Science.gov

Sample records for ocular adnexal igg4-related

  1. [Diagnosis of IgG4-related systemic disease in a patient with an ocular tumor associated with lung nodules].

    PubMed

    Vandenbos, F; Benchetrit, M; Tieulié, N; Ambrosetti, D; Chanalet, S; Burel-Vandenbos, F

    2012-12-01

    The IgG4-related systemic disease is a recently described entity of fibro-inflammatory systemic damage. Although initially described in some forms of pancreatitis, the disease can affect all organs. The common histological features include a lymphoplasmacytic infiltration (especially to IgG4), fibrosis and phlebitis. Elevated serum level of IgG4 is also often present. This rare but certainly underdiagnosed disease must be kept in mind of all clinician faced to a non-specific inflammatory lesion. We report a case of ocular inflammation and lung tumors in a patient of 84 years for which the diagnosis was made through immunolabelling with IgG4 in lesions biopsied. PMID:23159548

  2. Treatment options for ocular adnexal lymphoma (OAL)

    PubMed Central

    Cohen, Victoria Mary Lendrum

    2009-01-01

    Most lymphomas that involve the ocular adnexal structure are low grade, B cell, non-Hodgkin’s lymphomas. The treatment depends upon the grade and stage of the disease. High grade lymhoma requires treatment with systemic chemotherapy whereas the localized low grade (extranodal marginal zone lymphoma) can be successfully managed with local radiotherapy. Chlamydia psittaci infection is associated with low grade ocular lymphoma; however there is wide geographic variation in the strength of this association. Blanket antibiotic therapy is not advised unless there is proof of an infective agent. The monoclonal antibody, rituximab, may be successful for CD20 positive lymphoma, although it is likely that rituximab will have better long-term results when used in combination with systemic chemotherapy. PMID:20054418

  3. IgG4-related spinal pachymeningitis.

    PubMed

    Lu, Zhang; Tongxi, Liu; Jie, Luo; Yujuan, Jiao; Wei, Jiang; Xia, Liu; Yumin, Zheng; Xin, Lu

    2016-06-01

    The aim of this study is to study the clinical, laboratory, imaging pathology, and prognosis features of IgG4-related spinal pachymeningitis. We worked with a 55-year-old man suffering from IgG4-related spinal pachymeningitis who had the most widespread lesion in his dura mater. We also review previous related studies and discuss the clinical characteristics of this rare disease. In total, eight IgG4-related spinal pachymeningitis patients have been reported in the literature since 2009. They were mostly male patients, 51.7 ± 11.9 years old on average. Cervical and thoracic vertebrae were the most common sites for lesions. The most prominent symptom was varying numbness and weakness of the limbs and/or body associated with spinal cord compression. There was one patient (1/5) with elevated serum IgG4 levels and three patients (3/3) with increased cerebrospinal fluid (CSF) IgG4 index. Positive histopathologic findings are the strongest basis for a diagnosis. All the patients with IgG4-related spinal pachymeningitis responded well to glucocorticoid therapy. IgG4-related spinal pachymeningitis is an orphan disease that mainly occurs in cervical and thoracic vertebrae. Older males are the most susceptible group. Serum IgG4 levels were consistently normal in these cases, so analysis of CSF for IgG4 production (IgG4 index) could become a useful tool. Pathological findings remain the gold standard for diagnosis. Most patients responded favorably to glucocorticoid treatment. PMID:26567899

  4. Current concepts of ocular adnexal surgery

    PubMed Central

    Borrelli, Maria; Geerling, Gerd

    2013-01-01

    Ophthalmic Plastic and Reconstructive Surgery is a specialized area of ophthalmology that deals with the management of deformities and abnormalities of the eyelids, lacrimal system and the orbit. An ophthalmoplastic surgeon is able to identify and correct abnormalities of the ocular adnexae such as ectropion, lid retraction, conjunctival scarring with severe entropion, that can cause secondary ocular surface disorders; manage patients with watering eye, and when needed intervene with a dacryocystorhinostomy by external or endonasal approach and moreover minimize disfigurement following enucleation or evisceration and prevent further corneal damage, alleviate complains of tearing and grittiness, but also cosmetic complaints in patients with Graves’ orbitopathy. Aim of this manuscript was to review current established and recently evolving surgical procedures. PMID:26504698

  5. IgG4-related skin disease.

    PubMed

    Tokura, Y; Yagi, H; Yanaguchi, H; Majima, Y; Kasuya, A; Ito, T; Maekawa, M; Hashizume, H

    2014-11-01

    IgG4-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4(+) plasma cells. IgG4-RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4-RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4-mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis-like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1-3 are induced by direct infiltration of IgG4(+) plasma cells, while the other types (4-7) are caused by secondary mechanisms. IgG4-related skin disease is defined as IgG4(+) plasma-cell-infiltrating skin lesions that form plaques, nodules or tumours (types 1-3), but may manifest secondary lesions caused by IgG4(+) plasma cells and/or IgG4 (types 4-7). PMID:25065694

  6. Ocular adnexal non-Hodgkin’s lymphoma: a review of epidemiology and risk factors

    PubMed Central

    Moslehi, Roxana; Schymura, Maria J; Nayak, Seema; Coles, F Bruce

    2013-01-01

    Ocular adnexal non-Hodgkin’s lymphoma (NHL), the most common form of ophthalmic NHL, has a unique incidence pattern showing a steady and rapid increase in the past few decades, nearly equal rates among both genders, and predominance among Asians/Pacific Islanders. No major cause for ocular adnexal NHL has been identified, although infectious agents, immune disorders and genetic/epigenetic factors have all been implicated in its etiology. Identifying putative risk factors and biologic mechanisms leading to carcinogenesis in ocular adnexal NHL may enable implementation of effective preventive and/or therapeutic approaches for this malignancy. This article summarizes current knowledge on epidemiology of ocular adnexal NHL and the role of various potential risk factors in its etiology. PMID:23976898

  7. IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis.

    PubMed

    Kawano, Mitsuhiro; Yamada, Kazunori

    2016-08-01

    Immunoglobulin G4-related kidney disease (IgG4-RKD) is the collective name encompassing renal parenchymal and renal pelvic lesions. The hallmark of renal parenchymal lesions of IgG4-related disease is plasma cell-rich tubulointerstitial nephritis with numerous IgG4-positive plasma cells and characteristic fibrosis. In addition, glomerular lesions are sometimes present, with membranous glomerulonephritis being the most common. Although IgG4-RKD shows good responsiveness to corticosteroid therapy, follow-up imaging studies have revealed that partial cortical scars persist when the start of therapy is delayed. In this review, the authors provide an overview of the latest knowledge of IgG4-RKD, focusing in particular on its pathological and imaging characteristic features. PMID:27466797

  8. Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease.

    PubMed

    Nakanuma, Yasuni; Ishizu, Yoji; Zen, Yoh; Harada, Kenichi; Umemura, Takeji

    2016-08-01

    Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system. Two types of hepatic parenchymal involvement have been reported in IgG4-RD: IgG4-related autoimmune hepatitis (AIH) and IgG4-hepatopathy. Moreover, only three cases of IgG4-related AIH have been reported. Immunoglobulin G4-related AIH is clinicopathologically similar to AIH, except for an elevated serum IgG4 level and heavy infiltration of IgG4-positive plasma cells in the liver tissue. Interestingly, IgG4-related AIH can be complicated by well-known IgG4-RD(s). Immunoglobulin G4-hepatopathy, which includes various histopathological lesions encountered in the liver of patients with type I autoimmune pancreatitis, is classified into five histological categories: portal inflammation, large bile duct damage, portal sclerosis, lobular hepatitis, and cholestasis. Immunoglobulin G4-hepatopathy is currently a collective term covering hepatic lesions primarily or secondarily related to IgG4-related sclerosing cholangitis and type 1 autoimmune pancreatitis. In conclusion, the liver is not immune to IgG4-RD, and at least two types of hepatic involvement in IgG4-RD have been reported: IgG4-related AIH and IgG4-hepatopathy. Additional studies are required to clarify their precise clinical significance with respect to IgG4-RD and inherent liver diseases. PMID:27466793

  9. The Role of Infectious Agents in the Etiology of Ocular Adnexal Neoplasia

    PubMed Central

    Verma, Varun; Shen, Defen; Sieving, Pamela C.; Chan, Chi-Chao

    2008-01-01

    Given the fact that infectious agents contribute to around 18% of human cancers worldwide, it would seem prudent to explore their role in neoplasms of the ocular adnexa: primary malignancies of the conjunctiva, lacrimal glands, eyelids, and orbit. By elucidating the mechanisms by which infectious agents contribute to oncogenesis, the management, treatment, and prevention of these neoplasms may one day parallel what is already in place for cancers such as cervical cancer, hepatocellular carcinoma, gastric mucosa-associated lymphoid tissue lymphoma and gastric adenocarcinoma. Antibiotic treatment and vaccines against infectious agents may herald a future with a curtailed role for traditional therapies of surgery, radiation, and chemotherapy. Unlike other malignancies for which large epidemiological studies are available, analyzing ocular adnexal neoplasms is challenging as they are relatively rare. Additionally, putative infectious agents seemingly display an immense geographic variation that has led to much debate regarding the relative importance of one organism versus another. This review discusses the pathogenetic role of several microorganisms in different ocular adnexal malignancies, including human papilloma virus in conjunctival papilloma and squamous cell carcinoma, human immunodeficiency virus in conjunctival squamous carcinoma, Kaposi sarcoma-associated herpes virus or human herpes simplex virus-8 (KSHV/HHV-8) in conjunctival Kaposi sarcoma, Helicobacter pylori (H. pylori,), Chlamydia, and hepatitis C virus in ocular adnexal mucosa-associated lymphoid tissue lymphomas. Unlike cervical cancer where a single infectious agent, human papilloma virus, is found in greater than 99% of lesions, multiple organisms may play a role in the etiology of certain ocular adnexal neoplasms by acting through similar mechanisms of oncogenesis, including chronic antigenic stimulation and the action of infectious oncogenes. However, similar to other human malignancies

  10. Topiramate-induced maculopathy in IgG4-related disease

    PubMed Central

    DaCosta, Joanna; Younis, Saad

    2016-01-01

    This report describes a case of reversible topiramate-induced maculopathy in a 32-year-old female patient with IgG4-related disease. The patient presented with decreased vision associated with anterior uveitis and cystoid macula edema, which was unresponsive to oral and topical steroids. Following topiramate cessation, both cystoid macula edema and vision improved. The ocular side effects of topiramate and putative pharmacological mechanisms for topiramate-induced maculopathy in the context of IgG4-related disease are discussed. This report highlights that neurologists and ophthalmologists should be aware that patients presenting with topiramate-associated maculopathy should be advised to discontinue topiramate promptly to prevent irreversible loss of vision. PMID:27354829

  11. IgG4-Related Disease in a Urachal Tumor.

    PubMed

    Dum, Travis W; Zhang, Da; Lee, Eugene K

    2014-01-01

    IgG4-related disease is a newly recognized fibroinflammatory disorder that has the ability to affect nearly every organ system. It is characterized by tumefactive lesions and fibrosis and closely mimics neoplasms. Only one case of IgG4-related bladder mass has been reported in the literature, but there are no reports of IgG4-related disease in a urachal mass. Herein, we report a 26-year-old male who initially presented with symptoms of recurrent UTI. Work-up revealed a 6 cm urachal tumor, a 1.4 cm pulmonary lesion, and mediastinal lymphadenopathy; all metabolically active on PET scan and suspicious for urachal adenocarcinoma. Lung lesion fine needle aspiration and TURBT pathology revealed inflammation but no evidence of malignancy. The patient underwent a partial cystectomy and umbilectomy with pathology demonstrating dense plasmacytic cells, a high rate of immunohistochemistry staining positive for IgG4 plasma cells, a storiform pattern of fibrosis, and an obliterative phlebitis. Furthermore, the patient had an elevated serum IgG4 level of 227 mg/dL (range 2.4-121 mg/dL). IgG4-related disease is a newly recognized fibroinflammatory disorder that can mimic neoplastic processes and a high index of suspicion and accurate tissue pathology is necessary for an accurate diagnosis. PMID:25202466

  12. IgG4-related disease of the rectum

    PubMed Central

    Choi, Sung-Bong; Lim, Chul-Hyun; Cha, Myung-Guen

    2016-01-01

    IgG4-related disease is a relatively new disease entity characterized by elevated serum IgG4 levels and marked infiltration of IgG4-positive plasma cells in lesions. Organ enlargement or nodular lesions consisting of abundant infiltration of lymphocytes and IgG4-positive plasma cells and fibrosis are seen in various organs throughout. We encountered a patient with an inflammatory pseudotumor of the rectum, which was histopathologically confirmed to be an IgG4-related disease. The patient was a 28-year-old woman who had constipation for 3 months. The endoluminal ultrasonography showed a lesion that was heterogeneous and low echogenic in lower rectum. The result of colonoscopic biopsy findings was of chronic proctitis with lymphoid aggregates. For a confirmative diagnosis, excision was performed. Histopathological examination represented plasma cell infiltration and fibrosis. Immunohistochemistry revealed prominence of IgG4-positive plasma cells and confirmed the diagnosis of IgG4-related disease. The patient is currently under observation on low-dose oral prednisolone without relapse. PMID:27186575

  13. Large vessel involvement by IgG4-related disease

    PubMed Central

    Perugino, Cory A.; Wallace, Zachary S.; Meyersohn, Nandini; Oliveira, George; Stone, James R.; Stone, John H.

    2016-01-01

    Abstract Objectives: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions. Reports have described inflammatory aortitis and periaortitis, the latter in the setting of retroperitoneal fibrosis (RPF), but have not distinguished adequately between these 2 manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly defined. We describe the clinical features, radiology findings, and treatment response in a cohort of 36 patients with IgG4-RD affecting large blood vessels. Methods: Clinical records of all patients diagnosed with IgG4-RD in our center were reviewed. All radiologic studies were reviewed. We distinguished between primary large blood vessel inflammation and secondary vascular involvement. Primary involvement was defined as inflammation in the blood vessel wall as a principal focus of disease. Secondary vascular involvement was defined as disease caused by the effects of adjacent inflammation on the blood vessel wall. Results: Of the 160 IgG4-RD patients in this cohort, 36 (22.5%) had large-vessel involvement. The mean age at disease onset of the patients with large-vessel IgG4-RD was 54.6 years. Twenty-eight patients (78%) were male and 8 (22%) were female. Thirteen patients (36%) had primary IgG4-related vasculitis and aortitis with aneurysm formation comprised the most common manifestation. This affected 5.6% of the entire IgG4-RD cohort and was observed in the thoracic aorta in 8 patients, the abdominal aorta in 4, and both the thoracic and abdominal aorta in 3. Three of these aneurysms were complicated by aortic dissection or contained perforation. Periaortitis secondary to RPF accounted for 27 of 29 patients (93%) of secondary vascular involvement by IgG4-RD. Only 5 patients demonstrated evidence of both primary and secondary blood vessel involvement. Of those treated with rituximab, a majority responded positively. Conclusions: IgG4-RD is a distinctive, unique, and treatable cause of large-vessel vasculitis. It can also involve blood vessels secondary to perivascular tumefactive lesions. The most common manifestation of IgG4-related vasculitis is aortitis with aneurysm formation. The most common secondary vascular manifestation is periaortitis with relative sparing of the aortic wall. Both primary vasculitis and secondary vascular involvement respond well to B cell depletion therapy. PMID:27428181

  14. IgG4-related Orbital Disease and Its Mimics in a Western Population.

    PubMed

    Ferry, Judith A; Klepeis, Veronica; Sohani, Aliyah R; Harris, Nancy Lee; Preffer, Frederic I; Stone, John H; Grove, Arthur; Deshpande, Vikram

    2015-12-01

    Although chronic inflammatory disorders of the ocular adnexa are relatively common, their pathogenesis is in many cases poorly understood. Recent investigation suggests that many cases of sclerosing orbital inflammation are a manifestation of IgG4-related disease; however, most patients reported have been Asian, and it is not clear whether the results of studies from the Far East can be reliably extrapolated to draw conclusions about Western patients. We evaluated 38 cases previously diagnosed as orbital inflammatory pseudotumor or chronic dacryoadenitis to determine whether our cases fulfill the criteria for IgG4-RD (IgG4-related dacryoadenitis when involving the lacrimal gland, and IgG4-related sclerosing orbital inflammation when involving orbital soft tissue). Fifteen patients had IgG4-related dacryoadenitis or orbital inflammation. These patients included 9 men and 6 women, aged 24 to 77 years (median, 64 y). Lesions involved orbital soft tissue (8 cases), lacrimal gland (6 cases), and canthus (1 case). In 1 case, focal in situ follicular neoplasia was seen in a background of IgG4-RD. In another case, a clonal IGH gene rearrangement was detected. Four patients with IgG4-RD had evidence of IgG4-RD in other anatomic sites. Five patients, 1 man and 4 women, aged 26 to 74 years (median 50 y) had orbital lesions (2 involving lacrimal gland, 3 involving soft tissue) suspicious for, but not diagnostic of, IgG4-RD. Of 16 patients with IgG4-RD or probable IgG4-RD with information available regarding the course of their disease, 11 patients experienced recurrent or persistent orbital disease. However, no patient developed lymphoma, and no patient died of complications of IgG4-RD. Eighteen patients had lesions not representing IgG4-RD. They included 6 male and 12 female individuals aged 6 to 77 years (median, 47 y). These patients had a variety of diseases, including granulomatosis with polyangiitis (3 cases), Rosai-Dorfman disease (1 case), nonspecific chronic

  15. IgG4-Related Disease: A Multispecialty Condition

    PubMed Central

    da Fonseca, Emanuela Pimenta; Santiago, Mittermayer Barreto

    2014-01-01

    IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases. PMID:25506457

  16. Therapeutic approach to IgG4-related disease

    PubMed Central

    Brito-Zerón, Pilar; Kostov, Belchin; Bosch, Xavier; Acar-Denizli, Nihan; Ramos-Casals, Manuel; Stone, John H.

    2016-01-01

    Abstract To review the reported evidence on the therapeutic management of IgG4-related disease (IgG4-RD) in clinical practice. A systematic search of the literature was conducted. The primary outcome measured was the rate of efficacy of first-line therapeutic approaches. Secondary outcomes measured included the rate of disease relapse, the outcome of untreated patients, the rate of patients without drug therapy at the end of follow-up, the rate of side effects, and mortality. The MOOSE, AHRQ, STROBE, and GRACE recommendations/statements were followed. The results of the systematic search strategy yielded 62 studies that included a total of 3034 patients. Complete information about first-line therapeutic regimens was detailed in 1952 patients, including glucocorticoid-based regimens in 1437 (74%), drug-free regimens in 213 (11%), and other therapies in 38 (2%). No therapy (wait and see management) was reported in 264 (13%) patients. The efficacy of monotherapy with glucocorticoids was specified in 1220 patients, of whom 97% had a therapeutic response. Relapses, however, were reported in 464/1395 (33%) patients despite typically short follow-up periods. Therapeutic efficacy was reported in 219/231 (95%) of relapses treated with glucocorticoids, 56/69 (81%) of those treated with azathioprine, 16/22 (72%) of those treated with other immunosuppressive agents, and in the 9 cases treated with rituximab (100%). In 14 studies, the authors detailed the outcome of 159/246 patients with wait-and-see management; spontaneous improvement or resolution was reported in 68 (43%) cases. Wide heterogeneity was observed with respect to the first-line therapeutic approaches used for the different organ-specific disease subsets, including significant differences in the mean dose of glucocorticoids used. Nearly 70% of reported IgG4-RD patients are treated with oral glucocorticoids in monotherapy. However, the therapeutic management is heavily influenced by geographical, epidemiological, and clinical factors, especially with respect to the predominant organ affected. The frequency of glucocorticoid failure to induce sustained remissions both during and after treatment and the assessment of glucocorticoid toxicity in IgG4-RD require further study. PMID:27368010

  17. Molecular and Genomic Aberrations in Chlamydophila psittaci Negative Ocular Adnexal Marginal Zone Lymphomas

    PubMed Central

    Zhu, Daxing; Ikpatt, Offiong F; Dubovy, Sander R; Lossos, Chen; Natkunam, Yasodha; Chapman-Fredricks, Jennifer R.; Fan, Yao-Shan; Lossos, Izidore S.

    2013-01-01

    The etiology and pathogenesis of ocular adnexal extranodal marginal zone lymphoma (OAEMZL) are still unknown and the association with Chlamydophila psittaci (C. psittaci) has been shown in only some geographic regions. Herein we comprehensively examined the frequency of chromosomal translocations as well as CARD11, MYD88 (L265P) and A20 mutations /deletions in 45 C. psittaci negative OAEMZLs. t(14;18)(q32;q21) IGH-MALT1 and t(11;18)(q21;q21) API2-MALT1 were not detected in any of the analyzed tumors while 3 tumors harbored IGH translocations to an unidentified partner. CARD11 mutations were not found in all the analyzed tumors while MYD88 L265P mutation was detected in 3 (6.7%) tumors. A20 mutations and deletions were each detected in 7(15.6%) and 6(13.3%) of the tumors, respectively. Therefore, the observed genetic aberrations could account for the activation of NF-kB signaling pathway in only a minority of the cases. Further studies are needed to identify the molecular mechanisms underlying the pathogenesis of OAEMZL. PMID:23720088

  18. Chlamydophila psittaci-negative ocular adnexal marginal zone lymphomas express self polyreactive B-cell receptors.

    PubMed

    Zhu, D; Bhatt, S; Lu, X; Guo, F; Veelken, H; Hsu, D K; Liu, F-T; Alvarez Cubela, S; Kunkalla, K; Vega, F; Chapman-Fredricks, J R; Lossos, I S

    2015-07-01

    The pathogenesis of Chlamydophila psittaci-negative ocular adnexal extranodal marginal zone lymphomas (OAEMZLs) is poorly understood. OAEMZLs are monoclonal tumors expressing a biased repertoire of mutated surface immunoglobulins. Antigenic activation of the B-cell receptor (BCR) may have a role in the pathogenesis of these lymphomas. We have analyzed the reactivity of recombinant OAEMZL immunoglobulins. OAEMZL antibodies reacted with self-human antigens, as demonstrated by enzyme-linked immunosorbent assays, HEp-2 immunofluorescence and human protein microarrays. All the analyzed recombinant antibodies (rAbs) exhibited polyreactivity by comprehensive protein array antibody reactivity and some rAbs also demonstrated rheumatoid factor activity. The identity of several reactive antigens was confirmed by microcapillary reverse-phase high-performance liquid chromatography nano-electrospray tandem mass spectrometry. The tested rAbs frequently reacted with shared intracellular and extracellular self-antigens (for example, galectin-3). Furthermore, these self-antigens induced BCR signaling in B cells expressing cognate surface immunoglobulins derived from OAEMZLs. These findings indicate that interactions between self-antigens and cognate OAEMZL tumor-derived BCRs are functional, inducing intracellular signaling. Overall, our findings suggest that self-antigen-induced BCR stimulation may be implicated in the pathogenesis of C. psittaci-negative OAEMZLs. PMID:25676418

  19. IgG4-related disease in the sinonasal cavity accompanied by intranasal structure loss.

    PubMed

    Inoue, Akiko; Wada, Kota; Matsuura, Kentaro; Osafune, Hiroshi; Ida, Yutaro; Kosakai, Arifumi; Edamatsu, Hideo

    2016-02-01

    IgG4-related disease was recently proposed under the classification of systemic chronic inflammatory disease. In the field of otolaryngology, organ-specific diagnostic criteria have been established for the occurrence of this condition in the salivary glands, but not in the sinonasal cavity. Here we report a case involving a 70-year-old man with IgG4-related disease in the sinonasal cavity. The patient, with the chief complaint of nasal bleeding, first visited a physician. However, the patient experienced recurrent bleeding with intranasal structure loss and was subsequently referred to our hospital. His IgG4 level was elevated, and histopathological examination of a tissue sample obtained from the edematous sphenoid sinus showed increased IgG4-positive plasma cells and storiform fibrosclerosis. A definitive diagnosis of IgG4-related rhinosinusitis was made on the basis of comprehensive criteria for IgG4-related disease. The disease showed a progressively destructive course that was clearly different from that of chronic sinusitis and represented a very rare case of IgG4-related rhinosinusitis. IgG4-related disease originating in the sinonasal cavity is rare, and, to the best of our knowledge, this is the first primary case of IgG4-related disease that originated in one side of the sinonasal cavity and showed progressive destruction. PMID:26104224

  20. Retroperitoneal disorders associated with IgG4-related autoimmune pancreatitis.

    PubMed

    Hara, Noboru; Kawaguchi, Makoto; Takeda, Keisuke; Zen, Yoh

    2014-11-28

    IgG4-related autoimmune pancreatitis is frequently accompanied by relevant lesions in the genitourinary tract and retroperitoneal organs, which cause various clinical problems, ranging from non-specific back pain or bladder outlet obstruction to renal failure. The diagnosis of IgG4-related retroperitoneal fibrosis requires a multidisciplinary approach, including serological tests, histological examination, imaging analysis, and susceptibility to steroid therapy. Radiological examinations are helpful to diagnose this condition, but surgical resection is occasionally unavoidable to exclude malignancy, particularly for patients with isolated retroperitoneal involvement. Steroid therapy is the treatment of choice for this condition, the same as for other manifestations of IgG4-related disease. For patients with severe ureteral obstruction, additional ureteral stenting needs to be considered prior to steroid therapy to preserve the renal function. Some papers have suggested that IgG4-related disease can affect male reproductive organs including the prostate and testis. IgG4-related prostatitis usually causes lower urinary tract symptoms, such as dysuria and pollakisuria. Patients sometimes state that corticosteroids given for IgG4-related disease at other sites relieve their lower urinary tract symptoms, which leads us to suspect prostatic involvement in this condition. Because of the limited number of publications available, further studies are warranted to better characterize IgG4-related disease in male reproductive organs. PMID:25469023

  1. Characteristics, diagnosis and therapeutic strategies for IgG4-related orbital disease.

    PubMed

    Oles, Krzysztof; Sładzień, Jacek; Bartuś, Krzysztof; Leszczyńska, Joanna; Bojanowska, Emila; Krakowczyk, Łukasz; Mika, Joanna

    2016-06-01

    Thanks to detailed studies conducted in recent years, a new disease syndrome was identified in 2001. It is known as a IgG4-related disease and its differentiation is based on the analysis of IgG4 levels in the affected tissues. The IgG4-related disease is considered to be a generalized pathological process involving a wide spectrum of various disorders that may affect distant organs. Orbital IgG4-related disease is a recently reported issue that may prove important for the elucidation of the etiology of idiopathic, lymphoplasmacytic or fibrotic disorders of various organs, including the orbits. In this article, we are describing epidemiology and differential diagnostics of IgG4-related orbital disease with particular focus on pseudotumors, MALT lymphomas and lymphocyte/plasma cell infiltrations of the orbit. We are also discussing therapeutic possibilities currently available in the management of the disease. PMID:27116895

  2. Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

    PubMed Central

    Kim, Seokhwi; Bae, Hyunsik; Choi, Misun; Kim, Binnari; Heo, Jin Seok; Kim, Ho Seong; Choi, Seung Hee; Jang, Kee-Taek

    2016-01-01

    IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease. PMID:26755360

  3. IgG4-related disease manifesting as an acute gastric-pericardial fistula.

    PubMed

    Frydman, James; Grunner, Shahar; Kluger, Yoram

    2014-11-28

    IgG4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of IgG4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the IgG4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for IgG4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to IgG4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of IgG4-related disease. PMID:25469052

  4. IgG4-related disease manifesting as an acute gastric-pericardial fistula

    PubMed Central

    Frydman, James; Grunner, Shahar; Kluger, Yoram

    2014-01-01

    IgG4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of IgG4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the IgG4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for IgG4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to IgG4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of IgG4-related disease. PMID:25469052

  5. Nontuberculous mycobacterial infection with concurrent IgG4-related lymphadenopathy.

    PubMed

    Liu, Ting-Ting; Weng, Shao-Wen; Wang, Ming-Chung; Huang, Wan-Ting

    2016-03-01

    Disseminated nontuberculous mycobacteria (NTM) infection with concurrent IgG4-related lymphadenopathy has not been reported. We described a patient with neutralizing autoantibodies to interferon-gamma (IFN-γ) and elevated levels of serum IgG4 presenting with generalized lymphadenopathy and reactive dermatosis. Histologically, lymph nodes (LNs) showed effaced nodal architecture with polymorphic infiltrates, mimicking angioimmunoblastic T-cell lymphoma. Both the absolute number and the ratio of IgG4+ plasma cells to IgG+ plasma cells were increased. Mycobacterium abscessus was isolated from cultures of LNs, and demonstrated by polymerase chain reaction-restriction fragment length polymorphism. The skin biopsy showed neutrophilic dermatosis, consistent with Sweet syndrome. The patient met the criteria of both adult-onset immunodeficiency syndrome and IgG4-related lymphadenopathy. This case provides evidence of disseminated NTM infection with concurrent type III IgG4-related lymphadenopathy in the patient with anti-IFN-γ autoantibodies. PMID:26660641

  6. Coexistence of Acute Crescent Glomerulonephritis and IgG4-Related Kidney Disease

    PubMed Central

    Lu, Zeyuan; Yin, Jianyong; Bao, Hongda; Jiao, Qiong; Wu, Huijuan; Wu, Rui; Xue, Qin; Wang, Niansong; Zhang, Zhigang; Wang, Feng

    2016-01-01

    Introduction IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that may involve almost each organ or system. IgG4-related kidney disease (IgG4-RKD) refers to renal lesions associated with IgG4-RD. The most frequent morphological type of renal lesions is IgG4-related tubulointerstitial nephritis (IgG4-TIN) which is associated with increased IgG4-positive plasma cell infiltration and interstitial fibrosis. Case Report Herein, we present a rare case with coexisting IgG4-RKD and acute crescent glomerulonephritis with concomitant severe tubulointerstitial lesions instead of classic IgG4-TIN. Conclusion IgG4-RKD and acute crescent glomerulonephritis can occur in the same patient. This case may give us a clearer viewpoint of the disease. PMID:27504450

  7. IgG4-Related Esophageal Disease Presenting as Esophagitis Dissecans Superficialis With Chronic Strictures.

    PubMed

    Dumas-Campagna, Myriam; Bouchard, Simon; Soucy, Genevieve; Bouin, Mickael

    2014-08-01

    IgG4-related disease is a recently recognized autoimmune systemic disorder that has been described in various organs. The disease is characterized histologically by a dense lymphoplasmocytic infiltrate of IgG4-positive cells, storiform fibrosis and can be associated with tumefactive lesions. IgG4-related disease involving the upper gastrointestinal tract is rare and only two previous case reports have reported IgG4-related esophageal disease. We report the case of a 63-year-old female patient with a long-standing history of severe dysphagia and odynophagia with an initial diagnosis of reflux esophagitis. Symptoms persisted despite anti-acid therapy and control esophagogastroduodenoscopy (EGD) revealed endoscopic images consistent with esophagitis dissecans superficialis (sloughing esophagitis). An underlying autoimmune process was suspected and immunosuppressant agents were tried to control her disease. The patient eventually developed disabling dysphagia secondary to multiple chronic esophageal strictures. A diagnosis of IgG4-related disease was eventually made after reviewing esophageal biopsies and performing an immunohistochemical study with an anti-IgG4 antibody. Treatment attempts with corticosteroids and rituximab was not associated with a significant improvement of the symptoms of dysphagia and odynophagia, possibly because of the chronic nature of the disease associated with a high fibrotic component. Our case report describes this unique case of IgG4-related esophageal disease presenting as chronic esophagitis dissecans with strictures. We also briefly review the main histopathological features and treatment options in IgG4-related disease. PMID:24883156

  8. IgG4-related Hashimoto's thyroiditis--a new variant of a well known disease.

    PubMed

    Luiz, Henrique Vara; Gonçalves, Diogo; Silva, Tiago Nunes da; Nascimento, Isabel; Ribeiro, Ana; Mafra, Manuela; Manita, Isabel; Portugal, Jorge

    2014-11-01

    Hashimoto's thyroiditis (HT) has been characterized for many years as a well-defined clinicopathologic entity, but is now considered a heterogeneous disease. IgG4-related HT is a new subtype characterized by thyroid inflammation rich in IgG4-positive plasma cells and marked fibrosis. It may be part of the systemic IgG4-related disease. We report a case of a 56-year-old Portuguese man who presented with a one-month history of progressive neck swelling and dysphagia. Laboratory testing revealed increased inflammatory parameters, subclinical hypothyroidism and very high levels of thyroid autoantibodies. Cervical ultrasound (US) demonstrated an enlarged and heterogeneous thyroid gland and two hypoechoic nodules. US-guided fine needle aspiration cytology was consistent with lymphocytic thyroiditis. The patient was submitted to total thyroidectomy and microscopic examination identified typical findings of HT, marked fibrosis limited within the thyroid capsule and lymphoplasmacytic infiltration, with >50 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of >40%. After surgery, serum IgG4 concentration was high-normal. Symptoms relief and reduction in laboratory inflammatory parameters were noticed. Thyroid function is controlled with levothyroxine. To our knowledge we report the first case of IgG4-related HT in a non-Asian patient. We also perform a review of the literature regarding IgG4-related disease and IgG4-related HT. Our case highlights this new variant of the well known HT, and helps physicians in recognizing its main clinical features, allowing for proper diagnosis and treatment. PMID:25465611

  9. [IgG4-related disease treatment in 2014: Update and literature review].

