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1

Vasculitis  

MedlinePLUS

... Long-term treatment with medicines often can control the signs and symptoms of chronic vasculitis. Rarely, vasculitis doesn't respond well to treatment. This can lead to disability and even death. Much is still unknown about ...

2

Vasculitis  

Microsoft Academic Search

Systemic vasculitis is one of the most challenging diseases for physicians from a diagnostic perspective. Clinical symptoms\\u000a vary according to blood vessel size and organ systems involved. This article outlines manifestations, diagnosis, and management\\u000a of the vasculitides.

Anita Krishnamurthy; Gurtej S. Cheema; M. Eric Gershwin

2006-01-01

3

Hypersensitivity Vasculitis  

MedlinePLUS

Hypersensitivity Vasculitis joseph July 18, 2012 No Comments What is Hypersensitivity vasculitis? Hypersensitivity vasculitis (HV) is often used to ... blood vessels, called a leukocytoclastic vasculitis. What causes Hypersensitivity vasculitis? HV may be caused by a specific ...

4

Pediatric Vasculitis  

PubMed Central

Synopsis Childhood vasculitis is a challenging and complex group of conditions that are multisystem in nature and often require integrated care from multiple subspecialties including rheumatology, dermatology, cardiology, nephrology, neurology, and gastroenterology. Vasculitis is defined as the presence of inflammation in the blood vessel wall. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. This review explores the classification and general features of pediatric vasculitis as well as the clinical presentation, diagnostic evaluation, and therapeutic options for the most common vasculitides. PMID:22560577

Weiss, Pamela F.

2012-01-01

5

Urticarial Vasculitis  

MedlinePLUS

... results will open in a new tab or window. Search MedlinePlus : Participation in Clinical Trials is one way you can help advance research into finding better treatments and perhaps even a cure for vasculitis! VCRC- ...

6

Vasculitis Foundation  

MedlinePLUS

... Drive Life As A Zebra Fundraiser 7th Annual Western Mass Golf Open Andrea Pittini – Life, Vasculitis Awareness ... Day on Saturday, October 25, 2014 at Toronto Western Hospital, Toronto, Ontario, Canada. The one-day symposium ...

7

Hypocomplementemic Urticarial Vasculitis Syndrome  

PubMed Central

Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case. PMID:22328958

Christensen, Jim; McCarty, Morgan

2012-01-01

8

Headaches and vasculitis.  

PubMed

Vasculitis is a spectrum of clinicopathologic disorders defined by inflammation of arteries of veins of varying caliber with variable tissue injury. Headache may be an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. A suspicion of vasculitis based on the history, clinical examination, or laboratory studies warrants prompt evaluation and treatment to forestall progression and avert cerebral ischemia or infarction. PMID:24703534

Younger, David S

2014-05-01

9

Autoimmune testicular vasculitis.  

PubMed

A 38-year-old man presented for evaluation of new-onset left testicular pain with swelling. Testicular ultrasound revealed multiple intraparenchymal hypoechoic areas in the inferior portion of the left testicle. The patient was treated with a radical inguinal orchiectomy. Pathologic evaluation revealed intratesticular vasculitis, with involvement of medium-sized arteries. This appears to be a rare case of isolated testicular vasculitis, in the absence of any systemic symptoms. PMID:12736037

Raj, Ganesh V; Ellington, Kenneth S; Polascik, Thomas J

2003-05-01

10

Vasculitis in childhood.  

PubMed

Inadequate understanding of the pathogenesis and etiology of vascular inflammation continues to hinder progress in the diagnosis and treatment of pediatric vasculitis. The greatest amount of work is being done in the most common vasculitides of childhood, including Kawasaki disease and Henoch-Schönlein purpura. Discussion of rarer types of vasculitis, on the other hand, such as antineutrophil cytoplasmic antibody-positive small vessel diseases, is largely restricted to case reports. Most aspects of the care of children with Wegener granulomatosis and microscopic polyangiitis are derived by extrapolating from data about adults. Virtually no data are available concerning ways in which these diseases may be different in children. PMID:11604599

Yalcindag, A; Sundel, R

2001-09-01

11

[Symptomatic or secondary allergic vasculitis. Pathogenesis of cutaneous vasculitis].  

PubMed

The histopathologic alterations of the allergic cutaneous vasculitis (fibrinoid necrosis of the walls of the small blood vessels with infiltration of neuthrophils in Karyorrexis) is seen in several entities. It is possible to divide such entities in two groups: a) Primitive allergic vasculitis in which the vascular lesion is dominant and. b) Secondary allergic vasculitis in which the vascular alterations, not constant, are integrants of variable histopathologic conditions. Many classifications have deen done on clinical and pathological bases. Such classifications are not satisfactory. A classification in base of physiopathologic and immunologic mechanisms could permit adequate therapeutic planning. In this paper we consider the secondary vasculitis only. The secondary angitis are seen in: 1) Difuse colagen diseases. 3) Conditions related to infections or inflamatory processes. 3) Angitis produced by drugs. 4) Conditions produced by insects or parasites bites. 5) Heterogeneous group that include diferent tipes of not necrotizing sistemic angitis in uhich, some times, trombosant and necrotizing processes are associated. PMID:1240560

Corrales Padilla, H

1975-01-01

12

Discontinuation of therapies in vasculitis.  

PubMed

For most patients with vasculitis, treatment will result in prevention of mortality and also lead to clinical remission. This increased survival is of course most welcome, but the burden of surviving an episode of acute vasculitis consists of the effects of the disease as well as the adverse events from treatment. Therefore, we have begun to explore the possibility of withdrawing treatment in order to avoid long-term medication toxicities. Whilst this will reduce short-term side effects, if withdrawal leads to subsequent uncontrolled flares of disease, the need for additional therapy may outweigh any benefit from a drug-free holiday. For very mild forms of vasculitis, such as isolated skin vasculitis, the best option may be to avoid treatment altogether. In those patients with vasculitis secondary to an identifiable agent such as drug toxicity or an infectious organism, discontinuing the offending drug or treating the infection will usually resolve or cure the vasculitis. In patients with localised vasculitis, surgical removal of the affected area can be curative. Other forms of vasculitis have a self-limited duration, after which there does not appear to be any clinical evidence of disease, such as is the case for the majority of patients with giant cell arteritis. By contrast, in many forms of vasculitis, especially those associated with the presence of anti-neutrophil cytoplasm antibody (ANCA), relapse occurs in at least half the patients. Where glucocorticoid therapy is used for any length of time, in doses of >5 mg/day, side effects are almost universal. Adding a concomitant agent in the attempt to shorten the course and/or reduce the dose of glucocorticoid treatment may be effective, but can also result in toxicity from the alternative agent, and leaves the patient on immunosuppressive therapy. More toxic therapy, such as cyclophosphamide, usually is administered only for a limited time or cumulative amount, in order to achieve induction of remission or flare in severe disease. The advent of targeted biologic therapy offers the opportunity to provide more effective, less toxic and perhaps more long-lasting control of disease. Rituximab in small-vessel vasculitis can result in long-lasting control of disease, for 18 months or more, from a single course of treatment. Suppression of the interleukin-6 pathway may be effective in large-vessel vasculitis. Unfortunately, none of these therapies is capable of 'cure' for the majority of patients. Therefore, discontinuation of therapy remains unachievable for most patients with vasculitis, at least in the first few years of disease. Short courses of intensive, aggressive therapy are followed by the use of maintenance treatment. Long-term follow-up studies are required to determine the potential benefit of early, more effective control of vasculitis. PMID:24129146

Luqmani, Raashid A

2013-01-01

13

Cerebral vasculitis associated with cocaine abuse  

SciTech Connect

A case of cerebral vasculitis in a previously healthy 22-year-old man with a history of cocaine abuse is described. Cerebral angiograms showed evidence of vasculitis. A search for possible causes other than cocaine produced no results. The authors include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis.

Kaye, B.R.; Fainstat, M.

1987-10-16

14

Cerebral vasculitis in relapsing polychondritis.  

PubMed

Recurrent inflammation of cartilage in multiple sites is a hallmark of relapsing polychondritis (RP). Neurologic complications of this disease have begun to attract increasing attention, but the neuropathologic basis of these complications has not been described. We report a patient with RP whose autopsy showed extensive cerebral and systemic vasculitis. PMID:3336448

Stewart, S S; Ashizawa, T; Dudley, A W; Goldberg, J W; Lidsky, M D

1988-01-01

15

Silica exposure and systemic vasculitis.  

PubMed Central

Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis. PMID:14644669

Mulloy, Karen B

2003-01-01

16

Medium-size-vessel vasculitis  

PubMed Central

Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis. PMID:19946711

Eleftheriou, Despina; Brogan, Paul A.

