopathogenesis of sarcoidosis has been well elucidated over the past few years. There is abundant evidence for T-cell activation in sarcoidosis leading to activation of both Th1 and Th17 inflammatory cascades. Therapies targeting T-cell activation, Th1 pathways (such as the interleukin-6 inhibitors), Th17 pathway mediators, and others have been Food and Drug Administration approved or under investigation to treat a variety of autoimmune inflammatory diseases, but have not been studied in sarcoidosis. Targeting the p38 mitogen-activated protein kinases and the ubiquitine proteasome system with new agents may also represent a novel therapeutic option for patients with sarcoidosis. Rising morbidity and mortality rates for patients with sarcoidosis strongly support the need to develop more effective anti-inflammatory therapies to treat chronic disease.

  9. Histologic Review of Sarcoidosis in a Neck Lymph Node.

    PubMed

    Welter, Shannon M; DeLuca-Johnson, Javier; Thompson, Keith

    2017-08-29

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology. It may occur at any age, but is most commonly seen in young to middle age adults. Sarcoidosis remains more common in women regardless of geographic or racial boundaries. Although the etiology remains unclear, the most common explanation is that sarcoidosis is a disease of immunologic dysregulation triggered by an as yet unidentified environmental or microbial antigen in genetically susceptible persons. We review a case of sarcoidosis with was initially diagnosed in a neck lymph node that was removed for evaluation of metastatic squamous cell carcinoma with a discussion on the clinical and histologic characteristic of the disease.

  10. New molecular targets for the treatment of sarcoidosis

    PubMed Central

    Chiarchiaro, Jared; Chen, Bill B.; Gibson, Kevin F.

    2016-01-01

    Purpose of review Sarcoidosis is a chronic granulomatous disease typically affecting the lung, lymph nodes, and other organ systems. Evidence suggests that the morbidity and mortality rates for sarcoidosis in the USA are rising, despite widespread use of anti-inflammatory therapies. In this review, we survey new therapies that target specific inflammatory pathways in other diseases (such as rheumatoid arthritis, Crohn’s disease, and psoriasis) that are similar to pathways relevant to sarcoidosis immunopathogenesis, and therefore, represent potentially new sarcoidosis therapies. Recent findings Immunopathogenesis of sarcoidosis has been well elucidated over the past few years. There is abundant evidence for T-cell activation in sarcoidosis leading to activation of both Th1 and Th17 inflammatory cascades. Therapies targeting T-cell activation, Th1 pathways (such as the interleukin-6 inhibitors), Th17 pathway mediators, and others have been Food and Drug Administration approved or under investigation to treat a variety of autoimmune inflammatory diseases, but have not been studied in sarcoidosis. Targeting the p38 mitogen-activated protein kinases and the ubiquitine proteasome system with new agents may also represent a novel therapeutic option for patients with sarcoidosis. Summary Rising morbidity and mortality rates for patients with sarcoidosis strongly support the need to develop more effective anti-inflammatory therapies to treat chronic disease. PMID:27454074

  11. Current and emerging pharmacological treatments for sarcoidosis: a review

    PubMed Central

    Beegle, Scott H; Barba, Kerry; Gobunsuy, Romel; Judson, Marc A

    2013-01-01

    The treatment of sarcoidosis is not standardized. Because sarcoidosis may never cause significant symptoms or organ dysfunction, treatment is not mandatory. When treatment is indicated, oral corticosteroids are usually recommended because they are highly likely to be effective in a relative short period of time. However, because sarcoidosis is often a chronic condition, long-term treatment with corticosteroids may cause significant toxicity. Therefore, corticosteroid sparing agents are often indicated in patients requiring chronic therapy. This review outlines the indications for treatment, corticosteroid treatment, and corticosteroid sparing treatments for sarcoidosis. PMID:23596348

  12. Validation of the King's Sarcoidosis Questionnaire (KSQ) in a Dutch sarcoidosis population.

    PubMed

    Van Manen, Mirjam J G; Wapenaar, Monique; Strookappe, Bert; Drent, Marjolein; Elfferich, Marjon; de Vries, Jolanda; Gosker, Harry R; Birring, Surinder S; Patel, Amit S; van den Toorn, Leon; van den Blink, Bernt; Boomars, Karin; Hoitsma, Elske; Wijsenbeek, Marlies S

    2016-03-29

    The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing health status using five modules (General Health Status, Lung, Eyes, Skin, Medication) in patients with sarcoidosis. The KSQ was only validated in one English sarcoidosis cohort. The aim of this study was to validate the KSQ in a Dutch sarcoidosis population. The KSQ was translated according to international guidelines and tested in interviews with patients. Consecutive outpatients completed multiple questionnaires twice, two weeks apart. Construct validity, internal consistency and repeatability were determined. Of the 98 patients included 85 had lung, 22 skin and 24 eye disease. There was good construct validity of the KSQ General Health Status module against the World Health Organization Quality of Life-BREF questionnaire. The Medication module correlated weak to moderate with most questionnaires. The correlations with organ-specific questionnaires varied from strong for Eyes (r=0.75), Skin (r=-0.62) to moderate for Lung (r=-0.45 with MRC breathlessness scale). Internal consistency was good for all KSQ modules (Cronbach's α 0.72-0.93). Intraclass correlation coefficients (0.70-0.90) and Bland-Altman plots showed good repeatability of the KSQ. The Dutch KSQ is the first translation of the English KSQ, validated in a Dutch sarcoidosis population.

  13. Ocular metastases

    PubMed Central

    Cohen, V M L

    2013-01-01

    The eye is a rare site for disseminated malignancy because of the absence of a lymphatic system. Metastases to the ocular structures occur by haematogenous spread and therefore the parts of the eye with the best vascular supply are most likely to be affected. Many patients with Stage 4 carcinomatosis (distal metastatic spread) already have a history of a previous primary cancer. However, this is not always the case for lung cancer as this can metastasise early to the uveal tract and therefore the ophthalmologist may be the first to discover the presence of terminal metastatic disease. Broadly speaking, treatment options are focused on improving the patients' quality of life if visual acuity is threatened. Long-term side effects of treatment need to be considered as systemic cancer treatments and therefore patient life expectancy is improving. In this manuscript, presented at the Cambridge symposium 2012, the diagnosis and challenges involved in the management of ocular metastases are presented. PMID:23222564

  14. Ocular onchocerciasis

    PubMed Central

    Thylefors, B.

    1978-01-01

    Well over 20 million people in the world are infected with Onchocerca volvulus and it is probable that 200 000-500 000 people are blind as a result of this infection, which is the most important cause of blindness in certain areas of Africa and Latin America. Treatment of the disease is difficult and often produces serious adverse reactions in the patient. Combined use of diethylcarbamazine citrate and suramin is still the most suitable form of treatment. Screening for the early detection of cases at high risk of ocular manifestations must be organized, and their treatment undertaken, if blindness is to be avoided. Prevention of ocular onchocerciasis is feasible, using vector control methods to reduce transmission, but the procedures are costly and may have to be maintained for many years. Research is needed to improve treatment and to find a chemoprophylactic agent or a preventive vaccine. PMID:307448

  15. Gene-environment interactions in sarcoidosis

    PubMed Central

    Culver, Daniel A.; Newman, Lee S.; Kavuru, Mani S.

    2007-01-01

    Susceptibility to most human diseases is polygenic, with complex interactions between functional polymorphisms of single genes governing disease incidence, phenotype, or both. In this context, the contribution of any discrete gene is generally modest for a single individual, but may confer substantial attributable risk on a population level. Environmental exposure can modify the effects of a polymorphism, either by providing a necessary substrate for development of human disease or because the effects of a given exposure modulate the effects of the gene. In several diseases, genetic polymorphisms have been shown to be context-dependent, i.e. the effects of a genetic variant are realized only in the setting of a relevant exposure. Since sarcoidosis susceptibility is dependent on both genetic and environmental modifiers, the study of gene-environment interactions may yield important pathogenetic information and will likely be crucial for uncovering the range of genetic susceptibility loci. However, the complexity of these relationships implies that investigations of gene-environment interactions will require the study of large cohorts with carefully-defined exposures and similar clinical phenotypes. A general principle is that the study of gene-environment interactions requires a sample size at least several-fold greater than for either factor alone. To date, the presence of environmental modifiers has been demonstrated for one sarcoidosis susceptibility locus, HLA-DQB1, in African-American families. This article reviews general considerations obtaining for the study of gene-environment interactions in sarcoidosis. It also describes the limited current understanding of the role of environmental influences on sarcoidosis susceptibility genes. PMID:17560304

  16. Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab

    PubMed Central

    Alawdah, Laila; Nahari, Ahmad; Alshahrani, Dayel; Fagih, Musa; Ghazi, Shahid; Al-Hussaini, Abdulrahman

    2016-01-01

    Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months with hematemesis and development of hepatosplenomegaly. The second case was a 9-year-old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa. Gastric and esophageal biopsies had shown noncaseating granulomatous inflammation. The first case had histopathological evidence of granulomatous hepatitis, and both cases demonstrated lung nodularity on computed tomography chest. The boy had elevated angiotensin-converting enzyme level. Given the multisystem involvement with significant chest findings, tissue findings of granulomatous disease, and negative workup for other causes of granulomatous inflammation, both cases were diagnosed with active disseminated sarcoidosis, and treated with corticosteroids. The girl continued to be symptom-free for 4 years after tapering steroid therapy. The boy had relapses off steroids and the disease was brought into remission for 5 years off steroid therapy by infliximab. Pediatric GI sarcoidosis is a rare disease that exhibits heterogeneity in natural course. The chronic relapsing progressive form of the disease might benefit from infliximab therapy. PMID:27748327

  17. Coexistent Sarcoidosis and Tuberculosis: A Case Report.

    PubMed

    Carbonelli, Cristiano; Giuffreda, Ernesto; Palmiotti, Antonio; Loizzi, Domenico; Lococo, Filippo; Carpagnano, Elisiana; Lacedonia, Donato; Sollitto, Francesco; Foschino, Maria Pia

    2017-02-09

    Necrotizing granulomatous diseases of the lungs are usually dependent on a narrow range of differential diagnoses. Tuberculosis (TB) is responsible for the largest number of cases, while necrotizing sarcoidosis is generally considered a rare and easily distinguishable disease substantially based on histological features. However, this entity has become a viable diagnosis in the absence of mycobacteria isolation or when a remarkable clinical improvement cannot be achieved with the combination of anti-TB drugs at full dosage. The classic manifestations of TB and sarcoidosis have an overlapping range for which it is sometimes difficult to make a clinical diagnosis. Furthermore, the role of mycobacteria as a trigger antigen capable of evoking the clinical expression of sarcoidosis is a hypothesis supported by evidence from some cases. We report a case of bilateral tuberculous pleurisy in a 45-year-old male native of a North-African region with an atypical severe multisystem disease characterized by a fever resistant to anti-TB therapy and respondent to corticosteroid treatment. The choice to continue both steroid and anti-TB therapy proved to be correct for the late evidence of TB mycobacterial growth only on pleural specimens. The case described is suggestive of a coexistent systemic sarcoid manifestation and low-antigen TB, which is an underrecognized entity in the medical literature.

  18. [Morpheiform sarcoidosis as atypical manifestation of sarcoidosis. Review of the literature and differential diagnosis].

    PubMed

    Castellanos-González, María; Picazo Talavera, María Remedios

    2016-09-16

    Sarcoidosis is an idiopathic multisystem granulomatous disease that commonly involves the skin in 25% of affected patients. Because lesions assume a vast array of morphologies, a classification dividing them into specific (with presence of typical granulomas in the biopsy) or nonspecific (not containing granulomas) has been proposed. In the first group the variant morpheaform is considered exceptional. We review the cases reported in the literature and describe the possible differential diagnosis. We highlight the importance of recognizing the very atypical presentation of sarcoidosis and its ability to mimic morpheaform or sclerosis diseases in our patients.

  19. Noncoding RNAs, New Players in Pulmonary Medicine and Sarcoidosis.

    PubMed

    Salamo, Oriana; Mortaz, Esmaeil; Mirsaeidi, Mehdi

    2017-08-29

    Noncoding RNAs (ncRNAs) are coded by 98% of the human genomic DNA. They are grouped into two major classes according to their length: small ncRNAs and long ncRNAs. They regulate genome organization and stability, and physiological processes that maintain cellular homeostasis. Recently, there has emerged a large interest in ncRNAs because of their significant roles in the development of several inflammatory diseases, including sarcoidosis. Some of them were introduced as novel markers for the disease activity, such increased levels of miRNA-34a that was found in peripheral blood mononuclear cells of sarcoidosis patients reemphasizing the inflammatory component in sarcoidosis. They are also important key factors in the outcome of sarcoidosis. Dysregulation of miRNA-let7f leads to overexpression of profibrotic factors and could be related to the pathogenesis of pulmonary fibrosis in patients with sarcoidosis due to the positive effect on collagen expression and deposition. However, many unanswered questions remain about the association of ncRNAs and sarcoidosis. By understanding the functions of ncRNAs in Th1 and Th-17 cells, we may be able to uncover the mechanism of action of those cells in sarcoidosis. The diagnostic potential in noncoding RNA is remarkable. Indeed, further translational research is needed to define the RNA gene fingerprint of different sarcoidosis stages.

  20. Cardiac sarcoidosis resembling panic disorder: a case report.

    PubMed

    Tokumitsu, Keita; Demachi, Jun; Yamanoi, Yukichi; Oyama, Shigeto; Takeuchi, Junko; Yachimori, Koji; Yasui-Furukori, Norio

    2017-01-13

    Sarcoidosis is a systemic disease of unknown etiology, in which granulomas develop in various organs, including the skin, lungs, eyes, or heart. It has been reported that patients with sarcoidosis are more likely to develop panic disorder than members of the general population. However, there are many unknown factors concerning the causal relationship between these conditions. We present the case of a 57-year-old woman who appeared to have panic disorder, as she experienced repeated panic attacks induced by transient complete atrioventricular block, associated with cardiac sarcoidosis. Psychotherapy and pharmacotherapy were not effective in the treatment of her panic attacks. However, when we implanted a permanent pacemaker and initiated steroid treatment for cardiac sarcoidosis, panic attacks were ameliorated. Based on these findings, we diagnosed the patient's symptoms as an anxiety disorder associated with cardiac sarcoidosis, rather than panic disorder. This report highlights the importance of considering cardiac sarcoidosis in the differential diagnosis of panic disorder. This cardiac disease should be considered especially in patients have a history of cardiac disease (e.g., arrhythmia) and atypical presentations of panic symptoms. Panic disorder is a psychiatric condition that is typically diagnosed after other medical conditions have been excluded. Because the diagnosis of sarcoidosis is difficult in some patients, caution is required. The palpitations and symptoms of heart failure associated with cardiac sarcoidosis can be misdiagnosed as psychiatric symptoms of panic disorder. The condition described in the current case study appears to constitute a physical disease, the diagnosis of which requires significant consideration and caution.

  1. Cardiac Sarcoidosis: Is it More Common in Men?

    PubMed

    Martusewicz-Boros, Magdalena M; Boros, Piotr W; Wiatr, Elżbieta; Kempisty, Anna; Piotrowska-Kownacka, Dorota; Roszkowski-Śliż, Kazimierz

    2016-02-01

    Sarcoidosis is a systemic granulomatous disease which predominantly affects the lungs, although granulomas can also involve all other organs, including the heart. Cardiac sarcoidosis (CS) may occur at any stage of the disease and may be the cause of sudden cardiac death, even in a previously asymptomatic patient. The aim of this study was to evaluate the incidence of CS in a large group of patients diagnosed or followed up due to sarcoidosis. We performed a retrospective analysis of patients at our institution discharged with the final diagnosis "sarcoidosis" (ICD-10: D86) from January 2008 to October 2012. Only those with biopsy (from respiratory tract or lymph nodes) confirmed diagnosis of sarcoidosis were included. We then selected the subset of patients with cardiac involvement due to sarcoidosis confirmed by positive magnetic resonance imaging. The study covered 1375 consecutive sarcoidosis patients (51 % men), who were hospitalized during 5 years. Multiorgan disease was detected in 160 cases (11.7 %), and cardiac involvement was found in 64 patients (4.7 % of all), 70.3 % of whom were men. Twelve of those with CS were in stage I, 48 in stage II, and four in stage III. The odds ratio for having cardiac involvement in men compared to women was 2.3 (95 % CI 1.36-4.0, p = 0.002). Cardiac involvement in sarcoidosis was diagnosed in the similar percentage as in previously published data but was significantly more frequently in men.

  2. The association between osteopontin gene polymorphisms, osteopontin expression and sarcoidosis.

    PubMed

    Lavi, Hadas; Assayag, Miri; Schwartz, Assaf; Arish, Nissim; Fridlender, Zvi G; Berkman, Neville

    2017-01-01

    Sarcoidosis is a systemic inflammatory disease of unknown etiology. Osteopontin (SPP1, OPN) is an extra cellular matrix glycoprotein and cytokine with a known role in granuloma formation and in autoimmune and inflammatory diseases. To determine whether plasma OPN levels are elevated in patients with sarcoidosis and compare the frequency of four single nucleotide polymorphism (SNPs) variants in the OPN gene in sarcoidosis patients compared to healthy controls. Demographic and clinical information, radiological studies and pulmonary function tests were evaluated in 113 patients with sarcoidosis and in 79 healthy controls. Blood samples were analyzed for SNPs of the OPN gene and for plasma OPN and CRP levels. Association between clinical features of disease and OPN levels as well as SNP frequencies was determined. Plasma OPN levels were higher in sarcoidosis patients than in healthy subjects, (median: 217 vs 122ng/ml, p<0.001). Area under the curve for receiver operator curves (ROC) was 0.798 (0.686-0.909 95% CI.) No differences were observed between sarcoidosis patients and controls in the frequency of any of the SNPs evaluated. Presence of lung parenchymal involvement was associated with SNP distribution at rs1126772 (p = 0.02). We found no correlation between SNPs distribution and plasma OPN levels. Osteopontin protein levels are elevated in sarcoidosis. We found no evidence for an association between SNPs on the osteopontin gene and plasma OPN levels or the presence of sarcoidosis, however, an association between genotype and several phenotypic clinical parameters of disease was observed.

  3. Increased Risk of Atherosclerosis in Patients with Sarcoidosis.

    PubMed

    Bargagli, Elena; Rosi, Elisabetta; Pistolesi, Massimo; Lavorini, Federico; Voltolini, Luca; Rottoli, Paola

    2017-08-10

    Sarcoidosis is a systemic granulomatous disease of unknown etiology. Recent studies demonstrated that its pathogenesis is related with enhanced oxidative stress (protein carbonylation and lipid peroxidation) and alterations in the circulating lipid profile. Alterations of lipid metabolism (including the reduction in high-density lipoprotein cholesterol levels and apolipoprotein A1 concentrations) induce plasma membrane, bronchial and lung capillary endothelial cell damage in sarcoidosis patients. Dyslipidemia is associated with increased oxidative stress, diminished overall antioxidative protection and increased risk for atherosclerosis. Very recently increased cardiovascular biomarkers (in particular alterations of lipoprotein A and d-dimer concentrations) were observed in sarcoidosis patients, mainly in those with a high risk of atherosclerosis. Chitotriosidase, a biomarker of sarcoidosis activity and macrophage activation, is increased in serum and bronchoalveolar lavage fluid of patients with sarcoidosis as well as in patients with atherosclerosis. Lipidomics and other recent methodologies allowed the discovery of proteins involved in lipid metabolism and sarcoidosis pathogenesis, such as serum amyloid A, a biomarker of sarcoidosis activity, involved in innate immune response, inflammation and apolipoprotein metabolism. In this review lipid metabolism alteration and atherosclerosis risk in sarcoidosis patients were discussed in order to contribute to this novel and interesting research topic. © 2017 S. Karger AG, Basel.

  4. The clinical and immunologic features of pulmonary fibrosis in sarcoidosis

    PubMed Central

    PATTERSON, KAREN C.; HOGARTH, KYLE; HUSAIN, ALIYA N.; SPERLING, ANNE I.; NIEWOLD, TIMOTHY B.

    2014-01-01

    Sarcoidosis is a multisystem, granulomatous disease that most often affects the lungs. The clinical course is highly variable; many patients undergo spontaneous remission, but up to a third of patients progresses to a chronic disease course. The development of pulmonary fibrosis (PF) in a subset of patients with chronic disease has a negative impact on morbidity and mortality. While sarcoidosis-associated PF can be progressive, it is often referred to as “burnt out” disease, a designation reflecting inactive granulomatous inflammation. The immune mechanisms of sarcoidosis-associated PF are not well understood. It is not clear if fibrotic processes are active from the onset of sarcoidosis in predisposed individuals, or whether a profibrotic state develops as a response to ongoing inflammation. Transforming growth factor β (TGF-β) is an important profibrotic cytokine, and in sarcoidosis, distinct genotypes of TGF-β have been identified in those with PF. The overall cytokine profile in sarcoidosis-associated PF has not been well characterized, although a transition from a T helper 1 to a T helper 2 signature has been proposed. Macrophages have important regulatory interactions with fibroblasts, and the role of alveolar macrophages in sarcoidosis-associated PF is a compelling target for further study. Elucidating the natural history of sarcoidosis-associated PF will inform our understanding of the fundamental derangements, and will enhance prognostication and the development of therapeutic strategies. PMID:22683422

  5. Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association

    PubMed Central

    Grados, Aurélie; Ebbo, Mikael; Bernit, Emmanuelle; Veit, Véronique; Mazodier, Karin; Jean, Rodolphe; Coso, Diane; Aurran-Schleinitz, Thérèse; Broussais, Florence; Bouabdallah, Reda; Gravis, Gwenaelle; Goncalves, Anthony; Giovaninni, Marc; Sève, Pascal; Chetaille, Bruno; Gavet-Bongo, Florence; Weitten, Thierry; Pavic, Michel; Harlé, Jean-Robert; Schleinitz, Nicolas

    2015-01-01

    Abstract The association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported. We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients. Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series. This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse. PMID:26181571

  6. Diagnosis of sarcoidosis from a biopsy of a dilated mammary duct.

    PubMed

    Mason, Callan; Yang, Robert; Hamilton, Raynal; Punar, Metin; Shah, Zeeshan; Spigel, Joseph; Wang, Jean

    2017-04-01

    Sarcoidosis is an immunologic disease of unknown etiology that manifests most frequently within the lungs or associated lymph nodes. Sarcoidosis involving the breast is seen in <1% of cases and usually is diagnosed in patients with multisystem disease. The clinical and imaging presentations of sarcoidosis of the breast can be variable. Though uncommon, sarcoidosis should be considered in the differential diagnosis of a breast lesion, and given that imaging characteristics cannot distinguish between sarcoidosis and malignancy, all breast lesions in patients with sarcoidosis should be biopsied. Our case study demonstrates a diagnosis of sarcoidosis in an asymptomatic patient presenting with a single dilated duct and associated filling defect within the right breast.

  7. Pulmonary sarcoidosis: An important differential diagnosis in transbronchial lung biopsies

    PubMed Central

    Mahapatra, Qury S.; Sahai, Kavita; Rathi, K. R.; Singh, Sarvinder; Sharma, Shruti

    2014-01-01

    Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Lungs and lymphatics are the principal sites affected by this disease. The disorder is often not suspected by physicians. Materials and Methods: This was a retrospective study done on 140 transbronchial lung biopsies received for histopathological examination in the Department of Pathology for 1 year in a multispeciality tertiary care hospital, in Delhi. Results: Out of 140 transbronchial lung biopsies studied, 13 cases of sarcoidosis were diagnosed histopathologically. In these patients a clinical, pathological, and radiological corelation was done. And a final diagnosis of sarcoidosis was given after excluding other granulomatous lesions. Conclusion: Transbronchial lung biopsies have become an important tool in the diagnosis of sarcoidosis in present time. Hence sarcoidosis should be considered as a differential diagnosis when dealing with granulomatous lesions in lung biopsies. PMID:24778476

  8. Cryptococcal meningitis in an HIV negative patient with systemic sarcoidosis.

    PubMed Central

    Botha, R J; Wessels, E

    1999-01-01

    A case of Cryptococcus neoformans meningitis is described in an HIV negative patient with undiagnosed systemic sarcoidosis. The patient presented with signs of meningitis together with generalised lymphadenopathy and hepatosplenomegaly. Cryptococcal meningitis was diagnosed on lumbar puncture. She was treated with intravenous amphotericin B but died within two weeks of admission. Necropsy revealed lesions in the lungs, liver, spleen, lymph nodes, small intestine, and bone marrow consistent with sarcoidosis. Microscopically the lesions contained non-caseating epithelioid cell granulomas typical of sarcoidosis. No Schaumann or Hamazaki-Wesenberg bodies were identified. Cryptococcus neoformans meningitis is generally associated with immunosuppressive disorders. As T cell abnormalities have been described in sarcoidosis, this could have been a case of opportunistic infection. Although rare, sarcoidosis merits consideration in patients with cryptococcal disease in the absence of HIV infection. Images PMID:10711260

  9. Recognition of distinctive patterns of gallium-67 distribution in sarcoidosis

    SciTech Connect

    Sulavik, S.B.; Spencer, R.P.; Weed, D.A.; Shapiro, H.R.; Shiue, S.T.; Castriotta, R.J. )

    1990-12-01

    Assessment of gallium-67 ({sup 67}Ga) uptake in the salivary and lacrimal glands and intrathoracic lymph nodes was made in 605 consecutive patients including 65 with sarcoidosis. A distinctive intrathoracic lymph node {sup 67}Ga uptake pattern, resembling the Greek letter lambda, was observed only in sarcoidosis (72%). Symmetrical lacrimal gland and parotid gland {sup 67}Ga uptake (panda appearance) was noted in 79% of sarcoidosis patients. A simultaneous lambda and panda pattern (62%) or a panda appearance with radiographic bilateral, symmetrical, hilar lymphadenopathy (6%) was present only in sarcoidosis patients. The presence of either of these patterns was particularly prevalent in roentgen Stages I (80%) or II (74%). We conclude that simultaneous (a) lambda and panda images, or (b) a panda image with bilateral symmetrical hilar lymphadenopathy on chest X-ray represent distinctive patterns which are highly specific for sarcoidosis, and may obviate the need for invasive diagnostic procedures.

  10. What is the neutrophil/lymphocyte ratio in sarcoidosis?

    PubMed

    Gungor, S; Akturk, U A; Yalcinsoy, M; Kocak, N D; Goksenoglu, N C; Altunbey, S A; Bekir, S A; Guven, A A O; Sucu, P; Kuver, S U; Aksoy, E; Duman, D; Agca, M C; Oztas, S; Akkaya, E; Karakurt, Z

    2016-01-01

    Information regarding the Neutrophil/Lymphocyte ratio (NLR) in sarcoidosis and the data from studies recommending its use as an indicator of inflammation and in the differential diagnosis and prognosis, are limited. With this study, it was aimed to obtain data regarding the NLR level in the patients at the time of presentation to the hospital and to determine the characteristics of patients in whom the NLR value was > 2. During the study period, of the 3434 patients with the sub-diagnosis of D86, 1300 cases whose complete blood count values had been recorded at the time of presentation were included in the study. Of the cases, 40 % were pulmonary sarcoidosis, 7 % were pulmonary sarcoidosis with sarcoidosis of the lymph nodes, 8 % were lymph node sarcoidosis, 1 % were sarcoidosis, of other combined areas, and 40 % of the cases were sarcoidosis that were unspecified. The F/M of the cases were 947/353, and the average age of the cases was 44. When the sarcoidosis groups were grouped into NLR < 2 (Group 1) and NLR ≥ 2 (Gorup 2), 27 % were Group 1, 73 % were Group 2, and a significant correlation was found between the two groups. When the inflammatory indicators were compared with NLR, the PLT/MPV was found to be statistically insignificant, and the ACE, ESR and CRP were found to be statistically significant. The Neutrophil/Lymphocyte ratio in the complete blood count, which is an easy and cheap test, can be used as an indicator of inflammation in Sarcoidosis. In clinical practice, wide-based studies comprising the activity and the staging in the prognosis of sarcoidosis are required (Tab. 2, Fig. 2, Ref. 26).

  11. Dual Analysis for Mycobacteria and Propionibacteria in Sarcoidosis BAL

    PubMed Central

    Oswald-Richter, Kyra A.; Beachboard, Dia C.; Seeley, Erin H.; Abraham, Susamma; Shepherd, Bryan E.; Jenkins, Cathy A.; Culver, Daniel A.; Caprioli, Richard M.; Drake, Wonder P.

    2012-01-01

    Purpose Sarcoidosis is a non-caseating granulomatous disease for which a role for infectious antigens continues to strengthen. Recent studies have reported molecular evidence of mycobacteria or propionibacteria. We assessed for immune responses against mycobacterial and propionibacterial antigens in sarcoidosis bronchoalveolar lavage (BAL) using flow cytometry, and localized signals consistent with microbial antigens with sarcoidosis specimens, using matrix-assisted laser desorption ionization imaging mass spectrometry (MALDI-IMS). Methods BAL cells from 27 sarcoidosis, 14 PPD- controls, and 9 subjects with nontuberculosis mycobacterial (NTM) infections were analyzed for production of IFN-γ after stimulation with mycobacterial ESAT-6 and Propionibacterium acnes proteins. To complement the immunological data, MALDI-IMS was performed to localize ESAT-6 and Propionibacterium acnes signals within sarcoidosis and control specimens. Results CD4+ immunologic analysis for mycobacteria was positive in 17/27 sarcoidosis subjects, compared to 2/14 PPD-subjects, and 5/9 NTM subjects (p=00.008 and p=00.71 respectively, Fisher's exact test). There was no significant difference for recognition of P. acnes, which occurred only in sarcoidosis subjects that also recognized ESAT-6. Similar results were also observed for the CD8+ immunologic analysis. MALDI-IMS localized signals consistent with ESAT-6 only within sites of granulomatous inflammation, whereas P. acnes signals were distributed throughout the specimen. Conclusions MALDI-IMS localizes signals consistent with ESAT-6 to sarcoidosis granulomas, whereas no specific localization of P. acnes signals is detected. Immune responses against both mycobacterial and P. acnes are present within sarcoidosis BAL, but only mycobacterial signals are distinct from disease controls. These immunologic and molecular investigations support further investigation of the microbial community within sarcoidosis granulomas. PMID:22552860

  12. Efficacy and safety of TNF antagonists in sarcoidosis: data from the Spanish registry of biologics BIOBADASER and a systematic review.

    PubMed

    Maneiro, Jose Ramon; Salgado, Eva; Gomez-Reino, Juan J; Carmona, Loreto

    2012-08-01

    To evaluate the safety, efficacy, and effectiveness of TNF antagonists in patients with sarcoidosis. A descriptive study of a case series registered in BIOBADASER and a systematic review was performed. The search strategy of articles published between 1998 and July 2011 in Medline, Embase, and the Cochrane Library included synonyms of sarcoidosis and synonyms of TNF antagonists. Seven patients treated with infliximab (IFX) and 1 with etanercept (ETN) switched to IFX for inefficacy were registered in BIOBADASER 2.0. In 3, treatment is still ongoing. Reasons for discontinuation were serious adverse events in 2 cases, inefficacy in 2 cases, and complete clinical response in 2 cases. Eight serious adverse events were reported. In the selected 69 of 2262 reports and 1 abstract of the review, 232 patients (89.9%) were treated with IFX and 26 (10.0%) were treated with ETN. In 2 randomized clinical trials, favorable response of the lung disease was reported with IFX. In other randomized clinical trials, no improvement of ocular manifestations was reported with ETN. In the cases series, results were diverse. Mean weighted rates of adverse events, infections, serious infections, and malignancy were 39.9, 22.1, 5.9, and 1.0 per 100 patient-years, respectively. There is insufficient evidence to ensure the efficacy of TNF antagonists in sarcoidosis. Nevertheless, IFX may be effective in selected manifestations of the disease. Before starting treatment of sarcoidosis with IFX, a careful evaluation of the benefit/risk ratio must be considered on an individual basis. Copyright © 2012 Elsevier Inc. All rights reserved.

  13. Ocular dispersion

    NASA Astrophysics Data System (ADS)

    Hammer, Daniel X.; Noojin, Gary D.; Thomas, Robert J.; Stolarski, David J.; Rockwell, Benjamin A.; Welch, Ashley J.

    1999-06-01

    Spectrally resolved white-light interferometry (SRWLI) was used to measure the wavelength dependence of refractive index (i.e., dispersion) for various ocular components. The accuracy of the technique was assessed by measurement of fused silica and water, the refractive indices of which have been measured at several different wavelengths. The dispersion of bovine and rabbit aqueous and vitreous humor was measured from 400 to 1100 nm. Also, the dispersion was measured from 400 to 700 nm for aqueous and vitreous humor extracted from goat and rhesus monkey eyes. For the humors, the dispersion did not deviate significantly from water. In an additional experiment, the dispersion of aqueous and vitreous humor that had aged up to a month was compared to freshly harvested material. No difference was found between the fresh and aged media. An unsuccessful attempt was also made to use the technique for dispersion measurement of bovine cornea and lens. Future refinement may allow measurement of the dispersion of cornea and lens across the entire visible and near-infrared wavelength band. The principles of white- light interferometry including image analysis, measurement accuracy, and limitations of the technique, are discussed. In addition, alternate techniques and previous measurements of ocular dispersion are reviewed.

  14. Angiolymphoid hyperplasia with eosinophilia of the orbit and ocular adnexa: report of 5 cases.

    PubMed

    Azari, Amir A; Kanavi, Mozhgan R; Lucarelli, Mark; Lee, Vivian; Lundin, Ashley M; Potter, Heather D; Albert, Daniel M

    2014-05-01

    To report the clinical and histopathologic findings of ocular adnexal angiolymphoid hyperplasia with eosinophilia, an unusual but often misdiagnosed benign disorder. The ophthalmologic findings of angiolymphoid hyperplasia with eosinophilia with ocular adnexal involvement are variable and include eyelid swelling, ptosis, proptosis, and loss of vision. Imaging studies typically reveal a well-circumscribed mass in the orbit. The condition may resemble other diseases that involve the orbit and ocular adnexal tissue, such as lymphoma, hemangioma, sarcoidosis, and dermoid cyst. Histopathologic analysis reveals marked vascular proliferation with an accompanying inflammation composed of numerous eosinophils, lymphocytes, and plasma cells. Angiolymphoid hyperplasia with eosinophilia is a rare disease that can affect the ocular adnexal tissue. The clinical presentation is often nonspecific; therefore, histopathologic studies are essential for diagnosis and subsequent management of this benign condition.

  15. A Rare Presentation of Sarcoidosis as a Pancreatic Head Mass

    PubMed Central

    Mony, Shruti; Patil, Pradnya D.; English, Rebekah; Das, Ananya; Culver, Daniel A.

    2017-01-01

    Sarcoidosis is a multisystem granulomatous syndrome of unknown etiology with noncaseating epithelioid granulomas being the pathognomonic pathological finding. Sarcoidosis most commonly involves the lungs and involvement of the gastrointestinal (GI) tract is uncommon. Pancreatic sarcoidosis is very rare, especially when it is the presenting feature of sarcoidosis and can masquerade as pancreatic cancer. Tissue infiltration in pancreatic sarcoidosis can lead to either a diffuse nodular appearance or a mass-like lesion. We present an interesting case of a 47-year-old woman with a 10-pack-year history of smoking who presented with sharp epigastric pain, weight loss, and elevated lipase level. CT and MRI imaging showed a 4 cm × 5 cm heterogeneous pancreatic mass with a dilated pancreatic duct and peripancreatic lymphadenopathy. Endoscopic ultrasound guided FNA revealed noncaseating granulomas with no evidence of malignancy or atypical infection. CT of the chest revealed bilateral mediastinal and hilar adenopathy with calcification, without any parenchymal abnormalities, and her angiotensin-converting enzyme level was elevated at 170 U/L. The clinical picture pointed to the diagnosis of pancreatic sarcoidosis. Given the severity of gastrointestinal symptoms related to pancreatic sarcoidosis, prednisone therapy at 0.5 mg/kg/day was initiated with complete resolution of symptoms at 8 weeks. PMID:28321355

  16. Sarcoidosis and chronic beryllium disease: similarities and differences.

    PubMed

    Mayer, Annyce S; Hamzeh, Nabeel; Maier, Lisa A

    2014-06-01

    Chronic beryllium disease (CBD) is a granulomatous lung disease that may be pathologically and clinically indistinguishable from pulmonary sarcoidosis, except through use of immunologic testing, such as the beryllium lymphocyte proliferation test (BeLPT). Similar to sarcoidosis, the pulmonary manifestations of CBD are variable and overlap with other respiratory diseases. Definitive diagnosis of CBD is established by evidence of immune sensitization to beryllium and diagnostic bronchoscopy with bronchoalveolar lavage and transbronchial biopsy. However, the diagnosis of CBD can also be established on a medically probable basis in beryllium-exposed patients with consistent radiographic imaging and clinical course. Beryllium workers exposed too much higher levels of beryllium in the past demonstrated a much more fulminant disease than is usually seen today. Some extrapulmonary manifestations similar to sarcoidosis were noted in these historic cohorts, although with a narrower spectrum. Extrapulmonary manifestations of CBD are rare today. Since lung-predominant sarcoidosis can very closely resemble CBD, CBD is still misdiagnosed as sarcoidosis when current or past exposure to beryllium is not recognized and no BeLPT is obtained. This article describes the similarities and differences between CBD and sarcoidosis, including clinical and diagnostic features that can help physicians consider CBD in patients with apparent lung-predominant sarcoidosis. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  17. Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis

    PubMed Central

    Bacha, Dhouha; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; BelHadj, Najet

    2015-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties. PMID:26900444

  18. Sarcoidosis: an update on current pharmacotherapy options and future directions.

    PubMed

    Brito-Zerón, Pilar; Pérez-Alvarez, Roberto; Pallarés, Lucio; Retamozo, Soledad; Baughman, Robert P; Ramos-Casals, Manuel

    2016-12-01

    Sarcoidosis is a systemic disease of unknown etiology characterized by the development of non-caseating epitheloid granulomas. The lungs are the most commonly involved organ (>90% of cases), followed by the lymph nodes, the skin, and the eyes. Areas covered: This review summarizes current pharmacotherapy options and future directions for the development of new therapies. Glucocorticoids are the first-line therapy for sarcoidosis. For patients with the most severe forms of sarcoidosis (who will need glucocorticoids for long periods) and for those intolerant or refractory, immunosuppressive drugs are used as sparing agents. The management of extrathoracic sarcoidosis must be tailored to the specific organ or organs involved; however, there is limited data from controlled trials to guide the treatment of these patients. The emergence of biological therapies has increased the therapeutic armamentarium available to treat sarcoidosis, with monoclonal anti-TNF agents being the most promising, but their use is still limited by a lack of licensing and costs. Expert commentary: The treatment of sarcoidosis is still not totally standardized. New effective therapies are urgently needed to enable the reduction or replacement of long-term therapy with glucocorticoids in patients with sarcoidosis.

  19. Multimodality evaluation of musculoskeletal sarcoidosis: Imaging findings and literature review.

    PubMed

    Aptel, S; Lecocq-Teixeira, S; Olivier, P; Regent, D; Teixeira, P Gondim; Blum, A

    2016-01-01

    Whilst the detailed X-ray features of thoracic manifestations of sarcoidosis are now clearly defined and known by most radiologists, the same does not apply to osteoarticular and muscular features of the disease, which may however raise major diagnostic problems, either because they are the presenting features of the disease (7% of cases) or because they develop during its course. The bony lesions of sarcoid dactylitis (classical Perthes-Jüngling disease) are very characteristic and well known. Many other presentations of bone and bone marrow sarcoidosis may however raise major diagnostic difficulties, particularly uni- or multifocal osteolytic and sclerotic forms of the disease. The articular manifestations of sarcoidosis are difficult to distinguish from those of the other inflammatory and degenerative arthropathies. The muscular lesions in sarcoidosis are generally clinically silent and therefore often missed. MRI has shown them to be very common in active sarcoidosis. Acute forms carry a good prognosis whereas chronic lesions are a presenting feature of multi-organ sarcoidosis. Finally, clinicians should always think about the possibility of an iatrogenic origin for musculoskeletal abnormalities seen in sarcoidosis, particularly those related to corticosteroid therapy. Copyright © 2015 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

  20. A rare presentation of sarcoidosis with nasal bone involvement

    PubMed Central

    Uslu, Selen; Korkmaz, Hakan; Çetinkol, Yeliz

    2016-01-01

    Background: Sarcoidosis is a multisystem granulomatous inflammatory disease that is induced by infectious or noninfectious environmental antigens in a genetically susceptible host. Tuberculosis and sarcoidosis are two diseases with similar clinical and pathologic findings. The link between these two diseases has been extensively studied. Objective: Herein we describe a case of sarcoidosis associated with tuberculosis, treated for tuberculosis, and, 1 year, later presented with a nasal dorsal lump and skin lesions on the extremities. Methods: Case report with clinical description. Results: Our patient had a history of skin and cervical lymphadenopathy symptoms 1 year earlier and was treated with antituberculosis drugs in an outer medical center. Therapy had cured cervical lymphadenopathies, with no improvement in skin lesions. On appearance of the nasal dorsal lump, she presented to our outpatient clinic. We retrieved the previous specimens of the patient, which revealed coexistence of necrotizing granulomas with non-necrotizing granulomas, which was strongly indicative of the coexistence of tuberculosis and sarcoidosis. Radiologic, histopathologic, and microbiologic investigation revealed the diagnosis of sarcoidosis with nasal, cutaneous, and pulmonary involvement. Treatment with prednisolone and hydroxychloroquine resulted in dramatic improvement of nasal bone, pulmonary, and skin lesions within 2 weeks. Conclusion: The clinical presentation of sarcoidosis can be complex, and the differential diagnosis from tuberculosis can be challenging. Atypical clinical pictures also can cause delays in diagnosis and proper management. In patients with granulomatous lesions that are unresponsive to antituberculosis therapy, physicians must be alerted to the possibility of coexistent sarcoidosis. PMID:27103561

  1. Sarcoidosis presenting as non-scarring non-scalp alopecia.

    PubMed

    Dan, Luke; Relic, John

    2016-08-01

    In this article we describe a 39-year-old man who presented with non-scarring non-scalp alopecia of his limbs as the initial presentation of sarcoidosis. Alopecia is a rare cutaneous manifestation of sarcoidosis. A literature review has found only one other example of sarcoidosis presenting as non-scarring non-scalp alopecia in an area other than the scalp in a patient who was otherwise asymptomatic. Several reported cases have described scarring alopecia of the scalp, which is the area of skin most commonly affected by sarcoidosis. There has been one documented case of sarcoidosis manifesting as total body non-scarring alopecia in a patient who had systemic symptoms of sarcoidosis. Other cases have presented rare cutaneous manifestations of sarcoidosis but in all these cases several other organ systems have been involved, and the patient has had systemic symptoms on presentation or the cutaneous presentation did not include non-scalp non-scarring alopecia. © 2015 The Australasian College of Dermatologists.

  2. Necrotizing sarcoid granulomatosis--is it different from nodular sarcoidosis?

    PubMed

    Popper, H H; Klemen, H; Colby, Th V; Churg, A

    2003-05-01

    Necrotizing sarcoid granulomatosis (NSG) was initially defined as a granulomatosis with features in between sarcoidosis and Wegener's granulomatosis (WG), but without extrapulmonary involvement. Subsequent reports have shown that extrapulmonary involvement does exist, and some have suggested NSG as a variant of sarcoidosis. We studied 10 cases from 3 institutions, and compared clinical and histologic features with those of nodular sarcoidosis and WG. We have analyzed the 10 cases for mycobacterial chaperonin and for the insertion sequence 6110 by PCR. Nodular aggregates of granulomas in NSG were similar to those seen in nodular sarcoidosis. Granulocytic vasculitis, a hallmark of WG was not seen in any of the NSG cases. Granulomatous vasculitis was a common feature in cases of NSG, and did not differ from that seen in sarcoidosis. The only unique feature of NSG is infarct-like necrosis, induced by the vasculitis, which might also be interpreted as a function of the duration of the vasculitis, leading ultimately to vascular obstruction. NSG based on our morphologic findings is best classified as a variant of nodular sarcoidosis. In contrast to our findings in sarcoidosis mycobacterial DNA was not found in any of the 10 cases.

  3. The etiologic role of infectious antigens in sarcoidosis pathogenesis

    PubMed Central

    Celada, Lindsay J.; Hawkins, Charlene; Drake, Wonder P.

    2015-01-01

    Sarcoidosis is a granulomatous disease of unknown etiology, characterized by a Th1 immunophenotype, most commonly involving the lung, skin, lymph node and eyes. Molecular and immunologic studies continue to strengthen the association of sarcoidosis with infectious antigens, particularly those derived from Propionibacterium and Mycobacterium species. Independent studies report the presence of microbial nucleic acids and proteins within sarcoidosis specimens. Complementary immunologic studies also support the role of infectious agents in sarcoidosis pathogenesis. Th-1 immune responses directed against mycobacterial virulence factors have been detected within sarcoidosis diagnostic bronchoalveolar lavage (BAL). Th1 and Th17 immune responses against propionibacteria have also been reported. More recently, case reports and clinical trials from Japanese, European and American investigators have emerged regarding the efficacy of antimicrobials against Propionibacterium and Mycobacterium species on pulmonary and cutaneous sarcoidosis. While these clinical investigations are not conclusive, they support increasing efforts to identify novel therapeutics, such as antimicrobials, that will impact the observed increase in sarcoidosis morbidity and mortality. PMID:26593133

  4. Predictors of objective cough frequency in pulmonary sarcoidosis.

    PubMed

    Sinha, Aish; Lee, Kai K; Rafferty, Gerrard F; Yousaf, Nadia; Pavord, Ian D; Galloway, James; Birring, Surinder S

    2016-05-01

    Cough is a common symptom of pulmonary sarcoidosis. This study aimed to quantify cough frequency, and investigate its relationship with cough reflex sensitivity, pulmonary function and health status.32 patients with pulmonary sarcoidosis were compared with 40 healthy controls. Cough reflex sensitivity to capsaicin, objective 24-h cough counts, cough-specific health status, cough severity and cough triggers were measured. The predictors of cough frequency in sarcoidosis were determined in a multivariate analysis.Objective cough frequency was significantly raised in patients with sarcoidosis compared with healthy controls (p<0.001) and patients with cough had an impaired health status. Patients with pulmonary sarcoidosis had a heightened cough reflex sensitivity compared with healthy controls (p<0.001). Only cough reflex sensitivity was significantly associated with objective cough frequency in multivariate analysis, explaining 42% of the variance (p<0.001). There was no association between cough frequency, lung function, number of organs involved, chest radiograph stage or serum angiotensin-converting enzyme levels.Cough is a common and significant symptom in patients with sarcoidosis. Ambulatory objective cough monitoring provides novel insights into the determinants of cough in sarcoidosis, suggesting that cough reflex sensitivity may be more important than lung function and other measures of disease severity, and this should be investigated further. Copyright ©ERS 2016.

  5. Cardiac sarcoidosis diagnosed by multimodality imaging.

    PubMed

    Fujikura, Kana; Garcia, Mario J

    2017-09-01

    A 66-year-old woman presented with frequent premature ventricular contractions (PVC) and akinesis of the basal septum on echocardiography. Coronary angiography was normal. Cardiac magnetic resonance showed mid-wall enhancement. Positron emission tomography showed a perfusion defect at the same location using 13N-ammonia, but increased 18-fluorodeoxyglucose uptake. These findings supported the diagnosis of cardiac sarcoidosis. High-dose steroids initially reduced frequency of PVCs but had to be withdrawn due to severe side effects. An ICD was implanted. Our case demonstrates the utility of multimodality imaging to diagnose and guide management of this entity. © 2017, Wiley Periodicals, Inc.

  6. Anti-cyclic citrullinated peptide antibodies in patients with sarcoidosis.

    PubMed

    Kobak, Senol; Ylmaz, H; Sever, F; Duran, A; Sen, N

    2014-10-20

    Anti-cyclic citrullinated peptide (anti-CCP) antibodies have a high predictive value in rheumatoid arthritis (RA) patients and are associated with disease severity. Sarcoidosis is a chronic inflammatory disease characterized by non-calcified granuloma formations. To determining the prevalence of anti-CCP antibodies in patients with sarcoidosis, and identifying a possible correlation with clinical and laboratory findings. Forty-two patients presenting to the rheumatology polyclinic and diagnosed with sarcoidosis as a result of the examinations made, 45 RA patients and 45 healthy subjects were included in the study. Demographic, clinical, serological and radiological data of all patients were recorded. Anti-CCP antibodies were evaluated by using a second-generation ELISA method. Rheumatoid factor (RF) IgM was determined with the nephelometry method. Forty-two patients (10 males) were included in the study. Mean patient age was 45.2 years (20-70 years) and mean duration of disease was 3.5 years. Two sarcoidosis patients (4.7%) and 38(84.4%) RA patients were found to be positive for anti-CCP antibodies while the antibody wasn't detected in any healthy subject. The two sarcoidosis patients found positive for anti-CCP were also diagnosed with rheumatoid arthritis. RF positivity was detected in 7 sarcoidosis patients (16.6%) and in only one subject in the control group. The prevalence of anti-CCP antibodies in patients with sarcoidosis was found to be significantly lower than RA patients and similar with the healthy control group. This result shows that anti-CCP antibodies don't have an important role in the pathogenesis of sarcoidosis, but could be important in revealing the overlap syndromes of sarcoidosis-RA.