    PubMed

    Grados, A; Ebbo, M; Jean, E; Bernit, E; Harlé, J-R; Schleinitz, N

    2015-06-01

    IgG4-related disease is an inflammatory disorder characterized by a polyclonal lymphoplasmacytic tissue infiltrate, with numerous IgG4+ plasmocytes, evolving toward fibrosis. The disease is heterogeneous and affects several tissues and organs synchroneously or metachroneously. Both the fibrosis and the tumor forming characteristics of the disease can be responsible of irreversible tissue damage. For these reasons treatment is usually necessary. A dramatic response is usually observed with steroid treatment but relapses are frequent. Immunosuppressive agents and rituximab are used as second line treatments. We review here previous studies on treatment and suggest general recommendations for the treatment and follow up of patients with IgG4-related disease. PMID:25595874

  10. A case of refractory IgG4-related peripheral neuropathy with severe axonal damage.

    PubMed

    Suzuki, Yu; Shiraishi, Makoto; Yamada, Koji; Doi, Masatomo; Kato, Masayuki; Hasegawa, Yasuhiro

    2016-05-31

    A 78-year-old man presented complaining of tingling and pain. Neurological examination revealed dysesthesia and hypothermesthesia below both knees and areflexia in the lower extremities. Laboratory data revealed elevated serum levels of immunoglobulin IgG4 and para-aortic, and mesenteric lymphadenopathy was evident on plain computed tomography of the abdomen. Microscopic findings of a bone marrow biopsy specimen showed occlusion of blood vessels with IgG4-positive plasma cells. IgG4-related disease was diagnosed because the bone marrow biopsy exhibited > 10 IgG4-positive plasma cells per high-power field. Treatment was initiated with prednisolone starting at 30 mg/day, but no improvement in neurological symptoms was achieved. Sural nerve biopsy demonstrated obstructive thromboangiitis with severe loss of myelin and axons. Further investigations are needed to elucidate the relationship between obstructive thromboangiitis and steroid-resistant IgG4-related peripheral neuropathy. PMID:27098901

  11. Recurrent Mastoiditis Mimics IgG4 Related Disease: A Potential Diagnostic Pitfall.

    PubMed

    Deshpande, Vikram; Zane, Nicolas A; Kraft, Stefan; Stone, John H; Faquin, William C

    2016-09-01

    IgG4-related disease (IgG4-RD) is a recently recognized entity that causes progressive fibrosis and formation of mass lesions. IgG4-RD can be diagnosed histologically by its hallmarks of storiform fibrosis, prominent lymphoplasmacytic infiltrate, and obliterative phlebitis, accompanied by the infiltration of excessive numbers of IgG4-positive plasma cells as well as elevations in serum IgG4 concentrations. A recent publication reported a case of IgG4-RD in the mastoid sinus, representing a new anatomic location for this disease. We report two additional cases of IgG4-RD occurring in the mastoid and causing clinical mastoiditis. The presenting symptoms were varied-tinnitus, hearing loss, and cranial nerve palsies. All three cases showed a dense lymphoplasmacytic infiltrate, storiform type fibrosis as well as elevated numbers of IgG4 positive plasma cells. The three patients responded to immunosuppressive therapy that included steroids and Rituximab. We further investigated 162 consecutive mastoiditis cases at our institution in order to determine the frequency of IgG4-RD as a previously unrecognized cause of mastoiditis. Within this latter cohort we identified nine cases of mastoiditis that had two of the histologic features of IgG4-RD, specifically storiform fibrosis and a dense lymphoplasmacytic infiltrate. Two of these cases showed >50 IgG4-positive plasma cells per high-power field with IgG4-IgG ratio of >40 %, thus fulfilling histological criteria for IgG4-RD. However, both were due to severe acute or chronic infection. In conclusion, we reaffirm IgG4 related mastoiditis as a distinct but uncommon cause of recurrent mastoiditis. The diagnosis of IgG4-related mastoiditis should be rendered with caution, and only after the exclusion of potential mimickers, particularly infection. PMID:27091207

  12. Hypermethylation of MST1 in IgG4-related autoimmune pancreatitis and rheumatoid arthritis

    SciTech Connect

    Fukuhara, Takataro; Tomiyama, Takashi; Yasuda, Kaneki; Ueda, Yoshihiro; Ozaki, Yoshio; Son, Yonsu; Nomura, Shosaku; Uchida, Kazushige; Okazaki, Kazuichi; Kinashi, Tatsuo

    2015-08-07

    The serine/threonine kinase Mst1 plays important roles in the control of immune cell trafficking, proliferation, and differentiation. Previously, we reported that Mst1 was required for thymocyte selection and regulatory T-cell functions, thereby the prevention of autoimmunity in mice. In humans, MST1 null mutations cause T-cell immunodeficiency and hypergammaglobulinemia with autoantibody production. RASSF5C(RAPL) is an activator of MST1 and it is frequently methylated in some tumors. Herein, we investigated methylation of the promoter regions of MST1 and RASSF5C(RAPL) in leukocytes from patients with IgG4-related autoimmune pancreatitis (AIP) and rheumatoid arthritis (RA). Increased number of CpG methylation in the 5′ region of MST1 was detected in AIP patients with extrapancreatic lesions, whereas AIP patients without extrapancreatic lesions were similar to controls. In RA patients, we detected a slight increased CpG methylation in MST1, although the overall number of methylation sites was lower than that of AIP patients with extrapancreatic lesions. There were no significant changes of the methylation levels of the CpG islands in the 5′ region of RASSF5C(RAPL) in leukocytes from AIP and RA patients. Consistently, we found a significantly down-regulated expression of MST1 in regulatory T cells of AIP patients. Our results suggest that the decreased expression of MST1 in regulatory T cells due to hypermethylation of the promoter contributes to the pathogenesis of IgG4-related AIP. - Highlights: • Mst1 controls immune cells trafficking, cell proliferation and differentiation. • Autoimmune pancreatitis (AIP) is an idiopathic pancreatitis affecting multiple organs. • Decreased MST1 expression and increased CpG methylation of promoter of MST1 in AIP. • Slight increased CpG methylation of MST1 in rheumatoid arthritis patients. • MST1 contributes pathogenesis of IgG4-related AIP.

  13. Mitochondrial-dependent Autoimmunity in Membranous Nephropathy of IgG4-related Disease

    PubMed Central

    Buelli, Simona; Perico, Luca; Galbusera, Miriam; Abbate, Mauro; Morigi, Marina; Novelli, Rubina; Gagliardini, Elena; Tentori, Chiara; Rottoli, Daniela; Sabadini, Ettore; Saito, Takao; Kawano, Mitsuhiro; Saeki, Takako; Zoja, Carlamaria; Remuzzi, Giuseppe; Benigni, Ariela

    2015-01-01

    The pathophysiology of glomerular lesions of membranous nephropathy (MN), including seldom-reported IgG4-related disease, is still elusive. Unlike in idiopathic MN where IgG4 prevails, in this patient IgG3 was predominant in glomerular deposits in the absence of circulating anti-phospholipase A2 receptor antibodies, suggesting a distinct pathologic process. Here we documented that IgG4 retrieved from the serum of our propositus reacted against carbonic anhydrase II (CAII) at the podocyte surface. In patient's biopsy, glomerular CAII staining increased and co-localized with subepithelial IgG4 deposits along the capillary walls. Patient's IgG4 caused a drop in cell pH followed by mitochondrial dysfunction, excessive ROS production and cytoskeletal reorganization in cultured podocytes. These events promoted mitochondrial superoxide-dismutase-2 (SOD2) externalization on the plasma membrane, becoming recognizable by complement-binding IgG3 anti-SOD2. Among patients with IgG4-related disease only sera of those with IgG4 anti-CAII antibodies caused low intracellular pH and mitochondrial alterations underlying SOD2 externalization. Circulating IgG4 anti-CAII can cause podocyte injury through processes of intracellular acidification, mitochondrial oxidative stress and neoantigen induction in patients with IgG4 related disease. The onset of MN in a subset of patients could be due to IgG4 antibodies recognizing CAII with consequent exposure of mitochondrial neoantigen in the context of multifactorial pathogenesis of disease. PMID:26137589

  14. Pulmonary inflammatory myofibroblastic tumor and IgG4-related inflammatory pseudotumor: a diagnostic dilemma.

    PubMed

    Bhagat, Priyanka; Bal, Amanjit; Das, Ashim; Singh, Navneet; Singh, Harkant

    2013-12-01

    IgG4-related inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) share morphological features like a prominent fibroblastic/myofibroblastic proliferation and the presence of inflammatory cells. Since IPT is managed conservatively and IMT is treated by surgical excision, it is important to differentiate these two lesions. The aim of this study is to highlight morphological and immunohistochemical features that distinguish IPT and IMT. Clinicopathological characteristics of cases diagnosed as pulmonary IPT or IMT from 1997 to 2013 were reviewed. The histological features were studied on hematoxylin and eosin-stained sections. Immunohistochemistry was done for IgG, IgG4, ALK-1, SMA, desmin, and CD34 for classification into IPT and IMT. Of the ten patients, seven were male and the age ranged from 4 to 58 years. The tumor size ranged from 1.5 to 4.0 cm in diameter. Histologically, proliferation of bland-looking spindle cells along with fibrosis and an inflammatory infiltrate comprising of lymphocytes and plasma cells were the common morphological features of both lesions. The spindle cell proliferation was more marked in IMT whereas lymphoplasmacytic infiltrate was more prominent in IPT. Obstructive phlebitis was observed only in cases of IPT. IgG4 expression was noted in IPT, and the number of IgG4-positive plasma cells and the ratio of IgG4+/IgG+ plasma cells were significantly lower in IMT than in IgG4-related IPT. Expression of anaplastic lymphoma kinase (ALK) was observed only in IMT, but not in IgG4-related IPT. The proportion of proliferating spindle cells, lymphoplasmacytic infiltrate, obstructive phlebitis, IgG4+ plasma cells and the ratio of IgG4+/IgG+ plasma cells, and ALK expression are helpful in differentiating these morphologically similar but biologically different lesions, which require different treatment modalities. PMID:24100523

  15. Intracranial spread of IgG4-related disease via skull base foramina.

    PubMed

    Rice, C M; Spencer, T; Bunea, G; Scolding, N J; Sloan, P; Nath, U

    2016-06-01

    IgG4-related disease (IgG4-RD) is a newly recognised, multiorgan, inflammatory disease, and its full clinical spectrum remains undefined. We present a biopsy-proven case of IgG4-RD presenting with a parapharyngeal mass with intracranial extension and possible involvement of the brain parenchyma. We highlight the importance of considering the diagnosis in those presenting with tumefactive lesions, leptomeningitis or pachymeningitis and emphasise the value of securing a tissue diagnosis so that appropriate long-term treatment can be instigated and complications avoided. PMID:26856357

  16. Primary cutaneous extranodal marginal zone B-cell lymphoma of the eyelid skin: Diagnostic clues and distinction from other ocular adnexal diseases.

    PubMed

    Stagner, Anna M; Jakobiec, Frederick A; Freitag, Suzanne K

    2016-01-01

    A 60-year-old man developed a rubbery thickening and erythema of his left lateral upper and lower eyelids and lateral canthus over several months. He was treated for an extended period of time for blepharitis and chalazia. Incisional biopsy eventually disclosed microscopically a hypercellular lymphoid population sparing the epidermis that surrounded adnexal structures and infiltrated between orbicularis muscle fibers. Immunohistochemically, the lesion was found to be composed of neoplastic, kappa-restricted B cells with an equal number of reactive T cells and small reactive follicles. The diagnosis was a primary cutaneous extranodal marginal zone B-cell lymphoma of the eyelid skin (EMZL). We review the distinguishing clinical, histopathologic, and immunohistochemical features of cutaneous EMZL and contrast those with EMZL of other ocular adnexal sites. Also offered is a differential diagnosis of cutaneous lymphomas of the eyelid skin, which are predominately T-cell lesions. PMID:26545575

  17. Extensive Metastatic Cholangiocarcinoma Associated With IgG4-Related Sclerosing Cholangitis Misdiagnosed as Isolated IgG4-Related Sclerosing Cholangitis

    PubMed Central

    Zhang, Yi-An; Shen, Xi-Zhong; Zhu, Ji-Min; Liu, Tao-Tao

    2015-01-01

    Abstract As cholangiographic features of IgG4-related sclerosing cholangitis (IgG4-SC) resemble those of cholangiocarcinoma, it is highly confusing between the 2 conditions on the basis of cholangiographic findings. This study presents a case of extensive metastatic cholangiocarcinoma with IgG4-SC misdiagnosed as isolated IgG4-SC, and reviews recent studies of the 2 diseases. A 56-year-old man with no family history of malignant tumors or liver diseases presented with recurrent mild abdominal pain and distention for 3 months. Magnetic resonance cholangiopancreatography showed a 3.7 cm nodular lesion with unclear boundary in segment VI of the liver. Serum IgG4 and CA19-9 were slightly elevated. Histopathological examination was consistent with the consensus statement on the pathology of IgG4-SC. IgG4-SC was thus considered. Due to his mild symptoms, glucocorticoid was not given at first. However, 3 months after his first admission, he had more severe abdominal pain and further elevated serum CA19-9. Actually he was found suffering from extensive metastatic cholangiocarcinoma with IgG4-SC by exploratory laparotomy. The present case serves as a reminder that extensive metastatic cholangiocarcinoma with or without IgG4-SC may be misdiagnosed as an isolated IgG4-SC case if one relies solely on elevated serum and tissue IgG4 levels. We emphasize on the importance of repeated core needle biopsy or exploratory laparoscopy/laparotomy before immunosuppressive drugs are given, and on follow-up of imaging findings and serum CA19-9 once immunosuppressive therapy is started. PMID:26559312

  18. Colovesical fistula caused by glucocorticoid therapy for IgG4-related intrapelvic mass.

    PubMed

    Yabuuchi, Yohei; Matsubayashi, Hiroyuki; Matsuzaki, Masato; Shiomi, Akio; Moriguchi, Michihisa; Kawamura, Ichiro; Ito, Ichiro; Ono, Hiroyuki

    2015-12-16

    IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can occur in almost all systemic organs and generally responds to corticosteroid treatment. We report a rare case of an IgG4-related intrapelvic mass lesion that responded to steroid therapy but caused a fistula between the sigmoid colon and bladder. A 71-year-old man was followed after treatment for hepatocellular carcinoma. Follow-up computed tomography (CT) incidentally depicted left hydronephrosis with an ill-demarcated intrapelvic mass lesion. This lesion was histologically diagnosed as IgG4-RD by open biopsy, and peroral steroid therapy was initiated. One month after starting steroids, a colovesical fistula was detected by follow-up CT. A colostomy and urethral catheterization were emergently performed. The patient recovered and the mass lesion was drastically minimized by the initiation of glucocorticoids; however, he still needs urethral catheterization. IgG4-RD develops in various systemic organs and generally responds well to steroids. Clinicians must be watchful for the complications of responses to corticosteroids, such as fistulization, when the mass lesion of IgG4-RD is adjacent to multiple luminal organs. PMID:26677450

  19. IgG4-related renal disease: clinical and pathological characteristics.

    PubMed

    Kuroda, Naoto; Nao, Tomoya; Fukuhara, Hideo; Karashima, Takashi; Inoue, Keiji; Taniguchi, Yoshinori; Takeuchi, Mai; Zen, Yoh; Sato, Yasuharu; Notohara, Kenji; Yoshino, Tadashi

    2014-01-01

    IgG4-related disease is a recently established systemic condition. Tubulointerstitial nephritis is the most common renal manifestation. Glomerular lesions, particularly membranous glomerulonephritis, can develop simultaneously. Some patients present with serological renal dysfunction associated with elevated IgG or IgE levels and hypocomplementemia, while others are incidentally found to have abnormalities in kidneys on imaging. A majority of patients with IgG4-related kidney disease have similar lesions at other anatomical sites, which help us to suspect this condition. Serum IgG4 elevation (>135 mg/dL) is the most, although not entirely, specific marker for the diagnosis. Imaging findings varies from small nodules to bilateral diffuse abnormalities. In addition to the renal parenchyma, the renal pelvis and perirenal adipose tissue can be affected. Histological features include dense lymphoplasmacytic infiltration, storiform or "bird's eye" fibrosis (highlighted by PAM stain), and IgG4-positive plasma cell infiltration (>10 cells/high-power field and IgG4/IgG-positive cell ratio >40%). Immune complex deposition is detectable in the tubular basement membrane by immunofluorescence and/or electron microscopy. Patients usually respond well to corticosteroids, but highly active diseases may require other immunosuppressive therapies. Further investigations will be required to fully understand pathophysiology underlying this emerging condition. PMID:25337295

  20. Current Concepts and Diagnosis of IgG4-Related Pancreatitis (Type 1 AIP).

    PubMed

    Kawa, Shigeyuki

    2016-08-01

    Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer. However, because AIP responds favorably to corticosteroid treatment, careful differentiation from pancreatic cancer is required. An AIP diagnosis is currently based on the 2011 International Consensus Diagnostic Criteria for AIP, which are based on high sensitivity, selectivity, and accuracy. Over the long term, AIP can progress to a chronic condition, with pancreatic stone formation and atrophy resembling that of chronic pancreatitis. Although AIP has been linked to the complication of malignancies, it remains controversial whether an association exists between the disease and tumor formation. PMID:27466795

  1. Nasal manifestations of IgG4-related disease: A report of two cases.

    PubMed

    Ohno, Keiko; Matsuda, Yoko; Arai, Tomio; Kimura, Yurika

    2015-12-01

    IgG4-related disease (IgG4-RD) is a recently recognized clinical disease entity characterized by elevated serum IgG4, tumefaction, tissue infiltration of IgG4-positive plasma cells and fibrosis. IgG4-RD may occur, either synchronously or metachronously, in a variety of organs throughout the body. We describe herein two representative cases of the nasal manifestations of IgG4-RD, characterized by diffuse, crusty, erosive lesions on nasal mucosa. Oral steroid administration was effective in treating these nasal manifestations. We report a decrease in IgG4 positive plasma cell infiltrates in nasal mucosa biopsy specimens after steroid therapy, demonstrating that infiltration of IgG4-positive cells is reversible. PMID:26060132

  2. Co-existing ligneous conjunctivitis and IgG4-related disease.

    PubMed

    Chiang, Wei-Yu; Liu, Ting-Ting; Huang, Wan-Ting; Kuo, Ming-Tse

    2016-07-01

    Herein, we elucidate that ligneous conjunctivitis (LC) was proved as an IgG4-related disease (IgG4-RD) by a series of pathologic studies from primary and recurrent episodes of an LC patient. LC was diagnosed based on clinical presentation and pathological appearance; furthermore, combined with serological examination and immunohistochemical study, the case also conformed to the diagnosis of IgG4-RD. The IgG4-RD, broadly discussed in recent times, is an idiopathic disease entity with tissue fibrosis possibly involving multiple organs. To the best of our knowledge, IgG4-RD has never been reported with LC. By reporting the clinical course and literature review, we should pay attention to the association between these two diseases. PMID:27609168

  3. Serum Immunoglobulin Free Light Chain Assessment in IgG4-Related Disease

    PubMed Central

    Grados, Aurélie; Boucraut, José; Vély, Frédéric; Aucouturier, Pierre; Rigolet, Aude; Terrier, Benjamin; Saadoun, David; Ghillani-Dalbin, Pascale; Costedoat-Chalumeau, Nathalie; Harlé, Jean Robert; Schleinitz, Nicolas

    2013-01-01

    Immunoglobulin free light chains are produced in excess during normal antibody synthesis. Their evaluation is commonly used in case of a monoclonal gammopathy. In polyclonal hypergammaglobulinemia related to the Sjögren syndrome or systemic lupus, erythematosus serum free light chain levels are increased and could correlate with disease activity. We show here that the κ (P < 0.0001) and λ (P = 0.0003) free light chains and the κ : λ ratio (P = 0.0049) are increased in sixteen patients with IgG4-related disease when compared to healthy controls. The increase of κ and λ free light chains probably reflects the marked polyclonal B cell activation of the disease. We could not assess in this small cohort of patients a significative correlation of serum free light chain levels and disease activity or extension. PMID:23878543

  4. Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

    PubMed Central

    Carbone, Teresa; Azêdo Montes, Ricardo; Andrade, Beatriz; Lanzieri, Pedro; Mocarzel, Luis

    2015-01-01

    IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD. PMID:25838962

  5. Multicentric Castleman Disease With Tubulointerstitial Nephritis Mimicking IgG4-related Disease: Two Case Reports.

    PubMed

    Zoshima, Takeshi; Yamada, Kazunori; Hara, Satoshi; Mizushima, Ichiro; Yamagishi, Masakazu; Harada, Kenichi; Sato, Yasuharu; Kawano, Mitsuhiro

    2016-04-01

    Multicentric Castleman disease is a benign lymphoproliferative disorder with heterogenous clinical symptoms and involves systemic organs in addition to lymph nodes. Elevated serum IgG4 levels and IgG4-positive plasma cell (IgG4+PC) infiltrates have been reported in lymph nodes, lung and skin in some multicentric Castleman disease cases, resembling IgG4-related disease (IgG4-RD) histologically. However, no report has been available regarding IgG4+PC infiltration in the kidneys of multicentric Castleman disease. Here, we report 2 cases of multicentric Castleman disease complicated by IgG4-related disease (IgG4-RD) histologically. However, there has been no report published on PC-rich tubulointerstitial nephritis, lymphadenopathy, with numerous IgG4+PC infiltration, and elevated serum IgG4 levels, mimicking IgG4-RD. The blood examinations revealed systemic inflammation and elevated C-reactive protein and interleukin-6 levels. Corticosteroid therapy was partially effective in both cases, and combination therapy of corticosteroid and tocilizumab was needed in both cases. Moreover, after triple therapy with corticosteroid, rituximab and cyclophosphamide were used in 1 case to tame the severe inflammation. The present cases suggest that if continuously elevated serum C-reactive protein levels and partial corticosteroid responsiveness are encountered, multicentric Castleman disease should be considered rather than IgG4-RD as a differential diagnosis even if serum IgG4 is elevated and IgG4+PCs infiltrate systemic organs. PMID:26598921

  6. Clinical relevance of Küttner tumour and IgG4-related dacryoadenitis and sialoadenitis

    PubMed Central

    Furukawa, S; Moriyama, M; Kawano, S; Tanaka, A; Maehara, T; Hayashida, J-N; Goto, Y; Kiyoshima, T; Shiratsuchi, H; Ohyama, Y; Ohta, M; Imabayashi, Y; Nakamura, S

    2015-01-01

    Objectives Küttner tumour (KT), so-called chronic sclerosing sialoadenitis, is characterised by concomitant swelling of the submandibular glands secondary to strong lymphocytic infiltration and fibrosis independent of sialolith formation. However, recent studies have indicated that some patients with KT develop high serum levels of IgG4 and infiltration of IgG4-positive plasma cells, namely IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease. The aim of this study was to clarify the clinical and pathological associations between KT and IgG4-DS. Materials and Methods Fifty-four patients pathologically diagnosed with KT or chronic sialoadenitis were divided into two groups according to the presence or absence of sialolith (KT-S (+) or KT-S (−), respectively). Results There were no significant differences in the clinical findings, including the mean age, sex and disease duration, between the two groups. All patients in the KT-S (+) group showed unilateral swelling without infiltration of IgG4-positive plasma cells or a history of other IgG4-related diseases (IgG4-RD), while those in the KT-S (−) group showed bilateral swelling (37.5%), strong infiltration of IgG4-positive plasma cells (87.5%) and a history of other IgG4-RD (12.5%). Conclusions These results suggest an association between the pathogeneses of KT-S (−) and IgG4-DS, but not KT-S (+). PMID:24844187

  7. Rituximab for the Treatment of IgG4-Related Tubulointerstitial Nephritis

    PubMed Central

    McMahon, Blaithin A.; Novick, Tessa; Scheel, Paul J.; Bagnasco, Serena; Atta, Mohamed G.

    2015-01-01

    Abstract Immunoglobulin type gamma 4 (IgG4)-related disease is a relatively newly described clinical entity characterized by a distinctive histopathological appearance, increased numbers of IgG4 positive plasma cells and often, but not always, elevated serum IgG4 concentrations. The most common renal manifestation of IgG4-related disease is tubulointerstitial nephritis marked with proteinuria, hematuria, decreased kidney function, hypocomplementemia, and radiologic abnormalities. Renal biopsy characteristics include dense lymphoplasmacytic tubulointerstitial nephritis that stains for IgG4, storiform fibrosis, and immune complex deposition in the interstitium and along tubule basement membranes. Treatment traditionally consists of prolonged glucocorticoids but cases refractory to glucocorticoids have been reported. We report a case of a 58-year-old Caucasian man who presented with fatigue, 50 pound weight loss, dyspnea, lymphadenopathy, and nephromegaly. The patient was first misdiagnosed as chronic interstitial nephritis secondary to renal sarcoid and was treated with repeated doses of prednisone. On his third relapse, he underwent a repeat renal biopsy and a diagnosis of IgG4-tubulointerstitial nephritis was confirmed. He was refractory to treatment with prednisone. The patient received Rituximab and had prompt sustained improvement in renal function. At 1 year post Rituximab treatment, his serum creatinine remains at baseline and imaging study revealed reduction in his kidney size. This is the first case report using Rituximab as a steroid sparing option for refractory IgG4-tubulointerstitial nephritis. More information is needed on the long-term effects of using of B-cell depleting agents for glucocorticoid resistant IgG4-tubulointerstitial nephritis. PMID:26266393

  8. A case of non-lacrimal immunoglobulin G4 (IgG4)-related orbital disease with mastitis.

    PubMed

    Farooq, Tahir Ali; Mudhar, Hardeep; Sandramouli, S

    2016-01-01

    IgG4-related orbital disease is a recognised cause for orbital inflammation. As its awareness increases and diagnostic accuracy improves there will be an increased number of cases being identified. This unique case demonstrates for the first time, with histological evidence, a case of a non-lacrimal IgG4-related orbital disease with concurrent IgG4-related mastitis. We describe a 47 year old who presented with a supraorbital swelling and mass. This was initially successfully treated with oral steroids and was later excised on recurrence. Immunohistochemical and blood serum analysis confirmed IgG4-related orbital disease. On systemic enquiry she was found to have a mass of the breast, which was shown to be IgG4-related mastitis. She is currently asymptomatic with no sign of recurrence and is under long-term surveillance. This case highlights the importance of systemic work up in patients presenting with orbital foci of IgG4 disease. PMID:26700191

  9. IgG4-Related Lung Disease without Elevation of Serum IgG4 Level: A Case Report.

    PubMed

    Kang, Min Kyu; Cho, Yongseon; Han, Minsoo; Jung, Sun Young; Moon, Kyoung Min; Kim, Jinyoung; Kim, Ju Ri; Lee, Dong-Kyu; Park, Jun Hyung; Chung, So Hee

    2016-07-01

    Since IgG4-related pancreatitis was first reported in 2001, IgG4-related disease has been identified in other organs such as salivary gland, gallbladder, thyroid, retroperitoneum and kidney; but lung invasion is rare. A 63-year-old man presented with hemoptysis at the pulmonary clinic and chest computed tomography revealed about 4.1 cm irregular shaped mass with spiculated margin at the left upper lobe. Despite no elevation of serum IgG4 level, he was finally diagnosed as IgG4-related lung disease by transthoracic needle biopsy. After treatment with oral glucocorticoids, hemoptysis disappeared and the size of lung mass was decreased. PMID:27433179

  10. IgG4-Related Lung Disease without Elevation of Serum IgG4 Level: A Case Report

    PubMed Central

    Kang, Min Kyu; Han, Minsoo; Jung, Sun Young; Moon, Kyoung Min; Kim, Jinyoung; Kim, Ju Ri; Lee, Dong-kyu; Park, Jun Hyung; Chung, So Hee

    2016-01-01

    Since IgG4-related pancreatitis was first reported in 2001, IgG4-related disease has been identified in other organs such as salivary gland, gallbladder, thyroid, retroperitoneum and kidney; but lung invasion is rare. A 63-year-old man presented with hemoptysis at the pulmonary clinic and chest computed tomography revealed about 4.1 cm irregular shaped mass with spiculated margin at the left upper lobe. Despite no elevation of serum IgG4 level, he was finally diagnosed as IgG4-related lung disease by transthoracic needle biopsy. After treatment with oral glucocorticoids, hemoptysis disappeared and the size of lung mass was decreased. PMID:27433179

  11. Comprehensive genomic profiling of orbital and ocular adnexal lymphomas identifies frequent alterations in MYD88 and chromatin modifiers: new routes to targeted therapies.

    PubMed

    Cani, Andi K; Soliman, Moaaz; Hovelson, Daniel H; Liu, Chia-Jen; McDaniel, Andrew S; Haller, Michaela J; Bratley, Jarred V; Rahrig, Samantha E; Li, Qiang; Briceño, César A; Tomlins, Scott A; Rao, Rajesh C

    2016-07-01

    Non-Hodgkin lymphoma of the orbit and ocular adnexa is the most common primary orbital malignancy. Treatments for low- (extra-nodal marginal zone and follicular lymphomas) and high-grade (diffuse large B-cell lymphoma) are associated with local and vision-threatening toxicities. High-grade lymphomas relapse frequently and exhibit poor survival rates. Despite advances in genomic profiling and precision medicine, orbital and ocular adnexal lymphomas remain poorly characterized molecularly. We performed targeted next-generation sequencing (NGS) profiling of 38 formalin-fixed, paraffin-embedded orbital and ocular adnexal lymphomas obtained from a single-center using a panel targeting near-term, clinically relevant genes. Potentially actionable mutations and copy number alterations were prioritized based on gain- and loss-of-function analyses, and catalogued, approved, and investigational therapies. Of 36 informative samples, including marginal zone lymphomas (n=20), follicular lymphomas (n=9), and diffuse large B-cell lymphomas (n=7), 53% harbored a prioritized alteration (median=1, range 0-5/sample). MYD88 was the most frequently altered gene in our cohort, with potentially clinically relevant hotspot gain-of-function mutations identified in 71% of diffuse large B-cell lymphomas and 25% of marginal zone lymphomas. Prioritized alterations in epigenetic modulators were common and included gain-of-function EZH2 and loss-of-function ARID1A mutations (14% of diffuse large B-cell lymphomas and 22% of follicular lymphomas contained alterations in each of these two genes). Single prioritized alterations were also identified in the histone methyltransferases KMT2B (follicular lymphoma) and KMT3B (diffuse large B-cell lymphoma). Loss-of-function mutations and copy number alterations in the tumor suppressors TP53 (diffuse large B-cell and follicular lymphoma), CDKN2A (diffuse large B-cell and marginal zone lymphoma), PTEN (diffuse large B-cell lymphoma), ATM (diffuse large B

  12. IgG4-related disease: pathophysiologic insights drive emerging treatment approaches.

    PubMed

    Stone, John H

    2016-01-01

    IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect essentially any organ. The disease shows similar histopathology findings across organ systems, consisting of a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4 itself appears to be a reactive phenomenon rather than the primary disease driver. Recent investigations have focused on the interactions between cells of the B cell lineage and a novel CD4+ SLAMF7+ cytotoxic T cells capable of promoting fibrosis. Plasmablasts appear to play a crucial role along with B cells in the presentation of antigen to this T cell. IgG4-RD is marked by responsiveness to glucocorticoids, but frequent disease relapse, the inability to taper glucocorticoids completely, and steroid toxicity are problematic. Targeted treatment approaches against the B cell lineage appear promising, and therapeutic efforts focused upon the CD4+ SLAMF7+ cytotoxic T cell may also be feasible. PMID:27586808

  13. The Pathology of IgG4-Related Disease in the Bile Duct and Pancreas.

    PubMed

    Zen, Yoh

    2016-08-01

    Immunoglobulin G4-related disease (IgG4-RD) in the pancreatobiliary system manifests as sclerosing cholangitis (SC), hepatic inflammatory pseudotumors, and type 1 autoimmune pancreatitis (AIP). The pathology of IgG4-RD involves an inflammatory process and fibrogenic pathway, the combination of which damages the affected organs. Fibroinflammatory injury is characterized by three microscopic findings: a diffuse lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis. Although the diagnosis of IgG4-related pancreatocholangitis is relatively straightforward in surgical specimens, the current clinical requirement is to diagnose patients using biopsy samples, which remains challenging. Histological differential diagnoses include primary SC, follicular cholangitis/pancreatitis, SC with granulocytic epithelial lesions, and type 2 AIP. Although the massive infiltration of IgG4-positive plasma cells is a histological hallmark of IgG4-RD, many other immune cells (e.g., Th2 lymphocytes, regulatory T cells, and M2 macrophages) appear to be strongly involved in orchestral immune reactions. PMID:27466794

  14. IgG4-related disease: A relatively new concept for clinicians.

    PubMed

    Vasaitis, Lilian

    2016-01-01

    IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy. The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression. About 30-50% of patients are atopic or have mild eosinophilia. IgG4-RD predominantly affects middle-aged male patients. The cornerstones of diagnosis of the disease are compatible clinical features and typical histopathology. Swelling of salivary and lacrimal glands, lymphadenopathy, and type 1 autoimmune pancreatitis (AIP) are the most common manifestations of the disease. However, other tissues and organs, such as retroperitoneum, lung, kidney, aorta, upper airways, thyroid gland, meninges, heart, mesenterium and skin may be involved. Typical histopathology is lymphoplasmacytic infiltration abundant in IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis. Elevated serum IgG4 concentration supports the diagnosis. Characteristic imaging features such as a "capsule-like rim" surrounding the pancreatic lesions is highly specific to type 1 AIP. 18F-fluorodeoxyglucose positron emission tomography/computed tomography enables mapping the sites of inflammation, permits evaluation of the extent of the disease, helps in guiding biopsy decision, and may be used in monitoring response to treatment. Glucocorticoids alone or in combination with B-cell depletion with rituximab induces prompt clinical response to IgG4-RD. This article reviews the current understanding, different clinical manifestations, and approaches to diagnosis and treatment of IgG4-RD. PMID:26481243

  15. Corticosteroid Therapy for a Patient with Relapsing Polychondritis Complicated by IgG4-Related Disease.

    PubMed

    Yamasue, Mari; Nureki, Shin-Ichi; Matsumoto, Hiroyuki; Kan, Takamasa; Hashimoto, Takehiro; Ushijima, Ryoichi; Usagawa, Yuko; Kadota, Jun-Ichi

    2016-01-01

    Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease, coexistence of RP and IgG4-RD is rare. We herein report a case of RP complicated by IgG4-RD. A 63-year-old man developed recurrent bilateral ear pain and swelling, recurrent blurred and decreased vision, and migratory multiple joint pain, sequentially within one year. Fourteen months after the first symptom, he experienced dry cough and dyspnea with exertion. A computed tomography (CT) scan detected interstitial pneumonia, swelling of bilateral submandibular glands, bilateral hilar and mediastinal lymphadenopathy, and several nodules in bilateral kidneys. His serum levels of IgG and IgG4 were elevated. The biopsy specimen of auricular cartilage showed infiltrations of inflammatory cells and fibrosis consistent with RP. The IgG4-positive cells were not observed in auricular cartilage. The patient met the diagnostic criteria of RP, including bilateral auricular chondritis, conjunctivitis, iritis and polyarthritis. The biopsy specimens of lung and kidney revealed the significant infiltrations of IgG4-positive plasma cells and fibrosis. We also diagnosed him as having IgG4-RD, affecting bilateral submandibular glands, hilar and mediastinal lymph nodes, lungs, and kidneys. Thus, RP preceded the onset of IgG4-RD. Corticosteroid therapy improved the symptoms and CT scan findings. In conclusion, RP and IgG4-RD do coexist; however, the pathogenesis of their coexistence is unknown. PMID:27396510