2009-01-01

17

Chlamydophila pneumoniae Infection Induced Nodular Vasculitis  

PubMed Central

We present the case history of a 48-year-old male patient with Chlamydophila (Chlamydia) pneumoniae who developed a nodular vasculitis. He developed a cutaneous vasculitis with the onset of respiratory symptoms. The diagnosis of Chlamydophila pneumoniae infection was based on serology. Since this infection is very common in our population, although often asymptomatic, it should be systematically considered as a causative agent of nodular vasculitis. PMID:22220148

Sakuma, H.; Niiyama, S.; Amoh, Y.; Katsuoka, K.

2011-01-01

18

Gallbladder vasculitis associated with type-1 cryoglobulinemia.  

PubMed

A patient with type I cryoglobulinemia and monoclonal gammopathy of uncertain significance was found to have acute gallbladder vasculitis. The most prominent manifestation was upper abdominal pain in the setting of normal liver tests. An abdominal ultrasound demonstrated a thickened gallbladder wall, along with gallstones. HIDA scanning showed a nonfunctioning gallbladder with an edematous and thickened wall. There was characteristic leukocytoclastic vasculitis affecting the gallbladder. The patient recovered uneventfully subsequent to cholecystectomy. Gallbladder vasculitis should be considered in patients with unexplained upper abdominal pain and systemic vasculitis. PMID:11281178

Rajvanshi, P; Atac, B S; Seno, R; Gupta, S

2001-02-01

19

Educational Needs of Patients With Systemic Vasculitis  

ClinicalTrials.gov

Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

2014-07-11

20

Recent aspects of vasculitis and future direction.  

PubMed

Vasculitis is pathologically identified as specific cellular inflammation, vessel destruction, and tissue necrosis. Current classifications of vasculitis such as the Chapel Hill Classification (CHCC) and American College of Rheumatology (ACR) guidelines are not sufficiently adequate for clinicians to diagnose vasculitis. The biomarkers that are currently in clinical use such as PR3-ANCA and MPO-ANCA, only help in diagnosing small vessel vasculitis and their sensitivity and specificity are not sufficient. However, recent developments related to the pathogenesis and etiopathogenesis of vasculitis have the potential to contribute to new and improved biomarkers. The determination of diverse roles of ANCA and synergistic effects of infection, genetic, environmental factors and drugs on pathogenesis is quite important. The demonstration of a new autoantibody directed to hLAMP-2 and the resemblance to some microbial structures, in addition to the determination of the possible roles of hepatitis B and C on vasculitis are important findings. These hints may lead to new biomarker developments, providing a better method to diagnose vasculitis. The evidence on T cell immunity as circulatory and lesional will likely contribute to the development of new drugs for vasculitis. PMID:21921363

Aras, Gülfidan

2011-01-01

21

Urticarial Reactions: Vascular Erythema, Urticaria, Vasculitis  

PubMed Central

Vascular erythemas, including urticaria and vasculitis, represent diagnostic and therapeutic challenges. A careful systemic approach to history and physical examination should be followed by appropriate investigations to rule out systemic disease. Chronic urticaria patients should be physically tested to identify cholinergic, dermagraphic, and cold-induced responses. Food diaries and careful drug history may be important to identify exacerbating factors in chronic urticaria. A skin biopsy is necessary to diagnose vasculitis. Therapy of any underlying cause is supplemented with H1 antihistamines in urticaria patients, while vasculitis requires a different anti-inflammatory approach. PMID:21263956

Sibbald, R. Gary

1987-01-01

22

Hypersensitivity Vasculitis with Leukocytoclastic Vasculitis Associated with Alpha-1-Proteinase Inhibitor  

PubMed Central

Prolastin is a commercially available form of alpha-1-antitrypsin (AAT) that is derived from pooled human plasma and used for treatment of severe alpha-1-antitrypsin deficiency (AATD). We describe a patient with AATD who developed presumed hypersensitivity vasculitis (HV) following a Prolastin infusion. Hypersensitivity vasculitis (HV), or cutaneous vasculitis, is characterized by inflammation of the small vessels of the skin with resultant ischemia to the distally supplied areas. To our knowledge, this is the first reported case of presumed hypersensitivity vasculitis following Prolastin infusion. PMID:20204065

Mwirigi, Nicola W.; Thomas, Charles F.

2009-01-01

23

Hypersensitivity vasculitis with leukocytoclastic vasculitis associated with alpha-1-proteinase inhibitor.  

PubMed

Prolastin is a commercially available form of alpha-1-antitrypsin (AAT) that is derived from pooled human plasma and used for treatment of severe alpha-1-antitrypsin deficiency (AATD). We describe a patient with AATD who developed presumed hypersensitivity vasculitis (HV) following a Prolastin infusion. Hypersensitivity vasculitis (HV), or cutaneous vasculitis, is characterized by inflammation of the small vessels of the skin with resultant ischemia to the distally supplied areas. To our knowledge, this is the first reported case of presumed hypersensitivity vasculitis following Prolastin infusion. PMID:20204065

Mwirigi, Nicola W; Thomas, Charles F

2009-01-01

24

[Vasculitis--interdisciplinary diagnosis: radiology].  

PubMed

Determination of disease extension and disease activity are in the foreground of diagnostic imaging in vasculitides. There are several radiologic modalities available each having specific indications. Magnetic resonance imaging (MRI) readily depicts granulomas and mucosal inflammations in the paranasal sinuses, nasal cavity and orbits. Computed tomography detects osseous lesions of the skull. Due to its superb sensitivity MRI is an established screening modality for CNS vasculitides, although there are limitations with regard to specificity. In spite of its limited accuracy in most institutions angiography is still required for radiological confirmation of CNS vasculitis. Perfusion and diffusion MR-imaging may combine the advantages of 'conventional' MRI and angiography. By now the method is not fully validated for vasculitides, however. Vascular disease in Takayasu's arteritis and in giant cell arteritis involving predominantly large and medium sized vessels is readily diagnosed by non invasive magnetic resonance angiography. Percutaneous transluminal angioplasty has proven to be an effective and save therapeutic modality for the cure of vascular stenoses and occlusions. Plain film radiography in two planes is the established modality for pulmonary imaging. In pulmonary vasculitides a more thorough analysis of lung disease is provided by high resolution computed tomography. Diagnostic imaging does substantially assist in the interdisciplinary management of patients suffering from vasculitides. PMID:11584721

Reuter, M; Biederer, J; Müller-Hülsbeck, S; Heller, M

2001-08-01

25

Prognosis and ICU outcome of systemic vasculitis  

PubMed Central

Background Systemic vasculitis may cause life threatening complications requiring admission to an intensive care unit (ICU). The aim of this study was to evaluate outcomes of systemic vasculitis patients admitted to the ICU and to identify prognosis factors. Methods During a ten-year period, records of 31 adult patients with systemic vasculitis admitted to ICUs (median age: 63 y.o, sex ratio M/F: 21/10, SAPS II: 40) were reviewed including clinical and biological parameters, use of mechanical ventilation, catecholamine or/and dialysis support. Mortality was assessed and data were analyzed to identify predictive factors of outcome. Results Causes of ICU admissions were active manifestation of vasculitis (n?=?19), septic shock (n?=?8) and miscellaneous (n?=?4). Sixteen patients (52%) died in ICU. By univariate analysis, mortality was associated with higher SOFA (p?=?0.006) and SAPS II (p?=?0.004) scores. The need for a catecholamine support or/and a renal replacement therapy, and the occurrence of an ARDS significantly worsen the prognosis. By multivariate analysis, only SAPS II (Odd ratio: 1.16, 95% CI [1.01; 1.33]) and BVAS scores (Odd ratio: 1.16, 95% CI?=?[1.01; 1.34]) were predictive of mortality. Conclusion The mortality rate of severe vasculitis requiring an admission to ICU was high. High levels of SAPS II and BVAS scores at admission were predictive of mortality. PMID:24083831

2013-01-01

26

Reproductive Health in Men and Women With Vasculitis  

ClinicalTrials.gov

Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

2014-06-25

27

Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis  

PubMed Central

Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV. PMID:21507463

Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.

2011-01-01

28

Pathogenesis of ANCA-Associated Vasculitis  

PubMed Central

Antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) are a group of systemic vasculitis characterized by inflammation and necrosis of blood vessel walls. Genetic, epigenetic and environmental factors contribute to the etiology and pathogenesis of AAV. Based on currently available clinical and experimental evidence, it is reasonable to conceptualize that in predisposed patients, different triggers can lead to the production of autoantibodies (ANCA) that in the context of an inflammatory environment can cause tissue inflammation and vascular injury. Several different pathways and mechanisms in the pathogenesis of AAV are described in this contemporary review. PMID:20688244

Cartin-Ceba, Rodrigo; Peikert, Tobias; Specks, Ulrich

2012-01-01

29

Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation  

PubMed Central

Propylthiouracil (PTU) is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA) in few patients with Graves’ disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves’ disease. PMID:23776917

Ayturk, Semra; Demir, Mustafa Volkan; Yaylac?, Selcuk; Tamer, Ali

2013-01-01

30

Idiopathic granulomatous vasculitis: response to immunosuppressive therapy.  