  7. Ocular surface reconstruction update.

    PubMed

    Shimmura, Shigeto; Tsubota, Kazuo

    2002-08-01

    Ocular surface reconstruction (OSR) is now a standard procedure in the treatment of severe ocular surface disorders. The past few years have revealed the long-term results of patients who were operated on during the early stages of OSR development, and we now have a more realistic view of the benefits and limits of the procedure. On the other hand, further understanding of the physiologic role played by the amniotic membrane (AM) has opened doors to further refined techniques in treating these patients. This review will introduce some of the major contributions made during the past years in the advancement of OSR. Clinically, we are at a stage of reviewing the pros and cons of the various transplantation techniques. Identification of factors crucial for a successful OSR procedure will further improve surgical results. Basic researchers are on the verge of identifying the so-called limbal stem cells, and further understanding of AM physiology will lead the way to tissue engineering techniques as another alternative in OSR surgery.

  8. [Necrotizing systemic sarcoidosis with pulmonary and central nervous system involvement].

    PubMed

    Ríos Fernández, R; Callejas-Rubio, J L; Guerrero Fernández, M; Serrano Falcón, M M; Ortego-Centeno, N

    2008-01-01

    Sarcoidosis is a multisystemic disease which diagnosis depends on the presence of nonnecrotizing granulomas in the biopsy. However there are variants such as necrotizing sarcoidal granulomas or nodular sarcoidosis which have atypical findings and make difficult the differential diagnosis with other infectious processes. We describe a case of a man who develops granulomas with extensive necrosis in a systemic sarcoidosis that affected the lung and the central nervous system. This finding made us to make the diagnosis of tuberculosis and delay the specific treatment.

  9. Development of Hodgkin lymphoma in a patient with sarcoidosis.

    PubMed

    Gediz, F; Yilmaz, A F; Payzin, B; Yuksel, T; Calli, A O; Kobak, S

    2017-08-03

    Sarcoidosis is a chronic granulomatous disease of unknown etiology characterized by non-caseified granulomas in many different organs and systems. The disease most frequently manifests with bilateral hilar lymphadenopathy and infiltrations in the lungs and skin, as well as with eye lesions. It may mimic a number of systemic diseases and/or accompany them. The development of lymphoma in patients with sarcoidosis or the co-occurrence of both diseases is rarely reported in the literature. In this paper we report a female patient followed up with sarcoidosis for three years who developed Hodgkin lymphoma, according to the results of the investigations and biopsy results.

  10. Orbital sarcoidosis presenting as diffuse swelling of the lower eyelid.

    PubMed

    Lee, Jeong Kyu; Moon, Nam Ju

    2013-02-01

    The author reports a case of orbital Sarcoidosis in a 70-year-old female that initially presented as diffuse swelling of the lower eyelid. The patient complained of painless swelling of the left lower lid without palpable mass, and a computerized tomography (CT) scan of the orbit was unremarkable. A serum angiotensin converting enzyme level was elevated, and hilar lymphadenopathy was noted on the chest CT. The patient underwent surgical debulking for histologic confirmation, which led to a final diagnosis of sarcoidosis involving the orbital fat. Unexplained chronic eyelid swelling without a mass should be considered a possible ophthalmic manifestation of orbital sarcoidosis.

  11. CON4EI: CONsortium for in vitro Eye Irritation testing strategy - EpiOcular™ time-to-toxicity (EpiOcular ET-50) protocols for hazard identification and labelling of eye irritating chemicals.

    PubMed

    Kandarova, Helena; Letasiova, Silvia; Adriaens, Els; Guest, Robert; Willoughby, Jamin A; Drzewiecka, Agnieszka; Gruszka, Katarzyna; Alépée, Nathalie; Verstraelen, Sandra; Van Rompay, An R

    2017-08-30

    Assessment of acute eye irritation potential is part of the international regulatory requirements for testing of chemicals. The objective of the CON4EI (CONsortium for in vitro Eye Irritation testing strategy) project was to develop tiered testing strategies for eye irritation assessment for all drivers of classification. A set of 80 reference chemicals (38 liquids and 42 solids) was tested with eight different alternative methods. Here, the results obtained with reconstructed human cornea-like epithelium (RhCE) EpiOcular™ in the EpiOcular time-to-toxicity Tests (Neat and Dilution ET-50 protocols) are presented. The primary aim of this study was to evaluate whether test methods can discriminate chemicals not requiring classification for serious eye damage/eye irritancy (No Category) from chemicals requiring classification and labelling for Category 1 and Category 2. In addition, the predictive capacity in terms of in vivo drivers of classification was investigated. The chemicals were tested in two independent runs by MatTek In Vitro Life Science Laboratories. Results of this study demonstrate very high specificity of both test protocols. With the existing prediction models described in the SOPs, the specificity of the Neat and Dilution method was 87% and 100%, respectively. The Dilution method was able to correctly predicting 66% of GHS Cat 2 chemicals, however, prediction of GHS Cat 1 chemicals was only 47%-55% using the current protocols. In order to achieve optimal prediction for all three classes, a testing strategy was developed which combines the most predictive time-points of both protocols and for tests liquids and solids separately. Using this new testing strategy, the sensitivity for predicting GHS Cat 1 and GHS Cat 2 chemicals was 73% and 64%, respectively and the very high specificity of 97% was maintained. None of the Cat 1 chemicals was underpredicted as GHS No Category. Further combination of the EpiOcular time-to-toxicity protocols with other

  12. Unmasking sarcoidosis following surgery for Cushing disease.

    PubMed

    Diernaes, Jon E F; Bygum, Anette; Poulsen, Per L

    2016-01-01

    We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed clinically and a suspicion of underlying sarcoidosis was substantiated by lung imaging and elevated plasma interleukin (IL)-2 receptor. One month later, the lesions spontaneously resolved without therapy other than maintenance glucocorticoid replacement. Physicians should be aware that patients undergoing successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease.

  13. Unmasking sarcoidosis following surgery for Cushing disease

    PubMed Central

    Diernaes, Jon E.F.; Bygum, Anette; Poulsen, Per L.

    2016-01-01

    ABSTRACT We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed clinically and a suspicion of underlying sarcoidosis was substantiated by lung imaging and elevated plasma interleukin (IL)-2 receptor. One month later, the lesions spontaneously resolved without therapy other than maintenance glucocorticoid replacement. Physicians should be aware that patients undergoing successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease. PMID:27375832

  14. Molecular Analysis of Sarcoidosis Tissues for Mycobacterium Species DNA

    PubMed Central

    Pei, Zhiheng; Pride, David T.; Collins, Robert D.; Cover, Timothy L.; Blaser, Martin J.

    2002-01-01

    We performed polymerase chain reaction analysis, for Mycobacterium species 16S rRNA, rpoB, and IS6110 sequences, on 25 tissue specimens from patients with sarcoidosis and on 25 control tissue specimens consisting of mediastinal or cervical lymph nodes and lung biopsies. Mycobacterium species 16S rRNA sequences were amplified from 12 (48%) rpoB sequences from 6 (24%) of the sarcoidosis specimens. In total, 16S rRNA or rpoB sequences were amplified from 15 sarcoidosis specimens (60%) but were not detected in any of the control tissues (p=0.00002, Chi square). In three specimens, the sequences resembled Mycobacterium species other than M. tuberculosis. All specimens with sequences consistent with M. tuberculosis were negative for IS6110. We provide evidence that one of a variety of Mycobacterium species, especially organisms resembling M. tuberculosis, is found in most patients with sarcoidosis. PMID:12453366

  15. Sarcoidosis mimicking a venous ulcer: a case report.

    PubMed

    Joshi, Smita S; Romanelli, Paolo; Kirsner, Robert S

    2009-11-01

    Sarcoidosis--a chronic, multisystem disease of unknown etiology characterized by noncaseating granulomas--may cause ulcerative lesions, particularly in African American women. A case of ulcerative sarcoidosis mimicking a venous ulcer is presented. The patient is a 44-year-old African American hypertensive, obese woman with a nonhealing medially based lower leg ulcer of 3 years' duration clinically consistent with a venous ulcer. The ulcer did not heal with compression therapy and pentoxifylline. Subsequent biopsies showed granulomatous inflammation consistent with sarcoidosis. When intralesional triamcinolone was added to compression therapy, the ulcer resolved after 3 months. Given its propensity toward formation on the lower extremities and ulcerative and atrophic appearance, ulcerative sarcoidosis should be considered in the differential diagnosis of a venous ulcer refractory to standard therapy, especially in African American women.

  16. Isolated cutaneous sarcoidosis: a new insight into the old entity.

    PubMed

    Kumar H K, Sharath; M N, Gayathri; M, Bharathi; T, Ravikumar

    2013-08-01

    Cutaneous lesions are a part of common manifestation in multi-systemic sarcoidosis and skin involvement occurs in 20% to 35% of the patients with systemic disease. An exclusive cutaneous involvement is rare and it is reported in about 4%-5% of the patients of sarcoidosis. These lesions have been classified into specific and non-specific, depending on the presence of non- caseating granulomas, on histopathologic studies. In the present study, we are reporting two cases of isolated cutaneous sarcoidosis without systemic manifestations, which is a rare presentation. Sarcoidosis will continue to challenge even the most experienced clinicians with respect to a high degree of suspicion and further investigations for diagnosing and treating this disorder.

  17. An Isolated Mass in the Palm, Starting Manifestation of Sarcoidosis

    PubMed Central

    Ghorbanhoseini, Mohammad; Yazdi, Hamidreza; Naseh, Hossein; Hafezi, Poopak

    2016-01-01

    Sarcoidosis is a systemic disease that is characterized with noncaseating granulomatous nodules which present in multiple organs specially lungs (90%). Incidence of masses due to Sarcoidosis in upper extremity is low and most cases present in association with involvement of pulmonary hilary lymph nodes. In this article we present a rare case of Sarcoidosis which presented as a single soft tissue mass in hand without osseous or pulmonary hillary lymph node involvement. Incidence of involvement of musculoskeletal system is 1-5 %, mostly it occurs in small bones in hands and feet. In most cases involvement of soft tissue in extremities is accompanied with bone lesions. Those cases of soft tissue involvement are generally coincide with pulmonary lymph nodules. To the authors` knowledge, this is the first case of Sarcoidosis that presents without spreading in bones or pulmonary hilar lymph nodes. PMID:26894226

  18. [Systemic sarcoidosis: a diagnostic challenge in pediatrics. Case report].

    PubMed

    Díaz Angarita, Tomás; Morales Camacho, William; Lozano Neira, Laura; Plata Ortiz, Jessica; Zárate Taborda, Linda

    2016-10-01

    Sarcoidosis is a granulomatous inflammatory disease of unknown etiology, evidenced most often in young adults, which can compromise several organs, mainly lung, lymph nodes, eyes and skin. The presence of the disease in pediatric population is rare. Denomination in pediatrics is made based on age and clinical manifestations of the patient. The aim of this report is to present a case of systemic sarcoidosis in a pediatric patient without lung involvement with severe extrapulmonary manifestations. Fifteen year old patient who debuted with nonspecific symptoms (emesis, weight loss). Radiographic studies without lung involvement but with findings suggestive of Langerhans cell histiocytosis; however, histopathological report (bone and lymph) showed the presence of chronic granulomatous nonnecrotizing disease, sarcoidosis type. Childhood sarcoidosis is a rare and difficult entity to diagnose, mainly by the lack of specific biomarkers for diagnosis.

  19. Sarcoidosis of the skin: a review for the pulmonologist.

    PubMed

    Lodha, Saurabh; Sanchez, Miguel; Prystowsky, Stephen

    2009-08-01

    With vastly heterogeneous morphologic manifestations, sarcoidosis is one of the "great imitators" of medicine. Because there is no specific confirmatory test, the diagnosis rests on clinical acumen coupled with supportive information from tissue or blood evaluation and the exclusion of other diseases. The characteristic histologic pattern of noncaseating, epithelioid cell granulomas is not always present in skin lesions, which may be visually distinctive or diverse in appearance. As a result of their high incidence of respiratory disease, patients with sarcoidosis frequently seek care from pulmonologists who may become their primary health-care providers. Physicians who treat patients with sarcoidosis should be aware of the disease's diverse organ manifestations, but particularly those appearing on the skin because these can be disfiguring, have prognostic importance, and may not be readily diagnosed even by skin specialists. In this comprehensive review, we sought to illustrate this diversity and to update the diagnostic approach, histologic spectrum, and therapeutic strategies involved in cutaneous sarcoidosis.

  20. Concomitant axillary mycobacteriosis and neuro-sarcoidosis: diagnostic pitfalls

    PubMed Central

    Meckenstock, Roderich; Therby, Audrey; Chapelon-Abric, Catherine; Nifle, Chantal; Beressi, Jean Paul; Lebas, Constance; Greder–Belan, Alix

    2011-01-01

    There are many similarities between mycobacteriosis, in particular, tuberculosis, and sarcoidosis such as predominant intrathoracic localisation (even if all organs and tissues may be concerned), great variability of phenotypic expression, and granulomatous inflammatory reaction, caseous necrosis not being an absolute criterion of tuberculosis. Moreover, microbial (or mycobacterial?) agents may play a role in the pathogenesis of sarcoidosis which remains a diagnosis of exclusion particularly in atypical cases. The authors report a case of a non-immunocompromised female patient who presented, simultaneously, isolated axillary tubercular adenitis and neuro-sarcoidosis without any other localisation. This case illustrates the difficulty to distinguish between both of these two diseases and thus to choose an adequate treatment when diagnosis is not proven. Moreover, our patient (human leucocyte antigen B27 positive) presented symptoms of spondylarthritis which also may have a nosological link with tuberculosis or sarcoidosis. PMID:22679226

  1. Sarcoidosis and primary biliary cirrhosis with co-existing myositis

    PubMed Central

    Hughes, P.; McGavin, C. R.

    1997-01-01

    In a small number of cases the co-existence of primary biliary cirrhosis and sarcoidosis is assumed from clinical serological and histological findings. A case of sarcoidosis is reported in which the M2 antibody, a highly specific marker for primary biliary cirrhosis, was detected. The patient also developed a severe myositis and a possible overlap syndrome is discussed. 




 PMID:9059489

  2. Coexistence of pulmonary tuberculosis and sarcoidosis: a diagnostic dilemma.

    PubMed

    Mandal, Sanjay Kumar; Ghosh, Sudip; Mondal, Soumya Sarathi; Chatterjee, Sumanta

    2014-12-19

    Tuberculosis and sarcoidosis are multisystem diseases having different aetiology and management; however, they have similar clinical and histological characteristics. Very rarely they may coexist. We report a rare case of a 38-year-old woman who presented with chronic cough, low-grade fever and respiratory distress that was initially diagnosed as miliary tuberculosis. Diagnosis was supported by positive mycobacterial culture and initially responded to antitubercular treatment, but later recurrences led to further investigations and the diagnosis of coexisting sarcoidosis.

  3. The association between osteopontin gene polymorphisms, osteopontin expression and sarcoidosis

    PubMed Central

    Lavi, Hadas; Assayag, Miri; Schwartz, Assaf; Arish, Nissim; Fridlender, Zvi G.; Berkman, Neville

    2017-01-01

    Background Sarcoidosis is a systemic inflammatory disease of unknown etiology. Osteopontin (SPP1, OPN) is an extra cellular matrix glycoprotein and cytokine with a known role in granuloma formation and in autoimmune and inflammatory diseases. Objective To determine whether plasma OPN levels are elevated in patients with sarcoidosis and compare the frequency of four single nucleotide polymorphism (SNPs) variants in the OPN gene in sarcoidosis patients compared to healthy controls. Methods Demographic and clinical information, radiological studies and pulmonary function tests were evaluated in 113 patients with sarcoidosis and in 79 healthy controls. Blood samples were analyzed for SNPs of the OPN gene and for plasma OPN and CRP levels. Association between clinical features of disease and OPN levels as well as SNP frequencies was determined. Results Plasma OPN levels were higher in sarcoidosis patients than in healthy subjects, (median: 217 vs 122ng/ml, p<0.001). Area under the curve for receiver operator curves (ROC) was 0.798 (0.686–0.909 95% CI.) No differences were observed between sarcoidosis patients and controls in the frequency of any of the SNPs evaluated. Presence of lung parenchymal involvement was associated with SNP distribution at rs1126772 (p = 0.02). We found no correlation between SNPs distribution and plasma OPN levels. Conclusions Osteopontin protein levels are elevated in sarcoidosis. We found no evidence for an association between SNPs on the osteopontin gene and plasma OPN levels or the presence of sarcoidosis, however, an association between genotype and several phenotypic clinical parameters of disease was observed. PMID:28253271

  4. Racial Difference in Sarcoidosis Mortality in the United States

    PubMed Central

    Machado, Roberto F.; Schraufnagel, Dean; Sweiss, Nadera J.; Baughman, Robert P.

    2015-01-01

    BACKGROUND: The clinical presentation and outcome of sarcoidosis varies by race. However, the race difference in mortality outcome remains largely unknown. METHODS: We studied mortality related to sarcoidosis from 1999 through 2010 by examining data on multiple causes of death from the National Center for Health Statistics. We compared the comorbid conditions between sarcoidosis-related deaths with deaths caused by car accidents (previously healthy control subjects) and rheumatoid arthritis (chronic disease control subjects) in both African Americans and Caucasians. RESULTS: From 1999 through 2010, sarcoidosis was reported as an immediate cause of death in 10,348 people in the United States with a combined overall mean age-adjusted mortality rate of 2.8 per 1 million person-years. Of these, 6,285 were African American and 3,984 Caucasian. The age-adjusted mortality rate for African Americans was 12 times higher than for Caucasians. African Americans died at an earlier age than Caucasians. African Americans living in the District of Columbia and North Carolina and Caucasians living in Vermont had higher mortality rates. Although the total sarcoidosis age-adjusted mortality rate had not changed over the 12 year period studied, this rate increased for Caucasians (R = 0.747, P = .005) but not for African Americans. Compared with the control groups, pulmonary hypertension was significantly more common in individuals with sarcoidosis. CONCLUSIONS: This nationwide population-based study exposes a significant difference in ethnicity and sex among people dying of sarcoidosis in the United States. Pulmonary hypertension investigation should be considered in all patients with sarcoidosis, especially African Americans. PMID:25188873

  5. Epidemiology of cutaneous sarcoidosis, 1976-2013: a population-based study from Olmsted County, Minnesota.

    PubMed

    Ungprasert, P; Wetter, D A; Crowson, C S; Matteson, E L

    2016-10-01

    The epidemiology of cutaneous sarcoidosis is not well-characterized as only referral-based studies are available. To characterize the epidemiology of cutaneous sarcoidosis, with emphasis on annual incidence and clinical characteristics, from 1976 to 2013. Inception cohorts of patients with incident isolated cutaneous sarcoidosis and incident systemic sarcoidosis with cutaneous involvement in 1976-2013 in Olmsted County, Minnesota, United States were identified based on comprehensive individual medical record review. Inclusion in the isolated cutaneous sarcoidosis cohort required physician diagnosis and skin biopsy showing non-necrotizing granuloma. Inclusion in the systemic sarcoidosis with cutaneous involvement cohort required presence of systemic sarcoidosis and cutaneous lesions. Presence of systemic sarcoidosis was determined by physician diagnosis supported by histopathology of non-necrotizing granuloma, characteristic radiologic features of intrathoracic sarcoidosis and exclusion of other granulomatous diseases. Cutaneous lesions were defined as either sarcoidosis-specific or non-specific. There were 62 cases with sarcoidosis-specific cutaneous lesions (36 cases of sarcoidosis-specific cutaneous lesions and 26 cases of isolated cutaneous sarcoidosis) which corresponded to an incidence of 1.9 per 100 000 population. The female to male ratio was 2.1 : 1. Plaques, papules and subcutaneous nodules were the most commonly observed cutaneous lesions. There was no significant difference in cutaneous presentation between those who had isolated skin disease and those who had skin disease in association with systemic sarcoidosis. Prognosis of cutaneous sarcoidosis was favourable, as over 90% of patients had a good response to either glucocorticoids, hydroxychloroquine or tetracycline antibiotics. This study has a significant limitation, in that the studied population was predominantly Caucasians who generally have a lower prevalence of skin disease. The incidence of

  6. Accuracy of Diagnostic Coding for Sarcoidosis in Electronic Databases: A Population-Based Study.

    PubMed

    Ungprasert, Patompong; Matteson, Eric L; Crowson, Cynthia S

    2017-10-09

    Epidemiologic study of sarcoidosis utilizing electronic databases has been increasingly popular. However, the accuracy of diagnostic codes for sarcoidosis is unknown. The medical record-linkage system of the Rochester Epidemiology Project was searched to identify all potential adult cases of sarcoidosis between January 1, 1995 and December 31, 2013 in Olmsted County, Minnesota, using the International Classification of Diseases, Ninth Revision (ICD-9) code 135 (sarcoidosis). Complete medical records of those potential cases were individually reviewed. The diagnosis of sarcoidosis was confirmed by the presence of non-caseating granuloma on histopathology, radiographic findings of intrathoracic sarcoidosis, and compatible clinical presentations. Positive predictive value (PPV) was estimated as the number of patients verified to have sarcoidosis divided by the number of patients with a diagnostic code for sarcoidosis. The study cohort included 366 patients with at least one code for sarcoidosis. Of these, 224 cases of confirmed sarcoidosis were identified, resulting in PPV of 61.2% (95% CI 56.0-66.2%). A total of 268 patients in the database had a code for sarcoidosis on least two occasions separated by at least 30 days. Of these, there were 205 cases of confirmed sarcoidosis. The PPV for having the code at least twice was 76.5% (95% CI 71.0-81.4%). The PPV of ICD-9 code for sarcoidosis is relatively low and, thus, further verification is required for studies using electronic databases.

  7. Hepatic sarcoidosis: pathogenesis, clinical context, and treatment options.

    PubMed

    Syed, Umer; Alkhawam, Hassan; Bakhit, Mena; Companioni, Rafael A Ching; Walfish, Aron

    2016-09-01

    Sarcoidosis is typically characterized as a non-caseating granulomatous disease that has the ability to affect multiple different organ systems. Although extra-thoracic sarcoidosis can occur in the presence and also without lung involvement, isolated extra-pulmonary disease is rare. The liver is the third most commonly affected organ system after the lungs and lymph nodes. When discussing hepatic sarcoidosis it is important to keep in mind that many patients in this population may not present as one would typically expect since most of the patients are asymptomatic or have mild presentations. Therefore, the diagnosis can be difficult at times since no single laboratory or imaging study can definitively diagnose this systemic disease. In the rare case of some patients where there is difficulty in discerning between different pathologies, the use of image-guided tissue biopsy may be necessary to establish a diagnosis. At the current time, there are no clear guidelines for the management of hepatic sarcoidosis and are mostly dependent on a patient's clinical status at time of presentation. The current body of research in regard to treatment suggests steroids to be the mainstay of therapy. However, there is a role for additional immunosuppressive therapy in cases where the initial treatment is refractory to steroids. In this manuscript, we discussed the pathogenesis of liver sarcoidosis and context of its presentation. In addition, the differential diagnosis and imaging evaluation in this population is discussed. Finally, treatment options are reviewed in setting of previous studies for liver sarcoidosis.

  8. Angiotensin converting enzyme I/D polymorphism and sarcoidosis risk.

    PubMed

    Yang, H; Mo, T; Nie, W; Li, B

    2016-01-15

    This meta-analysis investigates the associations of angiotensin-converting enzyme (ACE) polymorphism and risk of sarcoidosis. Two reviewers independently searched three databases including PubMed, EMBASE, and Cochrane database to identify published studies. Full texts of the selected studies were accessed and related data was extracted using a standardized data extraction form. A total of 18 studies contained a total of 1626 patients with sarcoidosis in case group and 2465 healthy controls in control group. Results of the current meta-analysis revealed that ACE DD genotype was associated with a significantly increased risk of sarcoidosis (OR=1.21; 95% CI, 1.06-1.38; I2=48%). In the race subgroup analysis, Asians with ACE DD genotype showed no significant increased risk of sarcoidosis (OR=1.37; 95% CI, 0.94-1.99; I2=78%). Caucasians with ACE DD genotype had an increased sarcoidosis risk (OR=1.16; 95% CI, 1.01-1.36; I2=24%). Our meta-analysis indicated that the ACE DD genotype correlated with an increased risk of sarcoidosis.

  9. Cardiac Sarcoidosis Diagnosed by Incidental Lymph Node Biopsy.

    PubMed

    Matsuda, Jun; Fujiu, Katsuhito; Roh, Solji; Tajima, Miyu; Maki, Hisataka; Kojima, Toshiya; Ushiku, Tetsuo; Nawata, Kan; Takeda, Norihiko; Watanabe, Masafumi; Akazawa, Hiroshi; Komuro, Issei

    2017-02-07

    Cardiac involvement in systemic sarcoidosis sometimes provokes life-threatening ventricular tachyarrhythmia. Steroid administration is one of the fundamental anti-arrhythmia therapies. For an indication of steroid therapy, a definitive diagnosis of sarcoidosis is required.(1)) However, cases that are clearly suspected of cardiac sarcoidosis based on their clinical courses sometimes do not meet the current diagnostic criteria and result in the loss of an appropriate opportunity to perform steroid therapy.Here we report a case that was diagnosed as sarcoidosis by incidental biopsy of an inguinal lymph node during cardiac resuscitation for cardiac tamponade.(2)) While the inguinal lymph node was not swollen on computed tomography, a specimen obtained from an incidental biopsy during the exposure of a femoral vessel for the establishment of extracorporeal cardio-pulmonary resuscitation showed a non-caseating granuloma.This findings suggest a non-swelling lymph node biopsy might be an alternative strategy for the diagnosis for sarcoidosis if other standard strategies do not result in a diagnosis of sarcoidosis.

  10. Evidence for a genetic component in familial sarcoidosis

    SciTech Connect

    Rybicki, B.A.; Harrington, D.; Major, M.

    1994-09-01

    Sarcoidosis is a disease with reported familial clustering, but a putative hereditary component has not been established. We analyzed 33 sarcoid index cases with a positive family history and their 596 1st and 2nd degree relatives to determine if the distribution of disease was consistent with a genetic etiology. Prevalence of sarcoidosis was twice as high in 1st degree relatives of index cases compared to 2nd degree relatives (8.5% vs. 3.9%; p=0.016). Recurrence risk ratios (defined as the risk of disease in relatives divided by the population prevalence) were calculated in 2nd degree relatives after making an ascertainment correction. A single gene model fit the observed results better than a polygenic model for a range of sarcoidosis prevalance. A greater shared environment among siblings compared to parents and offspring did not appear to increase risk of sarcoidosis based on a higher prevalance of disease in the latter (7.1% vs. 10.7%; p=0.313). However, the age of diagnosis in affected parent-offspring pairs was more consistent with a combined genetic and environmental etiology than either component alone. In summary, these results suggest a genetic component, possibly a single gene, increases disease risk in family members of sarcoidosis cases. A segregation analysis is planned to more thoroughly evaluate the evidence for both genetic and non-genetic transmission of sarcoidosis in these families.

  11. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol.

    PubMed

    Moller, David R; Koth, Laura L; Maier, Lisa A; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K; Collman, Ronald G; Hamzeh, Nabeel; Sweiss, Nadera J; Zhang, Yingze; O'Neal, Scott; Senior, Robert M; Becich, Michael; Hochheiser, Harry S; Kaminski, Naftali; Wisniewski, Stephen R; Gibson, Kevin F

    2015-10-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant's clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis.

  12. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol

    PubMed Central

    Koth, Laura L.; Maier, Lisa A.; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K.; Collman, Ronald G.; Hamzeh, Nabeel; Sweiss, Nadera J.; Zhang, Yingze; O’Neal, Scott; Senior, Robert M.; Becich, Michael; Hochheiser, Harry S.; Kaminski, Naftali; Wisniewski, Stephen R.; Gibson, Kevin F.

    2015-01-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant’s clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis. PMID:26193069

  13. Sarcoidosis with optic nerve and retinal involvement.

    PubMed

    Gass, J D; Olson, C L

    1976-06-01

    A patient with the typical funduscopic changes of sarcoidosis died following an accident. The histopathological findings in one eye were correlated with the ophthalmoscopic observations made several days prior to death. Gross and histopathological examination revealed that the characteristic whitish-yellow focal retinal exudates, the perivascular exudates with an appearance like candle wax drippings, the preretinal and intravitreal nodules, and the localized tumefactions on the inner surface of the retina and optic nerve head noted ophthalmoscopically were a result of the nodular proliferation of epithelioid cells. These cells appeared to be derived primarily from blood vessels in the retina and optic nerve head. Although subpigment epithelial granulomas were found, there were no granulomas present within the choroid or elsewhere within the uvea.

  14. "End-stage" pulmonary fibrosis in sarcoidosis.

    PubMed

    Teirstein, Alvin T; Morgenthau, Adam S

    2009-02-01

    Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation.

  15. Idiopathic giant cell myocarditis and cardiac sarcoidosis.

    PubMed

    Blauwet, Lori A; Cooper, Leslie T

    2013-11-01

    Idiopathic giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare disorders that cause cardiomyopathy, often with ventricular arrhythmias or heart block. Infection, autoimmune processes, and genetics have all been implicated in the pathogenesis of these diseases, but the etiology for both diseases is likely a complex multifactorial process. Both GCM and CS are generally progressive despite treatment with standard heart failure and arrhythmia therapies. Making the diagnosis of GCM or CS on initial clinical presentation is possible in only a small percentage of patients, so myocardial tissue diagnosis is required. The use of multiple noninvasive imaging modalities may aid in diagnosis and assessment of response to treatment. Establishing the diagnosis of GCM or CS early is crucial, as tailored immunosuppressive treatment may significantly alter the clinical course of these patients. The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.

  16. Molecular Analysis of Sarcoidosis Granulomas Reveals Antimicrobial Targets.

    PubMed

    Rotsinger, Joseph E; Celada, Lindsay J; Polosukhin, Vasiliy V; Atkinson, James B; Drake, Wonder P

    2016-07-01

    Sarcoidosis is a granulomatous disease of unknown cause. Prior molecular and immunologic studies have confirmed the presence of mycobacterial virulence factors, such as catalase peroxidase and superoxide dismutase A, within sarcoidosis granulomas. Molecular analysis of granulomas can identify targets of known antibiotics classes. Currently, major antibiotics are directed against DNA synthesis, protein synthesis, and cell wall formation. We conducted molecular analysis of 40 sarcoidosis diagnostic specimens and compared them with 33 disease control specimens for the presence of mycobacterial genes that encode antibiotic targets. We assessed for genes involved in DNA synthesis (DNA gyrase A [gyrA] and DNA gyrase B), protein synthesis (RNA polymerase subunit β), cell wall synthesis (embCAB operon and enoyl reductase), and catalase peroxidase. Immunohistochemical analysis was conducted to investigate the locale of mycobacterial genes such as gyrA within 12 sarcoidosis specimens and 12 disease controls. Mycobacterial DNA was detected in 33 of 39 sarcoidosis specimens by quantitative real-time polymerase chain reaction compared with 2 of 30 disease control specimens (P < 0.001, two-tailed Fisher's test). Twenty of 39 were positive for three or more mycobacterial genes, compared with 1 of 30 control specimens (P < 0.001, two-tailed Fisher's test). Immunohistochemistry analysis localized mycobacterial gyrA nucleic acids to sites of granuloma formation in 9 of 12 sarcoidosis specimens compared with 1 of 12 disease controls (P < 0.01). Microbial genes encoding enzymes that can be targeted by currently available antimycobacterial antibiotics are present in sarcoidosis specimens and localize to sites of granulomatous inflammation. Use of antimicrobials directed against target enzymes may be an innovative treatment alternative.

  17. Delayed diagnosis of sarcoidosis is common in Brazil*

    PubMed Central

    Rodrigues, Mauri Monteiro; Coletta, Ester Nei Aparecida Martins; Ferreira, Rimarcs Gomes; Pereira, Carlos Alberto de Castro

    2013-01-01

    OBJECTIVE: To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil. METHODS: We evaluated patients with a biopsy-proven diagnosis of sarcoidosis, using a questionnaire that addressed the following: time since symptom onset and since the first medical visit; and the number and specialty of the physicians visited. We divided the patients by the timeliness of the diagnosis-timely (< 6 months) and delayed (≥ 6 months)-comparing the two groups in terms of systemic and pulmonary symptoms; extrathoracic involvement; spirometric data; radiological staging; level of education; income; and tuberculosis (diagnosis and treatment). RESULTS: We evaluated 100 patients. The median number of physicians consulted was 3 (range, 1-14). In 11 cases, sarcoidosis was diagnosed at the first visit. In 54, the first physician seen was a general practitioner. The diagnosis of sarcoidosis was timely in 41 patients and delayed in 59. The groups did not differ in terms of gender; race; type of health insurance; level of education; income; respiratory/systemic symptoms; extrathoracic involvement; and radiological staging. In the delayed diagnosis group, FVC was lower (80.3 ± 20.4% vs. 90.5 ± 17.1%; p = 0.010), as was FEV1 (77.3 ± 19.9% vs. 86.4 ± 19.5%; p = 0.024), misdiagnosis with and treatment for tuberculosis (≥ 3 months) also being more common (24% vs. 7%, p = 0.032, and 20% vs. 0%; p = 0.002, respectively). CONCLUSIONS: The diagnosis of sarcoidosis is often delayed, even when the imaging is suggestive of sarcoidosis. Delayed diagnosis is associated with impaired lung function at the time of diagnosis. Many sarcoidosis patients are misdiagnosed with and treated for tuberculosis. PMID:24310626

  18. Lichenoid sarcoidosis: a case with clinical and histopathological lichenoid features.

    PubMed

    Garrido-Ruiz, Maria C; Enguita-Valls, Ana B; de Arriba, Marta González; Vanaclocha, Francisco; Peralto, Jose Luis Rodriguez

    2008-06-01

    Sarcoidosis is a chronic multisystemic granulomatous disease of unknown etiology, characterized by the formation of noncaseating granulomas in the involved organs. Cutaneous involvement is about 25% with different clinical expressions, the lichenoid pattern being one of the rarest types of cutaneous sarcoidosis. Lichenoid sarcoidosis clinically manifests with multiple scale papules involving extensive skin areas, especially the trunk, limbs, and face mimicking a lichen planus. Although diverse histologic patterns have been previously related, a lichenoid granulomatous infiltrate involving the dermo-epidermal junction has never been reported in lichenoid sarcoidosis. We report a case of a 43-year-old woman presenting with skin-colored pruritic papules, slightly scaling in trunk, extremities, and ears. These symptoms condition continued to expand and worsen for several years. The patient was otherwise in good health with no lymphadenopathies. Histopathologic examination of a skin biopsy showed an upper dermal granulomatous infiltrate of epithelioid cells, without necrosis, distributed in a lichenoid pattern with many cytoid bodies. We consider this may be the first case presenting a characteristic microscopic granulomatous lichen-like pattern in the setting of a clinically lichenoid type of sarcoidosis.

  19. Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis.

    PubMed

    Mizuno, Kana; Nguyen, Chuyen Thi Hong; Ueda-Hayakawa, Ikuko; Okamoto, Hiroyuki

    2017-05-01

    Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62-year-old female was diagnosed with sarcoidosis by chest-abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non-caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. An atypical sarcoidosis involvement in FDG PET/CT

    PubMed Central

    Robin, Philippe; Benigni, Paolo; Feger, Benoit; Salaun, Pierre-Yves; Abgral, Ronan

    2016-01-01

    Abstract Rationale: Sarcoidosis is an idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis which involve various organs. Laryngeal involvement is extremely rare, with a prevalence of about 0.5 to 1%. Diagnoses: Here we present a case of laryngeal involvement of sarcoidosis demonstrated on 18F-Fluorodesoxyglucose Positron-Emission Tomography/Computed Tomography (FDG PET/CT). Patient concerns: A 63 year-old man suffering from dysphonia was referred to our department for characterization of laryngeal lesion suspicious for cancer with non-informative biopsy, the sample was not sufficient for diagnosis. Interventions: FDG PET/CT showed a pathological uptake on the right vocal cord, but also highlighted a bilateral uptake in intrathoracic hilar lymphadenopathy areas, typically found in several inflammatory diseases. Outcomes: New laryngeal targeted biopsies revealed non-caseating epithelioid granulomas suggesting sarcoidosis involvement. After 6 months of systemic steroid treatment, FDG PET/CT showed a significant decrease of the laryngeal uptake. Lessons: This case shows the usefulness of FDG PET/CT to accurately assess inflammatory activity in rare extra-pulmonary sarcoidosis involvement. Moreover, this case emphasizes that FDG PET/CT is an interesting tool for assessing therapeutic efficacy of inflammatory diseases such as sarcoidosis. PMID:28033265

  1. Sarcoidosis of the pineal gland: an unusual presentation of neurosarcoidosis

    PubMed Central

    Yang, Isaac; Delpolyi, Amy; Sughrue, Michael E.; Rubenstein, James; Bollen, Andrew W.

    2014-01-01

    Introduction Sarcoidosis is an inflammatory disease characterized by noncaseating granulomas that is rarely found as primary CNS pathology. We report an unusual case of sarcoidosis involving the pineal gland with radiographic, histopathology, and clinical data. Case report A 45-year-old man without evidence of systemic sarcoidosis presented with a history of gradual onset of blurry vision and diplopia that progressed over 3 months. MR imaging demonstrated an enhancing mass in the pineal region. A suboccipital craniotomy was performed with resection of the mass through a supra-cerebellar infratentorial approach. Histopathologic analysis did not reveal a pineoblastoma but instead revealed noncaseating granulomas within the pineal gland. Extensive hematologic laboratory examinations, cerebral spinal fluid studies, and cultures for infection were all negative. This mass lesion was diagnosed as solitary neurosarcoidosis of the pineal gland, without dissemination. The patient was treated with steroids and at 4-year follow-up is asymptomatic with an unremarkable MRI scan. Conclusion This is an unusual case of pineal sarcoidosis mimicking a tumor with associated MRI, CT and histopathologic findings reported together. Although rare, sarcoidosis of the pineal gland should not be excluded from a comprehensive differential diagnosis of an enhancing pineal region mass. PMID:18759061

  2. Current concepts regarding calcium metabolism and bone health in sarcoidosis.

    PubMed

    Baughman, Robert P; Papanikolaou, Ilias

    2017-09-01

    Vitamin D supplementation is widespread used in the general population. In sarcoidosis, up to 50% of patients, especially postmenopausal women and those taking corticosteroids, show evidence of increased bone fragility. The purpose of this review is to provide an evidence-based rationale on how to treat sarcoidosis patients with bone health issues. Evidence from observational studies show that decreased 25-hydroxy vitamin D is common in sarcoidosis. However, the great majority of sarcoidosis patents have normal or often elevated levels of 1,25-dihydroxy vitamin D (calcitriol), a marker associated with disease activity. High calcitriol levels may often be associated with hypercalcemia and hypercalcuria. The few interventional randomized controlled studies in the field, suggest that vitamin D supplementation may not be well tolerated because of hypercalcemia, moreover without substantial benefit on bone health and risk for fractures in these patients. Vitamin D supplementation may be withheld in sarcoidosis patients with bone fragility, unless calcitriol levels are below normal limits. A treating scheme is proposed.

  3. Carpal Tunnel Syndrome in Sarcoidosis: A Case Report of a Rare Neurologic Manifestation

    PubMed Central

    Sonambekar, Ajinkya; Gupta, Nikhil; Swadi, Akanksha; Tomar, Laxmikant Ramkumarsingh

    2016-01-01

    Introduction Sarcoidosis is a multisystemic inflammatory disease with myriad clinical manifestations. Neurologic involvement in sarcoidosis is uncommon. Peripheral neuropathic presentations include mononeuropathy, mononeuritis multiplex, and generalized sensory, motor, autonomic, and sensorimotor polyneuropathies. Case Presentation We report a case of carpal tunnel syndrome caused by sarcoidosis in a 30-year-old woman. Other causes of carpal tunnel syndrome were ruled out. The patient responded well to the standard line of corticosteroid treatment and wrist splinting. Discussion Carpal tunnel syndrome caused by sarcoidosis is a rare presentation. The mechanism of neurologic involvement in sarcoidosis is not clear. PMID:27643973

  4. Fatigue in sarcoidosis and idiopathic pulmonary fibrosis: differences in character and severity between diseases.

    PubMed

    Atkins, Christopher Peter; Gilbert, Daniel; Brockwell, Claire; Robinson, Sue; Wilson, Andrew Malcolm

    2016-08-01

    Sarcoidosis and idiopathic pulmonary fibrosis (IPF) are two common forms of interstitial lung disease. Fatigue is a recognised feature of sarcoidosis but an association between IPF and fatigue has not been investigated. To investigate the frequency and severity of fatigue in these groups, and variables affecting fatigue scores. A cross-sectional questionnaire study of patients with sarcoidosis and IPF followed-up at a single hospital was undertaken. Questionnaire data included validated measures of fatigue, anxiety, depression, sleepiness and dyspnoea, plus measures of disease severity including spirometry data. Questionnaires were administered to 232 patients (82 healthy volunteers, 73 sarcoidosis patients and 77 IPF patients). Sarcoidosis patients had statistically higher sleepiness scores but no significant difference was seen between overall measures of fatigue, anxiety or depression. Stratification by severity revealed a non-statistically significant tendency towards more severe fatigue scores in sarcoidosis. Regression analysis failed to identify any significant predictor variables measured in the sarcoidosis cohort, though in the IPF group both dyspnoea and sleepiness scores were significant predictors of fatigue (R2=0.74). Both sarcoidosis and IPF patients suffer with fatigue, although sarcoidosis patients tended towards reporting more severe fatigue scores, suggesting a subgroup with severe fatigue. The fatigue experienced by the two groups appears to be different; sarcoidosis patients report greater frequency of mental fatigue whereas IPF patients appear to suffer exhaustion, potentially related to dyspnoea. Dyspnoea and sleepiness scores modeled the majority of fatigue in the IPF group, whereas no single factor was able to predict fatigue in sarcoidosis.

  5. Detection of HTLV-I proviral DNA in sarcoidosis.

    PubMed

    Yajima, A; Kawada, A; Aragane, Y; Tezuka, T

    2001-01-01

    'Sarcoidosis-lymphoma syndrome' is known as an association of sarcoidosis with malignant lymphoma. We report a 56-year-old woman with systemic sarcoidosis who was seropositive for antibody against human T cell lymphoma/leukemia virus type I (HTLV-I). This patient showed integration of HTLV-I proviral DNA within cutaneous sarcoid nodules, but not in peripheral blood mononuclear cells. Neither atypical lymphocytes nor a T cell receptor beta1 gene rearrangement were observed in peripheral blood mononuclear cells or in cutaneous nodules, indicating that the patient did not have a smouldering type of adult T cell lymphoma/leukemia. Detection of integration of HTLV-I proviral DNA in cutaneous sarcoid nodules could suggest that the sarcoid nodules might have been generated as a protective response to chronic stimuli of HTLV-I.

  6. Bone cysts in sarcoidosis: what is their clinical significance?

    PubMed

    Yanardağ, Halil; Pamuk, Omer Nuri

    2004-09-01

    The incidence of bone cysts in sarcoidosis, a multisystemic disease, varies in different series, and these cysts are generally asymptomatic. We evaluated bone cysts in 516 sarcoidosis patients with available hand X-rays and in 200 patients with foot X-rays. Bone cysts were present in 21 cases (19 females, two males). The cysts were localized in phalanges of the hand in all cases, in feet in five cases, and in nasal bone in one. Six patients with bone cysts also had lupus pernio. Among those with cystic bone lesions, females were more frequent; extrapulmonary and skin involvement and lupus pernio were significantly more common. We diagnosed cystic bone lesions in 3.6% of our sarcoidosis patients and conclude that cysts were more frequent in females and in patients with lupus pernio.

  7. Anomalies in the dominant sarcoidosis paradigm justify its displacement.

    PubMed

    Reich, Jerome M

    2017-04-01

    The prevailing paradigm defines sarcoidosis as a disease of unknown etiology characterized by a systemic noncaseating epithelioid granulomatous response (SGR). This formulation fails to account for the elusiveness of the etiological agent, the nature of the Kveim response, the paradox of cutaneous delayed type hypersensitivity anergy in a setting of intense immune response and the appearance of SGR, indistinguishable from sarcoidosis, in persons with lymphohematogenous and solid neoplasms and a variety of cellular immune deficiencies. Displacing this formulation with an evidence-based alternative in which the SGR is viewed as an etiologically diverse, primitive immunological fallback due to inefficient cellular immune processing eliminates these shortcomings, providing a unifying accounting for the puzzles and violations of expectations associated with the prevailing paradigm. Its clinical import resides in the reported annual three-percent increase in sarcoidosis mortality in the past two decades (Swigris et al., 2011), which may be attributable, in part, to unneeded suppressive treatment.

  8. Sarcoidosis detected due to tattoo swellings in an Indian female.

    PubMed

    Ghorpade, Ashok

    2015-01-01

    A female patient presented with asymptomatic swellings in old blue-black tattoo marks of her forearms. On careful skin examination, few raised erythematous, circular plaques were also found on the non-tattooed skin on her back, about which she was unaware. The diagnosis of cutaneous and systemic sarcoidosis was confirmed after classical histopathological findings from skin lesions from her non tattooed back and from papulonodular tattooed lesions, ruling out other causes of granulomas and supported by a negative Mantoux test, raised serum angiotensin converting enzyme level, high resolution computed tomography thorax, etc. She responded well to oral steroid therapy. The importance of a careful skin examination, easy access provided by skin lesions in suspected cases of cutaneous/systemic sarcoidosis, the value of sudden tattoo changes in the diagnosis here and the possible contribution of tattoos toward the causation of cutaneous and/or systemic sarcoidosis are highlighted.

  9. Coexistence of sarcoidosis and adult onset Still disease.

    PubMed

    Semiz, Huseyin; Kobak, Senol

    2017-05-19

    Sarcoidosis is a chronic, inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It can be presented with bilateral hilar lymphadenopathy, skin lesions, eye involvement and locomotor system findings. Adult onset Still disease (AOSD) is a chronic inflammatory disease which presents with fever, arthritis and typical skin rashes. The disease is rare and can be misdiagnosed due to the absence of typical clinical and laboratory findings. The association of sarcoidosis and AOSD has not been previously reported in the literature. Herein we reported the development of AOSD in a patient followed by the diagnosis of sarcoidosis. The patient did not respond to high-dose corticosteroids and methotrexate therapy, and the disease was under control with anti-IL-6 (Tocilizumab) drug. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  10. Expression profiling elucidates a molecular gene signature for pulmonary hypertension in sarcoidosis

    PubMed Central

    Singla, Sunit; Zhou, Tong; Javaid, Kamran; Abbasi, Taimur; Casanova, Nancy; Zhang, Wei; Ma, Shwu-Fan; Wade, Michael S.; Noth, Imre; Sweiss, Nadera J.; Garcia, Joe G. N.