  16. Rituximab for the treatment of IgG4-related orbital disease: experience from five cases

    PubMed Central

    Wu, A; Andrew, N H; Tsirbas, A; Tan, P; Gajdatsy, A; Selva, D

    2015-01-01

    Purpose To review the clinical efficacy and safety of rituximab for treatment of IgG4-related orbital disease (IgG4-ROD). Design Retrospective multicentre interventional case series. Methods Chart review for five cases of biopsy-confirmed IgG4-ROD (IgG4+>10/HPF, ratio of IgG4+/IgG+>40%) treated with rituximab. Information retrieved included the dosing schedule, adverse events and the magnitude, temporality, and duration of the clinical response. Results All cases of IgG4-ROD were either steroid dependent or steroid resistant. Rituximab doses for induction therapy included two doses of 1000 mg at 2-weekly intervals, and four doses at 375 mg/m2 at weekly intervals. Two months after starting rituximab, three cases achieved complete clinical resolution and two cases achieved partial clinical resolution. Complete radiological resolution occurred in one case, and partial radiological resolution in three cases. Three cases received rituximab maintenance therapy and one case was commenced on mycophenolate. No relapse occurred during a mean follow-up of 33 months (range: 7–65 months). One disease relapse occurred when the dosing interval of rituximab maintenance therapy was extended to 6–monthly intervals; remission was swiftly achieved with rituximab reinduction therapy. The only adverse effects reported were one episode of fatigue lasting 1 week and two episodes of orbital discomfort. Conclusion Rituximab may be an effective treatment option for IgG4-ROD that is steroid dependent or steroid intolerant. Rituximab therapy resulted in swift clinical and radiological improvement, many months free of relapse, and few side effects. PMID:25341435

  17. Erdheim-Chester Disease as a Mimic of IgG4-Related Disease

    PubMed Central

    Gianfreda, Davide; Musetti, Claudio; Nicastro, Maria; Maritati, Federica; Cobelli, Rocco; Corradi, Domenico; Vaglio, Augusto

    2016-01-01

    Abstract Immunoglobulin-G4 (IgG4)-related disease (IgG4RD) is a fibro-inflammatory disorder characterized by tissue-infiltrating IgG4+ plasma cells, and, often, high serum IgG4. Several autoimmune, infectious, or proliferative conditions mimic IgG4RD. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by foamy histiocytic infiltration, fibrosis, and chronic inflammation. ECD and IgG4RD manifestations may overlap. A patient presented with huge fibrous retroperitoneal masses causing compression on neighboring structures; the case posed the challenge of the differential diagnosis between IgG4RD and ECD mainly because of a prominent serum and tissue IgG4 response. Retroperitoneal biopsy led to the diagnosis of ECD; the V600E BRAF mutation was found. Treatment with the BRAF inhibitor vemurafenib was started. Treatment failed to induce mass regression and the patient died after 3 months of therapy. Prompted by this case, we examined serum and tissue IgG4 in a series of 15 ECD patients evaluated at our center, and found that approximately one-fourth of the cases have increased IgG4 in the serum and often in the tissue. The differential diagnosis between IgG4RD and ECD can be challenging, as some ECD patients have prominent IgG4 responses. This suggests the possibility of common pathogenic mechanisms between ECD and IgG4RD. PMID:27227923

  18. Therapeutic approach to IgG4-related disease: A systematic review.

    PubMed

    Brito-Zerón, Pilar; Kostov, Belchin; Bosch, Xavier; Acar-Denizli, Nihan; Ramos-Casals, Manuel; Stone, John H

    2016-06-01

    To review the reported evidence on the therapeutic management of IgG4-related disease (IgG4-RD) in clinical practice.A systematic search of the literature was conducted. The primary outcome measured was the rate of efficacy of first-line therapeutic approaches. Secondary outcomes measured included the rate of disease relapse, the outcome of untreated patients, the rate of patients without drug therapy at the end of follow-up, the rate of side effects, and mortality. The MOOSE, AHRQ, STROBE, and GRACE recommendations/statements were followed.The results of the systematic search strategy yielded 62 studies that included a total of 3034 patients. Complete information about first-line therapeutic regimens was detailed in 1952 patients, including glucocorticoid-based regimens in 1437 (74%), drug-free regimens in 213 (11%), and other therapies in 38 (2%). No therapy (wait and see management) was reported in 264 (13%) patients. The efficacy of monotherapy with glucocorticoids was specified in 1220 patients, of whom 97% had a therapeutic response. Relapses, however, were reported in 464/1395 (33%) patients despite typically short follow-up periods. Therapeutic efficacy was reported in 219/231 (95%) of relapses treated with glucocorticoids, 56/69 (81%) of those treated with azathioprine, 16/22 (72%) of those treated with other immunosuppressive agents, and in the 9 cases treated with rituximab (100%). In 14 studies, the authors detailed the outcome of 159/246 patients with wait-and-see management; spontaneous improvement or resolution was reported in 68 (43%) cases. Wide heterogeneity was observed with respect to the first-line therapeutic approaches used for the different organ-specific disease subsets, including significant differences in the mean dose of glucocorticoids used.Nearly 70% of reported IgG4-RD patients are treated with oral glucocorticoids in monotherapy. However, the therapeutic management is heavily influenced by geographical, epidemiological, and clinical factors, especially with respect to the predominant organ affected. The frequency of glucocorticoid failure to induce sustained remissions both during and after treatment and the assessment of glucocorticoid toxicity in IgG4-RD require further study. PMID:27368010

  19. IgG4-related disease presenting as a lung mass and weight loss: Case report and review of the literature

    PubMed Central

    Grewal, Kevin; Cohen, Paul; Kwon, Jeff S.; Kaufman, David A.

    2015-01-01

    We describe a case of IgG4-related lung disease presenting as a lung mass with associated weight loss. IgG4-related disease is a systemic sclerosing disorder that causes fibrotic, often tumor-like manifestations that variably effect different organ systems. The clinical presentation of IgG4-related disease is protean. Timely recognition and diagnosis requires awareness on the part of clinicians and pathologists to the variable manifestations of this newly recognized disorder. We offer a concise review of the pulmonary manifestations, diagnosis and treatment of IgG4-related lung disease.

  20. IgG4-related Disease: A Mass Lesion in the Intrarenal Sinus near the Renal Pelvis.

    PubMed

    Inenaga, Jun-Ichi; Ueno, Toshiharu; Kawada, Masahiro; Imafuku, Aya; Mise, Koki; Sumida, Keiichi; Hiramatsu, Rikako; Hasegawa, Eiko; Hayami, Noriko; Suwabe, Tatsuya; Hoshino, Junichi; Sawa, Naoki; Takaichi, Kenmei; Fujii, Takeshi; Ohashi, Kenichi; Okaneya, Toshikazu; Ubara, Yoshifumi

    2015-01-01

    A 52-year-old Japanese woman was admitted to our hospital with the renal pelvic mass lesion detected on a health screening examination. The surgical specimen contained a mass exhibiting the histological features of immunoglobulin (Ig)G4-related disease, including lymphoplasmacytic infiltration and sclerosis with numerous IgG4-producing plasma cells. Postoperatively, an elevation of the serum IgG4 level was confirmed at 403 mg/dL; however, there was no evidence of tubulointerstitial nephritis or glomerulopathy, including membranous nephropathy, and the urothelium of the renal pelvis was intact without inflammation. We herein report this case in which IgG4-related disease of the renal pelvic region presented with a mass lesion in the intrarenal sinus near the renal pelvis, not 'pyelitis' (as described by Stone). PMID:26234232

  1. Diagnostic Value of Serum IgG4 for IgG4-Related Disease

    PubMed Central

    Hao, Mingju; Liu, Min; Fan, Gaowei; Yang, Xin; Li, Jinming

    2016-01-01

    Abstract Many studies about serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD) have been reported. However, these studies had relatively small sample sizes and the diagnostic accuracy values varied much between them. The aim of this study was to perform a meta-analysis to evaluate the diagnostic value of serum IgG4 for IgG4-RD. We conducted a search of relevant articles using MEDLINE, EMBASE, Web of Science, SCOPUS, and Cochrane Library databases published before December 2015. Studies those assessed the diagnostic accuracy of serum IgG4 for IgG4-RD and those provided the cut-off value for serum IgG4 were included. Data were synthesized using the random-effect model. Statistical analysis was performed using STATA with the MIDAS module and Meta-DiSc 1.4 software. A total of 9 case-control studies were analyzed, which included 1235 patients with IgG4-RD and 5696 overall controls. The pooled estimate, for a cut-off value ranged from 135 to 144 mg/dL, produced a sensitivity of 87.2% (95% CI, 85.2–89.0%) and a specificity of 82.6% (95% CI, 81.6–83.6%). The positive likelihood ratio (PLR), negative likelihood ratio (NLR), and diagnostic odds ratio (DOR) were 6.48 (95% CI, 3.98–10.57), 0.14 (95% CI, 0.09–0.21), and 45.15 (95% CI, 23.41–87.06), respectively. The area under the curve (AUC) of the summary receiver operating characteristic curve (SROC) was 0.94 (0.92–0.96). When a cut-off value of 2-fold the upper limit of normal was used (ranged from 270 to 280 mg/dL), the pooled sensitivity was 63% (95% CI, 60.0–66.0%), and the specificity was 94.8% (95% CI, 94.1–95.4%). The PLR, NLR, and DOR were 13.3 (95% CI, 7.39–24.0), 0.41 (95% CI, 0.29–0.58) and 33.42 (95% CI, 13.88–80.43), respectively. The AUC of the SROC was 0.92 (0.90–0.94). Only a relatively small number of studies were included, and significant heterogeneity was observed in this meta-analysis. Serum IgG4 is a modestly effective marker to diagnose IgG4-RD. Doubling the cut-off value for IgG4 could not improve the overall test characteristics. A high specificity inevitably accompanies with a significant sacrifice in sensitivity. PMID:27227950

  2. [IgG4-related kidney disease: a long-term follow up case of pseudotumor of the renal pelvis].

    PubMed

    Tsuzaka, Yasuo; Ookubo, Kazuki; Sugiyama, Kazutaka; Morimoto, Hirohiko; Amano, Hiroyuki; Oota, Nobutaka; Kuriki, Ken; Homma, Yukio

    2014-04-01

    A 69-year-old man had undergone left ureteronephrectomy because of a left renal pelvic tumor, however the pathological diagnosis was inflammatory pseudotumor. About 1 year later, computed tomography showed a mass at the right kidney near the hilar. Ureterorenoscopy and urine cytology were performed, and their results showed no evidence of malignancy. He had been followed closely without therapy. The mass increased in size during follow-up, and we reviewed the surgical specimen of the left ureteronephrectomy. Immunohistochemical studies revealed diffuse infiltration by IgG4 positive plasma cell. His serum IgG4 was high. We diagnosed him as IgG4-related kidney disease. In response to treatment with corticosteroid, the size of the tumor and serum IgG4 levels decreased. Most reported cases of IgG4-related disease involving kidney have a history of prior pancreatic involvement. We report a rare long term follow-up case of IgG4-related kidney disease without pancreatic involvement. PMID:24908817

  3. Pericardiobiliary Fistulation: A Rare Complication of Therapeutic ERCP in a Patient With IgG4-Related Sclerosing Cholangitis

    PubMed Central

    Paranandi, Bharat; Joshi, Deepak; Johnson, Gavin J.

    2015-01-01

    A 70-year-old man presented with acute coronary syndrome 3 weeks after plastic stent insertion for hilar biliary stricturing secondary to IgG4-related sclerosing cholangitis (IgG4-SC). Imaging demonstrated haemopericardium due to proximal migration of the plastic biliary stent through the liver capsule and diaphragm into the pericardial sac. The stent was endoscopically removed and a pericardiocentesis was performed. The patient's clinical condition rapidly improved. We illustrate an unusual but potentially serious complication that may arise from migration of a biliary stent and discuss a management strategy. PMID:26203452

  4. Chronic sclerosing sialadenitis of the submandibular gland: an entity of IgG4-related sclerosing disease

    PubMed Central

    Wei, Tzu-Wei; Lien, Ching-Feng; Hsu, Tun-Yen; He, Hong-Lin

    2015-01-01

    Chronic sclerosing sialadenitis typically involves the submandibular gland. It usually occurs in the middle-aged and elderly adults with a slight male predominance. Recent evidences have suggested that it is an entity of IgG4-related sclerosing disease and has distinct histopathological features, such as a dense lymphoplasmacytic infiltrate, sclerosis and obliterative phlebitis. It is important to discriminate this entity from other diseases, trying to give effective treatment to the patients. In this report, we described a patient having chronic sclerosing sialadenitis in the submandibular gland. PMID:26339446

  5. Serosal involvement in IgG4-related disease: report of two cases and review of the literature.

    PubMed

    González-Moreno, Juan; Losada-López, Inés; Gállego-Lezaun, Cristina; García-Gasalla, Mercedes; Gómez Bellvert, Cristina; Ortego Centeno, Norberto

    2016-07-01

    IgG4-related disease (IgG4-RD) is a recently described entity characterized by lymphoplasmacytic infiltrates, usually mimicking tumors, affecting almost every organ or system. Nevertheless, serosal involvement has been rarely reported. In this article, we report two cases of IgG4-RD with serosal involvement and review the literature. Because of the varied clinical pictures found in our review, we suggest a new terminology for the description of IgG4-RD with serosal involvement. PMID:27207156

  6. IgG4-related Lung Disease Associated with Autoimmune Hemolytic Anemia: A Case Report and a Literature Review.

    PubMed

    Noguchi, Shingo; Yatera, Kazuhiro; Jinbo, Mitsutaka; Yamada, Sohsuke; Shimabukuro, Ikuko; Yamasaki, Kei; Kido, Takashi; Ishimoto, Hiroshi; Yoshii, Chiharu; Mukae, Hiroshi

    2016-01-01

    We herein report a case of IgG4-related lung disease (IgG4-RLD) associated with autoimmune hemolytic anemia (AIHA). A 73-year-old Japanese female visited our hospital for an examination following an abnormal chest X-ray in 1999. She was diagnosed with bronchiolitis and AIHA, and treatment with prednisolone was started. After seven years, she visited our department due to a cough. Chest computed tomography (CT) demonstrated focal consolidation with ground-glass attenuations and thickened bronchial walls in the bilateral lungs. She was clinically diagnosed and treated for bronchial asthma. CT findings had shown no changes, and a lung biopsy was performed using video-assisted thoracic surgery at eleven years from the first diagnosis of AIHA. The pathological findings demonstrated the presence of peribronchovascular lymphoplasmacytic infiltrates with stromal fibrotic changes, admixed with many IgG4-positive plasma cells. Furthermore, the patient's serum IgG4 level was high, and her CT findings did not show any obvious abnormal findings in the any organs other than the lungs. She was diagnosed with IgG4-RLD based on the findings. We believe that this case report of IgG4-RLD associated with AIHA is clinically helpful for a better understanding of these diseases, although there are five reported cases of IgG4-related disease associated with AIHA. PMID:27580552

  7. IgG4-related Sclerosing Cholangitis with No Biliary Stricture but Severe Thickening of the Bile Duct Wall.

    PubMed

    Shimizu, Shuya; Naitoh, Itaru; Nakazawa, Takahiro; Hayashi, Kazuki; Miyabe, Katsuyuki; Kondo, Hiromu; Nishi, Yuji; Yoshida, Michihiro; Umemura, Shuichiro; Hori, Yasuki; Kato, Akihisa; Ohara, Hirotaka; Kuno, Toshiya; Takahashi, Satoru; Joh, Takashi

    2016-01-01

    We herein report a case of a 56-year-old man with IgG4-related sclerosing cholangitis (IgG4-SC) with no biliary stricture, but with a severely thickened bile duct wall. Contrast-enhanced computed tomography showed diffuse swelling of the pancreas and thickening of the common bile duct (CBD) wall with delayed enhancement. Obvious diffuse wall thickening of the CBD was observed on endoscopic ultrasonography. However, endoscopic retrograde cholangiography showed no biliary stricture in the CBD that had thickened. Although IgG4-SC has been classified by a stenotic lesion on cholangiography, we should be aware of some IgG4-SC cases showing only bile duct wall thickness without any biliary stricture. PMID:27301508

  8. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details.

    PubMed

    Umehara, Hisanori; Okazaki, Kazuichi; Masaki, Yasufumi; Kawano, Mitsuhiro; Yamamoto, Motohisa; Saeki, Takako; Matsui, Shoko; Sumida, Takayuki; Mimori, Tsuneyo; Tanaka, Yoshiya; Tsubota, Kazuo; Yoshino, Tadashi; Kawa, Shigeyuki; Suzuki, Ritsuro; Takegami, Tsutomu; Tomosugi, Naohisa; Kurose, Nozomu; Ishigaki, Yasuhito; Azumi, Atsushi; Kojima, Masaru; Nakamura, Shigeo; Inoue, Dai

    2012-02-01

    IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz's disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor. Although IgG4RD forms a distinct, clinically independent disease category and is attracting strong attention as a new clinical entity, many questions and problems still remain to be elucidated, including its pathogenesis, the establishment of diagnostic criteria, and the role of IgG4. Here we describe the concept of IgG4RD and up-to-date information on this emerging disease entity. PMID:21881964

  9. IgG4-related disease and its pathogenesis—cross-talk between innate and acquired immunity

    PubMed Central

    Nakajima, Akio; Nakamura, Takuji; Kawanami, Takafumi; Tanaka, Masao; Dong, Lingli; Kawano, Mitsuhiro

    2014-01-01

    IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world. The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4+ plasma cells. Although the clinical manifestations in various organs have been established, the pathogenesis of IgG4-RD is still unknown. Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published. However, many questions still remain, including questions about the pathogenesis of IgG4-RD and the roles of IgG4. In this review, we discuss the pathogenesis of IgG4-RD by focusing on the cross-talk between innate and acquired immunity. PMID:25024397

  10. Rituximab for the Treatment of IgG4-Related Tubulointerstitial Nephritis: Case Report and Review of the Literature.

    PubMed

    McMahon, Blaithin A; Novick, Tessa; Scheel, Paul J; Bagnasco, Serena; Atta, Mohamed G

    2015-08-01

    Immunoglobulin type gamma 4 (IgG4)-related disease is a relatively newly described clinical entity characterized by a distinctive histopathological appearance, increased numbers of IgG4 positive plasma cells and often, but not always, elevated serum IgG4 concentrations. The most common renal manifestation of IgG4-related disease is tubulointerstitial nephritis marked with proteinuria, hematuria, decreased kidney function, hypocomplementemia, and radiologic abnormalities. Renal biopsy characteristics include dense lymphoplasmacytic tubulointerstitial nephritis that stains for IgG4, storiform fibrosis, and immune complex deposition in the interstitium and along tubule basement membranes. Treatment traditionally consists of prolonged glucocorticoids but cases refractory to glucocorticoids have been reported.We report a case of a 58-year-old Caucasian man who presented with fatigue, 50 pound weight loss, dyspnea, lymphadenopathy, and nephromegaly. The patient was first misdiagnosed as chronic interstitial nephritis secondary to renal sarcoid and was treated with repeated doses of prednisone. On his third relapse, he underwent a repeat renal biopsy and a diagnosis of IgG4-tubulointerstitial nephritis was confirmed. He was refractory to treatment with prednisone. The patient received Rituximab and had prompt sustained improvement in renal function. At 1 year post Rituximab treatment, his serum creatinine remains at baseline and imaging study revealed reduction in his kidney size.This is the first case report using Rituximab as a steroid sparing option for refractory IgG4-tubulointerstitial nephritis. More information is needed on the long-term effects of using of B-cell depleting agents for glucocorticoid resistant IgG4-tubulointerstitial nephritis. PMID:26266393

  11. Long-Term Outcome and Patterns of Failure in Primary Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Treated With Radiotherapy

    SciTech Connect

    Hashimoto, Naoki; Sasaki, Ryohei; Nishimura, Hideki; Yoshida, Kenji; Miyawaki, Daisuke; Nakayama, Masao; Uehara, Kazuyuki; Okamoto, Yoshiaki; Ejima, Yasuo; Azumi, Atsushi; Matsui, Toshimitsu; Sugimura, Kazuro

    2012-03-15

    Purpose: To evaluate the long-term treatment outcome and disease behavior of primary ocular adnexal MALT (mucosa-associated lymphoid tissue) lymphoma (POAML) after treatment with radiotherapy. Methods and Materials: Seventy-eight patients (42 male, 36 female) diagnosed with stage I POAML between 1991 and 2010 at Kobe University Hospital were included. The median age was 60 years (range, 22-85 years). The median radiation dose administered was 30.6 Gy. Rituximab-based targeted therapy and/or chemotherapy was performed in 20 patients (25.6%). Local control (LC), recurrence-free survival (RFS), and overall survival (OS) rates were calculated using the Kaplan-Meier method. Results: The median follow-up duration was 66 months. Major tumor sites were conjunctiva in 37 patients (47.4%), orbita in 29 (37.2%), and lacrimal glands in 12 (15.4%). The 5- and 10-year OS rates were 98.1% and 95.3%, respectively. The 5- and 10-year LC rates were both 100%, and the 5- and 10-year RFS rates were 88.5% and 75.9%, respectively. Patients treated with a combination of radiotherapy and targeted therapy and/or chemotherapy had a trend for a better RFS compared with those treated with radiotherapy alone (p = 0.114). None developed greater than Grade 2 acute morbidity. There were 14 patients who experienced Grade 2 morbidities (cataract: 14; retinal disorders: 7; dry eye: 3), 23 patients who had Grade 3 morbidities (cataract: 23; dry eye: 1), and 1 patient who had Grade 4 glaucoma. Conclusions: Radiotherapy for POAML was shown to be highly effective and safe for LC and OS on the basis of long-term observation. The absence of systemic relapse in patients with combined-modality treatment suggests that lower doses of radiation combined with targeted therapy may be worth further study.

  12. Localized Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Treated With Radiation Therapy: A Long-Term Outcome in 86 Patients With 104 Treated Eyes

    SciTech Connect

    Harada, Ken; Murakami, Naoya; Kitaguchi, Mayuka; Sekii, Shuhei; Takahashi, Kana; Yoshio, Kotaro; Inaba, Koji; Morota, Madoka; Ito, Yoshinori; Sumi, Minako; Suzuki, Shigenobu; Tobinai, Kensei; Uno, Takashi; Itami, Jun

    2014-03-01

    Purpose: To evaluate the natural history, behavior of progression, prognostic factors, and treatment-related adverse effects of primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (POAML). Methods and Materials: Eighty-six patients with histologically proven stage I POAML treated with radiation therapy at National Cancer Center Hospital, Tokyo between 1990 and 2010 were retrospectively reviewed. The median age was 56 years (range, 18-85 years). The median dose administered was 30 Gy (range, 30-46 Gy). Seventy-seven patients (90%) were treated by radiation therapy alone. Results: The median follow-up duration was 9 years (range, 0.9-22 years). The 5- and 10-year overall survival (OS) rates were 97.6% and 93.5%, respectively, and no patients died of lymphoma. Patients with tumor sizes ≥4 cm showed a greater risk of contralateral relapse (P=.012). Six patients with contralateral relapse were seen and treated by radiation therapy alone, and all the lesions were controlled well, with follow-up times of 3 to 12 years. There was 1 case of local relapse after radiation therapy alone, and 3 cases of relapse occurred in a distant site. Cataracts developed in 36 of the 65 eyes treated without lens shielding and in 12 of the 39 patients with lens shielding (P=.037). Conclusions: The majority of patients with POAML showed behavior consistent with that of localized, indolent diseases. Thirty gray of local irradiation seems to be quite effective. The initial bilateral involvement and contralateral orbital relapses can be also controlled with radiation therapy alone. Lens shielding reduces the risk of cataract.

  13. Recent advances in the concept and pathogenesis of IgG4-related disease in the hepato-bilio-pancreatic system.

    PubMed

    Okazaki, Kazuichi; Yanagawa, Masahito; Mitsuyama, Toshiyuki; Uchida, Kazushige

    2014-09-01

    Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of "biliary diseases with pancreatic counterparts." Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD. PMID:25228969

  14. Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

    PubMed Central

    2011-01-01

    Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology. PMID:21205323

  15. Diagnostic performance of serum IgG4 level for IgG4-related disease: a meta-analysis

    PubMed Central

    Xu, Wen-long; Ling, Ying-chun; Wang, Zhi-kai; Deng, Fang

    2016-01-01

    An elevated serum IgG4 level is one of the most useful factors in the diagnosis of IgG4-related disease (IgG4-RD). In this study, we performed a meta-analysis of the published articles assessing the diagnostic accuracy of serum IgG4 concentrations for IgG4-RD. The databases of MEDLINE/PubMed, EMBASE and Web of Science were systematically searched for relevant studies. Sensitivities and specificities of serum IgG4 in each study were calculated, and the hierarchical summary receiver operating characteristic (HSROC) model with a random effects model were employed to obtain the individual and pooled estimates of sensitivities and specificities. In total, twenty-three studies comprising 6048 patients with IgG4-RD were included in the meta-analysis. The pooled sensitivity was 85% with a 95% confidence interval (CI) of 78–90%; the pooled specificity was 93% with a 95% CI of 90–95%. The HSROC curve for quantitative serum IgG4 lies closer to the upper left corner of the plot, and the area under the curve (AUC) was 0.95 (95% CI 0.93, 0.97), which suggested a high diagnostic accuracy of serum IgG4 for the entity of IgG4-RD. Our study suggests that serum IgG4 has high sensitivity and specificity in the diagnosis of IgG4-RD. PMID:27558881

  16. Diagnostic performance of serum IgG4 level for IgG4-related disease: a meta-analysis.

    PubMed

    Xu, Wen-Long; Ling, Ying-Chun; Wang, Zhi-Kai; Deng, Fang

    2016-01-01

    An elevated serum IgG4 level is one of the most useful factors in the diagnosis of IgG4-related disease (IgG4-RD). In this study, we performed a meta-analysis of the published articles assessing the diagnostic accuracy of serum IgG4 concentrations for IgG4-RD. The databases of MEDLINE/PubMed, EMBASE and Web of Science were systematically searched for relevant studies. Sensitivities and specificities of serum IgG4 in each study were calculated, and the hierarchical summary receiver operating characteristic (HSROC) model with a random effects model were employed to obtain the individual and pooled estimates of sensitivities and specificities. In total, twenty-three studies comprising 6048 patients with IgG4-RD were included in the meta-analysis. The pooled sensitivity was 85% with a 95% confidence interval (CI) of 78-90%; the pooled specificity was 93% with a 95% CI of 90-95%. The HSROC curve for quantitative serum IgG4 lies closer to the upper left corner of the plot, and the area under the curve (AUC) was 0.95 (95% CI 0.93, 0.97), which suggested a high diagnostic accuracy of serum IgG4 for the entity of IgG4-RD. Our study suggests that serum IgG4 has high sensitivity and specificity in the diagnosis of IgG4-RD. PMID:27558881

  17. T helper 2 and regulatory T-cell cytokine production by mast cells: a key factor in the pathogenesis of IgG4-related disease.

    PubMed

    Takeuchi, Mai; Sato, Yasuharu; Ohno, Kyotaro; Tanaka, Satoshi; Takata, Katsuyoshi; Gion, Yuka; Orita, Yorihisa; Ito, Toshihiro; Tachibana, Tomoyasu; Yoshino, Tadashi

    2014-08-01

    IgG4-related disease is a systemic disorder with unique clinicopathological features and uncertain etiological features and is frequently related to allergic disease. T helper 2 and regulatory T-cell cytokines have been reported to be upregulated in the affected tissues; thus, the production of these cytokines by T helper 2 and regulatory T cells has been suggested as an important factor in the pathogenesis of IgG4-related disease. However, it is not yet clear which cells produce these cytokines in IgG4-related disease, and some aspects of the disorder cannot be completely explained by T-cell-related processes. To address this, we analyzed paraffin-embedded sections of tissues from nine cases of IgG4-related submandibular gland disease, five cases of submandibular sialolithiasis, and six cases of normal submandibular gland in order to identify potential key players in the pathogenesis of IgG4-related disease. Real-time polymerase chain reaction analysis confirmed the significant upregulation of interleukin (IL)4, IL10, and transforming growth factor beta 1 (TGFβ1) in IgG4-related disease. Interestingly, immunohistochemical studies indicated the presence of mast cells expressing these cytokines in diseased tissues. In addition, dual immunofluorescence assays identified cells that were double-positive for each cytokine and for KIT, which is expressed by mast cells. In contrast, the distribution of T cells did not correlate with cytokine distribution in affected tissues. We also found that the mast cells were strongly positive for IgE. This observation supports the hypothesis that mast cells are involved in IgG4-related disease, as mast cells are known to be closely related to allergic reactions and are activated in the presence of elevated non-specific IgE levels. In conclusion, our results indicate that mast cells produce T helper 2 and regulatory T-cell cytokines in tissues affected by IgG4-related disease and possibly have an important role in disease pathogenesis. PMID:24390219

  18. Chronic Mastitis in Egypt and Morocco: Differentiating between Idiopathic Granulomatous Mastitis and IgG4-Related Disease.

    PubMed

    Allen, Steven G; Soliman, Amr S; Toy, Kathleen; Omar, Omar S; Youssef, Tamer; Karkouri, Mehdi; Ayad, Essam; Abdel-Aziz, Azza; Hablas, Ahmed; Tahri, Ali; Oltean, Hanna N; Kleer, Celina G; Merajver, Sofia D

    2016-09-01

    Idiopathic granulomatous mastitis (IGM) is a benign, frequently severe chronic inflammatory lesion of the breast. Its etiology remains unknown and reported cases vary in their presentation and histologic findings with an optimal treatment algorithm yet to be described owing mainly to the disease's heterogeneity. IgG4-related disease (IgG4-RD) is a newly recognized systemic fibroinflammatory condition characterized by a dense lymphoplasmacytic infiltrate with many IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. Immunosuppressive therapy is considered to be an effective first-line therapy for IgG4-RD. We sought to clarify and classify chronic mastitis according to the histologic findings of IgG4-RD mastitis with respect to IGM and to develop a robust diagnostic framework to help select patients for optimal treatment strategies. Using the largest collection to date (43 cases from Egypt and Morocco), we show that despite sharing many features, IGM and IgG4-RD mastitis are separate diseases. To diagnostically separate the diseases, we created a classification schema-termed the Michigan Classification-based upon our large series of cases, the consensus statement on IgG4-RD, and the histologic description of IGM in the literature. Using our classification, we discerned 17 cases of IgG4-RD and 8 cases of IGM among the 43 chronic mastitis cases, with 18 indeterminate cases. Thus, our Michigan Classification can form the basis of rational stratification of chronic mastitis patients between these two clinically and histopathologically heterogeneous diseases. PMID:27279578

  19. IgG4-related hypophysitis presenting as diabetes insipidus with tubulo-interstital nephritis and mediastinal lymphadenopathy

    PubMed Central

    Mustafa, Waheed; Sheaff, Michael T; Khan, Sami

    2016-01-01

    Summary IgG4-related disease (IgG4-RD) is a rare but increasingly recognised condition, emerging as a clinical entity following the observation of the associations of autoimmune pancreatitis. IgG4-RD is characterised by extensive infiltration of IgG4-positive plasma cells into multiple organs and raised serum IgG4 levels. Clinical manifestations of IgG4 disease classically include autoimmune pancreatitis, lacrimal or salivary gland infiltration (formerly known as Mikulicz disease) and retroperitoneal fibrosis. More rarely, IgG4 disease can cause pituitary hypophysitis. Although most frequently described in middle-aged males, the epidemiology and pathogenesis of the disease remain largely undefined. Nevertheless, an understanding of the wide variety of clinical manifestations of this multi-system condition is undeniably important given the often excellent outcomes following treatment. We describe an unusual presentation of IgG4 disease with isolated diabetes insipidus secondary to pituitary hypophysitis. The patient in question subsequently developed chest pain secondary to mediastinal lymphadenopathy and tubulo-interstitial nephritis leading to renal dysfunction. He was successfully treated with oral steroids and had regular follow-up, and remains well at follow-up 2 years later. Learning points IgG4 disease, although rare, is increasing in prevalence largely due to increased recognition of its clinical manifestations, including autoimmune pancreatitis, lacrimal or salivary gland infiltration, retroperitoneal fibrosis and, more rarely, lymphocytic hypophysitis presenting as diabetes insipidus. IgG4 disease is highly treatable, and symptoms may show complete resolution with administration of steroids, highlighting the importance of correct and timely diagnosis. Causes of lymphocytic hypophysitis are varied and not distinguishable radiologically. Given the difficulty in biopsying the pituitary, careful attention must be paid to the systemic clinical presentation to provide clues as to the underlying disorder. PMID:27398219

  20. Distribution and components of interstitial inflammation and fibrosis in IgG4-related kidney disease: analysis of autopsy specimens.