PubMed Central

A case of idiopathic granulomatous vasculitis (disseminated visceral giant cell arteritis) is described in an old woman, the seventh case of this rare disorder reported to date. The main organ affected was the liver and, to our knowledge, this is the first patient to be diagnosed while still alive and the only case to have received medical treatment. It is also the first time that muscular involvement has been documented in this condition. Cyclophosphamide treatment resulted in disappearance of symptoms and increase in weight. The patient died of an unrelated condition. Images PMID:7665707

Alguacil-Garcia, G F; Moreno-Requena, J; Martinez-Albadalejo, M; Hallal-Hachem, H; Gonzalez-Pina, B; de Paco-Moya, M

1995-01-01

31

Leukocytoclastic vasculitis caused by drug additives.  

PubMed

Chronic cutaneous small vessel (leukocytoclastic) vasculitis (LCV) is a process believed to be related to the presence of circulating immune complexes. The most frequent causes and associated disorders are medications, infections, collagen vascular disorders, paraproteinemias, and, rarely, neoplasia. Reports of food or food additives as a causative factor for LCV have appeared but are rare. We report a patient with chronic cutaneous LCV in whom the presumed cause was an excipient (a dye) used in the capsule form of lithium carbonate. Furthermore, ingestion of foods containing dyes results in a disease flare in our patient. PMID:8169261

Lowry, M D; Hudson, C F; Callen, J P

1994-05-01

32

The impact of vasculitis on patients' social participation and friendships  

PubMed Central

Objectives Our objective is to explore how vasculitis, affects patients’ friendships and social participation. Methods Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants’ written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participant’s responses, the coders determined how themes were interrelated across participants. Results Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At limes, this negative impact was related to structural changes in patients’ social networks due to loss of friendships. Reduced social participation was also associated with friends’ inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. Conclusion The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients’ ability to engage in dialogue about their illness with their friends. PMID:22325346

Carpenter, Delesha M.; Meador, Amy E.; Elstad, Emily A.; Hogan, Susan L.; DeVellis, Robert F.

2013-01-01

33

Cerebral large vessel vasculitis in systemic lupus erythematosus.  

PubMed

Neuropsychiatric systemic lupus erythematosus (NPSLE) is defined by involvement of the central nervous system in systemic lupus erythematosus (SLE), with a wide range of both neurological and psychiatric manifestations. Although its aetiopathogenesis is not fully elucidated, NPSLE seems to be a consequence of cerebral vascular pathology including thromboembolism, small-vessel vasculopathy and, in rare cases, true vasculitis. Cerebral vasculitis is rare, and cerebral large-vessel vasculitis in SLE is even more unusual. We report the case of a female patient with the diagnosis of SLE. She presented with stroke-like symptoms, headache and vertigo, and palpable purpura on her legs. Further investigations revealed that she suffered from both vasculitis of the cerebral large vessels and coexisting cutaneous small-vessel vasculitis. PMID:24969082

Böckle, B C; Jara, D; Aichhorn, K; Junker, D; Berger, T; Ratzinger, G; Sepp, N T

2014-11-01

34

Cryoglobulinemic vasculitis in a patient with CREST syndrome.  

PubMed

Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. PMID:24852904

Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D

2014-10-01

35

[Vasculitis : New nomenclature of the Chapel Hill consensus conference 2012].  

PubMed

Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis. PMID:25269877

Holl-Ulrich, K

2014-11-01

36

Optic disc vasculitis in epidemic dropsy.  

PubMed

During an outbreak of epidemic dropsy in Delhi, 233 patients were studied. Retinal changes including venous dilatation and tortuosity, haemorrhages and disc oedema were observed. A clinical picture compatible with type I optic disc vasculitis was seen in 13 eyes and that of type II in 3 eyes. Fluorescein angiography was carried out in 23 randomly selected cases. Relevant angiographic findings included dilated and tortuous retinal veins, prominent vascular staining, blocked fluorescence, microaneurysms, disc oedema and peripapillary dye spillage. Presence of positive angiographic findings correlated well with the severity of the systemic disease, glaucoma, however, revealed no correlation. Papillophlebitis, a new ocular manifestation of Argemone mexicana oil toxicity, as also the fluorescein angiographic picture in epidemic dropsy is being reported for the first time in the literature. PMID:3430862

Sachdev, M S; Sood, N N; Mohan, M; Sachdev, H P; Gupta, S K

1987-01-01

37

Livedo vasculitis associated with regional cytophagic histiocytoses.  

PubMed

We report a 70-year-old woman who developed recurrent multiple ulcers and residual atrophic scars on her legs and feet. The histology showed fibrinoid material in the dermal vessel walls causing partial to complete occlusion of the lumina. Capillary tufting and dilatation were also noted in the dermis. There was no vasculitis or panniculitis. Extravasated red blood cells were seen throughout the dermis; these were engulfed by benign-appearing histiocytes around the dermal-fat junction. As she was generally well and there was no evidence of the presence of cytophagic cells in her extracutaneous organs, she was diagnosed with regional phagocytic histiocytoses supposedly triggered by extravasated red blood cells. PMID:10659504

Kuno, Y; Numata, T; Tsuji, T

1999-12-01

38

Rituximab induces remission in refractory HCV associated cryoglobulinaemic vasculitis  

PubMed Central

Case report: The patient was a 45 year old woman with HCV associated cryoglobulinaemic vasculitis, with purpura, arthralgia, constitutional symptoms, and a polyneuropathy. A malignant NHL was found as underlying lymphoproliferative disease. At this stage the disease was refractory to interferon ?2b and ribavirin and to subsequent immunosuppressive treatment with cyclophosphamide. Six rituximab infusions targeting the CD20 antigen on cells of the B cell lineage induced remission of the vasculitis. Bone marrow biopsy disclosed absence of the NHL. Remission has subsequently been maintained and HCV eliminated with the new pegylated interferon ?2b and ribavirin for nearly one year. Conclusions: Transition of the underlying "benign" lymphoproliferative disease to a malignant lymphoma may result in difficult to treat HCV associated cryoglobulinaemic vasculitis. Rituximab offers a new possibility for inducing remission in refractory HCV associated cryoglobulinaemic vasculitis and the lymphoproliferative disorder. After remission, HCV may subsequently be eliminated with pegylated interferon ?2b and ribavirin. PMID:14644867

Lamprecht, P; Lerin-Lozano, C; Merz, H; Dennin, R; Gause, A; Voswinkel, J; Peters, S; Gutzeit, O; Arlt, A; Solbach, W; Gross, W

2003-01-01

39

Pulmonary vasculitis as the first manifestation of rheumatoid arthritis  

PubMed Central

In this report, we describe a 61-year-old man that presented with isolated pulmonary vasculitis and a positive anti-cyclic citrullinated peptide (CCP) antibody. Within a few months, the patient developed the symmetric polyarthritis consistent with rheumatoid arthritis (RA). Because the anti-CCP antibody is highly specific for RA and vasculitis is a known association of RA, we suspect the pulmonary vasculitis in this patient was the first manifestation of underlying RA. This case extends on previous reports that have shown that lung disease may predate the development of articular RA and that anti-CCP positivity and lung disease may represent a pre-RA phenotype. To our knowledge, this is the first case report of pulmonary vasculitis as the first manifestation of RA.

Tourin, Olga; de la Torre Carazo, Salvador; Smith, Daniel R.; Fischer, Aryeh

2013-01-01

40

Vasculitis Syndromes of the Central and Peripheral Nervous Systems  

MedlinePLUS

... blurred vision, or blindness seizures, convulsions stroke or transient ischemic attack (TIA, sometimes also called a “mini- ... supports The Vasculitis Clinical Research Consortium (VCRC), a network of academic medical centers, patient support organizations, and ...

41

[Vasculitis with cutaneous necrosis induced by oral contraceptive].  