    2016-01-01

    Abstract Pulmonary hypertension (PH), when it complicates sarcoidosis, carries a poor prognosis, in part because it is difficult to detect early in patients with worsening respiratory symptoms. Pathogenesis of sarcoidosis occurs via incompletely characterized mechanisms that are distinct from the mechanisms of pulmonary vascular remodeling well known to occur in conjunction with other chronic lung diseases. To address the need for a biomarker to aid in early detection as well as the gap in knowledge regarding the mechanisms of PH in sarcoidosis, we used genome-wide peripheral blood gene expression analysis and identified an 18-gene signature capable of distinguishing sarcoidosis patients with PH (n = 8), sarcoidosis patients without PH (n = 17), and healthy controls (n = 45). The discriminative accuracy of this 18-gene signature was 100% in separating sarcoidosis patients with PH from those without it. If validated in a large replicate cohort, this signature could potentially be used as a diagnostic molecular biomarker for sarcoidosis-associated PH. PMID:28090288

  11. Diagnosis of sarcoidosis from a biopsy of a dilated mammary duct

    PubMed Central

    Yang, Robert; Hamilton, Raynal; Punar, Metin; Shah, Zeeshan; Spigel, Joseph; Wang, Jean

    2017-01-01

    Sarcoidosis is an immunologic disease of unknown etiology that manifests most frequently within the lungs or associated lymph nodes. Sarcoidosis involving the breast is seen in <1% of cases and usually is diagnosed in patients with multisystem disease. The clinical and imaging presentations of sarcoidosis of the breast can be variable. Though uncommon, sarcoidosis should be considered in the differential diagnosis of a breast lesion, and given that imaging characteristics cannot distinguish between sarcoidosis and malignancy, all breast lesions in patients with sarcoidosis should be biopsied. Our case study demonstrates a diagnosis of sarcoidosis in an asymptomatic patient presenting with a single dilated duct and associated filling defect within the right breast. PMID:28405081

  12. Laryngeal sarcoidosis: presentation and management in the pediatric population.

    PubMed

    Strychowsky, Julie E; Vargas, Sara O; Cohen, Ezra; Vielman, Rene; Son, Mary Beth; Rahbar, Reza

    2015-09-01

    Sarcoidosis is a disease characterized by systemic non-necrotizing granulomas of unknown etiology. Laryngeal sarcoidosis is extremely uncommon, especially among pediatric patients. The clinical presentation and management of this entity in the pediatric population are poorly understood. A comprehensive search in PubMed was conducted to identify all cases in the published literature. We also present a case of isolated pediatric laryngeal sarcoidosis and outline the multidisciplinary approach to evaluation and management. A previously healthy 13-year-old female presented with a five-month history of mild dysphonia, dyspnea on exertion, and diffuse supraglottic edema. Biopsy showed non-necrotizing granulomas. Treatment with methotrexate led to marked improvement. The literature search identified seven previously published cases of pediatric laryngeal sarcoidosis, four in which disease was isolated to the larynx. All patients presented with a symptomatic and diffusely edematous supraglottis. Diagnoses were based on supraglottic biopsies showing non-necrotizing granulomas; all other possible etiopathologies were excluded. Three patients responded to corticosteroid therapy alone, one patient to tumor necrosis factor (TNF) inhibitor and methotrexate, and the remainder to a combination of corticosteroid therapy and surgical debulking. Laryngeal sarcoidosis in the pediatric population is challenging to diagnose and manage. When epithelioid granulomas are encountered histologically, other causes of granulomatous inflammation must be ruled out before a diagnosis of sarcoidosis can be made. Corticosteroid therapy alone may be ineffective. Medical therapy with methotrexate alone or in combination with TNF inhibitors versus surgical debulking alone or as part of multimodality treatment should be considered. A multidisciplinary approach with involvement of an otolaryngologist, pathologist, and rheumatologist is suggested. Copyright © 2015 Elsevier Ireland Ltd. All rights

  13. Ocular disease and driving.

    PubMed

    Wood, Joanne M; Black, Alex A

    2016-09-01

    As the driving population ages, the number of drivers with visual impairment resulting from ocular disease will increase given the age-related prevalence of ocular disease. The increase in visual impairment in the driving population has a number of implications for driving outcomes. This review summarises current research regarding the impact of common ocular diseases on driving ability and safety, with particular focus on cataract, glaucoma, age-related macular degeneration, hemianopia and diabetic retinopathy. The evidence considered includes self-reported driving outcomes, driving performance (on-road and simulator-based) and various motor vehicle crash indices. Collectively, this review demonstrates that driving ability and safety are negatively affected by ocular disease; however, further research is needed in this area. Older drivers with ocular disease need to be aware of the negative consequences of their ocular condition and in the case where treatment options are available, encouraged to seek these earlier for optimum driving safety and quality of life benefits.

  14. Quantitative immunoelectrophoretic analysis in patients with tuberculosis and sarcoidosis

    PubMed Central

    Clarke, H. G. Minchin; Freeman, T.; Hickman, R.; Pryse-Phillips, W. E. M.

    1970-01-01

    A study of 12 immunologically distinct serum proteins has been made in two conditions, tuberculosis and sarcoidosis, which have in common the formation of giant-cell granulomata. Significantly (p<0·001) higher concentrations of five proteins [α2 group component (GC), caeruloplasmin (caer), haptoglobin (hpt), haemopexin (hpx), and α1 easily precipitable glycoprotein (α1 PGp)] were found in tuberculosis. No significant differences from normal were found in patients with sarcoidosis. The results obtained compared to the available figures for conventional electrophoresis and the so-called `acute phase reaction' are discussed. Images PMID:4098613

  15. Thermography in ocular inflammation

    PubMed Central

    Kawali, Ankush A

    2013-01-01

    Background and Objectives: The purpose of this study was to evaluate ocular inflammatory and non-inflammatory conditions using commercially available thermal camera. Materials and Methods: A non-contact thermographic camera (FLIR P 620) was used to take thermal pictures of seven cases of ocular inflammation, two cases of non-inflammatory ocular pathology, and one healthy subject with mild refractive error only. Ocular inflammatory cases included five cases of scleritis, one case of postoperative anterior uveitis, and a case of meibomian gland dysfunction with keratitis (MGD-keratitis). Non-inflammatory conditions included a case of conjunctival benign reactive lymphoid hyperplasia (BRLH) and a case of central serous chorio-retinopathy. Thermal and non-thermal photographs were taken, and using analyzing software, the ocular surface temperature was calculated. Results: Patient with fresh episode of scleritis revealed high temperature. Eyes with MGD-keratitis depicted lower temperature in clinically more affected eye. Conjunctival BRLH showed a cold lesion on thermography at the site of involvement, in contrast to cases of scleritis with similar clinical presentation. Conclusion: Ocular thermal imaging is an underutilized diagnostic tool which can be used to distinguish inflammatory ocular conditions from non-inflammatory conditions. It can also be utilized in the evaluation of tear film in dry eye syndrome. Its applications should be further explored in uveitis and other ocular disorders. Dedicated “ocular thermographic” camera is today's need of the hour. PMID:24347863

  16. Cluster bomb ocular injuries.

    PubMed

    Mansour, Ahmad M; Hamade, Haya; Ghaddar, Ayman; Mokadem, Ahmad Samih; El Hajj Ali, Mohamad; Awwad, Shady

    2012-01-01

    To present the visual outcomes and ocular sequelae of victims of cluster bombs. This retrospective, multicenter case series of ocular injury due to cluster bombs was conducted for 3 years after the war in South Lebanon (July 2006). Data were gathered from the reports to the Information Management System for Mine Action. There were 308 victims of clusters bombs; 36 individuals were killed, of which 2 received ocular lacerations and; 272 individuals were injured with 18 receiving ocular injury. These 18 surviving individuals were assessed by the authors. Ocular injury occurred in 6.5% (20/308) of cluster bomb victims. Trauma to multiple organs occurred in 12 of 18 cases (67%) with ocular injury. Ocular findings included corneal or scleral lacerations (16 eyes), corneal foreign bodies (9 eyes), corneal decompensation (2 eyes), ruptured cataract (6 eyes), and intravitreal foreign bodies (10 eyes). The corneas of one patient had extreme attenuation of the endothelium. Ocular injury occurred in 6.5% of cluster bomb victims and 67% of the patients with ocular injury sustained trauma to multiple organs. Visual morbidity in civilians is an additional reason for a global ban on the use of cluster bombs.

  17. Cluster Bomb Ocular Injuries

    PubMed Central

    Mansour, Ahmad M.; Hamade, Haya; Ghaddar, Ayman; Mokadem, Ahmad Samih; El Hajj Ali, Mohamad; Awwad, Shady

    2012-01-01

    Purpose: To present the visual outcomes and ocular sequelae of victims of cluster bombs. Materials and Methods: This retrospective, multicenter case series of ocular injury due to cluster bombs was conducted for 3 years after the war in South Lebanon (July 2006). Data were gathered from the reports to the Information Management System for Mine Action. Results: There were 308 victims of clusters bombs; 36 individuals were killed, of which 2 received ocular lacerations and; 272 individuals were injured with 18 receiving ocular injury. These 18 surviving individuals were assessed by the authors. Ocular injury occurred in 6.5% (20/308) of cluster bomb victims. Trauma to multiple organs occurred in 12 of 18 cases (67%) with ocular injury. Ocular findings included corneal or scleral lacerations (16 eyes), corneal foreign bodies (9 eyes), corneal decompensation (2 eyes), ruptured cataract (6 eyes), and intravitreal foreign bodies (10 eyes). The corneas of one patient had extreme attenuation of the endothelium. Conclusions: Ocular injury occurred in 6.5% of cluster bomb victims and 67% of the patients with ocular injury sustained trauma to multiple organs. Visual morbidity in civilians is an additional reason for a global ban on the use of cluster bombs. PMID:22346132

  18. Microbial Lineages in Sarcoidosis: A Metagenomic Analysis Tailored for Low Microbial Content Samples.

    PubMed

    Clarke, Erik L; Lauder, Abigail P; Hofstaedter, Casey E; Hwang, Young; Fitzgerald, Ayannah S; Imai, Ize; Biernat, Wojciech; Rękawiecki, Bartłomiej; Majewska, Hanna; Dubaniewicz, Anna; Litzky, Leslie A; Feldman, Michael D; Bittinger, Kyle; Rossman, Milton D; Patterson, Karen C; Bushman, Frederic D; Collman, Ronald G

    2017-08-28

    The etiology of sarcoidosis is unknown, but microbial agents are suspected as triggers. We sought to identify bacterial, fungal or viral lineages in specimens from sarcoidosis patients enriched relative to controls using metagenomic DNA sequencing. Since DNA from environmental contamination contributes disproportionately to samples with low authentic microbial content, we developed improved methods for filtering environmental contamination. We analyzed specimens from sarcoidosis subjects (n=93), non-sarcoidosis control subjects (n=72) and various environmental controls (n=150). Sarcoidosis specimens consisted of two independent sets of formalin-fixed, paraffin-embedded lymph node biopsies, bronchoalveolar lavage (BAL), Kveim reagent, and fresh granulomatous spleen from a sarcoidosis patient. All specimens were analyzed by bacterial 16S and fungal ITS rRNA gene sequencing. In addition, BAL was analyzed by shotgun sequencing of fractions enriched for viral particles, and Kveim and spleen were subjected to whole-genome shotgun sequencing. In one tissue set, fungi in the Cladosporiaceae family were enriched in sarcoidosis compared to non-sarcoidosis tissues; in the other tissue set, we detected enrichment of several bacterial lineages in sarcoidosis, but not Cladosporiaceae. BAL showed limited enrichment of Aspergillus fungi. Several microbial lineages were detected in Kveim and spleen, including Cladosporium. No microbial lineage was enriched in more than one sample type after correction for multiple comparisons. Metagenomic sequencing revealed enrichment of microbes in single types of sarcoidosis samples, but limited concordance across sample types. Statistical analysis accounting for environmental contamination was essential to avoiding false positives.

  19. An epidemiological perspective of the pathology and etiology of sarcoidosis.

    PubMed

    Sawahata, Michiru; Sugiyama, Yukihiko

    2016-08-01

    To update current knowledge on the pathology and etiology of sarcoidosis, here we review previous epidemiological research and discuss age-related differences and historical changes in the clinical characteristics of sarcoidosis we identified over the last four decades in Japan. Extrathoracic lymph node involvement was more common in young patients, while extrathoracic involvement of non-lymphatic organs and hypercalcemia were more common in older patients. Most patients in their 20s presented with bilateral hilar lymphadenopathy, but this was consistently less common among older patients. Over time, the distribution of age at diagnosis has shifted toward the older age group in the United States, Denmark, and Japan. In Japan, the incidence rate has been decreasing among young people, but there has consistently been a second peak among postmenopausal women. Age-related differences in the clinical presentation of sarcoidosis may reflect the pathways of causative antigens and the strengthening of immunoregulatory mechanisms with age. Internal and external environmental factors, such as exposure to diverse microorganisms, ovarian insufficiency, and active vitamin D deficiency, that may contribute to the onset of sarcoidosis must be identified in order to develop strategies for prevention and treatment.

  20. Adipophilin expression in necrobiosis lipoidica, granuloma annulare, and sarcoidosis.

    PubMed

    Schulman, Joshua M; LeBoit, Philip E

    2015-03-01

    Necrobiosis lipoidica (NL), granuloma annulare (GA), and sarcoidosis usually are distinguished by clinical presentation and routine microscopy, but their distinction can sometimes be challenging. Historically, a clue to diagnosing NL or GA has been the identification of lipid droplets in the areas of altered collagen, but such studies have required fresh frozen tissue, making them impractical. Here, we present the first report of immunohistochemical staining to detect adipophilin, a membrane protein in lipid droplets, in NL (n = 12), GA (n = 19), sarcoidosis (n = 12), and, as a control for nonspecific tissue damage, nongranulomatous cutaneous necrosis (n = 13). Four patterns of labeling were identified: (1) extracellular, within zones of altered collagen; (2) both intracellular and extracellular, after the distribution of palisaded or scattered histiocytes; (3) intracellular, within clustered histiocytes; and (4) periadnexal. All cases of NL demonstrated pattern 1; nearly all cases of GA (18/19) demonstrated pattern 2; most sarcoidosis (10/12) demonstrated pattern 3; and nongranulomatous necrosis demonstrated either pattern 4 (6/13) or did not stain (6/13), confirming that the antibody to adipophilin did not adhere nonspecifically to the damaged tissue. An additional set of 3 biopsies with overlapping or partially sampled features of NL, GA, and/or sarcoidosis subsequently confirmed the potential utility of adipophilin staining in diagnostically challenging cases. We conclude that the pattern of adipophilin expression is a useful adjunct in the evaluation of granulomatous dermatitis.

  1. Unilateral apical infiltrate as an initial presentation of pulmonary sarcoidosis.

    PubMed

    Tice, A W

    1981-11-01

    A unilateral, apical, pulmonary infiltrate was seen in an Air Force weapon systems officer stationed in the Philippines as an initial presentation of pulmonary sarcoidosis. The most obvious diagnosis for that geographic area is tuberculosis. Diagnosis must be pursued to evaluate all differential possibilities, with resort to open-lung or bronchoscopic biopsy, if necessary.

  2. Multifactorial Etiology Pulmonary Hypertension in a Patient with Sarcoidosis

    PubMed Central

    Lucas Vinícius da Fonseca, Barreto; Felipe Naze Rodrigues, Cavalcante; Joselina Luzia Menezes, Oliveira; Marcos Antônio, Almeida-Santos; José Augusto Soares, Barreto-Filho; Antônio Carlos Sobral, Sousa

    2016-01-01

    Differential diagnosis between pre- and postcapillary pulmonary hypertension (PH) in patients with diastolic heart failure (DHF) is a challenge in clinical practice. The presence of PH is implicated in worse prognosis in patients with this disease. This case report approaches the process of investigation of pulmonary hypertension in adult patient with DHF, double mitral lesion, and sarcoidosis with poor clinical outcome. PMID:27800190

  3. Systemic sarcoidosis complicated of acute renal failure: about 12 cases.

    PubMed

    Mahfoudhi, Madiha; Mamlouk, Habiba; Turki, Sami; Kheder, Adel

    2015-01-01

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, biological and histological profile in these cases and then to indicate the interest to consider the diagnosis of sarcoidosis in cases of unexplained renal failure. Extra-renal complications, therapeutic modalities and the outcome were determined in all patients. Our series involved 12 women with an average age of 40 years. Biological investigations showed an abnormal normocalcemia in 7 cases, a hypercalcemia in 5 cases, a hypercalciuria in 10 cases and polyclonal hypergammaglobulinemia in 7 cases. An acute renal failure was found in all patients with a median creatinin of 520 umol/L. For all patients, the renal echography was normal however, the kidney biopsy showed tubulo-interstitial nephritis. The extra-renal signs highlighting pulmonary interstitial syndrome in 5 cases, a sicca syndrome in 4 cases, mediastinal lymph nodes in 2 cases, a lymphocytic alveolitis in 3 cases, an anterior granulomatous uveitis in 2 cases and a polyarthritis in 5 cases. Five patients benefited of hemodialysis. The treatment consisted of corticosteroid in all cases. The follow up was marked by complete resolution of clinical and biological signs. The diagnosis of renal sarcoidosis must be done quickly to prevent renal failure.

  4. Renal failure due to granulomatous interstitial nephritis after pulmonary sarcoidosis.

    PubMed

    van Dorp, W T; Jie, K; Lobatto, S; Weening, J J; Valentijn, R M

    1987-01-01

    Two patients, who had had pulmonary sarcoidosis, developed renal failure due to sarcoid granulomatous interstitial nephritis after their original pulmonary symptoms had subsided. Treatment with prednisone resulted in almost complete recovery of renal function. Lysozyme and angiotensin-converting enzyme levels and gallium scintigraphy can be of diagnostic value.

  5. The intricate relationship of histoplasmosis and sarcoidosis: a case report.

    PubMed

    Mathur, Poonam; Zurlo, John J; Crook, Tonya J

    2014-06-27

    Histoplasmosis is an endemic mycosis with most cases of clinical illness reported in North and Central America. Rarely, patients develop progressive disseminated histoplasmosis with extrapulmonary manifestations. These infections are fatal if not appropriately treated. We report a case of progressive disseminated histoplasmosis presenting with fever, progressive dyspnea, and pancytopenia in a 51-year-old Caucasian man who had been treated with chronic steroids for a diagnosis of sarcoidosis made 20 years previously. His presentation was initially mistaken for sarcoidosis but, fortunately, laboratory results showed hematologic abnormalities, and the diagnosis of histoplasmosis was made by bone marrow biopsy. Sarcoidosis reduces T cell activity, and the addition of steroids for treatment causes further immunosuppression and vulnerability for development of a disseminated infection. The diagnosis of histoplasmosis depends mainly on clinical presentation and host factors. Although there are diagnostic laboratory tests available, clinicians may need to diagnose histoplasmosis by history and physical examination alone and treat empirically, since awaiting Histoplasma-specific laboratory results would delay initiation of treatment. Primary care providers, hospitalists, and subspecialists alike should be aware of the overlap in clinical and radiological presentations of sarcoidosis and histoplasmosis, and when and how to pursue diagnostic testing for endemic mycoses, since these infections can be fatal in immunosuppressed patients without appropriate treatment.

  6. Assessment of Cardiac Sarcoidosis with Advanced Imaging Modalities

    PubMed Central

    Akasaka, Takashi

    2014-01-01

    Sarcoidosis is a chronic systemic disease of unknown etiology that is characterized by the presence of noncaseating epithelioid granulomas, usually in multiple organs. Several studies have shown that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in genetically susceptible individuals. Cardiac involvement may occur and lead to an adverse outcome: the heart mechanics will be affected and that causes ventricular failure, and the cardiac electrical system will be disrupted and lead to third degree atrioventricular block, malignant ventricular tachycardia, and sudden cardiac death. Thus, early diagnosis and treatment of this potentially devastating disease is critically important. However, sensitive and accurate imaging modalities have not been established. Recent studies have demonstrated the promising potential of cardiac magnetic resonance imaging (MRI) and 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG PET) in the diagnosis and assessment of cardiac sarcoidosis (CS). In this review, we discuss the epidemiology, etiology, histological findings, and clinical features of sarcoidosis. We also introduce advanced imaging including 18F-FDG PET and cardiac MRI as more reliable diagnostic modalities for CS. PMID:25250336

  7. Identifying novel biomarkers in sarcoidosis using genome-based approaches

    PubMed Central

    Knox, Kenneth S.; Garcia, Joe G.N.

    2015-01-01

    Synopsis We briefly review conventional biomarkers used clinically to 1) support a diagnosis and 2) monitor disease progression in patients with sarcoidosis. We describe potential new biomarkers identified by genome-wide screening and the approaches to discover these biomarkers. PMID:26593137

  8. Diagnosis of Ocular Tuberculosis.

    PubMed

    Ang, Marcus; Vasconcelos-Santos, Daniel V; Sharma, Kusum; Accorinti, Massimo; Sharma, Aman; Gupta, Amod; Rao, Narsing A; Chee, Soon-Phaik

    2016-07-05

    Ocular tuberculosis remains a presumptive clinical diagnosis, as the gold standard tests for diagnosing ocular tuberculosis are often not useful: Mycobacterium tuberculosis cultures require weeks to process on Lowenstein-Jenson media and have low yield from ocular samples; while acid-fast bacilli smears or polymerase chain reaction detection of M. tuberculosis DNA have low sensitivities. Thus, diagnosis is often based on suggestive clinical signs, which are supported by positive investigations: tuberculin skin test or interferon-gamma release assays; chest X-ray findings suggestive of pulmonary tuberculosis, and/or evidence of associated systemic tuberculosis infections in the absence of other underlying disease. The aim of this review is to provide an update on the methods of diagnosing ocular tuberculosis, and discuss the challenges of its diagnosis. We also suggest a step-ladder approach to a more accurate diagnosis of ocular tuberculosis by combining the available diagnostic tests.

  9. GSTT1 and GSTM1 gene polymorphisims in sarcoidosis.

    PubMed

    Coskun, Funda; Karkucak, Mutlu; Yilmaz, Dilber; Yakut, Tahsin; Uzaslan, Esra

    2016-10-07

    Sarcoidosis is a granulomatous disease of unknown cause, which affects all systems, especially the lungs and the lymphatic system. Genetic and environmental factors are held accountable for the etiology. Based on the general opinion, sarcoidosis develops after exposure to a specific environmental agent by genetically susceptible individuals.  The present study aimed to evaluate the disease susceptibility of the GSTT1 and GSTM1 gene polymorphisms in the patients with sarcoidosis. The present study included 78 patients; 38 patients with histopathologically verified sarcoidosis and 40 control subjects. Multiplex PCR method was used to determine the GSTT1 and GSTM1 gene polymorphisms. The genotype was determined based on the bands formed in the agarose gel electrophoresis. The statistical analysis was done using the chi-square test. The positive/negative genotype rates were 79%/21% and 53%/47%, respectively in the case group for the GSTT1 and GSTM1 gene polymorphisms, whereas the positive/negative genotype rates were 77%/23% and 55%/45% in the control group. There was no statistically significant difference in the positive and negative genotypes compared with the case group and the control group for the GSTT1 and GSTM1 gene polymorphisms (p > 0.05). The results from the present study suggest that there is not any association with the control group for the disease susceptibility of the GSTT1 and GSTM1 gene polymorphisms in patients with sarcoidosis, and this result should be supported by large-scale studies because of the limited number of cases in the present study.

  10. The clinical significance of hematologic parameters in patients with sarcoidosis.

    PubMed

    Dirican, Nigar; Anar, Ceyda; Kaya, Sule; Bircan, Haci Ahmet; Colar, Huseyin Halil; Cakir, Munire

    2016-01-01

    Sarcoidosis is a multisystemic inflammatory granulomatous disease of unknown etiology. No suitable biomarkers are available to evaluate the prognosis of this disease, which still has an unpredictable clinical course. The aim of this study was to evaluate the potential clinical usefulness of hematologic markers. We investigated 172 subjects: 116 patients with sarcoidosis and 56 healthy individuals at Suleyman Demirel University and Dr. Suat Seren Chest Diseases and Thoracic Surgery Training Hospital. Complete blood count, demographics and pulmonary function test data from sarcoidosis patients between 2008 and 2013 were evaluated and collated retrospectively. The cut-off values were determined by calculating the neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) of the patients. The cut-off values were determined as 2 and 8.95 for NLR and MPV, respectively. NLRs were significantly higher in sarcoidosis patients than in healthy controls (P < 0.001) and were directly correlated with erythrocyte sedimentation rate (ESR) levels (R = 0.183, P = 0.017). Receiver operator characteristic (ROC) curve analysis revealed a 0.83 [confidence interval (CI) 68.8%-88.4%] area under the curve, 80% sensitivity and 59% specificity at the cut-off of NLR. Higher NLRs (≥2) were detected in patients with sarcoidosis than in the control group (P < 0.001). Also, high NLRs were more frequent in patients with extrapulmonary involvement (P = 0.031). MPV values were not different between control and patient groups. NLR may be a biomarker with good sensitivity that is easily detected in serum. It can be proposed in clinical practice to identify a patient's prognosis. However, large prospective studies are required to further demonstrate the prognostic significance of these values. © 2014 John Wiley & Sons Ltd.

  11. Identification of Immune-Relevant Factors Conferring Sarcoidosis Genetic Risk

    PubMed Central

    Fischer, Annegret; Ellinghaus, David; Nutsua, Marcel; Hofmann, Sylvia; Montgomery, Courtney G.; Iannuzzi, Michael C.; Rybicki, Benjamin A.; Petrek, Martin; Mrazek, Frantisek; Pabst, Stefan; Grohé, Christian; Grunewald, Johan; Ronninger, Marcus; Eklund, Anders; Padyukov, Leonid; Mihailovic-Vucinic, Violeta; Jovanovic, Dragana; Sterclova, Martina; Homolka, Jiri; Nöthen, Markus M.; Herms, Stefan; Gieger, Christian; Strauch, Konstantin; Winkelmann, Juliane; Boehm, Bernhard O.; Brand, Stephan; Büning, Carsten; Schürmann, Manfred; Ellinghaus, Eva; Baurecht, Hansjörg; Lieb, Wolfgang; Nebel, Almut; Müller-Quernheim, Joachim; Franke, Andre

    2015-01-01

    Rationale: Genetic variation plays a significant role in the etiology of sarcoidosis. However, only a small fraction of its heritability has been explained so far. Objectives: To define further genetic risk loci for sarcoidosis, we used the Immunochip for a candidate gene association study of immune-associated loci. Methods: Altogether the study population comprised over 19,000 individuals. In a two-stage design, 1,726 German sarcoidosis cases and 5,482 control subjects were genotyped for 128,705 single-nucleotide polymorphisms using the Illumina Immunochip for the screening step. The remaining 3,955 cases, 7,514 control subjects, and 684 parents of affected offspring were used for validation and replication of 44 candidate and two established risk single-nucleotide polymorphisms. Measurements and Main Results: Four novel susceptibility loci were identified with genome-wide significance in the European case-control populations, located on chromosomes 12q24.12 (rs653178; ATXN2/SH2B3), 5q33.3 (rs4921492; IL12B), 4q24 (rs223498; MANBA/NFKB1), and 2q33.2 (rs6748088; FAM117B). We further defined three independent association signals in the HLA region with genome-wide significance, peaking in the BTNL2 promoter region (rs5007259), at HLA-B (rs4143332/HLA-B*0801) and at HLA-DPB1 (rs9277542), and found another novel independent signal near IL23R (rs12069782) on chromosome 1p31.3. Conclusions: Functional predictions and protein network analyses suggest a prominent role of the drug-targetable IL23/Th17 signaling pathway in the genetic etiology of sarcoidosis. Our findings reveal a substantial genetic overlap of sarcoidosis with diverse immune-mediated inflammatory disorders, which could be of relevance for the clinical application of modern therapeutics PMID:26051272

  12. Thyroid function and autoimmunity in sarcoidosis: a case-control study.

    PubMed

    Ilias, I; Panoutsopoulos, G; Batsakis, C; Nikolakakou, D; Filippou, N; Christakopoulou, I

    1998-12-01

    To evaluate thyroid function and the presence of antithyroid autoantibodies in patients with sarcoidosis, compared to patients with chronic obstructive pulmonary disease (controls). Sera were obtained from 26 patients (19 women and 7 men) with active sarcoidosis, age and sex matched to 26 patients with diagnosed chronic obstructive pulmonary disease. Baseline thyrotropin, total triiodothyronine and thyroxine, antithyroglobulin autoantibodies, and antithyroid peroxidase autoantibodies were analyzed. Only antithyroglobulin autoantibodies were significantly elevated in sarcoidosis patients (p=0.041, Wilcoxon two-sample test). Basic thyroid function parameters, with the exception of antithyroglobulin autoantibodies, were within the normal limits in patients with sarcoidosis. Their presence should be interpreted as another characteristic of a generalized immune dysfunction in sarcoidosis. Screening of thyroid disease in patients with sarcoidosis does not seem necessary.

  13. Systemic sarcoidosis with hypercalcaemia, hypothalamic-pituitary dysfunction and thyroid involvement.

    PubMed

    Alsahwi, Nassib; Blavo, Delali; Karanchi, Harsha

    2016-08-05

    Sarcoidosis is a multisystem granulomatous disorder. The lungs are the principal organs affected, however, extrapulmonary involvement including disorders of the pituitary and thyroid glands has been reported but presentation with multiple endocrine manifestations is rare. We report the case of a 36-year-old African-American woman who presented with hypercalcaemia, abnormal thyroid function studies and secondary amenorrhoea. On workup including laboratory, radiological testing and biopsy she was diagnosed with sarcoidosis with multi-organ involvement. Endocrine manifestations included non-parathyroid hormone mediated hypercalcaemia related to sarcoidosis, thyroid involvement with sarcoidosis and hypothalamic-pituitary involvement with a sellar and suprasellar mass associated with secondary adrenal insufficiency, secondary hypogonadism, growth hormone deficiency and secondary hypothyroidism. We report to the best of our knowledge the first case of simultaneous multiple endocrine manifestations of sarcoidosis that included hypercalcaemia, hypopituitarism and sarcoidosis of the thyroid gland. 2016 BMJ Publishing Group Ltd.

  14. Sarcoidosis during etanercept treatment for rheumatoid arthritis in women with a history of bilateral oophorectomy.

    PubMed

    Sawahata, Michiru; Sigiyama, Yukihiko; Yamasawa, Hideaki; Miki, Atsushi; Yamamoto, Hisashi; Muto, Shigeaki; Yamamoto, Hironori; Bando, Masashi

    2016-08-01

    nd in immune-mediated inflammatory diseases, such as rheumatoid arthritis (RA). Paradoxically, this treatment induces sarcoidosis in a small population of RA patients as a class effect. A safer anti-TNF therapeutic strategy requires understanding of the risk factors for sarcoidosis. In Japan, TNF inhibitor was introduced in 2003. We reviewed 226 consecutive patients (65 men and 161 women) who were newly diagnosed with sarcoidosis between 2003 and 2012 at Jichi Medical University Hospital, Japan. We detected 3 cases in which sarcoidosis developed during etanercept treatment for RA. All 3 cases were women who had undergone bilateral oophorectomy more than 20 years earlier. Taken together with our previous epidemiologic findings of a consistently maintained second peak after menopause in the age-specific distribution of sarcoidosis in women over four decades, long-term insidious ovarian dysfunction was a possible risk factor for sarcoidosis under certain conditions, especially during etanercept treatment.

  15. Key messages from a rare case of annular sarcoidosis of scalp

    PubMed Central

    Bhushan, Premanshu; Thatte, Sarvesh S.; Singh, Avninder

    2016-01-01

    Sarcoidosis, a multisystem disease of obscure etiology, is characterized by the formation of noncaseating epithelioid cell granulomas in several organs or tissues. The diagnosis of sarcoidosis requires a compatible clinical picture, histologic demonstration of noncaseating granulomas, and exclusion of other diseases capable of producing similar histology or clinical features. The lung is the most commonly affected organ, but the skin is frequently involved. Sarcoidosis occurs worldwide and affects all ages and races with female predominance. Scalp involvement is decidedly rare among the myriad cutaneous manifestations of sarcoidosis. Alopecia is common in sarcoidosis and is generally scarring in nature. Annular sarcoidosis is a rare morphology and annular sarcoid of scalp is seldom reported. Herein we present a case of annular scalp sarcoid with systemic involvement and without alopecia. PMID:27294057

  16. Serum ACE Level in Sarcoidosis Patients with Typical and Atypical HRCT Manifestation

    PubMed Central

    Kahkouee, Shahram; Samadi, Katayoon; Alai, Ali; Abedini, Atefeh; Rezaiian, Lida

    2016-01-01

    Summary Background Sarcoidosis is an inflammatory disease that affects multiple organs. Before widespread use of computed tomography (CT), the severity of sarcoidosis was assessed based on chest X-ray abnormalities. HRCT can distinguish between active inflammatory changes and irreversible fibrosis. In this study, we analyzed different ACE levels in 148 patients diagnosed with sarcoidosis. Material/Methods We categorized these patients based on their HRCT results into four groups: 1) patients diagnosed with chronic disease; 2) patients diagnosed with non-chronic disease; 3) patients who exhibited typical HRCT changes; and 4) patients who exhibited atypical HRCT changes. Afterward the mean ACE level of each group was calculated and compared. Result The HRCT scans of chronic sarcoidosis patients tended to show more atypical sarcoidosis patterns. Moreover, there was a reverse correlation between chronicity and ACE level (P-value <0.05). Conclusions HRCT is another modality which would be useful when the diagnosis of sarcoidosis is not definite. PMID:27733890

  17. Sarcoidosis among U.S. Navy enlisted men, 1965-1993.

    PubMed

    1997-06-13

    Sarcoidosis is a multisystem granulomatous disease of unknown etiology with highest incidence among young and middle-aged adults. In the United States, the risk for sarcoidosis is substantially higher among blacks than among other races (1,2); however, the reasons for this association are unknown. In response to the occurrence of a case of sarcoidosis in a U.S. Navy (USN) enlisted man, CDC's National Institute for Occupational Safety and Health (NIOSH) analyzed USN data on cases of sarcoidosis diagnosed among active-duty enlisted personnel during 1965-1993. This report summarizes the findings of this analysis, which indicate that the incidence of sarcoidosis declined among USN enlisted men during 1965-1993, particularly among blacks, and that the risk for sarcoidosis was statistically associated with the assignment of USN enlisted men to aircraft carriers.

  18. Clinical presentations and diagnostic work-up in sarcoidosis: a series of Turkish cases (clinics and diagnosis of sarcoidosis).

    PubMed

    Kıter, Göksel; Müsellim, Benan; Cetinkaya, Erdoğan; Türker, Hatice; Kunt Uzaslan, A Esra; Yentürk, Esin; Uzun, Oğuz; Sağlam, Leyla; Özdemir Kumbasar, Özlem; Celik, Gökhan; Okumuş, Gülfer; Arbak, Peri Meram; Altıay, Gündeniz; Tabak, Levent; Sakar Coşkun, Ayşın; Erturan, Serdar; Türktaş, Haluk; Yalnız, Enver; Akkoçlu, Atilla; Öğüş, Candan; Doğan, Ömer Tamer; Özkan, Metin; Özkan, Serir; Uzel, Fatma Işıl; Öngen, Gül

    2011-01-01

    Sarcoidosis is an idiopathic granulomatous disease. It usually affects the lung. The diagnosis may be problematic since the known causes of granulomatous inflammation must be excluded. This multicenter study aimed to evaluate the clinical presentations and diagnostic approaches of sarcoidosis. The study protocol was sent via internet, and the participants were asked to send the information (clinical, radiological and diagnostic) on newly diagnosed sarcoidosis cases. 293 patients were enrolled within two years. Pulmonary symptoms were found in 73.3% of the patients, and cough was the most common one (53.2%), followed by dyspnea (40.3%). Constitutional symptoms were occured in half of the patients. The most common one was fatigue (38.6%). The most common physical sign was eritema nodosum (17.1%). The most common chest radiograhical sign was bilateral hilar lymphadenomegaly (78.8%). Staging according to chest X-ray has revealed that most of the patients were in Stage I and Stage II (51.9% and 31.7%, respectively). Sarcoidosis was confirmed histopathologically in 265 (90.4%) patients. Although one-third of the bronchoscopy was revealed normal, mucosal hyperemi (19.8%) and external compression of the bronchial wall (16.8%) were common abnormal findings. The 100% success rate was obtained in mediastinoscopy among the frequently used sampling methods. Transbronchial biopsy was the most frequently used method with 48.8% success rate. Considering sarcoidosis with its most common and also rare findings in the differential diagnosis, organizing the related procedures according to the possibly effected areas, and the expertise of the team would favour multimodality diagnosis.

  19. Comorbid autoimmune diseases in patients with sarcoidosis: A nationwide case-control study in Taiwan.

    PubMed

    Wu, Chi-Hung; Chung, Pei-I; Wu, Chen-Yi; Chen, Yen-Ta; Chiu, Yun-Wen; Chang, Yun-Ting; Liu, Han-Nan

    2017-04-01

    The association between sarcoidosis and autoimmune comorbidities has been reported, however, it has seldom been confirmed by a large nationwide study. Our study aimed to clarify the association between sarcoidosis and autoimmune comorbidities in the Taiwanese. A total of 1237 patients with sarcoidosis and 4948 age- and sex-matched control subjects were selected from the National Health Insurance Research Database of Taiwan from 1997 to 2010. Multiple logistic regressions were performed to calculate the odds of comorbidities between the two groups. The prevalence of sarcoidosis was 2.17/100 000 individuals in Taiwan. Sarcoidosis patients tended to run a higher risk of autoimmune comorbidities than the control group (17.6% vs 9.4%, P < 0.05). Autoimmune thyroid disease (adjusted odd ratio [aOR], 1.32; 95% confidence interval [CI], 1.05-1.64), Sjögren's syndrome (aOR, 11.6; 95% CI, 4.36-31.0) and ankylosing spondylitis (aOR, 3.80; 95% CI, 2.42-5.97) were significantly associated with sarcoidosis. The sex-stratified analyses were carried out to demonstrate a significant association of sarcoidosis with ankylosing spondylitis in both sexes, but with autoimmune thyroid disease in male patients and with Sjögren's syndrome female patients, respectively. Besides, the diagnosis of the autoimmune comorbidities strongly associated with sarcoidosis tended to be established after that of sarcoidosis. This study demonstrated that patients with sarcoidosis tended to have autoimmune thyroid disease, Sjögren's syndrome and ankylosing spondylitis, and the diagnosis of sarcoidosis usually preceded that of associated comorbidities. Clinicians should be alert to autoimmune comorbidities in patients with sarcoidosis. © 2016 Japanese Dermatological Association.

  20. Nail Sarcoidosis with and without Systemic Involvement: Report of Two Cases.

    PubMed

    Noriega, Leandro; Criado, Paulo; Gabbi, Tatiana; Avancini, João; Di Chiacchio, Nilton

    2015-09-01

    The most commonly seen nail change in sarcoidosis, often associated with chronic systemic disease, is onychodystrophy. Imaging of hands, feet and chest is of paramount importance when nail sarcoidosis is suspected, for evaluation of bone cysts and pulmonary involvement, respectively. We report 2 cases of sarcoidosis that developed nail involvement - one with pulmonary disease, and the other without any evidence of systemic involvement, which is very rare.

  1. Nail Sarcoidosis with and without Systemic Involvement: Report of Two Cases

    PubMed Central

    Noriega, Leandro; Criado, Paulo; Gabbi, Tatiana; Avancini, João; Di Chiacchio, Nilton

    2015-01-01

    The most commonly seen nail change in sarcoidosis, often associated with chronic systemic disease, is onychodystrophy. Imaging of hands, feet and chest is of paramount importance when nail sarcoidosis is suspected, for evaluation of bone cysts and pulmonary involvement, respectively. We report 2 cases of sarcoidosis that developed nail involvement – one with pulmonary disease, and the other without any evidence of systemic involvement, which is very rare. PMID:27170939

  2. Peripheral Blood Gene Expression as a Novel Genomic Biomarker in Complicated Sarcoidosis

    PubMed Central

    Sweiss, Nadera J.; Chen, Edward S.; Moller, David R.; Knox, Kenneth S.; Ma, Shwu-Fan; Wade, Michael S.; Noth, Imre; Machado, Roberto F.; Garcia, Joe G. N.

    2012-01-01

    Sarcoidosis, a systemic granulomatous syndrome invariably affecting the lung, typically spontaneously remits but in ∼20% of cases progresses with severe lung dysfunction or cardiac and neurologic involvement (complicated sarcoidosis). Unfortunately, current biomarkers fail to distinguish patients with remitting (uncomplicated) sarcoidosis from other fibrotic lung disorders, and fail to identify individuals at risk for complicated sarcoidosis. We utilized genome-wide peripheral blood gene expression analysis to identify a 20-gene sarcoidosis biomarker signature distinguishing sarcoidosis (n = 39) from healthy controls (n = 35, 86% classification accuracy) and which served as a molecular signature for complicated sarcoidosis (n = 17). As aberrancies in T cell receptor (TCR) signaling, JAK-STAT (JS) signaling, and cytokine-cytokine receptor (CCR) signaling are implicated in sarcoidosis pathogenesis, a 31-gene signature comprised of T cell signaling pathway genes associated with sarcoidosis (TCR/JS/CCR) was compared to the unbiased 20-gene biomarker signature but proved inferior in prediction accuracy in distinguishing complicated from uncomplicated sarcoidosis. Additional validation strategies included significant association of single nucleotide polymorphisms (SNPs) in signature genes with sarcoidosis susceptibility and severity (unbiased signature genes - CX3CR1, FKBP1A, NOG, RBM12B, SENS3, TSHZ2; T cell/JAK-STAT pathway genes such as AKT3, CBLB, DLG1, IFNG, IL2RA, IL7R, ITK, JUN, MALT1, NFATC2, PLCG1, SPRED1). In summary, this validated peripheral blood molecular gene signature appears to be a valuable biomarker in identifying cases with sarcoidoisis and predicting risk for complicated sarcoidosis. PMID:22984568

  3. Sarcoidosis Th17 Cells are ESAT-6 Antigen Specific but Demonstrate Reduced IFN-γ Expression

    PubMed Central

    Richmond, Bradley W.; Ploetze, Kristen; Isom, Joan; Chambers-Harris, Isfahan; Braun, Nicole A.; Taylor, Thyneice; Abraham, Susamma; Mageto, Yolanda; Culver, Dan A.; Oswald-Richter, Kyra A.; Drake, Wonder P.

    2013-01-01

    Rationale Sarcoidosis is a granulomatous disease of unknown etiology. Many patients with sarcoidosis demonstrate antigen-specific immunity to mycobacterial virulence factors. Th-17 cells are crucial to the immune response in granulomatous inflammation, and have recently been shown to be present in greater numbers in the peripheral blood and bronchoalveolar lavage (BAL) fluid (BALF) of sarcoidosis patients than healthy controls. It is unclear whether Th-17 cells in sarcoidosis are specific for mycobacterial antigens, or whether they have similar functionality to control Th-17 cells. Methods Flow cytometry was used to determine the numbers of Th-17 cells present in the peripheral blood and BALF of patients with sarcoidosis, the percentage of Th-17 cells that were specific to the mycobacterial virulence factor ESAT-6, and as well as to assess IFN-γ expression in Th-17 cells following polyclonal stimulation. Results Patients with sarcoidosis had greater numbers of Th-17 cells in the peripheral blood and BALF than controls and produced significantly more extracellular IL-17A (p=0.03 and p=0.02, respectively). ESAT-6 specific Th-17 cells were present in both peripheral blood and BALF of sarcoidosis patients (p<0.001 and p=0.03, respectively). After polyclonal stimulation, Th-17 cells from sarcoidosis patients produced less IFN-γ than healthy controls. Conclusions Patients with sarcoidosis have mycobacterial antigen-specific Th-17 cells peripherally and in sites of active sarcoidosis involvement. Despite the Th1 immunophenotype of sarcoidosis immunology, the Th-17 cells have reduced IFN-γ expression, compared to healthy controls. This reduction in immunity may contribute to sarcoidosis pathogenesis. PMID:23073617

  4. Ocular Emergencies: Red Eye.

    PubMed

    Tarff, Andreina; Behrens, Ashley

    2017-05-01

    "Red eye" is used as a general term to describe irritated or bloodshot eyes. It is a recognizable sign of an acute/chronic, localized/systemic underlying inflammatory condition. Conjunctival injection is most commonly caused by dryness, allergy, visual fatigue, contact lens overwear, and local infections. In some instances, red eye can represent a true ocular emergency that should be treated by an ophthalmologist. A comprehensive assessment of red eye conditions is required to preserve the patients visual function. Severe ocular pain, significant photophobia, decreased vision, and history of ocular trauma are warning signs demanding immediate ophthalmological consultation. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. Nanocrystal for ocular drug delivery: hope or hype.

    PubMed

    Sharma, Om Prakash; Patel, Viral; Mehta, Tejal

    2016-08-01

    The complexity of the structure and nature of the eye emanates a challenge for drug delivery to formulation scientists. Lower bioavailability concern of conventional ocular formulation provokes the interest of researchers in the development of novel drug delivery system. Nanotechnology-based formulations have been extensively investigated and found propitious in improving bioavailability of drugs by overcoming ocular barriers prevailing in the eye. The advent of nanocrystals helped in combating the problem of poorly soluble drugs specifically for oral and parenteral drug delivery and led to development of various marketed products. Nanocrystal-based formulations explored for ocular drug delivery have been found successful in achieving increase in retention time, bioavailability, and permeability of drugs across the corneal and conjunctival epithelium. In this review, we have highlighted the ocular physiology and barriers in drug delivery. A comparative analysis of various nanotechnology-based ocular formulations is done with their pros and cons. Consideration is also given to various methods of preparation of nanocrystals with their patented technology. This article highlights the success achieved in conquering various challenges of ocular delivery by the use of nanocrystals while emphasizing on its advantages and application for ocular formulation. The perspectives of nanocrystals as an emerging flipside to explore the frontiers of ocular drug delivery are discussed.

  6. A Very Rare Case -  Hairy Cell Leukemia in Patient with Sarcoidosis.

    PubMed

    Karadurmus, N; Erdem, G; Basaran, Y; Naharci, I; Tasci, C; Dogan, T; Ifran, A; Kaptan, K; Saglam, K; Beyan, C

    2015-01-01

    Although the coexistence of hairy cell leukemia with sarcoidosis has been reported in a few cases in the literature, in our case the patient had been diagnosed and followed about 10 years with sarcoidosis and massive splenomegaly. It has been demonstrated that T helper 1 cells exist in organs influenced by sarcoidosis. These cells produce IL-2 and IFN-γ and induce a nonspecific inflammatory response and granuloma formation. Also these cytokines may play a role in the development of hairy cell leukemia.Key words: hairy cell leukemia -  sarcoidosis - massive splenomegaly.

  7. Familial sarcoidosis in Finland and Hokkaido, Japan--a comparative study.

    PubMed

    Pietinalho, A; Ohmichi, M; Hirasawa, M; Hiraga, Y; Löfroos, A B; Selroos, O

    1999-06-01

    Two or more cases of sarcoidosis in one family is not unusual. To compare the frequencies of familial sarcoidosis in Finland and Hokkaido, Japan, and to analyse the type of associations reported, we collected data on all patients visiting hospitals for sarcoidosis in 1984 in Finland (1378 patients) and Hokkaido (208 patients), including information about familial sarcoidosis. We also analysed the familial cases seen among 571 sarcoidosis patients diagnosed at the Mjölbolsta hospital in Finland from 1955 to 1987 and among 686 Japanese patients seen in Sapporo from 1964 to 1988. In 1984, 50 sarcoidosis patients visiting Finnish hospitals and nine sarcoidosis patients in Hokkaido reported as familial cases. Of the sarcoidosis patients seen in Finland at the Mjolbolsta hospital in 1955-1987, 27 had a family member with the same disease, while this number was 20 in the Sapporo hospital in 1964-1988. Those surveys give a prevalence of familial sarcoidosis in Finland of 3.6-4.7% and in Hokkaido of 2.9-4.3%. Among familial cases, the dominating relationships were sister-brother and mother-child relationships.

  8. Transcriptome profiles in sarcoidosis and their potential role in disease prediction.

    PubMed

    Schupp, Jonas C; Vukmirovic, Milica; Kaminski, Naftali; Prasse, Antje

    2017-09-01

    Sarcoidosis is a systemic disease defined by the presence of nonnecrotizing granuloma in the absence of any known cause. Although the heterogeneity of sarcoidosis is well characterized clinically, the transcriptome of sarcoidosis and underlying molecular mechanisms are not. The signal of all transcripts, small and long noncoding RNAs, can be detected using microarrays or RNA-Sequencing. Analyzing the transcriptome of tissues that are directly affected by granulomas is of great importance to understand biology of the disease and may be predictive of disease and treatment outcome. Multiple genome wide expression studies performed on sarcoidosis affected tissues were published in the last 11 years. Published studies focused on differences in gene expression between sarcoidosis vs. control tissues, stable vs. progressive sarcoidosis, as well as sarcoidosis vs. other diseases. Strikingly, all these transcriptomics data confirm the key role of TH1 immune response in sarcoidosis and particularly of interferon-γ (IFN-γ) and type I IFN-driven signaling pathways. The steps toward transcriptomics of sarcoidosis in precision medicine highlight the potentials of this approach. Large prospective follow-up studies are required to identify signatures predictive of disease progression and outcome.

  9. Sarcoidosis with involvement of the paranasal sinuses - a retrospective analysis of 12 biopsy-proven cases

    PubMed Central

    2013-01-01

    Background Extrapulmonary involvement by sarcoidosis is observed in about 30–40% of patients with sarcoidosis. Little is known about the frequency and clinical characteristics of sinonasal sarcoidosis. Methods We retrospectively analyzed 12 cases of biopsy-proven sinonasal sarcoidosis. Patients were identified from a patient population of 1360 patients with sarcoidosis at the Outpatient Clinic for Sarcoidosis and Rare Lung Diseases at LungClinic Grosshansdorf, a tertiary care hospital for respiratory medicine. Results The most frequent signs and symptoms were nasal polyps (4 cases), epistaxis (3 cases), nasal crusts (8 cases) and anosmia (5 cases). Pulmonary sarcoidosis of the patients was staged as stage I (n = 1) and stage II (n = 11) on chest radiographs. Spirometry was normal in 11 patients. 7 patients had a diffusion capacity of the lung for carbon monoxide of less than 90% of predicted. Other organs were affected in 8 patients. All patients received systemic corticosteroid treatment and most patients received topical steroids. 5 patients received steroid sparing agents. Repeated sinus surgery had to be performed in 4 patients. Conclusions Sinonasal involvement is a rare disease manifestation of sarcoidosis with a frequency slightly lower than 1% in our patient population. The clinical course of sinonasal sarcoidosis can be complicated by relapse despite systemic immunosuppressive treatment and repeated sinus surgery. PMID:24070015

  10. [The morphological examination in the differential diagnosis of tuberculosis and sarcoidosis].