    PubMed

    Hara, Satoshi; Kawano, Mitsuhiro; Mizushima, Ichiro; Harada, Kenichi; Takata, Takuma; Saeki, Takako; Ubara, Yoshifumi; Sato, Yasuharu; Nagata, Michio

    2016-09-01

    IgG4-related kidney disease (IgG4-RKD) occasionally progresses to chronic renal failure and is pathologically characterized by IgG4-positive lymphoplasmacyte-rich tubulointerstitial nephritis with storiform fibrosis (bird's-eye pattern fibrosis). Although radiology reveals a heterogeneous distribution of affected areas in this disease, their true distribution within the whole kidney is still unknown because of difficulty in estimating this from needle biopsy samples. Using 5 autopsy specimens, the present study histologically characterized the distribution and components of interstitial inflammation and fibrosis in IgG4-RKD. Interstitial lymphoplasmacytic infiltration or fibrosis was observed in a variety of anatomical locations such as intracapsular, subcapsular, cortical, perivascular, and perineural regions heterogeneously in a patchy distribution. They tended to be more markedly accumulated around medium- and small-sized vessels. Storiform fibrosis was limited to the cortex. Immunostaining revealed nonfibrillar collagens (collagen IV and VI) and fibronectin predominance in the cortical lesion, including storiform fibrosis. In contrast, fibril-forming collagens (collagen I and III), collagen VI, and fibronectin were the main components in the perivascular lesion. In addition, α-smooth muscle actin-positive myofibroblasts were prominently accumulated in the early lesion and decreased with progression, suggesting that myofibroblasts produce extracellular matrices forming a peculiar fibrosis. In conclusion, perivascular inflammation or fibrosis of medium- and small-sized vessels is a newly identified pathologic feature of IgG4-RKD. Because storiform fibrosis contains mainly nonfibrillar collagens, "interstitial fibrosclerosis" would be a suitable term to reflect this. The relation between the location and components of fibrosis determined in whole kidney samples provides new clues to the pathophysiology underlying IgG4-RKD. PMID:27246178

  1. A Rare Case of Diffuse Large B-cell Lymphoma in a Patient with IgG4-Related Autoimmune Pancreatitis.

    PubMed

    Nishimura, Yoshito; Iwamuro, Masaya; Ocho, Kazuki; Hasegawa, Kou; Kimura, Kosuke; Hanayama, Yoshihisa; Kondo, Eisei; Tanaka, Takehiro; Otsuka, Fumio

    2016-08-01

    A 61-year-old Japanese man with IgG4-related autoimmune pancreatitis was referred to our hospital because of perspiration during food intake. Abdominal computed tomography (CT) with contrast media revealed multiple mesenteric lymphadenopathies. An open surgical abdominal biopsy and subsequent histopathological analysis revealed abnormally large lymphoid cells that were negative for CD3, CD5, and c-myc and positive for CD20 and bcl-2, leading to a diagnosis of diffuse large B-cell lymphoma. Here, we discuss the risk of malignancies, particularly malignant lymphoma in patients with IgG4-related disease. The importance of pathological analysis to reach the appropriate diagnosis in such cases should be emphasized. PMID:27549673

  2. Diagnostic Performance of Serum IgG4 Levels in Patients With IgG4-Related Disease

    PubMed Central

    Yu, Kuang-Hui; Chan, Tien-Ming; Tsai, Ping-Han; Chen, Ching-Hui; Chang, Pi-Yueh

    2015-01-01

    Abstract The aim of this study is to study the clinical features and diagnostic performance of IgG4 in Chinese populations with IgG4-related diseases (IgG4-RDs). The medical records of 2901 adult subjects who underwent serum IgG4 level tests conducted between December 2007 and May 2014 were reviewed. Serum concentrations of IgG4 were measured in 2901 cases, including 161 (5.6%) patients with IgG4-RD and 2740 (94.4%) patients without IgG4-RD (non-IgG4-RD group). The mean age of the IgG4-RD patients was 58.4 ± 16.1 years (range: 21–87), and 48 (29.8%) were women. The mean serum IgG4 level was significantly much higher in IgG4-RD patients than in non-IgG4-RD (1062.6 vs 104.3 mg/dL, P < 0.001) participants. For IgG4 >135 mg/dL, the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio (LR)+, and LR− were 86%, 77%, 18%, 99%, 3.70, and 0.19, respectively. When the upper limit of normal was doubled for an IgG4 >270 mg/dL, the corresponding data were 75%, 94%, 43%, 98%, 12.79, and 0.26, respectively. For IgG4 >405 mg/dL (tripling the upper limit of normal), the corresponding data were 62%, 98%, 68%, 98%, 37.00, and 0.39, respectively. When calculated according to the manufacturer's package insert cutoff (>201 mg/dL) for the diagnosis of IgG4-RD, the corresponding sensitivity, specificity, PPV, NPV, LR+, and LR− were 80%, 89%, 29%, 99%, 7.00, and 0.23, respectively. For IgG4 >402 mg/dL (>2× the upper limit of the normal range), the corresponding data were 62%, 98%, 68%, 98%, 36.21, and 0.39, respectively. For IgG4 >603 mg/dL (>3× the upper limit of the normal range), the corresponding data were 50%, 99%, 84%, 97%, 90.77 and 0.51, respectively. The optimal cutoff value of serum IgG4 (measured by nephelometry using a Siemens BN ProSpec instrument and Siemens reagent) for the diagnosis of IgG4-RD was 248 mg/dL, the sensitivity and specificity were 77.6% and 92.8%, respectively. The present study demonstrated that 2 or 3 times the upper limit of the manufacturer's reference range of the IgG4 level was a useful marker for the diagnosis of various types of IgG4-RD and the optimal cutoff level was 248 mg/dL. PMID:26469909

  3. Purely cutaneous sclerosing IgG4-related disease of the cephalic region: case report and a mini-review of the clinical and pathological aspects.

    PubMed

    Muscardin, Luca; Paolino, Giovanni; Panetta, Chiara; Donati, Pietro

    2016-06-01

    IgG4-related disease is a recently defined emerging entity. Many different organs may be affected by this disease: pancreas, salivary and lacrimal glands, liver, peritoneum and lung. Also the skin may be affected, as secondary localization, while as primary cutaneous localization it has been rarely described. A male patient presented at our Institute with a two-year history of sclerosing erythematous nodules of the scalp. Histological examination showed a T-lymphocyte (CD3+) infiltrate with interspersed plasmacytoid cells and the interposition of a fibrosclerotic tissue. We found numerous IgG4+ cells at the periphery of the nodular structures, while the serum levels of IgG4 and the remaining blood chemistry analysis were normal. Only a few cases of primitive cutaneous pseudo-lymphomatous IgG4-related disease have been described in the literature. Our case showed the same clinical and histologic features of those previously described; the etiology of IgG4-related diseases remains to be elucidated. PMID:26340764

  4. Plasmacytoid Dendritic Cell Activation and IFN-α Production Are Prominent Features of Murine Autoimmune Pancreatitis and Human IgG4-Related Autoimmune Pancreatitis.

    PubMed

    Arai, Yasuyuki; Yamashita, Kouhei; Kuriyama, Katsutoshi; Shiokawa, Masahiro; Kodama, Yuzo; Sakurai, Toshiharu; Mizugishi, Kiyomi; Uchida, Kazushige; Kadowaki, Norimitsu; Takaori-Kondo, Akifumi; Kudo, Masatoshi; Okazaki, Kazuichi; Strober, Warren; Chiba, Tsutomu; Watanabe, Tomohiro

    2015-10-01

    The abnormal immune response accompanying IgG4-related autoimmune pancreatitis (AIP) is presently unclear. In this study, we examined the role of plasmacytoid dendritic cell (pDC) activation and IFN-α production in this disease as well as in a murine model of AIP (MRL/Mp mice treated with polyinosinic-polycytidylic acid). We found that the development of AIP in treated MRL/Mp mice occurred in parallel with pancreatic accumulation of pDCs producing IFN-α, and with pDC depletion and IFN-α-blocking studies, we showed that such accumulation was necessary for AIP induction. In addition, we found that the pancreas of treated MRL/Mp mice contained neutrophil extracellular traps (NETs) shown previously to stimulate pDCs to produce IFN-α. Consistent with these findings, we found that patients with IgG4-related AIP also exhibited pancreatic tissue localization of IFN-α-expressing pDCs and had significantly higher serum IFN-α levels than healthy controls. In addition, the inflamed pancreas of these patients but not controls also contained NETs that were shown to be capable of pDC activation. More importantly, patient pDCs cultured in the presence of NETs produced greatly increased levels of IFN-α and induced control B cells to produce IgG4 (but not IgG1) as compared with control pDCs. These data suggest that pDC activation and production of IFN-α is a major cause of murine AIP; in addition, the increased pDC production of IFN-α and its relation to IgG4 production observed in IgG4-related AIP suggest that this mechanism also plays a role in the human disease. PMID:26297761

  5. IgG4-Related Kidney Disease in a Patient With History of Breast Cancer: Findings on 18F-FDG PET/CT.

    PubMed

    Krebs, Simone; Monti, Serena; Seshan, Surya; Fox, Josef; Mannelli, Lorenzo

    2016-08-01

    A 64-year-old African American woman with history of stage III breast carcinoma, 3 years after complete response to therapy, presents with progressive fatigue, increasing arthralgia, and unintentional weight loss of 15 lb in 3 months. An F-FDG PET demonstrated new diffuse FDG avidity of the renal parenchyma, new FDG-avid foci in pancreas and lungs, and new FDG-avid lymph nodes above and below the diaphragm. While a retroperitoneal lymph node biopsy was inconclusive, a kidney biopsy resulted in diagnosis of diffuse, severe, IgG4-related tubulointerstitial nephritis. Treatment with corticosteroid led to complete resolution of the symptoms and PET findings. PMID:27187735

  6. Probable IgG4-related sclerosing disease presenting as a gastric submucosal tumor with an intense tracer uptake on PET/CT: a case report.

    PubMed

    Otsuka, Ryota; Kano, Masayuki; Hayashi, Hideki; Hanari, Naoyuki; Gunji, Hisashi; Hayano, Koichi; Matsubara, Hisahiro

    2016-12-01

    A 44-year-old man consulted an internist because of abnormalities in an upper gastrointestinal series. It showed an elevated lesion with central depression in the greater curvature of the middle part of the stomach. Upper gastrointestinal endoscopy showed an elevated lesion with central depression, bridging hold, and no abnormalities of the gastric mucosa in the greater curvature of the middle part of the stomach. Endoscopic ultrasonography showed a submucosal tumor derived from the muscle layer of the stomach. Computed tomography showed a 22-mm tumor in the upper part of the stomach. Integrated position emission tomography/computed tomography (PET/CT) showed an intense tracer uptake by the tumor. Based on these findings, a gastrointestinal stromal tumor was suspected and laparoscopic endoscopic cooperative surgery was performed. A histopathological examination showed lymphoplasmacytic infiltration and fibrosis, and an immunohistochemical analysis showed the infiltration of IgG4-positive lymphoplasmacytic cells. The probable diagnosis was IgG4-related sclerosing disease of the stomach. We herein describe a rare case of probable IgG4-related sclerosing disease which presented as a gastric submucosal tumor. PET/CT is a useful imaging technique for the diagnosis and follow-up of this disease. PMID:27059471

  7. Characterization and Comparison of Patient Subgroups Suspicious for IgG4-Related Disease and Malignant Lymphoma in Patients Followed-up for Sjögren's Syndrome.

    PubMed

    Szántó, Antónia; Szabó, Katalin; Nagy, Gábor; Molnár, Csaba; Zeher, Margit

    2016-07-01

    Differential diagnosis of patients with Sjögren's syndrome (SS), IgG4-related disease (IgG4-RD) and SS patients having high risk for lymphoma (LHR) can be challenging. Some patients with IgG4-RD might be misdiagnosed as having SS. There are special symptoms of SS that raise the possibility of IgG4-RD whereas other symptoms identify patients as having LHR. The purpose of this study was to characterize and compare patients with SS, possible IgG4-RD and SS patients with LHR. Sixty-five SS patients were divided into 4 subgroups according to having possible IgG4-RD (n = 15), LHR (n = 16), eligible for both aforementioned groups (n = 20) and not eligible for either group (n = 14), respectively. Four patients fulfilled the diagnostic criteria for IgG4-RD. The serum levels of IgG4 were significantly higher in patients suspicious for IgG4-RD compared to that of LHR patients (0.46 g/l vs. 0.12 g/l, p = 0.032). Shared features of the patient groups (salivary gland swelling (SGS) and lymphadenopathy), were separately analysed: SGS patients had higher IgG4/IgG ratio (p = 0.036), lymphadenopathic patients had higher IgG4 levels (p = 0.042). Some patients may be "hidden" under the diagnosis of SS. Although patients with LHR and patients with possible IgG4-RD share some symptoms, they differ significantly regarding IgG4 levels and IgG4/IgG ratio. PMID:26786867

  8. DNA Microarray Analysis of Submandibular Glands in IgG4-Related Disease Indicates a Role for MARCO and Other Innate Immune-Related Proteins

    PubMed Central

    Ohta, Miho; Moriyama, Masafumi; Maehara, Takashi; Gion, Yuka; Furukawa, Sachiko; Tanaka, Akihiko; Hayashida, Jun-Nosuke; Yamauchi, Masaki; Ishiguro, Noriko; Mikami, Yurie; Tsuboi, Hiroto; Iizuka-Koga, Mana; Kawano, Shintaro; Sato, Yasuharu; Kiyoshima, Tamotsu; Sumida, Takayuki; Nakamura, Seiji

    2016-01-01

    Abstract IgG4-related disease (IgG4-RD) is a novel systemic disease entity characterized by elevated serum IgG4 and tissue infiltration of IgG4-positive plasma cells accompanied by severe fibrosis. Although recent studies demonstrated that innate immune cells including monocytes and macrophages might promote local fibrosis and IgG4 production, the pathological mechanism remains unclear. In this study, we sought to identify the disease-associated genes, especially innate immune molecules. Gene expression was analyzed by DNA microarray in submandibular glands (SMGs) from patients with IgG4-RD (n = 5), chronic sialoadenitis (CS) (n = 3), and controls (n = 3). Differentially expressed genes (DEGs) were validated by real-time polymerase chain reaction (PCR) and immunohistochemical staining in IgG4-RD (n = 18), CS (n = 4), Sjögren syndrome (n = 11), and controls (n = 10). Gene expression patterns in the 3 groups were quite different from each other by the pvclust method and principal components analysis. In IgG4-RD, 1028 upregulated genes and 692 downregulated genes were identified as DEGs (P < 0.05). Gene Ontology (GO) term analysis indicated that the upregulated DEGs in IgG4-RD encoded proteins involved in T/B cell activation and chemotaxis. PCR validated significantly higher expression of macrophage receptor with collagenous structure (MARCO), a pattern-recognition receptor, in IgG4-RD compared with the other groups (P < 0.01). Immunohistochemical analysis confirmed that the expression pattern of MARCO was similar to that of the M2 macrophage marker CD163. MARCO was identified as a disease-associated molecule in IgG4-RD by DNA microarray. Moreover, M2 macrophages might contribute to the initiation of IgG4-RD via MARCO. PMID:26886650

  9. DNA Microarray Analysis of Submandibular Glands in IgG4-Related Disease Indicates a Role for MARCO and Other Innate Immune-Related Proteins.

    PubMed

    Ohta, Miho; Moriyama, Masafumi; Maehara, Takashi; Gion, Yuka; Furukawa, Sachiko; Tanaka, Akihiko; Hayashida, Jun-Nosuke; Yamauchi, Masaki; Ishiguro, Noriko; Mikami, Yurie; Tsuboi, Hiroto; Iizuka-Koga, Mana; Kawano, Shintaro; Sato, Yasuharu; Kiyoshima, Tamotsu; Sumida, Takayuki; Nakamura, Seiji

    2016-02-01

    IgG4-related disease (IgG4-RD) is a novel systemic disease entity characterized by elevated serum IgG4 and tissue infiltration of IgG4-positive plasma cells accompanied by severe fibrosis. Although recent studies demonstrated that innate immune cells including monocytes and macrophages might promote local fibrosis and IgG4 production, the pathological mechanism remains unclear. In this study, we sought to identify the disease-associated genes, especially innate immune molecules.Gene expression was analyzed by DNA microarray in submandibular glands (SMGs) from patients with IgG4-RD (n = 5), chronic sialoadenitis (CS) (n = 3), and controls (n = 3). Differentially expressed genes (DEGs) were validated by real-time polymerase chain reaction (PCR) and immunohistochemical staining in IgG4-RD (n = 18), CS (n = 4), Sjögren syndrome (n = 11), and controls (n = 10).Gene expression patterns in the 3 groups were quite different from each other by the pvclust method and principal components analysis. In IgG4-RD, 1028 upregulated genes and 692 downregulated genes were identified as DEGs (P < 0.05). Gene Ontology (GO) term analysis indicated that the upregulated DEGs in IgG4-RD encoded proteins involved in T/B cell activation and chemotaxis. PCR validated significantly higher expression of macrophage receptor with collagenous structure (MARCO), a pattern-recognition receptor, in IgG4-RD compared with the other groups (P < 0.01). Immunohistochemical analysis confirmed that the expression pattern of MARCO was similar to that of the M2 macrophage marker CD163.MARCO was identified as a disease-associated molecule in IgG4-RD by DNA microarray. Moreover, M2 macrophages might contribute to the initiation of IgG4-RD via MARCO. PMID:26886650

  10. Erdheim-Chester Disease as a Mimic of IgG4-Related Disease: A Case Report and a Review of a Single-Center Cohort.

    PubMed

    Gianfreda, Davide; Musetti, Claudio; Nicastro, Maria; Maritati, Federica; Cobelli, Rocco; Corradi, Domenico; Vaglio, Augusto

    2016-05-01

    Immunoglobulin-G4 (IgG4)-related disease (IgG4RD) is a fibro-inflammatory disorder characterized by tissue-infiltrating IgG4 plasma cells, and, often, high serum IgG4. Several autoimmune, infectious, or proliferative conditions mimic IgG4RD. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by foamy histiocytic infiltration, fibrosis, and chronic inflammation. ECD and IgG4RD manifestations may overlap.A patient presented with huge fibrous retroperitoneal masses causing compression on neighboring structures; the case posed the challenge of the differential diagnosis between IgG4RD and ECD mainly because of a prominent serum and tissue IgG4 response.Retroperitoneal biopsy led to the diagnosis of ECD; the V600E BRAF mutation was found. Treatment with the BRAF inhibitor vemurafenib was started.Treatment failed to induce mass regression and the patient died after 3 months of therapy. Prompted by this case, we examined serum and tissue IgG4 in a series of 15 ECD patients evaluated at our center, and found that approximately one-fourth of the cases have increased IgG4 in the serum and often in the tissue.The differential diagnosis between IgG4RD and ECD can be challenging, as some ECD patients have prominent IgG4 responses. This suggests the possibility of common pathogenic mechanisms between ECD and IgG4RD. PMID:27227923

  11. Management of ocular, orbital, and adnexal trauma

    SciTech Connect

    Spoor, T.C.; Nesi, F.A.

    1988-01-01

    This book contains 20 chapters. Some of the chapter titles are: The Ruptured Globe: Primary Care; Corneal Trauma, Endophthalmitis; Antibiotic Usage; Radiology of Orbital Trauma; Maxillofacial Fractures; Orbital Infections; and Basic Management of Soft Tissue Injury.

  12. Ocular Adnexal Lymphoma Presenting with Visual Loss

    PubMed Central

    Gulati, Shuchi; Corrêa, Zélia M.; Karim, Nagla; Medlin, Stephen

    2016-01-01

    Context: Elderly patients with visual loss often have age-related macular degeneration, diabetic retinopathy, glaucoma, and cataract as common causes of visual loss. Other less common etiologies should be considered, especially in those presenting with systemic associations. Case Report: The patient discussed in our review is an 80-year-old female, with a history of diabetic retinopathy and macular degeneration who presented with a sudden deterioration of vision. While this was initially attributed to diabetic retinopathy, she was eventually noted to have a salmon patch lesion in her conjunctiva, diagnosed on biopsy to be a diffuse large B-cell lymphoma. Conclusion: Because of the significant rate of disseminated disease among patients with lymphomas in the orbit that carries a worse prognosis, early diagnosis is essential to promote better overall survival of these patients. We describe here a patient diagnosed with conjunctival lymphoma associated with pronounced visual loss and review the literature on this subject. PMID:27011948

  13. Diagnostic Value of Serum IgG4 for IgG4-Related Disease: A PRISMA-compliant Systematic Review and Meta-analysis.

    PubMed

    Hao, Mingju; Liu, Min; Fan, Gaowei; Yang, Xin; Li, Jinming

    2016-05-01

    Many studies about serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD) have been reported. However, these studies had relatively small sample sizes and the diagnostic accuracy values varied much between them.The aim of this study was to perform a meta-analysis to evaluate the diagnostic value of serum IgG4 for IgG4-RD.We conducted a search of relevant articles using MEDLINE, EMBASE, Web of Science, SCOPUS, and Cochrane Library databases published before December 2015.Studies those assessed the diagnostic accuracy of serum IgG4 for IgG4-RD and those provided the cut-off value for serum IgG4 were included.Data were synthesized using the random-effect model. Statistical analysis was performed using STATA with the MIDAS module and Meta-DiSc 1.4 software.A total of 9 case-control studies were analyzed, which included 1235 patients with IgG4-RD and 5696 overall controls. The pooled estimate, for a cut-off value ranged from 135 to 144 mg/dL, produced a sensitivity of 87.2% (95% CI, 85.2-89.0%) and a specificity of 82.6% (95% CI, 81.6-83.6%). The positive likelihood ratio (PLR), negative likelihood ratio (NLR), and diagnostic odds ratio (DOR) were 6.48 (95% CI, 3.98-10.57), 0.14 (95% CI, 0.09-0.21), and 45.15 (95% CI, 23.41-87.06), respectively. The area under the curve (AUC) of the summary receiver operating characteristic curve (SROC) was 0.94 (0.92-0.96). When a cut-off value of 2-fold the upper limit of normal was used (ranged from 270 to 280 mg/dL), the pooled sensitivity was 63% (95% CI, 60.0-66.0%), and the specificity was 94.8% (95% CI, 94.1-95.4%). The PLR, NLR, and DOR were 13.3 (95% CI, 7.39-24.0), 0.41 (95% CI, 0.29-0.58) and 33.42 (95% CI, 13.88-80.43), respectively. The AUC of the SROC was 0.92 (0.90-0.94).Only a relatively small number of studies were included, and significant heterogeneity was observed in this meta-analysis.Serum IgG4 is a modestly effective marker to diagnose IgG4-RD. Doubling the cut-off value for IgG4 could not improve the overall test characteristics. A high specificity inevitably accompanies with a significant sacrifice in sensitivity. PMID:27227950

  14. IgG4-related tubulointerstitial nephritis associated with only lymphadenopathy and without elevated serum IgG4 or renal imaging abnormalities: a case report and literature review.

    PubMed

    Qiao, Xi; Wang, Lihua; Wang, Chen; Gao, Lifang; Yao, Shulei; Wu, Liran; Zhang, Xiaoqin

    2015-01-01

    IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common renal manifestation of IgG4-related kidney disease (IgG4-RKD) and may cause acute or chronic renal dysfunction. Imaging often shows heterogeneous densities in the kidneys, such as a mass or multiple nodules. Serology usually demonstrates high levels of serum IgG4 and total IgG. Most patients have other organs involvement by IgG4 related disease. Although lymphadenopathy is frequently observed in patients with IgG4-TIN, it is rarely presented as the only extrarenal lesion. Herein, we present a rare case of IgG4-TIN associated with only lymphadenopathy and without elevated serum IgG4 or renal imaging abnormalities. A 61-year-old Chinese man was admitted to our hospital with seven months history of generalized lymphadenopathy and five months history of renal dysfunction. His renal imaging was normal. He had no current or previous clinical, radiographic, and/or histologic evidence of other organ involvement except for the lymphadenopathy. Renal biopsy indicated plasma cell-rich TIN with an increased number of IgG4-positive plasma cells and storiform fibrosis. Repeated lymph nodes biopsy revealed IgG4-related lymphadenopathy. However, he did not have elevated serum IgG4 or total IgG levels. Oral prednisone therapy improved his renal function and lymphadenopathy. These findings supported our final diagnosis of IgG4-TIN. Clinicians should be aware of this condition and steroid therapy should be considered for such patients. An early diagnosis and appropriate therapy can induce remission and preserve renal function. PMID:26770608

  15. Anterior Orbit and Adnexal Amyloidosis

    PubMed Central

    Al Hussain, Hailah; Edward, Deepak P.

    2013-01-01

    Purpose: To describe six cases of anterior orbital and adnexal amyloidosis and to report on proteomic analysis to characterize the nature of amyloid in archived biopsies in two cases. Materials and Methods: The clinical features, radiological findings, pathology, and outcome of six patients with anterior orbit and adnexal amyloidosis were retrieved from the medical records. The biochemical nature of the amyloid was determined using liquid chromatography/mass spectroscopy archived paraffin-embedded tissue in two cases. Results: Of the six cases, three had unilateral localized anterior orbit and lacrimal gland involvement. Four of the six patients were female with an average duration of 12.8 years from the time of onset to presentation eyelid infiltration by amyloid caused ptosis in five cases. CT scan in patients with lacrimal gland involvement (n = 3) demonstrated calcified deformable anterior orbital masses and on pathological exmaintionamyloid and calcific deposits replaced the lacrimal gland acini. Ptosis repair was performed in three patients with good outcomes. One patient required repeated debulking of the mass and one patient had recurrenct disease. Proteomic analysis revealed polyclonal IgG-associated amyloid deposition in one patient and AL kappa amyloid in the second patient. Conclusion: Amyloidosis of the anterior orbit and lacrimal gland can present with a wide spectrum of findings with good outcomes after surgical excision. The nature of amyloid material can be precisely determined in archival pathology blocks using diagnostic proteomic analysis. PMID:24014979

  16. Role of Fine Needle Aspiration Cytology as a Diagnostic Tool in Orbital and Adnexal Lesions

    PubMed Central

    Khan, Lubna; Malukani, Kamal; Malaiya, Siddharth; Yeshwante, Prashant; Ishrat, Saba; Nandedkar, Shirish S.

    2016-01-01

    Purpose: To evaluate the role of fine needle aspiration (FNAC) as a diagnostic tool in cases of orbital and ocular adnexal masses. Cytological findings were correlated with histopathological diagnosis wherever possible. Methods: FNAC was performed in 29 patients of different age groups presenting with orbital and ocular adnexal masses. Patients were evaluated clinically and investigated by non-invasive techniques before fine needle aspiration of the masses. Smears were analyzed by a cytologist in all cases. Further, results of cytology were compared with the histopathological diagnosis. Results: The age of patients ranged from 1 to 68 years (mean: 29.79±19.29). There were 14 males and 15 females with a male to female ratio of 0.93:1. Out of 29 cases, 26 aspirates were cellular. Cellularity was insufficient in three (10.34%) aspirates. Out of 26 cellular aspirates, 11 were non-neoplastic while 15 were neoplastic on cytology. Subsequent histopathologic examination was done in 21/26 cases. Concordance rate of FNAC in orbital and ocular adnexal mass lesions with respect to the precise histologic diagnosis was 90%. Conclusion: When properly used in well-indicated patients (in cases where a diagnosis cannot be made by clinical and imaging findings alone), FNAC of orbital and periorbital lesions is an invaluable and suitable adjunct diagnostic technique that necessitates close cooperation between the ophthalmologist and cytologist. However, nondiagnostic aspirates may sometimes be obtained, and an inconclusive FNAC should not always be ignored. PMID:27621787

  17. Diagnosis and Management of Adnexal Masses.

    PubMed

    Biggs, Wendy S; Marks, Sarah Tully

    2016-04-15

    Adnexal masses can have gynecologic or nongynecologic etiologies, ranging from normal luteal cysts to ovarian cancer to bowel abscesses. Women who report abdominal or pelvic pain, increased abdominal size or bloating, difficulty eating, or rapid satiety that occurs more than 12 times per month in less than a year should be evaluated for ovarian cancer. Pelvic examination has low sensitivity for detecting an adnexal mass; negative pelvic examination findings in a symptomatic woman should not deter further workup. Ectopic pregnancy must be ruled out in women of reproductive age. A cancer antigen 125 (CA 125) test may assist in the evaluation of an adnexal mass in appropriate patients. CA 125 levels are elevated in conditions other than ovarian cancer. Because substantial overlap in CA 125 levels between pre- and postmenopausal women may occur, this level alone is not recommended for differentiating between a benign and a malignant adnexal mass. Transvaginal ultrasonography is the first choice for imaging of an adnexal mass. Large mass size, complexity, projections, septation, irregularity, or bilaterality may indicate cancer. If disease is suspected outside of the ovary, computed tomography may be indicated; magnetic resonance imaging may better show malignant characteristics in the ovary. Serial ultrasonography and periodic measurement of CA 125 levels may help in differentiating between benign or potentially malignant adnexal masses. If an adnexal mass larger than 6 cm is found on ultrasonography, or if findings persist longer than 12 weeks, referral to a gynecologist or gynecologic oncologist is indicated. PMID:27175840

  18. Antibiotic therapy for ocular infection.

    PubMed Central

    Snyder, R W; Glasser, D B

    1994-01-01

    Infections of the eye can rapidly damage important functional structures and lead to permanent vision loss or blindness. Broad-spectrum antibiotics should be administered to the appropriate site of infection as soon as a diagnosis is made. Topical drops are preferred for corneal and conjunctival infections. Intravitreal antibiotics, and possibly subconjunctival and parenteral antibiotics, are preferred for endophthalmitis. Parenteral antibiotics are recommended for infection in deep adnexal structures. We review specific aspects of antibiotic therapy for ocular and periocular infection. PMID:7856158

  19. Ocular Hypertension

    MedlinePlus

    ... Español Eye Health / Eye Health A-Z Ocular Hypertension Sections What Is Ocular Hypertension? Ocular Hypertension Causes ... Hypertension Diagnosis Ocular Hypertension Treatment What Is Ocular Hypertension? Written by: Kierstan Boyd Reviewed by: J Kevin ...

  20. Rituximab as Single Agent in Primary MALT Lymphoma of the Ocular Adnexa

    PubMed Central

    Annibali, Ombretta; Chiodi, Francesca; Sarlo, Chiara; Cortes, Magdalena; Quaranta-Leoni, Francesco M.; Quattrocchi, Carlo; Bianchi, Antonella; Bonini, Stefano; Avvisati, Giuseppe

    2015-01-01

    Ocular Adnexal Lymphomas are the first cause of primary ocular malignancies, and among them the most common are MALT Ocular Adnexal Lymphomas. Recently systemic immunotherapy with anti-CD20 monoclonal antibody has been investigated as first-line treatment; however, the optimal management for MALT Ocular Adnexal Lymphomas is still unknown. The present study evaluated retrospectively the outcome of seven consecutive patients with primary MALT Ocular Adnexal Lymphomas, of whom six were treated with single agent Rituximab. All patients received 6 cycles of Rituximab 375 mg/mq every 3 weeks intravenously. The overall response rate was 100%; four patients (67%) achieved a Complete Remission, and two (33%) achieved a partial response. In four patients an additional Rituximab maintenance every 2-3 months was given for two years. After a median follow-up of 29 months (range 8–34), no recurrences were observed, without of therapy- or disease-related severe adverse events. None of the patients needed additional radiotherapy or other treatments. Rituximab as a single agent is highly effective and tolerable in first-line treatment of primary MALT Ocular adnexal Lymphomas. Furthermore, durable responses are achievable with the same-agent maintenance. Rituximab can be considered the agent of choice in the management of an indolent disease in whom the “quality of life” matter is of primary importance. PMID:26425558

  1. Microcystic adnexal carcinoma following radiotherapy in childhood

    SciTech Connect

    Borenstein, A.; Seidman, D.S.; Trau, H.; Tsur, H. )

    1991-04-01

    A 36-year-old man was treated by radiotherapy for tinea capitis many years before discovery of microcystic adnexal carcinoma (MAC). Because of patient's refusal of any surgical intervention, we were able to follow the natural course of this tumor for 13 years. This case emphasizes the typical slow development of (MAC). The implication of the association of MAC and radiotherapy are discussed.

  2. Adnexal torsion: review of the literature.

    PubMed

    Sasaki, Kirsten J; Miller, Charles E

    2014-01-01

    Adnexal torsion is one of a few gynecologic surgical emergencies. Misdiagnosis or delay in treatment can have permanent sequelae including loss of an ovary with effect on future fertility, peritonitis, and even death. A PubMed search was performed between 1985 and 2012 for reviews, comparative studies, and case reports to provide a review of the epidemiology, risk factors, clinical presentation, common laboratory and imaging findings, and treatments of adnexal torsion. Common symptoms of torsion include pain, nausea, and vomiting, with associated abdominal or pelvic tenderness, and may differ in premenarchal and pregnant patients. Laboratory and imaging findings including ultrasound with Doppler analysis, computed tomography, and magnetic resonance imaging can assist in making the diagnosis but should not trump clinical judgment; normal Doppler flow can be observed in up to 60% of adnexal torsion cases. Treatment depends on the individual patient but commonly includes detorsion, even if the adnexae initially seem necrotic, with removal of any associated cysts or salpingo-oophorectomy, because recurrence rates are higher with detorsion alone or detorsion with only cyst aspiration. PMID:24126258

  3. Adnexal mass evaluation in the emergency department.

    PubMed

    Morgan, A

    2001-08-01

    Female patients who have stable vital signs presenting to the emergency department with abdominopelvic pain and an adnexal mass can be extremely difficult to manage. However, by performing a rapid problem-oriented history and physical with emphasis on the age of patient, menstrual history, and pelvic exam, a detailed differential diagnosis can be compiled. With the addition of a complete blood count and HCG, if appropriate, and a CA-125 for your consultant, additional information can be obtained while a ultrasound examination is being performed. With the above information, consultation and disposition should be readily accomplished. PMID:11554288

  4. Meibomian glands and ocular surface inflammation.

    PubMed

    Suzuki, Tomo; Teramukai, Satoshi; Kinoshita, Shigeru

    2015-04-01

    The purpose of this review was to systematically analyze publications related to the role of meibomian gland disease in ocular surface inflammation, with special reference to meibomitis as an inflammatory form of meibomian gland dysfunction (MGD). Meibomian gland inflammation is often present with the ocular surface inflammation in conditions such as blepharokeratoconjunctivitis, ocular rosacea, and phlyctenular keratitis, but its contribution is often overlooked, especially in younger subjects. This can result in misdiagnosis, mistreatment, and, sometimes, severe visual impairment. We identified a related disease entity, seen predominantly in young patients, of ocular surface inflammation associated with meibomitis, which we termed meibomitis-related keratoconjunctivitis. Its specific clinical features are similar to those observed in the above-mentioned diseases, and the inflammatory form of MGD was found to be closely involved in the ocular surface inflammation seen in those four diseases, based on our statistical evaluation. The diagnosis and management of meibomitis, an inflammatory form of MGD, is vital for the successful treatment of the induced ocular surface inflammation. We propose that the ocular surface and the adnexal meibomian glands should be considered as one unit, i.e., the "meibomian gland and ocular surface" (MOS), when encountered in the clinical setting. PMID:25881997

  5. Gastrointestinal Stromal Tumor: May Mimic Adnexal Mass.

    PubMed

    Karaca, Nilay; Akpak, Yasam Kemal; Tatar, Zeynep; Batmaz, Gonca; Erken, Aslihan

    2016-02-01

    Gastrointestinal stromal tumors (GISTs) are rare tumor of the gastrointestinal tract. GISTs occur in the entire gastrointestinal tract and may also arise from the retroperitoneum, omentum and mesenteries. They are originated from gastrointestinal pacemaker cells (Cajal's interstitial cells) and range from benign tumors to sarcomas at all sites of occurrence. Diagnosis of GIST could be deceptive because of their similarity in appearance to gynecological neoplasms. We would like to present a case of a woman with GIST in the small intestine giving a imprint of an adnexal mass was diagnosed correctly during surgery. The diagnosis and treatment of GIST has been reformed over the past years. It is crucial to separate GISTs from possible misdiagnosis because their prognosis and treatment could be unlike clearly. The purpose of this case is to evaluate this rarely seen clinical entity, and thus, make some contribution to the literature. PMID:26383211

  6. Gastrointestinal Stromal Tumor: May Mimic Adnexal Mass

    PubMed Central

    Karaca, Nilay; Akpak, Yaşam Kemal; Tatar, Zeynep; Batmaz, Gonca; Erken, Aslihan

    2016-01-01

    Gastrointestinal stromal tumors (GISTs) are rare tumor of the gastrointestinal tract. GISTs occur in the entire gastrointestinal tract and may also arise from the retroperitoneum, omentum and mesenteries. They are originated from gastrointestinal pacemaker cells (Cajal’s interstitial cells) and range from benign tumors to sarcomas at all sites of occurrence. Diagnosis of GIST could be deceptive because of their similarity in appearance to gynecological neoplasms. We would like to present a case of a woman with GIST in the small intestine giving a imprint of an adnexal mass was diagnosed correctly during surgery. The diagnosis and treatment of GIST has been reformed over the past years. It is crucial to separate GISTs from possible misdiagnosis because their prognosis and treatment could be unlike clearly. The purpose of this case is to evaluate this rarely seen clinical entity, and thus, make some contribution to the literature. PMID:26383211

  7. Isolated adnexal torsion in a 20-week spontaneous twin pregnancy

    PubMed Central

    Kahramanoglu, Ilker; Eroglu, Vasfiye; Turan, Hasan; Kaval, Gizem; Sal, Veysel; Tokgozoglu, Nedim

    2016-01-01

    Background Adnexal torsion can be a life-threatning condition in pregnancy, while the risk of late diagnosis is increased, in second and third trimester in particular. Laparoscopy is an effective approach in diagnosis and treatment of adnexal torsion. However, entry to abdomen may be challenging in more advanced pregnancies. Case report Herein, we report a case of adnexal torsion during 20th week of twin pregnancy, which was detorsioned laparoscopically. The woman delivered healthy infants at her 36th week of pregnancy. Discussion Adnexal torsion as a cause of acute abdomen may be kept in mind in pregnants, even if there is no predisposing factor. Laparoscopy may be performed safely in 2nd trimester for acute abdomen. PMID:27129134

  8. 21 CFR 866.6050 - Ovarian adnexal mass assessment score test system.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ovarian adnexal mass assessment score test system... immunological Test Systems § 866.6050 Ovarian adnexal mass assessment score test system. (a) Identification. An ovarian/adnexal mass assessment test system is a device that measures one or more proteins in serum...