PubMed

Multiple ulcerated and necrotic lesions developed in a 29-year-old woman. Response to treatment during the next few months was minimal. The most plausible explanation was drug-induced vasculitis caused by contraceptive pills (Microgynon: levonorgestrel 0.15 mg and ethinyl oestradiol 0.03 mg). The diagnosis of vasculitis was confirmed by histopathological and immunofluorescent studies. Urticarial manifestations had developed into necrotizing inflammation and the lesions only began to heal after discontinuation of the pills. Contraceptive pills are not included among the drugs causing necrotizing vasculitis, and to the best of our knowledge, this is the first report. Although contraceptive pills are commonly implicated in the induction of erythema nodusom or vasculitis, such a severe reaction with necrosis is rare. PMID:1885102

Mosovich, B; Biton, A; Avinoach, I

1991-04-15

42

Anti-TNF-Alpha Therapy and Systemic Vasculitis  

PubMed Central

TNF-? is a pleiotropic cytokine, which plays a major role in the pathogenesis of numerous autoimmune and/or inflammatory systemic diseases. Systemic vasculitis constitutes a group of rare diseases, characterized by inflammation of the arterial or venous vessel wall, causing stenosis and thrombosis. Treatment of the different type of vasculitis mainly relies on steroids and immunosuppressive drugs. In case of refractory or relapsing diseases, however, a second line of treatment may be required. Anti-TNF-? drugs have been used in this setting during the last 15 years with inconsistent results. We reviewed herein the use of anti-TNF-? therapy in different kind of vasculitis and concluded that, except for Behcet's disease, this therapeutic option has not demonstrated significant improvement in the treatment of vasculitis. PMID:24719524

Kaplanski, Gilles

2014-01-01

43

Chronic Periaortitis (Retroperitoneal Fibrosis) Concurrent with Recurrent Cutaneous Eosinophilic Vasculitis  

PubMed Central

Chronic periaortitis (CP) is usually accompanied by at least mild manifestations of systemic autoimmunity; however, skin manifestations are rare. Here, we report an 82-year-old woman presenting with a pruritic annular eosinophilic dermatosis that led to the diagnosis of recurrent cutaneous eosinophilic vasculitis (RCEV) coexisting with a latent CP. The present paper is reminder that a CP should be included as a potential differential diagnosis in the elaboration of patients with cutaneous vasculitis that is suspicious of underlying autoimmunity. PMID:23198176

Kiorpelidou, Despoina; Gaitanis, Georgios; Zioga, Aikaterini; Tsili, Athina C.; Bassukas, Ioannis D.

2011-01-01

44

Pulmonary Pathology: LC22-1 SEVERAL TOPICS OF PULMONARY VASCULITIS AND PULMONARY HEMORRHAGE SYNDROMES.  

PubMed

Vasculitis is defined as inflammation of a vessel wall. Hemorrhage is defined as the escape of blood from vessels. If pulmonary vasculitis and pulmonary hemorrhage are directly connected that inflammation of blood vessels leads to rupture and then hemorrhage, the classification of the pulmonary vasculitis and pulmonary hemorrhage syndromes would be simple. There are many schemes for classifying vasculitis, such as McCluskey 1983, Jennette 1997, Vuong 2002, and Saleh 2005. Classifications can be made by etiology, histologic reaction, size and type of vessels, and associated diseases. Recently CHCC1994 is revised to CHCC 2012. Vasculitis and mimics of pulmonary vasculitis includes idiopathic necrotizing vasculitis syndromes, miscellaneous systemic disorders including collagen-vascular diseases, pulmonary hemorrhage syndromes, vascular involvement in lymphoproliferative disorders, pulmonary hypertensive arterial changes, and granulomatous vasculitis. My presentation reviews all over and focuses on pathological features of pulmonary vasculitis and pulmonary hemorrhage syndromes. PMID:25188158

Matsubara, Osamu

2014-10-01

45

Complement in ANCA-Associated Vasculitis  

PubMed Central

Antineutrophil cytoplasmic autoantibodies (ANCA) are the likely cause for necrotizing small vessel vasculitis and crescentic glomerulonephritis. Unlike other forms of crescentic glomerulonephritis induced by immune complexes or anti-glomerular basement membrane (anti-GBM) antibodies that have conspicuous vessel wall immunoglobulin and complement, there is a paucity, although usually not an absence, of vessel wall immunoglobulin and complement in ANCA-associated glomerulonephritis. In spite of this comparatively lower level and more localized distribution of vessel wall complement, experimental and clinical observations strongly incriminate alternative complement pathway activation as critically important in the pathogenesis of ANCA disease. Experimental data in animal models and in vitro experiments demonstrate that primed neutrophils are activated by ANCA, which generates C5a that engages C5a receptors on neutrophils. This attracts and in turn primes more neutrophils for activation by ANCA. In patients with ANCA disease, plasma levels of C3a, C5a, soluble C5b-9 and Bb have been reported to be higher in active disease than in remission, whereas no difference was reported in plasma C4d in active versus remission ANCA disease. Thus, experimental and clinical data support the hypothesis that ANCA-induced neutrophil activation activates the alternative complement pathway and generates C5a. C5a not only recruits additional neutrophils through chemotaxis but also primes neutrophils for activation by ANCA. This creates a self-fueling inflammatory amplification loop that results in the extremely destructive necrotizing vascular injury. PMID:24161040

Jennette, J Charles; Xiao, Hong; Hu, Peiqi

2014-01-01

46

CNS Vasculitis in Autoimmune Disease: MR Imaging Findings and Correlation with Angiography  

Microsoft Academic Search

BACKGROUND AND PURPOSE: MR findings in CNS vasculitis and their correlation with angiography have not been clearly defined. We therefore explored three hypotheses regarding CNS vasculitis associated with autoimmune disease: 1) MR imaging is highly sensitive; 2) a typical MR appearance exists; and, 3) MR and angiographic findings correlate well. METHODS: We studied 18 patients with CNS vasculitis associated with

Martin G. Pomper; Timothy J. Miller; John H. Stone; William C. Tidmore; David B. Hellmann

1999-01-01

47

Leukocytoclastic vasculitis and acute allergic interstitial nephritis following ceftriaxone exposure  

PubMed Central

Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis is a small vessel inflammatory disease which mainly involves the postcapillary venules. A 17-year-old girl developed palpable purpura over lower limbs and acute allergic interstitial nephritis 5 days after exposure to ceftriaxone. Skin biopsy from the lesion over lower limbs confirmed the diagnosis of LCV. Discontinuation of drug and treatment with steroid resulted in resolution of skin lesions and normalization of kidney functions. Beta-lactams are commonly used antibiotics in various types of infection in day-to-day practice. LCV, a rare complication of ceftriaxone should be kept in mind while using this drug.

Agrawal, Sachin R.; Rajput, Atul; Jain, A. P.

2014-01-01

48

Secondary Syphilis with Nodular Vasculitis Mimicking Beh?et's Disease  

PubMed Central

Although, erythema nodosum is a common skin manifestation associated with syphilis, nodular vasculitis is a rare feature. Here, we describe a case of a 22-year-old, human immunedeficiency virus negative, non-immunocompromised man who developed recurrent oral and scrotal ulcers with nodular lesions of the lower extremitie. Behçet's disease was initially suspected, however, his serologic test for syphilis was positive, and he was thus diagnosed with secondary syphilis, with a skin biopsy showing nodular vasculitis. The patient was treated with benzathine penicillin, and the skin lesions disappeared after treatment. PMID:24475361

Jo, Jaemin; Kim, Jae Wang; Kim, Jinseok; Yu, Jung Re

2013-01-01

49

Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis  

PubMed Central

BACKGROUND/AIMS—Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathophysiology of the disease, and to propose a treatment strategy.?METHODS—A total of 220 French gastroenterology departments were contacted to review patients with unexplained small bowel strictures. Of 17 responses, 12 corresponded to a diagnosis of CMUSE. These patients were hospitalised between 1965 and 1993 and their medical records were reviewed.?RESULTS—All patients (mean age 42.1 (4.4) years) had intestinal and five had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient had heterozygous type I C2 deficiency (28 base pair gene deletion). Two to 25 (mean 8.3 (1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils.?CONCLUSIONS—CMUSE is an independent entity characterised by steroid sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.???Keywords: cryptogenetic multifocal ulcerous stenosing enteritis; vasculitis; small intestine PMID:11171822

Perlemuter, G; Guillevin, L; Legman, P; Weiss, L; Couturier, D; Chaussade, S

2001-01-01

50

Progress in treatment of ANCA-associated vasculitis  

PubMed Central

Autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis (AAV) is characterised by inflammation of blood vessels. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide transformed AAV from a fatal condition to a largely treatable condition. Over the past 30 years, considerable progress has been made refining immunosuppressive regimens with a focus on minimising toxicity. There is, however, a high unmet need in the treatment of AAV. A proportion of patients are refractory to current therapies; 50% experience a relapse within 5 years and treatment toxicity contributes to mortality and chronic disability. As knowledge of the pathogenesis of vasculitis grows, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of vasculitis. Lymphocyte-targeted and cytokine-targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes. As rare diseases, treatment advances in vasculitis depend on international collaborative research networks both to establish an evidence base for newer agents and to develop recommendations for patient management. PMID:22569190

2012-01-01

51

Vasculitis as the basis of cutaneous lesions in Reiter's disease  

Microsoft Academic Search

The cutaneous lesions of Reiter's disease (RD) and pustular psoriasis (PP) are said to be histologically similar and often indistinguishable. We encountered three cases of RD in which biopsy specimens of lesions clinically compatible with keratoderma blenorrhagicum showed a pustular psoriasiform tissue reaction in conjunction with a subjacent superficial leukocytoclastic vasculitis (LCV). In an attempt to ascertain if these changes

C. M Magro; A. N Crowson; R Peeling

1995-01-01

52

Assessment of damage in vasculitis: expert ratings of damage  

PubMed Central

Objectives. Current measures of damage in vasculitis do not account for the possibility that some forms of damage may exert greater impact than others. As part of an international effort to revise how damage is quantified in vasculitis clinical research, an exercise was performed to measure expert ratings of damage items. Methods. Members of the Vasculitis Clinical Research Consortium and European Vasculitis Study Group were given a list of 129 items of damage related to WG and microscopic polyangiitis (MPA). Participants were asked to rate each item of damage on an integer scale from 0 to 10, where 10 represented the most severe form of damage and 0 indicated ‘no impact’. Results. A multidisciplinary panel of 50 investigators from North America, Europe and Australia–New Zealand participated. The highest median ratings (8–10) were assigned to items of damage associated with malignancy, tissue ischaemia, the central nervous system and cardiopulmonary manifestations. The mean scores ranged from 1.3 to 9.5. The highest s.d.s (?2.5) were associated with forms of damage that may benefit from surgical intervention or may not be causally associated with WG or MPA. Lower scores were assigned by nephrologists in comparison with rheumatologists and by Americans in comparison to Europeans, although the difference in median ranks used by these groups was not statistically significant (P > 0.05 for the comparisons). Conclusions. This exercise represents an important step in the development of a weighting system that may increase the utility of damage index scores for the assessment of patients with vasculitis. PMID:19454608

Jayne, David; Luqmani, Raashid; Merkel, Peter A.