    PubMed

    Dvorkovskaia, I V; Maĭskaia, M Iu; Nasyrov, R A; Baranova, O P; Ariel', B M

    2014-01-01

    The paper considers the results of histological and bacterioscopic examinations of biopsy specimens from the lung and mediastinal lymph nodes of 120 patients with an unclear diagnosis of tuberculosis and sarcoidosis and with problem of their differential diagnosis. One hundred and five of these patients were microscopically diagnosed as having either tuberculosis or sarcoidosis. Additional examination of differently stained sections and that using immunohistochemical M. tuberculosis detection were conducted in 15 cases, which could diagnose tuberculosis and sarcoidosis in 7 and 8 patients, respectively. Bacterioscopic examination, in terms of extreme variability the phenotypic properties of mycobacteria, is of decisive importance in the differential diagnosis of tuberculosis and sarcoidosis.

  11. Ocular bobbing with survival

    PubMed Central

    Clarke, Charles; Stern, Richard

    1976-01-01

    A case is described of prolonged ocular bobbing with survival after subarachnoid haemorrhage from a pontine arteriovenous malformation. Hitherto, this sign has usually been associated with a rapidly fatal outcome. Images PMID:1255213

  12. Ultrasonography in ocular trauma.

    PubMed

    Dastevska-Djosevska, Emilija

    2013-01-01

    Ultrasonography is a non-invasive, simple and effective diagnostic method which enables visualization and evaluation of intraocular injury degree in cloudy eye media. The basic aim of this investigation was to find out the frequency of various types of ocular injuries using ultrasonography and to make an analysis of their frequency in relation to gender and age. This retrospective study included 182 patients hospitalized at the Clinic of Ophthalmology in Skopje due to mechanical eye trauma. The patients underwent ultrasonography on the Alcon Ultrascan Imagining System apparatus and Sonomed EZ Scan AB 5500+. B scan technique was used primarily, while the A scan had a positive and correlative role. Ocular trauma was more present in males (85.2%) compared to females (14.8%). 49.5% of the patients had open, and 50.5% had closed globe injuries. The most represented age group in ocular injuries was the age ranged from 51 to 60 years. There was no significant difference between the type of mechanical injury and the age (Chi-Squares=5.52 p=0.47895025). Ultrasonography showed that the most frequent pathologic result, both in open and closed globe injuries, was vitreous hemorrhage. Ultrasonography has an irreplaceable role in the clinical evaluation and management of ocular trauma. It showed that the most frequent finding in ocular trauma was vitreous haemorrhage, and the male gender was more frequently exposed to ocular trauma.

  13. An oral lesion as the primary clinical manifestation of sarcoidosis.

    PubMed

    Gill, I; Siddiqi, J

    2017-03-16

    An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. An incisional biopsy of the lesion was carried out, which unexpectedly revealed the presence of a non-caseating granulomatous inflammatory reaction. A referral was sent to the respiratory medicine team and a diagnosis was later confirmed of stage II sarcoidosis. This case report highlights the need for clinicians to be aware of all possible causes of oral lesions, including rare manifestations of underlying systemic disease.

  14. Diabetes insipidus secondary to sarcoidosis presenting with caseating granuloma.

    PubMed

    Alam, Taimour; Thomas, Steven

    2011-03-03

    Diabetes insipidus is a rare complication of sarcoid infiltration of the hypothalamic-pituitary region. Non-caseating granuloma formation is typical of sarcoidosis. Anterior and posterior pituitary function may be affected. MRI coupled with endocrinology assessment is the usual method of investigation. A 25-year-old Caucasian male with no significant medical history presented with polyuria and polydipsia. Water deprivation test confirmed diabetes insipidus. CT scanning of the chest confirmed lymphadenopathy. Lymph node biopsy revealed caseating granuloma. Extensive investigation for tuberculosis was negative. The patient was started on intranasal desmopressin and steroids with marked improvement in symptoms. This is the first reported case of neurosarcoidosis with diabetes insipidus and caseation on histology that we are aware of. Differentiating between caseation due to sarcoidosis and tuberculosis on histology is possible by the use of special stains. Return of normal endocrine function is unusual and the patient is likely to require desmopressin therapy for life.

  15. [Sudden cardiac death due to sarcoidosis. Case report].

    PubMed

    Sejben, István; Som, Zoltán; Cserni, Gábor

    2017-07-01

    Sarcoidosis is a systemic granulomatous disease of unknown aetiology, which is characterized by bilateral hilar lymphadenopathy and pulmonary disease. Clinically detected cardiac involvement occurs in 5% of sarcoid patients, although cardiac manifestations are discovered in 25% of the cases at autopsy. Sarcoid heart disease frequently causes atrioventricular block. The authors present the case of a 44-year-old man with bradycardia. On admission, second degree Mobitz II, then third degree atrioventricular block was diagnosed. Coronarography showed normal coronary arteries. 2.5 years following artificial Biotronik Entovis DR type pacemaker implantation, sudden cardiac death occurred. Autopsy revealed sarcoidosis with cardiac, pulmonary, splenic, renal and lymph node involvement. In case of young or middle-aged patients with atrioventricular block, it is best to search for other causes if the most common coronary origin can be excluded. Orv Hetil. 2017; 158(27): 1067-1070.

  16. Isolated extraocular orbital mass: a rare presentation of sarcoidosis.

    PubMed

    Sah, Birendra P; Sharma, Bikram; Iannuzzi, Michael C

    2016-10-07

    We report a case of orbital sarcoidosis in a 66 year old male who presented with one month history of right eye swelling and intermittent diplopia. MRI revealed an enhancing infiltrative soft tissue mass in the inferior aspect of the right orbit and biopsy of the mass demonstrated non-necrotizing granulomas. Chest CT scan was normal and PET scan showed no other organ involvement. He was treated with tapering doses of prednisone over six months. Although relapse occurred while tapering prednisone to 20 mg per day, he responded well to the addition of azathioprine with complete resolution of visual difficulties and orbital the mass on repeat MRI. Sarcoidosis, presenting as an isolated orbital mass is rare, can be successfully treated and should be included in differential diagnosis.

  17. Sarcoidosis of the lacrimal gland as a first manifestation.

    PubMed

    Peralta-Gómez, M Y; Ávila-Ocampo, K A; Huerta-Velázquez, S; Rivera-Salgado, M I

    2017-06-10

    The first manifestation of sarcoidosis is usually at the pulmonary level. The case is described of a 40-year-old female patient, who presented with an increased volume of the lacrimal gland and mechanical ptosis of upper left eyelid as the first expression of this disease. The diagnosis of systemic sarcoidosis with primary presentation of the lacrimal gland was made after performing several immunological studies with negative results, imaging studies, and taking of glandular and lymph node biopsies. A favourable response was achieved with oral methotrexate treatment. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  18. Diabetes insipidus secondary to sarcoidosis presenting with caseating granuloma

    PubMed Central

    Alam, Taimour; Thomas, Steven

    2011-01-01

    Diabetes insipidus is a rare complication of sarcoid infiltration of the hypothalamic-pituitary region. Non-caseating granuloma formation is typical of sarcoidosis. Anterior and posterior pituitary function may be affected. MRI coupled with endocrinology assessment is the usual method of investigation. A 25-year-old Caucasian male with no significant medical history presented with polyuria and polydipsia. Water deprivation test confirmed diabetes insipidus. CT scanning of the chest confirmed lymphadenopathy. Lymph node biopsy revealed caseating granuloma. Extensive investigation for tuberculosis was negative. The patient was started on intranasal desmopressin and steroids with marked improvement in symptoms. This is the first reported case of neurosarcoidosis with diabetes insipidus and caseation on histology that we are aware of. Differentiating between caseation due to sarcoidosis and tuberculosis on histology is possible by the use of special stains. Return of normal endocrine function is unusual and the patient is likely to require desmopressin therapy for life. PMID:22707619

  19. Advances in imaging for diagnosis and management of cardiac sarcoidosis.

    PubMed

    Aggarwal, Niti R; Snipelisky, David; Young, Phillip M; Gersh, Bernard J; Cooper, Leslie T; Chareonthaitawee, Panithaya

    2015-09-01

    Sarcoidosis is a multisystem granulomatous disorder with a high prevalence of cardiac involvement. Cardiac sarcoidosis (CS) may be life threatening due to end-stage cardiomyopathy and sudden cardiac death. The frequent absence of specific symptoms and lack of a diagnostic 'gold standard' pose challenges in the diagnosis of CS. Endomyocardial biopsy, although specific, has an unacceptably low sensitivity. Non-invasive cardiac imaging has a huge role in the assessment of patients with known or suspected CS. This comprehensive review compares the diagnostic accuracy, along with advantages and disadvantages, of established and emerging imaging modalities for CS. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

  20. Bilateral parotitis as the initial presentation of childhood sarcoidosis.

    PubMed

    Banks, Gretchen C; Kirse, Daniel J; Anthony, Evelyn; Bergman, Simon; Shetty, Avinash K

    2013-01-01

    The differential diagnosis of bilateral parotid gland enlargement in children includes infectious, inflammatory, and neoplastic disorders. We present the case of a 13-year-old male who presented with a 5-week history of bilateral parotid swelling. On exam, both parotid glands were nontender, smooth, and diffusely enlarged. He had slightly elevated inflammatory markers, but other lab results were normal. A neck CT revealed symmetric enlargement of the parotid, submandibular, and sublingual glands. A chest CT revealed scattered peripheral pulmonary nodules and bilateral hilar adenopathy. A parotid gland biopsy showed multiple noncaseating granulomas with multinucleated giant cells surrounded by lymphocytes, consistent with the diagnosis of sarcoidosis. Special stains for acid-fast and fungal organisms were negative. Using this illustrative case, we discuss the differential diagnosis of bilateral salivary gland enlargement in children and review the etiology, diagnosis, clinical manifestations, and treatment of pediatric sarcoidosis. Copyright © 2013 Elsevier Inc. All rights reserved.

  1. Undiagnosed renal sarcoidosis in a patient with chronic interstitial nephritis.

    PubMed

    Dong, Wenfang; Qiu, Bin; Liu, Hongfeng; He, Leren

    2017-09-05

    A 53-year-old female was admitted to hospital, with acute elevation of SCr and hypercalcemia, on a 5-year history of chronic interstitial nephritis and stage III chronic kidney disease (CKD). Extensive workup failed to yield a definitive diagnosis concerning the cause of the disorder. Intense uptake of (18)F-FDG in the spleen and liver was detected by PET/CT imaging with negative angiotensin-converting enzyme (ACE) in serum. The spleen and the hypermetabolism nodules of the liver were resected for histopathologic examination and turned out to be noncaseating granulomas, likely sarcoidosis. Combined with clinical features, the final diagnosis for this patient was sarcoidosis, involving the kidneys, spleen, liver, and lungs.

  2. Circulating cytokines in sarcoidosis: Phenotype-specific alterations for fibrotic and non-fibrotic pulmonary disease

    PubMed Central

    Patterson, Karen C.; Franek, Beverly S.; Müller-Quernheim, Joachim; Sperling, Anne I.; Sweiss, Nadera J.; Niewold, Timothy B.

    2013-01-01

    Aims Sarcoidosis is a granulomatous disease of unknown etiology marked by tremendous clinical heterogeneity. Many patients enter remission with good long-term outcomes. Yet, chronic disease is not uncommon, and this important phenotype remains understudied. Identified alterations in local and circulating cytokines—specifically targeted for study, and often in the acute phase of disease—have informed our growing understanding of the immunopathogenesis of sarcoidosis. Our aim was to evaluate a broad panel of circulating cytokines in patients with chronic sarcoidosis. Among those with chronic disease, pulmonary fibrosis occurs in only a subset. To gain more insight into the determinants of the fibrotic response, we also determined if the phenotypes of fibrotic and non-fibrotic pulmonary sarcoidosis have distinct cytokine profiles. Results In patients with sarcoidosis compared to controls, IL-5 was decreased, and IL-7 was increased. Both of these comparisons withstood rigorous statistical correction for multiple comparisons. GM-CSF met a nominal level of significance. We also detected an effect of phenotype, where IL-5 was significantly decreased in non-fibrotic compared to fibrotic pulmonary sarcoidosis, and compared to controls. Compared to controls, there was a trend towards a significant increase in IL-7 in fibrotic, but not in non-fibrotic pulmonary sarcoidosis. In contrast, compared to controls, there was a trend towards a significant increase in GM-CSF in non-fibrotic, but not in fibrotic pulmonary sarcoidosis. Conclusions In a comprehensive evaluation of circulating cytokines in sarcoidosis, we found IL-5, IL-7, and GM-CSF to be altered. These findings provide a window into the immunopathogenesis of sarcoidosis. IL-7 is a novel sarcoidosis cytokine and, as a master regulator of lymphocytes, is an attractive target for further studies. By observing an effect of phenotype upon cytokine patterns, we also identify specific immune alterations which may

  3. Sarcoidosis in an Italian province. Prevalence and environmental risk factors

    PubMed Central

    Beghè, Deborah; Dall’Asta, Luca; Garavelli, Claudia; Pastorelli, Augusto Alberto; Muscarella, Marilena; Saccani, Gloria; Aiello, Marina; Corradi, Massimo; Stacchini, Paolo; Chetta, Alfredo; Bertorelli, Giuseppina

    2017-01-01

    Background and aim Sarcoidosis is a systemic granulomatous inflammatory disease whose causes are still unknown and for which epidemiological data are often discordant. The aim of our study is to investigate prevalence and spatial distribution of cases, and identify environmental exposures associated with sarcoidosis in an Italian province. Methods After georeferentiation of cases, the area under study was subdivided with respect to Municipality and Health Districts and to the altitude in order to identify zonal differences in prevalence. The bioaccumulation levels of 12 metals in lichen tissues were analyzed, in order to determine sources of air pollution. Finally, the analysis of the correlation between metals and between pickup stations was performed. Results 223 patients were identified (58.3% female and 41.7% male of total) and the mean age was 50.6±15.4 years (53.5±15.5 years for the females and 46.5±14.4 for the males). The mean prevalence was 49 per 100.000 individuals. However, we observed very heterogeneous prevalence in the area under study. The correlations among metals revealed different deposition patterns in lowland area respect to hilly and mountain areas. Conclusions The study highlights a high prevalence of sarcoidosis cases, characterized by a very inhomogeneous and patchy distribution with phenomena of local aggregation. Moreover, the bioaccumulation analysis was an effective method to identify the mineral particles that mostly contribute to air pollution in the different areas, but it was not sufficient to establish a clear correlation between the onset of sarcoidosis and environmental risk factors. PMID:28475583

  4. Sarcoidosis associated with infliximab therapy in ulcerative colitis

    PubMed Central

    Gîlcă, Georgiana-Emmanuela; Diaconescu, Smaranda; Bălan, Gheorghe Gh.; Timofte, Oana; Ştefănescu, Gabriela

    2017-01-01

    Abstract Rationale: Although immunomodulatory therapy has been clearly stated as an important landmark in treatment of ulcerative colitis, significantly improving the quality of life for patients with inflammatory bowel disease, there are several aspects to be considered regarding the possible side-effects of anti-TNF alpha agents. In spite of a good safety profile, biologic TNF antagonists may induce paradoxical inflammation, which can manifest as sarcoid-like granulomatosis, consisting of noncaseating granulomas in the affected organs. Patient concerns: We report the case of a 30-year-old male patient, with no personal or familial history of lung disease, with a personal history of ulcerative colitis (UC), under clinical remission following infliximab therapy in maintenance dose, who was admitted for treatment administration, but also for dyspnea, nocturnal sweating, and nonproductive cough. Diagnoses: Based on clinical manifestations, biological landmarks excluding various infections, CT scan, fibrobronchoscopy with bronchoalveolar lavage for culture and immunohistochemical examination, followed by mediastinoscopy with sampling of paratracheal lymph node, which underwent histopathological examination, the patient was diagnosed with drug- induced stage II pulmonary sarcoidosis. Interventions: Since the patient had developed severe allergic reaction after being administered Infliximab at admission, the biological treatment was immediately discontinued. Following the diagnosis of pulmonary sarcoidosis, corticotherapy was initiated. Patient outcomes: After corticotherapy was initiated, the patient had a favorable outcome at 3 months reevaluation, both regarding the course of ulcerative colitis and sarcoidosis. Lessons: Patients under biological therapy using anti-TNF alpha agents must be carefully monitored, in order to early identify potential paradoxical inflammation (such as sarcoidosis) as a side-effect. The drug-related pulmonary disease tends to improve upon

  5. Biopsy proven pituitary sarcoidosis presenting as a possible adenoma.

    PubMed

    Prayson, Richard A

    2016-12-01

    Sarcoidosis is a well-recognized systemic granulomatous process which involves the central nervous system in 5-15% of patients. One of the more frequent sites of central nervous system involvement is the pituitary and hypothalamic region. Involvement of the sellar region by sarcoidosis is overall an infrequent occurrence, comprising less than 1% of all intrasellar lesions. Patients typically present with an infiltrative lesion on imaging studies and clinically with symptoms related to diabetes insipidus or hyperprolactinemia. This report describes a 38-year-old woman who initially presented with a variety of symptoms including headaches, light sensitivity, nausea and vomiting, acute visual changes, cold intolerance, amenorrhea, decreased libido, fatigue and galactorrhea. She had an elevated serum prolactin level and evidence of oligoclonal bands in the cerebrospinal fluid. Imaging studies discovered a 1.8cm mass involving the pituitary gland and compressing the optic chiasm. The lesion was excised and microscopically was marked by a chronic inflammatory cell infiltrate and scattered nonnecrotizing granulomas. Stains and microbiologic cultures failed to demonstrate microorganisms. There was no evidence of other organ involvement on postoperative imaging. She was treated with prednisone with improvement of symptoms and subsequently required methotrexate to treat left eye pain and blurred vision, 29months after her surgery. Achieving treatment control in patients with pituitary and hypothalamic improvement in sarcoidosis still remains a challenge.

  6. Sudden unexpected death due to severe pulmonary and cardiac sarcoidosis.

    PubMed

    Ginelliová, Alžbeta; Farkaš, Daniel; Farkašová Iannaccone, Silvia; Vyhnálková, Vlasta

    2016-09-01

    In this paper we report the autopsy findings of a 57 year old woman who died unexpectedly at home. She had been complaining of shortness of breath, episodes of dry coughing, and nausea. Her past medical and social history was unremarkable. She had no previous history of any viral or bacterial disease and no history of oncological disorders. Autopsy revealed multiple grayish-white nodular lesions in the pleura and epicardial fat and areas resembling fibrosis on the cut surface of the anterior and posterior wall of the left ventricle and interventricular septum. Histological examination of the lungs and heart revealed multiple well-formed noncaseating epithelioid cell granulomas with multinucleated giant cells. Death was attributed to myocardial ischemia due to vasculitis of intramural coronary artery branches associated with sarcoidosis. Sarcoidosis is a multisystemic disease of unknown etiology characterized by the formation of noncaseating epithelioid cell granulomas in the affected organs and tissues. The diagnosis of sarcoidosis in this case was established when other causes of granulomatous disease such as tuberculosis, berylliosis, hypersensitivity pneumonitis, and giant cell myocarditis had been reasonably excluded.

  7. Myocardial contractile patterns predict future cardiac events in sarcoidosis.

    PubMed

    Chen, Jian; Lei, Juan; Scalzetti, Ernest; McGrath, Mary; Feiglin, David; Voelker, Robert; Wang, Jingfeng; Iannuzzi, Michael C; Liu, Kan

    2017-09-09

    The poor prognosis of cardiac sarcoidosis (CS) underscores the need for risk stratification. We evaluated 84 consecutive sarcoidosis patients who were referred for echocardiographic studies for cardiac symptoms or abnormal electrocardiograms. In 54 patients without previous diagnosis of CS or other known structural heart disease, 13 reached endpoints during (median) 24 months follow up. Significantly impaired peak systolic longitudinal strain in their original echocardiograms were identified in 13 of 17 left ventricular segments, clustering in the free wall, interventricular septum and apex. The regional (including 13 clustered segments) peak systolic longitudinal strain (RPSLS) were significantly impaired in patients with endpoints, compared with those without [(-11.4 ± 4.45) vs. (-18.7 ± 3.76) %, P < 0.00001]. Cox multivariate regression analysis revealed that RPSLS was independently associated with endpoints (HR 1.24; 95% CI 1.08-1.42, P = 0.002). Receiver operating characteristic curve suggested a cut-off RPSLS value of -15.0% (84.6% sensitivity and 86.8% specificity) to predict the occurrence of endpoints. Impaired RPSLS correlates with risk of adverse cardiac events in patients with extra-cardiac sarcoidosis.

  8. Detection of Myocardial Sarcoidosis by Thallium 201 Imaging

    PubMed Central

    Haywood, L. Julian; Sharma, Om P.; Siegel, Michael E.; Siegel, Robert J.; Gottlieb, Stephen L.; Caldwell, John; Siemsen, Jan K.

    1982-01-01

    Thallium 201 myocardial perfusion scanning revealed focal left ventricular defects at rest in four of 30 patients (13 percent) with systemic sarcoidosis. All the defects decreased in size during thallium stress imaging, a finding opposite of that usually seen in myocardial ischemia. Nine patients had abnormal right ventricular visualization at rest. Thus, 11 of 30 (37 percent) had abnormal resting myocardial scand. Two of the four patients with left ventricular defects were studied serially for up to 1.5 years to evaluate response to corticosteroid therapy. The myocardial defect diminished in one patient, whereas the second patient, maintained for 2 years on low doses of steroids, had no resolution. In patients suspected of focal myocardial involvement by sarcoidosis on thallium scanning dynamic changes in scan defects can occur during exercise which are the reverse of those usually seen in coronary artery disease. These defects probably represent myocardial sarcoid. Right ventricular uptake generally appears to correlate with the severity of pulmonary involvement by sarcoidosis, yet does not appear to be a specific finding. ImagesFigure 1Figure 2 PMID:7143469

  9. Pulmonary granulomatous reaction: talc pneumoconiosis or chronic sarcoidosis?

    PubMed Central

    Tukiainen, P; Nickels, J; Taskinen, E; Nyberg, M

    1984-01-01

    A chronic pulmonary granulomatous reaction was associated with an almost identical clinical picture in two patients exposed to talc. In both patients lung biopsy showed the deposition of talc particles and a heavy granulomatous reaction. At the time of diagnosis the Kveim test result was negative in both patients, urinary calcium excretion was normal, and there were no extrapulmonary manifestations and no response to steroid treatment. These findings point against sarcoidosis. The serum angiotensin-converting enzyme level, however, was raised in both patients. It was concluded that the patient who was exposed to talc in the rubber industry had a true talc pneumoconiosis. The other patient, who was exposed to cosmetic talcum powder, suffered from chronic sarcoidosis with talc deposition in the lungs, since an enlarged axillar lymph node containing granulomatous inflammation was discovered after two years' follow up. These cases show that it may be extremely difficult to differentiate between chronic sarcoidosis and talc pneumoconiosis even after careful clinical and histological analysis. Images PMID:6691939

  10. The "mystery" of cutaneous sarcoidosis: facts and controversies.

    PubMed

    Tchernev, G; Cardoso, J C; Chokoeva, A A; Verma, S B; Tana, C; Ananiev, J; Gulubova, M; Philipov, S; Kanazawa, N; Nenoff, P; Lotti, T; Wollina, U

    2014-01-01

    The reason why the cutaneous form of sarcoidosis is well known in the literature is because of its spectrum of manifestations granting it the fame of a Great Imitator. The mystery shrouding the pathogenesis of this rare cutaneous disease is still there (in spite of the fundamental progress of the various diagnostic methods in current day medicine). The production of the morphological substrate - the epithelioid cell granuloma - which is considered to be characteristic of skin sarcoidosis, could, however, also be the end result of a reaction to i) various specific infectious agents such as Leishmaniasis cutis, coccidioidomycosis, etc., ii) certain residual bacterial or other mycobacterial antigens which, at the moment of setting the diagnosis are - by definition - non-infectious but still immunogenic, as well as iii) different tumor antigens in lesional tissue or other location. Often, differentiating between sarcodiosis and a sarcoid-like reaction, based on the updated criteria for cutaneous sarcoidosis, is problematic to downright impossible. A future characterization of the genetic signature of the two conditions, as well as the implementation of additional mandatory panels for i) the identification of certain infectious or ii) non-infectious but immunogenic and iii) tumor antigens in the epithelioid cell granuloma (or in another location in the organism), could be a considerable contribution to the process of differentiating between the two above-mentioned conditions. This will create conditions for greater accuracy when setting the subsequent therapeutic approaches.

  11. Genitourinary sarcoidosis: An essential review for the practicing clinician

    PubMed Central

    Block, Norman L.; Kava, Bruce R.

    2017-01-01

    Introduction: Sarcoidosis is a multisystem disease that commonly involves the lungs, but may also present with extrapulmonary manifestations. Genitourinary (GU) tract involvement has been traditionally thought to be rare, but that view may underestimate the true prevalence of the disease due to the often, silent presentation thereof. Methods: The literature pertaining to sarcoidosis from the general systemic point of view, etiology and therapy and with regard to specific organs was reviewed by identifying key words in a PubMed search. That material with special relevance to the Indian experience was emphasized. Results: There are a number of isolated case reports in the literature describing symptomatic and asymptomatic GU tract sarcoidosis. The world literature associated with the generalized syndrome was reviewed and summarized. Specific aspects of GU involvement are presented for each organ of the GU tract. Conclusions: It is critical for the practicing clinician to have a working knowledge of the clinical manifestations of this disease as it involves the GU tract, as well as to be able to distinguish it from tuberculosis and the various malignancies that affect this organ system. PMID:28197023

  12. Right ventricular sarcoidosis: is it time for updated diagnostic criteria?

    PubMed

    Vakil, Kairav; Minami, Elina; Fishbein, Daniel P

    2014-04-01

    A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient's clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient's right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient's case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.

  13. Moyamoya disease associated with pulmonary sarcoidosis--case report.

    PubMed

    Takenaka, K; Ito, M; Kumagai, M; Yamakawa, H; Sugimoto, Y; Yamakawa, H; Nishimura, Y; Sakai, N

    1998-09-01

    A 61-year-old female presented with a unique case of moyamoya disease associated with pulmonary sarcoidosis. She was admitted for sudden onset of left temporalgia with episode of numbness on face, tongue, and upper extremity on the right side. The next morning, she had symptoms of Gerstmann syndrome and her ability to speak was disturbed. Her medical history included radical resection of lung cancer on the right side. She had no symptoms of pulmonary sarcoidosis. Neuroimaging showed an infarction in the left occipital lobe. Angiography showed occlusions of the bilateral internal carotid arteries at the supraclinoid portions. Subsequently, a left superficial temporal artery-middle cerebral artery anastomosis with encephalo-myo-synangiosis was performed. Ninety-three days after admission, she suddenly developed dyspnea which resulted in death 3 hours later. Autopsy findings showed typical epithelioid granulomas of sarcoid type in the lymph nodes of the peribronchus, lung, and liver. Thrombotic emboli were found in the bilateral pulmonary arteries, and marked fibrous intimal thickening in the bilateral internal carotid arteries. Immunological reaction with inflammatory events may cause pathological changes in patients with moyamoya disease or sarcoidosis. The co-incidence in this case suggests that some common inflammatory events may be involved in the pathogenesis of these diseases.

  14. Lesion of a lower lip scar--an initial presentation of sarcoidosis.

    PubMed

    Ribeiro de Castro, Maria Cristina; Maya, Tullia Cuzzi; Pereira, Lea Freitas; Lugon, Nilza Viana; Ramos-e-Silva, Marcia

    2002-09-01

    The development of sarcoidosis at the site of previous scar is unusual and may be the first sign of systemic disease. Sarcoidosis on a lower lip scar persisting for 13 years, which pointed to examination for internal manifestations, is presented along with a review of the subject

  15. Risk of fragility fracture among patients with sarcoidosis: a population-based study 1976-2013.

    PubMed

    Ungprasert, P; Crowson, C S; Matteson, E L

    2017-02-16

    Incidence of fragility fracture of a population-based cohort of 345 patients with sarcoidosis was compared with age and sex-matched comparators. The incidence of fragility fracture was higher among patients with sarcoidosis with hazard ratio (HR) of 2.18.

  16. A case of isolated sacral and pelvic sarcoidosis diagnosed by bone marrow biopsy.

    PubMed

    Garwood, Amy S; Mikuls, Ted R

    2003-10-01

    Although sarcoidosis frequently involves bone, it rarely presents as isolated osseous disease. We present a case in which sarcoidosis is limited to the sacrum and pelvis, a case that underscores the potential importance of both magnetic resonance imaging (MRI) and bone marrow biopsy in establishing the diagnosis. Additionally, we report the interval resolution of bony disease by MRI following glucocorticoid treatment.

  17. Management of cardiac sarcoidosis in the United States: a Delphi study.

    PubMed

    Hamzeh, Nabeel Y; Wamboldt, Frederick S; Weinberger, Howard D

    2012-01-01

    No formal guidelines exist to guide physicians caring for patients with sarcoidosis in their screening for management of patients with cardiac sarcoidosis. We conducted a modified Delphi study to investigate if a consensus could be reached on the best approaches for screening for and management of cardiac sarcoidosis. A modified Delphi study design with two rounds of questionnaires was used to investigate if a consensus existed among sarcoid experts in the United States on the best management approaches for cardiac sarcoidosis. Experts were identified based on their national reputation as sarcoid experts and by being actively involved in sarcoidosis clinics at their institutions. Overall agreement was low to moderate. Agreement was reached on the role of history, physical examination, and 12-lead ECG in screening, echocardiogram, Holter monitor, myocardial fluorodeoxyglucose PET scan, and cardiac MRI in workup, and steroids in treatment. Agreement was not reached on the role of signal-averaged ECG in screening, optimum dose of prednisone, use of steroid-sparing agents, and duration of treatment. Several comments underscore the diverse approaches and uncertainty that exist in managing cardiac sarcoidosis. Our study highlights the dilemma that sarcoid experts face in their approach to cardiac sarcoidosis. It also highlights the lack of agreement among sarcoid experts on key aspects of diagnosis and management and stresses the importance of collaborative efforts to investigate the best strategies for screening for and management of cardiac sarcoidosis.

  18. [Budd-Chiari syndrome: a rare complication of hepatic sarcoidosis (about one case)].

    PubMed

    Sghier, Ismael Ait; Billah, Nabil Moatassim

    2016-01-01

    Sarcoidotic involvement of the liver is frequent, albeit uncommonly symptomatic. Anicteric cholestasis and portal hypertension are the main complications. Budd-Chiari syndrome is a little known and exceptional complication of hepatic sarcoidosis. We present a case of A 45-year-old woman suffering from hepatic sarcoidosis who developed Budd-Chiari syndrome.

  19. Sarcoidosis Following Anti-PD-1 and Anti-CTLA-4 Therapy for Metastatic Melanoma.

    PubMed

    Reddy, Swathi B; Possick, Jennifer D; Kluger, Harriet M; Galan, Anjela; Han, Dale

    2017-10-01

    Immune checkpoint inhibitors represent the newest treatment for stage IV melanoma. These agents are generally well tolerated, however severe immune-related adverse effects have been noted in a small, but clinically significant percentage of patients. Specifically, sarcoidosis is a known potential complication following anti-CTLA-4 therapy. We present 2 cases of pulmonary and cutaneous sarcoidosis developing in patients with stage IV melanoma. Both patients were treated with ipilimumab and anti-PD-1 therapy, and both experienced good oncologic responses to treatment; neither had evidence of preexisting sarcoidosis. Of note, both patients developed sarcoidosis only after undergoing immune checkpoint inhibitor therapy. In 1 patient, sarcoidosis developed after initiation of anti-PD-1 therapy, 3 months after the last dose of anti-CTLA-4 monotherapy, suggesting a synergistic immune dysmodulating effect of both checkpoint inhibitors. Ultimately, both patients' symptoms and radiologic findings resolved with corticosteroid treatment, and both patients have tolerated retreatment with PD-1 inhibitors. Sarcoidosis is a rare complication of immune checkpoint inhibitors and can manifest with severe pulmonary manifestations. However, sarcoidosis in this setting is responsive to corticosteroids and does not necessarily recur with retreatment. It is yet unclear whether the development of sarcoidosis in these patients represents unmasking of preexisting autoimmune tendencies or is a marker of oncologic response.

  20. Economic burden of sarcoidosis in a commercially-insured population in the United States.

    PubMed

    Rice, J Bradford; White, Alan; Lopez, Andrea; Conway, Alexandra; Wagh, Aneesha; Nelson, Winnie W; Philbin, Michael; Wan, George J

    2017-10-01

    Sarcoidosis is a multi-system inflammatory disorder characterized by the presence of non-caseating granulomas in involved organs. Patients with sarcoidosis have a reduced quality-of-life and are at an increased risk for several comorbidities. Little is known about the direct and indirect cost of sarcoidosis following the initial diagnosis. To provide an estimate of the healthcare resource utilization (HCRU) and costs borne by commercial payers for sarcoidosis patients in the US. Patients with a first diagnosis of sarcoidosis between January 1, 1998 and March 31, 2015 ("index date") were selected from a de-identified privately-insured administrative claims database. Sarcoidosis patients were required to have continuous health plan enrollment 12 months prior to and following their index dates. Propensity-score (1:1) matching of sarcoidosis patients with non-sarcoidosis controls was carried out based on a logistic regression of baseline characteristics. Burden of HCRU and work loss (disability days and medically-related absenteeism) were compared between the matched groups over the 12-month period following the index date ("outcome period"). A total of 7,119 sarcoidosis patients who met the selection criteria were matched with a control. Overall, commercial payers incurred $19,714 in mean total annual healthcare costs per sarcoidosis patient. The principle cost drivers were outpatient visits ($9,050 2015 USD, 46%) and inpatient admissions ($6,398, 32%). Relative to controls, sarcoidosis patients had $5,190 (36%) higher total healthcare costs ($19,714 vs $14,524; p < 0.001). Sarcoidosis patients also had significantly more work loss days (15.9 vs 11.3; p < 0.001) and work loss costs ($3,288 vs $2,527; p < 0.001) than matched controls. Sarcoidosis imposes an estimated total direct medical cost of $1.3-$8.7 billion to commercial payers, and an indirect cost of $0.2-$1.5 billion to commercial payers in work loss. Sarcoidosis imposes a significant economic

  1. The diagnosis of sarcoidosis pleurisy by medical thoracoscopy: report of three cases.

    PubMed

    Akçay, Sule; Pinelli, Valentina; Marchetti, Gian Pietro; Tassi, Gian Franco

    2008-01-01

    Pleural effusion in sarcoidosis is infrequent. The data on thoracoscopic observations of sarcoidosis pleurisy are limited. The present study describes three cases of sarcoid-related pleurisy diagnosed by medical thoracoscopy and discusses the thoracoscopic features of sarcoid pleuritis. The appearance of pleural nodules was completely different in the three cases, and the distribution of nodules of sarcoidosis was heterogenously located in the pleural surfaces. Pleural disease in sarcoidosis could be proved easily by sampling visible nodules, and pleural fluid could be aspirated without complications during thoracoscopy. Due to symptomatic pleurisy of sarcoidosis, therapy was given as systemic corticosteroid. In conclusion, thoracoscopy may be an appropriate alternative technique to obtain an accurate diagnosis in sarcoid pleurisy.

  2. Sudden cardiac death and sarcoidosis of the heart in a young patient.

    PubMed

    Jotterand, Morgane; Grabherr, Silke; Lobrinus, Johannes Alexandre; Michaud, Katarzyna

    Sarcoidosis is a granulomatous disease of unknown etiology affecting any organ, microscopically characterized by noncaseating granulomata. Cardiac involvement in sarcoidosis has been reported. It might be symptomatic or not and even revealed by sudden death. Heart conduction system is rarely investigated at autopsy, even in cases of sudden cardiac death. We present a case of a 32-year-old woman who died suddenly. The examination of the heart conduction system revealed a cardiac sarcoidosis that could explain the sudden death. The review of clinical data of the patient revealed some symptoms consistent/in agreement with this hypothesis. Cardiac sarcoidosis remains a diagnostic challenge and can be easily missed, clinically and pathologically. The retrospective analysis of clinical data and autopsy results of fatal and unusual cases might help to better understand sarcoidosis and its clinical presentations. Examination of the cardiac conduction system is crucial in selected cases of sudden cardiac death. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Presumed ocular histoplasmosis.

    PubMed

    Thuruthumaly, Catherine; Yee, David Chin; Rao, Prabakar Kumar

    2014-11-01

    To update the knowledge on the risk factors and treatment options for choroidal neovascular membrane due to ocular histoplasmosis and to provide a treatment algorithm. Smoking has been shown to be a strong risk factor in the development of choroidal neovascularization. Alleles that have been identified as a risk factor for the development of choroidal neovascularization in age-related macular degeneration have not shown to be associated with Presumed Ocular Histoplasmosis Syndrome-related choroidal neovascularization. Treatment has largely moved away from submacular surgery and macular photocoagulation to antivascular endothelial growth factor therapy. This review highlights the devastating vision loss that may occur in ocular histoplasmosis from the development of an atrophic scar at the fovea or following choroidal neovascularization. Many therapies have been tried with varied amounts of success. Antivascular endothelial growth factor therapy appears to be the gold-standard treatment with the possibility of combined photodynamic therapy in refractory cases.

  4. Increased disease activity in a patient with sarcoidosis after high dose interleukin 2 treatment for metastatic renal cancer.

    PubMed

    Logan, T F; Bensadoun, E S

    2005-07-01

    Sarcoidosis is a disease of unknown aetiology in which cytokines such as interleukin 2 (IL-2) are thought to play an important role. We present the case history of a 48 year old man with sarcoidosis who received treatment with high dose IL-2 for metastatic renal cell cancer, following which he developed hypercalcaemia characterised by a raised level of 1,25-dihydroxyvitamin D (1,25-(OH)2-D3), a finding consistent with sarcoidosis associated hypercalcaemia. The increased activity in his sarcoidosis following IL-2 treatment provides direct supportive evidence for the role of IL-2 in the pathogenesis of sarcoidosis.

  5. What Is an Ocular Migraine?

    MedlinePlus

    ... When to seek help What is an ocular migraine? Is it a sign of something serious? Answers ... the other which might have more–serious complications. Migraine aura involving your vision In some cases, ocular ...

  6. Ocular complications of boxing.

    PubMed

    Bianco, M; Vaiano, A S; Colella, F; Coccimiglio, F; Moscetti, M; Palmieri, V; Focosi, F; Zeppilli, P; Vinger, P F

    2005-02-01

    To investigate the prevalence of ocular injuries in a large population of boxers over a period of 16 years, in particular, the most severe lesions that may be vision threatening. Clinical records of the medical archive of the Italian Boxing Federation were analysed. A total of 1032 boxers were examined from February 1982 to October 1998. A complete ophthalmological history was available for 956, who formed the study population (a total of 10 697 examinations). The following data were collected: age when started boxing; duration of competitive boxing career (from the date of the first bout); weight category; a thorough ocular history. The following investigations were carried out: measurement of visual acuity and visual fields, anterior segment inspection, applanation tonometry, gonioscopy, and examination of ocular fundus. Eighty age matched healthy subjects, who had never boxed, formed the control group. Of the 956 boxers examined, 428 were amateur (44.8%) and 528 professional (55.2%). The median age at first examination was 23.1 (4.3) years (range 15-36). The prevalence of conjunctival, corneal, lenticular, vitreal, ocular papilla, and retinal alterations in the study population was 40.9% compared with 3.1% in the control group (p< or =0.0001). The prevalence of serious ocular findings (angle, lens, macula, and peripheral retina alterations) was 5.6% in boxers and 3.1% in controls (NS). Boxing does not result in a higher prevalence of severe ocular lesions than in the general population. However, the prevalence of milder lesions (in particular with regard to the conjunctiva and cornea) is noteworthy, justifying the need for adequate ophthalmological surveillance.

  7. Ocular complications of boxing

    PubMed Central

    Bianco, M; Vaiano, A; Colella, F; Coccimiglio, F; Moscetti, M; Palmieri, V; Focosi, F; Zeppilli, P; Vinger, P

    2005-01-01

    Objectives: To investigate the prevalence of ocular injuries in a large population of boxers over a period of 16 years, in particular, the most severe lesions that may be vision threatening. Methods: Clinical records of the medical archive of the Italian Boxing Federation were analysed. A total of 1032 boxers were examined from February 1982 to October 1998. A complete ophthalmological history was available for 956, who formed the study population (a total of 10 697 examinations). The following data were collected: age when started boxing; duration of competitive boxing career (from the date of the first bout); weight category; a thorough ocular history. The following investigations were carried out: measurement of visual acuity and visual fields, anterior segment inspection, applanation tonometry, gonioscopy, and examination of ocular fundus. Eighty age matched healthy subjects, who had never boxed, formed the control group. Results: Of the 956 boxers examined, 428 were amateur (44.8%) and 528 professional (55.2%). The median age at first examination was 23.1 (4.3) years (range 15–36). The prevalence of conjunctival, corneal, lenticular, vitreal, ocular papilla, and retinal alterations in the study population was 40.9% compared with 3.1% in the control group (p⩽0.0001). The prevalence of serious ocular findings (angle, lens, macula, and peripheral retina alterations) was 5.6% in boxers and 3.1% in controls (NS). Conclusions: Boxing does not result in a higher prevalence of severe ocular lesions than in the general population. However, the prevalence of milder lesions (in particular with regard to the conjunctiva and cornea) is noteworthy, justifying the need for adequate ophthalmological surveillance. PMID:15665199

  8. Diagnostic Utility of Angiotensin-Converting Enzyme in Sarcoidosis: A Population-Based Study.

    PubMed

    Ungprasert, Patompong; Carmona, Eva M; Crowson, Cynthia S; Matteson, Eric L

    2016-02-01

    Sarcoidosis is a disease with heterogenous clinical presentations. Diagnosis of sarcoidosis is often challenging with the lack of gold standard tests. In this study, we investigated the diagnostic utility of angiotensin-converting enzyme (ACE) for diagnosis of sarcoidosis. A cohort of Olmsted County, Minnesota residents who were diagnosed with sarcoidosis between January 1, 1984 and December 31, 2013 was identified based on individual medical record review. ACE levels recorded in the medical records of all subjects at the time of diagnosis were extracted. Comparator subjects were residents of Olmsted County, Minnesota who had ACE levels tested the same time period but did not have a diagnosis of sarcoidosis. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and the c-statistic of high versus low/normal ACE to diagnose sarcoidosis were calculated. A total of 3277 Olmsted County residents age ≥18 years had at least one ACE test in 1984-2013. The sarcoidosis incidence cohort contained 295 Olmsted County residents diagnosed with sarcoidosis in 1984-2013. Of these, ACE tests were obtained in 251. The sensitivity and specificity of high ACE for diagnosis of sarcoidosis were 41.4 % (95 % CI 35.3-47.8 %) and 89.9 % (95 % CI 88.8-91.0 %), respectively. The PPV and NPV in this population were 25.4 % (95 % CI 21.3-29.9 %) and 94.9 % (95 % CI 85.0-87.4 %). This study demonstrated a poor sensitivity and insufficient specificity of high ACE for diagnosis of sarcoidosis suggesting a limited role of ACE in clinical practice.

  9. Upper Airway Obstruction Requiring Emergent Tracheostomy Secondary to Laryngeal Sarcoidosis: A Case Report

    PubMed Central

    Ryu, Changwan; Herzog, Erica L.; Pan, Hongyi; Homer, Robert; Gulati, Mridu

    2017-01-01

    Patient: Female, 35 Final Diagnosis: Laryngeal sarcoidosis Symptoms: Hoarseness • stridor Medication: — Clinical Procedure: Tracheostomy Specialty: Otolaryngology Objective: Rare disease Background: Laryngeal sarcoidosis is a rare extrapulmonary manifestation of sarcoidosis, accounting for 0.33–2.1% of cases. A life-threatening complication of laryngeal sarcoidosis is upper airway obstruction. In this report we describe our experience in the acute and chronic care of a patient who required an emergent tracheostomy, with the aim to provide further insight into this difficult to manage disease. Case Report: A 37-year-old African American female with a 10-year history of stage 1 sarcoidosis presented with severe dyspnea. Laryngeal sarcoidosis was diagnosed three years previously, and she remained stable on low-dose prednisone until six months prior to admission, at which time she self-discontinued her prednisone for the homeopathic treatment Nopalea cactus juice. Her physical examination was concerning for impending respiratory failure as she presented with inspiratory stridor and hoarseness. Laryngoscopy showed a retroflexed epiglottis obstructing the glottis with edematous arytenoids and aryepiglottic folds. Otolaryngology performed an emergent tracheostomy to secure her airway and obtained epiglottic biopsies, which were consistent with sarcoidosis. She was eventually discharged home on prednisone 60 mg daily. Following months of corticosteroids, a laryngoscopy showed the epiglottis continuing to obstruct the glottis. The addition of methotrexate to a tapered dosage of prednisone 10 mg daily was unsuccessful, and she remains on prednisone 20 mg daily for disease control. Conclusions: Laryngeal sarcoidosis, a rare extrapulmonary manifestation of sarcoidosis, uncommonly presents as the life-threatening complication of complete upper airway obstruction. As such, laryngeal sarcoidosis is associated with significant morbidity and mortality, requiring a high index

  10. Blockade of the Programmed Death-1 Pathway Restores Sarcoidosis CD4+ T-Cell Proliferative Capacity

    PubMed Central

    Braun, Nicole A.; Celada, Lindsay J.; Herazo-Maya, Jose D.; Abraham, Susamma; Shaginurova, Guzel; Sevin, Carla M.; Grutters, Jan; Culver, Daniel A.; Dworski, Ryszard; Sheller, James; Massion, Pierre P.; Polosukhin, Vasiliy V.; Johnson, Joyce E.; Kaminski, Naftali; Wilkes, David S.; Oswald-Richter, Kyra A.

    2014-01-01

    Rationale: Effective therapeutic interventions for chronic, idiopathic lung diseases remain elusive. Normalized T-cell function is an important contributor to spontaneous resolution of pulmonary sarcoidosis. Up-regulation of inhibitor receptors, such as programmed death-1 (PD-1) and its ligand, PD-L1, are important inhibitors of T-cell function. Objectives: To determine the effects of PD-1 pathway blockade on sarcoidosis CD4+ T-cell proliferative capacity. Methods: Gene expression profiles of sarcoidosis and healthy control peripheral blood mononuclear cells were analyzed at baseline and follow-up. Flow cytometry was used to measure ex vivo expression of PD-1 and PD-L1 on systemic and bronchoalveolar lavage–derived cells of subjects with sarcoidosis and control subjects, as well as the effects of PD-1 pathway blockade on cellular proliferation after T-cell receptor stimulation. Immunohistochemistry analysis for PD-1/PD-L1 expression was conducted on sarcoidosis, malignant, and healthy control lung specimens. Measurements and Main Results: Microarray analysis demonstrates longitudinal increase in PDCD1 gene expression in sarcoidosis peripheral blood mononuclear cells. Immunohistochemistry analysis revealed increased PD-L1 expression within sarcoidosis granulomas and lung malignancy, but this was absent in healthy lungs. Increased numbers of sarcoidosis PD-1+ CD4+ T cells are present systemically, compared with healthy control subjects (P < 0.0001). Lymphocytes with reduced proliferative capacity exhibited increased proliferation with PD-1 pathway blockade. Longitudinal analysis of subjects with sarcoidosis revealed reduced PD-1+ CD4+ T cells with spontaneous clinical resolution but not with disease progression. Conclusions: Analogous to the effects in other chronic lung diseases, these findings demonstrate that the PD-1 pathway is an important contributor to sarcoidosis CD4+ T-cell proliferative capacity and clinical outcome. Blockade of the PD-1 pathway may be a

  11. Immunogenetics of ocular inflammatory disease.

    PubMed

    Levinson, R D

    2007-02-01

    Ocular inflammatory disease comprises of a diverse group of clinical entities that may result from autoimmune processes, infections, or both. While many individual ocular inflammatory diseases are quite rare, ocular inflammation is one of the more common causes of visual disability, including blindness, in the developed world. Better understanding of ocular inflammatory disease is an important step in designing more sophisticated therapies that may help prevent loss of visual function for these patients.