  9. 21 CFR 866.6050 - Ovarian adnexal mass assessment score test system.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Ovarian adnexal mass assessment score test system... immunological Test Systems § 866.6050 Ovarian adnexal mass assessment score test system. (a) Identification. An ovarian/adnexal mass assessment test system is a device that measures one or more proteins in serum...

  10. 21 CFR 866.6050 - Ovarian adnexal mass assessment score test system.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Ovarian adnexal mass assessment score test system... immunological Test Systems § 866.6050 Ovarian adnexal mass assessment score test system. (a) Identification. An ovarian/adnexal mass assessment test system is a device that measures one or more proteins in serum...

  11. 21 CFR 866.6050 - Ovarian adnexal mass assessment score test system.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ovarian adnexal mass assessment score test system... immunological Test Systems § 866.6050 Ovarian adnexal mass assessment score test system. (a) Identification. An ovarian/adnexal mass assessment test system is a device that measures one or more proteins in serum...

  12. Single-Site Laparoscopic Management of a Large Adnexal Mass

    PubMed Central

    Scribner, Dennis R.; Weiss, Patrice M.

    2013-01-01

    Introduction: Single-site laparoscopy is gaining acceptance in many surgical fields including gynecology. The purpose of this report is to demonstrate the technique and outcome for removing a large adnexal mass through a single site. Case Description: A 41-y-old female was referred to gynecology oncology for increased abdominal girth for 3 mo. An ultrasound confirmed a benign-appearing, 37-cm left adnexal mass. The mass was removed through a single-site laparoscopic incision with the aid of drainage and a morcellator. The operating time was 84 min. The patient was discharged 2 h and 35 min later with full return to normal activity in 5 d. Conclusion: Large, benign-appearing adnexal masses can be managed safely with superior cosmetic results using single-site laparoscopy. PMID:23925036

  13. Ovarian function, reproduction, and later operations following adnexal surgery.

    PubMed

    Ranney, B; Chastain, D

    1978-05-01

    Among 3102 patients who had major gynecologic operations, only 223 (7.18%) needed adnexal operations during which reproductive potential was preserved. Operations involved ovaries (156), tubes (55), and embryologic cysts (32). After recovery 53.69% of these patients had viable babies (64.46% of patients under age 30). Patients were examined up to 28 years after operations, an average 11.32 years. When last examined. most patients still retained physiologic evidence of ovarian hormonal function; 20 patients had passed through spontaneous menopause, at an average age of 49.4 years. Fifty-two patients needed subsequent pelvic operations, but only 7 for possibly related conditions (4 repeat tubal pregnancies and 3 cyst-adenomas). All but one twisted adnexal mass, with gangrene, originated as a paramesonephric duct cyst. Seventeen adnexal operations were associated with pregnancy: 15 mothers had healthy babies, and 2 aborted. Details concerning numbers and characteristics of specific lesions are included in the paper. PMID:652198

  14. Nontuberculous Mycobacterial Ocular Infections: A Systematic Review of the Literature

    PubMed Central

    Kheir, Wajiha J.; Sheheitli, Huda; Abdul Fattah, Maamoun; Hamam, Rola N.

    2015-01-01

    Nontuberculous or atypical mycobacterial ocular infections have been increasing in prevalence over the past few decades. They are known to cause periocular, adnexal, ocular surface and intraocular infections and are often recalcitrant to medical therapy. These infections can potentially cause detrimental outcomes, in part due to a delay in diagnosis. We review 174 case reports and series on nontuberculous mycobacterial (NTM) ocular infections and discuss etiology, microbiology, risk factors, diagnosis, clinical presentation, and treatment of these infections. History of interventions, trauma, foreign bodies, implants, contact lenses, and steroids are linked to NTM ocular infections. Steroid use may prolong the duration of the infection and cause poorer visual outcomes. Early diagnosis and initiation of treatment with multiple antibiotics are necessary to achieve the best visual outcome. PMID:26106601

  15. Fertility following laparoscopic management of benign adnexal cysts.

    PubMed

    Canis, M; Bassil, S; Wattiez, A; Pouly, J L; Manhes, H; Mage, G; Bruhat, M A

    1992-04-01

    Fertility following laparoscopic treatment of benign adnexal cysts without ovarian suture was studied retrospectively. Patients with endometriomas or who were previously infertile were excluded. Thirty-eight patients treated conservatively were included, 10 after partial resection of functional cysts, 23 after an ovarian cystectomy and six after treatment of a paraovarian cyst. One patient had two cysts. The overall intrauterine pregnancy rate was 92%; one patient had an ectopic pregnancy (2.6%). From these results, we conclude that fertility following laparoscopic treatment of adnexal cysts appears to be normal. Technical guidelines to improve laparoscopic cystectomy are proposed. PMID:1387882

  16. Spectrum of cutaneous and soft tissue lesions in two Carney complex patients-adnexal induction versus authentic adnexal neoplasms.

    PubMed

    Kacerovska, Denisa; Requena, Luis; Michal, Michal; Grossmann, Petr; Treskova, Inka; Roucka, Patrik; Kazakov, Dmitry V

    2012-10-01

    Two unusual Carney complex patients are described. In one of them, several cutaneous biopsies revealed myxoid lesions that were rather more close to authentic adnexal neoplasms with myxoid stroma than to a "myxoma with an epithelial component." These included lesions resembling trichofolliculoma, infundibular cyst, and trichodiscoma. Additionally, 1 soft tissue myxoma was unique in the sense that it greatly resembled a cardiac myxoma, begging the question whether this could represent an embolus from the patient's cardiac myxoma. Given the large size and complexity and heterogeneity of the cutaneous lesions, the authors suggest that these may represent authentic cutaneous neoplasms accompanied by myxoid stroma and not adnexal elements induced by the stroma. However, the latter mechanism is well recognized and demonstrated by our second patient in whom adnexal-type elements in the cutaneous lesion were clearly induced by the myxoid stroma but were unusually complex by manifesting panfollicular and also sebaceous differentiation. PMID:22588545

  17. [Ocular syphilis].

    PubMed

    Chiquet, C; Khayi, H; Puech, C; Tonini, M; Pavese, P; Aptel, F; Romanet, J-P

    2014-04-01

    Syphilis is a sexually transmitted disease caused by Treponema pallidum. Previously known as the "great imitator", this disease can have numerous and complex manifestations. The ophthalmologist should suspect the diagnosis in patients with uveitis or optic neuropathy and high-risk sexual behavior and/or another sexually transmitted disease (such as HIV) or those presenting with posterior placoid chorioretinitis or necrotising retinitis. Ocular involvement in acquired syphilis is rare, tending to occur during the secondary and tertiary stages of the disease. Syphilis may affect all the structures of the eye, but uveitis (accounting for 1-5% of the uveitis in a tertiary referral center) is the most common ocular finding. Granulomatous or non-granulomatous iridocyclitis (71%), panuveitis, posterior uveitis (8%) and keratouveitis (8%) are often described. In the secondary stage, the meninges and the central nervous system can be affected, sometimes with no symptoms, which justifies performing lumbar puncture in patients with uveitis and/or optic neuropathy. The diagnosis of ocular syphilis requires screening with a non-treponemal serology and confirmation with a treponemal-specific test. Parenterally administered penicillin G is considered first-line therapy for all stages of ocular syphilis. Systemic corticosteroids are an appropriate adjunct treatment for posterior uveitis, scleritis and optic neuritis if ocular inflammation is severe. Prolonged follow-up is necessary because of the possibility of relapse of the disease. With proper diagnosis and prompt antibiotic treatment, the majority of cases of ocular syphilis can be cured. PMID:24655791

  18. A pelvic retroperitoneal Schwannoma presenting as an adnexal mass.

    PubMed

    Khatib, R A; Khalil, A M; Saba, M I; Aswad, N K; Mroueh, A M

    1994-05-01

    Solitary nerve sheath tumors such as benign schwannomas arising in the pelvic retroperitoneum are infrequently reported. We report a case of a benign retroperitoneal pelvic schwannoma that presented with pelvic pain and an adnexal mass. Complete surgical excision was achieved only after transection of the S1 nerve root on the left side. The adjacent vascular and urinary channels sustained no injuries and the patient had minimal neurologic deficit. PMID:8188087

  19. Added Value of Assessing Adnexal Masses with Advanced MRI Techniques

    PubMed Central

    Thomassin-Naggara, I.; Balvay, D.; Rockall, A.; Carette, M. F.; Ballester, M.; Darai, E.; Bazot, M.

    2015-01-01

    This review will present the added value of perfusion and diffusion MR sequences to characterize adnexal masses. These two functional MR techniques are readily available in routine clinical practice. We will describe the acquisition parameters and a method of analysis to optimize their added value compared with conventional images. We will then propose a model of interpretation that combines the anatomical and morphological information from conventional MRI sequences with the functional information provided by perfusion and diffusion weighted sequences. PMID:26413542

  20. Optical coherence tomography imaging of ocular and periocular tumours

    PubMed Central

    Medina, Carlos A; Plesec, Thomas; Singh, Arun D

    2014-01-01

    Optical coherence tomography (OCT) has become pivotal in the practice of ophthalmology. Similar to other ophthalmic subspecialties, ophthalmic oncology has also incorporated OCT into practice. Anterior segment OCT (AS-OCT), ultra-high resolution OCT (UHR-OCT), spectral domain OCT (SD-OCT) and enhanced depth imaging OCT (EDI-OCT), have all been described to be helpful in the diagnosis, treatment planning and monitoring response of ocular and periocular tumours. Herein we discuss the role of OCT including the advantages and limitations of its use in the setting of common intraocular and adnexal tumours. PMID:24599420

  1. 76 FR 16350 - Medical Devices; Ovarian Adnexal Mass Assessment Score Test System; Labeling; Black Box Restrictions

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-23

    ... HUMAN SERVICES Food and Drug Administration 21 CFR Part 866 Medical Devices; Ovarian Adnexal Mass... regulation classifying ovarian adnexal mass assessment score test systems to restrict these devices so that a... mass assessment score test system into class II (special controls). DATES: Submit either electronic...

  2. Periocular anterior adnexal anatomy and clinical adnexal examination of the adult Asian elephant (Elephas maximus).

    PubMed

    Wong, Michael A; Isaza, Ramiro; Cuthbert, J Kelly; Brooks, Dennis E; Samuelson, Don A

    2012-12-01

    Formalin preserved ocular-associated anterior adnexa tissues from five necropsied Asian elephants (Elephas maximus) were dissected with attention to the palpebrae, conjunctiva, nictitating membranes, nasolacrimal ducts, and periocular glandular tissues. Gross and histologic examination revealed that lacrimal and tarsal glands were not present. Evidence of the lacrimal drainage apparatus, including lacrimal punctae or any remnant of lacrimal sacs, was also absent. In contrast, well-developed sebaceous glands associated with accessory hairs along the palpebrae were exceptionally abundant. Mixed-secreting accessory lacrimal glands were noted in the deep stroma posterior to the tarsus of both palpebrae and the gland of the nictitating membrane. Apparently, the Asian elephant has developed a novel tear system in the absence of lacrimal and tarsal (meibomian) glands. Clinical examinations and bacterial cultures of the visible periocular tissues were performed on eight living adult Asian elephants to confirm the postmortem anatomic findings and provide guidance to the clinician during examination of the elephant conjunctiva. PMID:23272346

  3. 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

  4. 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.

  5. Ocular pharmacology.

    PubMed

    Novack, Gary D; Robin, Alan L

    2016-05-01

    Ophthalmic diseases include both those analogous to systemic diseases (eg, inflammation, infection, neuronal degeneration) and not analogous (eg, cataract, myopia). Many anterior segment diseases are treated pharmacologically through eye drops, which have an implied therapeutic index of local therapy. Unlike oral dosage forms administered for systemic diseases, eyedrops require patients not only to adhere to treatment, but to be able to accurately perform-ie, instill drops correctly. Anatomical and physiological barriers make topical delivery to the anterior chamber challenging-in some cases more challenging than absorption through the skin, nasal passages, or gut. Treatment of the posterior segment (eg, vitreous, retina, choroid, and optic nerve) is more challenging due to additional barriers. Recently, intravitreal injections have become a standard of care with biologics for the treatment of macular degeneration and other diseases. Although the eye has esterases, hydroxylases, and transporters, it has relatively little CYP450 enzymes. Because it is challenging to obtain drug concentrations at the target site, ocular clinical pharmacokinetics, and thus pharmacokinetic-pharmacodynamic interactions, are rarely available. Ophthalmic pharmaceuticals require consideration of solubility, physiological pH, and osmolarity, as well as sterility and stability, which in turn requires optimal pharmaceutics. Although applied locally, ocular medications may be absorbed systemically, which results in morbidity and mortality (eg, systemic hypotension, bronchospasm, and bradycardia). PMID:26360129

  6. Investigation and Management of Adnexal Masses in Pregnancy

    PubMed Central

    Cavaco-Gomes, João; Jorge Moreira, Cátia; Rocha, Anabela; Mota, Raquel; Paiva, Vera; Costa, Antónia

    2016-01-01

    Adnexal masses can be found in 0.19 to 8.8% of all pregnancies. Most masses are functional and asymptomatic and up to 70% resolve spontaneously in the second trimester. The main predictors of persistence are the size (>5 cm) and the imagiological morphocomplexity. Those that persist carry a low risk of malignancy (0 to 10%). Most malignant masses are diagnosed at early stages and more than 50% are borderline epithelial neoplasms. Ultrasound is the preferred method to stratify the risk of complications and malignancy, allowing medical approach planning. Pregnancy and some gestational disorders may modify the levels of tumor markers, whereby their interpretation during pregnancy should be cautious. Large masses are at increased risk of torsion, rupture, and dystocia. When surgery is indicated, laparoscopy is a safe technique and should ideally be carried out in the second trimester of pregnancy. PMID:27119043

  7. Clinical management of incidental findings on pelvic adnexal masses.

    PubMed

    Dias, Daniel Spadoto; Bueloni-Dias, Flávia Neves; Delmanto, Armando; Tonon, Ângela Favorito Santarém; Tayfour, Najla Mohamad; Traiman, Paulo; Dias, Rogério

    2015-01-01

    Due to widespread use of pelvic and transvaginal ultrasound in routine gynecological evaluation, the incidental finding of adnexal masses has led to discussions about management in asymptomatic patients regarding the risk of ovarian cancer. Transvaginal ultrasonography remains the modality of choice in the evaluation of suspicious characteristics. The combined analysis of ultrasound morphological parameters with Doppler study, serum carcinoma antigen 125 and investigation of a symptom index may improve diagnosis. Surgical approach should be considered whenever there are suspicious images, rapid growth of cysts, changes in the appearance compared to the initial evaluation or when the patient has symptoms. Future studies on genetic and molecular mechanisms may help explain the pathophysiology of ovarian cancer, improving early diagnosis and treatment. PMID:26603011

  8. Dermoscopy of Skin Adnexal Neoplasms:A Continuous Challenge.

    PubMed

    di Meo, Nicola; Stinco, Giuseppe; Gatti, Alessandro; Fadel, Mattia; Vichi, Silvia; Trevisan, Giusto

    2016-06-01

    Dear Editor, Apocrine hidradenoma is a rare benign adnexal tumor related to the more common poroma, as they both originate from sweat glands. Hidradenoma usually has an eccrine differentiation, but an apocrine differentiation is possible. Due to its rarity and non-specific clinical appearance it is difficult to differentiate it from other malignant cutaneous lesions. In this challenging task, dermatoscopy could be particularly helpful to better describe, recognize, and differentiate these lesions. Unfortunately, the literature offers only few dermatoscopic descriptions of this rare cutaneous neoplasm. A 70-year-old woman in fair general condition was referred to our Department for an asymptomatic 10×8 mm single nodule on her left scapula. This nodule was red, dome shaped, well circumscribed, firm, and mildly tender (Figure 1). The patient reported that it had been present since approximately 7 months. The lesion was neither painful nor itchy and there was no bleeding. A skin examination did not show any other lesions with the same features or other suspicious lesions. Dermatoscopy revealed milky-pinkish areas with dotted vessels, linear-irregular vessels, and hairpin vessels: some of these aspects can also be found in amelanotic melanoma (1). There were also homogeneous blue areas similar to lacunae, characteristic but not exclusive to vascular or sarcomatous neoplasms and basal cell carcinoma (1,2). Furthermore, small ulcerations covered by an amber crust were identified, which can usually be found in basal cell carcinoma. Regarding the background of the lesion, we noticed peculiar, translucent, pinkish, soft, large lobular areas (Figure 2). On the basis of this analysis, we suspected an atypical presentation of a basal cell carcinoma, an amelanotic melanoma, or a sarcomatous skin neoplasm. A metastasis of an unknown solid tumor was also taken into consideration. The histological examination revealed an adnexal neoplasm and specifically an apocrine hidradenoma

  9. Cutaneous and ocular manifestations of neurocutaneous syndromes.

    PubMed

    Chernoff, Karen A; Schaffer, Julie V

    2016-01-01

    Neurocutaneous syndromes are a heterogeneous group of congenital and hereditary disorders with manifestations in the skin and the nervous system, usually together with ocular features that represent diagnostic clues and potential sources of morbidity. Dermatologists and ophthalmologists often need to work together in identifying and managing patients with these conditions; herein, we focus on classic and under-recognized neurocutaneous syndromes. We begin with autosomal dominant genodermatoses characterized by hamartomas and tumors in the skin, eyes, and central nervous system: neurofibromatosis type 1, tuberous sclerosis complex, and PTEN hamartoma-tumor syndrome. This is followed by a discussion of two mosaic disorders, Sturge-Weber syndrome and neurocutaneous melanocytosis. In addition to providing an update on clinical presentations and evaluation of patients with these conditions, we review recent insights into their pathogenesis, drawing attention to relationships among the diseases on a molecular level and implications regarding treatment. We also highlight the major features of other neurocutaneous syndromes that have ocular findings plus pigmentary, vascular, hyperkeratotic, adnexal, connective tissue, photosensitive, and inflammatory manifestations in the skin. PMID:26903185

  10. Case report: Ovarian cavernous haemangioma presenting as a heavily calcified adnexal mass.

    PubMed

    Kim, M Y; Rha, S E; Oh, S N; Lee, Y J; Jung, E S; Byun, J Y

    2008-11-01

    Haemangioma is an extremely rare tumour of the ovary. We describe the CT and MRI findings of a heavily calcified ovarian haemangioma in a 69-year-old woman presenting with adnexal torsion. CT showed a well-circumscribed mass with extensive punctate calcifications, located posterior to the uterus. MR images showed dark signal intensities of the mass on both T(1) and T(2) weighted images, with poor enhancement of the mass on contrast-enhanced T(1) weighted images. MR images also demonstrated a beak-like protrusion from the adnexal mass, representing the adnexal torsion. This is the first case report of a heavily calcified ovarian haemangioma in the English literature. Although rare, ovarian haemangioma should be included in the differential diagnosis of a heavily calcified adnexal mass. PMID:18941040

  11. Histomorphological and Immunohistochemical Reappraisal of Cutaneous Adnexal Tumours: A Hospital Based Study

    PubMed Central

    Fatima, Uroos; Malaviya, Anil K.

    2016-01-01

    Background. Diagnosing adnexal tumours of the skin is a challenge due to their wide variety, infrequent occurrence in practice, and confusing morphological picture. Aims and Objectives. The present study aims to observe the spectrum of adnexal tumours at our institute and to evaluate them based on histomorphological, histochemical, and immunohistochemical methods either alone or in combination for proper identification and classification. Materials and Methods. A partly retrospective and partly prospective study was conducted on adnexal skin tumours over a period of 6 years. Relevant clinical profile was recorded. Histopathological examination was carried out and special stains were applied as and when required. Immunohistochemistry was performed where diagnosis with routine stains was not possible. Results. A total of 150 skin tumour biopsies were received. There were 87 keratotic tumours, 39 adnexal tumours, and 24 melanocytic tumours. Amongst the adnexal tumours, 51.3% eccrine, 30.8% follicular, and 17.9% sebaceous tumours were seen. In five cases, histological diagnosis was troublesome where immunohistochemistry helped in making final diagnosis. Limitations. The sample size is small. Conclusion. Histomorphology is confirmatory in majority of the adnexal tumours but few rare lesions that mimic internal malignancy require a panel of immunomarkers to rule out other possible differentials. PMID:27034895

  12. Molten metal-related ocular thermal burn: report on two cases.

    PubMed

    Arici, Ceyhun; Iskeleli, Guzin; Atalay, Eray; Mangan, Mehmet Serhat; Kilic, Belgin

    2015-01-01

    We report two cases of severe thermal burns on the ocular surface and its adnexal appendages that developed secondary to exposure to molten heavy metal with a melting temperature of near-thousand degree Celsius. Despite aggressive intervention and strict monitoring, the profound inflammation caused significant damage to the ocular surface, ending up in an intractable infection with an unfavorable outcome. The heat of the molten metal at impact, the heat-retaining capacity of the heavy metal, the total area of the ocular surface exposed to the molten metal, and the duration of exposure determined the severity of the injury. The unfavorable outcome, despite an intensive treatment, in terms of visual acuity and cosmetic appearance, should be explicitly explained to the patient, and a psychiatrist consultation should be considered if necessary. PMID:26677043

  13. Eccrine or apocrine poroma? Six poromas with divergent adnexal differentiation.

    PubMed

    Harvell, J D; Kerschmann, R L; LeBoit, P E

    1996-02-01

    We describe six cases of benign eccrine poroma-like neoplasms with divergent adnexal differentiation. Four cases exhibited sebaceous differentiation in the form of individual or clustered sebocytes with or without sebaceous ducts. One case showed both sebaceous and hair follicle differentiation, and one case showed sebaceous and possible apocrine secretory differentiation. Clinically, most were skin-colored, red, or purple papules or nodules. One patient had a preoperative diagnosis of Bowen's disease, with an erythematous plaque. None recurred following biopsy. Previous reports of similar lesions have suggested a possible role for human papilloma virus (HPV) in their pathogenesis; however, immunohistochemical staining for HPV structural antigens was negative in all six of these cases. Similarities to previously reported cases of eccrine poroma-like neoplasms with sebaceous differentiation are discussed. Given the evidence of sebaceous and follicular differentiation seen in this study and the common embryologic origin of follicular, sebaceous, and apocrine structures, it follows that at least some benign neoplastic proliferations with histopathologic features of "eccrine" poroma could be of apocrine origin. PMID:8721584

  14. Skin adnexal neoplasms—part 2: An approach to tumours of cutaneous sweat glands

    PubMed Central

    Obaidat, Nidal A; Alsaad, Khaled O; Ghazarian, Danny

    2007-01-01

    Tumours of cutaneous sweat glands are uncommon, with a wide histological spectrum, complex classification and many different terms often used to describe the same tumour. Furthermore, many eccrine/apocrine lesions coexist within hamartomas or within lesions with composite/mixed differentiation. In addition to the eccrine and apocrine glands, two other skin sweat glands have recently been described: the apoeccrine and the mammary‐like glands of the anogenital area. In this review (the second of two articles on skin adnexal neoplasms), common as well as important benign and malignant lesions of cutaneous sweat glands are described, and a summary for differentiating primary adnexal neoplasms from metastatic carcinoma is outlined, striving to maintain a common and acceptable terminology in this complex subject. Composite/mixed adnexal tumours are also discussed briefly. PMID:16882695

  15. Benign Vulvar Adnexal Tumours - A 5 year Study in a Tertiary Care Hospital

    PubMed Central

    Tiwana, Kanwardeep Kaur; Nibhoria, Sarita; Kaur, Harpreet; Bajaj, Akanksha; Phutela, Richa

    2015-01-01

    Despite of the fact that the vulva contains a high density of apocrine and anogenital mammary glands in addition to eccrine glands and folliculosebaceous units, the benign vulvar adnexal tumours are rare. Though the varied clinical presentation and diverse histopathological spectrum of vulvar neoplasms has amazed the pathologists, only few studies have been reported in literature. The present five year study consists of only five cases of benign vulvar neoplasms depicting their rarity. Hidradenoma papilliferum and syringoma were the most common entities followed by Chondroid syringoma. The aim of our study is to explore and highlight the histopathological diversity of benign vulvar adnexal tumours reflecting the relative frequency of these structures. PMID:27047567

  16. Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge

    PubMed Central

    Ferraz, Catarina; Nunes, Teresa; Fonseca, Elsa; Vaz, Luísa Guedes

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement. PMID:25705537

  17. A case of adenoid cystic carcinoma associated with IgG4-related disease

    PubMed Central

    Shimo, Tsuyoshi; Yao, Mayumi; Takebe, Yuichiro; Ono, Yuko; Obata, Kyoichi; Kurio, Naito; Ibaragi, Soichiro; Yoshioka, Norie; Kishimoto, Koji; Yanagi, Yoshinobu; Nagatsuka, Hitoshi; Sasaki, Akira

    2015-01-01

    Introduction Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition associated with elevated serum IgG4 levels and tissue infiltration by IgG4-expressing plasma cells. We present a case of adenoid cystic carcinoma (ACC) of the submandibular gland with possible involvement of IgG4-RD. Presentation of case The patient was a 59-year-old man presenting with a swollen right submandibular gland. Laboratory tests revealed IgG4 levels of 176 mg/dl (reference range: 4.8–105). An initial open biopsy for histological diagnosis showed chronic sialadenitis. The region was monitored on an outpatient basis, and finally the right submandibular was totally resected because malignant tumor could not be excluded. Histological examination of the submandibular gland showed an ACC with lymphocytic infiltration containing many IgG4-positive plasma cells in the tumor stroma. Discussion We have described a case that indicated a possible involvement of ACC with IgG4-RD. This allows us to speculate that longstanding IgG4-RD may progress to malignancy or infiltration of IgG4-positive plasma cells through the signals of tumor stimuli. Further investigations are required to determine the potential pathogenic mechanism underlying this unique tumor. Conclusion This case underscores that caution is needed in the diagnosis of masses with high serum IgG4 levels, as the differential diagnosis includes malignancy. PMID:25781921

  18. Autoimmune Pancreatitis and IgG4 Related Disease in Three Children

    PubMed Central

    Chong, Sze Yee; Coleman, Lee; MacGregor, Duncan; Hardikar, Winita; Oliver, Mark R.

    2016-01-01

    We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation. PMID:27622194

  19. Clinicopathological features of Riedel's thyroiditis associated with IgG4-related disease in Japan.

    PubMed

    Takeshima, Ken; Inaba, Hidefumi; Ariyasu, Hiroyuki; Furukawa, Yasushi; Doi, Asako; Nishi, Masahiro; Hirokawa, Mitsuyoshi; Yoshida, Akira; Imai, Ryoukichi; Akamizu, Takashi

    2015-01-01

    Riedel's thyroiditis (RT) is a rare chronic fibrosing disorder characterized by a hard, infiltrative lesion in the thyroid gland, which is often associated with multifocal fibrosclerosis. Immunoglobulin G4-related disease (IgG4-RD) is typified by infiltration of IgG4-positive plasma cells into multiple organs, resulting in tissue fibrosis and organ dysfunction. In order to evaluate the clinicopathological features of RT and its relationship with IgG4-RD, we performed a Japanese literature search using the keywords "Riedel" and "Riedel's thyroiditis." We used the electronic databases Medline and Igaku Chuo Zasshi, the latter of which is the largest medical literature database in Japan. The diagnosis of RT was based on the presence of a fibroinflammatory process with extension into surrounding tissues. Only 10 patients in Japan fulfilled RT diagnostic criteria during the 25-year period between 1988 and 2012. Two patients with confirmed IgG4/IgG immunohistochemical findings demonstrated 43 and 13 IgG4-positive plasma cells per high-power field, respectively, and the IgG4-positive/IgG-positive plasma cell ratios of 20% and less than 5%. Of the 10 patients with RT, two received glucocorticoids, one of whom experienced marked shrinkage of the thyroid lesion. One patient had extra-thyroid involvement in the form of retroperitoneal fibrosis. Although the clinicopathological features of RT suggest that IgG4-RD may be the underlying condition in some cases, further investigation is needed to clarify the etiology of RT in relation to IgG4-RD. PMID:26052139

  20. IgG4-related disease: current challenges and future prospects

    PubMed Central

    Lang, David; Zwerina, Jochen; Pieringer, Herwig

    2016-01-01

    Immunoglobulin G4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with a characteristic histopathological appearance that can affect various organs. Although numerous single-organ manifestations have been described more than a century ago, its systemic nature and unique features were only discovered in the last 2 decades, when IgG4-RD emerged as a new entity of disease. IgG4-RD is usually considered a rare disease, but its true epidemiology has not yet been fully clarified. Also, despite recent advances in the identification of the underlying immunological processes, its pathophysiology is only incompletely understood till now. The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, histological, and serological analyses. However, no finding alone is specific for IgG4-RD. Therefore, its diagnosis requires careful interpretation of examination results in context with the patient’s clinical appearance as well as the exclusion of a broad variety of differential diagnoses. The past years brought rapid advances concerning this novel disease entity: diagnostic criteria, further insights into the underlying immunological processes, new biomarkers, and novel therapeutic approaches were proposed and widened the knowledge in the field of IgG4-RD. Still, a greater number of questions remain unanswered, and many recent developments require further discussion and proof from clinical trials. This review should give an overview on current knowledge and future perspectives in epidemiology, pathophysiology, diagnosis, and therapy of IgG4-RD. PMID:26929632

  1. Autoimmune Pancreatitis and IgG4 Related Disease in Three Children.

    PubMed

    Bolia, Rishi; Chong, Sze Yee; Coleman, Lee; MacGregor, Duncan; Hardikar, Winita; Oliver, Mark R

    2016-07-01

    We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation. PMID:27622194

  2. Clinicopathological analysis of salivary gland tissue from patients with IgG4-related disease.

    PubMed

    Takano, Kenichi; Nomura, Kazuaki; Abe, Ayumi; Kamekura, Ryuta; Yamamoto, Motohisa; Ichimiya, Shingo; Takahashi, Hiroki; Himi, Tetsuo

    2016-07-01

    Conclusion The diagnosis of immunoglobulin G4-related disease (IgG4-RD) should be based on the morphology of tissue biopsy, and this study recommends a submandibular gland (SMG) biopsy for accurate diagnosis and to exclude malignant disease. Objective To clarify which type of biopsy specimen (SMG or labial salivary gland [LSG]) should be taken from patients with IgG4-RD. Methods This study included 33 patients with IgG4-RD (21 women; 12 men) who were subjected to both SMG and LSG biopsies at Sapporo Medical University between 2011-2015. Tissues obtained from the SMG and LSG specimens were evaluated. Results All SMG specimens satisfied the diagnostic criteria for IgG4-RD, whereas 19 (57.6%) LSG specimens satisfied the diagnostic criteria for IgG4-RD. Histological evaluation showed fibrosis in all the SMG specimens and in eight LSG specimens (24.2%). Obliterative phlebitis was seen in nine SMG specimens (27.3%), but it was absent in all the LSG specimens. PMID:27007955

  3. IgG4-Related Lung Disease Associated with Usual Interstitial Pneumonia.

    PubMed

    Schneider, Frank; Veraldi, Kristen L; Levesque, Marc C; Colby, Thomas V; S Yi, Eunhee

    2016-01-01

    We report a case of immunoglobulin(Ig)G4-related disease with the radiologic and histopathological manifestations resembling usual interstitial pneumonia (UIP). The patient was a 62-year-old man who presented with progressive dyspnea of insidious onset. High resolution computed tomography of the chest showed lower-lobe predominant peripheral reticulation and traction bronchiectasis but no honeycomb change. Microscopic examination of the surgical lung biopsy showed characteristic features of UIP including architectural distortion by fibrosis with peripheral and paraseptal accentuation, scattered fibroblast foci and microscopic honeycomb change. In addition there were prominent multifocal lymphoplasmacytic infiltrates with a marked increase of IgG4-positive plasma cells (79 per high power field in hot spots) and high IgG4/IgG ratio (up to 67%). The serum IgG4 level was elevated at 760 mg/dl (reference range 9-89), with normal levels for the other IgG subclasses and negative serologic markers for autoimmune diseases. The patient's symptoms improved significantly with oral corticosteroid treatment. PMID:27053971