2009-01-01

53

Paraneoplastic leukocytoclastic vasculitis as an initial presentation of malignant pleural mesothelioma: a case report  

PubMed Central

Introduction Vasculitis has been associated with malignancies, more commonly hematological rather than solid malignancies. Due to the rarity of these conditions and the lack of a temporal association, the relationship between vasculitis and malignancy remains unclear. Paraneoplastic vasculitis as a phenomenon of lung cancer has been described in the literature. To the best of our knowledge, this is the first case report of leukocytoclastic vasculitis being an initial presentation of malignant pleural mesothelioma. Case presentation We report the case of an 84-year old Greek man who presented to our facility with an erythematous, pruritic and purpuric rash affecting his limbs. This was biopsy-proven to be leukocytoclastic vasculitis and treated conservatively with topical corticosteroids as well as oral prednisolone, with good results. Six months later, he was diagnosed as having malignant pleural mesothelioma. As he remained asymptomatic from his malignancy, no systemic chemotherapy was instituted. He had a recurrence of biopsy-proven leukocytoclastic vasculitis two months after he was diagnosed as having mesothelioma, which again settled with conservative measures. Conclusions It is important to remain vigilant with regard to the association between leukocytoclastic vasculitis and malignancies. A diagnosis of vasculitis requires a search for malignancies as well as other possible etiologies. This is particularly of relevance when the vasculitis becomes chronic, recurrent or treatment is no longer effective. Should our patient have experienced refractory vasculitis, we would have instituted systemic chemotherapy to treat the underlying malignancy. PMID:22937937

2012-01-01

54

Sudden death due to isolated segmentary coronary vasculitis.  

PubMed

Coronary vasculitis is a group of conditions occurring either independently or associated with another diseases characterized by an inflammation of the blood vessel's wall and subsequent fibrinoid necrosis, occlusion, stenosis, or aneurismal dilatations. Coronary vasculitis leading to sudden cardiac death has rarely been described in the scientific literature.We present the case of an 18-year-old football player who collapsed when playing football. The patient remained in a deep coma (glasgow coma scale = 3) in the hospital for another 4 hours before dying. During hospitalization, he was diagnosed with acute anterolateral myocardial infarction with ST elevation. An autopsy was performed the next day, and on the coronary vessels were identified a dilatation with luminal extension, which, based on clinical and pathological criteria, was considered to be an isolated, coronary polyarteritis nodosa. PMID:25361059

Dermengiu, Dan; Hostiuc, Sorin; Cristian Curca, George; Constantin Rusu, Mugurel; Paparau, Cristian; Ceausu, Mihai

2014-12-01

55

New pathophysiological insights and treatment of ANCA-associated vasculitis  

Microsoft Academic Search

ANCA-associated-vasculitis (AAV) comprises three different diseases entities: Churg–Strauss syndrome, microscopic polyangiitis, and Wegener's granulomatosis. AAV is an autoimmune disease with complex pathophysiology. Anti-neutrophil cytoplasmic antibodies (ANCAs) with specificity for proteinase-3 (PR3) or myeloperoxidase (MPO) are hallmarks of AAV and have a pivotal role in disease development. In addition to ANCA, the cellular immune system contributes to the pathogenesis of the

Benjamin Wilde; Pieter van Paassen; Oliver Witzke; Jan Willem Cohen Tervaert

2011-01-01

56

ANCA-associated vasculitis with renal involvement: an outcome analysis  

Microsoft Academic Search

Background. The anti-neutrophil cytoplasmic anti- body (ANCA)-associated vasculitides are a group of heterogeneous diseases. This study was undertaken to investigate the outcome of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and renal- limited vasculitis (RLV). Furthermore, we analysed the differences in patients with proteinase 3-ANCA (PR3-ANCA) and those with myeloperoxidase-ANCA (MPO-ANCA), which have not been assessed in a homogeneously treated group

Sven Weidner; Steffen Geuss; Syrus Hafezi-Rachti; Andrea Wonka; Harald D. Rupprecht

2004-01-01

57

Cytomegalovirus-related necrotising vasculitis mimicking Henoch-Schönlein syndrome.  

PubMed

Viral vasculitides have been previously reported in the literature, the role of infections in their pathogenesis ranging from direct cause to trigger event. Here we report the case of a 3-year-old immunocompetent girl who developed a systemic vasculitis leading to ileal perforation, mimicking a full blown picture of Henoch-Schönlein purpura. High dosage steroid treatment was started, with good response. The anatomopathological examination of the resected gastrointestinal tract showed features of necrotising vasculitis and cytomegalovirus (CMV)-related inclusion bodies in the endothelial cells, with direct correlation to vascular damage. The causative role of viral infection was revealed by the presence of CMV DNA in patient's blood and positive IgG titer against the virus. Steroid therapy was then tapered: the patient achieved clinical remission, which still persists after a six-months follow-up. Our report suggests that CMV vasculitis is probably more frequent than previously thought, even in immunocompetent patients, with a protean clinical presentation, mimicking other types of vasculitides. PMID:24854375

D'Alessandro, Matteo; Buoncompagni, Antonella; Minoia, Francesca; Coccia, Maria C; Martini, Alberto; Picco, Paolo

2014-01-01

58

Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris  

PubMed Central

We present the case of a 64-year-old woman who has suffered from pustulosis palmaris et plantaris for 10 years. At the first examination, many erythematous lesions with purpura, blood crusts, and blisters were present in the lower legs and dorsum of the feet. Painful swelling in the sternal region and dorsal pain were also noted. Elevation of the CRP and myogenic enzyme levels, and liver and renal dysfunctions were noted on blood testing. Histopathologically, leukocytoclastic vasculitis was noted in small blood vessels in the whole dermal layers, and deposition of IgM and C3 in the vascular wall was detected by the direct immunofluorescence techniques. Based on these findings, cutaneous small vessel vasculitis was diagnosed. Because the patient complained of a toothache during the clinical course, an X-ray examination was performed. On pantomography, a radicular cyst and apical periodontitis were noted. The tooth symptoms changed with exacerbation and remission of the skin symptoms. These findings indicate that odontogenic infection is very likely to be a cause of cutaneous small vessel vasculitis in a manner similar to pustulosis palmaris et plantaris. PMID:22548039

Kosaka, Motoko; Kato, Tokue; Kawana, Seiji

2012-01-01

59

Primary vasculitides and vasculitis confined to skin: clinical features and new pathogenic aspects  

Microsoft Academic Search

\\u000a Abstract Cutaneous vasculitis is a heterogeneous group of disorders, and may occur with virtually all syndromes of vasculitis. It\\u000a can occur as an isolated dermatologic disorder or as a manifestation of a potentially life-threatening systemic vasculitis.\\u000a Cutaneous manifestations vary depending on the underlying cause, the size of the vessel involved and the severity and type\\u000a of inflammation. In this short

Elena Csernok; Wolfgang Ludwig Gross

2000-01-01

60

Leflunomide-induced DRESS syndrome with renal involvement and vasculitis.  