  12. Ocular involvement of brucellosis.

    PubMed

    Bazzazi, Nooshin; Yavarikia, Alireza; Keramat, Fariba

    2013-01-01

    A 29-year-old male diagnosed with brucellosis a week earlier was referred to the ophthalmology clinic with visual complaints. On examination, visual acuity was 20/25, he had conjunctival injection on slit lamp examination. There was also bilateral optic disk swelling plus retinal hyperemia (optic disc hyperemia and vascular tortuosity) and intraretinal hemorrhage on funduscopy. The patient was admitted and treated with cotrimoxazole, rifampin, doxycycline and prednisolone for 2 months. Ocular manifestations subsided gradually within 6 months after treatment. Brucellosis can affect the eye and lead to serious ocular complications. Early diagnosis and prompt treatment should be considered in endemic areas.

  13. Correction of ocular dystopia.

    PubMed

    Janecka, I P

    1996-04-01

    The purpose of this study was to examine results with elective surgical correction of enophthalmos. The study was a retrospective assessment in a university-based referral practice. A consecutive sample of 10 patients who developed ocular dystopia following orbital trauma was examined. The main outcome measures were a subjective evaluation by patients and objective measurements of patients' eye position. The intervention was three-dimensional orbital reconstruction with titanium plates. It is concluded that satisfactory correction of enophthalmos and ocular dystopia can be achieved with elective surgery using titanium plates. In addition, intraoperative measurements of eye position in three planes increases the precision of surgery.

  14. New classification of ocular foreign bodies.

    PubMed

    Shukla, Bhartendu

    2016-12-01

    Foreign bodies (FBs) in the eye are usually classified as intraocular (IOFB) or extraocular (EOFB). In IOFB the FB is within the eye ball and in EOFB it is outside. This classification seems oversimplified. Hence a new classification is proposed on the basis of FB locations, in which adnexal FBs (in orbit, lids, con- junctiva and lacrimal apparatus) are also included. These are further classified according to their exact location. FBs can also be classified in many other ways. Besides IOFB and EOFB, another condition IMFB (intramural foreign body) is also described. The FBs are situated within cornea or sclera and are neither IOFB nor EOFB. Ocular trauma also includes trauma to ocular adnexa and hence the terms IOFB and EOFB have been replaced by IGFB (intraglobal foreign body) and EGFB (extraglobal foreign body).

  15. Sarcoidosis with Takayasu arteritis: a model of overlapping granulomatosis. A report of seven cases and literature review.

    PubMed

    Chapelon-Abric, Catherine; Saadoun, David; Marie, Isabelle; Comarmond, Cloé; Desbois, Anne Claire; Domont, Fanny; Savey, Léa; Cacoub, Patrice

    2017-08-08

    To describe the features of exceptional coexisting Takayasu arteritis (TA) and sarcoidosis, two conditions of unknown cause associated with a common immunologic pattern. We report seven cases of concomitant sarcoidosis-Takayasu or Takayasu-like vasculitis, observed in two referral centers between 1995 and 2015. All patients were female. The mean age at sarcoidosis diagnosis and TA diagnosis was 36 and 37 years, respectively. Sarcoidosis occurred in 86% of cases before or together with TA. Sarcoidosis always had a classic expression except for one renal localization. Sarcoidosis was not severe and mostly non-treated (86%). In all cases of TA, supra-aortic arteries were involved; in only two TA cases a more diffuse inflammatory arterial involvement was noted. In one case, Takayasu arteritis occurred despite immunosuppressive therapy given for sarcoidosis. All patients received for TA a treatment with corticosteroids associated with methotrexate (four cases), infliximab (one case) or tocilizumab (one case). After a mean follow-up of 89 months, TA always improved and no death was observed. TA stands as pathology associated with sarcoidosis. TA occurred in three cases among 50. When sarcoidosis preceded TA, a recovery of sarcoidosis was achieved mostly without treatment. TA is a prognostic and therapeutic factor. Immunosuppressive treatment, including steroids, led to a good prognosis for TA as well as for sarcoidosis. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  16. Ocular rosacea: a dermatologic perspective.

    PubMed

    Webster, Guy; Schaller, Martin

    2013-12-01

    As many as 50% of patients given the diagnosis of cutaneous rosacea also have ocular rosacea. Conservative figures indicate that approximately 10 million patients are affected by ocular rosacea in the United States alone. Despite this prevalence, ocular symptoms of rosacea are often improperly diagnosed, particularly when they occur in the absence of skin involvement.

  17. Sarcoidosis vs. Sarcoid-like reactions: The Two Sides of the same Coin?

    PubMed

    Tchernev, Georgi; Tana, Claudio; Schiavone, Cosima; Cardoso, José-Carlos; Ananiev, Julian; Wollina, Uwe

    2014-07-01

    Differentiating between sarcoidosis as an autonomous disease and sarcoid-like reactions requires considerable efforts. The epithelioid cell granuloma is not equivalent to sarcoidosis because it may be identified in a number of infectious and noninfectious disorders, including neoplastic diseases. At the current state of knowledge, accurate distinction between different causes of epithelioid cell granulomas is in many cases not possible. Despite being characteristic of sarcoidosis and sarcoid-like reactions, the epithelioid cell granuloma is not their synonym, as numerous other causes can give rise to such a type of granulomatous infiltrate. Its etiology should be sought through careful additional investigations, including the genetic signature of both conditions.Sarcoid-like reactions may be grouped generally into several subtypes. The differentiation between each one of them requires a certain combination of diagnostic tests. The major objective of these tests is to exclude or to prove the presence of an infectious, tumoral, or immunogenic antigen on the one hand, and to characterize the genetic profile of the affected patients (for example, sarcoidosis-specific genes) on the other. Only thus may one accurately differentiate between the two pathologic conditions described earlier in the abstract.The clear differentiation between sarcoidosis as a separate disease and sarcoid-like pathologies leads to the more precise clarification of the final diagnosis, which may in turn allow for a more appropriate therapy and improvement in the quality of life of the patients. Equating sarcoid granulomas with sarcoidosis can lead to serious consequences in a number of patients. Sadly enough, after scrutinizing the current available data in the world literature, one cannot find criteria to allow such distinction in a high percentage of the investigated cases.This critical review provides a completely new pathogenetic and diagnostic algorithm, helping in the differentiation

  18. Ocular Screening System

    NASA Technical Reports Server (NTRS)

    1985-01-01

    The NASA imaging processing technology, an advanced computer technique to enhance images sent to Earth in digital form by distant spacecraft, helped develop a new vision screening process. The Ocular Vision Screening system, an important step in preventing vision impairment, is a portable device designed especially to detect eye problems in children through the analysis of retinal reflexes.

  19. Ocular Screening System

    NASA Technical Reports Server (NTRS)

    1985-01-01

    An ocular screening system designed for safe, convenient screening of large groups was developed at Marshall Space Flight Center, leading to the formation of Medical Sciences Corporation. The system identifies visual defects accurately and inexpensively, and includes a photorefractor telephoto lens and an electronic flash. Medical Sciences Corporation is using the device to test at schools, industrial plants, etc.

  20. Instrument Measures Ocular Counterrolling

    NASA Technical Reports Server (NTRS)

    Levitan, Barry M.; Reschke, Millard F.; Spector, Lawrence N.

    1991-01-01

    Compact, battery-powered, noninvasive unit replaces several pieces of equipment and operator. Instrument that looks like pair of goggles with small extension box measures ocular counterrotation. Called "otolith tilt-translation reinterpretation" (OTTR) goggles, used in studies of space motion sickness. Also adapted to use on Earth and determine extent of impairment in patients who have impaired otolith functions.

  1. Robotic ocular surgery.

    PubMed

    Tsirbas, A; Mango, C; Dutson, E

    2007-01-01

    Bimanual, three-dimensional robotic surgery has proved valuable for a variety of surgical procedures. To examine the use of a commercially available surgical robot for ocular microsurgery. Using a da Vinci surgical robot, ocular microsurgery was performed with repair of a corneal laceration in a porcine model. The experiments were performed on harvested porcine eyes placed in an anatomical position using a foam head on a standard operating room table. A video scope and two, 360 degrees -rotating, 8-mm, wrested-end effector instruments were placed over the eye with three robotic arms. The surgeon performed the actual procedures while positioned at a robotic system console that was located across the operating room suite. Each surgeon placed three 10-0 sutures, and this was documented with still and video photography. Ocular microsurgery was successfully performed using the da Vinci surgical robot. The robotic system provided excellent visualisation, as well as controlled and delicate placement of the sutures at the corneal level. Robotic ocular microsurgery is technically feasible in the porcine model and warrants consideration for evaluation in controlled human trials to deploy functioning remote surgical centres in areas without access to state-of-the-art surgical skill and technology.

  2. Pattern of ocular trauma.

    PubMed

    Hossain, M M; Mohiuddin, A A; Akhanda, A H; Hossain, M I; Islam, M F; Akonjee, A R; Ali, M

    2011-07-01

    This prospective observational study was conducted in the department of Ophthalmology Mymensingh Medical College Hospital during the period of November, 2009 to October, 2010. Two hundred & fifty (250) patients of both sexes and all ages with ocular trauma were selected randomly for this study. A detailed history of patients, duration of trauma, relation of trauma with work, visual status prior to injury, any surgery prior to injury & patients were alcoholic or not were taken. Male patients were 190(76%) and female patients were 60(24%). Majority of patients were 11-20 years group (39.2%). Most of patients (40%) attended into hospital within 60 hours of ocular trauma. Accidental occupational trauma were more common (51.2%) and assault injury were less common (12.8%). Greater number of ocular trauma was caused by sharp objects (59.2%) and less number of ocular trauma was caused by chemical injuries (2.4%). Open globe injuries were more common (62%) than closed globe injury (38%). Visual acuity on admission between 6/60 to PL comprises highest number (64%) and also on discharge between 6/60 to PL comprises highest number of cases (50%). Most of the patients came from poor socioeconomic group (60%).

  3. [Ocular syphilis: 9 cases].

    PubMed

    Gauthier, A S; Tea, S; Hustache Mathieu, L; Schwartz, C; Saleh, M; Delbosc, B

    2016-11-01

    Syphilis is a disease with ocular involvement may be opening and cover all ocular structures. The objective of the study was to retrospectively identify and describe all cases of ocular syphilis diagnosed in the ophthalmology department of Besançon University Hospital from March 2004 to April 2014. Between March 2004 and April 2014, we diagnosed and monitored 9 male patients (13 eyes) with ocular syphilis (1 interstitial keratitis, chorioretinitis associated with a 1 hyalite, 3 panuvéites, 1 chorioretinitis after placoid, 3 bilateral papillary edema). Patients had a mean age of 52.6 years (32-77 years) at diagnosis and the inaugural symptoms were in 100% of cases decreased visual acuity. The follow-up period ranged from 1 month to 5 years. The curative treatment included intravenous antibiotics penicillin G for 3 weeks. All patients had an improvement in their AV (AV initial range "counting fingers" and 0.6 versus AV final between 0.5 and 1 in decimal scale). Syphilis is a disease currently on the rise. It must be systematically sought before any inflammatory disease of the eye or papilledema. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  4. Robotic ocular surgery

    PubMed Central

    Tsirbas, A; Mango, C; Dutson, E

    2007-01-01

    Background Bimanual, three‐dimensional robotic surgery has proved valuable for a variety of surgical procedures. Aims To examine the use of a commercially available surgical robot for ocular microsurgery. Methods Using a da Vinci surgical robot, ocular microsurgery was performed with repair of a corneal laceration in a porcine model. The experiments were performed on harvested porcine eyes placed in an anatomical position using a foam head on a standard operating room table. A video scope and two, 360°‐rotating, 8‐mm, wrested‐end effector instruments were placed over the eye with three robotic arms. The surgeon performed the actual procedures while positioned at a robotic system console that was located across the operating room suite. Each surgeon placed three 10‐0 sutures, and this was documented with still and video photography. Results Ocular microsurgery was successfully performed using the da Vinci surgical robot. The robotic system provided excellent visualisation, as well as controlled and delicate placement of the sutures at the corneal level. Conclusions Robotic ocular microsurgery is technically feasible in the porcine model and warrants consideration for evaluation in controlled human trials to deploy functioning remote surgical centres in areas without access to state‐of‐the‐art surgical skill and technology. PMID:17020903

  5. Anti-TNF-α therapy in refractory uveitis associated with sarcoidosis: Multicenter study of 17 patients.

    PubMed

    Riancho-Zarrabeitia, Leyre; Calvo-Río, Vanesa; Blanco, Ricardo; Mesquida, Marina; Adan, Alfredo M; Herreras, José M; Aparicio, Ángel; Peiteado-Lopez, Diana; Cordero-Coma, Miguel; García Serrano, José Luis; Ortego-Centeno, Norberto; Maíz, Olga; Blanco, Ana; Sánchez-Bursón, Juan; González-Suárez, Senén; Fonollosa, Alejandro; Santos-Gómez, Montserrat; González-Vela, Carmen; Loricera, Javier; Pina, Trinitario; González-Gay, Miguel A

    2015-12-01

    To assess anti-TNF-α therapy response in uveitis associated with sarcoidosis refractory to conventional immunosuppressive therapy. Open-label, multicenter, retrospective study on patients with sarcoid uveitis who underwent anti-TNF-α therapy because of inadequate response to conventional therapy including corticosteroids and at least 1 systemic synthetic immunosuppressive drug. The main outcome measurements were degree of anterior and posterior chamber inflammation, visual acuity, macular thickness, and immunosuppression load. A total of 17 patients (8 men; 29 affected eyes; mean ± standard deviation age 38.4 ± 16.8; range: 13-76 years) were studied. The patients had bilateral hilar lymphadenopathy (58.8%), lung parenchyma involvement (47.1%), peripheral lymph nodes (41.2%), and involvement of other organs (52.9%). Angiotensin-converting enzyme was elevated in 58.8%. The most frequent ocular pattern was bilateral chronic relapsing panuveitis. The first biologic agent used was adalimumab in 10 (58.8%) and infliximab in 7 (41.2%) cases. Infliximab 5mg/kg intravenously every 4-8 weeks and adalimumab 40mg subcutaneously every 2 weeks were the most common administration patterns. In most cases anti-TNF-α therapy was given in combination with immunosuppressive drugs. The mean duration of follow-up was 33.9 ± 17.1 months. Significant improvement was observed following anti-TNF-α therapy. Baseline results versus results at 2 years from the onset of biologic therapy were the following: the median of cells in the ocular anterior chamber (interquartile range-IQR) 0.5 (0-2) versus 0 (0-0) (p = 0.003), vitritis 0 (0-1.25) versus 0 (0-0) (p = 0.008), macular thickness (391.1 ± 58.8 versus 247 ± 40.5µm) (p = 0.028), and visual acuity 0.60 ± 0.33 versus 0.74 ± 0.27; p = 0.009. The median daily (interquartile range) dose of prednisone was also reduced from 10 (0-30)mg at the onset of the anti-TNF-α therapy to 0 (0-0)mg at 2 years (p = 0.02). Significant reduction was

  6. Vitamin D Supplementation: Not So Simple in Sarcoidosis.

    PubMed

    Sodhi, Amik; Aldrich, Thomas

    2016-09-01

    Americans are increasingly receiving vitamin D supplementation, often based on low-measured 25-hydroxy-vitamin D (25-OH-vit D). In sarcoidosis, there is often increased metabolism of 25-OH-vit D to 1,25-dihydroxy-vitamin D (1,25-OH-vit D), so 25-OH-vit D may remain low, despite high levels of 1,25-OH-vit D. In such cases, vitamin D supplementation may lead to hypercalcemia. We randomly selected 196 patients with sarcoidosis who received at least 1 prescription of vitamin D between 2005 and 2011 and 196 control patients. Primary outcome was the incidence of hypercalcemia during the 2 years following the vitamin D prescription. A secondary outcome was the proportion of patients who had received vitamin D prescriptions and who had adequate blood work performed before the prescription. The 25-OH-vit D and 1,25-OH-vit D levels were measured in only 70% and 23%, respectively, of those receiving supplementation. Hypercalcemia was noted more frequently in the group that received vitamin D (42.3%) as compared with the nonsupplemented group (18.3%), P < 0.0001. Patients who received a vitamin D prescription developed moderate and severe hypercalcemia more frequently (12.8%) as compared to the group that did not receive vitamin D (3.6%), P = 0.001. In multivariate analysis, having a prescription for vitamin D increased the risk of developing hypercalcemia to approximately 2-fold. The risk of developing hypercalcemia (odds ratio = 4.1) was increased with renal failure. Our study demonstrates that a substantial proportion of patients with sarcoidosis who receive vitamin D are not getting appropriate pretesting. This increases their risk for developing hypercalcemia. Copyright © 2016 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  7. Tadalafil therapy for sarcoidosis-associated pulmonary hypertension

    PubMed Central

    Baughman, R. P.; Aris, R.; Engel, P.; Donohue, J. F.

    2016-01-01

    Abstract Sarcoidosis-associated pulmonary hypertension (SAPH) is estimated to occur in at least 5% or more of sarcoidosis patients, and it contributes to significant morbidity and mortality. Optimal therapy for SAPH is not well established. We performed a 24-week open-label trial of tadalafil for SAPH at 2 academic medical centers. Subjects were required to have confirmed sarcoidosis plus a right heart catheterization within 12 months of enrollment showing a mean pulmonary artery pressure ≥ 25 mmHg, a pulmonary artery wedge pressure ≤ 15 mmHg, and a calculated pulmonary vascular resistance ≥ 3 Wood units. Subjects received 20 mg/day of tadalafil for the first 4 weeks and then 40 mg/day for the subsequent 20 weeks. Sixteen patients were screened, 12 of whom met criteria for enrollment. At 24 weeks, there was no overall improvement in 6-minute walk distance (6MWD). Five of the 12 subjects dropped out of the study early (2 for social reasons, 3 for medical reasons). There was no significant change in short form 36, St. George’s respiratory questionnaire, or maximum Borg dyspnea scores over the 24 weeks. There were no significant adverse events or laboratory abnormalities clearly related to tadalafil in the cohort. The study did not meet the primary end point of change in 6MWD because of the small sample size. Tadalafil was generally safely administered in this cohort of SAPH patients. There was a relatively high dropout rate but no major adverse events and no clinical worsening. Larger studies are needed to explore this question further. (Trial registration: ClinicalTrials.gov identifier: NCT01324999) PMID:28090299

  8. Mycobacterium tuberculosis Antigen 85A Induces Th-1 Immune Responses in Systemic Sarcoidosis

    PubMed Central

    Hajizadeh, Rana; Sato, Hiroe; Carlisle, James; Nadaf, Michele T.; Evans, Whitney; Shepherd, Bryan E.; Miller, Robert F.; Kalams, Spyros A.; Drake, Wonder Puryear

    2014-01-01

    Sarcoidosis is a granulomatous disease of unknown etiology, characterized by a Th-1 immunophenotype. Although humoral immune responses by sarcoidosis subjects to mycobacterial proteins have been detected, mycobacterial antigens capable of inducing cellular immune responses in sarcoidosis subjects have not been reported. We used the enzyme-linked immunospot assay to assess for recognition of the Mycobacterium tuberculosis mycolyl transferase, Antigen 85A, by peripheral blood mononuclear cells from 25 sarcoidosis subjects, 22 PPD− (purified protein derivative) healthy volunteers, and 16 PPD+ healthy subjects. Reactivity to Ag85A whole protein was observed in 15 of 25 sarcoidosis subjects compared to 2 of 22 PPD− subjects (p = 0.0006, Fisher’s exact test) and to 14 of 16 PPD+ subjects (p = 0.084, Fisher’s exact test). Monoclonal antibody against HLA-DR inhibited recognition. In addition to immune recognition of Ag85A whole protein, peptide-mapping studies identified four immunogenic Ag85A peptides, which induced Th-1 immune responses in individual sarcoidosis subjects, suggesting that multiple epitopes from a mycobacterial protein may have a role in sarcoidosis immunopathogenesis. PMID:17357846

  9. Physical activity and training in sarcoidosis: review and experience-based recommendations.

    PubMed

    Strookappe, Bert; Saketkoo, Lesley Ann; Elfferich, Marjon; Holland, Anne; De Vries, Jolanda; Knevel, Ton; Drent, Marjolein

    2016-10-01

    Sarcoidosis is a multisystemic inflammatory disorder with a great variety of symptoms, including fatigue, dyspnea, pain, reduced exercise tolerance and muscle strength. Physical training has the potential to improve exercise capacity and muscle strength, and reduce fatigue. The aim of this review and survey was to present information about the role of physical training in sarcoidosis and offer practical guidelines. A systematic literature review guided an international consensus effort among sarcoidosis experts to establish practice-basic recommendations for the implementation of exercise as treatment for patients with various manifestations of sarcoidosis. International sarcoidosis experts suggested considering physical training in symptomatic patients with sarcoidosis. Expert commentary: There is promising evidence of a positive effect of physical training. Recommendations were based on available data and expert consensus. However, the heterogeneity of these patients will require modification and program adjustment of the standard rehabilitation format for e.g. COPD or interstitial lung diseases. An optimal training program (types of exercise, intensities, frequency, duration) still needs to be defined to optimize training adjustments, especially reduction of fatigue. Further randomized controlled trials are needed to consolidate these findings and optimize the comprehensive care of sarcoidosis patients.

  10. Prognostic markers of sarcoidosis: an analysis of patients from everyday pneumological practice.

    PubMed

    Doubková, Martina; Pospíšil, Zdeněk; Skřičková, Jana; Doubek, Michael

    2015-10-01

    Although sarcoidosis is a well-known disease, sarcoidosis patient outcome and the role of prognostic markers in everyday practice have not yet been well described. Therefore, the goal of the present study was to analyse the fate of sarcoidosis patients and the factors influencing the disease's behaviour in routine practice. The study was a retrospective analysis of 306 consecutive patients (181 females and 125 males; median age 45 years) diagnosed with sarcoidosis in the years 2003-2012. Through the use of uniparametric analysis, it was determined that the stage of the disease, organ involvement, CD4/CD8 ratio in the bronchoalveolar lavage fluid (BALF), serum and urine calcium levels, smoking, and pulmonary functions had a significant influence on spontaneous sarcoidosis resolution. Multiparametric analysis revealed that spontaneous resolution was significantly positively influenced by female gender, a less advanced disease stage, a higher CD4/CD8 ratio in the BALF, lymphocytosis in the BALF and normal pulmonary functions at diagnosis. During follow-up, only three patients died. The causes of death were respiratory failure with pneumonia (n = 2) and heart attack caused by sarcoidosis heart involvement (n = 1). The data show sarcoidosis as a disorder with high variability in clinical and laboratory findings but with good general prognosis. © 2014 John Wiley & Sons Ltd.

  11. Coexistence of early onset sarcoidosis and partial interferon-γ receptor 1 deficiency.

    PubMed

    Çakan, Mustafa; Keskindemirci, Gonca; Aydoğmuş, Çiğdem; Akı, Hilal; Hatipoğlu, Nevin; Kıyak, Aysel; Aydoğan, Gönül; Aktay-Ayaz, Nuray

    2016-01-01

    Pediatric sarcoidosis comprises a spectrum of childhood granulomatous inflammatory conditions. Pathological hallmark of the disease is granuloma formation that is seen in the affected tissues and almost any organ or system can be involved. There are two forms of pediatric sarcoidosis. One is seen in older children and the clinical picture is very similar to that of adult sarcoidosis and the other one is seen in early childhood. Sarcoidosis in early childhood can be divided as Blau syndrome (familial form) and early onset sarcoidosis (sporadic form). In both of the diseases there is a defect in the NOD2/CARD15 gene. The typical triad of early onset sarcoidosis is polyarthritis, dermatitis and uveitis. Interferon-γ receptor 1 deficiency is caused by defects in the IFNγR1 gene and non-tuberculosis mycobacterial pathogens are the leading causes of infections that start in early childhood. Herein we report a patient who presented with the symptoms of early onset sarcoidosis and also had partial interferon-γ receptor 1 deficiency that presented with BCG-osis. In addition to anti-mycobacterial treatment, methotrexate and prednisolone were used in therapy.

  12. Dermoscopy could be useful in differentiating sarcoidosis from necrobiotic granulomas even after treatment with systemic steroids

    PubMed Central

    Ramadan, Shahira; Hossam, Dalia; Saleh, Marwah A.

    2016-01-01

    Background: Diagnosing cutaneous sarcoidosis and necrobiotic granulomas is challenging. Objective: Assessing the value of dermoscopy in differentiating cutaneous sarcoidosis from necrobiotic granulomas and evaluating whether their dermoscopic features will be altered after treatment. Methods: Nineteen cutaneous sarcoidosis and 11 necrobiotic granuloma patients (2 necrobiosis lipoidica, 4 granuloma annulare and 5 rheumatoid nodule) were included in this study. The diagnosis was confirmed by skin biopsy. The lesions were examined using non-contact polarized dermoscope (Dermlite 2 HR-Pro; 3Gen, San Juan Capistrano, CA). Results: Ten out of 19 cutaneous sarcoidosis patients and 7/11 necrobiotic cases group were receiving treatments (topical, intralesional or systemic steroids ± chloroquine) but still have cutaneous lesions. Treatment duration in the sarcoidosis group ranged from 2 months to 10 years (median 3 years) and in the necrobiotic cases group ranged from 3 months to 16 years (median 2 years). Pink homogenous background, translucent orange areas, white scar-like depigmentation and fine white scales were significantly associated with the cutaneous sarcoidosis compared to necrobiotic cases group. On the other hand mixed pink, white and yellowish background was significantly associated with the necrobiotic cases group. No significant difference in the dermoscopic findings was detected between treated and non-treated patients. Conclusion: Some dermoscopic findings are shared between the cutaneous sarcoidosis group and the necrobiotic cases group, yet dermoscopy could be a useful aid in differentiating them even after treatment. PMID:27648379

  13. Sarcoidosis in patients with mixed connective tissue disease: clinical, genetic, serological and histological observations.

    PubMed

    Szodoray, Peter; Szollosi, Zoltan; Gyimesi, Edit; Takacs, Istvan; Mekkel, Gabriella; Vegh, Judit; Szilagyi, Anna; Zeher, Margit; Szegedi, Gyula; Bodolay, Edit

    2008-06-01

    The objective of this study was to investigate how the development of sarcoidosis influences the disease course of mixed connective tissue disease (MCTD). The cellular composition of MCTD-associated sarcoidosis granulomas was evaluated and also the disease-accompanying T-cell activation and alterations of the serum cytokine levels were measured before and after the therapy. The HLA-DR specific alleles were also assessed. We present two cases with MCTD coexisting sarcoidosis. Serum concentrations of Th1 and Th2 cytokines were assessed by ELISA. Peripheral blood CD3+ total T-cell numbers, CD4+ and CD8+ T-cell subset were determined by flow cytometry. Furthermore, hematoxylin-eosin staining and immunhistochemistry were performed for histological assessment. HLA-DR specific alleles were determined by using PCR-SSP. Elevated number of activated T-cells and high Th1 cytokine levels were detected, mainly IFN-gamma and TNF-alpha. Histologically, CD4+ and CD8+ T-cells were present in the sarcoidosis infiltrations. The haplotypes were to some extent dissimilar from the HLA-DR genotype from patients with MCTD, or sarcoidosis alone. Sarcoidosis enhances the activation of MCTD, based on the laboratory and clinical findings. Our results show that the inflammation is mainly in the effector phase, while granuloma formation is characteristic of the resolution phase of the disease. The assessment of the cytokine network in sarcoidosis-associated MCTD enables us to select the most effective, individualized therapy protocol for these patients.

  14. Extensive intramuscular manifestation of sarcoidosis with initially missed diagnosis and delayed therapy: a case report.

    PubMed

    Meyer, Niklaus; Sutter, Reto; Schirp, Udo; Gutzeit, Andreas

    2017-08-24

    Sarcoidosis is a multisystemic granulomatous disorder, which in nearly all cases involves the lungs and other organs. Isolated forms of sarcoidosis within the muscles, but without lung involvement, are extremely rare and can lead to delayed or even false diagnosis. A 52-year-old white, Swiss man presented with painful arm cramps and a history of symptoms over the previous 3 years. In the initial clinical investigation, our patient also showed edema in both legs without any other complaints. After performing an magnetic resonance imaging scan of his extremities and a positron emission tomography/computed tomography scan, diffuse myositis was described. The subsequent muscle biopsy provided the surprising diagnosis of muscle sarcoidosis, without involvement of the lungs or any other organ. After starting therapy with glucocorticoids, his symptoms improved immediately. Sarcoidosis is a common disorder, which in most cases affects the lungs. In this case report an isolated sarcoidosis is described without lung involvement, but with involvement of the muscles of the extremities and the trunk. Reported cases of sarcoidosis only involving skeletal muscle and without lung involvement are extremely rare. Radiologists should consider this presentation of sarcoidosis to avoid delayed diagnosis and therapy.

  15. Gene expression profiles in granuloma tissue reveal novel diagnostic markers in sarcoidosis.

    PubMed

    Christophi, George P; Caza, Tiffany; Curtiss, Christopher; Gumber, Divya; Massa, Paul T; Landas, Steve K

    2014-06-01

    Sarcoidosis is an immune-mediated multisystem disease characterized by the formation of non-caseating granulomas. The pathogenesis of sarcoidosis is unclear, with proposed infectious or environmental antigens triggering an aberrant immune response in susceptible hosts. Multiple pro-inflammatory signaling pathways have been implicated in mediating macrophage activation and granuloma formation in sarcoidosis, including IFN-γ/STAT-1, IL-6/STAT-3, and NF-κB. It is difficult to distinguish sarcoidosis from other granulomatous diseases or assess disease severity and treatment response with histopathology alone. Therefore, development of improved diagnostic tools is imperative. Herein, we describe an efficient and reliable technique to classify granulomatous disease through selected gene expression and identify novel genes and cytokine pathways contributing to the pathogenesis of sarcoidosis. We quantified the expression of twenty selected mRNAs extracted from formalin-fixed paraffin embedded (FFPE) tissue (n = 38) of normal lung, suture granulomas, sarcoid granulomas, and fungal granulomas. Utilizing quantitative real-time RT-PCR we analyzed the expression of several genes, including IL-6, COX-2, MCP-1, IFN-γ, T-bet, IRF-1, Nox2, IL-33, and eotaxin-1 and revealed differential regulation between suture, sarcoidosis, and fungal granulomas. This is the first study demonstrating that quantification of target gene expression in FFPE tissue biopsies is a potentially effective diagnostic and research tool in sarcoidosis.

  16. Cryptococcosis in sarcoidosis: cryptOsarc, a comparative study of 18 cases.

    PubMed

    Bernard, C; Maucort-Boulch, D; Varron, L; Charlier, C; Sitbon, K; Freymond, N; Bouhour, D; Hot, A; Masquelet, A C; Valeyre, D; Costedoat-Chalumeau, N; Etienne, M; Gueit, I; Jouneau, S; Delaval, P; Mouthon, L; Pouget, J; Serratrice, J; Brion, J-P; Vaylet, F; Bremont, C; Chennebault, J M; Jaffuel, S; Broussolle, C; Lortholary, O; Sève, P

    2013-06-01

    To describe the main characteristics and the treatment of cryptococcosis in patients with sarcoidosis. Multicenter study including all patients notified at the French National Reference Center for Invasive Mycoses and Antifungals. Retrospective chart review. Each case was compared with two controls without opportunistic infections. Eighteen cases of cryptococcosis complicating sarcoidosis were analyzed (13 men and 5 women). With 2749 cases of cryptococcosis registered in France during the inclusion period of this study, sarcoidosis accounted for 0.6% of all the cryptococcosis patients and for 2.9% of the cryptococcosis HIV-seronegative patients. Cryptococcosis and sarcoidosis were diagnosed concomitantly in four cases; while sarcoidosis was previously known in 14/18 patients, including 12 patients (67%) treated with steroids. The median rate of CD4 T cells was 145 per mm(3) (range: 55-1300) and not related to steroid treatment. Thirteen patients had cryptococcal meningitis (72%), three osteoarticular (17%) and four disseminated infections (22%). Sixteen patients (89%) presented a complete response to antifungal therapy. After a mean follow-up of 6 years, no death was attributable to cryptococcosis. Extra-thoracic sarcoidosis and steroids were independent risk factors of cryptococcosis in a logistic regression model adjusted with the sex of the patients. Cryptococcosis is a significant opportunistic infection during extra-thoracic sarcoidosis, which occurs in one-third of the cases in patients without any treatment; it is not associated to severe CD4 lymphocytopenia and has a good prognosis.

  17. WHOQOL-bREF questionnaire as a measure of quality of life in sarcoidosis.

    PubMed

    Alilović, Marija; Peros-Golubicić, Tatjana; Radosević-Vidacek, Biserka; Koscec, Adrijana; Tekavec-Trkanjec, Jasna; Solak, Mirsala; Hećimović, Ana; Smojver-Jezek, Silvana

    2013-09-01

    The aim of this study was to evaluate the usefulness of a short form of the WHOQOL questionnaire, the WHOQOL-BREF, which consists of 24 questions, in evaluating quality of life (QOL) in sarcoidosis patients. A group of 97 sarcoidosis patients and a matched group of 97 healthy controls took part in the study. Their QOL was examined by means of the WHOQOL-BREF, and the respiratory functions were measured in sarcoidosis patients. The WHOQOL-BREF revealed significantly poorer QOL of sarcoidosis patients in the domains of Physical and Psychological Health in comparison to healthy controls. In contrast, sarcoidosis patients perceived their QOL significantly better than healthy controls in the domains of Social Relations and Environment. Differences between sarcoidosis patients and healthy controls were found in several items from the WHOQOL-BREF and some of them were modified by gender. However, the WHOQOL-BREF did not prove to be a sensitive measure of fatigue, which is the most common symptom in sarcoidosis patients.

  18. Sarcoidosis HLA class II genotyping distinguishes differences of clinical phenotype across ethnic groups

    PubMed Central

    Sato, Hiroe; Woodhead, Felix A.; Ahmad, Tariq; Grutters, Jan C.; Spagnolo, Paolo; van den Bosch, Jules M.M.; Maier, Lisa A.; Newman, Lee S.; Nagai, Sonoko; Izumi, Takateru; Wells, Athol U.; du Bois, Roland M.; Welsh, Kenneth I.

    2010-01-01

    The HLA class II (DRB1 and DQB1) associations with sarcoidosis have been studied by several groups but often without consistent results. In this paper, we consider the hypothesis that observed inconsistencies relate to distinct, genetically encoded disease phenotypes which differ in prevalence between centres. We therefore typed HLA-DRB1 and DQB1 in 340 UK, 139 Dutch and 163 Japanese sarcoidosis patients and, respectively, 354, 218 and 168 healthy controls from these populations. We applied consistent phenotyping and genotyping and investigated associations between HLA class II alleles and distinct disease phenotypes within and between ethnic groups. DRB1*01 and DQB1*0501 are protective against all manifestations of sarcoidosis. Lung-predominant sarcoidosis is associated with DRB1*12 and *14. Löfgren's syndrome is a common sarcoidosis phenotype in the Dutch and is strongly associated with the DRB1*0301 allele. This phenotype is not seen among the Japanese in whom DRB1*0301 is absent. The same allele is protective for UK uveitis. Sarcoid uveitis is common in Japan. The DRB1*04–DQB1*0301 haplotype is a risk factor for this disease manifestation in Japanese and UK subjects but protective for sarcoidosis overall. We show that distinct sarcoidosis phenotypes have similar genetic associations across ethnic groups. The disease case mix differs between centres and may be explained by different ethnic allelic frequencies. PMID:20685690

  19. Acute kidney injury as the presenting manifestation of sarcoidosis: A case series and review of literature.

    PubMed

    Rajkumar, Theepika; Lea-Henry, Tom; Chacko, Bobby

    2017-10-04

    Acute kidney injury is rarely the presenting feature of sarcoidosis. We present a case series of patients whose diagnosis of sarcoidosis was only brought to light by the development of renal impairment. Concurrent hypercalcaemia was noted, prompting further investigation. The patients discussed experienced a significant and rapid improvement in both renal function and hypercalcaemia in response to therapy with prednisolone. This is out of keeping with previous reports of sarcoidosis-induced renal impairment. Our case series highlights the importance of testing for hypercalcaemia in the context of acute kidney injury. Sarcoidosis is primarily a disease of the lungs and reticuloendothelial system, however the prevalence of renal involvement with sarcoidosis may be under-recognised. The renal manifestations of sarcoidosis are discussed in the context of the current literature. Furthermore, from our experience, we postulate that in the context of sarcoidosis-induced renal injury, concurrent hypercalcaemia may present prior to the development of chronic renal injury and therefore these patients may be more likely to recover renal function. This article is protected by copyright. All rights reserved.

  20. Sudden cardiac death in infiltrative cardiomyopathies: sarcoidosis, scleroderma, amyloidosis, hemachromatosis.

    PubMed

    Lubitz, Steven A; Goldbarg, Seth H; Mehta, Davendra

    2008-01-01

    Sarcoidosis, scleroderma, amyloidosis, and hemachromatosis are systemic infiltrative disorders that commonly affect the heart. Owing to their potential for diffuse organ involvement, these diseases may present with myriad clinical manifestations. Conduction system abnormalities are common, and each of these disorders has been associated with sudden cardiac death. In this review, we summarize the epidemiology, clinical features, diagnosis, and treatment of each of these entities. We place special emphasis on existing literature as it pertains to risk stratification and therapy aimed at the prevention of sudden cardiac death in these infiltrative cardiomyopathies.

  1. Thoracic Sarcoidosis: Imaging with High Resolution Computed Tomography

    PubMed Central

    Singh, Sarvinder; Jain, Megha; Singh, Satyendra Narayan; Sharma, Rajat Kumar

    2017-01-01

    Introduction Sarcoidosis is a disease of unknown aetiology that primarily affects the lungs. Clinical and radiological findings with demonstration of non caseating granulomas on pathology is utilised for diagnosing the disease. Aim To assess and evaluate the features of thoracic sarcoidosis on High Resolution Computed Tomography (HRCT) chest. Materials and Methods A total of 40 (31 males and 9 females) cases of pulmonary sarcoidosis in a period of three years were included in this study. Patients underwent detailed clinical evaluation, imaging, Pulmonary Function Tests (PFT) and pathological confirmation of disease. Chest radiograph was obtained in all patients. HRCT was done on 16 slice Computed Tomography (CT) using 1 mm slice thickness and high spatial frequency algorithm for image re-construction. Images were viewed and evaluated using appropriate lung and mediastinal windows. The lymph nodes were classified as hilar and mediastinal with Maximum Short Axis Diameter (MSAD) more than 10 mm taken as cut-off for enlargement. Pulmonary opacities were classified as nodules (micronodules 1-4 mm and macronodules >5 mm), reticular opacities, fibrotic lesions, ground glass opacities and consolidations. Nodule distribution classified as perilymphatic centrilobular and random. Repeat scanning done on follow up or as clinically indicated. Results A total of five patients had Stage I disease, 24 patients had Stage II disease, eight patients had Stage III disease and three patients had stage IV disease. Mediastinal lymphdenopathy present in 29 patients. Bilateral hilar adenopathy was the predominant pattern seen in 22 patients. Lung parenchymal lesions excluding end stage disease noted in 32 patients. The characteristic HRCT lung parenchymal involvement of micronodules with a perilymphatic distribution was seen in 26 patients. HRCT features of predominant upper and middle lobe distribution seen in majority of patients. Documented atypical lesions and the characteristic

  2. HTLV-1 seroprevalance in sarcoidosis. A clinical and laboratory study in northeast of Iran.

    PubMed

    Saghafi, Massoud; Rezaieyazdi, Zahra; Nabavi, Shima; Mirfeizi, Zahra; Sahebari, Maryam; Salari, Masoumeh

    2017-02-09

    Sarcoidosis is an autoimmune multiorgan granulomatosis disease with unknown origin. Some environmental factors such as viruses may induce the disease in genetically susceptible individuals. Human T cell lymphotropic virus type 1 (HTLV-1) can dysregulate the human immune system and the role of this virus in the pathogenesis of autoimmune diseases has been investigated and documented, such as in uveitis. In this study, we have focused on the seroprevalence of HTLV-1 in sarcoidosis in comparison to the normal population in the northeast of Iran, an endemic area for HTLV-1. This cross-sectional study enrolled 125 patients with established sarcoidosis to evaluate the frequency of HTLV-1 and compare it with the normal population of Mashhad, Iran. Participants' blood samples were analyzed for HTLV-1 antibody by an enzyme-linked immunosorbent assay kit. Positive results were confirmed by polymerase chain reaction method. Finally, data were analyzed using SPSS 11. Among sarcoidosis patients 106 (84.8%) patients had a history of acute course and 19 (15.2%) had chronic sarcoidosis. Four percent of the patients versus 2.12% of the Mashhad population were HTLV-1 positive with no statistical difference (P = 0.201). In age- and sex-matched selected controls, 3.6% were HTLV-1 positive again with no statistical difference by sarcoidosis group (P = 0.52). There was no statistical difference between arthritis, erythema nodusom, uveitis, constitutional symptoms, abnormal chest radiography (parahilar lymphadenopathy) and computed tomography scan findings, respiratory symptoms, sex, the course of the sarcoidosis in HTLV-1 positive and negative sarcoidosis patients. The frequency of HTLV-1 in 125 sarcoidosis patients was 4%. In comparison with prevalence of HTLV-1 in Mashhad, HTLV-1 seroprevalence did not show any significant difference. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  3. Appendectomy, tonsillectomy, and risk for sarcoidosis - A hospital-based case-control study in Japan.

    PubMed

    Sawahata, Michiru; Nakamura, Yosikazu; Sugiyama, Yukihiko

    2017-05-01

    The role of surgery in the onset of sarcoidosis is unclear. We investigated whether surgery is an internal environmental factor for sarcoidosis onset within the Japanese population. We enrolled 222 patients diagnosed with sarcoidosis (78 men, 144 women) who were admitted to our department between 1984 and 2012. We also enrolled 529 control subjects (251 men, 278 women), who were matched for sex, age at admission, and year of admission. Surgical history, family history, and smoking status were evaluated. Multivariate analysis correlated history of appendectomy (OR, 1.55; 95% CI, 1.05-2.29) and tonsillectomy (OR, 2.79; 95% CI, 0.91-8.56) with the occurrence of sarcoidosis; other surgical procedures had no correlation. In women, appendectomy had a stronger association with sarcoidosis (OR, 1.69; 95% CI, 1.05-2.73), as opposed to that in men (OR, 1.39; 95% CI, 0.68-2.85). This association was greater in women aged ≥45 years than in those aged <45 years. There was a stronger correlation between tonsillectomy and sarcoidosis in women (OR, 3.30; 95% CI, 0.88-12.39), than in men (OR, 1.26; 95% CI, 0.10-16.52). ORs for sarcoidosis were 5.55 (95% CI, 2.02-15.27) and 0.97 (95% CI, 0.52-1.84) in women aged ≥45 years with a history of appendectomy at <20 years and ≥20 years, respectively, with the former being statistically significant. Appendix and tonsil removal was associated with sarcoidosis onset, suggesting their potential protective role against sarcoidosis development. Further studies are needed to minimize possible confounding factors. Copyright © 2017 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

  4. The Association between ANXA11 Gene Polymorphisms and Sarcoidosis: a Meta-Analysis and systematic review.

    PubMed

    Zhou, Hongfei; Diao, Mengyuan; Zhang, Mingyue

    2016-08-01

    The associations of ANXA11 gene polymorphisms and susceptibility to sarcoidosis have been evaluated in recent years. However, the results remain controversial, especially in different ethnicity. To assess the associations between ANXA11 and sarcoidosis, we conducted this meta-analysis. Articles were searched in MEDLINE, EMBASE and PubMed from their establishment date to August of 2014, and 4,567 sarcoidosis patients and 4,278 controls from 6 studies were included. The strength of associations was determined by ORs with 95% CIs. The associations between ANXA11 SNP rs1049550, rs2573346, rs2789679 polymorphisms and sarcoidosis risk were assessed using additive, recessive and dominant models. ANXA11 SNP rs2573346 and rs2789679 T allele conferred protection against sarcoidosis (OR: 0.664, 95% CI: 0.607-0.726 for rs2573346, and OR: 0.698, 95% CI: 0.640-0.762 for rs2789679). For SNP rs1049550, individuals carrying the ''T'' allele (TT+CT) had a nearly 46% increased risk for the development of sarcoidosis, when compared with CC homozygotes (OR: 1.461, 95% CI: 1.183-1.803) in overall population. A significant association was also found in additive model (OR: 1.477, 95% CI: 1.328-1.642 for CC vs. CT; OR: 0.610, 95% CI: 0.412-0.905 for TT vs. CC). In addition, ethnicity factors may contribute to the disease risk. The meta-analysis revealed that ''T'' allele of ANXA11 SNP rs2573346 and rs2789679 conferred protection against sarcoidosis. ''C'' allele of SNP rs1049550 may be a risk factor for sarcoidosis in overall population. Our study shows that ANXA11 closely associated with the development of sarcoidosis but further studies in different ethnicity were needed.

  5. Risk of Malignancy Among Patients With Sarcoidosis: A Population-Based Cohort Study.

    PubMed

    Ungprasert, Patompong; Crowson, Cynthia S; Matteson, Eric L

    2017-01-01

    To investigate the risk of malignancy in patients with sarcoidosis in a population-based cohort. A cohort of Olmsted County, Minnesota, residents diagnosed with sarcoidosis between January 1, 1976 and December 31, 2013 was identified based on individual medical record review. For each sarcoidosis subject, 2 sex- and age-matched comparator subjects without sarcoidosis were randomly selected. Cases and comparators were then cross-indexed with the Mayo Clinic Cancer Registry, which collected data on every type of malignancy except for nonmelanoma skin cancer, for malignancy ascertainment. A total of 345 incident cases of sarcoidosis and 690 comparators were identified. There was no difference in the prevalence of malignancy at the index date between the 2 groups (4.3% among cases and 4.3% among comparators; P = 1.0). During followup, 36 patients with sarcoidosis and 91 subjects without sarcoidosis developed malignancy, with a cumulative incidence at 10 years of 3.8% and 7.1%, respectively. The difference corresponded to a hazard ratio (HR) of 0.72 (95% confidence interval [95% CI] 0.49-1.06). The cumulative incidences at 10 years for individual types of malignancy were also similar between the 2 groups, with nonsignificant HRs. However, subgroup analysis found that cases with extrathoracic involvement were at higher risk of incident hematologic malignancy compared with cases without extrathoracic involvement (HR 1.87 [95% CI 1.09-3.22]). Risk of malignancy was similar among patients with sarcoidosis compared to nonsarcoidosis subjects. However, the risk of incident hematologic malignancy was significantly higher among patients with sarcoidosis with extrathoracic involvement compared to patients without extrathoracic disease. © 2016, American College of Rheumatology.

  6. Upper Airway Obstruction Requiring Emergent Tracheostomy Secondary to Laryngeal Sarcoidosis: A Case Report.

    PubMed

    Ryu, Changwan; Herzog, Erica L; Pan, Hongyi; Homer, Robert; Gulati, Mridu

    2017-02-13

    BACKGROUND Laryngeal sarcoidosis is a rare extrapulmonary manifestation of sarcoidosis, accounting for 0.33-2.1% of cases. A life-threatening complication of laryngeal sarcoidosis is upper airway obstruction. In this report we describe our experience in the acute and chronic care of a patient who required an emergent tracheostomy, with the aim to provide further insight into this difficult to manage disease. CASE REPORT A 37-year-old African American female with a 10-year history of stage 1 sarcoidosis presented with severe dyspnea. Laryngeal sarcoidosis was diagnosed three years previously, and she remained stable on low-dose prednisone until six months prior to admission, at which time she self-discontinued her prednisone for the homeopathic treatment Nopalea cactus juice. Her physical examination was concerning for impending respiratory failure as she presented with inspiratory stridor and hoarseness. Laryngoscopy showed a retroflexed epiglottis obstructing the glottis with edematous arytenoids and aryepiglottic folds. Otolaryngology performed an emergent tracheostomy to secure her airway and obtained epiglottic biopsies, which were consistent with sarcoidosis. She was eventually discharged home on prednisone 60 mg daily. Following months of corticosteroids, a laryngoscopy showed the epiglottis continuing to obstruct the glottis. The addition of methotrexate to a tapered dosage of prednisone 10 mg daily was unsuccessful, and she remains on prednisone 20 mg daily for disease control. CONCLUSIONS Laryngeal sarcoidosis, a rare extrapulmonary manifestation of sarcoidosis, uncommonly presents as the life-threatening complication of complete upper airway obstruction. As such, laryngeal sarcoidosis is associated with significant morbidity and mortality, requiring a high index of suspicion for timely diagnosis and treatment.