  4. IgG4- related disease: an orphan disease with many faces

    PubMed Central

    2014-01-01

    Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder (ORPHA284264). Although patients have been described more than 100 years ago, the systemic nature of this disease has been recognized in the 21st century only. Type 1 autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD. Typically, lymphoplasmacellular inflammation, storiform fibrosis and obliterative phlebitis are found in IgG4-RD biopsies and the tissue invading plasma cells largely produce IgG4. Elevated serum IgG4 levels are found in many but not all patients. Consequently, diagnostic criteria for IgG4-RD have been proposed recently. Treatment is largely based on clinical experience and retrospective case series. Glucocorticoids are the mainstay of therapy, although adjunctive immunosuppressive agents are used in relapsing patients. This review summarizes current knowledge on clinical manifestations, pathophysiology and treatment of IgG4-RD. PMID:25026959

  5. IgG4-Related Lung Disease Associated with Usual Interstitial Pneumonia

    PubMed Central

    Schneider, Frank; Veraldi, Kristen L.; Levesque, Marc C.; Colby, Thomas V.; S. Yi, Eunhee

    2016-01-01

    We report a case of immunoglobulin(Ig)G4-related disease with the radiologic and histopathological manifestations resembling usual interstitial pneumonia (UIP). The patient was a 62-year-old man who presented with progressive dyspnea of insidious onset. High resolution computed tomography of the chest showed lower-lobe predominant peripheral reticulation and traction bronchiectasis but no honeycomb change. Microscopic examination of the surgical lung biopsy showed characteristic features of UIP including architectural distortion by fibrosis with peripheral and paraseptal accentuation, scattered fibroblast foci and microscopic honeycomb change. In addition there were prominent multifocal lymphoplasmacytic infiltrates with a marked increase of IgG4-positive plasma cells (79 per high power field in hot spots) and high IgG4/IgG ratio (up to 67%). The serum IgG4 level was elevated at 760 mg/dl (reference range 9-89), with normal levels for the other IgG subclasses and negative serologic markers for autoimmune diseases. The patient’s symptoms improved significantly with oral corticosteroid treatment. PMID:27053971

  6. The Treatment of IgG4-Related Diseases in the Hepatobiliary-Pancreatic System.

    PubMed

    Kamisawa, Terumi; Kuruma, Sawako; Chiba, Kazuro

    2016-08-01

    An accurate diagnosis should be made before treatment of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis. Once a diagnosis has been established, steroids are the standard therapy and symptoms are the major indications. Before steroid therapy, obstructive jaundice and hyperglycemia should be controlled. An initial dose of 0.6 mg/kg/d of oral prednisolone is administered for 2 to 4 weeks, and is gradually tapered over 2 to 3 months. After steroid therapy has begun, blood and imaging tests are performed periodically. Patients with a poor response to steroids should be reevaluated on suspicion of malignancy. To prevent relapse, maintenance therapy using low-dose prednisolone (2.5-5 mg/d) for 1 to 3 years is recommended in Japan. Proximal biliary stricture is reported to be a predictor of relapse. Readministration and dose-up of steroids are effective for relapses. In Western countries, immunosuppressive drugs and rituximab have also been shown to be effective. The optimal treatment regimen should be addressed in future randomized, controlled clinical trials. PMID:27466798

  7. 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

  8. Immunohistochemical study of calretinin in normal skin and cutaneous adnexal proliferations.

    PubMed

    González-Guerra, Elena; Kutzner, Heinz; Rutten, Arno; Requena, Luis

    2012-07-01

    Calretinin is a calcium-binding protein member of the EF-hand family. The presence of calretinin has been demonstrated in certain stages of the cellular cycle in a wide variety of normal and neoplastic tissues. The main aims of our study were (1) to investigate what structures of the normal skin and cutaneous adnexal proliferations express immunoreactivity for calretinin and (2) to determine the value of immunohistochemical expression for calretinin as a marker for follicular, sebaceous, apocrine, and eccrine differentiation in cutaneous adnexal proliferations. We studied 139 biopsy specimens, including 10 cases of normal skin of different locations and 129 benign and malignant cutaneous adnexal proliferations. In normal skin, we found that calretinin is expressed in the innermost cell layer of the outer root sheath in anagen hair follicle, in both the duct and sebolemma of the sebaceous gland, in the secretory portion of eccrine glands, and in mast cells of the stroma. In cutaneous adnexal proliferations, we found strong immunoreactivity for calretinin in tricholemmal cysts, tricholemmomas/inverted follicular keratoses, tumors of follicular infundibulum, and in some basal cell carcinomas. Focal positivity was also seen in trichoadenomas, trichoblastomas/trichoepitheliomas, pilomatricomas, proliferating tricholemmal tumors, pilar sheath acanthomas, trichofolliculomas, follicular hybrid cysts, cutaneous mixed tumors, steatocystomas, sebaceous hyperplasias, and sebaceomas. These results demonstrate that immunohistochemical study for calretinin may be helpful to identify the innermost cell layer of the outer root sheath in anagen hair follicle and the cutaneous adnexal proliferations showing differentiation toward this structure. Calretinin immunoreactivity supports eccrine differentiation in some sweat gland neoplasms, and it is also useful in identifying neoplasms with ductal sebaceous differentiation. PMID:22343111

  9. Pediatric ocular phthiriasis infestation.

    PubMed

    Kairys, D J; Webster, H J; Terry, J E

    1988-02-01

    Although pubic lice infestation of ocular regions is relatively uncommon, the optometrist needs to be aware of the diagnosis and treatment of louse-infested patients. A case report of ocular phthiriasis is presented along with a discussion of its etiology, clinical diagnosis and management. PMID:3361053

  10. Ultrasound Image Discrimination between Benign and Malignant Adnexal Masses Based on a Neural Network Approach.

    PubMed

    Aramendía-Vidaurreta, Verónica; Cabeza, Rafael; Villanueva, Arantxa; Navallas, Javier; Alcázar, Juan Luis

    2016-03-01

    The discrimination between benign and malignant adnexal masses in ultrasound images represents one of the most challenging problems in gynecologic practice. In the study described here, a new method for automatic discrimination of adnexal masses based on a neural networks approach was tested. The proposed method first calculates seven different types of characteristics (local binary pattern, fractal dimension, entropy, invariant moments, gray level co-occurrence matrix, law texture energy and Gabor wavelet) from ultrasound images of the ovary, from which several features are extracted and collected together with the clinical patient age. The proposed technique was validated using 106 benign and 39 malignant images obtained from 145 patients, corresponding to its probability of appearance in general population. On evaluation of the classifier, an accuracy of 98.78%, sensitivity of 98.50%, specificity of 98.90% and area under the curve of 0.997 were calculated. PMID:26715189

  11. Ocular manifestation of Ichthyosis

    PubMed Central

    Al-Amry, Mohammad A.

    2015-01-01

    Purpose Ichthyosis is a rare dermato-ocular disease. This study evaluates the presenting ocular signs, symptoms, complications and prognosis of ichthyosis in a case series from Saudi Arabia. Methods A retrospective chart review was performed for 11 patients with ichthyosis who presented to King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia, over the last 20 years. Results The most common presenting ocular diagnosis was ectropion of both the lids. Two patients developed corneal perforation with poor prognosis. Most of the patients underwent skin grafting to repair eyelid ectropion. The visual prognosis was excellent because timely surgical interventions were performed. Hence the rate of corneal complications such as perforation was low. Conclusion The most ocular presentation of ichthyosis is ectropion of both the upper and lower lids. Despite good visual prognosis, there were some devastating corneal complications such as perforation with unpredictable outcomes. PMID:26949357

  12. Ultrasound follow up of an adnexal mass has the potential to save lives.

    PubMed

    Ormsby, Eleanor L; Pavlik, Edward J; van Nagell, John R

    2015-11-01

    Ovarian cancer is among the most dreaded cancers since it is often found at a late stage where the opportunity for extended survival is poor. Ultrasound has been utilized in several ovarian cancer screening trials in asymptomatic women in order to detect ovarian cancer at early stages where survival rates are high. Efforts to improve screening for ovarian cancer are ongoing. While ovarian cancer screening in asymptomatic women is not recommended for clinical application currently, the care of women with adnexal masses found by ultrasound in clinical practice can benefit from close evaluation of the evidence obtained from large prospective ovarian cancer screening trials and by relating this evidence to recent advances in the understanding of different types of ovarian cancer. Post-menopausal women who have an adnexal mass discovered by ultrasound have a much higher risk of developing ovarian cancer than women with normal ultrasound. The preponderance of reported evidence indicates that ultrasound monitoring of an adnexal mass is safe, cost effective and can achieve an improved positive predictive value in detecting ovarian cancer when ovarian abnormalities resolve during surveillance. Proposals to arbitrarily discontinue ultrasound monitoring can negatively impact patient care and generate medical-legal actions, especially because there is no evidence to support safe discontinuation. In this review, we outline a rationale for continuing ultrasound surveillance of ovarian abnormalities. PMID:26116103

  13. Laparoscopic surgery for treating adnexal masses during the first trimester of pregnancy

    PubMed Central

    Minig, Lucas; Otaño, Lucas; Cruz, Pilar; Patrono, María Guadalupe; Botazzi, Cecilia; Zapardiel, Ignacio

    2016-01-01

    OBJECTIVE: To evaluate the feasibility and safety of laparoscopic surgery for treating adnexal masses during the first trimester of pregnancy. STUDY DESIGN: An observational study of a prospective collection of data of all pregnant women who underwent laparoscopic surgery for adnexal masses during the first trimester of pregnancy between January 1999 and November 2012 at the Obstetrics and Gynecology Department of the Italian Hospital of Buenos Aires, Buenos Aires, Argentina was performed. RESULTS: A total of 13 women were included. The median (range) gestational age at the moment of surgical procedure was 7 weeks (range: 5-12 weeks). The main indication of surgery was cyst torsion in four cases (30.7%) and rupture of ovarian cysts in four cases (30.7%). Other indications included persistent ovarian cyst in three patients (23%) and heterotopic pregnancy in two cases (15.3%). Neither surgical complications nor spontaneous abortions occurred in any of the cases and the post-operative period was uneventful in all the cases. No cases of intrauterine growth retardation, preterm delivery, congenital defects, or neonatal complications were registered. CONCLUSION: The treatment of complicated adnexal masses by laparoscopic surgery during the first trimester of pregnancy appears to be a safe procedure both for the mother and for the foetus. Additional research on a larger number of cases is still needed to support these conclusions. PMID:26917915

  14. Usefulness of T2*-weighted MRI in the detection of adnexal torsion

    PubMed Central

    Kawai, Nobuyuki; Kanematsu, Masayuki; Kawaguchi, Shimpei; Kojima, Toshihisa; Furui, Tatsuro; Morishige, Ken-ichirou; Matsuo, Masayuki

    2016-01-01

    Background The usefulness of T2*-weighted (T2*W) imaging for the detection of adnexal torsion has yet to be determined. Purpose To assess the usefulness of T2*W imaging for detecting and differentiating adnexal torsion. Material and Methods Eight patients with eight ovaries with torsion and 44 patients with 72 ovaries without torsion were included in this study. All patients underwent 1.5-T magnetic resonance imaging (MRI) including T2*W images. The frequency and distribution of hypointensity on T2*W images were compared between ovaries with torsion and ovaries without torsion. Results Hypointensity on T2*W images was significantly more frequent in ovaries with torsion than in ovaries without torsion (75% vs. 36%; P < 0.05). Among patients with hypointensity on T2*W images, the frequency of diffuse hypointensity was significantly higher in ovaries with torsion than in ovaries without torsion (83% vs. 0%; P < 0.01); whereas the frequency of focal hypointensity was significantly lower in ovaries with torsion than in ovaries without torsion (17% vs. 100%; P < 0.01). Conclusion The presence and distribution of hypointensity on T2*W images may play a supplementary role in the detection of adnexal torsion. PMID:27478621

  15. Epidemiology of Patients Hospitalized for Ocular Trauma in the Chaoshan Region of China, 2001–2010

    PubMed Central

    Cao, He; Li, Liping; Zhang, Mingzhi

    2012-01-01

    Background The burden and pattern of ocular trauma in China are poorly known and not well studied. We aimed at studying the epidemiological characteristics of patients hospitalized for ocular trauma at major ophthalmology departments in the largest industrial base of plastic toys in China. Methods A retrospective study of ocular trauma cases admitted to 3 tertiary hospitals in China from 1st January 2001 to 31st December 2010 was performed. Results The study included a total of 3,644 injured eyes from 3,559 patients over the 10-year period: 2,008 (55.1%) open-globe injuries, 1,580 (43.4%) closed-globe injuries, 41 (1.1%) chemical injuries, 15 (0.4%) thermal injuries and 678 (18.6%) ocular adnexal injuries. The mean age of the patients was 29.0±16.8 years with a male-to-female ratio of 5.2∶1 (P = 0.007). The most frequent types of injury were work-related injuries (1,656, 46.5%) and home-related injuries (715, 20.1%). The majority of injuries in males (56.2%) and females (36.0%) occurred in the 15–44 age group and 0–14 age group, respectively. The final visual acuity correlated with the initial visual acuity (Spearman’s correlation coefficient = 0.659; P<0.001). The Ocular Trauma Score also correlated with the final visual acuity (Spearman’s correlation coefficient = 0.655; P<0.001). Conclusions This analysis provides an epidemiological study of patients who were hospitalized for ocular trauma. Preventive efforts are important for both work-related and home-related eye injuries. PMID:23118997

  16. Pediatric genetic ocular tumors

    PubMed Central

    Rouhani, Behnaz; Ramasubramanian, Aparna

    2014-01-01

    Pediatric genetic ocular tumors include malignancies like retinoblastoma and phakomatosis like neurofibromatosis, tuberous sclerosis, von Hippel-Lindau syndrome, and nevoid basal cell carcinoma syndrome. It is important to screen for ocular tumors both for visual prognosis and also for systemic implications. The phakomatosis comprise of multitude of benign tumors that are aysmptomatic but their detection can aid in the diagnosis of the syndrome. Retinoblastoma is the most common malignant intraocular tumor in childhood and with current treatment modalities, the survival is more than 95%. It is transmitted as an autosomal dominant fashion and hence the offsprings of all patients with the germline retinoblastoma need to be screened from birth. This review discusses the various pediatric genetic ocular tumors discussing the clinical manifestation, diagnosis and treatment.

  17. Glycobiology of ocular angiogenesis.

    PubMed

    Markowska, Anna I; Cao, Zhiyi; Panjwani, Noorjahan

    2014-12-01

    Ocular neovascularization can affect almost all the tissues of the eye: the cornea, the iris, the retina, and the choroid. Pathological neovascularization is the underlying cause of vision loss in common ocular conditions such as diabetic retinopathy, retinopathy of prematurity and age-related macular neovascularization. Glycosylation is the most common covalent posttranslational modification of proteins in mammalian cells. A growing body of evidence demonstrates that glycosylation influences the process of angiogenesis and impacts activation, proliferation, and migration of endothelial cells as well as the interaction of angiogenic endothelial cells with other cell types necessary to form blood vessels. Recent studies have provided evidence that members of the galectin class of β-galactoside-binding proteins modulate angiogenesis by novel carbohydrate-based recognition systems involving interactions between glycans of angiogenic cell surface receptors and galectins. This review discusses the significance of glycosylation and the role of galectins in the pathogenesis of ocular neovascularization. PMID:25108228

  18. Ocular toxicity of fludarabine

    PubMed Central

    Ding, Xiaoyan; Herzlich, Alexandra A; Bishop, Rachel; Tuo, Jingsheng; Chan, Chi-Chao

    2008-01-01

    The purine analogs, fludarabine and cladribine represent an important class of chemotherapy agents used to treat a broad spectrum of lymphoid malignancies. Their toxicity profiles include dose-limiting myelosuppression, immunosuppression, opportunistic infection and severe neurotoxicity. This review summarizes the neurotoxicity of high- and standard-dose fludarabine, focusing on the clinical and pathological manifestations in the eye. The mechanisms of ocular toxicity are probably multifactorial. With increasing clinical use, an awareness of the neurological and ocular vulnerability, particularly to fludarabine, is important owing to the potential for life- and sight-threatening consequences. PMID:18461151

  19. Potential harms outweigh benefits of indefinite monitoring of stable adnexal masses.

    PubMed

    Suh-Burgmann, Elizabeth; Kinney, Walter

    2015-12-01

    The management of women with asymptomatic adnexal masses should aim to balance potential benefit with potential harm. While masses with highly worrisome features or other signs of malignancy should be referred for surgery, the vast majority of masses have an indeterminate or benign appearance and are candidates for observation. Evidence supports the use of initial short-term serial ultrasound in distinguishing between benign and malignant masses. However, benefit from prolonged, potentially life-long monitoring of stable masses has not been demonstrated. Since the goal of monitoring an adnexal mass is to observe for worrisome growth or increasing complexity as an indicator of malignancy, if the mass remains stable, the likelihood of malignancy and therefore, the potential benefit of observation wanes with time. The recognition that Type 2 high grade serous cancers, which are responsible for the majority of deaths from ovarian cancer, arise from fallopian tube rather than ovarian precursors, further diminishes the likelihood that monitoring a stable ovarian mass will lead to early diagnosis of high grade disease. While some Type 1 cancers may develop from ovarian precursors, the available data suggest that any measurable benefit of monitoring known lesions for detection of these cancers is realized within the first year of observation. The argument in favor of indefinite, potentially life-long monitoring of stable masses also fails to adequately account for the risks of perpetual imaging, which include the risk of incidental findings, an increased likelihood of unnecessary surgery, patient anxiety and cost. It is not always better to order a test than not order a test. Given the absence of evidence of benefit, observation of stable small adnexal masses should be limited in duration in order to minimize potential harms. PMID:26363476

  20. Comparison of Adnexal Mass in Women Undergoing Mass Excision During the Antepartum Period and Cesarean Section

    PubMed Central

    Saghafi, Nafiseh; Roodsary, Zohreh Yousefi; Kadkhodaeian, Sima; Mofrad, Maliheh Hasanzadeh; Farahabadi, Elham Hosseini; Hoseinyfarahabady, Mohammadreza

    2016-01-01

    Objectives The frequency of adnexal masses in pregnant women ranges from 0.1% to 4%. Selecting the right approach to manage the subsequent intervention remains one of the most controversial challenges among gynecologists. Our aim in this cross-sectional study was to clarify the clinical-pathological differences among the adnexal masses that are excised during either the antepartum period or cesarean section (CS). Methods In this study, we assessed 11,000 pregnancy cases referred to the Qaem Hospital in the Mashhad University of Medical Sciences, Iran, between 2010 and 2014. In total, 53 pregnant women with adnexal masses (other than non-gynecological mass and ectopic pregnancy) were selected for further investigation. We divided patients into two groups (group A and group B). Patients of group A had a diagnosed tumor that was excised antepartum while patients in group B had a mass taken out during CS. We then assembled data based on maternal age, parity, gestational age, surgery type, delivery mode, size and location of the tumor, complications, presentations, histopathological diagnosis, and ultrasonography findings for further analysis. Results The major proportion of masses (62.3%) were excised during CS whereas the remainder (37.7%) were removed antepartum. The mean size of the detected tumor for benign and malignant cases was 10.0 cm and 13.8 cm in group A, and 8.0 cm and 9.3 cm in group B, respectively. There was a statistically significant difference observed between patients in the two groups regarding the benign/malignant status of the mass (p = 0.008), its size (p = 0.019) and simplicity/complexity (p = 0.004). Conclusions The rate of malignant tumors was considerably higher in women who had antepartum mass excision compared to those with mass resection during CS. Also, tumors were larger (and more complex) in patients in group A compared to group B. PMID:27162593

  1. Cavernous hemangioma presenting as a right adnexal mass in a child.

    PubMed

    Correa-Rivas, María S; Colón-González, Gloria; Lugo-Vicente, Humberto

    2003-09-01

    This is the case of an 11-year-old girl who presented with a right adnexal mass and vague abdominal symptoms since seven months prior to her hospital admission for surgery. CT-scan and sonographic images were those of a benign lesion, probably ovarian torsion or infarction. Serum tumoral markers were normal. A right salpingo-oophorectomy and appendectomy were performed. Pathology examination revealed a cavernous hemangioma of the ovary. The clinicopathologic presentation of this unusual benign ovarian tumor is discussed. PMID:14619460

  2. Preoperative Evaluation of Risk of Ovarian Malignancy Algorithm Index in Prediction of Malignancy of Adnexal Masses

    PubMed Central

    Farzaneh, Farah; Honarvar, Zahra; Yaraghi, Mansoore; Yaseri, Mehdi; Arab, Maliheh; Hosseini, Maryamsadat; Ashrafgangoi, Tahereh

    2014-01-01

    Background: Differentiation between benign and malignant ovarian neoplasms is essential to create a system for patient referrals. Objectives: The aim of the present prospective trial was to analyze the value of the risk of ovarian malignancy algorithm (ROMA) in prediction of adnexal masses malignancy in pre- and post-menopause women before operation. Materials and Methods: Preoperative serum samples were tested for CA125 and HE4 using fully automated methods (Abbott architect) and gained best cutoff. The ROMA index was analyzed in 99 patients (including 68 pre-menopause and 31 menopause) with adnexal masses referred to Imam Hossein Hospital/Tehran/Iran and had been scheduled for operation. The pathological results showed 43 cases (22 menopause) with malignant adnexal masses and 56 cases (9 menopauses) with benign adnexal masses. Demographical data, clinical symptoms and the ROMA index were separately analyzed and contrasted in benign and malignant in both menopause and pre-menopause patients. Results: The only significant difference was the older age of the malignant group vs. benign group (P = 0.001) regarding demographic findings. As concerns the clinical symptoms, presence of abdominal discomfort in pre-diagnosis period was the only significant parameter in malignant group (P = 0.001). Additionally, data analysis of patients as a total group showed that specificity (96.4%), positive predictive value (PPV) (94.1%), area under the curve (AUC) (0.907), and diagnostic accuracy (DA) (86.9%) of the ROMA were higher than HE4 (91.1%, 85.7%, 0.857 and 81.8%. respectively) and CA125 (87.9%, 67.3%, 0.828 and 75.8%, respectively) alone. Besides, negative predictive value (NPV) (86.4%) and sensitivity (86.1%) of CA125 were higher than HE4 (79.7% and 69.8%, respectively). In contrast, specificity of HE4 (91.1%) was higher than CA125 (67.9%). Data analysis of patients as two groups (pre and post menopause groups) showed the same results. Conclusions: Specificity, DA and AUC of

  3. Cystic acantholytic dyskeratosis of the vulva: An unusual presentation of a follicular adnexal neoplasm

    PubMed Central

    Torres, Kara Melissa Tiangco; Junkins-Hopkins, Jacqueline M.

    2016-01-01

    Acantholytic dyskeratosis (AD) is a histologic pattern seen in Darier's disease or dyskeratosis follicularis, warty dyskeratoma, and transient AD. This pattern is characterized by suprabasilar clefting, acantholysis, and formation of corps ronds and grains. We present a case of AD that is unique based on its genital location and cystic architecture. A 53-year-old woman presented with an otherwise asymptomatic cyst on her left vulva of uncertain duration. On microscopic examination, there were fragments of cystic epithelium with areas of hypergranulosis, acantholysis, corps ronds, and corps grains formation. These features are felt by the authors to be a unique presentation of a follicular adnexal neoplasm. PMID:27559500

  4. [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. PMID:27187879

  5. 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.

  6. Homocysteine in ocular diseases.

    PubMed

    Ajith, Thekkuttuparambil Ananthanarayanan; Ranimenon

    2015-10-23

    Homocysteine (Hcy) is a derived sulfur-containing and non-proteinogenic amino acid. The metabolism of Hcy occurs either through the remethylation to methionine or transsulfuration to cysteine. Studies have identified hyperhomocysteinemia (HHcy) as one of the possible risk factors for a multitude of diseases including vascular, neurodegenerative and ocular diseases. Association of HHcy with eye diseases such as retinopathy, pseudoexfoliative glaucoma maculopathy, cataract, optic atrophy and retinal vessel atherosclerosis is established. The molecular mechanism underlying these ocular diseases has been reported as impaired vascular endothelial function, apoptosis of retinal ganglion cells, extracellular matrix alterations, decreased lysyl oxidase activity and oxidative stress. The formed homocysteine-thiolactone in HHcy has stronger cytotoxicity and pro-inflammatory properties which can induce lens opacification and optic nerve damage. The metabolism of Hcy requires enzymes with vitamins such as folic acid, vitamins B12 and B6. Despite the mixed conclusion of various studies regarding the level of these vitamins in elder people, studies recommended the treatment with folate and B12 to reduce Hcy levels in subjects with or without any defect in the enzymes involved in its metabolism. The levels of Hcy, folate, B6 as well as B12 should be measured early in patients with visual impairment that would aid to screen patients for life-threatening disorders related with HHcy. Elder patients may supplement with these vitamins in order to attenuate the ocular damages. This article discusses the association of Hcy in ocular diseases and the possible mechanism in the pathogenesis. PMID:26343924

  7. 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.

  8. The Value of Ultrasound Monitoring of Adnexal Masses for Early Detection of Ovarian Cancer.

    PubMed

    Suh-Burgmann, Elizabeth; Kinney, Walter

    2016-01-01

    Although ultrasound has so far been found to be ineffective as a screening tool for ovarian cancer, it is commonly used as a means of evaluating or following ovarian or adnexal masses once they are detected. We review the use of serial ultrasound for the management of adnexal masses and propose an approach to monitoring based on an understanding of the overall risk of cancer among the population in question and an assessment of how the potential benefit of monitoring compares with potential risk. In our approach, masses that are symptomatic, large (>10 cm), associated with an elevated CA 125 level or overt signs of malignancy, or that are determined to have a worrisome appearance by stringent ultrasound criteria should be evaluated surgically. Women with masses that have none of these characteristics should be offered monitoring. Short-term initial ultrasound monitoring carries significant potential benefit in terms of aiding detection of early malignancy and avoidance of unnecessary surgery. However, if a mass remains stable but persistent, the potential benefit of ongoing monitoring wanes with time, whereas the potential harms, in terms of patient anxiety, cost, and the risk of incidental findings and unnecessary surgery increase. Therefore, monitoring of stable lesions should be limited in duration in order to limit potential harms from overtreatment and overdiagnosis. PMID:26904503

  9. The Value of Ultrasound Monitoring of Adnexal Masses for Early Detection of Ovarian Cancer

    PubMed Central

    Suh-Burgmann, Elizabeth; Kinney, Walter

    2016-01-01

    Although ultrasound has so far been found to be ineffective as a screening tool for ovarian cancer, it is commonly used as a means of evaluating or following ovarian or adnexal masses once they are detected. We review the use of serial ultrasound for the management of adnexal masses and propose an approach to monitoring based on an understanding of the overall risk of cancer among the population in question and an assessment of how the potential benefit of monitoring compares with potential risk. In our approach, masses that are symptomatic, large (>10 cm), associated with an elevated CA 125 level or overt signs of malignancy, or that are determined to have a worrisome appearance by stringent ultrasound criteria should be evaluated surgically. Women with masses that have none of these characteristics should be offered monitoring. Short-term initial ultrasound monitoring carries significant potential benefit in terms of aiding detection of early malignancy and avoidance of unnecessary surgery. However, if a mass remains stable but persistent, the potential benefit of ongoing monitoring wanes with time, whereas the potential harms, in terms of patient anxiety, cost, and the risk of incidental findings and unnecessary surgery increase. Therefore, monitoring of stable lesions should be limited in duration in order to limit potential harms from overtreatment and overdiagnosis. PMID:26904503

  10. Correlation between ocular perfusion pressure and ocular pulse amplitude in glaucoma, ocular hypertension, and normal eyes

    PubMed Central

    Figueiredo, Bruno P; Cronemberger, Sebastião; Kanadani, Fabio N

    2013-01-01

    Background The purpose of this study was to investigate the correlation between ocular perfusion pressure and ocular pulse amplitude in glaucoma, ocular hypertension, and normal eyes. Methods Ninety eyes from 90 patients were included. Thirty patients had been recently diagnosed with glaucoma and had no previous history of treatment for ocular hypotension, 30 had elevated intraocular pressure (IOP) without evidence of glaucoma, and 30 had normal IOP (<21 mmHg) with no detectable glaucomatous damage. Goldmann applanation tonometry (GAT), dynamic contour tonometry (DCT), blood pressure measurement, pachymetry, Humphrey visual field, and routine ophthalmic examination was performed in each patient. Ocular perfusion pressure was calculated as the difference between mean arterial pressure and IOP. The ocular pulse amplitude was given by DCT. The Pearson correlation coefficient was used to compare the glaucomatous and ocular hypertensive groups, and comparisons with the normal IOP group were done using the Spearman’s rank correlation coefficient. Results Mean IOP by DCT was 22.7 ± 4.3 mmHg in the glaucoma group, 22.3 ± 2.8 mmHg in the ocular hypertension group, and 14.3 ± 1.6 mmHg in the control group. Mean IOP by GAT was 19.0 ± 5.1 mmHg for glaucoma, 22.4 ± 2.1 mmHg for ocular hypertension, and 12.9 ± 2.2 mmHg for controls. Mean ocular pulse amplitude was 3.4 ± 1.2 mmHg in the glaucoma group, 3.5 ± 1.2 mmHg in the ocular hypertension group, and 2.6 ± 0.9 mmHg in the control group. Mean ocular perfusion pressure was 46.3 ± 7.9 mmHg in the glaucoma group, 46.3 ± 7.9 mmHg in the ocular hypertension group, and 50.2 ± 7.0 mmHg in controls. No significant correlation between ocular perfusion pressure and ocular pulse amplitude was found in any of the groups (P = 0.865 and r = −0.032, P = 0.403 and r = −0.156, P = 0.082 and ρ = −0.307 for glaucoma, ocular hypertension, and normal eyes, respectively). Conclusion There is no significant correlation between

  11. 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

  12. 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. PMID:25704934

  13. 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

  14. An ocular motility conundrum.

    PubMed

    McElnea, Elizabeth Margaret; Stephenson, Kirk; Lanigan, Bernie; Flitcroft, Ian

    2014-01-01

    Two siblings, an 11-year-old boy and a 7-year-old girl presented with bilateral symmetrical ptosis and limited eye movements. Having already been reviewed on a number of occasions by a variety of specialists in multiple hospital settings a diagnosis of their ocular motility disorder had remained elusive. We describe their cases, outline the differential diagnosis and review the investigations performed which were influential in finally making a diagnosis. PMID:25349186

  15. Ocular Proton Therapy Centers

    NASA Astrophysics Data System (ADS)

    Kacperek, Andrzej

    This chapter describes a review of proton therapy (PT) centers and the techniques used for the treatment of ocular lesions. The role of ion beam therapy (IBT) for eye treatments, principally choroidal melanomas, has become well established among the competing treatment modalities. More national centers now offer PT for these lesions, but not necessarily in a hospital environment. Significant improvements in eye treatment planning, patient positioning, and QA dosimetry have been realized, to the benefit of treatment efficiency and accuracy of dose delivery.

  16. Rare Skin Adnexal and Melanocytic Tumors Arising in Ovarian Mature Cystic Teratomas: A Report of 3 Cases and Review of the Literature.

    PubMed

    Moulla, Alexandra A; Magdy, Nesreen; Francis, Nicholas; Taube, Janis; Ronnett, Brigitte M; El-Bahrawy, Mona

    2016-09-01

    Mature teratoma of the ovary is the most common primary ovarian tumor accounting for 15% (10%-20%) of all ovarian neoplasms. Skin and skin adnexal structures are the most common elements identified in mature teratomas. Benign and malignant skin tumors can arise in ovarian teratomas, the most common being epithelial tumors. Melanocytic and adnexal tumors developing in a teratoma are rare and can be easily overlooked. We report 3 cases and review melanocytic and skin adnexal tumors encountered in ovarian teratomas. PMID:26974995

  17. Adnexal Torsion

    MedlinePlus

    ... abdomen (abdominal cavity) and the tissues lining it. Did You Know... The ovary sometimes twists, causing sudden, ... is removed (called oophorectomy). Resources In This Article Did You Know 1 Did You Know... Noncancerous Gynecologic ...