PubMed

DRESS or drug reaction (or rash) with eosinophilia and systemic symptoms belongs to the severe cutaneous adverse reaction group and is characterized by hematological abnormalities and visceral organ involvement. Although most often related with anticonvulsant and sulfonamide use, it is reported with numerous other drugs. We report an unusual case of DRESS syndrome due to Leflunomide, also complicated by renal involvement in the form of granulomatous interstitial nephritis and vasculitis. On a review of the literature, eight similar cases were found, and these are discussed. PMID:23271613

Pinto, Benzeeta; Dhir, Varun; Krishnan, Sabari; Nada, Ritambhra

2013-05-01

61

Leukocytoclastic vasculitis presenting with acute haemorrhagic oedema of the scrotum  

PubMed Central

Fournier’s gangrene is a form of rapidly progressive necrotising fasciitis involving the soft tissues of the male genitalia. The treatment for this urologic emergency is immediate surgical debridement. Misdiagnosis of Fournier’s may lead to unnecessary surgical interventions, making careful recognition of systemic signs of illness crucial. The authors present a case of a 48-year-old patient who presented emergently with massive scrotal oedema, erythema and pain suspicious for Fournier’s gangrene and systemic signs of illness, including palpable purpura and leukocytoclastic vasculitis on biopsy. PMID:22679273

Reyner, Karina; Dahm, Philipp

2011-01-01

62

Kawasaki Disease as a Systemic Vasculitis in Childhood  

PubMed Central

Kawasaki disease is a disease of unknown etiology that most frequently affects infants and children under 5 years of age. Inflammation occurs in medium-sized muscular arteries throughout the body including the coronary artery, being classified as a systemic vasculitis syndrome. Histopathological investigations of Kawasaki disease have mainly focused on the coronary artery because it is directly associated with the cause of death. However, to identify the cause and pathology of Kawasaki disease, it is necessary to investigate lesions of whole organs. Thus, we attempted to review lesions in organs other than the heart and hypotheses of pathogenesis recently attracting attention. PMID:23555407

2010-01-01

63

Treatment of Primary Systemic Vasculitis with the Inosine Monophosphate Dehydrogenase Inhibitor Mycophenolic Acid  

Microsoft Academic Search

Despite advances in the treatment of vasculitis, modern therapies fail to induce or maintain remission in a significant proportion of patients. Mycophenolic acid is increasingly used to treat vasculitis syndromes. Here, we consider relevant pharmacokinetic and pharmacodynamic properties of mycophenolate, with emphasis on the impact of renal impairment, and we review the existing evidence for and current trials of mycophenolate

Thomas F. Hiemstra; Rachel B. Jones; David R. W. Jayne

2010-01-01

64

Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis  

PubMed Central

A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later. PMID:24653851

Kobak, Senol; Yilmaz, Hatice; Karaarslan, Ahmet; Yalcin, Murat

2014-01-01

65

Leukocytoclastic vasculitis in a patient with ankylosing spondylitis.  

PubMed

A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later. PMID:24653851

Kobak, Senol; Yilmaz, Hatice; Karaarslan, Ahmet; Yalcin, Murat

2014-01-01

66

T Cell-Macrophage Interactions and Granuloma Formation in Vasculitis  

PubMed Central

Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage–T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever, and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell–macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy. PMID:25309534

Hilhorst, Marc; Shirai, Tsuyoshi; Berry, Gerald; Goronzy, Jorg J.; Weyand, Cornelia M.

2014-01-01

67

Gemcitabine-induced large vessel vasculitis demonstrated by PET CT: a rare, important side effect.  

PubMed

Gemcitabine is a nucleoside analogue used widely across haemato-oncology. Side effects are generally predictable, and typically consist of cytopenia, nausea, and infection. As the present case clearly demonstrates, gemcitabine is in rare cases associated with life-threatening large vessel vasculitis, which can involve the aorta. It is important to consider gemcitabine-induced vasculitis in non-specifically unwell patients with raised inflammatory markers and fever of unknown origin, with or without signs of vascular compromise. Early recognition, cessation of gemcitabine therapy, and high-dose steroids are critical for a good outcome. PET CT is valuable to diagnose large vessel vasculitis and monitor treatment response. PMID:24584910

Eyre, Toby A; Gooding, Sarah; Patel, Ishita; Moore, Niall; Hatton, Chris; Collins, Graham P

2014-06-01

68

Issues in trial design for ANCA-associated and large-vessel vasculitis.  

PubMed

Randomized clinical trials (RCTs) have informed the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, although challenges still exist. The evidence base for treating large-vessel vasculitis (LVV) is weaker, but initiatives to standardize diagnostic criteria and outcome measures, and to validate biomarkers in LVV, together with newly initiated RCTs should start to address this need. In this Perspectives, we discuss the prerequisites for RCTs in vasculitis, existing trial evidence, continuing unmet needs, potential therapeutic avenues to explore and considerations in the design of future trials. PMID:24798573

Tarzi, Ruth M; Mason, Justin C; Pusey, Charles D

2014-08-01

69

Advances in pathogenesis and treatment of ANCA-associated vasculitis.  

PubMed

Anti-neutrophil cytoplasmic autoantibodies (ANCA) directed to proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA) are sensitive and specific markers for their associated diseases, granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) and microscopic polyangiitis (MPA), respectively. Clinical observations suggest but do not prove that ANCA are involved in the pathogenesis of GPA and MPA. In vivo and in vitro experimental data strongly suggest if not prove that MPO-ANCA underlie the pathological lesions seen in MPO-ANCA associated MPA. This is less clear for PR3-ANCA associated GPA in which, besides small-vessel vasculitis, granulomatous inflammation is apparent. Here, cellular immunity appears to play an additional role. Insight into the pathogenetic events involved in these diseases has resulted in new ways of treatment that target the specific pathways that underlie the development of the lesions. PMID:25336033

Kallenberg, Cees G M

2014-10-01

70

Intravitreal bevacizumab for macular edema due to occlusive vasculitis.  

PubMed

Systemic vasculitides can cause retinal vascular pathology, including cotton-wool spots, retinal hemorrhages, vascular occlusion, and capillary nonperfusion. Two main causes of visual decline include macular edema and retinal neovascularization. Presumably, both of these complications are caused by increased intraocular levels of vascular growth and permeability factors. We report a patient with occlusive retinal vasculitis associated with mixed connective tissue disease who was treated with intravitreal bevacizumab for chronic macular edema. One month after treatment, visual acuity improved from 20/80 to 20/60, and foveal thickness decreased from 543 microns to 306 microns. This effect persisted for at least 3 months after treatment. No complications, including increased retinal ischemia, were observed. PMID:17564933

Margolis, Ron; Lowder, Careen Y; Sears, Jonathan E; Kaiser, Peter K

2007-01-01

71

Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis  

PubMed Central

BACKGROUND Cyclophosphamide and glucocorticoids have been the cornerstone of remission-induction therapy for severe antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis for 40 years. Uncontrolled studies suggest that rituximab is effective and may be safer than a cyclophosphamide-based regimen. METHODS We conducted a multicenter, randomized, double-blind, double-dummy, noninferiority trial of rituximab (375 mg per square meter of body-surface area per week for 4 weeks) as compared with cyclophosphamide (2 mg per kilogram of body weight per day) for remission induction. Glucocorticoids were tapered off; the primary end point was remission of disease without the use of prednisone at 6 months. RESULTS Nine centers enrolled 197 ANCA-positive patients with either Wegener’s granulomatosis or microscopic polyangiitis. Baseline disease activity, organ involvement, and the proportion of patients with relapsing disease were similar in the two treatment groups. Sixty-three patients in the rituximab group (64%) reached the primary end point, as compared with 52 patients in the control group (53%), a result that met the criterion for noninferiority (P<0.001). The rituximab-based regimen was more efficacious than the cyclophosphamide-based regimen for inducing remission of relapsing disease; 34 of 51 patients in the rituximab group (67%) as compared with 21 of 50 patients in the control group (42%) reached the primary end point (P = 0.01). Rituximab was also as effective as cyclophosphamide in the treatment of patients with major renal disease or alveolar hemorrhage. There were no significant differences between the treatment groups with respect to rates of adverse events. CONCLUSIONS Rituximab therapy was not inferior to daily cyclophosphamide treatment for induction of remission in severe ANCA-associated vasculitis and may be superior in relapsing disease. (Funded by the National Institutes of Allergy and Infectious Diseases, Genentech, and Biogen; ClinicalTrials.gov number, NCT00104299.) PMID:20647199

Stone, John H.; Merkel, Peter A.; Spiera, Robert; Seo, Philip; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St. Clair, E. William; Turkiewicz, Anthony; Tchao, Nadia K.; Webber, Lisa; Ding, Linna; Sejismundo, Lourdes P.; Mieras, Kathleen; Weitzenkamp, David; Ikle, David; Seyfert-Margolis, Vicki; Mueller, Mark; Brunetta, Paul; Allen, Nancy B.; Fervenza, Fernando C.; Geetha, Duvuru; Keogh, Karina A.; Kissin, Eugene Y.; Monach, Paul A.; Peikert, Tobias; Stegeman, Coen; Ytterberg, Steven R.; Specks, Ulrich

2011-01-01

72

Hepatitis C-Associated Mixed Cryoglobulinemic Vasculitis Induces Differential Gene Expression in Peripheral Mononuclear Cells  