  7. Value of neutrophil/lymphocyte ratio in the differential diagnosis of sarcoidosis and tuberculosis

    PubMed Central

    Iliaz, Sinem; Iliaz, Raim; Ortakoylu, Gonenc; Bahadir, Ayse; Bagci, Belma Akbaba; Caglar, Emel

    2014-01-01

    INTRODUCTION: The differential diagnosis of sarcoidosis creates a challange due to tuberculosis also having lung and lymph node involvement. Because both diseases show granulomatous inflammation, it may not be possible to distinguish tuberculosis and sarcoidosis in pathological specimens. As a result of the complexity in the differential diagnosis of sarcoidosis and tuberculosis, new markers for differentiation are being investigated. OBJECTIVE: The aim of our study is to investigate the value of neutrophil/lymphocyte ratio (NLR) as a possible marker in differentiating sarcoidosis and tuberculosis. MATERIALS AND METHODS: In our study, 51 acid-fast bacilli (AFB) positive and/or culture-positive patients with pulmonary tuberculosis, ​​40 patients with biopsy-proven sarcoidosis and a control group consisting of 43 patients were included. In our study, information was collected retrospectively based on hospital records. RESULTS: Leukocyte and neutrophil counts, NLR, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) were significantly higher, and albumin was significantly lower in the tuberculosis group compared with sarcoidosis (for all parameters P < 0.001). The most appropriate cut-off value of NLR to distinguish tuberculosis from sarcoidosis was determined as 2.55. For this cut-off value of NLR there was 79% sensitivity, 69% specificity, 73% positive predictive value (PPV), 75% negative predictive value (NPV), and area under the curve (AUC) was 0.788. For differentiation of sarcoidosis from tuberculosis, accuracy of the NLR test according to this cut-off value was found as 76%. CONCLUSION: NLR as a little known marker in respiratory medicine was found to be supportive in differentiation of tuberculosis and sarcoidosis. More studies on this issue is needed. PMID:25276243

  8. Clinicopathological study of glomerular diseases associated with sarcoidosis: a multicenter study

    PubMed Central

    2013-01-01

    Background The association between sarcoidosis and glomerular diseases has not been extensively investigated in a large series and the potential features of this uncommon association remain to be determined. Methods We retrospectively identified 26 patients with biopsy-proven glomerular lesions that occurred in a sarcoidosis context. Potential remission of glomerular disease and sarcoidosis under specific treatment (steroid and/or immunosuppressive agents) was recorded for all patients. Demographic, clinical and biological characteristics were assessed at the time of kidney biopsy for each patient. Therapeutic data were analyzed for all patients. Results Glomerular disease occurred after the diagnosis of sarcoidosis in 11 of 26 cases (42%) (mean delay of 9.7 years). In six patients (23%), the glomerulopathy preceded the sarcoidosis diagnosis (mean delay 8 years). In the last nine patients (35%), both conditions occurred simultaneously. The most frequent glomerular disease occurring in sarcoidosis patients was membranous nephropathy in eleven cases. Other glomerular lesions included IgA nephropathy in six cases, focal segmental glomerulosclerosis in four patients, minimal change nephrotic syndrome for three patients and proliferative lupus nephritis in two patients. Granulomatous interstitial nephritis was associated with glomerular disease in six patients and was exclusively found in patients in whom the both disease occurred simultaneously. In nine patients with simultaneous glomerular and sarcoidosis diseases, we observed a strong dissociation between glomerular disease and sarcoidosis in terms of steroid responsiveness. At the end of the follow-up (mean of 8.4 years), six patients had reached end-stage renal disease and three patients had died. Conclusions A wide spectrum of glomerular lesions is associated with sarcoidosis. The close temporal relationship observed in some patients suggests common causative molecular mechanisms of glomerular injury but

  9. A Circulating Micro-RNA Signature Serves as a Diagnostic and Prognostic Indicator in Sarcoidosis.

    PubMed

    Ascoli, Christian; Huang, Yue; Schott, Cody; Turturice, Benjamin A; Metwally, Ahmed; Perkins, David L; Finn, Patricia W

    2017-08-16

    Micro-RNAs (miRNAs) act as post-transcriptional regulators of gene expression. In sarcoidosis, aberrant miRNA expression may enhance immune responses mounted against an unknown antigenic agent. We tested whether a distinct miRNA signature functions as a diagnostic biomarker and explored its role as an immune modulator in sarcoidosis. Expression of miRNAs from peripheral blood mononuclear cells from subjects that met clinical and histopathologic criteria for sarcoidosis were compared to those from matched controls in the ACCESS study. Signature miRNAs were determined by miRNA microarray analysis and validated by quantitative reverse transcription PCR (RT-qPCR). Microarray analysis identified 54 differentially expressed feature mature human miRNAs between groups. Significant feature miRNAs that distinguish sarcoidosis from controls were selected by use of probabilistic models adjusted for clinical variables. Eight signature miRNAs were chosen to verify the diagnosis of sarcoidosis in a validation cohort and distinguished sarcoidosis from controls with a positive predictive value of 88%. We identified both novel and previously described genes and molecular pathways associated with sarcoidosis as targets of these signature miRNAs. Additionally, we demonstrate that signature miRNAs (hsa-miR-150-3p and hsa-miR-342-5p) are significantly associated with reduced lymphocytes and airflow limitations, known markers of poor prognosis. Together, these findings suggest that a circulating miRNA signature serves as a non-invasive biomarker that supports the diagnosis of sarcoidosis. Future studies will test the miRNA signature as a prognostication tool associated with poor clinical outcomes in sarcoidosis.

  10. Exacerbation of recalcitrant cutaneous sarcoidosis with adalimumab - a paradoxical effect? A case report*

    PubMed Central

    Santos, Guida; Sousa, Lourdes Emerenciano; João, Alexandre Miguel Bruno Lopes

    2013-01-01

    The paradoxical adverse effects of tumor necrosis factor-alpha (TNF-alpha) antagonists have been described frequently as a result of the widespread use of these drugs. Among the TNF-alpha blocking agents, few reports exist relating the use of adalimumab in cutaneous sarcoidosis, although all of them show good results. More recently, sarcoidosis onsets have been reported with various TNF-alpha inhibitors. The current case is, to our knowledge, the first to describe the exacerbation of cutaneous lesions of sarcoidosis treated with adalimumab. PMID:24346872

  11. Sarcoidosis on tattoos: a review of the literature from 1939 to 2011.

    PubMed

    Kluger, N

    2013-08-01

    Sarcoidosis is an autoimmune disease of unknown etiology characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs. Cutaneous sarcoidosis occurs in approximately 25% of the cases. Sarcoid reactions on old scars, traumatized skin sites and around embedded foreign material have long been observed. For the past 70 years, sarcoidal granulomas on tattoos and permanent make-up have also been documented. Granulomatous and sarcoidal tattoo reactions may be the first and sometimes only cutaneous manifestation of systemic sarcoidosis. This review summarizes the currently available data on this topic and discusses the issues related to the diagnosis, management and physiopathogeny of sarcoidal reactions on tattoos.

  12. Pulmonary sarcoidosis mimicking tuberculosis: Importance of the galaxy sign on thoracic computed tomography.

    PubMed

    Gorospe Sarasúa, Luis; Ureña-Vacas, Almudena; Arrieta, Paola; Santos-Carreño, Astrid Lucía; Navas-Elorza, Enrique; de la Puente-Bujidos, Carlos

    2017-08-29

    Sarcoidosis and tuberculosis are two common granulomatous conditions that may share clinical and radiological presentations. The galaxy sign (sarcoid galaxy sign) is a characteristic radiological sign of pulmonary sarcoidosis on thoracic computed tomography (CT). We present the case of a patient with sarcoidosis that was initially misdiagnosed as tuberculosis, in whom the galaxy sign on CT was useful as it suggested the correct diagnosis. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  13. A granulomatous conundrum: Concurrent necrobiosis lipoidica, cutaneous sarcoidosis and erythema nodosum in a nondiabetic patient.

    PubMed

    Valecha, Nishant; Bennett, Genevieve; Yip, Leona

    2017-02-17

    Necrobiosis lipoidica (NL) and cutaneous sarcoidosis are granulomatous disorders with a largely unknown aetiopathogenesis. Evidence of co-existing NL and sarcoidosis in the same patient may suggest a degree of overlap between these entities through shared granulomatous inflammatory pathways. Occasionally, one condition can mimic the other, making their distinction difficult. We report a novel case of a non-diabetic woman who presented with concurrent NL, cutaneous sarcoidosis and erythema nodosum. We discuss some of the complexities distinguishing these entities and propose that they may represent different stages of the same granulomatous process linked through yet unknown pathomechanisms.

  14. Acquired anhidrosis associated with systemic sarcoidosis: Quantification of nerve fibers around eccrine glands by confocal microscopy.

    PubMed

    Nishida, M; Namiki, T; Sone, Y; Hashimoto, T; Tokoro, S; Hanafusa, T; Yokozeki, H

    2017-08-10

    Neurological disorders can cause hypohidrosis and/or anhidrosis by disturbing either the central or the peripheral nervous systems.(1-3) Although a syringotropic variant of cutaneous sarcoidosis causes dysfunction of sweating, systemic sarcoidosis rarely causes hypohidrosis or anhidrosis.(4,5) Here we present a novel case of an acquired anhidrosis in a patient with systemic sarcoidosis. Furthermore, we developed a novel methodology to quantify nerve fibers around eccrine glands using confocal microscopy and found that nerve fibers around eccrine glands in anhidrotic areas are significantly decreased compared to hidrotic areas. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  15. Isolated Gastrointestinal Sarcoidosis Involving Multiple Gastrointestinal Sites Presenting as Chronic Diarrhea

    PubMed Central

    Gaye, Bilkisu; Makary, Raafat; Monteiro, Carmela; Eid, Emely

    2016-01-01

    Sarcoidosis is a chronic and systemic disorder characterized by the formation of non-caseating granulomas. Very few cases of isolated gastrointestinal sarcoidosis have been reported, and even fewer, if any, report gastrointestinal sarcoidosis within multiple gastrointestinal sites concomitantly. We present a 42-year-old white man with chronic diarrhea and abdominal pain for more than 3 years. Mucosal biopsies revealed non-caseating microgranulomas in the stomach, throughout the small intestine, colon, and rectum. Prednisone therapy was initiated with a rapid improvement in symptoms and complete resolution of diarrhea within 3 weeks. PMID:28119949

  16. Ocular delivery of macromolecules

    PubMed Central

    Kim, Yoo-Chun; Chiang, Bryce; Wu, Xianggen; Prausnitz, Mark R.

    2014-01-01

    Biopharmaceuticals are making increasing impact on medicine, including treatment of indications in the eye. Macromolecular drugs are typically given by physician-administered invasive delivery methods, because non--invasive ocular delivery methods, such as eye drops, and systemic delivery, have low bioavailability and/or poor ocular targeting. There is a need to improve delivery of biopharmaceuticals to enable less-invasive delivery routes, less-frequent dosing through controlled-release drug delivery and improved drug targeting within the eye to increase efficacy and reduce side effects. This review discusses the barriers to drug delivery via various ophthalmic routes of administration in the context of macromolecule delivery and discusses efforts to develop controlled-release systems for delivery of biopharmaceuticals to the eye. The growing number of macromolecular therapies in the eye needs improved drug delivery methods that increase drug efficacy, safety and patient compliance. PMID:24998941

  17. Sudden death from systemic sarcoidosis: a case of legal medicine.

    PubMed

    Zoja, R; Andreola, S; Gentile, G; Rancati, A

    2012-03-01

    The sarcoid condition of vital organs such as heart, lungs, liver and brain, may constitute, though rarely, a dangerous situation for survival. In forensic pathology, sudden death related to such disease represents an unusual event occurring in subjects who die unexpectedly in spite of their previous good health, and whose autopsy reveals, mainly, the involvement of heart or the central nervous system (CNS). The Authors describe a case of sudden death due to systemic sarcoidosis with atypical presentation in a young South American nulliparous woman showing, as the only symptom, occasional episodes of spotting during the last two months of her life. The only noteworthy finding from the autopsy resulted in secondary obstructive hydrocephalus. The subsequent forensic toxicological examination was negative, whereas the histopathological examination, conducted by means of post-fixation techniques and standard coloring methods on the viscera removed during autopsy, revealed useful data to determine the cause of death, consisting of a diffuse inflammatory reaction with giant cells and epithelioid cells of sarcoid type localized in the CNS and in the genitourinary apparatus. The case presented, ranking among deaths occurred unexpectedly, is a useful opportunity both for clinical remarks, given its inherent diagnostic difficulties, especially in the presence of atypical symptoms, and for legal medicine ones, given the inclusion of sarcoidosis in cases of sudden death that can give rise to criminal investigations.

  18. Vertebral sarcoidosis: demonstration of bone involvement by computerized axial tomography

    SciTech Connect

    Dinerstein, S.L.; Kovarsky, J.

    1984-08-01

    A report is given of a rare case of vertebral sarcoidosis with negative conventional spinal x-ray films, yet with typical cystic lesions of the spine found incidentally during abdominal computerized axial tomography (CAT). The patient was a 28-year-old black man, who was admitted for evaluation of a 1 1/2-year history of diffuse myalgias, intermittent fever to 102 F orally, bilateral hilar adenopathy, and leukopenia. A technetium polyphosphate bone scan revealed diffuse areas of increased uptake over the sternum, entire vertebral column, and pelvis. Conventional x-ray films of the cervical, thoracic, and lumbar spine, and an AP view of the pelvis were all normal. Chest x-ray film revealed only bilateral hilar adenopathy. During the course of an extensive negative evaluation for infection, an abdominal CAT scan was done, showing multiple, small, sclerotic-rimmed cysts at multiple levels of the lower thoracic and lumbar spine. Bone marrow biopsy revealed only changes consistent with anemia of chronic disease. Mediastinal lymph node biopsy revealed noncaseating granulomas. A tentative diagnosis of sarcoidosis was made, and treatment with prednisone, isoniazid and rifampin was begun. Within two weeks of initiation of prednisone therapy, the patient was symptom-free. A repeat technetium polyphosphate bone scan revealed only a small residual area of mildly increased uptake over the upper thoracic vertebrae.

  19. Ocular sparganosis from Assam

    PubMed Central

    Nath, Reema; Gogoi, Rajendra Nath

    2015-01-01

    Sparganosis is caused by plerocercoid larvae of the Pseudophyllidea tapeworms of the genus Spirometra. Though prevalent in East Asian and south east Asian countries like China, Japan, Korea, Taiwan, Vietnam, Thailand; yet very few cases are reported from India. We report a case of migrating sub-conjunctival ocular sparganosis mimicking scleritis which later on developed into orbital cellulitis from Dibrugarh, Assam, North-eastern part of India. This case is reported for its rarity. PMID:25709957

  20. Human ocular anatomy.

    PubMed

    Kels, Barry D; Grzybowski, Andrzej; Grant-Kels, Jane M

    2015-01-01

    We review the normal anatomy of the human globe, eyelids, and lacrimal system. This contribution explores both the form and function of numerous anatomic features of the human ocular system, which are vital to a comprehensive understanding of the pathophysiology of many oculocutaneous diseases. The review concludes with a reference glossary of selective ophthalmologic terms that are relevant to a thorough understanding of many oculocutaneous disease processes.

  1. Caveats and truths in genetic, clinical, autoimmune and autoinflammatory issues in Blau syndrome and early onset sarcoidosis.

    PubMed

    Caso, Francesco; Costa, Luisa; Rigante, Donato; Vitale, Antonio; Cimaz, Rolando; Lucherini, Orso Maria; Sfriso, Paolo; Verrecchia, Elena; Tognon, Sofia; Bascherini, Vittoria; Galeazzi, Mauro; Punzi, Leonardo; Cantarini, Luca

    2014-12-01

    Blau syndrome (BS) and early onset sarcoidosis (EOS) are, respectively, the familial and sporadic forms of the pediatric granulomatous autoinflammatory disease, which belong to the group of monogenic autoinflammatory syndromes. Both of these conditions are caused by mutations in the NOD2 gene, which encodes the cytosolic NOD2 protein, one of the pivotal molecules in the regulation of innate immunity, primarily expressed in the antigen-presenting cells. Clinical onset of BS and EOS is usually in the first years of life with noncaseating epithelioid granulomas mainly affecting joints, skin, and uveal tract, variably associated with heterogeneous systemic features. The dividing line between autoinflammatory and autoimmune mechanisms is probably not so clear-cut, and the relationship existing between BS or EOS and autoimmune phenomena remains unclear. There is no established therapy for the management of BS and EOS, and the main treatment aim is to prevent ocular manifestations entailing the risk of potential blindness and to avoid joint deformities. Nonsteroidal anti-inflammatory drugs, corticosteroids and immunosuppressive drugs, such as methotrexate or azathioprine, may be helpful; when patients are unresponsive to the combination of corticosteroids and immunosuppressant agents, the tumor necrosis factor-α inhibitor infliximab should be considered. Data on anti-interleukin-1 inhibition with anakinra and canakinumab is still limited and further corroboration is required. The aim of this paper is to describe BS and EOS, focusing on their genetic, clinical, and therapeutic issues, with the ultimate goal of increasing clinicians' awareness of both of these rare but serious disorders.

  2. Ocular injuries in automobile crashes.

    PubMed

    Huelke, D F; O'Day, J; Barhydt, W H

    1982-01-01

    Tempered windshields commonly used in Europe have been shown to be related to the relatively high incidence of ocular injuries. Although windshields of the High Penetration Resistant (HPR) type in cars in North America are not at all significantly involved in ocular injuries, still about 50 % of the injuries of the eye area are caused by glass. The HPR windshield probably is the main reason for the relatively low occurrence of ocular injuries in United States crashes compared to these injuries reported from countries with tempered windshields. No ocular injuries were observed among belted occupants in this study. It appears that the increased use of lap-shoulder belts would decrease the likelihood of occupant contact with the windshield, mirrors, roof support, steering wheel, and instrument panel- about two thirds of the occupant contacts related to ocular injury- and thus reduce the incidence of ocular injuries leading to decreased vision.

  3. Pulmonary tuberculosis followed by sarcoidosis in an HIV-infected patient: A case report and a simplified diagnostic flowchart for diagnosis and treatment of sarcoidosis.

    PubMed

    Mencarini, P; Bellagamba, R; Oliva, A; Ghirga, P; Giancola, M L; Corpolongo, A; Ascoli Bartoli, T; De Nardo, P; Baiocchini, A; Del Nonno, F; Narciso, P; Nicastri, E

    2016-01-01

    The diagnosis of sarcoidosis in a patient living with HIV infection is an uncommon event and a challenge for clinicians. Clinical manifestations are variable and fluctuating depending to adherence to ARV therapy and to the level of CD4 count. We analyze here one chronic case in which sarcoidosis appeared clinically two years after pulmonary tuberculosis. The course of the disease was influenced and prolonged by frequent interruptions of antiretroviral therapy. Moreover the diagnosis and the decision to treat have been delayed by the need of exclusion of other pathologies, principally tuberculosis reactivation/reinfection, other mycobacterial diseases, hematologic malignancies. We propose a simplified flowchart for diagnosis and follow up of sarcoidosis, which may also be applied to patients with HIV infection. Diagnosis of latent tuberculosis infection (LTBI) may be difficult in these patients, because the immunological paradox of sarcoidosis. For this reason, following exclusion of active tuberculosis, we advise to submit all sarcoidosis patients to IPT (isoniazid preventive therapy), when immunosuppressive therapy is started.

  4. [A case of lichenoid sarcoidosis with characteristic clinical and histopathological findings].

    PubMed

    Sanchez-Lopez, Josefa; Porriño-Bustamante, Ma Librada; Aneiros-Fernández, Jose; Naranjo-Sintes, Ramon; Fernández-Pugnaire, Ma Antonia

    2014-04-16

    Sarcoidosis is a multisystem inflammatory disease characterized by the formation of noncaseating granulomas in various organs and tissues. The majority of patients with systemic sarcoidosis will present with lung and lymph node involvement. In addition, 20% have skin involvement that may be the only manifestation of the disease or may be an important prognostic marker for involvement of other organs. There are multiple forms of presentation of cutaneous sarcoidosis, which may be a true challenge.We report a patient with a one month history of an eruption of skin colored papules. Some were grouped in a symmetrical distribution on the trunk, inner arms, and lumbar region. Pathologic examination revealed an infiltrate in the papillary dermis showing a band of noncaseating granulomas along with disruption of the basal lamina and lichenoid changes. The clinicopathological correlation confirmed the diagnosis of lichenoid sarcoidosis. We consider our case interesting owing to the clinical presentation and the lichenoid distribution of granulomas.

  5. Multiple bilateral pulmonary nodules masquerading as pulmonary metastasis; a case of nodular sarcoidosis

    PubMed Central

    Jafari, Mostafa; Farrokh, Donya; Mohammadpanah, Najmeh

    2016-01-01

    Sarcoidosis is a multi-system inflammatory disorder of unknown etiology that is manifested by the presence of non-caseating granulomas. Multiple pulmonary nodules are rare presentations of sarcoidosis. We report a case of nodular sarcoidosis in a young male of Middle-East origin who had initially presented with bilateral painful ankle edema. His chest X-ray showed multiple bilateral pulmonary nodules. A high resolution computed tomography scan of the chest demonstrated multiple pulmonary nodular lesions and also mediastinal and hilar lymphadenopathy. Subsequent biopsies revealed non-necrotizing granuloma with multi-nucleated giant cells indicative of sarcoidosis. An appropriate work-up was done to confirm the true nature of the nodules and facilitate treatment. PMID:27757192

  6. (18)F-fluoromisonidazole (FMISO) PET may have the potential to detect cardiac sarcoidosis.

    PubMed

    Manabe, Osamu; Hirata, Kenji; Shozo, Okamoto; Shiga, Tohru; Uchiyama, Yuko; Kobayashi, Kentaro; Watanabe, Shiro; Toyonaga, Takuya; Kikuchi, Hisaya; Oyama-Manabe, Noriko; Tamaki, Nagara

    2017-02-01

    (18)F-fluoromisonidazole (FMISO) is a positron emission tomography (PET) tracer that accumulates in hypoxic tissues. We here present a case of suspected cardiac sarcoidosis which was detected with increased FMISO uptake.

  7. Muscular Sarcoidosis Detected by F-18 FDG PET/CT in a Hypercalcemic Patient

    PubMed Central

    Han, Eun Ji; Jang, Yi Sun; Lee, In Suk; Lee, Jong Min; Kang, Siwon

    2013-01-01

    Sarcoidosis is a systemic granulomatous disease of unknown etiology that involves many organs, occasionally mimicking malignancy. We herein report a 50-yr-old woman of muscular sarcoidosis of chronic myopathic type, manifested by hypercalcemia and muscle wasting. Besides insignificant hilar lymphadenopathy, her sarcoidosis was confined to generalized atrophic muscles and therefore, F-18 FDG PET/CT alone among conventional imaging studies provided diagnostic clues for the non-parathyroid-related hypercalcemia. On follow-up PET/CT during low-dose steroid treatment, FDG uptake in the muscles disappeared whereas that in the hilar lymph nodes remained. PET/CT may be useful in the evaluation of unexpected disease extent and monitoring treatment response in suspected or known sarcoidosis patients. PMID:24015050

  8. Ocular neuropathy in peripheral neuropathies.

    PubMed

    Evliyaoglu, Ferhat; Karadag, Remzi; Burakgazi, Ahmet Z

    2012-11-01

    Ocular movements and coordination require complex and integrated functions of somatic and autonomic nervous systems. Neurological disorders affecting these nervous systems may cause ocular dysfunction involving extraocular muscles and pupils. In this article, the prevalence, clinical presentations, and management of ocular neuropathy related to certain peripheral neuropathies, including diabetic neuropathy, Guillain-Barré syndrome (GBS), chronic inflammatory neuropathies, human immunodeficiency virus (HIV)-associated neuropathy, and hereditary neuropathies, are examined in detail. Copyright © 2012 Wiley Periodicals, Inc.

  9. Proteomic Analysis of Kveim Reagent Identifies Targets of Cellular Immunity in Sarcoidosis.

    PubMed

    Eberhardt, Christian; Thillai, Muhunthan; Parker, Robert; Siddiqui, Nazneen; Potiphar, Lee; Goldin, Rob; Timms, John F; Wells, Athol U; Kon, Onn M; Wickremasinghe, Melissa; Mitchell, Donald; Weeks, Mark E; Lalvani, Ajit

    2017-01-01

    Kveim-reagent (Kv) skin testing was a historical method of diagnosing sarcoidosis. Intradermal injection of treated sarcoidosis spleen tissue resulted in a granuloma response at injection site by 4-6 weeks. Previous work indicates proteins as the possible trigger of this reaction. We aimed to identify Kv-specific proteins and characterise the ex vivo response of Peripheral Blood Mononuclear Cells (PBMCs) from sarcoidosis, tuberculosis and healthy control patients when stimulated with both Kv and selected Kv-specific proteins. Kv extracts were separated by 1D-SDS-PAGE and 2D-DIGE and then underwent mass spectrometric analysis for protein identification. Sarcoidosis and control PBMCs were first stimulated with Kv and then with three selected recombinant protein candidates which were identified from the proteomic analysis. PBMC secreted cytokines were subsequently measured by Multiplex Cytokine Assay. We observed significantly increased IFN-γ and TNF-α secretion from Kv-stimulated PBMCs of sarcoidosis patients vs. PBMCs from healthy volunteers (IFN-γ: 207.2 pg/mL vs. 3.86 pg/mL, p = 0.0018; TNF-α: 2375 pg/mL vs. 42.82 pg/mL, p = 0.0003). Through proteomic approaches we then identified 74 sarcoidosis tissue-specific proteins. Of these, 3 proteins (vimentin, tubulin and alpha-actinin-4) were identified using both 1D-SDS-PAGE and 2D-DIGE. Data are available via ProteomeXchange with identifier PXD005150. Increased cytokine secretion was subsequently observed with vimentin stimulation of sarcoidosis PBMCs vs. tuberculosis PBMCs (IFN-γ: 396.6 pg/mL vs 0.1 pg/mL, p = 0.0009; TNF-α: 1139 pg/mL vs 0.1 pg/mL, p<0.0001). This finding was also observed in vimentin stimulation of sarcoidosis PBMCs compared to PBMCs from healthy controls (IFN-γ: 396.6 pg/mL vs. 0.1 pg/mL, p = 0.014; TNF-α: 1139 pg/mL vs 42.29 pg/mL, p = 0.027). No difference was found in cytokine secretion between sarcoidosis and control PBMCs when stimulated with either tubulin or alpha-actinin-4

  10. Sarcoidosis or Sjögren syndrome? Clues to defining mimicry or coexistence in 59 cases.

    PubMed

    Ramos-Casals, Manuel; Brito-Zerón, Pilar; García-Carrasco, Mario; Font, Josep

    2004-03-01

    We present 5 new cases of coexisting sarcoidosis and Sjögren syndrome (SS) and review the literature for additional cases in order to analyze the clinical, immunologic, and histologic characteristics that may help physicians differentiate the mimicry of SS by sarcoidosis from a true coexistence of both autoimmune diseases. We considered the coexistence of sarcoidosis with SS to be when patients presented specific histologic patterns of both diseases, simultaneously or at different times.Fifty-nine patients were included in the analysis (54 identified in the literature search plus our 5 unpublished cases): 49 (83%) patients were female and 10 (17%) were male, with a mean age at diagnosis of 50 years. According to the histopathologic examination of the exocrine glands performed in 53 cases, we defined coexistence of sarcoidosis and SS in 28 cases, while in the remaining 25 patients, sarcoidosis mimicked SS. Clues to identifying when sarcoidosis coexists with SS were a higher prevalence of systemic manifestations (arthritis and uveitis) and positive immunologic parameters (antinuclear antibodies, rheumatoid factor, and anti-Ro/SS-A), as well as the existence of a focal sialadenitis (Chisholm-Mason score grades III-IV, with a CD4+ lymphocytic infiltration) in the salivary gland biopsy. In patients first diagnosed with primary SS, the appearance of some clinical features such as hilar adenopathies, uveitis, or hypercalcemia leads to the diagnosis of coexisting sarcoidosis. A careful application of the new American-European consensus criteria had a sensitivity of 93% and a specificity of 92% in identifying when SS coexists with sarcoidosis.In conclusion, the association of sarcoidosis with SS leads to a true coexistence of both diseases in more than half the patients described in the literature, while in the remaining patients, sarcoidosis mimics SS. In light of these results, sarcoidosis should not be considered as an exclusion criterion for the diagnosis of SS, and in

  11. Proteomic Analysis of Kveim Reagent Identifies Targets of Cellular Immunity in Sarcoidosis

    PubMed Central

    Parker, Robert; Siddiqui, Nazneen; Potiphar, Lee; Goldin, Rob; Timms, John F.; Wells, Athol U.; Kon, Onn M.; Wickremasinghe, Melissa; Mitchell, Donald; Weeks, Mark E.; Lalvani, Ajit

    2017-01-01

    Background Kveim-reagent (Kv) skin testing was a historical method of diagnosing sarcoidosis. Intradermal injection of treated sarcoidosis spleen tissue resulted in a granuloma response at injection site by 4–6 weeks. Previous work indicates proteins as the possible trigger of this reaction. We aimed to identify Kv-specific proteins and characterise the ex vivo response of Peripheral Blood Mononuclear Cells (PBMCs) from sarcoidosis, tuberculosis and healthy control patients when stimulated with both Kv and selected Kv-specific proteins. Methods Kv extracts were separated by 1D-SDS-PAGE and 2D-DIGE and then underwent mass spectrometric analysis for protein identification. Sarcoidosis and control PBMCs were first stimulated with Kv and then with three selected recombinant protein candidates which were identified from the proteomic analysis. PBMC secreted cytokines were subsequently measured by Multiplex Cytokine Assay. Results We observed significantly increased IFN-γ and TNF-α secretion from Kv-stimulated PBMCs of sarcoidosis patients vs. PBMCs from healthy volunteers (IFN-γ: 207.2 pg/mL vs. 3.86 pg/mL, p = 0.0018; TNF-α: 2375 pg/mL vs. 42.82 pg/mL, p = 0.0003). Through proteomic approaches we then identified 74 sarcoidosis tissue-specific proteins. Of these, 3 proteins (vimentin, tubulin and alpha-actinin-4) were identified using both 1D-SDS-PAGE and 2D-DIGE. Data are available via ProteomeXchange with identifier PXD005150. Increased cytokine secretion was subsequently observed with vimentin stimulation of sarcoidosis PBMCs vs. tuberculosis PBMCs (IFN-γ: 396.6 pg/mL vs 0.1 pg/mL, p = 0.0009; TNF-α: 1139 pg/mL vs 0.1 pg/mL, p<0.0001). This finding was also observed in vimentin stimulation of sarcoidosis PBMCs compared to PBMCs from healthy controls (IFN-γ: 396.6 pg/mL vs. 0.1 pg/mL, p = 0.014; TNF-α: 1139 pg/mL vs 42.29 pg/mL, p = 0.027). No difference was found in cytokine secretion between sarcoidosis and control PBMCs when stimulated with either tubulin

  12. HLA-DRB1*1101: A Significant Risk Factor for Sarcoidosis in Blacks and Whites

    PubMed Central

    Rossman, Milton D.; Thompson, Bruce; Frederick, Margaret; Maliarik, Mary; Iannuzzi, Michael C.; Rybicki, Benjamin A.; Pandey, Janardan P.; Newman, Lee S.; Magira, Eleni; Beznik-Cizman, Bojana; Monos, Dimitri

    2003-01-01

    Sarcoidosis is a granulomatous disorder of unknown etiology, associated with an accumulation of CD4+ T cells and a TH1 immune response. Since previous studies of HLA associations with sarcoidosis were limited by serologic or low-resolution molecular identification, we performed high-resolution typing for the HLA-DPB1, HLA-DQB1, HLA-DRB1, and HLA-DRB3 loci and the presence of the DRB4 or DRB5 locus, to define HLA class II associations with sarcoidosis. A Case Control Etiologic Study of Sarcoidosis (ACCESS) enrolled biopsy-confirmed cases (736 total) from 10 centers in the United States. Seven hundred six (706) controls were case matched for age, race, sex, and geographic area. We studied the first 474 ACCESS patients and case-matched controls. The HLA-DRB1 alleles were differentially distributed between cases and controls (P<.0001). The HLA-DRB1*1101 allele was associated (P<.01) with sarcoidosis in blacks and whites and had a population attributable risk of 16% in blacks and 9% in whites. HLA-DRB1-F47 was the amino acid residue most associated with sarcoidosis and independently associated with sarcoidosis in whites. The HLA-DPB1 locus also contributed to susceptibility for sarcoidosis and, in contrast to chronic beryllium disease, a non–E69-containing allele, HLA-DPB1*0101, conveyed most of the risk. Although significant differences were observed in the distribution of HLA class II alleles between blacks and whites, only HLA-DRB1*1501 was differentially associated with sarcoidosis (P<.003). In addition to being susceptibility markers, HLA class II alleles may be markers for different phenotypes of sarcoidosis (DRB1*0401 for eye in blacks and whites, DRB3 for bone marrow in blacks, and DPB1*0101 for hypercalcemia in whites). These studies confirm a genetic predisposition for sarcoidosis and present evidence for the allelic variation at the HLA-DRB1 locus as a major contributor. PMID:14508706

  13. HLA-DRB1*1101: a significant risk factor for sarcoidosis in blacks and whites.

    PubMed

    Rossman, Milton D; Thompson, Bruce; Frederick, Margaret; Maliarik, Mary; Iannuzzi, Michael C; Rybicki, Benjamin A; Pandey, Janardan P; Newman, Lee S; Magira, Eleni; Beznik-Cizman, Bojana; Monos, Dimitri

    2003-10-01

    Sarcoidosis is a granulomatous disorder of unknown etiology, associated with an accumulation of CD4+ T cells and a TH1 immune response. Since previous studies of HLA associations with sarcoidosis were limited by serologic or low-resolution molecular identification, we performed high-resolution typing for the HLA-DPB1, HLA-DQB1, HLA-DRB1, and HLA-DRB3 loci and the presence of the DRB4 or DRB5 locus, to define HLA class II associations with sarcoidosis. A Case Control Etiologic Study of Sarcoidosis (ACCESS) enrolled biopsy-confirmed cases (736 total) from 10 centers in the United States. Seven hundred six (706) controls were case matched for age, race, sex, and geographic area. We studied the first 474 ACCESS patients and case-matched controls. The HLA-DRB1 alleles were differentially distributed between cases and controls (P<.0001). The HLA-DRB1*1101 allele was associated (P<.01) with sarcoidosis in blacks and whites and had a population attributable risk of 16% in blacks and 9% in whites. HLA-DRB1-F(47) was the amino acid residue most associated with sarcoidosis and independently associated with sarcoidosis in whites. The HLA-DPB1 locus also contributed to susceptibility for sarcoidosis and, in contrast to chronic beryllium disease, a non-E(69)-containing allele, HLA-DPB1*0101, conveyed most of the risk. Although significant differences were observed in the distribution of HLA class II alleles between blacks and whites, only HLA-DRB1*1501 was differentially associated with sarcoidosis (P<.003). In addition to being susceptibility markers, HLA class II alleles may be markers for different phenotypes of sarcoidosis (DRB1*0401 for eye in blacks and whites, DRB3 for bone marrow in blacks, and DPB1*0101 for hypercalcemia in whites). These studies confirm a genetic predisposition for sarcoidosis and present evidence for the allelic variation at the HLA-DRB1 locus as a major contributor.

  14. Association of sarcoidosis with endothelial function, arterial wall properties, and biomarkers of inflammation.

    PubMed

    Siasos, Gerasimos; Tousoulis, Dimitris; Gialafos, Elias; Oikonomou, Evangelos; Zaromitidou, Marina; Aggeli, Constantina; Korompelis, Porfyrios; Kallianos, Anastasios; Rapti, Aggeliki; Zisimos, Konstantinos; Marinos, Georgios; Stefanadis, Christodoulos; Papavassiliou, Athanasios G

    2011-06-01

    Sarcoidosis is an inflammatory disease, which may affect vascular function. The study was designed to assess the impact of sarcoidosis on endothelial function and arterial stiffness. Eighty-seven sarcoidosis patients and eighty-seven matched healthy subjects (Cl) were included in the study. Sarcoidosis patients were divided into two groups. Group 1 included patients never treated and group 2 included patients receiving cortisone treatment. Endothelial function was evaluated by flow-mediated dilatation (FMD). Carotid-femoral pulse wave velocity (PWV) was measured as an index of aortic stiffness and augmentation index (AI75) as a measure of arterial wave reflections. Serum levels of soluble intercellular adhesion molecule-1 and tumor necrosis factor-α (TNF-α), were measured. In the totality of the population, sarcoidosis patients had significantly lower FMD (P < 0.01) and significantly higher AI75 (P < 0.05). There was also a significant difference, between group 1, and Cl in FMD and AI75, but there was no difference between group 2 and Cl in FMD and AI75. AI75 values were significantly correlated with serum levels of intercellular adhesion molecule-1 (ICAM-1) (r = 0.370, P < 0.01) and TNF-α (r = 0.219, P = 0.049). In the present study, we have shown that sarcoidosis patients have impaired endothelial function and increased arterial stiffness. Sarcoidosis patients on cortisone treatment had no differences compared to controls on the vascular parameters. Moreover, there was a significant correlation between inflammatory process and vascular function impairment. These findings indicate that sarcoidosis patients have impaired vascular function and increased inflammatory status, which may improve with cortisone treatment.

  15. Cardiac Sarcoidosis: The Impact of Age and Implanted Devices on Survival.

    PubMed

    Zhou, Ying; Lower, Elyse E; Li, Hui-Ping; Costea, Alexandru; Attari, Mehran; Baughman, Robert P

    2017-01-01

    To assess the clinical characteristics, diagnosis, and outcome of cardiac sarcoidosis in a single institution sarcoidosis clinic. Patients with cardiac sarcoidosis were identified using refined World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) criteria of highly probable and probable. Patient demographics, local and systemic treatments, and clinical outcome were collected. Of the 1,815 patients evaluated over a 6-year period, 73 patients met the WASOG criteria for cardiac sarcoidosis. The median age at diagnosis was 46 years, with a median follow-up of 8.8 years. Reduced left ventricular ejection fraction (LVEF) was the most common manifestation (54.8%). Patients with arrhythmias experienced ventricular tachycardia or severe heart block, (35.6% and 19.2%, respectively) with or without reduced LVEF. A total of 45 (61.6%) patients underwent cardiac PET scan and/or MRI, with 41 (91.1%) having a positive study. During follow-up, 10 patients (13.7%) either underwent transplant (n = 3) or died (n = 7) from sarcoidosis. Kaplan-Meier survival curves revealed 5- and 10-year survival rates of 95.5% and 93.4%, respectively. Univariate factors of age at diagnosis < 46 years, implantation of pacemaker or defibrillator, mycophenolate treatment, or LVEF > 40% were associated with improved survival. Cox regression analysis demonstrated that age ≥ 46 years and lack of an implanted pacemaker or defibrillator were the only independent predictors of mortality. The new WASOG criteria were able to characterize cardiac involvement in our sarcoidosis clinic. Age and lack of pacemaker or defibrillator were the significant predictors of mortality for cardiac sarcoidosis, and reduced LVEF < 40% was associated with worse prognosis. ClinicalTrials.gov; No.: NCT02356445; URL: www.clinicaltrials.gov. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  16. SPECT imaging with Tl-201 and Ga-67 in myocardial sarcoidosis

    SciTech Connect

    Kurata, C.; Sakata, K.; Taguchi, T.; Fukumoto, Y.; Miyata, H.; Aoshima, S.; Yamazaki, N. )

    1990-06-01

    Two patients with myocardial sarcoidosis are presented, both of whom underwent SPECT imaging with Tl-201 and Ga-67. The first had Ga-67 myocardial uptake with a Tl-201 defect, which disappeared with corticosteroid therapy. The second had multiple Tl-201 defects without Ga-67 uptake, which persisted despite corticosteroid therapy. Therefore, the combination of Tl-201 and Ga-67 imaging may be useful for recognizing myocardial sarcoidosis and for predicting the response to corticosteroid therapy.

  17. Cardiac sarcoidosis demonstrated by Tl-201 and Ga-67 SPECT imaging

    SciTech Connect

    Taki, J.; Nakajima, K.; Bunko, H.; Ohguchi, M.; Tonami, N.; Hisada, K. )

    1990-09-01

    Ga-67 and Tl-201 SPECT was performed to evaluate cardiac sarcoidosis in a 15-year-old boy. Tl-201 SPECT imaging showed decreased uptake in the inferior to lateral wall and Ga-67 accumulation in the area of decreased Tl-201 uptake. These findings suggested cardiac sarcoidosis, and cardiac biopsy confirmed this diagnosis. After corticosteroid therapy, myocardial uptake of Ga-67 disappeared and myocardial TI-201 uptake became more homogeneous.

  18. Isolated oculocutaneous sarcoidosis in a teenage male: a rare case report.

    PubMed

    Aggarwal, H K; Jain, D; Mittal, A; Dabas, G

    2016-09-09

    Sarcoidosis is an inflammatory disease with multisystem involvement characterized by the presence of noncaseating granulomas. It can affect virtually every organ of the body, with lung involvement being most common occurring in >90% of patients. Other organs affected are skin, eye and liver. Skin involvement is common, affecting 25-35%. Here we present a rare case of a 15 year-old male with isolated oculocutaneous sarcoidosis without systemic involvement.

  19. Obesity and Weight Gain in Relation to Incidence of Sarcoidosis in US Black Women

    PubMed Central

    Coogan, Patricia F.; Govender, Praveen; Berman, Jeffrey S.; Palmer, Julie R.; Rosenberg, Lynn

    2015-01-01

    BACKGROUND: Sarcoidosis, a systemic disorder characterized by chronic granulomatous inflammation, occurs more frequently among US black women, as do overweight and obesity. Little is known about the relation of overweight and obesity, which induce chronic inflammation, to incidence of sarcoidosis. METHODS: We assessed the relation of obesity and weight gain to the incidence of sarcoidosis in the Black Women’s Health Study, a follow-up study of 59,000 US black women aged 21 to 69 years at baseline in 1995. Information on weight at age 18 years, height, current weight, incident sarcoidosis, and covariates was collected at baseline and on biennial follow-up questionnaires. Cox regression models adjusted for age, education, geographic region, smoking, alcohol consumption, and physical activity were used to estimate incidence rate ratios (IRRs) and 95% CIs. RESULTS: From 1995 through 2011, 454 incident cases of sarcoidosis occurred during 707,557 person-years of follow-up. The incidence of sarcoidosis increased with increasing BMI and weight gain. The IRR was 1.40 (95% CI, 0.88-2.25) for BMI ≥ 30 kg/m2 at age 18 years relative to 20 to 24 kg/m2 (P trend = .18), 1.42 (95% CI, 1.07-1.89) for BMI ≥ 35 kg/m2 at baseline relative to 20 to 24 kg/m2 (P trend = .01), and 1.47 (95% CI, 1.10-1.97) for a weight gain between age 18 years and baseline of ≥ 30 kg relative to 0 to 9 kg (P trend = .16). In stratified analyses, there were significant trends of sarcoidosis incidence with increasing BMI and weight gain in women aged ≥ 45 years and ever smokers. CONCLUSIONS: The present study provides evidence that weight gain and obesity during adulthood are associated with increased sarcoidosis incidence. PMID:25411869

  20. Insertion/Deletion Polymorphisms and Serum Angiotensin-converting Enzyme Levels in Iranian Patients with Sarcoidosis

    PubMed Central

    JAVADI, Alireza; SHAMAEI, Masoud; ZAREI, Masoud; REZAEIAN, Lida; KIANI, Arda; ABEDINI, Atefeh

    2016-01-01

    Background: Sarcoidosis is a multisystem inflammatory disease of unknown origin with characterization of small granulomas. Angiotensin-converting enzyme (ACE) is a pathophysiologic marker of sarcoidosis. We present the ACE insertion/deletion (I/D) polymorphism in correlation with serum ACE level in Iranian patients with sarcoidosis. Methods: From Jan 2014 to Jan 2015, 102 Iranian patients who histopathologically diagnosed for sarcoidosis and 192 healthy age and sex-matched controls were recruited. PCR was used for detection of I/D polymorphism in ACE gene. Results: Frequency of II/ID/DD genotype in sarcoidosis disease was 17%, 35.5%, and 47.1%, respectively. The frequency of D allele was 0.65. A significant association between I/D genotypes and mean of sACE level was seen (DD=85.2±22.9, P<0.001). More frequent genotype in sarcoidosis patients was DD (47%), ID genotype (45.9%) was found more in controls. Logistic regression analysis adjusting age and sex showed that ID to II (OR=0.35, 95%CI=0.17–0.73, P=0.005) and DD to II (OR=2.11, 95%CI=0.98–4.54, P=0.05) could be considered as a predictor factor for the disease activity. No significant model for men in sarcoidosis group was seen, while women with II/ID were associated with a reduced risk for the disease. Conclusion: Although more regional studies with appropriate statistical scale must be done to provide a better diagnosis and prognostic tool for this disease, this study demonstrates that ID and DD genotype could be predictive factors for sarcoidosis. PMID:28032065

  1. Nivolumab-Induced Development of Pulmonary Sarcoidosis in Renal Cell Carcinoma.

    PubMed

    Zhang, Meng; Schembri, Geoffrey

    2017-09-01

    A 64-year-old woman with metastatic clear cell renal carcinoma who had been on nivolumab immunotherapy for 10 months was referred for a FDG PET scan to monitor disease progress. New bilateral mediastinal and hilar FDG-avid lymphadenopathy was noted. Pathology on subsequent mediastinal nodal biopsy showed well-formed epithelioid granulomas with no evidence of malignancy consistent with sarcoidosis. This case illustrates that pulmonary sarcoidosis can be induced by immunotherapy in the treatment of renal cell carcinoma.

  2. Transcriptional Blood Signatures Distinguish Pulmonary Tuberculosis, Pulmonary Sarcoidosis, Pneumonias and Lung Cancers

    PubMed Central

    Bloom, Chloe I.; Graham, Christine M.; Berry, Matthew P. R.; Rozakeas, Fotini; Redford, Paul S.; Wang, Yuanyuan; Xu, Zhaohui; Wilkinson, Katalin A.; Wilkinson, Robert J.; Kendrick, Yvonne; Devouassoux, Gilles; Ferry, Tristan; Miyara, Makoto; Bouvry, Diane; Dominique, Valeyre; Gorochov, Guy; Blankenship, Derek; Saadatian, Mitra; Vanhems, Phillip; Beynon, Huw; Vancheeswaran, Rama; Wickremasinghe, Melissa; Chaussabel, Damien; Banchereau, Jacques; Pascual, Virginia; Ho, Ling-pei; Lipman, Marc; O’Garra, Anne

    2013-01-01

    Rationale New approaches to define factors underlying the immunopathogenesis of pulmonary diseases including sarcoidosis and tuberculosis are needed to develop new treatments and biomarkers. Comparing the blood transcriptional response of tuberculosis to other similar pulmonary diseases will advance knowledge of disease pathways and help distinguish diseases with similar clinical presentations. Objectives To determine the factors underlying the immunopathogenesis of the granulomatous diseases, sarcoidosis and tuberculosis, by comparing the blood transcriptional responses in these and other pulmonary diseases. Methods We compared whole blood genome-wide transcriptional profiles in pulmonary sarcoidosis, pulmonary tuberculosis, to community acquired pneumonia and primary lung cancer and healthy controls, before and after treatment, and in purified leucocyte populations. Measurements and Main Results An Interferon-inducible neutrophil-driven blood transcriptional signature was present in both sarcoidosis and tuberculosis, with a higher abundance and expression in tuberculosis. Heterogeneity of the sarcoidosis signature correlated significantly with disease activity. Transcriptional profiles in pneumonia and lung cancer revealed an over-abundance of inflammatory transcripts. After successful treatment the transcriptional activity in tuberculosis and pneumonia patients was significantly reduced. However the glucocorticoid-responsive sarcoidosis patients showed a significant increase in transcriptional activity. 144-blood transcripts were able to distinguish tuberculosis from other lung diseases and controls. Conclusions Tuberculosis and sarcoidosis revealed similar blood transcriptional profiles, dominated by interferon-inducible transcripts, while pneumonia and lung cancer showed distinct signatures, dominated by inflammatory genes. There were also significant differences between tuberculosis and sarcoidosis in the degree of their transcriptional activity, the

  3. Clinical Characteristics and Outcome of Hepatic Sarcoidosis: A Population-Based Study 1976-2013.

    PubMed

    Ungprasert, Patompong; Crowson, Cynthia S; Simonetto, Douglas A; Matteson, Eric L

    2017-10-01

    Data on clinical manifestations and outcome of hepatic sarcoidosis are scarce. This study aimed to use a population-based cohort of patients with incident sarcoidosis to better describe the characteristics of hepatic sarcoidosis. A cohort of incident cases of sarcoidosis in Olmsted County, MN, USA, from 1976 to 2013 was identified from the database. Diagnosis was verified by individual medical record review. Confirmed cases of sarcoidosis were then reviewed for liver involvement. Data on clinical manifestations, imaging study, liver biochemical tests, treatment, and outcome were collected. Cumulative incidence of cirrhosis adjusted for the competing risk of death was estimated. A total of 345 cases of incident sarcoidosis were identified. Of these, 19 cases (6%) had liver involvement (mean age 46.1 years, 53% female and 79% Caucasian). Most patients had asymptomatic liver disease and were discovered in pursuit of abnormal biochemical tests and imaging studies. Alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) were elevated in the majority of patients (88 and 90%, respectively). Elevated transaminases were less common and less severe. About half of patients had abnormal imaging study with hypodense nodular lesions being the most common abnormality (six patients) followed by hepatomegaly (three patients). Liver biopsy revealed non-caseating granuloma in 88% (14 of 16 patients). A total of four patients developed cirrhosis. Involvement of the liver by sarcoidosis was seen in 6% of patients with sarcoidosis. The majority of patients were asymptomatic. Elevated ALP and GGT were the most common abnormal biochemical tests. Liver biopsy revealed non-caseating granuloma in almost all cases. Cirrhosis was seen in a significant number of patients. Generalizability of the observations to other populations may be limited, as the studied population was predominantly Caucasian. The prevalence of liver disease may be higher in more diverse populations.