  18. Pelvic Castleman's disease presenting as an adnexal tumor in a young woman.

    PubMed

    Lee, Jisun; Paek, Jiheum; Lee, Yong Hee; Kong, Tae Wook; Chang, Suk-Joon; Ryu, Hee-Sug

    2015-07-01

    Castleman's disease (CD) is a rare benign disorder of unknown etiology characterized by proliferation of lymphoid tissues. Seventy percent of this tumor occurs in the mediastinum and it is seldom found in neck, pancreas or pelvis. We report a case of asymptomatic pelvic CD initially presenting as an adnexal tumor in a 27-year-old woman. Initial transvaginal sonography revealed 7-cm-sized hyperechoic mass adjacent to the right ovary and the following abdominal computed tomography scanning showed the same sized mass located on the right extraperitoneal pelvic cavity. Laparoscopic mass excision was performed without any complication and pathological diagnosis was made as CD. CD should be included in the differential diagnosis of female pelvic masses which are noted in the pelvic cavity. In this report, we review the clinicopathological findings in a presentation of CD. PMID:26217605

  19. Adnexal Incarceration in a Posterior Pelvic Peritoneal Defect Mimics Ovarian Torsion.

    PubMed

    Jackson, Amanda M; Hope, Erica R; Phippen, Neil T

    2015-01-01

    Surgery for suspected ovarian torsion sometimes reveals unexpected sources of pelvic pain, such as internal hernias, adhesions, or anatomic defects. A 23-year-old nulligravida with Alagille syndrome was taken to the operating room with suspected ovarian torsion. Intraoperatively, the right adnexa bulged out of a right-sided, posterior peritoneal cleft that incarcerated most of the enlarged ovary. No ovarian torsion was identified. The left adnexa appeared to be normal; however, it dwelled within a left-sided posterior peritoneal cleft. The bilateral posterior peritoneal defects that housed the adnexa were likely of congenital etiology. Although adnexal incarceration is a rare finding at surgery for suspected ovarian torsion, it should be part of the differential diagnosis when evaluating acute pelvic pain. PMID:26044591

  20. Cutaneous adnexal neoplasms in biopsy specimens processed in the Department of Pathology, University of Malaya.

    PubMed

    Jayalakshmi, P; Looi, L M

    1996-07-01

    A review of consecutive biopsies of adnexal tumours from 112 patients, received by the Department of Pathology, University of Malaya, over a 13-year period was undertaken. The age range of the patients was from 1 to 84 years, with a mean of 29.8 years. Thirty-three (32%) patients were under 20 years of age. There were 68 females with a male to female ratio of 1.0:1.5. In 105 cases (93.7%), the neoplasm was solitary. The tumour measured less than 2 cm in the largest dimension in 103 cases (92%). The common sites of occurrence were the head and neck region (59%) and extremities (25%). Neoplasms of hair follicle origin accounted for 63.4% (71 cases) of all lesions. Intra-tumour deposition of amyloid was noted in one of the 14 cases of trichoepithelioma. PMID:8893922

  1. Ocular drug delivery.

    PubMed

    Gaudana, Ripal; Ananthula, Hari Krishna; Parenky, Ashwin; Mitra, Ashim K

    2010-09-01

    Ocular drug delivery has been a major challenge to pharmacologists and drug delivery scientists due to its unique anatomy and physiology. Static barriers (different layers of cornea, sclera, and retina including blood aqueous and blood-retinal barriers), dynamic barriers (choroidal and conjunctival blood flow, lymphatic clearance, and tear dilution), and efflux pumps in conjunction pose a significant challenge for delivery of a drug alone or in a dosage form, especially to the posterior segment. Identification of influx transporters on various ocular tissues and designing a transporter-targeted delivery of a parent drug has gathered momentum in recent years. Parallelly, colloidal dosage forms such as nanoparticles, nanomicelles, liposomes, and microemulsions have been widely explored to overcome various static and dynamic barriers. Novel drug delivery strategies such as bioadhesive gels and fibrin sealant-based approaches were developed to sustain drug levels at the target site. Designing noninvasive sustained drug delivery systems and exploring the feasibility of topical application to deliver drugs to the posterior segment may drastically improve drug delivery in the years to come. Current developments in the field of ophthalmic drug delivery promise a significant improvement in overcoming the challenges posed by various anterior and posterior segment diseases. PMID:20437123

  2. Infranuclear ocular motor disorders.

    PubMed

    Lueck, Christian J

    2011-01-01

    This chapter covers the very large number of possible disorders that can affect the three ocular motor nerves, the neuromuscular junction, or the extraocular muscles. Conditions affecting the nerves are discussed under two major headings: those in which the site of damage can be anatomically localized (e.g., fascicular lesions and lesions occurring in the subarachnoid space, the cavernous sinus, the superior orbital fissure, or the orbit) and those in which the site of the lesion is either nonspecific or variable (e.g., vascular lesions, tumors, "ophthalmoplegic migraine," and congenital disorders). Specific comments on the diagnosis and management of disorders of each of the three nerves follow. Ocular motor synkineses (including Duane's retraction syndrome and aberrant regeneration) and disorders resulting in paroxysms of excess activity (e.g., neuromyotonia) are then covered, followed by myasthenia gravis and other disorders that affect the neuromuscular junction. A final section discusses disorders of the extraocular muscles themselves, including thyroid disease, orbital myositis, mitochondrial disease, and the muscular dystrophies. PMID:21601071

  3. 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

  4. Ocular tuberculosis: current perspectives

    PubMed Central

    Shakarchi, Faiz I

    2015-01-01

    The World Health Organization currently estimates that nearly two billion people, or one-third of the world’s population, are infected by tuberculosis, and that roughly 10% of the infected people are symptomatic. Tuberculosis affects the lungs in 80% of patients, while in the remaining 20% the disease may affect other organs, including the eye. Uveitis can be seen concurrently with tuberculosis, but a direct association is difficult to prove. Ocular tuberculosis is usually not associated with clinical evidence of pulmonary tuberculosis, as up to 60% of extrapulmonary tuberculosis patients may not have pulmonary disease. The diagnosis of tuberculous uveitis is often problematic and in nearly all reported cases, the diagnosis was only presumptive. Tuberculous uveitis is a great mimicker of various uveitis entities and it can be considered in the differential diagnosis of any type of intraocular inflammation. It is still unknown if ocular manifestations result from a direct mycobacterium infection or hypersensitivity reaction and this is reflected on the management of tuberculous uveitis. Prevalence of tuberculosis as an etiology of uveitis may reach up to 10% in endemic areas. Tuberculous uveitis is a vision-threatening disease that inevitably leads to blindness if not properly diagnosed and treated. The aim of this review is to illustrate the various clinical features and management of presumed tuberculous uveitis. The current review focuses on the diagnostic criteria, significance of tuberculin skin test, and use of systemic corticosteroids in the management of tuberculous uveitis as recommended in recent publications. PMID:26648690

  5. Single Port Transumbilical Laparoscopic Surgery versus Conventional Laparoscopic Surgery for Benign Adnexal Masses: A Retrospective Study of Feasibility and Safety

    PubMed Central

    Wang, Si-Yun; Yin, Ling; Guan, Xiao-Ming; Xiao, Bing-Bing; Zhang, Yan; Delgado, Amanda

    2016-01-01

    Background: Single port laparoscopic surgery (SPLS) is an innovative approach that is rapidly gaining recognition worldwide. The aim of this study was to determine the feasibility and safety of SPLS compared to conventional laparoscopic surgery for the treatment of benign adnexal masses. Methods: In total, 99 patients who underwent SPLS for benign adnexal masses between December 2013 and March 2015 were compared to a nonrandomized control group comprising 104 conventional laparoscopic adnexal surgeries that were performed during the same period. We retrospectively analyzed multiple clinical characteristics and operative outcomes of all the patients, including age, body mass index, size and pathological type of ovarian mass, operative time, estimated blood loss (EBL), duration of postoperative hospital stay, etc. Results: No significant difference was observed between the two groups regarding preoperative baseline characteristics. However, the pathological results between the two groups were found to be slightly different. The most common pathological type in the SPLS group was mature cystic teratoma, whereas endometrioma was more commonly seen in the control group. Otherwise, the two groups had comparable surgical outcomes, including the median operation time (51 min vs. 52 min, P = 0.909), the median decreased level of hemoglobin from preoperation to postoperation day 3 (10 g/L vs. 10 g/L, P = 0.795), and the median duration of postoperative hospital stay (3 days vs. 3 days, P = 0.168). In SPLS groups, the median EBL and the anal exsufflation time were significantly less than those of the conventional group (5 ml vs. 10 ml, P < 0.001; 10 h vs. 22 h, P < 0.001). Conclusions: SPLS is a feasible and safe approach for the treatment of benign adnexal masses. Further study is required to better determine whether SPLS has significant benefits compared to conventional techniques. PMID:27231167

  6. 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

  7. 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.

  8. 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.

  9. 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

  10. 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

  11. Ocular complications of diabetes mellitus.

    PubMed

    Sayin, Nihat; Kara, Necip; Pekel, Gökhan

    2015-02-15

    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

  12. Ocular neuromyotonia after radiation therapy

    SciTech Connect

    Lessell, S.; Lessell, I.M.; Rizzo, J.F. III

    1986-12-15

    Ocular neuromyotonia is a paroxysmal monocular deviation that results from spasm of eye muscles secondary to spontaneous discharges from third, fourth, or sixth nerve axons. We observed this rare disorder in four patients who had been treated with radiation for tumors in the region of the sella turcica and cavernous sinus. Based on these cases and four others identified in the literature it would appear that radiation predisposes to a cranial neuropathy in which ocular neuromyotonia may be the major manifestation. Radiation appears to be the most common cause of ocular neuromyotonia.

  13. Ocular Blood Flow Autoregulation Mechanisms and Methods

    PubMed Central

    Luo, Xue; Shen, Yu-meng; Jiang, Meng-nan; Lou, Xiang-feng; Shen, Yin

    2015-01-01

    The main function of ocular blood flow is to supply sufficient oxygen and nutrients to the eye. Local blood vessels resistance regulates overall blood distribution to the eye and can vary rapidly over time depending on ocular need. Under normal conditions, the relation between blood flow and perfusion pressure in the eye is autoregulated. Basically, autoregulation is a capacity to maintain a relatively constant level of blood flow in the presence of changes in ocular perfusion pressure and varied metabolic demand. In addition, ocular blood flow dysregulation has been demonstrated as an independent risk factor to many ocular diseases. For instance, ocular perfusion pressure plays key role in the progression of retinopathy such as glaucoma and diabetic retinopathy. In this review, different direct and indirect techniques to measure ocular blood flow and the effect of myogenic and neurogenic mechanisms on ocular blood flow are discussed. Moreover, ocular blood flow regulation in ocular disease will be described. PMID:26576295

  14. Cystic adnexal mass in a 16-year-old female: Ovarian pathology or complication of a Müllerian anomaly?

    PubMed Central

    Santacana-Laffitte, Guido; Ruiz, Lorell; Pedrogo, Yasmin; Colon, Edgar

    2013-01-01

    Patient: Female, 16 Final Diagnosis: Pelvic mass Symptoms: None Medication: None Clinical Procedure: CT • MRI Specialty: Diagnostic radiology • pediatrics Objective: Unusual presentation of unknown etiology, Rare disease, Mistake in diagnosis Background: Müllerian anomalies encompass a wide variety of malformations in the female genital tract, usually associated with renal and anorectal malformations. Of these anomalies, approximately 11% are uterus didelphys, which occurs when midline fusion of the müllerian ducts is arrested to a variable extent. Case Report: We report the case of a 16-year-old female with uterine didelphys, jejunal malrotation, hematometra, hematosalpinx, and bilateral subcentimeter homogenous circular cystic-like renal lesions, who initially presented with left lower quadrant abdominal pain, non-bloody vomiting, and a history of irregular menstrual periods. Initial CT was confusing for an adnexal cystic mass, but further imaging disclosed the above müllerian anomalies. Conclusions: Müllerian anomalies may mimic other, more common, adnexal lesions; thus, adequate evaluation of suspicious cystic adnexal masses with multiple and advanced imaging modalities such as MRI is essential for adequate diagnosis and management. PMID:23826455

  15. Control of ocular inflammation.

    PubMed

    Wilkie, D A

    1990-05-01

    Although both topical and systemic anti-inflammatory agents have a place in veterinary ophthalmology, they play only a small role in overall patient management. They must be used appropriately to prevent ocular damage and loss of vision from inflammation and are not a replacement for a complete ophthalmic examination and specific treatment directed at the etiology of the problem. If used indiscriminately, they can result in local or systemic side effects or toxicities, many of which are worse than the initial problem for which they were selected. Just as topical corticosteroids are contraindicated with infectious keratitis, so are systemic corticosteroids contraindicated in patients with ocular inflammation resulting from a systemic infectious process. Anti-inflammatories must be used at the appropriate dosage and frequency. Use of corticosteroids that have low intraocular penetration for intraocular disease or corticosteroids with low potency is a waste of time and money. The most expensive medication is one that does not work. Avoid combination therapies when only a single medication is required. These do not save time or money and have the potential to result in the development of drug-related diseases. Diseases for which anti-inflammatory therapy has little or no indication include corneal scars, corneal edema, corneal pigmentation, corneal dystrophy, cataracts without inflammation, glaucoma, and retinal atrophy and degeneration. Last, remember that all commercially available ophthalmic medications are specifically formulated for use in the eye. Their pH, concentration, osmolality, and melting temperature all are designed to facilitate penetration. The use of dermal and otic preparations to treat ophthalmic problems is contraindicated. PMID:2194354

  16. Ocular involvement and visual outcome of herpes zoster ophthalmicus: review of 45 patients from Tunisia, North Africa

    PubMed Central

    2014-01-01

    Background Ocular complications of herpes zoster ophthalmicus (HZO) may lead to substantial visual impairment. The purpose of this study was to characterize and analyze ocular involvement and visual outcome of HZO in patients from Tunisia, North Africa. This study is a retrospective chart review of 51 eyes of 45 patients with HZO. Results Mean age was 44.5 years. Thirty patients (66.7%) were aged over 50 years. Twenty-four patients (53.3%) were male and 21 patients were female (46.7%). There was no statistically significant difference in gender distribution. Initial mean best corrected visual acuity (BCVA) was 20/50. Ocular manifestations included adnexal involvement (58.8%), keratitis (31.4%), keratouveitis (31.4%), isolated anterior uveitis (AU) (29.4%), intraocular pressure elevation (23.5%), oculomotor nerve palsy (5.8%), and optic neuritis (1.9%). Isolated AU (p < 0.001), isolated keratitis (p = 0.001), and intraocular pressure elevation (p = 0.013) were more likely to be concomitant to HZO active skin disease, while keratouveitis occurred more likely more than 1 month after HZO eruption (p < 0.001). AU and keratouveitis were more likely to be associated with age ≥ 50 years (p = 0.001 and p = 0.02, respectively). Ocular complications included neurotrophic keratopathy (1.9%), corneal opacity (5.9%), secondary glaucoma (7.8%), optic atrophy (1.9%), and postherpetic neuralgia (13.3%). Mean follow-up was 12 months. Mean final BCVA was 20/32; it was ≥ 20/40 in 78.4% of the eyes. Conclusions Our study provided epidemiologic and clinical data of HZO in a Tunisian population. AU and keratitis were the most common ocular complications. Neurotrophic keratopathy was scarce. The overall visual outcome is good, with about three quarters of the treated patients maintaining VA of 20/40 or better. PMID:25246984

  17. 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

  18. Single-Incision Single-Instrument Adnexal Surgery in Pediatric Patients

    PubMed Central

    Loux, Tara; Falk, Gavin A.; Gaffley, Michaela; Ortega, Stephanie; Ramos, Carmen; Malvezzi, Leopoldo; Knight, Colin G.; Burnweit, Cathy

    2015-01-01

    Introduction. Pediatric surgeons often practice pediatric gynecology. The single-incision single-instrument (SISI) technique used for appendectomy is applicable in gynecologic surgery. Methods. We retrospectively analyzed the records of patients undergoing pelvic surgery from 2008 to 2013. SISI utilized a 12 mm transumbilical trocar and an operating endoscope. The adnexa can be detorsed intracorporeally or extracorporealized via the umbilicus for lesion removal. Results. We performed 271 ovarian or paraovarian surgeries in 258 patients. In 147 (54%), the initial approach was SISI; 75 cases (51%) were completed in patients aged from 1 day to 19.9 years and weighing 4.7 to 117 kg. Conversion to standard laparoscopy was due to contralateral oophoropexy, solid mass, inability to mobilize the adnexa, large mass, bleeding, adhesions, or better visualization. When SISI surgery was converted to Pfannenstiel, the principal reason was a solid mass. SISI surgery was significantly shorter than standard laparoscopy. There were no major complications and the overall cohort had an 11% minor complication rate. Conclusion. SISI adnexal surgery is safe, quick, inexpensive, and effective in pediatric patients. SISI was successful in over half the patients in whom it was attempted and offers a scarless result. If unsuccessful, the majority of cases can be completed with standard multiport laparoscopy. PMID:26557994

  19. Adnexal germ cell carcinoma with bone metastases in pregnant women: case report and review.

    PubMed

    Tenorio-Guadalupe, María Del Rosario; Arsentales-Montalva, Valeria; Yonz-Buendía, Yessabell Sonia; Fiestas-Saldarriaga, Fabián; Pimentel-Álvarez, Patricia

    2016-01-01

    Germ cell carcinoma during pregnancy is rare. However, its detection has increased due to the use of ultrasound fetal monitoring in the antenatal care program. In this article, we present the case of a Germ cell carcinoma during pregnancy is rare. However, its detection has increased due to the use of ultrasound fetal monitoring in the antenatal care program. In this article, we present the case of a pregnant 27-year-old diagnosed with an adnexal germ cell carcinoma at six weeks of gestation, whose initial approach was local resection (suboptimal cytoreduction). Four weeks after surgery, the patient presented with grade IV peripheral neuropathy in the lower limbs; magnetic resonance imaging scan indicated an infiltrative lesion at D5. The local medical board decided on chemotherapy starting on the 19th week of gestation. The rest of the pregnancy period was uneventful and the patient had a cesarean section at 34 weeks of gestation and a live newborn with no complications. Unfortunately, four days after caesarean section, the patient died of a septic shock with respiratory focus. PMID:27602713

  20. Purinergic Receptors in Ocular Inflammation

    PubMed Central

    Guzman-Aranguez, Ana; Gasull, Xavier; Diebold, Yolanda; Pintor, Jesús

    2014-01-01

    Inflammation is a complex process that implies the interaction between cells and molecular mediators, which, when not properly “tuned,” can lead to disease. When inflammation affects the eye, it can produce severe disorders affecting the superficial and internal parts of the visual organ. The nucleoside adenosine and nucleotides including adenine mononucleotides like ADP and ATP and dinucleotides such as P1,P4-diadenosine tetraphosphate (Ap4A), and P1,P5-diadenosine pentaphosphate (Ap5A) are present in different ocular locations and therefore they may contribute/modulate inflammatory processes. Adenosine receptors, in particular A2A adenosine receptors, present anti-inflammatory action in acute and chronic retinal inflammation. Regarding the A3 receptor, selective agonists like N6-(3-iodobenzyl)-5′-N-methylcarboxamidoadenosine (CF101) have been used for the treatment of inflammatory ophthalmic diseases such as dry eye and uveoretinitis. Sideways, diverse stimuli (sensory stimulation, large intraocular pressure increases) can produce a release of ATP from ocular sensory innervation or after injury to ocular tissues. Then, ATP will activate purinergic P2 receptors present in sensory nerve endings, the iris, the ciliary body, or other tissues surrounding the anterior chamber of the eye to produce uveitis/endophthalmitis. In summary, adenosine and nucleotides can activate receptors in ocular structures susceptible to suffer from inflammatory processes. This involvement suggests the possible use of purinergic agonists and antagonists as therapeutic targets for ocular inflammation. PMID:25132732

  1. Radiotherapy for ocular tumours.

    PubMed

    Stannard, C; Sauerwein, W; Maree, G; Lecuona, K

    2013-02-01

    Ocular tumours present a therapeutic challenge because of the sensitive tissues involved and the necessity to destroy the tumour while minimising visual loss. Radiotherapy (RT) is one of several modalites used apart from surgery, laser, cryotherapy, and chemotherapy. Both external beam RT (EBRT) and brachytherapy are used. Tumours of the bulbar conjunctiva, squamous carcinoma and malignant melanoma, can be treated with a radioactive plaque: strontium-90, ruthenium-106 (Ru-106), or iodine-125 (I-125), after excision. If the tumour involves the fornix or tarsal conjunctiva, proton therapy can treat the conjunctiva and spare most of the eye. Alternatively, an I-125 interstitial implant can be used with shielding of the cornea and lens. Conjunctival mucosal-associated lymphoid tissue lymphoma can be treated with an anterior electron field with lens shielding and 25-30 Gray (Gy) in 2 Gy fractions. Discrete retinoblastoma (RB), too large for cryotherapy or thermolaser, or recurrent after these modalities, can be treated with plaque therapy, I-125, or Ru-106. For large RB, multiple tumours, or vitreous seeds the whole eye can be treated with an I-125 applicator, sparing the bony orbit, or with EBRT, under anaesthetic, using X-rays or proton therapy with vacuum contact lenses to fix the eyes in the required position. Post-enucleated orbits at risk for recurrent RB can be treated with an I-125 implant with shielding to reduce the dose to the bony orbit. Uveal malignant melanomas can be treated with plaque or proton therapy with excellent local control. Preservation of vision will depend on the initial size and location of the tumour. PMID:23174750

  2. Radiotherapy for ocular tumours

    PubMed Central

    Stannard, C; Sauerwein, W; Maree, G; Lecuona, K

    2013-01-01

    Ocular tumours present a therapeutic challenge because of the sensitive tissues involved and the necessity to destroy the tumour while minimising visual loss. Radiotherapy (RT) is one of several modalites used apart from surgery, laser, cryotherapy, and chemotherapy. Both external beam RT (EBRT) and brachytherapy are used. Tumours of the bulbar conjunctiva, squamous carcinoma and malignant melanoma, can be treated with a radioactive plaque: strontium-90, ruthenium-106 (Ru-106), or iodine-125 (I-125), after excision. If the tumour involves the fornix or tarsal conjunctiva, proton therapy can treat the conjunctiva and spare most of the eye. Alternatively, an I-125 interstitial implant can be used with shielding of the cornea and lens. Conjunctival mucosal-associated lymphoid tissue lymphoma can be treated with an anterior electron field with lens shielding and 25–30 Gray (Gy) in 2 Gy fractions. Discrete retinoblastoma (RB), too large for cryotherapy or thermolaser, or recurrent after these modalities, can be treated with plaque therapy, I-125, or Ru-106. For large RB, multiple tumours, or vitreous seeds the whole eye can be treated with an I-125 applicator, sparing the bony orbit, or with EBRT, under anaesthetic, using X-rays or proton therapy with vacuum contact lenses to fix the eyes in the required position. Post-enucleated orbits at risk for recurrent RB can be treated with an I-125 implant with shielding to reduce the dose to the bony orbit. Uveal malignant melanomas can be treated with plaque or proton therapy with excellent local control. Preservation of vision will depend on the initial size and location of the tumour. PMID:23174750

  3. 21 CFR 886.1040 - Ocular esthesiometer.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...) MEDICAL DEVICES OPHTHALMIC DEVICES Diagnostic Devices § 886.1040 Ocular esthesiometer. (a) Identification. An ocular esthesiometer is a device, such as a single-hair brush, intended to touch the cornea...

  4. 21 CFR 886.1040 - Ocular esthesiometer.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...) MEDICAL DEVICES OPHTHALMIC DEVICES Diagnostic Devices § 886.1040 Ocular esthesiometer. (a) Identification. An ocular esthesiometer is a device, such as a single-hair brush, intended to touch the cornea...

  5. 21 CFR 886.1040 - Ocular esthesiometer.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...) MEDICAL DEVICES OPHTHALMIC DEVICES Diagnostic Devices § 886.1040 Ocular esthesiometer. (a) Identification. An ocular esthesiometer is a device, such as a single-hair brush, intended to touch the cornea...

  6. 21 CFR 886.1040 - Ocular esthesiometer.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...) MEDICAL DEVICES OPHTHALMIC DEVICES Diagnostic Devices § 886.1040 Ocular esthesiometer. (a) Identification. An ocular esthesiometer is a device, such as a single-hair brush, intended to touch the cornea...

  7. GATA3 Expression in Normal Skin and in Benign and Malignant Epidermal and Cutaneous Adnexal Neoplasms

    PubMed Central

    de Peralta-Venturina, Mariza N.; Balzer, Bonnie L.; Frishberg, David P.

    2015-01-01

    Abstract: Initial investigations reported GATA3 to be a sensitive and relatively specific marker for mammary and urothelial carcinomas. Recently, GATA3 expression has been described in several other epithelial tumors. However, there has been only limited investigation of GATA3 expression in cutaneous epithelial tumors. The objective of this study was to examine the immunohistochemical expression of GATA3 in a wide variety of cutaneous epithelial neoplasms. GATA3 expression was evaluated in 99 benign and 63 malignant cutaneous epithelial tumors. GATA3 was consistently and usually strongly expressed in clear cell acanthoma, trichofolliculoma, trichoepithelioma, trichilemmoma, sebaceous adenoma, sebaceoma, apocrine hidrocystoma, apocrine tubular papillary adenoma, hidradenoma papilliferum, and syringocystadenoma papilliferum. Hidradenomas exhibited variable positive staining. Most poromas, syringomas, chondroid syringomas, cylindromas, and spiradenomas were negative or only focally and weakly positive. Focal staining was present in all pilomatrixomas. Thirteen of 14 basal cell carcinomas, 21 of 24 squamous carcinomas, and all 6 sebaceous carcinomas exhibited positive staining. The 1 apocrine carcinoma, both mucinous carcinomas, and 2 of 3 microcystic adnexal carcinomas also exhibited positive staining, whereas the 1 eccrine porocarcinoma and the 1 adenoid cystic carcinoma were negative. One of 11 Merkel cell carcinomas exhibited focal weak staining. Our findings demonstrate that GATA3 is expressed in a wide variety of benign and malignant cutaneous epithelial neoplasms. In addition to carcinomas of breast and urothelial origin and other more recently described GATA3-positive tumors, the differential diagnosis of a metastatic tumor of unknown primary origin that expresses GATA3 should also include a carcinoma of cutaneous epithelial origin. PMID:26595821

  8. Saccular impact on ocular torsion.

    PubMed

    De Graaf, B; Bos, J E; Groen, E

    1996-01-01

    When someone is tilted laterally, the shear force on the maculae of the utriculus and the sacculus is described by the sine and the cosine of the angle of tilt, respectively. So both the sacculus and the utriculus are stimulated, but in the literature, ocular torsion is normally attributed to utricular function alone (and, thus, seen as a response to y-axis linear acceleration). However, on the base of a series of experiments on a tilt chair, a linear track, human centrifuges, and during parabolic flights, we conclude that the sacculus contributes to ocular torsion as well (there is a response to z-axis linear acceleration). The data suggest that the ratio of the utricular and saccular impact on ocular torsion is 3:1. The utriculus generates conjugate and the sacculus disjunctive torsional eye movements. PMID:8886354

  9. The Ocular Surface Chemical Burns

    PubMed Central

    Baradaran-Rafii, Alireza; Djalilian, Ali R.

    2014-01-01

    Ocular chemical burns are common and serious ocular emergencies that require immediate and intensive evaluation and care. The victims of such incidents are usually young, and therefore loss of vision and disfigurement could dramatically affect their lives. The clinical course can be divided into immediate, acute, early, and late reparative phases. The degree of limbal, corneal, and conjunctival involvement at the time of injury is critically associated with prognosis. The treatment starts with simple but vision saving steps and is continued with complicated surgical procedures later in the course of the disease. The goal of treatment is to restore the normal ocular surface anatomy and function. Limbal stem cell transplantation, amniotic membrane transplantation, and ultimately keratoprosthesis may be indicated depending on the patients' needs. PMID:25105018

  10. Ocular Immune Privilege and Transplantation

    PubMed Central

    Taylor, Andrew W.

    2016-01-01

    Allografts are afforded a level of protection from rejection within immune-privileged tissues. Immune-privileged tissues involve mechanisms that suppress inflammation and promote immune tolerance. There are anatomical features, soluble factors, membrane-associated proteins, and alternative antigen-presenting cells (APC) that contribute to allograft survival in the immune-privileged tissue. This review presents the current understanding of how the mechanism of ocular immune privilege promotes tolerogenic activity by APC, and T cells in response to the placement of foreign antigen within the ocular microenvironment. Discussed will be the unique anatomical, cellular, and molecular mechanisms that lessen the chance for graft destroying immune responses within the eye. As more is understood about the molecular mechanisms of ocular immune privilege greater is the potential for using these molecular mechanisms in therapies to prevent allograft rejection. PMID:26904026

  11. [Ocular immune reconstitution inflammatory syndrome].

    PubMed

    Ma, N; Ye, J J

    2016-02-11

    Immune reconstitution inflammatory syndrome (IRIS) is a collection of inflammatory disorders associated with paradoxical worsening of preexisting infectious processes or emerging diseases or even dead after the initiation of highly active antiretroviral therapy (HAART) in human immunodeficiency virus (HIV) infected individuals in a period of recovery of immune function. Ocular immune reconstitution inflammatory syndrome is mainly caused by cytomegalovirus which performing a series of ocular inflammation accompanied with the increase of CD4+ T lymphocytes, such as cytomegalovirus retinitis, after HAART. With HAART widely used, the patients of IRIS gradually increased. But the clinical presentations of IRIS were various because of different pathogens. This review summarized the clinical manifestations, risk factors, diagnosis and treatment of ocular IRIS.(Chin J Ophthalmol, 2016, 51: 150-153). PMID:26906710

  12. Primary alveolar rhabdomyosarcoma of fallopian tube masquerading as a unilateral adnexal mass: A case report and literature review.

    PubMed

    Shahin, Nisreen Abu; Alqaisy, Amin; Zheng, Wenxin

    2015-01-01

    Rhabdomyosarcoma (RMS) is a high-grade sarcoma that predominantly affects children, and rarely, the adult population. RMS demonstrates three major histologic variants: Embryonal, alveolar, and pleomorphic. A limited number of documented pure RMS cases of the gynecologic organs in adult women are found in the literature. Of these reports, the fallopian tube (FT) is reported as the primary site in only three cases, those included one of embryonal and two of the pleomorphic histologic variants. Herein, we report the first case of alveolar RMS arising in the FT of an adult woman and presenting as a unilateral adnexal mass. PMID:26549082

  13. Air bags and ocular injuries.

    PubMed Central

    Stein, J D; Jaeger, E A; Jeffers, J B

    1999-01-01

    PURPOSE: This investigation retrospectively examined ocular injuries associated with air bag deployment to gain a better appreciation of potential risk factors in motor vehicle accidents. National statistics regarding the efficacy of air bags were reviewed. METHODS: Review of the literature from 1991 to 1998 identified 44 articles describing 97 patients with air-bag-induced ocular injuries. Variables extracted from each case were age, sex, height, position in the car, eye wear, vehicle impact speed, visual acuity, and specific ocular injuries. RESULTS: Corneal abrasions occurred in 49% of occupants, hyphemas in 43%, vitreous or retinal hemorrhages in 25%, and retinal tears or detachments in 15%. The globe was ruptured in 10 patients. Patients involved in higher-speed accidents (over 30 mph) sustained a greater percentage of vitreous or retinal hemorrhages and traumatic cataracts, while those at slower speeds were more prone to retinal tears or detachments. In a subset of 14 patients with serious ocular injuries, the impact speed of 11 patients was recorded at 30 mph or less. Slower speed may be a risk factor for some ocular injuries. Occupant height was not a significant factor. National statistics confirm that air bags reduce fatalities in motor vehicle accidents. However, children sitting in the front seat without a seat belt and infants in passenger-side rear-facing car seats are at risk for fatal injury. CONCLUSION: Air bags combined with seat belts are an effective means of reducing injury and death in adults during motor vehicle accidents. However, this study has documented a wide variety of ocular injuries associated with air bag deployment. It is hoped that researchers can develop modifications that continue to save lives while minimizing additional harm. Images FIGURE 1 FIGURE 2A FIGURE 2B FIGURE 2C FIGURE 2D FIGURE 3A FIGURE 3B FIGURE 4 FIGURE 5 FIGURE 7 FIGURE 8 PMID:10703118

  14. [Adverse ocular effects of vaccinations].

    PubMed

    Ness, T; Hengel, H

    2016-07-01

    Vaccinations are very effective measures for prevention of infections but are also associated with a long list of possible side effects. Adverse ocular effects following vaccination have been rarely reported or considered to be related to vaccinations. Conjunctivitis is a frequent sequel of various vaccinations. Oculorespiratory syndrome and serum sickness syndrome are considered to be related to influenza vaccinations. The risk of reactivation or initiation of autoimmune diseases (e. g. uveitis) cannot be excluded but has not yet been proven. Overall the benefit of vaccination outweighs the possible but very low risk of ocular side effects. PMID:27357302

  15. 21 CFR 886.1040 - Ocular esthesiometer.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ocular esthesiometer. 886.1040 Section 886.1040...) MEDICAL DEVICES OPHTHALMIC DEVICES Diagnostic Devices § 886.1040 Ocular esthesiometer. (a) Identification. An ocular esthesiometer is a device, such as a single-hair brush, intended to touch the cornea...

  16. Ultraviolet light and ocular diseases.

    PubMed

    Yam, Jason C S; Kwok, Alvin K H

    2014-04-01

    The objective of this study is to review the association between ultraviolet (UV) light and ocular diseases. The data are sourced from the literature search of Medline up to Nov 2012, and the extracted data from original articles, review papers, and book chapters were reviewed. There is a strong evidence that ultraviolet radiation (UVR) exposure is associated with the formation of eyelid malignancies [basal cell carcinoma (BCC) and squamous cell carcinoma (SCC)], photokeratitis, climatic droplet keratopathy (CDK), pterygium, and cortical cataract. However, the evidence of the association between UV exposure and development of pinguecula, nuclear and posterior subcapsular cataract, ocular surface squamous neoplasia (OSSN), and ocular melanoma remained limited. There is insufficient evidence to determine whether age-related macular degeneration (AMD) is related to UV exposure. It is now suggested that AMD is probably related to visible radiation especially blue light, rather than UV exposure. From the results, it was concluded that eyelid malignancies (BCC and SCC), photokeratitis, CDK, pterygium, and cortical cataract are strongly associated with UVR exposure. Evidence of the association between UV exposure and development of pinguecula, nuclear and posterior subcapsular cataract, OSSN, and ocular melanoma remained limited. There is insufficient evidence to determine whether AMD is related to UV exposure. Simple behaviural changes, appropriate clothing, wearing hats, and UV blocking spectacles, sunglasses or contact lens are effective measures for UV protection. PMID:23722672

  17. Therapeutical Management for Ocular Rosacea

    PubMed Central

    López-Valverde, Gloria; Garcia-Martin, Elena; Larrosa-Povés, José Manuel; Polo-Llorens, Vicente; Pablo-Júlvez, Luis E.

    2016-01-01

    Purpose The purpose of this study is to describe a case of ocular rosacea with a very complex evolution. Rosacea is a chronic dermatological disease that may affect the ocular structures up to 6-72% of all cases. This form is often misdiagnosed, which may lead to long inflammatory processes with important visual consequences for affected patients. Therefore, an early diagnosis and an adequate treatment are important. Methods We report the case of a 43-year-old patient who had several relapses of what seemed an episode of acute bacterial conjunctivitis. Two weeks later, he developed a corneal ulcer with a torpid evolution including abundant intrastromal infiltrators and calcium deposits. He was diagnosed with ocular rosacea and treated with systemic doxycycline and topical protopic. Results A coating with amniotic membrane was placed in order to heal the ulcer, but a deep anterior lamellar keratoplasty to restore the patient's vision because of the corneal transparency loss was necessary. Conclusions Ocular rosacea includes multiple ophthalmic manifestations ranging from inflammation of the eyelid margin and blepharitis to serious corneal affectations. A delayed diagnosis can result in chronic inflammatory conditions including keratinization and loss of corneal transparency, which lead to important visual sequelae for affected patients. PMID:27462249

  18. Adverse ocular reactions to drugs.

    PubMed Central

    Spiteri, M. A.; James, D. G.