PubMed Central

This study examines the distinct gene expression profile of peripheral blood mononuclear cells from patients with chronic hepatitis C infection and mixed cryoglobulinemic (MC) vasculitis. Our DNA microarray analysis indicates that hepatitis C virus (HCV)-associated MC vasculitis is characterized by compromised neutrophil function, impaired chemotaxis, and increased interferon-stimulated gene (ISG) expression, contributing to overall MC pathogenesis and end-organ damage. Increased ISG expression is suggestive of an enhanced endogenous interferon gene signature. PBMC depletion assays demonstrate that this increased expression is likely due to an activation of monocytes and not a direct result of B cell expansion. Notably, this monocyte activation of ISG expression in HCV-associated MC vasculitis suggests a poor predictor status of interferon-based treatment. Further analysis of PBMC gene expression profiles before and after in vivo B cell depletion therapy is critical to completely understanding the mechanisms of MC vasculitis pathogenesis. PMID:24904592

Sidharthan, Sreetha; Kim, Cheol-Woo; Murphy, Alison A.; Zhang, Xiaozhen; Yang, Jun; Lempicki, Richard A.; Sneller, Michael C.; Kottilil, Shyam

2014-01-01

73

Cerebral angiography as a guide for therapy in isolated central nervous system vasculitis  

SciTech Connect

The authors present a case of isolated central nervous system vasculitis documented by cerebral arteriography in which remission, using a treatment regimen of prednisone and cyclophosphamide, was guided by serial arteriography during a 15-month period.

Stein, R.L.; Martino, C.R.; Weinert, D.M.; Hueftle, M.; Kammer, G.M.

1987-04-24

74

Behçet’s disease: an immune-mediated vasculitis involving vessels of all sizes  

Microsoft Academic Search

Behçet’s disease is an immune-mediated vasculitis affecting both small and large vessels. Small-vessel vasculitis is the pathological\\u000a basis of the multiorgan involvement that results in protean clinical features. However, relapsing aphthous ulcers in the mouth\\u000a are considered the clinical hallmark and are often also observed over the genitalia. Both manifestations, in association with\\u000a uveitis, form the typical clinical triad. In

R. Rizzi; S. Bruno; R. Dammacco

1997-01-01

75

Translational Mini-Review Series on Immunology of Vascular Disease: Accelerated atherosclerosis in vasculitis  

PubMed Central

Premature atherosclerosis has been observed during the course of different systemic inflammatory diseases such as rheumatoid arthritis and sytemic lupus erythematosus. Remarkably, relatively few studies have been published on the occurrence of accelerated atherosclerosis in patients with vasculitis. In giant cell arteritis (GCA), mortality because of ischaemic heart disease is not increased. In addition, intima media thickness (IMT) is lower in patients with GCA than in age-matched controls. In contrast, IMT is increased significantly in Takayasu arteritis, another form of large vessel vasculitis occurring in younger patients. In Takayasu arteritis and in Kawasaki disease, a form of medium-sized vessel vasculitis, accelerated atherosclerosis has been well documented. In small vessel vasculitis because of anti-neutrophil cytoplasmic autoantibodies-associated vasculitis, cardiovascular diseases are a major cause of mortality. IMT measurements reveal conflicting results. During active disease these patients experience acceleration of the atherosclerotic process. However, when inflammation is controlled, these patients have atherosclerotic development as in healthy subjects. Several risk factors, such as diabetes and hypertension, are present more often in patients with vasculitis compared with healthy controls. In addition, steroids may be pro-atherogenic. Most importantly, many patients have impaired renal function, persistent proteinuria and increased levels of C-reactive protein, well-known risk factors for acceleration of atherosclerosis. Enhanced oxidation processes, persistently activated T cells and reduced numbers of regulatory T cells are among the many pathophysiological factors that play a role during acceleration of atherogenesis. Finally, autoantibodies that may be relevant for acceleration of atherosclerosis are found frequently in elevated titres in patients with vasculitis. Because patients have an increased risk for cardiovascular events, vasculitis should be treated with as much care as possible. In addition, treatment should be considered with angiotensin-converting-enzyme inhibitors and/or angiotensin receptor-1 blockers, statins and acetylsalicyl acid. Finally, classical risk factors for cardiovascular disease should be monitored and treated as much as possible. PMID:19309350

Tervaert, J W Cohen

2009-01-01

76

Mycophenolate mofetil in the treatment of SLE and systemic vasculitis: experience at a single university center.  

PubMed

Mycophenolate mofetil (MMF) is used off-label for systemic lupus erythematosus (SLE) and systemic vasculitis. The study aim was to investigate clinical use and treatment results with MMF in patients with SLE and systemic vasculitis. This study included patients with SLE or systemic vasculitis with ongoing or previous MMF treatment. Data on treatment outcome were obtained through medical record reviews. A total of 135 of 648 (21%) patients with SLE and 43 of 455 (9%) patients with systemic vasculitis had ongoing or previous MMF treatment. Among SLE patients, the most common organ manifestation at baseline (treatment start) was renal involvement (50%). Most of the systemic vasculitis patients had Wegener's granulomatosis (GPA) (65%). Median dose of MMF was 2000?mg/day. Glucocorticoid (GC) doses were significantly reduced during MMF treatment from 21.7?mg/day at baseline to 8.3?mg/day at 12 months (p?vasculitis patients. MMF appeared to be effective with a reasonable survival-on-drug and a GC-sparing effect. PMID:24399811

Lourdudoss, C; Vollenhoven, R van

2014-03-01

77

Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report  

PubMed Central

Introduction Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud’s arthropathy, and valvular heart disease have been reported. Case presentation A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. Conclusions We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy. PMID:25339233

2014-01-01

78

Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome.  

PubMed

Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922

Azami, Ahad; Maleki, Nasrollah; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra

2014-01-01

79

Circovirus in Tissues of Dogs with Vasculitis and Hemorrhage  

PubMed Central

We characterized the complete genome of a novel dog circovirus (DogCV) from the liver of a dog with severe hemorrhagic gastroenteritis, vasculitis, and granulomatous lymphadenitis. DogCV was detected by PCR in fecal samples from 19/168 (11.3%) dogs with diarrhea and 14/204 (6.9%) healthy dogs and in blood from 19/409 (3.3%) of dogs with thrombocytopenia and neutropenia, fever of unknown origin, or past tick bite. Co-infection with other canine pathogens was detected for 13/19 (68%) DogCV-positive dogs with diarrhea. DogCV capsid proteins from different dogs varied by up to 8%. In situ hybridization and transmission electron microscopy detected DogCV in the lymph nodes and spleens of 4 dogs with vascular compromise and histiocytic inflammation. The detection of a circovirus in tissues of dogs expands the known tropism of these viruses to a second mammalian host. Our results indicate that circovirus, alone or in co-infection with other pathogens, might contribute to illness and death in dogs. PMID:23628223

Li, Linlin; McGraw, Sabrina; Zhu, Kevin; Leutenegger, Christian M.; Marks, Stanley L.; Kubiski, Steven; Gaffney, Patricia; Dela Cruz Jr, Florante N.; Wang, Chunlin; Delwart, Eric

2013-01-01

80

Current and emerging techniques for ANCA detection in vasculitis.  

PubMed

Detection of antineutrophil cytoplasmic antibodies (ANCAs) is a well-established diagnostic test used to evaluate suspected necrotizing vasculitis of small blood vessels. Conditions associated with these antibodies, collectively referred to as ANCA-associated vasculitides, include granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). The diagnostic utility of ANCA testing depends on the type of assay performed and on the clinical setting. Most laboratories worldwide use standard indirect immunofluorescence tests (IFT) to screen for ANCA and then confirm positive IFT results with antigen-specific tests for proteinase 3 (PR3) and myeloperoxidase (MPO). Developments such as automated image analysis of immunofluorescence patterns, so-called third-generation PR3-ANCA and MPO-ANCA ELISA, and multiplex technology have improved the detection of ANCAs. However, challenges in routine clinical practice remain, including methodological aspects of IFT performance, the diverse antigen-specific assays available, the diagnostic value of testing in clinical settings and the prognostic value of serial ANCA monitoring in the prediction of disease relapse. This Review summarizes the available data on ANCA testing, discusses the usefulness of the various ANCA assays and advises on the clinical indications for the use of ANCA testing. PMID:24890776

Csernok, Elena; Moosig, Frank

2014-08-01

81

Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome  

PubMed Central

Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922

Azami, Ahad; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra

2014-01-01

82

Neuroborreliosis-associated cerebral vasculitis: long-term outcome and health-related quality of life.  