  4. Identification of HLA-DR–bound peptides presented by human bronchoalveolar lavage cells in sarcoidosis

    PubMed Central

    Wahlström, Jan; Dengjel, Jörn; Persson, Bengt; Duyar, Hüseyin; Rammensee, Hans-Georg; Stevanoviδc, Stefan; Eklund, Anders; Weissert, Robert; Grunewald, Johan

    2007-01-01

    Sarcoidosis is an inflammatory disease of unknown etiology, most commonly affecting the lungs. Activated CD4+ T cells accumulate in the lungs of individuals with sarcoidosis and are considered to be of central importance for inflammation. We have previously shown that Scandinavian sarcoidosis patients expressing the HLA-DR allele DRB1*0301 are characterized by large accumulations in the lungs of CD4+ T cells expressing the TCR AV2S3 gene segment. This association afforded us a unique opportunity to identify a sarcoidosis-specific antigen recognized by AV2S3+ T cells. To identify candidates for the postulated sarcoidosis-specific antigen, lung cells from 16 HLA-DRB1*0301pos patients were obtained by bronchoalveolar lavage. HLA-DR molecules were affinity purified and bound peptides acid eluted. Subsequently, peptides were separated by reversed-phase HPLC and analyzed by liquid chromatography–mass spectrometry. We identified 78 amino acid sequences from self proteins presented in the lungs of sarcoidosis patients, some of which were well-known autoantigens such as vimentin and ATP synthase. For the first time, to our knowledge, we have identified HLA-bound peptides presented in vivo during an inflammatory condition. This approach can be extended to characterize HLA-bound peptides in various autoimmune settings. PMID:17975675

  5. [Sarcoidosis of the skeleton. Review of the literature and case report. (author's transl)].

    PubMed

    Uehlinger, E; Wurm, K

    1976-08-01

    The frequency of sarcoidosis in the skeleton varies between 3 and 36%. Skeletal sarcoidosis is rare in early stages (Löfgren-syndrom), relatively frequent in late stages. The initial phase is characterized by the formation of miliary non-caseating epitheloid-cell granulomas in the bone marrow. The invasion of the bone marrow may either be tolerated by the bone tissue or it initiates a perifocal osteosclerosis or a osteolysis. Correspondingly the X-ray of the skeleton shows normal structure or focal osteosclerosis or osteolysis. Therefore in the first case the sarcoidosis cannot be identified by X-ray. Most frequent locations are the phalanges of the fingers and toes, less common the stem skelton (skull, vertebrae, pelvis) and very rare the long tubular bones. In most cases the skeletal sarcoidosis is well tolerated. Report of a case of osteosclerotic sarcoidosis of the pelvis of a 39-years old woman with generalized sarcoidosis which was diagnozed four years earlier. The X-rays of the phalanges were normal. The biopsy of the iliac crest shows miliary sarcoid granuloma of the bone marrow and accretion of lamellar bone on the surface of the bone trabeculi with a distinct mosaic pattern. Treatment with steroids during the following five years was ineffective.

  6. Vitreous lavage fluid and bronchoalveolar lavage fluid have equal diagnostic value in sarcoidosis.

    PubMed

    Maruyama, Kazuichi; Inaba, Tohru; Tamada, Tsutomu; Nakazawa, Toru

    2016-12-01

    Here, we elucidate the immunological features of both bronchoalveolar lavage fluid (BALF) and vitreous lavage fluid (VLF) samples from patients with histopathologically verified sarcoidosis. In addition, we assess the safety of vitrectomy in sarcoidosis patients by investigating the occurrence of complications and the recovery of visual acuity.Twenty-two eyes of 22 patients with tissue-proven sarcoidosis were enrolled in this study. BALF and VLF samples were obtained and compared in each patient, and the clinical course (including visual acuity) was followed. The presence of sarcoidosis was assessed with a flow cytometric analysis of T-lymphocytes in the BALF and VLF samples.Our results indicated that the CD4 T-cell population and the CD4/CD8 ratio were significantly higher in the VLF T-lymphocytes than the BALF T-lymphocytes. On the other hand, the CD8+ T-cell population was significantly lower in the VLF T-lymphocytes.Therefore, our findings suggest that VLF samples have a high diagnostic value (equal to that of BALF samples) for sarcoidosis. Moreover, we found that the sample collection did not affect visual acuity and that there were no adverse events after surgery. A flow cytometric analysis of a VLF sample may therefore be a useful adjunct in the diagnosis of sarcoidosis.

  7. Infliximab for chronic cutaneous sarcoidosis: a subset analysis from a double-blind randomized clinical trial.

    PubMed

    Baughman, Robert P; Judson, Marc A; Lower, Elyse E; Drent, Marjolein; Costabel, Ulrich; Flavin, Susan; Lo, Kim Hung; Barnathan, Elliot S

    2016-01-15

    Limited evidence exists demonstrating an effective treatment for chronic cutaneous sarcoidosis. To determine infliximab's effectiveness in sarcoidosis. We conducted a subset analysis from a randomized, double-blind, placebo-controlled trial for chronic pulmonary sarcoidosis to determine infliximab's effectiveness. Patients with chronic cutaneous sarcoidosis received infliximab (3 or 5 mg/kg) or placebo over 24 weeks. Of 138 patients, the subset analysis evaluated 17 patients with chronic facial and another 9 patients with nonfacial skin involvement. The SASI evaluated lesions for degree of erythema, desquamation, induration, and percentage of area involved. Facial and nonfacial lesions were scored in a blinded manner. Among 5 placebo-treated and 12 infliximab-treated patients, an improvement was observed with infliximab versus placebo in change from baseline to weeks 12 and 24 in desquamation (P<0.005) and induration (P<0.01) at week 24. Erythema, percentage of area involved and the evaluation of paired photographs did not reveal significant differences. Sample size; more extensive disease in placebo patients; chronic therapy upon enrollment; lung as primary organ of sarcoidosis involvement; limited investigator experience with SASI. Infliximab appears to be a beneficial treatment for chronic cutaneous sarcoidosis. The SASI scoring system demonstrated significant improvement versus placebo in lesion desquamation and induration.

  8. Sarcoidosis and squamous cell carcinoma: a connection documented in a case series of 3 patients.

    PubMed

    Berg, Sara A; Novoa, Roberto A; Stewart, Campbell; Sobanko, Joseph F; Miller, Christopher J; Rosenbach, Misha

    2016-12-01

    Sarcoidosis is a chronic multisystem disease characterized by the formation of noncaseating granulomas in multiple organs, including the skin. An association between multisystem sarcoidosis and an increased risk for malignancy has been established. Dermatologists should be aware of the increased risk for nonmelanoma skin cancers in patients with sarcoidosis. We report a series of 3 patients with primarily cutaneous sarcoidosis who presented with new-onset cutaneous squamous cell carcinoma (SCC). Two patients were black women and 1 patient presented with lesions of cutaneous sarcoidosis arising concurrently with SCCs in the same location, distinguishable only by biopsy. These cases highlight the association between sarcoidosis and an increased risk for SCC. Because dermatologists may be the primary clinicians caring for these patients, it is important that they remain aware of the increased risk for cutaneous malignancies and that they have a low threshold for biopsy of new and unusual skin lesions. Furthermore, 2 patients were black women, a population not commonly affected by skin cancer, which further exemplifies the need for comprehensive skin examinations in black patients. Although the precise mechanism for an increased risk for malignancy in these patients requires further investigation, chronic inflammation and immune dysregulation may play a role.

  9. Sarcoidosis diagnosed after September 11, 2001, among adults exposed to the World Trade Center disaster.

    PubMed

    Jordan, Hannah T; Stellman, Steven D; Prezant, David; Teirstein, Alvin; Osahan, Sukhminder S; Cone, James E

    2011-09-01

    Explore relationships between World Trade Center (WTC) exposures and sarcoidosis. Sarcoidosis has been reported after exposure to the WTC disaster. We ascertained biopsy-proven post-9/11 sarcoidosis among WTC Health Registry enrollees. Cases diagnosed after Registry enrollment were included in a nested case-control study. Controls were matched to cases on age, sex, race or ethnicity, and eligibility group (eg, rescue or recovery worker). We identified 43 cases of post-9/11 sarcoidosis. Twenty-eight incident cases and 109 controls were included in the case-control analysis. Working on the WTC debris pile was associated with sarcoidosis (odds ratio 9.1, 95% confidence interval 1.1 to 74.0), but WTC dust cloud exposure was not (odds ratio 1.0, 95% confidence interval 0.4 to 2.8). Working on the WTC debris pile was associated with an elevated risk of post-9/11 sarcoidosis. Occupationally exposed workers may be at increased risk. (C)2011The American College of Occupational and Environmental Medicine

  10. Vitreous lavage fluid and bronchoalveolar lavage fluid have equal diagnostic value in sarcoidosis

    PubMed Central

    Maruyama, Kazuichi; Inaba, Tohru; Tamada, Tsutomu; Nakazawa, Toru

    2016-01-01

    Abstract Here, we elucidate the immunological features of both bronchoalveolar lavage fluid (BALF) and vitreous lavage fluid (VLF) samples from patients with histopathologically verified sarcoidosis. In addition, we assess the safety of vitrectomy in sarcoidosis patients by investigating the occurrence of complications and the recovery of visual acuity. Twenty-two eyes of 22 patients with tissue-proven sarcoidosis were enrolled in this study. BALF and VLF samples were obtained and compared in each patient, and the clinical course (including visual acuity) was followed. The presence of sarcoidosis was assessed with a flow cytometric analysis of T-lymphocytes in the BALF and VLF samples. Our results indicated that the CD4 T-cell population and the CD4/CD8 ratio were significantly higher in the VLF T-lymphocytes than the BALF T-lymphocytes. On the other hand, the CD8+ T-cell population was significantly lower in the VLF T-lymphocytes. Therefore, our findings suggest that VLF samples have a high diagnostic value (equal to that of BALF samples) for sarcoidosis. Moreover, we found that the sample collection did not affect visual acuity and that there were no adverse events after surgery. A flow cytometric analysis of a VLF sample may therefore be a useful adjunct in the diagnosis of sarcoidosis. PMID:27930546

  11. Reduced expression of monocyte CD200R is associated with enhanced proinflammatory cytokine production in sarcoidosis

    PubMed Central

    Fraser, Simon D.; Sadofsky, Laura R.; Kaye, Paul M.; Hart, Simon P.

    2016-01-01

    In sarcoidosis, the proinflammatory cytokines interferon gamma, tumour necrosis factor and interleukin-6 are released by monocyte-derived macrophages and lymphocytes in the lungs and other affected tissues. Regulatory receptors expressed on monocytes and macrophages act to suppress cytokine production, and reduced expression of regulatory receptors may thus promote tissue inflammation. The aim of this study was to characterise the role of regulatory receptors on blood monocytes in patients with sarcoidosis. Cytokine release in response to stimulation of whole blood was measured in healthy controls and Caucasian non-smoking patients with sarcoidosis who were not taking disease modifying therapy. Expression of the regulatory molecules IL-10R, SIRP-α/β, CD47, CD200R, and CD200L was measured by flow cytometry, and functional activity was assessed using blocking antibodies. Stimulated whole blood and monocytes from patients with sarcoidosis produced more TNF and IL-6 compared with healthy controls. 52.9% of sarcoidosis patients had monocytes characterised by low expression of CD200R, compared with 11.7% of controls (p < 0.0001). Patients with low monocyte CD200R expression produced higher levels of proinflammatory cytokines. In functional studies, blocking the CD200 axis increased production of TNF and IL-6. Reduced expression of CD200R on monocytes may be a mechanism contributing to monocyte and macrophage hyper-activation in sarcoidosis. PMID:27929051

  12. Polycystic Liver Disease and Sarcoidosis: Unusual Coexisting Etiologies of Portal Hypertension

    PubMed Central

    Jagroop, Sophia; Parthvi, Rukma

    2017-01-01

    Both polycystic liver disease (PLD) and sarcoidosis can involve liver. Most of the time, liver disease in both conditions is asymptomatic, but they can rarely cause portal hypertension. Our aim is to report a case of a 51-year-old female with a history of adult dominant polycystic kidney disease (ADPKD) and sarcoidosis who presented with multiple episodes of hematemesis. An endoscopy showed grade 3 esophageal varices. A computed tomography (CT) scan of the abdomen showed ascites with polycystic liver, nodular contour, and calcified granuloma. PLD can cause portal hypertension due to fibrosis or large cysts compressing on the portal vein. On the other hand, sarcoidosis causes portal hypertension by formation of arteriovenous(AV) shunts or fibrosis in areas of granulomas. Both conditions are diagnosed on imaging. There is no approved medical treatment for PLD; the only curative treatment is liver transplantation. Asymptomatic hepatic sarcoidosis does not need any treatment. The recommended treatment is corticosteroids for both isolated and systemic sarcoidosis. ADPKD and sarcoidosis can involve multiple organs. The presence of both conditions can accelerate the disease process and could be a therapeutic challenge. Early abdominal imaging during the course of both diseases can improve the outcome by decreasing the diagnostic window. PMID:28280650

  13. Scar sarcoidosis on a finger mimicking a rapidly growing soft tissue tumour: a case report

    PubMed Central

    2012-01-01

    Background Scar sarcoidosis is a rare and uncommon but specific cutaneous manifestation of sarcoidosis. In general it arises in pre-existing scars deriving from mechanical traumas. As most surgeons dealing with scars might not be aware of cutaneous sarcoidosis and its different types of appearance the appropriate staging and treatment might be missed or at least delayed. To our knowledge this is the first case in literature of scar sarcoidosis on a finger. Case presentation We present a case of a 33-year-old carpenter who developed scar sarcoidosis on his right index finger 4 years after the tendon of the long digital flexor got accidentally cut by an angle grinder. He was referred due to a swelling of the finger suspected to be a malignant soft tissue tumour. The circumference of the affected finger had almost doubled, adding up to 94 mm. Incision biopsy revealed typical noncaseating granulomas. Further investigation showed a systemic extent of the disease with involvement of the lung. A systemic treatment with oral steroids led to an almost full regression of the swelling with restoration of function and resolution of lung infiltrates. Conclusion In case of a suspicious and/or progressive swelling a definite diagnosis should be achieved by biopsy within a short time to enable a proper treatment. If scar sarcoidosis is proven further investigation is necessary to exclude a systemical involvement. A surgical treatment of the swelling is not indicated. PMID:23031186

  14. Meta-analytical association between angiotensin-converting enzyme gene polymorphisms and sarcoidosis risk.

    PubMed

    Zhu, R; Bi, L Q; Kong, H; Tilley, S L; Wang, H; Xie, W P

    2015-04-17

    Previous reports identified an association between sarcoidosis and an insertion/deletion (I/D) polymorphism in angiotensin-converting enzyme. Our meta-analysis of articles published between March 1996 and June 2013 identified studies in the PubMed, EMBASE, and the China National Knowledge Infrastructure databases. We examined whether angiotensin-converting enzyme polymorphisms influence sarcoidosis susceptibility. The strength of the association between I/D polymorphisms and sarcoidosis risk was measured based on the odds ratio and 95% confidence interval. Analysis was based on recessive and dominant models. Ethnic subgroup analysis from 18 articles (1882 cases and 3066 controls) showed that DD homozygote carriers were at a slightly increased risk of sarcoidosis compared with II homozygotes and DI heterozygotes (P = 0.03). Comparison of DD plus DI vs II revealed no significant association with sarcoidosis in group and ethnic subgroup analysis. We found that the I/D polymorphism in the angiotensin-converting enzyme gene was not associated with a major risk of sarcoidosis.

  15. Differential Expression of MicroRNA and Predicted Targets in Pulmonary Sarcoidosis

    PubMed Central

    Crouser, Elliott D.; Julian, Mark W.; Crawford, Melissa; Shao, Guohong; Yu, Lianbo; Planck, Stephen R.; Rosenbaum, James T.; Nana-Sinkam, S. Patrick

    2014-01-01

    Background Recent studies show that various inflammatory diseases are regulated at the level of RNA translation by small non-coding RNAs, termed microRNAs (miRNAs). We sought to determine whether sarcoidosis tissues harbor a distinct pattern of miRNA expression and then considered their potential molecular targets. Methods and Results Genome-wide microarray analysis of miRNA expression in lung tissue and peripheral blood mononuclear cells (PBMCs) was performed and differentially expressed (DE)-miRNAs were then validated by real-time PCR. A distinct pattern of DE-miRNA expression was identified in both lung tissue and PBMCs of sarcoidosis patients. A subgroup of DE-miRNAs common to lung and lymph node tissues were predicted to target transforming growth factor (TGFβ)-regulated pathways. Likewise, the DE-miRNAs identified in PBMCs of sarcoidosis patients were predicted to target the TGFβ-regulated “wingless and integrase-1” (WNT) pathway. Conclusions This study is the first to profile miRNAs in sarcoidosis tissues and to consider their possible roles in disease pathogenesis. Our results suggest that miRNA regulate TGFβ and related WNT pathways in sarcoidosis tissues, pathways previously incriminated in the pathogenesis of sarcoidosis. PMID:22209793

  16. [Cryptococcal meningitis in a patient with a ventriculoperitoneal shunt and monitoring for pulmonary sarcoidosis].

    PubMed

    Baallal, H; El Asri, A C; Eljebbouri, B; Akhaddar, A; Gazzaz, M; El Mostarchid, B; Boucetta, M

    2013-02-01

    The fungus Cryptococcus neoformans can cause common opportunistic infection in acquired immune deficiency syndrome (AIDS) patients. But other conditions can be associated with sarcoidosis. Meningoencephalitis is the most common manifestation of this disease. One of the most important neurological complications is the development of intracranial hypertension (ICH), which may result in high morbidity and mortality. We report the case of a patient harboring a ventriculoperitoneal shunt, and having contracted a cryptococcal meningitis as a risk factor for pulmonary sarcoidosis. Brain MRI showed arachnoiditis, with a mass in contact with the right frontal horn. Indian ink staining of the cerebrospinal fluid (CSF) showed positivity that was confirmed by the identification of Cryptococcus neoformans after culture. The evolution was favorable under medical treatment with removal of material. The relationship between sarcoidosis and cryptococcosis, described in the literature is not coincidental but is a rare complication of sarcoidosis of potential severity (40% of mortality). Sarcoidosis is a common systemic disease that may increase host susceptibility to CNS cryptococcal infection without any other signs or symptoms of host immunosuppression. The diagnosis of cryptococcosis should be evoked as a differential diagnosis of neuro-sarcoidosis. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  17. Scar sarcoidosis on a finger mimicking a rapidly growing soft tissue tumour: a case report.

    PubMed

    Henrichs, Marcel-Philipp; Streitbürger, Arne; Gosheger, Georg; Surke, Carsten; Dierkes, Christian; Hardes, Jendrik

    2012-10-02

    Scar sarcoidosis is a rare and uncommon but specific cutaneous manifestation of sarcoidosis. In general it arises in pre-existing scars deriving from mechanical traumas. As most surgeons dealing with scars might not be aware of cutaneous sarcoidosis and its different types of appearance the appropriate staging and treatment might be missed or at least delayed. To our knowledge this is the first case in literature of scar sarcoidosis on a finger. We present a case of a 33-year-old carpenter who developed scar sarcoidosis on his right index finger 4 years after the tendon of the long digital flexor got accidentally cut by an angle grinder. He was referred due to a swelling of the finger suspected to be a malignant soft tissue tumour. The circumference of the affected finger had almost doubled, adding up to 94 mm. Incision biopsy revealed typical noncaseating granulomas. Further investigation showed a systemic extent of the disease with involvement of the lung. A systemic treatment with oral steroids led to an almost full regression of the swelling with restoration of function and resolution of lung infiltrates. In case of a suspicious and/or progressive swelling a definite diagnosis should be achieved by biopsy within a short time to enable a proper treatment. If scar sarcoidosis is proven further investigation is necessary to exclude a systemical involvement. A surgical treatment of the swelling is not indicated.

  18. Acute renal failure as a form of presentation of sarcoidosis in a young adult: a case report

    PubMed Central

    2014-01-01

    Introduction Sarcoidosis is a systemic granulomatous disease. Renal involvement is a rare initial presentation of this disease. Few articles on renal involvement as an initial presentation of sarcoidosis have been published in the literature. Case presentation A 26-year-old Caucasian woman presented with acute renal failure as an initial manifestation of sarcoidosis. Conclusions Renal involvement is an uncommon feature of sarcoidosis and it is essential to establish a fast and correct diagnosis because early therapy avoids progression to terminal renal failure. PMID:25124289

  19. Sarcoidosis and Histoplasmosis: Is One a Consequence of the Other? A Case Report and Review of the Literature

    PubMed Central

    Bansal, Anupam; Drewek, Rupali

    2015-01-01

    Sarcoidosis involves abnormal collections of inflammatory cells (granulomas) which may form as nodules in multiple organs. 90% of affected patients have respiratory tract abnormalities. We present a 61-year-old male with sarcoidosis who was admitted for respiratory distress. Fibrosing mediastinitis was seen in the chest computograph. Management was conservative and included steroids, antibiotics, and oxygen therapy. Sarcoidosis and fibrosing mediastinitis are rare. Fibrosing mediastinitis is more commonly seen with histoplasmosis. We explore the clinical similarities between histoplasmosis and sarcoidosis. We also explore the potential cause and effect relationship and workup for each disease entity. PMID:26345350

  20. Corticosteroid Responsive Sarcoidosis with Multisystemic Involvement Years after Initial Diagnosis: A Lymphoma Mimicker on 18-FDG PET/CT

    PubMed Central

    Acar, Turker; Savas, Recep; Kocacelebi, Kenan; Ucan, Eyup Sabri

    2015-01-01

    Sarcoidosis is a chronic multisystemic inflammatory disease characterized by noncaseating epithelioid cell granulomas. 18-Fluorodeoxyglucose positron-emission tomography/computer tomography (FDG-PET/CT) is increasingly used in routine clinical practice to assess active sarcoidosis because it can detect active inflammatory granulomatous disease. However, active sarcoidosis lesions are observed to be hypermetabolic on FDG-PET/CT much like malignancies, which may lead to misinterpretation on imaging. In this case report, we present a rare case of sarcoidosis with multisystem involvement including lung, lymph nodes, bone, pleura, and soft tissue that mimicked lymphoma on FDG-PET/CT and responded to corticosteroid treatment. PMID:26312138

  1. Ocular medications in children.

    PubMed

    Wallace, D K; Steinkuller, P G

    1998-11-01

    Many ocular medications are used by pediatricians or ophthalmologists caring for pediatric patients. Topical antibiotics are commonly prescribed for bacterial conjunctivitis, nasolacrimal duct obstructions, and ophthalmia neonatorum. Many new antiallergy eye drops are now available for the treatment of seasonal (hay fever) conjunctivitis. Dilating eye drops and antiglaucoma medications are generally used or prescribed by ophthalmologists, but pediatricians must be aware of their potentially serious systemic side effects. Before initiating treatment, physicians should evaluate the risks and benefits of ophthalmic medications, establish minimum dosages necessary to achieve a therapeutic benefit, and monitor children for local and systemic side effects.

  2. [Ocular biometry using Orbscan].

    PubMed

    Touzeau, O; Allouch, C; Borderie, V; Laroche, L

    2005-06-01

    Orbscan is a recent optical device that combines the Placido disk of the videokeratoscope and a scanning slit. The scanning slit measures the elevation of both the corneal surface (anterior and posterior) and the anterior iris-lens surface. Biometric measures of the anterior segment such as corneal thickness, anterior chamber depth, corneal diameter, and iridocorneal angle are obtained using spatial coordinates of various ocular surfaces. Orbscan is not only a corneal topograph but a versatile device capable of measuring the biometry of the anterior segment of the eye.

  3. Ocular surface tumors

    PubMed Central

    Othman, Ihab Saad

    2009-01-01

    Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, mucoepidermoid, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Ocular surface tumors include a variety of neoplasms originating from squamous epithelium, melanocytic tumors and lymphocytic resident cells of the conjunctival stroma. In this review, we highlight clinical features of these lesions, important diagnostic and investigative tools and standard care of management. PMID:21234217

  4. [Neurologic manifestations of sarcoidosis: A study of 18 cases].

    PubMed

    Touati, N; Mansour, M; Bedoui, I; Kacem, A; Derbali, H; Riahi, A; Messelmani, M; Zaouali, J; Fekih-Mrissa, N; Mrissa, R

    2015-11-01

    Sarcoidosis is a multisystemic granulomatous disease of unknown aetiology. Neurologic manifestations are found in 5 to 10% of cases. We conducted a retrospective study over 6-year period including 18 patients diagnosed with neurosarcoidosis in the Neurologic department of the Military Hospital of Instruction of Tunis. Clinical, radiological, therapeutic features and outcome were studied. The mean age was 43.44 years. Neurologic signs were the first symptom in 10 cases. Peripheral nervous system impairment was often found. Meningitis was noted in 8 cases. Biological tests are not contributive for the diagnosis. The brain magnetic resonance imaging was pathologic in 10 cases. Corticosteroids were administrated in the majority of cases. Eight patients did not show any sign of improvement. Ten cases improved with treatment. Diagnosis of neurosarcoidosis is difficult because of its clinical and radiological polymorphism. It is based on a clinical history suggestive of neurosarcoidosis, laboratory, imaging and histological studies. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  5. Ventricular tachycardia as the first manifestation of cardiac sarcoidosis

    PubMed Central

    Mehrhof, Felix; Stockburger, Martin; Schuette, Hartwig; Haverkamp, Wilhelm; Dietz, Rainer

    2009-01-01

    The case of a 32-year-old man with sustained ventricular tachycardia and hypotension is described. Following pharmacological treatment the patient switched to a sinus rhythm and was transferred to a university hospital for further diagnostic procedures and treatment. Cardiac catherisation ruled out underlying coronary artery disease, and cardiac MRI as well as echocardiography demonstrated a moderately reduced left ventricular ejection fraction, marked thickening of the interventricular septum and extensive intramural and epicardial infiltration of both ventricles. Endomyocardial biopsies were inconclusive; an implantable cardioverter defibrillator (ICD) was implanted in order to prevent a fatal arrhythmic event. Only repeated lymph node biopsies revealed typical findings of granulomatous disease, which together with the clinical course and the cardiac MRI findings strongly supported cardiac sarcoidosis. A few days after initiation of therapy with corticosteroids, the patient experienced the first of a number of ICD discharges, demanding aggressive anti-arrhythmic treatment regimen for the future. PMID:21686620

  6. Multifocal Defects and Splenomegaly in Sarcoidosis: A New Scintigraphic Pattern

    PubMed Central

    Iko, Benny O.; Chunwuba, Christian; Anderson, Javan E.; Greene, E. Josephine; Higginbotham, Edith; Thomas, Andrew

    1982-01-01

    Sarcoidosis involves the spleen in a significant percentage of patients afflicted with this disease. The most commonly applied modality for studying such patients is the 99mTc-sulfur colloid scan. Different patterns have been recognized: “normal” spleen and nonspecific splenic enlargement, with or without focal areas of photon deficiency, which was thought to represent an infarction in a previous report. We have encountered two patients showing splenomegaly and multifocal photon deficiencies secondary to sarcoid granulomata. These cases are presented to inform clinicians of a new pattern. Our patients were managed with systemic corticosteroids and palliation of pain. If symptoms persist or worsen, splenectomy may be necessary to avoid spontaneous rupture. An abbreviated differential diagnosis of the enlarged spleen with multiple focal defects is discussed. ImagesFigure 1Figure 2Figure 3 PMID:7131574

  7. MRI in ocular drug delivery.

    PubMed

    Li, S Kevin; Lizak, Martin J; Jeong, Eun-Kee

    2008-11-01

    Conventional pharmacokinetic methods for studying ocular drug delivery are invasive and cannot be conveniently applied to humans. The advancement of MRI technology has provided new opportunities in ocular drug-delivery research. MRI provides a means to non-invasively and continuously monitor ocular drug-delivery systems with a contrast agent or compound labeled with a contrast agent. It is a useful technique in pharmacokinetic studies, evaluation of drug-delivery methods, and drug-delivery device testing. Although the current status of the technology presents some major challenges to pharmaceutical research using MRI, it has a lot of potential. In the past decade, MRI has been used to examine ocular drug delivery via the subconjunctival route, intravitreal injection, intrascleral injection to the suprachoroidal space, episcleral and intravitreal implants, periocular injections, and ocular iontophoresis. In this review, the advantages and limitations of MRI in the study of ocular drug delivery are discussed. Different MR contrast agents and MRI techniques for ocular drug-delivery research are compared. Ocular drug-delivery studies using MRI are reviewed.

  8. Fetal ocular measurements by MRI.

    PubMed

    Li, Xiao Bing; Kasprian, Gregor; Hodge, Jacqueline C; Jiang, Xiao Li; Bettelheim, Dieter; Brugger, Peter C; Prayer, Daniela

    2010-11-01

    To present fetal magnetic resonance imaging (MRI) ocular measurement ranges by gestational age (GA) in normal and growth-restricted fetuses. A total of 298 pregnant women from the 18th to the 39th week of gestation were imaged using MRI. Ocular measurements including binocular distance (BOD), interocular distance (IOD), transverse ocular diameter (OD) and anterior-posterior (AP) OD were measured. The curve estimation analyses for linear, logarithmic and quadratic models were performed. The ocular measurements of the fetuses with intrauterine growth restriction (IUGR) were compared with that of the normal fetuses. The fetal eye resembles an ellipsoid with significantly longer OD and shorter AP (t = - 22.07, p < 0.001). The quadratic model was the best model in predicting growth of the fetal BOD, IOD, OD and AP. The ocular measurements of the fetuses with IUGR were significantly different from that of the normal fetuses (BOD: t = 3.58, p < 0.001; IOD: t = 5.73, p < 0.001; OD: t = 3.52, p < 0.001; AP: t = 2.19, p < 0.05). Fetal ocular growth can be readily assessed by fetal MRI. Using the normative data provided in this study, fetal ocular anomalies may be detected. Ocular size is frequently reduced in the condition of IUGR, with potential pathologic impact on postnatal vision.

  9. MRI in ocular drug delivery

    PubMed Central

    Li, S. Kevin; Lizak, Martin J.; Jeong, Eun-Kee

    2008-01-01

    Conventional pharmacokinetic methods for studying ocular drug delivery are invasive and cannot be conveniently applied to humans. The advancement of MRI technology has provided new opportunities in ocular drug-delivery research. MRI provides a means to non-invasively and continuously monitor ocular drug-delivery systems with a contrast agent or compound labeled with a contrast agent. It is a useful technique in pharmacokinetic studies, evaluation of drug-delivery methods, and drug-delivery device testing. Although the current status of the technology presents some major challenges to pharmaceutical research using MRI, it has a lot of potential. In the past decade, MRI has been used to examine ocular drug delivery via the subconjunctival route, intravitreal injection, intrascleral injection to the suprachoroidal space, episcleral and intravitreal implants, periocular injections, and ocular iontophoresis. In this review, the advantages and limitations of MRI in the study of ocular drug delivery are discussed. Different MR contrast agents and MRI techniques for ocular drug-delivery research are compared. Ocular drug-delivery studies using MRI are reviewed. PMID:18186077

  10. Unsuspected exposure to beryllium: potential implications for sarcoidosis diagnoses.

    PubMed

    Laczniak, Andrew N; Gross, Nathan A; Fuortes, Laurence J; Field, R William

    2014-07-21

    Exposure to Beryllium (Be) can cause sensitization (BeS) and chronic beryllium disease (CBD) in some individuals.  Even relatively low exposures may be sufficient to generate an asymptomatic, or in some cases a symptomatic, immune response. Since the clinical presentation of CBD is similar to that of sarcoidosis, it is helpful to have information on exposure to beryllium in order to reduce misdiagnosis. The purpose of this pilot study is to explore the occurrence of Be surface deposits at worksites with little or no previous reported use of commercially available Be products.  The workplaces chosen for this study represent a convenience sample of businesses in eastern Iowa. One hundred thirty-six surface dust samples were collected from 27 businesses for analysis of Be. The results were then divided into categories by the amount of detected Be according to U.S. Department of Energy guidelines as described in 10 CFR 850.30 and 10 CFR 850.31. Overall, at least one of the samples at 78% of the work sites tested contained deposited Be above the analytical limit of quantitation (0.035 µg beryllium per sample).  Beryllium was detected in 46% of the samples collected. Twelve percent of the samples exceeded 0.2 µg/100 cm² and 4% of the samples exceeded a Be concentration of 3 µg/100 cm². The findings from this study suggest that there may be a wider range and greater number of work environments that have the potential for Be exposure than has been documented previously.  These findings could have implications for the accurate diagnosis of sarcoidosis.

  11. Nonhuman Primate Ocular Biometry

    PubMed Central

    Augusteyn, Robert C.; Maceo Heilman, Bianca; Ho, Arthur; Parel, Jean-Marie

    2016-01-01

    Purpose To examine ocular growth in nonhuman primates (NHPs) from measurements on ex vivo eyes. Methods We obtained NHP eyes from animals that had been killed as part of other studies or because of health-related issues. Digital calipers were used to measure the horizontal, vertical, and anteroposterior globe diameters as well as corneal horizontal and vertical diameters of excised globes from 98 hamadryas baboons, 551 cynomolgus monkeys, and 112 rhesus monkeys, at ages ranging from 23 to 360 months. Isolated lens sagittal thickness and equatorial diameter were measured by shadowphotogrammetry. Wet and fixed dry weights were obtained for lenses. Results Nonhuman primate globe growth continues throughout life, slowing toward an asymptotic maximum. The final globe size scales with negative allometry to adult body size. Corneal growth ceases at around 20 months. Lens diameter increases but thickness decreases with increasing age. Nonhuman primate lens wet and dry weight accumulation is monophasic, continuing throughout life toward asymptotic maxima. The dry/wet weight ratio reaches a maximum of 0.33. Conclusions Nonhuman primate ocular globe and lens growth differ in several respects from those in humans. Although age-related losses of lens power and accommodative amplitude are similar, lens growth and properties are different indicating care should be taken in extrapolating NHP observations to the study of human accommodation. PMID:26780314

  12. Ocular surface temperature.

    PubMed

    Efron, N; Young, G; Brennan, N A

    1989-09-01

    A wide-field color-coded infra-red imaging device was applied to the measurement of i) the temperature profile across the ocular surface and ii) the temporal stability of central corneal temperature, on 21 subjects. The thermographs showed a pattern of ellipsoidal isotherms (major axis horizontal) approximately concentric about a temperature apex (coldest point) which was slightly inferior to the geometric center of the cornea (GCC). The GCC had a mean temperature (+/- SD) of 34.3 +/- 0.7 degrees C (range 32.8 to 35.4 degrees C). Temperature increased towards the periphery of the cornea with the limbus being 0.45 degrees C warmer than the GCC (p less than 0.0001). Following a blink, the GCC cooled at a mean (+/- SD) rate of 0.033 +/- 0.024 degrees C/s (p less than 0.0001) over the first 15s. Subjects whose corneas cooled more slowly following a blink demonstrated a greater capacity to avoid blinking for a prolonged period (p less than 0.05). This improved method of measuring ocular surface temperature has important applications in modeling corneal physiology and pathology.

  13. Multiple mycobacterial antigens are targets of the adaptive immune response in pulmonary sarcoidosis

    PubMed Central

    2010-01-01

    Introduction Sarcoidosis is a multisystem granulomatous disease for which the association with mycobacteria continues to strengthen. It is hypothesized that a single, poorly degradable antigen is responsible for sarcoidosis pathogenesis. Several reports from independent groups support mycobacterial antigens having a role in sarcoidosis pathogenesis. To identify other microbial targets of the adaptive immune response, we tested the ability of CD4+ and CD8+ T cells to recognize multiple mycobacterial antigens. Methods Fifty-four subjects were enrolled in this study: 31 sarcoidosis patients, nine non-tuberculosis mycobacterial (NTM) infection controls, and 14 PPD- controls. Using flow cytometry, we assessed for Th1 immune responses to ESAT-6, katG, Ag85A, sodA, and HSP. Results Alveolar T-cells from twenty-two of the 31 sarcoidosis patients produced a CD4+ response to at least one of ESAT-6, katG, Ag85A, sodA, or HSP, compared to two of 14 PPD- controls (p = 0.0008) and five of nine NTM controls (p = 0.44), while eighteen of the 31 sarcoidosis subjects tested produced a CD8+ response to at least one of the mycobacterial antigens compared to two of 14 PPD- controls (p = 0.009) and three of nine NTM controls (0.26). Not only did the BAL-derived T cells respond to multiple virulence factors, but also to multiple, distinct epitopes within a given protein. The detection of proliferation upon stimulation with the mycobacterial virulence factors demonstrates that these responses are initiated by antigen specific recognition. Conclusions Together these results reveal that antigen-specific CD4+ and CD8+ T cells responses to multiple mycobacterial epitopes are present within sites of active sarcoidosis involvement, and that these antigen-specific responses are present at the time of diagnosis. PMID:21092305

  14. Evaluation of Tpeak-Tend interval and Tpeak-end/QT ratio in patients with Sarcoidosis.

    PubMed

    Ucsular, Fatma; Karadeniz, Gülistan; Karadeniz, Cem; Yalnız, Enver; Demir, Melike

    2016-08-01

    rdiogram (ECG), can be used as a marker of transmural dispersion of repolarization. Also, Tp-e/QT and Tp-e/QTc ratios are used as an index of arrhythmogenesis. Prolonged Tp-e interval and Tp-e/QT ratio was found associated with sudden cardiac death in different clinical conditions. Novel ventricular repolarization parameters, Tp-e interval, Tp-e/QT and Tp-e/QTc ratios have not been evaluated in patients with sarcoidosis without cardiac symptoms previously.Therefore, we investigated Tp-e interval in patients with pulmonary sarcoidosis. Sixty-one consecutive patients diagnosed with biopsy-proven pulmonary sarcoidosis were retrospectively reviewed. The 12-lead ECG examinations were performed at speed of 25 mm/s while the patients were in supine position.  QT dispersion, Tp-e intervals and Tp-e/QT ratio were measured. Comparison of ECG parameters between two groups showed a significantly higher average heart rate (p< 0.05) in the patients with sarcoidosis than controls. QT dispersion was higher in patients group. Tp-e interval was significantly prolonged in the study group compared to the control group (92±21 ms and 85±14 ms, respectively; p< 0.05). Tp-e/QT and Tp-e/QTc ratios were significantly higher in patients with sarcoidosis compared to control subjects (all p values < 0.05). The novel repolarization parameters Tp-e interval, Tp-e/QT and Tp-e/QTc ratios are increased in patients with sarcoidosis without any cardiac symptoms. These parameters might be used as a marker for predicting the ventricular arrhythmias and sudden cardiac death in patients with sarcoidosis.

  15. Bioinformatics analysis of gene expression profile data to screen key genes involved in pulmonary sarcoidosis.

    PubMed

    Li, Hongyan; Zhao, Xiaonan; Wang, Jing; Zong, Minru; Yang, Hailing

    2017-01-05

    Sarcoidosis is a multisystemic inflammatory and granulomatous disease that occurs in almost all populations and affects multiple organs. Meanwhile, its most common manifestation is pulmonary sarcoidosis. This study aimed to identify effective biomarkers for the diagnosis and therapy of pulmonary sarcoidosis. GSE16538 was downloaded from Gene Expression Omnibus, including 6 pulmonary sarcoidosis samples and 6 normal lung samples. Then, differentially expressed genes (DEGs) were identified by limma package in R. After the expression values of the DEGs were extracted, hierarchical clustering analysis was performed for the DEGs using the pheatmap package in R. Subsequently, protein-protein interaction (PPI) pairs among the DEGs were searched by STRING or REACTOME databases, and then PPI networks were visualized by Cytoscape software. Using DAVID and KOBAS, functional and pathway enrichment analyses separately were performed for the DEGs involved in the PPI network. Total 208 DEGs were identified in pulmonary sarcoidosis samples, including 179 up-regulated genes and 29 down-regulated genes. Hierarchical clustering showed that the DEGs could clearly distinguish the pulmonary sarcoidosis samples from the normal lung samples. In the PPI network constructed by STRING database, CXCL9, STAT1, CCL5, CXCL11 and GBP1 had higher degrees and betweenness values, and could interact with each other. Functional enrichment showed that CXCL9, CXCL11 and CCL5 were enriched in immune response. Moreover, STAT1 was enriched in pathways of chemokine signaling pathway and JAK-STAT signaling pathway. CXCL9, CXCL11, STAT1, CCL5 and GBP1 might be implicated in pulmonary sarcoidosis through interacting with each other. Copyright © 2016 Elsevier B.V. All rights reserved.

  16. T wave amplitude in lead aVR as a novel diagnostic marker for cardiac sarcoidosis.

    PubMed

    Tanaka, Yoshihiro; Konno, Tetsuo; Yoshida, Shohei; Tsuda, Toyonobu; Sakata, Kenji; Furusho, Hiroshi; Takamura, Masayuki; Yoshimura, Kenichi; Yamagishi, Masakazu; Hayashi, Kenshi

    2017-03-01

    It is vital to identify cardiac involvement (CI) in patients with sarcoidosis as the condition could initially lead to sudden cardiac death. Although the T wave amplitude in lead aVR (TWAaVR) is reportedly associated with adverse cardiac events in various cardiovascular diseases, only scarce data are available concerning the utility of lead aVR in identifying CI in patients with sarcoidosis. We retrospectively investigated the diagnostic values of TWAaVR in patients with sarcoidosis in comparison with conventional electrocardiography parameters such as bundle branch block (BBB). From January 2006 to December 2014, 93 consecutive patients with sarcoidosis were enrolled (mean age, 55.7 ± 15.7 years; male, 31 %; cardiac involvement, n = 26). TWAaVR showed the greatest sensitivity (39 %) and specificity (92 %) in distinguishing between sarcoidosis patients with and without CI, at a cutoff value of -0.08 mV. The diagnostic value of BBB for cardiac involvement was significantly improved when combined with TWAaVR (sensitivity: 61-94 %, specificity: 97-89 %, area under the curve: 0.79-0.92, p = 0.018). Multivariate logistic regression analysis indicated that TWAaVR and BBB were independent electrocardiography parameters associated with CI. In summary, we observed that sarcoidosis patients exhibiting a high TWAaVR were likely to have CI. Thus, the application of a combination of BBB with TWAaVR may be useful when screening for CI in sarcoidosis patients.

  17. Inhibition of 5-fluorouracil-induced ocular irritation by ocular ice packs.

    PubMed

    Loprinzi, C L; Wender, D B; Veeder, M H; O'Fallon, J R; Vaught, N L; Dose, A M; Ghosh, C; Bartel, J; Leitch, J M

    1994-08-01

    This clinical trial was developed to determine whether ocular ice pack therapy would decrease 5-fluorouracil (5-FU)-induced ocular toxicity. Sixty-two patients who suffered from 5-FU-induced ocular toxicity, and were scheduled to receive another cycle of the chemotherapy that caused the ocular toxicity, were entered in this clinical trial. A randomized, crossover design was used, with patients documenting their ocular toxicity by the use of daily diaries. The results from the first cycle of treatment suggested that ocular ice pack therapy decreased 5-FU-induced ocular toxicity (P = 0.056). The 38 evaluable patients in the crossover analyses demonstrated decreased ocular toxicity with ocular ice pack therapy (p = .001). The ocular ice pack therapy was well tolerated by most of the study participants. Ocular ice pack therapy appears to lessen 5-FU-induced ocular toxicity to a clinically moderate degree. Better methods for decreasing 5-FU-induced ocular toxicity are necessary.

  18. Ocular involvement in pemphigus vulgaris.

    PubMed

    Akhyani, Maryam; Keshtkar-Jafari, Alireza; Chams-Davatchi, Cheyda; Lajevardi, Vahide; Beigi, Sara; Aghazadeh, Nessa; Rayati Damavandi, Maede; Arami, Shabnam

    2014-07-01

    Pemphigus vulgaris (PV) is an autoimmune disorder affecting the skin and mucous membranes. Ocular involvement in PV has been reported but its prevalence and clinical characteristics are not well defined. This prospective cross-sectional study of 103 PV patients was designed to determine the prevalence, clinical types and epidemiological trends of ocular involvement in a population of Iranian patients with PV. Ocular involvement was present in 17 (16.5%) patients. Conjunctivitis was the most prevalent type of ocular involvement (9/17, 52.9%), followed by erosion of the palpebral conjunctiva (7/17, 41.2%). Erosion of the bulbar conjunctiva was noted in only one patient (5.9%). The most commonly reported symptoms were eye irritation (76.5%) and redness (76.5%). No significant relation was found between ocular involvement and disease activity (partial remission or relapse). Mucoid discharge was significantly more common in patients with conjunctival erosions as compared to patients with conjunctivitis (P = 0.038). We conclude that ocular involvement is not rare in PV; 16.5% of PV patients develop ocular disease independent of the disease activity and extension. Conjunctivitis is the most common type of involvement, however, palpebral conjunctival erosion is more frequent than previously realized.

  19. Ocular Screening System

    NASA Technical Reports Server (NTRS)

    1987-01-01

    Used to detect eye problems in children through analysis of retinal reflexes, the system incorporates image processing techniques. VISISCREEN's photorefractor is basically a 35 millimeter camera with a telephoto lens and an electronic flash. By making a color photograph, the system can test the human eye for refractive error and obstruction in the cornea or lens. Ocular alignment problems are detected by imaging both eyes simultaneously. Electronic flash sends light into the eyes and the light is reflected from the retina back to the camera lens. Photorefractor analyzes the retinal reflexes generated by the subject's response to the flash and produces an image of the subject's eyes in which the pupils are variously colored. The nature of a defect, where such exists, is identifiable by atrained observer's visual examination.

  20. Sports related ocular injuries.

    PubMed

    Mishra, Avinash; Verma, Ashok K

    2012-07-01

    Every year > 600,000 sports and recreation related eye injuries occur, out of which roughly 13,500 of these result in permanent loss of sight. Up to 90% of these sports related eye injuries are preventable by using adequate eye protection equipment. Protective eyewear is made of polycarbonate, a highly impact-resistant plastic which is now easily available as prescription and non-prescription eyewear and all players should be encouraged to use them. The medical officers by educating their patients regarding the risks of eye injuries in various sports and the confirmed benefits of using protective equipment have the potential to prevent injury to over thousands of eyes every year. The medical fraternity can also play a very important role in educating the coaches, parents, and children and thus put an end to unnecessary blindness and vision loss from sports related ocular injuries, therefore ensuring a lifetime of healthy vision.

  1. Ocular leech infestation

    PubMed Central

    Lee, Yueh-Chang; Chiu, Cheng-Jen

    2015-01-01

    This case report describes a female toddler with manifestations of ocular leech infestation. A 2-year-old girl was brought to our outpatient clinic with a complaint of irritable crying after being taken to a stream in Hualien 1 day previous, where she played in the water. The parents noticed that she rubbed her right eye a lot. Upon examination, the girl had good fix and follow in either eye. Slit-lamp examination showed conjunctival injection with a moving dark black–brown foreign body partly attached in the lower conjunctiva. After applying topical anesthetics, the leech, measuring 1 cm in length, was extracted under a microscope. The patient began using topical antibiotic and corticosteroid agents. By 1 week after extraction, the patient had no obvious symptoms or signs, except for a limited subconjunctival hemorrhage, and no corneal/scleral involvement was observed. PMID:25784786

  2. Photorefractor ocular screening system

    NASA Technical Reports Server (NTRS)

    Richardson, John R. (Inventor); Kerr, Joseph H. (Inventor)

    1987-01-01

    A method and apparatus for detecting human eye defects, particularly detection of refractive error is presented. Eye reflex is recorded on color film when the eyes are exposed to a flash of light. The photographs are compared with predetermined standards to detect eye defects. The base structure of the ocular screening system is a folding interconnect structure, comprising hinged sections. Attached to one end of the structure is a head positioning station which comprises vertical support, a head positioning bracket having one end attached to the top of the support, and two head positioning lamps to verify precise head positioning. At the opposite end of the interconnect structure is a camera station with camera, electronic flash unit, and blinking fixation lamp, for photographing the eyes of persons being evaluated.