    1983-01-01

    Drugs acting on various parts of the body may also affect the eye insidiously. Increased awareness of such drug toxicity by the prescribing doctor should encourage him to consider effects on the cornea, lens, retina, optic nerve and elsewhere when checking the patient's progress. The following review concerns adverse ocular effects of systemic drug administration. PMID:6356101

  19. Ocular Toxoplasmosis: Lessons from Brazil

    Technology Transfer Automated Retrieval System (TEKTRAN)

    • A new attention to post-natally acquired infections. Previously, most attention was focused on infection during pregnancy, and the risk of congenital disease, with the feeling that infection in older individuals was benign, without a substantial risk of disease morbidity, such as ocular involvemen...

  20. Ocular manifestations of feline viral diseases.

    PubMed

    Stiles, Jean

    2014-08-01

    Feline viral diseases are common and cats can be presented with a variety of clinical manifestations. Ocular disease associated with viral pathogens is not unusual, particularly with viruses causing upper respiratory tract disease in cats, such as feline herpesvirus type 1 and feline calicivirus. These agents mainly cause ocular surface disease. Other viruses, such as feline immunodeficiency virus and feline coronavirus, can cause uveitis, while feline leukemia virus can induce ocular lymphosarcoma. This review covers the most common viral pathogens of cats that cause ocular manifestations, the specific features of the ocular diseases caused by these viruses and therapeutic recommendations. PMID:24461645

  1. Laparoendoscopic single-site surgery (LESS) versus conventional laparoscopic surgery for adnexal preservation: a randomized controlled study

    PubMed Central

    Cho, Yeon Jean; Kim, Mi-La; Lee, Soo Yoon; Lee, Hee Suk; Kim, Joo Myoung; Joo, Kwan Young

    2012-01-01

    Objective To compare the operative outcomes, postoperative pain, and subsequent convalescence after laparoendoscopic single-site surgery (LESS) or conventional laparoscopic surgery for adnexal preservation. Study design From December 2009 to September 2010, 63 patients underwent LESS (n = 33) or a conventional laparoscopic surgery (n = 30) for cyst enucleation. The overall operative outcomes including postoperative pain measurement using the visual analog scale (VAS) were evaluated (time points 6, 24, and 24 hours). The convalescence data included data obtained from questionnaires on the need for analgesics and on patient-reported time to recovery end points. Results The preoperative characteristics did not significantly differ between the two groups. The postoperative hemoglobin drop was higher in the LESS group than in the conventional laparoscopic surgery group (P = 0.048). Postoperative pain at each VAS time point, oral analgesic requirement, intramuscular analgesic requirement, and the number of days until return to work were similar in both groups. Conclusion In adnexa-preserving surgery performed in reproductive-age women, the operative outcomes, including satisfaction of the patients and convalescence after surgery, are comparable for LESS and conventional laparoscopy. LESS may be a feasible and a promising alternative method for scarless abdominal surgery in the treatment of young women with adnexal cysts PMID:22448110

  2. Ocular surface foreign bodies: novel findings mimicking ocular malignant melanoma

    PubMed Central

    Maudgil, A; Wagner, B E; Rundle, P; Rennie, I G; Mudhar, H S

    2014-01-01

    Purpose Malignant melanoma of the eye is an uncommon condition that is important to recognise. We describe three cases in which ocular foreign bodies have masqueraded as ocular malignant melanoma. Methods Interventional case reports. Results Case 1 describes diathermy-induced carbon particle implantation, during plaque therapy for the treatment of uveal melanoma, mimicking recurrence with extra-scleral invasion. Case 2 shows a foreign body called ‘mullite' mimicking conjunctival melanoma. Case 3 demonstrates a conjunctival foreign body called ‘illite' that mimicked a limbal melanocytic lesion, clinically thought to be either melanocytoma or melanoma. Conclusion This report highlights the importance of careful history taking, examination, and appropriate biopsy in cases of suspected malignant melanoma, to prevent unnecessary and potentially radical treatment. PMID:25104745

  3. [Mechanism of angiogenesis. Ocular involvement].

    PubMed

    Mocanu, Carmen

    2003-01-01

    Over the past several years, there has been important progress in the field of intrinsec mechanisms of ocular neovascularization. Immunohistological studies succeeded a better systematization of the factors that stimulates and inhibits this process. Their presence in different ocular normal structures, without any angiogenic activity, suggests a physiological balance between VEGF (vascular endothelial growth factor) with stimulatory effect on angiogenesis and PEDF (pigment epithelium derived factor) with inhibitory effect. It has been discussing the possibility of modification of physiological balance between VEGF and PEDF to induce the neovascularization process. The understanding of the physiopathological mechanisms of the substances implicated in inhibition of chorioretinal neovascularization makes to be real the expectations for the development of new treatments. PMID:15083677

  4. Ocular Complications of Chloroquine Therapy

    PubMed Central

    Lloyd, Lois A.; Hiltz, John W.

    1965-01-01

    Ocular complications of long-term chloroquine therapy were observed in 18 of 45 patients so treated. This therapy was used in patients with rheumatoid arthritis, lupus erythematosus, sarcoidosis, discoid lupus and other chronic “collagen disease”. Thirteen patients had reversible corneal opacifications, and seven had irreversible retinal changes, with visual loss and visual field defects. Pathological evidence of chloroquine retinopathy was obtained in one patient. Physicians are therefore warned to use this drug only after careful consideration. If it is used, repeated ocular examinations should include assessment of visual acuity, visual fields on a tangent screen and fundus examination through a dilated pupil. ImagesFig. 4Fig. 7Fig. 8 PMID:14275038

  5. [Difficulties in differential diagnosis of adnexal masses during pregnancy: the role of greyscale and color doppler sonography].

    PubMed

    Czekierdowski, A; Bednarek, W; Rogowska, W; Kotarski, J

    2001-12-01

    We have attempted to determine the accuracy of greyscale and color Doppler ultrasound in the differentiation of adnexal masses in pregnancy. The studied group included 2245 pregnant women from low risk population. Following criteria were evaluated: maximal diameter and volume of the tumor, echogenicity, presence of septa and papillary projections in grey scale sonography. Color Doppler analysis included blood vessel presence and arrangement and blood flow characteristics with the use of pulsatility (PI), resistive (RI) and systolic/diastolic (S/D) indices. Preoperative CA-125 serum levels were available in 11 patients. In 66 (2.94%) patients adnexal tumors were detected during routine ultrasound scan at the end of the first trimester. Twenty-seven masses (1.2%) persisted beyond 16 weeks of gestation and were subsequently surgically removed. Pathological diagnosis confirmed 19 serous cystadenomas, 4 endometriomas and 2 dermoids, one pedunculated myoma and one fibrothecoma. Mean size of the tumors was 79 Jmm (range: 43-245 mm), mean volume 166. lml (range: 30-1320 ml). Doppler indices values presented as mean, SD and range were as follows: PI = 1.26 +/- 0.71 (range: 0.57-3.84); RI = 0.61 +/- 0.15 (range: 0.33-0.89) and S/D = 2.62 +/- 0.98 (range: 1.17-4.91). Median serum concentration of CA-125 was 17 IU/ml (range: 8.4-1247 IU/ml). Only 3 of these women had elevated (> 35 IU/ml) levels: 2 endometriomas (344 IU/ml and 1247 IU/ml) and one myoma (37 IU/ml), respectively. Based on the sonographic findings two solid tumors were incorrectly classified as probably malignant (fibrothecoma and subserous myoma). Negative predictive value of ultrasound diagnosis in the studied population was therefore 92.6% (25 of 27). We conclude that although prenatal sonography has the potential to correctly classify most of adnexal masses, caution in risk assessment is needed especially when persistent solid tumor is found. PMID:11883264

  6. Ocular toxoplasmosis in AIDS patients.

    PubMed Central

    Gagliuso, D J; Teich, S A; Friedman, A H; Orellana, J

    1990-01-01

    We describe 16 cases of ocular and, in some patients, associated CNS toxoplasmosis in AIDS patients. T gondii is commonly associated with infection in the immunocompromised host. The lesions are most often seen in the CNS and eyes; involvement in the brain, heart, lung, liver, spleen, and lymph nodes may be observed. CNS involvement by toxoplasmosis may be an initial manifestation of AIDS and may be associated with discrete or diffuse lesions. CT scan and MR imaging may demonstrate a multitude of lesions often displaying the characteristic ring-shaped enhancement after contrast injection. Ocular involvement by toxoplasmosis, though less common than CNS involvement, is characterized by several features. These may be manifested as single or multifocal retinal lesions in one or both eyes or massive areas of retinal necrosis. Invariably these lesions are unassociated with a pre-existing retinochoroidal scar suggesting that the lesions are a manifestation of acquired rather than congenital disease. Presence of IgM antibodies may support this observation although antibody levels in AIDS patients may not reflect the magnitude of disease. Vitreous reaction is often minimal. Anterior uveitis has been reported in one case. Treatment of the ocular infection with pyrimethamine, clindamycin and sulfadiazine is effective in over 75% of patients. Once resolution of the ocular infection is observed, maintenance therapy is continued as relapses occur in the absence of treatment. Corticosteroid treatment is unnecessary and its use has been associated with the development of CMV retinitis. Other retinal infections in AIDS patients which should be considered in the differential diagnosis include CMV, herpetic-associated ARN and syphilis. Concomitant CMV and toxoplasmosis in the same eye have been seen. Images FIGURE 1 FIGURE 2 A FIGURE 2 B FIGURE 3 A FIGURE 3 B FIGURE 4 A FIGURE 4 B FIGURE 5 A FIGURE 5 B FIGURE 6 FIGURE 7 FIGURE 8 A FIGURE 8 B FIGURE 9 A FIGURE 9 B FIGURE 9 C PMID

  7. Facial asymmetry in ocular torticollis.

    PubMed

    Akbari, Mohammad Reza; Khorrami Nejad, Masoud; Askarizadeh, Farshad; Pour, Fatemeh Farahbakhsh; Ranjbar Pazooki, Mahsa; Moeinitabar, Mohamad Reza

    2015-01-01

    Torticollis can arise from nonocular (usually musculoskeletal) and ocular conditions. Some facial asymmetries are correlated with a history of early onset ocular torticollis supported by the presence of torticollis on reviewing childhood photographs. When present in an adult, this type of facial asymmetry with an origin of ocular torticollis should help to confirm the chronicity of the defect and prevent unnecessary neurologic evaluation in patients with an uncertain history. Assessment of facial asymmetry consists of a patient history, physical examination, and medical imaging. Medical imaging and facial morphometry are helpful for objective diagnosis and measurement of the facial asymmetry, as well as for treatment planning. The facial asymmetry in congenital superior oblique palsy is typically manifested by midfacial hemihypoplasia on the side opposite the palsied muscle, with deviation of the nose and mouth toward the hypoplastic side. Correcting torticollis through strabismus surgery before a critical developmental age may prevent the development of irreversible facial asymmetry. Mild facial asymmetry associated with congenital torticollis has been reported to resolve with continued growth after early surgery, but if asymmetry is severe or is not treated in the appropriate time, it might remain even with continued growth after surgery. PMID:27239567

  8. Transplant related ocular surface disorders: Advanced techniques for ocular surface rehabilitation after ocular complications secondary to hematopoietic stem cell transplantation.

    PubMed

    Stahl, Erin D; Mahomed, Faheem; Hans, Amneet K; Dalal, Jignesh D

    2016-05-01

    HSCT has been linked to the development of an assortment of ocular surface complications with the potential to lead to permanent visual impairment if left untreated or if not treated early in the course of disease. Strategies for therapy include maintenance of lubrication and tear preservation, prevention of evaporation, decreasing inflammation, and providing epithelial support. The ultimate aim of treatment is to prevent permanent ocular sequelae through prompt ophthalmology consultation and the use of advanced techniques for ocular surface rehabilitation. We describe several rehabilitation options of ocular surface complications occurring secondarily during the post-HSCT course. PMID:26869458

  9. [Diagnosis and treatment of ocular hypertension].

    PubMed

    Sun, Y Y; Chen, W W; Wang, N L

    2016-07-01

    Ocular hypertension is popular among people, with a prevalence of 3% to 10% in those older than 40 years old. Without proper intervention, over 10% of the patients with ocular hypertension would develop glaucoma in the following 5 to 10 years. Glaucoma has become one of the leading causes of blindness all over the world, which makes it essential for us to pay enough attention to the prevention and treatment of ocular hypertension. However, it is not cost-effective to treat all the patients with ocular hypertension. Certain side effects may also be caused with long-term medical treatment. Therefore, it is of great importance for ophthalmologists to identify the right time and use appropriate therapeutic methods. To introduce the knowledge of ocular hypertension, the definition, epidemiology, diagnosis, risk factors and treatment of ocular hypertension are reviewed in this article. (Chin J Ophthalmol, 2016, 52: 542-546). PMID:27531115

  10. Ocular Toxicity of Tyrosine Kinase Inhibitors

    PubMed Central

    Davis, Mary Elizabeth

    2016-01-01

    Purpose/Objectives To review common tyrosine kinase inhibitors, as well as their ocular side effects and management. Data Sources A comprehensive literature search was conducted using cINahl®, Pubmed, and cochrane databases for articles published since 2004 with the following search terms: ocular toxicities, tyrosine kinase inhibitors, ophthalmology, adverse events, eye, and vision. Data Synthesis Tyrosine kinase inhibitors can cause significant eye toxicity. Conclusions Given the prevalence of new tyrosine kinase inhibitor therapies and the complexity of possible pathogenesis of ocular pathology, oncology nurses can appreciate the occurrence of ocular toxicities and the role of nursing in the management of these problems. Implications for Nursing Knowledge of the risk factors and etiology of ocular toxicity of targeted cancer therapies can guide nursing assessment, enhance patient education, and improve care management. Including a review of eye symptoms and vision issues in nursing assessment can enhance early detection and treatment of ocular toxicity. PMID:26906134

  11. Tumors of the ocular surface: A review

    PubMed Central

    Honavar, Santosh G; Manjandavida, Fairooz P

    2015-01-01

    Tumors of the Ocular Surface clinically manifest with a very wide spectrum and include several forms of epithelial, stromal, caruncular, and secondary tumors. As a group, these tumors are seen commonly in the clinical practice of a comprehensive ophthalmologist, cornea specialist, and an ocular oncologist. This review is aimed to discuss the common tumors of the ocular surface and emphasize on their clinical diagnosis and appropriate management. PMID:25971163

  12. Ocular Syphilis among HIV-Infected Individuals

    PubMed Central

    Li, Jonathan Z.; Tucker, Joseph D.; Lobo, Ann-Marie; Marra, Christina M.; Davis, Benjamin T.; Papaliodis, George N.; Felsenstein, Donna; Durand, Marlene L.; Yawetz, Sigal; Robbins, Gregory K.

    2010-01-01

    We describe a human immunodeficiency virus (HIV)–infected individual with ocular manifestations of secondary syphilis. Twelve other cases of HIV-associated ocular syphilis are also presented. Six of 12 individuals had normal cerebrospinal fluid study results, and 3 patients required retreatment within 1.5 years. In patients with HIV infection, clinicians should be vigilant for ocular syphilis despite normal cerebrospinal fluid measures and for syphilis reinfection. PMID:20604717

  13. Microcystic adnexal carcinoma in a non-Caucasian patient: A case report and review of the literature

    PubMed Central

    CHEN, JINGXIN; YANG, SHIPING; CHEN, JIMIN; LIAO, TIANAN; DENG, WEI; LI, WEIZHONG

    2016-01-01

    Microcystic adnexal carcinoma (MAC) is extremely rare among Asians, with the majority of cases presenting in Caucasian individuals. The current study describes the case of a 38-year-old Chinese woman who presented with a 10 year history of a mass in the upper lip. A biopsy resulted in a diagnosis of MAC. The patient underwent complete surgical resection and the tumor was successfully excised. During 6 months of follow-up, there was no evidence of recurrence. To the best of our knowledge, the present case is the first Chinese case to be reported in the English literature, and is presented with the aim of increasing the awareness and aiding in the management of MAC in non-Caucasian populations. PMID:27073500

  14. A female adnexal tumor of probable Wolffian origin showing positive O-6-methylguanine-DNA methyltransferase methylation

    PubMed Central

    Kwon, Min Jung; Yun, Min Jeong

    2016-01-01

    Female adnexal tumor of probable Wolffian origin (FATWO) is a rare disease entity that arises from the mesonephric duct system. FATWO is different than other gynecological cancers in terms of embryology. Here, we describe the case of a 52-year-old woman with malignant FATWO. The patient underwent explorative laparotomy and surgical staging after a frozen section revealed malignancy. Detailed examination of the pathologic findings were consistent with FATWO. Counseling and further testing were provided to the patient to assess the risk of germline mutation and epigenetic change. An O-6-methylguanine-DNA methyltransferase gene methylation test was positive, and all other tests were normal. This is the first study to report a case of O-6-methylguanine-DNA methyltransferase methylation with FATWO in Korea. PMID:27462603

  15. [Glaucoma medications, preservatives and the ocular surface.

    PubMed

    Aptel, F; Labbé, A; Baudouin, C; Bron, A; Lachkar, Y; Sellem, E; Renard, J-P; Nordmann, J-P; Rouland, J-F; Denis, P

    2014-10-14

    Several clinical and experimental studies have demonstrated that ocular surface disease is common in glaucoma patients receiving chronic glaucoma drops, and that the preservatives in these drops play a major role in the occurrence of ocular surface disease. These ocular surface changes may induce both symptoms reported by the patients and anterior segment clinical signs, and should be systematically assessed by history and exam in all glaucoma patients. In these patients with ocular surface disease, reducing the amount of preservatives administered to the eye should be strived for, rather than adding additional eye drops to alleviate or mask the side effects of the glaucoma drops. PMID:25440185

  16. Ocular manifestations of infectious skin diseases.

    PubMed

    Sadowska-Przytocka, Anna; Czarnecka-Operacz, Magdalena; Jenerowicz, Dorota; Grzybowski, Andrzej

    2016-01-01

    Ocular complications of infectious skin diseases are a common occurrence. Managing the inflamed or infected eye in the emergency setting presents a diagnostic and therapeutic challenge to the emergency physician. Infectious agents may affect any part of the eye. Ocular findings may be the first sign of many infectious diseases, such as, for example, gonorrhea or chlamydia infection. Understanding the various forms of ocular involvement in these conditions is important, because untreated ophthalmic involvement can lead to severe vision loss. This review focuses on the significant ocular manifestations of the most common infectious diseases, including bacterial, viral, fungal, and parasitic infections, that both ophthalmologists and dermatologists may encounter. PMID:26903179

  17. Scleral Mechanisms Underlying Ocular Growth and Myopia

    PubMed Central

    Metlapally, Ravi; Wildsoet, Christine F.

    2015-01-01

    In the regulation of ocular growth, scleral events critically determine eye size and thus the refractive status of the eye. Increased scleral matrix remodeling can lead to exaggerated eye growth causing myopia and additionally increased risk of ocular pathological complications. Thus, therapies targeting these changes in sclera hold potential to limit such complications since sclera represents a relatively safe and accessible drug target. Understanding the scleral molecular mechanisms underlying ocular growth is essential to identifying plausible therapeutic targets in the sclera. This section provides a brief update on molecular studies that pertain to the sclera in the context of ocular growth regulation and myopia. PMID:26310158

  18. [Principles of treatment in ocular burns regarding the ocular surface and limbal stem cells].

    PubMed

    Potop, V; Dumitrache, Marieta

    2005-01-01

    The term ocular surface emphasizes the functional interdependence of the nonkeratinizing epithelium of cornea and conjunctiva. The limbal stem cells are responsible for replacement of corneal epithelium following ocular surface injuries. Over the past decades important advances in the management of chemical injury have occurred based on the application of theories on ocular surface and limbal stem cells. PMID:16245740

  19. Towards an evidence-based approach for diagnosis and management of adnexal masses: findings of the International Ovarian Tumour Analysis (IOTA) studies

    PubMed Central

    Kaijser, J.

    2015-01-01

    Whilst the outcomes for patients with ovarian cancer clearly benefit from centralised, comprehensive care in dedicated cancer centres, unfortunately the majority of patients still do not receive appropriate specialist treatment. Any improvement in the accuracy of current triaging and referral pathways whether using new imaging tests or biomarkers would therefore be of value in order to optimise the appropriate selection of patients for such care. An analysis of current evidence shows that such tests are now available, but still await recognition, acceptance and widespread adoption. It is therefore to be hoped that present guidance relating to the classification of ovarian masses will soon become more “evidence-based”. These promising tests include the International Ovarian Tumour Analysis (IOTA) LR2 model and ultrasound-based Simple Rules (SR). Based on a comprehensive recent meta-analysis both currently offer the optimal “evidence-based” approach to discriminating between cancer and benign conditions in women with adnexal tumours needing surgery. LR2 and SR are reliable tests having been shown to maintain a high sensitivity for cancer after independent external and temporal validation by the IOTA group in the hands of examiners with various levels of ultrasound expertise. They also offer more accurate triage compared to the existing Risk of Malignancy Index (RMI). The development of the IOTA ADNEX model represents an important step forward towards more individualised patient care in this area. ADNEX is a novel test that enables the more specific subtyping of adnexal cancers (i.e. borderline, stage 1 invasive, stage II-IV invasive, and secondary metastatic malignant tumours) and shares similar levels of accuracy to IOTA LR2 and SR for basic discrimination between cancer and benign disease. The IOTA study has made significant progress in relation to the classification of adnexal masses, however what is now needed is to see if these or new diagnostic tools

  20. Ocular drug delivery systems: An overview

    PubMed Central

    Patel, Ashaben; Cholkar, Kishore; Agrahari, Vibhuti; Mitra, Ashim K

    2014-01-01

    The major challenge faced by today’s pharmacologist and formulation scientist is ocular drug delivery. Topical eye drop is the most convenient and patient compliant route of drug administration, especially for the treatment of anterior segment diseases. Delivery of drugs to the targeted ocular tissues is restricted by various precorneal, dynamic and static ocular barriers. Also, therapeutic drug levels are not maintained for longer duration in target tissues. In the past two decades, ocular drug delivery research acceleratedly advanced towards developing a novel, safe and patient compliant formulation and drug delivery devices/techniques, which may surpass these barriers and maintain drug levels in tissues. Anterior segment drug delivery advances are witnessed by modulation of conventional topical solutions with permeation and viscosity enhancers. Also, it includes development of conventional topical formulations such as suspensions, emulsions and ointments. Various nanoformulations have also been introduced for anterior segment ocular drug delivery. On the other hand, for posterior ocular delivery, research has been immensely focused towards development of drug releasing devices and nanoformulations for treating chronic vitreoretinal diseases. These novel devices and/or formulations may help to surpass ocular barriers and associated side effects with conventional topical drops. Also, these novel devices and/or formulations are easy to formulate, no/negligibly irritating, possess high precorneal residence time, sustain the drug release, and enhance ocular bioavailability of therapeutics. An update of current research advancement in ocular drug delivery necessitates and helps drug delivery scientists to modulate their think process and develop novel and safe drug delivery strategies. Current review intends to summarize the existing conventional formulations for ocular delivery and their advancements followed by current nanotechnology based formulation developments

  1. Ocular perfusion pressure and ocular blood flow in glaucoma

    PubMed Central

    Cherecheanu, A Popa; Garhofer, G; Schmidl, D; Werkmeister, R; Schmetterer, L

    2013-01-01

    Glaucoma is a progressive optic neuropathy of unknown origin. It has been hypothesized that a vascular component is involved in glaucoma pathophysiology. This hypothesis has gained support from studies showing that reduced ocular perfusion pressure is a risk factor for the disease. The exact nature of the involvement is, however, still a matter of debate. Based on recent evidence we propose a model including primary and secondary insults in glaucoma. The primary insult appears to happen at the optic nerve head. Increased intraocular pressure and ischemia at the post-laminar optic nerve head affects retinal ganglion cell axons. Modulating factors are the biomechanical properties of the tissues and cerebrospinal fluid pressure. After this primary insult retinal ganglion cells function at a reduced energy level and are sensitive to secondary insults. These secondary insults may happen if ocular perfusion pressure falls below the lower limit of autoregulation or if neurovascular coupling fails. Evidence for both faulty autoregulation and reduced hyperemic response to neuronal stimulation has been provided in glaucoma patients. The mechanisms appear to involve vascular endothelial dysfunction and impaired astrocyte-vessel signaling. A more detailed understanding of these pathways is required to direct neuroprotective strategies via the neurovascular pathway. PMID:23009741

  2. Ocular perfusion pressure and ocular blood flow in glaucoma.

    PubMed

    Cherecheanu, A Popa; Garhofer, G; Schmidl, D; Werkmeister, R; Schmetterer, L

    2013-02-01

    Glaucoma is a progressive optic neuropathy of unknown origin. It has been hypothesized that a vascular component is involved in glaucoma pathophysiology. This hypothesis has gained support from studies showing that reduced ocular perfusion pressure is a risk factor for the disease. The exact nature of the involvement is, however, still a matter of debate. Based on recent evidence we propose a model including primary and secondary insults in glaucoma. The primary insult appears to happen at the optic nerve head. Increased intraocular pressure and ischemia at the post-laminar optic nerve head affects retinal ganglion cell axons. Modulating factors are the biomechanical properties of the tissues and cerebrospinal fluid pressure. After this primary insult retinal ganglion cells function at a reduced energy level and are sensitive to secondary insults. These secondary insults may happen if ocular perfusion pressure falls below the lower limit of autoregulation or if neurovascular coupling fails. Evidence for both faulty autoregulation and reduced hyperemic response to neuronal stimulation has been provided in glaucoma patients. The mechanisms appear to involve vascular endothelial dysfunction and impaired astrocyte-vessel signaling. A more detailed understanding of these pathways is required to direct neuroprotective strategies via the neurovascular pathway. PMID:23009741

  3. Anomalous phosphenes in ocular protontherapy

    NASA Astrophysics Data System (ADS)

    Khan, E.; Maréchal, F.; Dendale, R.; Mabit, C.; Calugaru, V.; Desjardin, L.; Narici, L.

    2010-04-01

    We have undertaken a clinical ground study of proton-induced light flashes (phosphenes). Patients treated at the Institut Curie - Centre de Protonthérapie in Orsay, France, received radiation therapy to cure ocular and skull-base cancers. Sixty percent of the patients treated for choroidal melanomas using 73 MeV protons report anomalous phosphenes. Delivering a radiation dose on the retina only is not sufficient to trigger the light flash. The present study may be the first indication of phosphenes triggered by protons of few tens of MeV.

  4. Translational models of ocular disease.

    PubMed

    Zeiss, Caroline J

    2013-07-01

    Animals provide indispensable models to translate basic mechanistic discoveries and realize their therapeutic potential in humans. Conversely, advances in human medicine often inform management of similar conditions in clinical veterinary medicine. In this paper, key experimental model species are introduced, with emphasis on genetic contributions of the mouse. Its role and those of larger animal models are described in common ocular research areas including intraocular neoplasia, corneal epithelial and stromal disease, cataract, uveitis, glaucoma, and retinal dystrophies. Emphasis is placed on those conditions shared by humans and domestic animals, with the intent of exploring how the study of comparable conditions in humans, domestic animals, and laboratory animals informs one another. PMID:23750503

  5. Raman Spectroscopy of Ocular Tissue

    NASA Astrophysics Data System (ADS)

    Ermakov, Igor V.; Sharifzadeh, Mohsen; Gellermann, Warner

    The optically transparent nature of the human eye has motivated numerous Raman studies aimed at the non-invasive optical probing of ocular tissue components critical to healthy vision. Investigations include the qualitative and quantitative detection of tissue-specific molecular constituents, compositional changes occurring with development of ocular pathology, and the detection and tracking of ocular drugs and nutritional supplements. Motivated by a better understanding of the molecular mechanisms leading to cataract formation in the aging human lens, a great deal of work has centered on the Raman detection of proteins and water content in the lens. Several protein groups and the hydroxyl response are readily detectable. Changes of protein compositions can be studied in excised noncataractous tissue versus aged tissue preparations as well as in tissue samples with artificially induced cataracts. Most of these studies are carried out in vitro using suitable animal models and conventional Raman techniques. Tissue water content plays an important role in optimum light transmission of the outermost transparent ocular structure, the cornea. Using confocal Raman spectroscopy techniques, it has been possible to non-invasively measure the water to protein ratio as a measure of hydration status and to track drug-induced changes of the hydration levels in the rabbit cornea at various depths. The aqueous humor, normally supplying nutrients to cornea and lens, has an advantageous anterior location for Raman studies. Increasing efforts are pursued to non-invasively detect the presence of glucose and therapeutic concentrations of antibiotic drugs in this medium. In retinal tissue, Raman spectroscopy proves to be an important tool for research into the causes of macular degeneration, the leading cause of irreversible vision disorders and blindness in the elderly. It has been possible to detect the spectral features of advanced glycation and advanced lipooxydation end products in

  6. Clinical and Immunological Responses in Ocular Demodecosis

    PubMed Central

    Kim, Jae Hoon; Chun, Yeoun Sook

    2011-01-01

    The purpose of this study was to investigate clinical and immunological responses to Demodex on the ocular surface. Thirteen eyes in 10 patients with Demodex blepharitis and chronic ocular surface disorders were included in this study and treated by lid scrubbing with tea tree oil for the eradication of Demodex. We evaluated ocular surface manifestations and Demodex counts, and analyzed IL-1β, IL-5, IL-7, IL-12, IL-13, IL-17, granulocyte colony-stimulating factor, and macrophage inflammatory protein-1β in tear samples before and after the treatment. All patients exhibited ocular surface manifestations including corneal nodular opacity, peripheral corneal vascularization, refractory corneal erosion and infiltration, or chronic conjunctival inflammatory signs before treatment. After treatment, Demodex was nearly eradicated, tear concentrations of IL-1β and IL-17 were significantly reduced and substantial clinical improvement was observed in all patients. In conclusion, we believe that Demodex plays an aggravating role in inflammatory ocular surface disorders. PMID:21935281

  7. Ocular diseases: immunological and molecular mechanisms

    PubMed Central

    Song, Jing; Huang, Yi-Fei; Zhang, Wen-Jing; Chen, Xiao-Fei; Guo, Yu-Mian

    2016-01-01

    Many factors, such as environmental, microbial and endogenous stress, antigen localization, can trigger the immunological events that affect the ending of the diverse spectrum of ocular disorders. Significant advances in understanding of immunological and molecular mechanisms have been researched to improve the diagnosis and therapy for patients with ocular inflammatory diseases. Some kinds of ocular diseases are inadequately responsive to current medications; therefore, immunotherapy may be a potential choice as an alternative or adjunctive treatment, even in the prophylactic setting. This article first provides an overview of the immunological and molecular mechanisms concerning several typical and common ocular diseases; second, the functions of immunological roles in some of systemic autoimmunity will be discussed; third, we will provide a summary of the mechanisms that dictate immune cell trafficking to ocular local microenvironment in response to inflammation. PMID:27275439

  8. Custom Ocular Prosthesis: A Palliative Approach

    PubMed Central

    Thakkar, Prachi; Patel, JR; Sethuraman, Rajesh; Nirmal, Narendra

    2012-01-01

    The goal of palliative care is the achievement of the best quality of life for patients and their families. Eyes are generally the first features of the face to be noticed. Loss of an eye is a traumatic event which has a crippling effect on the psychology of the patient. Several ocular and orbital disorders require surgical intervention that may result in ocular defects. An ocular prosthesis is fabricated to restore the structure, function, and cosmetics of the defects created by such conditions. Although an implant eye prosthesis has a superior outcome, due to economic factors it may not be a feasible option for all patients. Therefore, a custom-made ocular prosthesis is a good alternative. This case report presents a palliative treatment for a patient with an enucleated eye by fabricating a custom ocular prosthesis which improved his psychological, physical, social, functional, emotional and spiritual needs. PMID:22837616

  9. Ocular manifestations of frontonasal dysplasia.

    PubMed

    Roarty, J D; Pron, G E; Siegel-Bartelt, J; Posnick, J C; Buncic, J R

    1994-01-01

    The ophthalmologic findings associated with frontonasal dysplasia have not been defined previously in a large series of untreated children. We reviewed the ophthalmic manifestations of a series of patients with frontonasal dysplasia who were seen as part of their craniofacial evaluation. All had undergone a complete ophthalmologic examination before any manipulation of either the orbits or the soft tissues of the orbital contents. From 1986 to 1991, 23 patients with frontonasal dysplasia were seen; ophthalmologic abnormalities were found in 20 (87 percent). Abnormalities included significant refractive errors, strabismus, nystagmus, and eyelid ptosis. Three patients had amblyopia, a treatable cause of visual loss, from strabismus or anisometropia. Ten eyes in seven patients (30 percent) had severe structural anomalies, such as optic nerve hypoplasia, optic nerve colobomas, microphthalmia, cataract, corneal dermoid, or inflammatory retinopathy, that resulted in an acuity of 20/100 or worse. The high incidence of ocular abnormalities indicates that early assessment by an ophthalmologist should be part of the initial evaluation of patients with frontonasal dysplasia to detect treatable visual or ocular problems. PMID:8278482

  10. A comparison of clinical and surgical outcomes between laparo-endoscopic single-site surgery and traditional multiport laparoscopic surgery for adnexal tumors

    PubMed Central

    Lee, In Ok; Yoon, Jung Won; Chung, Dawn; Yim, Ga Won; Nam, Eun Ji; Kim, Sunghoon; Kim, Sang Wun

    2014-01-01

    Objective The purpose of this study was to compare clinical and surgical outcomes between laparo-endoscopic single-site (LESS) surgery and traditional multiport laparoscopic (TML) surgery for treatment of adnexal tumors. Methods Medical records were reviewed for patients undergoing surgery for benign adnexal tumors between January 2008 and April 2012 at our institution. All procedures were performed by the same surgeon. Clinical and surgical outcomes for patients undergoing LESS surgery using Glove port were compared with those patients undergoing TML surgery. Results A review of 129 patient cases undergoing LESS surgery using Glove port and 100 patient cases undergoing TML surgery revealed no significant differences in the baseline characteristics of the two groups. The median operative time was shorter in the LESS group using Glove port at 44 minutes (range, 19-126 minutes) than the TML group at 49 minutes (range, 20-196 minutes) (P=0.0007). There were no significant differences between in the duration of postoperative hospital stay, change in hemoglobin levels, pain score or the rate of complications between the LESS and TML groups. Conclusion LESS surgery showed comparable clinical and surgical outcomes to TML surgery, and required less operative time. Future prospective trials are warranted to further define the benefits of LESS surgery for adnexal tumor treatment. PMID:25264529