  3. Ocular histoplasmosis syndrome.

    PubMed

    Diaz, Rocio I; Sigler, Eric J; Rafieetary, Mohammad R; Calzada, Jorge I

    2015-01-01

    Ocular histoplasmosis syndrome (OHS) is a chorioretinal disorder with a distinct fundus appearance that is commonly found in regions endemic for Histoplasma capsulatum. Choroidal neovascularization (CNV) secondary to OHS is considered one of the principal causes of central vision loss among young adults in endemic areas. Although there is no consensus regarding its pathogenesis, evidence points to Histoplasma capsulatum as the most probable etiology. Once considered an intractable hemorrhagic maculopathy, CNVs are now treatable. Extrafoveal CNVs are successfully treated with laser photocoagulation. Subfoveal and juxtafoveal CNVs are managed with anti-vascular endothelial growth factor therapy, photodynamic therapy, or a combination of both. Modern imaging technologies such as spectral-domain optical coherence tomography have improved our diagnostic abilities, making it easier to monitor disease activity and CNV regression. We review the epidemiology, pathogenesis, clinical manifestations, differential diagnosis, and current treatment of this disease.

  4. Corticosteroids for ocular toxoplasmosis

    PubMed Central

    Jasper, Smitha; Vedula, Satyanarayana S; John, Sheeja S; Horo, Saban; Sepah, Yasir J; Nguyen, Quan Dong

    2014-01-01

    Background Ocular infestation with Toxoplasma gondii, a parasite, may result in inflammation in the retina, choroid, and uvea and consequently lead to complications such as glaucoma, cataract, and posterior synechiae. Objectives The objective of this systematic review was to assess the effects of adjunctive use of corticosteroids for ocular toxoplasmosis. Search methods We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2012, Issue 9), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE, (January 1950 to October 2012), EMBASE (January 1980 to October 2012), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to October 2012), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov) and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We searched the reference lists of included studies for any additional studies not identified by the electronic searches. We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 11 October 2012. Selection criteria We planned to include randomized and quasi-randomized controlled trials. Eligible trials would have enrolled participants of any age who were immunocompetent and were diagnosed with active ocular toxoplasmosis. Included trials would have compared anti-parasitic therapy plus corticosteroids versus anti-parasitic therapy alone, or different doses or times of initiation of corticosteroids. Data collection and analysis Two authors independently screened titles and abstracts retrieved from the electronic searches. We retrieved full-text articles of studies categorized as ‘unsure’ or ‘include’ after review of the abstracts. Two authors independently reviewed each full-text article. Discrepancies were

  5. Cornea: Window to Ocular Immunology

    PubMed Central

    Niederkorn, Jerry Y.

    2011-01-01

    The ocular surface is continuously exposed to environmental agents such as allergens, pollutants, and microorganisms, which could provoke inflammation. However, an array of anatomical, physiological, and immunological features of the ocular surface conspire to limit corneal inflammation and endow the eye with immune privilege. A remarkable example of ocular immune privilege is the success of corneal allografts, which unlike all other forms of organ transplantation, survive without the use of systemic immunosuppressive drugs or MHC matching. This review describes the anatomical, physiological, and dynamic immunoregulatory processes that contribute to immune privilege. PMID:21789035

  6. Ocular manifestations of feline herpesvirus.

    PubMed

    Andrew, S E

    2001-03-01

    Feline herpesvirus-1 (FHV-1) infection is ubiquitous in the domestic cat population worldwide. The most common clinical ocular manifestations of infection with FHV-1 are conjunctivitis and keratitis. This paper reviews the pathogenesis of feline herpesvirus-1 and discusses the various clinical ocular manifestations, diagnostic techniques and treatment of FHV-1-induced diseases. Ocular manifestations include: conjunctivitis, keratitis, stromal keratitis, keratoconjunctivitis sicca, ophthalmia neonatorium, symblepharon, corneal sequestrum, eosinophilic keratitis and anterior uveitis. Diagnostic techniques discussed include: virus isolation, fluorescent antibody testing, serum neutralising titers, ELISA and polymerase chain reaction. Various therapies are also discussed.

  7. Ocular complications of diabetes mellitus

    PubMed Central

    Sayin, Nihat; Kara, Necip; Pekel, Gökhan

    2015-01-01

    Diabetes mellitus (DM) is a important health problem that induces ernestful complications and it causes significant morbidity owing to specific microvascular complications such as, retinopathy, nephropathy and neuropathy, and macrovascular complications such as, ischaemic heart disease, and peripheral vasculopathy. It can affect children, young people and adults and is becoming more common. Ocular complications associated with DM are progressive and rapidly becoming the world’s most significant cause of morbidity and are preventable with early detection and timely treatment. This review provides an overview of five main ocular complications associated with DM, diabetic retinopathy and papillopathy, cataract, glaucoma, and ocular surface diseases. PMID:25685281

  8. Ocular myasthenia gravis: A review

    PubMed Central

    Nair, Akshay Gopinathan; Patil-Chhablani, Preeti; Venkatramani, Devendra V; Gandhi, Rashmin Anilkumar

    2014-01-01

    Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG. PMID:25449931

  9. Humoral immune profiling of mycobacterial antigen recognition in sarcoidosis and Löfgren's syndrome using high-content peptide microarrays.

    PubMed

    Ferrara, Giovanni; Valentini, Davide; Rao, Martin; Wahlström, Jan; Grunewald, Johan; Larsson, Lars-Olof; Brighenti, Susanna; Dodoo, Ernest; Zumla, Alimuddin; Maeurer, Markus

    2017-03-01

    Sarcoidosis is considered an idiopathic granulomatous disease, although similar immunological and clinical features with tuberculosis (TB) suggest mycobacterial involvement in its pathogenesis. High-content peptide microarrays (HCPM) may help to decipher mycobacteria-specific antibody reactivity in sarcoidosis. Serum samples from patients with sarcoidosis, Löfgren's syndrome, and TB, as well as from healthy individuals (12/group), were tested on HCPM containing 5964 individual peptides spanning 154 Mycobacterium tuberculosis proteins displayed as 15-amino acid stretches. Inclusion/exclusion and significance analyses were performed according to published methods. Each study group recognized 68-78% M. tuberculosis peptides at least once. M. tuberculosis epitope recognition by sarcoidosis patient sera was 42.7%, and by TB patient sera was 39.1%. Seven and 16 peptides were recognized in 9/12 (75%) and 8/12 (67%) sarcoidosis patient sera but not in TB patient sera, respectively. Nine (75%) and eight (67%) out of twelve TB patient sera, respectively recognized M. tuberculosis peptides that were not recognized in sarcoidosis patient sera. Specific IgG recognition patterns for M. tuberculosis antigens in sarcoidosis patients re-affirm mycobacterial involvement in sarcoidosis, providing biologically relevant targets for future studies pertaining to diagnostics and immunotherapy. Copyright © 2017. Published by Elsevier Ltd.

  10. Use of insurance claims data to determine prevalence and confirm a cluster of sarcoidosis cases in Vermont.

    PubMed

    Cragin, Lori A; Laney, A Scott; Lohff, Cortland J; Martin, Brennan; Pandiani, John A; Blevins, Lynn Z

    2009-01-01

    In 2006, the Vermont Department of Health was asked to respond to a potential cluster of sarcoidosis cases related to a Vermont office building. Sarcoidosis prevalence has not been formally described for the United States. A range of < 1-40/100,000 is commonly reported; however, we have not identified primary sources supporting this conclusion. Because of the wide prevalence range and lack of a local estimate, confirming existence of a cluster was difficult. We ascertained the prevalence of sarcoidosis cases in Vermont by using insurance claims data to determine whether or not a cluster of sarcoidosis cases was related to the office building. We calculated county and state annual prevalence proportions for sarcoidosis for 2004 and 2005 and annual building prevalences for 1992-2006. The pooled sarcoidosis case prevalence for Vermont was 66.1/100,000. The pooled building annual prevalence (1,128/100,000) was statistically different from the county in which the building is located (odds ratio = 15.5, 95% confidence interval 3.0, 50.3). We reported the first statewide sarcoidosis prevalence in the United States. This prevalence exceeded previous limited and unsubstantiated U.S. reports. Even with Vermont's elevated sarcoidosis prevalence, the presence of a cluster in this building was apparent.

  11. Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association: Report of 12 Cases and Review of the Literature.

    PubMed

    Grados, Aurélie; Ebbo, Mikael; Bernit, Emmanuelle; Veit, Véronique; Mazodier, Karin; Jean, Rodolphe; Coso, Diane; Aurran-Schleinitz, Thérèse; Broussais, Florence; Bouabdallah, Reda; Gravis, Gwenaelle; Goncalves, Anthony; Giovaninni, Marc; Sève, Pascal; Chetaille, Bruno; Gavet-Bongo, Florence; Weitten, Thierry; Pavic, Michel; Harlé, Jean-Robert; Schleinitz, Nicolas

    2015-07-01

    The association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported. We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients. Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series. This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse.

  12. Expression Profile of Six RNA-Binding Proteins in Pulmonary Sarcoidosis

    PubMed Central

    Novosadova, Eva; Hagemann-Jensen, Michael; Kullberg, Susanna; Kolek, Vitezslav; Grunewald, Johan; Petrek, Martin

    2016-01-01

    Background Sarcoidosis is characterised by up-regulation of cytokines and chemokine ligands/receptors and proteolytic enzymes. This pro-inflammatory profile is regulated post-transcriptionally by RNA-binding proteins (RBPs). We investigated in vivo expression of six RBPs (AUF1, HuR, NCL, TIA, TIAR, PCBP2) and two inhibitors of proteolytic enzymes (RECK, PTEN) in pulmonary sarcoidosis and compared it to the expression in four control groups of healthy individuals and patients with other respiratory diseases: chronic obstructive pulmonary disease (COPD), asthma and idiopathic interstitial pneumonias (IIPs). Methods RT-PCR was used to quantify the mRNAs in bronchoalveolar (BA) cells obtained from 50 sarcoidosis patients, 23 healthy controls, 30 COPD, 19 asthmatic and 19 IIPs patients. Flow cytometry was used to assess intracellular protein expression of AUF1 and HuR in peripheral blood T lymphocytes (PBTLs) obtained from 9 sarcoidosis patients and 6 healthy controls. Results Taking the stringent conditions for multiple comparisons into consideration, we consistently observed in the primary analysis including all patients regardless of smoking status as well as in the subsequent sub-analysis limited for never smokers that the BA mRNA expression of AUF1 (p<0.001), TIA (p<0.001), NCL (p<0.01) and RECK (p<0.05) was decreased in sarcoidosis compared to healthy controls. TIA mRNA was also decreased in sarcoidosis compared to both obstructive pulmonary diseases (COPD and asthma; p<0.001) but not compared to IIPs. There were several positive correlations between RECK mRNA and RBP mRNAs in BA cells. Also sarcoidosis CD3+, CD4+ and CD8+ PBTLs displayed lower mean fluorescence intensity of AUF1 (p≤0.02) and HuR (p≤0.03) proteins than control healthy PBTLs. Conclusion mRNA expressions of three RBPs (AUF1, TIA and NCL) and their potential target mRNA encoding RECK in BA cells and additionally protein expression of AUF1 and HuR in PBTLs were down-regulated in our sarcoidosis

  13. Immunological Evidence for the Role of Mycobacteria in Sarcoidosis: A Meta-Analysis.

    PubMed

    Fang, Chuling; Huang, Hui; Xu, Zuojun

    2016-01-01

    Sarcoidosis is a granulomatous disease, the etiology of which is currently unknown. The role of mycobacteria in the etiology of sarcoidosis has been extensively investigated. In this meta-analysis, we assessed the immunological evidence of the possible role of mycobacteria in the pathogenesis and development of sarcoidosis. We performed a systematic search of relevant articles from PubMed, Embase and Cochrane Library databases published between January 1990 and October 2015. Data extracted from the articles were analyzed with Review Manager 5.3 (Cochrane Collaboration, Oxford, UK). In this meta-analysis, 13 case-control studies (733 participants) were considered eligible according to our criteria. Methodological quality was assessed using the Newcastle-Ottawa Scale (NOS). The positivity incidence of the immune response (either the cell-mediated response or humoral response) in sarcoidosis patients was significantly higher than that in controls, as determined using fixed-effects model. The odds ratio (OR) of the positivity incidence of T-cell response in the patients with sarcoidosis versus the controls with PPD- or unknown PPD status was 5.54 (95% CI 3.56-8.61); the ORs were 16.70 (95% CI 8.19-34.08) and 1.48 (95% CI 0.74-2.96) for the two subgroups with PPD- controls and unknown PPD status respectively. However, the OR of the positivity incidence in patients with sarcoidosis versus PPD+ controls (latent tuberculosis infection; LTBI) was 0.26 (95% 0.10-0.66). Regarding the humoral response, pooled analysis of the positivity incidence revealed an OR (95%CI) of 20.43 (5.53-75.53) for the patients with sarcoidosis versus controls; the ORs were 11.93 (95% CI 2.15-66.27) and 41.97 (95% CI 5.24-336.15) in two subgroups of controls with PPD- and unknown PPD statuses respectively. Data on heterogeneity and evidence of publication bias were examined. This meta-analysis confirmed the existence of an association between mycobacteria (especially M.tuberculosis) and sarcoidosis

  14. X-Linked ocular albinism; Nettleship-Falls ocular albinism.

    PubMed

    Booth, Alexandria V; Soldano, Anthony C; Levine, Jonathan; Pomeranz, Miriam

    2008-05-15

    A 39-year-old man with foveal hypoplasia, nystagmus, and decreased visual acuity was found to have multiple, cutaneous, hypopigmented macules. Macromelanosomes were demonstrated in normal skin on histopathologic examination. The patient's constellation of findings along with a strong X-linked inheritance pattern in family members led to the diagnosis of X-linked ocular albinism, which is an uncommon condition that is characterized by congenital nystagmus, iris translucency, hypopigmentation of the ocular fundus, strabismus, foveal hypoplasia, photophobia, and impaired vision.

  15. Sarcoidosis in World Trade Center rescue workers presenting with rheumatologic manifestations.

    PubMed

    Bowers, Brian; Hasni, Sarfaraz; Gruber, Barry L

    2010-01-01

    The health consequences of the World Trade Center collapse are unknown, but likely to be significant and may take years to fully appreciate. Sarcoidosis is a multisystem inflammatory disorder of unknown etiology characterized pathologically by noncaseating granulomas. Inciting events, such as infectious agents or possible environmental exposures, have been postulated as the source of antigen exposure initiating an inflammatory cascade. We describe 2 cases of sarcoidosis in rescue workers with significant exposure from the World Trade Center collapse, who presented with extrapulmonary rheumatologic manifestations. Our first case involved a 33-year-old white New York City man detective found to have sarcoidosis following an evaluation of diffuse joint pain. The second case involved a 40-year-old African American man, New York City officer, who presented with uveitis, and was subsequently diagnosed with sarcoidosis. These 2 cases extend the spectrum of disorders resulting from the World Trade Center disaster and illustrate the need for clinicians to be aware of the diverse presentations of sarcoidosis in this patient population.

  16. Seasonal variation in incidence of sarcoidosis: a population-based study, 1976-2013.

    PubMed

    Ungprasert, Patompong; Crowson, Cynthia S; Matteson, Eric L

    2016-12-01

    Studies of seasonal patterns of incidence of sarcoidosis may provide a better understanding of potential environmental triggers of the disease. In this study, Olmsted County, Minnesota residents who were diagnosed with sarcoidosis between 1976 and 2013 were identified based on individual medical record review. The seasonal variation of incident sarcoidosis was then calculated. The age-adjusted and sex-adjusted incidence rate of sarcoidosis was lower in autumn (2.0/100 000; 95% CI 1.5 to 2.5) compared with winter (3.2/100 000; 95% CI 2.6 to 3.8), spring (2.8/100 000; 95% CI 2.2 to 3.4) and summer (2.9/100 000; 95% CI 2.2 to 3.5, p=0.024). Subgroup analysis per decade consistently showed lower incidence of sarcoidosis in autumn. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  17. Peroxisome proliferator-activated receptor gamma as modulator of inflammation in pulmonary sarcoidosis.

    PubMed

    Pejcić, Tatjana; Stanković, Ivana; Petković, Tatjana Radjenović; Borovac, Desa Nastasijević; Djordjević, Ivanka; Jeftović-Stoimenov, Tatjana

    2013-01-01

    Peroxisome proliferator-activated receptor (PPAR) includes the family of ligand-activated transcription factors which belong to the group of nuclear hormone receptors and are connected to retinoid, glucocorticoid and thyroid hormone receptors. There are three subtypes of PPARs: PPARalpha (also known as NR1C3), PPARgamma (known as NR1C1) and PPARdelta (known as PPARbeta or NR1C2). All of them take part in the metabolism, cell proliferation and immune response. PPARgamma and PPARalpha are identified as important immunomodulators and potentially represent an anti-inflammatory target for respiratory diseases. PPARgamma deficiency in the lungs has been observed in the inflammatory diseases such as asthma, pulmonary alveolar proteinosis, fibrosis and sarcoidosis, as well as in the animal models of the lung inflammation. A small number of papers concerned with PPARgamma in sarcoidosis pointto the lowered activity of this factor in the alveolar macrophages and a lowered gene expression for the PPARgamma, while the activity is preserved in healthy individuals. At the same time, an increased activity of the nuclear factor kappa B (NF-kappaB) in the bronchoalveolar lavage has been recorded in patients with sarcoidosis. The reason for the decrease in the production of PPARgamma in sarcoidosis remains unknown. Several possible mechanisms are mentioned: genetic defect with lowered production, down-regulation due to the increased values of IFN-gamma or an increased decomposition of PPARgamma. Further investigation will explain the mechanisms regarding the decreased production of PPARgamma in sarcoidosis.

  18. A Novel In Vitro Human Granuloma Model of Sarcoidosis and Latent Tuberculosis Infection.

    PubMed

    Crouser, Elliott D; White, Peter; Caceres, Evelyn Guirado; Julian, Mark W; Papp, Audrey C; Locke, Landon W; Sadee, Wolfgang; Schlesinger, Larry S

    2017-10-01

    Many aspects of pathogenic granuloma formation are poorly understood, requiring new relevant laboratory models that represent the complexity (genetics and diversity) of human disease. To address this need, we developed an in vitro model of granuloma formation using human peripheral blood mononuclear cells (PBMCs) derived from patients with active sarcoidosis, latent tuberculosis (TB) infection (LTBI), or normal healthy control subjects. PBMCs were incubated for 7 days with uncoated polystyrene beads or beads coated with purified protein derivative (PPD) or human serum albumin. In response to PPD-coated beads, PBMCs from donors with sarcoidosis and LTBI formed robust multicellular aggregates resembling granulomas, displaying a typical T-helper cell type 1 immune response, as assessed by cytokine analyses. In contrast, minimal PBMC aggregation occurred when control PBMCs were incubated with PPD-coated beads, whereas the response to uncoated beads was negligible in all groups. Sarcoidosis PBMCs responded to human serum albumin-coated beads with modest cellular aggregation and inflammatory cytokine release. Whereas the granuloma-like aggregates formed in response to PPD-coated beads were similar for sarcoidosis and LTBI, molecular profiles differed significantly. mRNA expression patterns revealed distinct pathways engaged in early granuloma formation in sarcoidosis and LTBI, and they resemble molecular patterns reported in diseased human tissues. This novel in vitro human granuloma model is proposed as a tool to investigate mechanisms of early granuloma formation and for preclinical drug discovery research of human granulomatous disorders. Clinical trial registered with www.clinicaltrials.gov (NCT01857401).

  19. Resolution of abnormal cardiac MRI T2 signal following immune suppression for cardiac sarcoidosis.

    PubMed

    Crouser, Elliott D; Ruden, Emily; Julian, Mark W; Raman, Subha V

    2016-08-01

    Cardiac MR (CMR) with late gadolinium enhancement is commonly used to detect cardiac damage in the setting of cardiac sarcoidosis. The addition of T2 mapping to CMR was recently shown to enhance cardiac sarcoidosis detection and correlates with increased cardiac arrhythmia risk. This study was conducted to determine if CMR T2 abnormalities and related arrhythmias are reversible following immune suppression therapy. A retrospective study of subjects with cardiac sarcoidosis with abnormal T2 signal on baseline CMR and a follow-up CMR study at least 4 months later was conducted at The Ohio State University from 2011 to 2015. Immune suppression treated participants had a significant reduction in peak myocardial T2 value (70.0±5.5 vs 59.2±6.1 ms, pretreatment vs post-treatment; p=0.017), and 83% of immune suppression treated subjects had objective improvement in cardiac arrhythmias. Two subjects who had received inadequate immune suppression treatment experienced progression of cardiac sarcoidosis. This report indicates that abnormal CMR T2 signal represents an acute inflammatory manifestation of cardiac sarcoidosis that is potentially reversible with adequate immune suppression therapy.

  20. Possible role of l-selectin in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis

    PubMed Central

    Kaseda, M; Kadota, J; Mukae, H; Kawamoto, S; Shukuwa, T; Iwashita, T; Matsubara, Y; Ishimatsu, Y; Yoshinaga, M; Abe, K; Kohno, S

    2000-01-01

    A number of adhesion molecules participate in the recruitment of inflammatory cells to the site of inflammation, and selectins together with their ligands are important in the early transient adhesion phase. In this study, we evaluated the role of l-selectin in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis. We measured serum and bronchoalveolar lavage fluid (BALF) concentrations of soluble (s)l-selectin using an ELISA. Serum and BALF concentrations of sl-selectin were significantly elevated in patients with sarcoidosis compared with control healthy subjects and idiopathic pulmonary fibrosis (IPF) patients (P < 0·05 and P < 0·01, respectively). The lymphocyte surface marker was also examined in peripheral blood and BALF by flow cytometric analysis. The percentage of CD3+CD62L+ cells (l-selectin-bearing T lymphocytes) was significantly lower in peripheral blood of sarcoidosis than in that of healthy subjects (P < 0·01). In contrast, the percentage of CD3+CD62L− cells (l-selectin-negative T lymphocytes) in BALF of patients with sarcoidosis was significantly higher than in healthy subjects (P < 0·05) and IPF patients (P < 0·01). Furthermore, there was a significant correlation between serum concentrations of sl-selectin and the number of l-selectin-negative T lymphocytes in BALF (r = 0·535, P < 0·01). Our results suggest that l-selectin may be involved in T lymphocyte alveolitis in patients with active pulmonary sarcoidosis. PMID:10886252

  1. Primary Cardiac Sarcoidosis with Syncope and Refractory Atrial Arrhythmia: A Case Report and Review of the Literature.

    PubMed

    Thangam, Manoj; Nathan, Sriram; Kar, Biswajit; Petrovic, Marija; Patel, Manish; Loyalka, Pranav; Buja, L Maximilian; Gregoric, Igor D

    2016-06-01

    We discuss the case of a 38-year-old black man who presented at our hospital with his first episode of syncope, recently developed atrial arrhythmias refractory to pharmacologic therapy, and a left atrial thrombus. He was diagnosed with primary cardiac sarcoidosis characterized by predominant involvement of the epicardium that caused atrial fibrillation and atrial flutter. Histologic analysis of his epicardial lesions yielded a diagnosis of sarcoidosis. This patient's atrial arrhythmia was successfully treated with a hybrid operation that involved resection of his atrial appendage, an Epicor maze procedure, and radiofrequency ablation during a catheter-based electrophysiologic study. The cardiac sarcoidosis was successfully managed with corticosteroid therapy. Our case report shows that sarcoidosis can initially manifest itself as syncope with new-onset atrial arrhythmia. Sarcoidosis is important in the differential diagnosis because of its progressive nature and its potential for treatment with pharmacologic, surgical, and catheter-based interventions.

  2. A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease

    PubMed Central

    Nogi, Shinichi; Sasaki, Noriko; Chinen, Naofumi; Honda, Kiri; Saito, Eiko; Wakabayashi, Takayuki; Yamada, Chiho; Suzuki, Yasuo

    2014-01-01

    Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM) from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis. PMID:25431723

  3. Primary Cardiac Sarcoidosis with Syncope and Refractory Atrial Arrhythmia: A Case Report and Review of the Literature

    PubMed Central

    Thangam, Manoj; Nathan, Sriram; Kar, Biswajit; Petrovic, Marija; Patel, Manish; Loyalka, Pranav; Buja, L. Maximilian

    2016-01-01

    We discuss the case of a 38-year-old black man who presented at our hospital with his first episode of syncope, recently developed atrial arrhythmias refractory to pharmacologic therapy, and a left atrial thrombus. He was diagnosed with primary cardiac sarcoidosis characterized by predominant involvement of the epicardium that caused atrial fibrillation and atrial flutter. Histologic analysis of his epicardial lesions yielded a diagnosis of sarcoidosis. This patient's atrial arrhythmia was successfully treated with a hybrid operation that involved resection of his atrial appendage, an Epicor maze procedure, and radiofrequency ablation during a catheter-based electrophysiologic study. The cardiac sarcoidosis was successfully managed with corticosteroid therapy. Our case report shows that sarcoidosis can initially manifest itself as syncope with new-onset atrial arrhythmia. Sarcoidosis is important in the differential diagnosis because of its progressive nature and its potential for treatment with pharmacologic, surgical, and catheter-based interventions. PMID:27303240

  4. Sarcoidosis in celiac disease: A page written by genetic variants in IL-18 miRNAs target site?

    PubMed

    Mormile, Raffaella

    2016-05-01

    Sarcoidosis is a chronic idiopathic granulomatous disease. Interleukin-18 (IL-18) has been strongly implicated in the pathogenesis of sarcoidosis. Sarcoidosis shows characteristic microRNAs (miRNAs) profiles. MiRNAs have recently emerged as a new class of modulators of gene expression. MiRNAs are involved in susceptibility to a number of autoimmune diseases promoting and inhibiting the gene expression of different Th1 pro-inflammatory cytokines including IL18. Sarcoidosis has been connected with a variety of autoimmune disorders including celiac disease (CD). CD is a chronic, immune-mediated condition of the small intestine caused by permanent intolerance to dietary gluten. IL-18 has been reported to play an important role in inducing and maintaining inflammation after gluten exposure. MiRNAs expression is significantly altered in CD patients. We hypothesize that sarcoidosis and CD may be the result of common genetic variants in IL-18 miRNA target site.

  5. Ocular tropism of respiratory viruses.

    PubMed

    Belser, Jessica A; Rota, Paul A; Tumpey, Terrence M

    2013-03-01

    Respiratory viruses (including adenovirus, influenza virus, respiratory syncytial virus, coronavirus, and rhinovirus) cause a broad spectrum of disease in humans, ranging from mild influenza-like symptoms to acute respiratory failure. While species D adenoviruses and subtype H7 influenza viruses are known to possess an ocular tropism, documented human ocular disease has been reported following infection with all principal respiratory viruses. In this review, we describe the anatomical proximity and cellular receptor distribution between ocular and respiratory tissues. All major respiratory viruses and their association with human ocular disease are discussed. Research utilizing in vitro and in vivo models to study the ability of respiratory viruses to use the eye as a portal of entry as well as a primary site of virus replication is highlighted. Identification of shared receptor-binding preferences, host responses, and laboratory modeling protocols among these viruses provides a needed bridge between clinical and laboratory studies of virus tropism.

  6. Ocular Tropism of Respiratory Viruses

    PubMed Central

    Rota, Paul A.; Tumpey, Terrence M.

    2013-01-01

    SUMMARY Respiratory viruses (including adenovirus, influenza virus, respiratory syncytial virus, coronavirus, and rhinovirus) cause a broad spectrum of disease in humans, ranging from mild influenza-like symptoms to acute respiratory failure. While species D adenoviruses and subtype H7 influenza viruses are known to possess an ocular tropism, documented human ocular disease has been reported following infection with all principal respiratory viruses. In this review, we describe the anatomical proximity and cellular receptor distribution between ocular and respiratory tissues. All major respiratory viruses and their association with human ocular disease are discussed. Research utilizing in vitro and in vivo models to study the ability of respiratory viruses to use the eye as a portal of entry as well as a primary site of virus replication is highlighted. Identification of shared receptor-binding preferences, host responses, and laboratory modeling protocols among these viruses provides a needed bridge between clinical and laboratory studies of virus tropism. PMID:23471620

  7. Ocular Health (OH) Fundoscope Exam

    NASA Image and Video Library

    2013-06-05

    Astronaut Karen Nyberg and Astronaut Chris Cassidy (partially visible), both Expedition 37 flight engineers, perform an Ocular Health (OH) Fundoscope Exam in the Destiny laboratory of the International Space Station

  8. Papulo-Nodular Reactions in Black Tattoos as Markers of Sarcoidosis: Study of 92 Tattoo Reactions from a Hospital Material.

    PubMed

    Sepehri, Mitra; Hutton Carlsen, Katrina; Serup, Jørgen

    2016-01-01

    Sarcoidosis is, from historical data, suggested to be more prevalent among patients with tattoo reactions. We aimed to evaluate this association in a systematic study. This is a consecutive study of patients with tattoo complications, diagnosed in the "Tattoo Clinic" at Bispebjerg University Hospital in Copenhagen, Denmark, from 2008 to 2015, based on clinical assessment and histology. From the overall group of 494 tattoo complications in 406 patients, 92 reactions in 72 patients showed a papulo-nodular pattern studied for local and systemic sarcoidosis, since sarcoidosis is expected to be nodular. Of the 92 reactions with a papulo-nodular pattern, 27 (29%) reactions in 19 patients were diagnosed as cutaneous or systemic sarcoidosis, supported by histology; 65 (71%) were diagnosed as non-sarcoidosis due to histology and no clinical sarcoid manifestations. "Rush phenomenon" with concomitant reaction in many other black tattoos, triggered by a recent tattoo with a papulo-nodular reaction, was observed in 70% in the sarcoidosis group and 28% in the non-sarcoidosis group, indicating a predisposing factor which may be autoimmune and linked with sarcoidosis. Agglomerates of black pigment forming foreign bodies may in the predisposed individual trigger widespread reaction in the skin and internal organs. Black tattoos with papulo-nodular reactions should be seen as markers of sarcoidosis. Papulo-nodular reactions may, as triggers, induce widespread reactions in other black tattoos - a "rush phenomenon" - depending on individual predisposition. Sarcoidosis is estimated to be 500-fold increased in papulo-nodular reactions compared to the prevalence in the general population, and the association with black tattoos is strong. © 2017 S. Karger AG, Basel.

  9. Diagnostic Value of the CD103+CD4+/CD4+ Ratio to Differentiate Sarcoidosis from Other Causes of Lymphocytic Alveolitis.

    PubMed

    Bretagne, Lisa; Diatta, Ibrahima-Dina; Faouzi, Mohamed; Nobile, Antoine; Bongiovanni, Massimo; Nicod, Laurent P; Lazor, Romain

    2016-01-01

    The CD103 integrin is present on CD4+ lymphocytes of the bronchial mucosa, but not on peripheral blood CD4+ lymphocytes. It has been hypothesized that CD4+ lymphocytes in pulmonary sarcoidosis originate from redistribution from the peripheral blood to the lung, and therefore do not bear the CD103 integrin. Some data suggest that a low CD103+ percentage among bronchoalveolar lavage fluid (BALF) CD4+ lymphocytes discriminates between sarcoidosis and other diagnoses. To determine the diagnostic value of BALF CD103+ to identify sarcoidosis among other causes of alveolar lymphocytosis in a large retrospective case series. Among 391 consecutive bronchoalveolar lavages performed at our institution and analyzed by flow cytometry, we identified 207 cases, which were grouped into nine diagnostic categories: sarcoidosis, tuberculosis, non-tuberculous infections, hypersensitivity pneumonitis, non-specific interstitial pneumonia, organizing pneumonia, drug-induced lung diseases, other interstitial lung diseases (ILDs), and other diagnoses. To assess the discriminative value of the CD103+CD4+/CD4+ ratio to distinguish sarcoidosis from other entities, areas under ROC curves (AUC) were calculated. Sarcoidosis patients (n = 53) had significantly lower CD103+CD4+/CD4+ ratios than patients in other diagnostic categories. The AUC was 62% for sarcoidosis compared to all other diagnoses, and 69% for sarcoidosis compared to other ILDs. When combining CD103+CD4+/CD4+ and CD4+/CD8+ ratios, the AUC increased to 76 and 78%, respectively. When applying previously published cut-offs to our population, the AUC varied between 54 and 73%. The CD103+CD4+/CD4+ ratio does not accurately discriminate between sarcoidosis and other causes of lymphocytic alveolitis, neither alone nor in combination with the CD4+/CD8+ ratio, and is not a powerful marker for the diagnosis of sarcoidosis. © 2016 S. Karger AG, Basel.

  10. Antibiotic Resistance Among Ocular Pathogens in the United States: Five-Year Results From the Antibiotic Resistance Monitoring in Ocular Microorganisms (ARMOR) Surveillance Study.

    PubMed

    Asbell, Penny A; Sanfilippo, Christine M; Pillar, Christopher M; DeCory, Heleen H; Sahm, Daniel F; Morris, Timothy W

    2015-12-01

    The Antibiotic Resistance Monitoring in Ocular Microorganisms (ARMOR) study is the only ongoing nationwide antibiotic resistance surveillance program specific to ocular pathogens. To report resistance rates and trends among common ocular isolates collected during the first 5 years of the ARMOR study. This antibiotic resistance surveillance study was performed at an independent central laboratory. Clinical centers across the United States were invited to submit ocular isolates of Staphylococcus aureus, coagulase-negative staphylococci (CoNS), Streptococcus pneumoniae, Haemophilus influenzae, and Pseudomonas aeruginosa. Isolates were collected from January 1, 2009, through December 31, 2013, and analyzed from January 16 to May 15, 2015. Minimum inhibitory concentrations for various antibiotic classes were determined by broth microdilution according to the guidelines of the Clinical and Laboratory Standards Institute. Minimum inhibitory concentrations were interpreted as susceptible, intermediate, or resistant based on established break points. A total of 3237 ocular isolates (1169 S aureus, 992 CoNS, 330 S pneumoniae, 357 H influenzae, and 389 P aeruginosa) were collected from 72 centers. Methicillin resistance was found among 493 S aureus isolates (42.2%; 95% CI, 39.3%-45.1%) and 493 CoNS isolates (49.7%; 95% CI, 46.5%-52.9%), and methicillin-resistant (MR) isolates had a high probability of concurrent resistance to fluoroquinolones, aminoglycosides, or macrolides (P < .001). Multidrug resistance to at least 3 additional antibiotic classes was found in 428 MR S aureus isolates (86.8%) and 381 MRCoNS isolates (77.3%). All staphylococcal isolates were susceptible to vancomycin. Resistance among S pneumoniae isolates was highest for azithromycin (113 isolates [34.2%]) whereas resistance among P aeruginosa and H influenzae was low against the antibiotics tested. Staphylococcal isolates from elderly patients were more likely to be MR, as were S aureus isolates

  11. [Ocular changes in dialysis patients].

    PubMed

    Popa, M; Nicoară, S

    2000-01-01

    The study analyzes the ocular aspects in patients receiving hemodialysis, in order to define the importance of the ophthalmological exam as prognosis and follow-up parameter. The prospective study includes 84 patients with renal insufficiency who received hemodialysis between 1994-1998. The ocular aspects and their connection with the dialysis and the basic disease are described and analyzed. The most important were the retinal vascular complications: hypertensive retinopathy, anterior optic ischaemic neuropathy, central retinal artery occlusion, diabetic retinopathy.

  12. Cicatricial changes in ocular pemphigus

    PubMed Central

    Chirinos-Saldaña, P; Zuñiga-Gonzalez, I; Hernandez-Camarena, J C; Navas, A; Ramirez-Luquin, T; Robles-Contreras, A; Jimenez-Martinez, M C; Ramirez-Miranda, A; Bautista-de Lucio, V M; Graue-Hernandez, E O

    2014-01-01

    Purpose To describe the clinical characteristics of ocular involvement in patients with pemphigus at an ophthalmological referral center. Methods A retrospective review was conducted on patients with the immunopathological diagnosis of pemphigus examined between 1 January 2000 and 1 April 2010. Uncorrected distance visual acuity (UDVA), best corrected distance visual acuity (BCVA), ocular symptoms, and ocular surface inflammatory and scarring changes were assessed. Results A total of 15 patients were identified, with a mean age of 68.27±14.35 years, and 80% (n=12) were female. Extraocular involvement was reported in one patient. All of the eyes showed cicatricial changes in the conjunctiva. In all, 6 eyes (20%) were classified as stage I; 12 eyes (40%) as stage II; 10 eyes (33%) as stage III; and 2 eyes (7%) as stage IV. A statistically significant association was found between BCVA and the severity of ocular involvement. The mean BCVA logMAR was 1.66 (20/914), with a range from logMAR 0 (20/20) to logMAR 4 (NLP). Other ocular diseases were found in 8 (53.3%), systemic diseases in 10 (66.7%), and the use of pemphigus-inducing drugs in 10 patients (66.7%). Conclusions The present report represents the largest series of ocular involvement in pemphigus confirmed by immunopathology. The clinical manifestations varied from conjunctival hyperemia to corneal scarring and perforation. There was a strong association between scarring changes and low BCVA. Ocular and systemic diseases as well as the use of pemphigus-inducing drugs may predispose to ocular cicatricial changes observed in this series. PMID:24480839

  13. Ocular toxicity of targeted therapies.

    PubMed

    Renouf, Daniel J; Velazquez-Martin, Juan P; Simpson, Rand; Siu, Lillian L; Bedard, Philippe L

    2012-09-10

    Molecularly targeted agents are commonly used in oncology practice, and many new targeted agents are currently being tested in clinical trials. Although these agents are thought to be more specific and less toxic then traditional cytotoxic chemotherapy, they are associated with a variety of toxicities, including ocular toxicity. Many of the molecules targeted by anticancer agents are also expressed in ocular tissues. We reviewed the literature for described ocular toxicities associated with both approved and investigational molecularly targeted agents. Ocular toxicity has been described with numerous approved targeted agents and also seems to be associated with several classes of agents currently being tested in early-phase clinical trials. We discuss the proposed pathogenesis, monitoring guidelines, and management recommendations. It is important for oncologists to be aware of the potential for ocular toxicity, with prompt recognition of symptoms that require referral to an ophthalmologist. Ongoing collaboration between oncologists and ocular disease specialists is critical as the use of molecularly targeted agents continues to expand and novel targeted drug combinations are developed.

  14. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis.

    PubMed

    Harzallah, Amel; Kaaroud, Hayet; Boubaker, Karima; Barbouch, Samia; Goucha, Rim; Hamida, Fethi Ben; Abdallah, Taieb Ben

    2017-01-01

    Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

  15. Sarcoidosis with Pancreatic Mass, Endobronchial Nodules, and Miliary Opacities in the Lung.

    PubMed

    Matsuura, Shun; Mochizuka, Yasutaka; Oishi, Kyohei; Miyashita, Koichi; Naoi, Hyogo; Mochizuki, Eisuke; Mikura, Shinichiro; Tsukui, Masaru; Koshimizu, Naoki; Ohata, Akihiko; Suda, Takahumi

    2017-09-25

    Sarcoidosis affects multiple organs and rarely has unusual manifestations. A 78-year-old woman was referred to our hospital for coughing symptoms. A chest computed tomography (CT) scan revealed bilateral diffuse miliary patterns and right pleural effusion. Bronchoscopy showed multiple nodules in the carina and the bronchus intermedius. A CT scan of her abdomen revealed hypovascular lesions involving the pancreatic head and body. A transbronchial lung biopsy, bronchial mucosal biopsy, and endoscopic ultrasound-guided fine-needle aspiration of the pancreatic mass demonstrated non-caseating granulomas. We diagnosed the patient with sarcoidosis. She received no treatment for sarcoidosis and has been followed up for one year, during which no pulmonary disease progression had been observed and the pancreatic masses partially regressed.

  16. Diffuse Anterior Retinoblastoma with Sarcoidosis-Like Nodule

    PubMed Central

    Kitazawa, Koji; Nagata, Kenji; Yamanaka, Yukito; Kuwahara, Yasumichi; Iehara, Tomoko; Kinoshita, Shigeru; Sotozono, Chie

    2015-01-01

    Background Retinoblastomas account for 4% of malignancies in children, 1-2% of which are diffuse infiltrating retinoblastomas. Diffuse anterior retinoblastoma is rare and does not involve the retina. Here, we report on a diffuse anterior retinoblastoma with large sarcoidosis-like nodules on the iris that were responsive to anti-inflammatory therapy. Case We present a 6-year-old girl who had anterior uveitis with white nodules on the iris and posterior surface of the cornea in her right eye. The nodules initially responded well to anti-inflammatory treatment. However, anterior segment optical coherence tomography (AS-OCT) showed that the nodules gradually grew, shrinking the iris. We then collected the aqueous humor for diagnosis. A biopsy revealed clusters of small cells with a high nuclear-to-cytoplasm ratio with partial rosette formation. Therefore, we diagnosed diffuse anterior retinoblastoma without retinal involvement and performed enucleation of the right eye. The histopathology demonstrated undifferentiated cells similar to those seen on the biopsy, and tumor cells invaded the iris stroma, posterior surface of the cornea, ciliary body, and sclera. After the enucleation, she underwent chemotherapy and remains alive. Conclusion A differential diagnosis of retinoblastoma should be considered when white nodules refractory to anti-inflammatory therapy occur in the eye, even in the absence of obvious retinal masses. AS-OCT findings are useful in assessing retinoblastoma. PMID:26955346

  17. Extending Admixture Mapping to Nuclear Pedigrees: Application to Sarcoidosis

    PubMed Central

    McKeigue, Paul M.; Colombo, Marco; Agakov, Felix; Datta, Indrani; Levin, Albert; Favro, David; Gray-Montgomery, Courtney; Iannuzzi, Michael C.; Rybicki, Benjamin A.

    2013-01-01

    We describe statistical methods that extend the application of admixture mapping from unrelated individuals to nuclear pedigrees, allowing existing pedigree-based collections to be fully exploited. Computational challenges have been overcome by developing a fast algorithm that exploits the factorial structure of the underlying model of ancestry transitions. This has been implemented as an extension of the program ADMIXMAP. We demonstrate the application of the method to a study of sarcoidosis in African Americans that has previously been analyzed only as an admixture mapping study restricted to unrelated individuals. Although the ancestry signals detected in this pedigree analysis are generally similar to those detected in the earlier analysis of unrelated cases, we are able to extract more information and this yields a much sharper exclusion map; using the classical criterion of an LOD score of minus 2, the pedigree analysis is able to exclude a risk ratio of 2 or more associated with African ancestry over 96% of the genome, compared with only 83% in the earlier analysis of unrelated individuals only. Although the pedigree extension of ADMIXMAP can use ancestry-informative markers only at relatively low density, it can use imputed ancestry states from programs such as WINPOP or HAPMIX that use dense SNP marker genotypes for admixture mapping. This extends both the efficiency and the range of application of this powerful gene mapping method. PMID:23371909

  18. TL1A/DR3 axis involvement in the inflammatory cytokine network during pulmonary sarcoidosis.

    PubMed

    Facco, M; Cabrelle, A; Calabrese, F; Teramo, A; Cinetto, F; Carraro, S; Martini, V; Calzetti, F; Tamassia, N; Cassatella, M A; Semenzato, G; Agostini, C

    2015-01-01

    TNF-like ligand 1A (TL1A), a recently recognized member of the TNF superfamily, and its death domain receptor 3 (DR3), firstly identified for their relevant role in T lymphocyte homeostasis, are now well-known mediators of several immune-inflammatory diseases, ranging from rheumatoid arthritis to inflammatory bowel diseases to psoriasis, whereas no data are available on their involvement in sarcoidosis, a multisystemic granulomatous disease where a deregulated T helper (Th)1/Th17 response takes place. In this study, by flow cytometry, real-time PCR, confocal microscopy and immunohistochemistry analyses, TL1A and DR3 were investigated in the pulmonary cells and the peripheral blood of 43 patients affected by sarcoidosis in different phases of the disease (29 patients with active sarcoidosis, 14 with the inactive form) and in 8 control subjects. Our results demonstrated a significant higher expression, both at protein and mRNA levels, of TL1A and DR3 in pulmonary T cells and alveolar macrophages of patients with active sarcoidosis as compared to patients with the inactive form of the disease and to controls. In patients with sarcoidosis TL1A was strongly more expressed in the lung than the blood, i.e., at the site of the involved organ. Additionally, zymography assays showed that TL1A is able to increase the production of matrix metalloproteinase 9 by sarcoid alveolar macrophages characterized, in patients with the active form of the disease, by reduced mRNA levels of the tissue inhibitor of metalloproteinase (TIMP)-1. These data suggest that TL1A/DR3 interactions are part of the extended and complex immune-inflammatory network that characterizes sarcoidosis during its active phase and may contribute to the pathogenesis and to the progression of the disease.

  19. Triggering receptor expressed on myeloid cells-1 and 2 in bronchoalveolar lavage fluid in pulmonary sarcoidosis.

    PubMed

    Suchankova, Magda; Bucova, Maria; Tibenska, Elena; Tedlova, Eva; Demian, Juraj; Majer, Ivan; Novosadova, Helena; Tedla, Miroslav; Paulovicova, Ema; Kantarova, Daniela

    2013-04-01

    Pulmonary sarcoidosis (PS) is characterized by the formation of granulomas in the lungs and has been associated with infection by microorganisms. Triggering receptor expressed on the surface of myeloid cells (TREM)-1 is overexpressed in response to infection while TREM-2 is involved in granuloma formation. We hypothesized that these receptors are overexpressed in PS and might be useful for diagnostic testing. Cell surface TREM-1 and TREM-2 expression in cells obtained at bronchoalveolar lavage (BAL) was measured in individuals with sarcoidosis (n = 26) and compared with that seen in individuals with other interstitial lung diseases (ILD) (n = 27). TREM-1 and TREM-2 expression was significantly increased in sarcoidosis compared with other ILD: total number of TREM-1, P = 0.0039 (23.81 vs 13.50 cells/μl), TREM-2, P < 0.0001 (32.81 vs 7.76 cells/μl); percentage of TREM-1: P = 0.0002 (41.30% vs 15.70%), TREM-2: P < 0.0001 (34% vs 9.60%); and mean fluorescence of TREM-1: P = 0.0005 (5.43 vs 1.96), TREM-2: P = 0.0011 (6.85 vs 2.77). Increase in both of these receptors seems to be typical for PS. In discriminating sarcoidosis from other ILD, the specificity (96%) and sensitivity (72%) of the combination of TREM-1 and TREM-2 was high. Increased TREM-1 and TREM-2 cell surface expression is observed in sarcoidosis. Evaluation of BAL cell expression of both of these receptors may serve as a diagnostic marker for sarcoidosis. © 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology.

  20. Relationship between radiologic patterns, pulmonary function values and bronchoalveolar lavage fluid cells in newly diagnosed sarcoidosis

    PubMed Central

    Zeleckienė, Ingrida; Matačiūnas, Mindaugas; Puronaitė, Roma; Jurgauskienė, Laimutė; Malickaitė, Radvilė; Strumilienė, Edita; Gruslys, Vygantas; Zablockis, Rolandas; Danila, Edvardas

    2017-01-01

    Background The aim of the present study was to identify specious radiologic and/or physiologic prognostic marker(s), which lead to optimize of the patient follow-up frequency. Methods Eighty consecutive patients with newly diagnosed pulmonary sarcoidosis. Patients underwent chest radiography, high-resolution computed tomography (HRCT) examination, pulmonary function tests (PFT), bronchoscopy with bronchoalveolar lavage (BAL) and lung biopsy, and bronchoalveolar lavage fluid (BALF) cell examination. Results The reduction in PFT values seen in radiological sarcoidosis stage III was greater than that seen in stages I and II. The percentage of neutrophils in the lungs was found to increase in stages II and III. PFT indices were correlated negatively with the consolidation and ground glass opacities CT scores, but not with the micronodule or macronodule scores. The rise in the percentage of BALF lymphocytes was associated with the restriction pattern of PFT. The diagnostic value of BALF for sarcoidosis was higher when the typical radiologic patterns of stage I disease were found and that smoking decreased the diagnostic value of CD4/CD8 ratio. Conclusions This study supports the opinion that the staging of the pulmonary sarcoidosis with chest X-rays is still valuable from the prognostic point of view, because significant correlations between the radiologic stages of sarcoidosis and PFT parameters were found. Chest HRCT was significantly superior to chest X-ray in detecting mediastinal and pulmonary parenchymal changes. However, the prognostic role of HRCT needs to be better investigated evaluating serial examinations. Only consolidation and ground glass scores (neither of which are frequently found in sarcoidosis) hold prognostic value, since these were negatively correlated with PFT parameters. PMID:28203410