PubMed

Neuroborreliosis affects the nervous system after systemic infection with the spirochete Borrelia burgdorferi. Previously, cerebral vasculitis has been regarded as an extremely rare complication of neuroborreliosis. The data on the long-term outcome in patients with cerebral vasculitis due to neuroborreliosis are limited. The objective of this study was to perform a longitudinal analysis of cases of neuroborreliosis-associated cerebral vasculitis. We recruited all patients (n = 11) diagnosed with neuroborreliosis-associated in three neurological departments in an East German region. Inclusion criteria were sudden neurological deficits, magnetic resonance (MR) imaging findings that conform to cerebral ischemia or brain infarction, intrathecal synthesis of borrelia-specific antibodies, and non-atherosclerotic pathology of brain supplying arteries. Vasculitic changes were detected by digital subtraction angiography, MR angiography and/or transcranial Doppler ultrasound. Outcomes were measured by the modified Rankin scale (mRS) and EuroQoL Index. Cerebral vasculitis is a rare complication of Lyme disease (0.3% of all cases in the endemic area). Ten out of 11 patients diagnosed with neuroborreliosis-associated vasculitis cerebral vasculitis using clinical, radiological and immunological criteria developed ischemic stroke or transient ischemic attacks (TIA), 7 patients had recurrent stroke. Vasculitic alterations could be demonstrated in 8 patients that all except one developed ischemic lesions. The median mRS was 3 (range 0-4) at admission and 2 (range 0-6) at discharge. The posterior circulation was affected in 8 of 11 patients; thrombosis of the basilar artery was detected in 2 patients, one died in the acute stage. Neuroborreliosis can cause recurrent stroke or TIA on the basis of cerebral vasculitis. Lumbar puncture is needed for detection of this potentially life-threatening condition. Early recognition and adequate therapy would possibly improve outcome. PMID:23329377

Back, Tobias; Grünig, Steffi; Winter, Yaroslav; Bodechtel, Ulf; Guthke, Kersten; Khati, Diana; von Kummer, Rüdiger

2013-06-01

83

Genome-Wide Association Study of Hepatitis C Virus- and Cryoglobulin-Related Vasculitis  

PubMed Central

Objectives The host genetic basis of mixed cryoglobulin vasculitis is not well understood and has not been studied in large cohorts. A genome-wide association study was conducted among 356 HCV RNA positive individuals with cryoglobulin-related vasculitis and 447 ethnically-matched, HCV RNA positive controls. Methods All cases had both serum cryoglobulins as well as a vasculitis syndrome. A total of 899,641 markers from the Illumina HumanOmni1-Quad chip were analyzed using logistic regression adjusted for sex, as well as genetically-determined ancestry. Replication of select single nucleotide polymorphisms (SNPs) was conducted using 91 cases and 180 controls, adjusting for sex and country of origin. Results The most significant associations were identified on chromosome 6 near the NOTCH4 and MHC class II genes. A genome-wide significant association was detected on chromosome 6 at SNP rs9461776 (OR= 2.16, p=1.16E-07) between HLA-DRB1 and DQA1: this association was further replicated in additional independent samples (meta-analysis p=7.1×10?9). Conclusions A genome-wide significant association with cryoglobulin related vasculitis was identified with SNPs near NOTCH4 and MHC Class II genes. The two regions are correlated and it is difficult to disentangle which gene is responsible for the association with MC vasculitis in this extended MHC region. PMID:25030430

Zignego, Anna Linda; Wojcik, Genevieve L.; Cacoub, Patrice; Visentini, Marcella; Casato, Milvia; Mangia, Alessandra; Latanich, Rachel; Charles, Edgar; Gragnani, Laura; Terrier, Benjamin; Piazzola, Valeria; Dustin, Lynn B.; Khakoo, Salim I.; Busch, Michael P.; Lauer, George M.; Kim, Arthur Y.; Alric, Laurent; Thomas, David L.; Duggal, Priya

2014-01-01

84

Levamisole-Contaminated Cocaine: An Emergent Cause of Vasculitis and Skin Necrosis  

PubMed Central

The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care. PMID:24778656

Mobarakai, Neville

2014-01-01

85

Levamisole-contaminated cocaine: an emergent cause of vasculitis and skin necrosis.  

PubMed

The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care. PMID:24778656

Souied, Osama; Baydoun, Hassan; Ghandour, Zahraa; Mobarakai, Neville

2014-01-01

86

Transduction of a Foreign Histocompatibility Gene into the Arterial Wall Induces Vasculitis  

NASA Astrophysics Data System (ADS)

Autoimmune vasculitis represents a disease characterized by focal inflammation within arteries at multiple sites in the vasculature. Therapeutic interventions in this disease are empirical and often unsuccessful, and the mechanisms of immune injury are not well-defined. The direct transfer of recombinant genes and their expression in the arterial wall provides an opportunity to explore the pathogenesis and treatment of vascular disease. In this report, an animal model for vasculitis has been developed. Inflammation has been elicited by direct gene transfer of a foreign class I major histocompatibility complex gene, HLA-B7, to specific sites in porcine arteries. Transfer and expression of this recombinant gene was confirmed by a polymerase chain reaction and immunohistochemistry, and cytolytic T cells specific for HLA-B7 were detected. These findings demonstrate that expression of a recombinant gene in the vessel wall can induce a focal immune response and suggest that vessel damage induced by cell-mediated immune injury can initiate vasculitis.

Nabel, Elizabeth G.; Plautz, Gregory; Nabel, Gary J.

1992-06-01

87

Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.  

PubMed

Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis. PMID:25147987

Gammon, Bryan; Longmire, Michelle; DeClerck, Brittney

2014-09-01

88

Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis  

EPA Science Inventory

ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...

89

Toxoplasmosis associated with digital vasculitis and immunodeficiency--a dilemma in diagnosis.  

PubMed Central

We present a case of acquired toxoplasmosis associated with severe digital vasculitis--a previously unreported finding. The rise in Toxoplasma gondii antibody titres may have been attenuated by impaired cellular immunity, and this poses a problem for diagnosis. PMID:6483704

Ellis, M. E.; Webb, A. K.; Dunbar, E. M.

1984-01-01

90

eNOS inhibits the development of autoimmune-mediated vasculitis  

PubMed Central

Objective Many different genes or mediators have been implicated in promoting the development of vasculitis, although little is known regarding the mechanisms that normally act to suppress lesion formation. eNOS (NOS3) has been shown to inhibit vascular inflammation in many different model systems, but its roles in the pathogenesis of vasculitis have not been elucidated. The aim of this study was to determine the functions of eNOS in the initiation and progression of vasculitic lesion formation. Methods Nos3 mutant MRL/MpJ-Faslpr mice were generated and comprehensively evaluated and compared to controls for the development of autoimmune disease, including vasculitic lesion formation and glomerulonephritis. Results Nos3?/? MRL/MpJ-Faslpr mice had accelerated onset and increased incidence of renal vasculitis compared to Nos3+/+ controls. In contrast, no significant differences in severity of glomerulonephritis were observed between groups. Vasculitis was also observed in eNOS deficient mice in other organs, including increased expression in the lung. Ultrastructural analyses of renal lesions revealed the presence of electron dense deposits in affected arteries, while IgG, IgA, and C3 deposition was observed in some vessels in Nos3?/? kidneys. In addition, eNOS deficient mice showed increased levels of circulating IgG-IgA immune complexes at 20 weeks of age compared to Nos3+/+ MRL/MpJ-Faslpr and Nos3?/? C57BL/6 mice. Conclusion These findings strongly indicate that eNOS serves as a negative regulator of vasculitis in MRL/MpJ-Faslpr mice, and further suggest that NO produced by this enzyme may be critical for inhibiting lesion formation and vascular damage in human vasculitic diseases. PMID:22933338

Schoeb, Trenton R.; Jarmi, Tambi; Hicks, M. John; Henke, Scott; Zarjou, Abolfazl; Suzuki, Hitoshi; Kramer, Philip; Novak, Jan; Agarwal, Anupam; Bullard, Daniel C.

2012-01-01

91

Immunological and clinical follow up of hepatitis C virus associated cryoglobulinaemic vasculitis  

PubMed Central

OBJECTIVE—To study immunological markers and compare these markers with standard measures for the clinical and immunological follow up of vasculitis activity in hepatitis C virus (HCV) associated cryoglobulinaemic vasculitis (CV).?METHODS—Serial serum samples from eight patients with newly diagnosed HCV associated CV were followed during interferon ? treatment induced remission of the CV. Vasculitis activity and disease extent were evaluated with the Birmingham vasculitis activity score (BVAS) and disease extent index (DEI). Cryoglobulinaemia, complement levels (C3c, C4, and CH50), rheumatoid factor (RF), autoantibodies such as antinuclear antibodies, soluble interleukin 2 receptor (sIL2r), soluble intercellular adhesion molecule-1 (sICAM-1), and soluble CD30 (sCD30) were determined.?RESULTS—All patients achieved either complete or partial remission of their CV during interferon ? treatment. There was a significant reduction in vasculitis activity and disease extent (BVAS, DEI), cryoglobulinaemia, RF, sIL2r, sICAM-1, and sCD30. Complement C3c levels increased significantly during this period. Erythrocyte sedimentation rate and levels of complement C4 and CH50 did not change significantly. Both clinical measures (BVAS and DEI) correlated significantly only with C3c and sCD30.?CONCLUSIONS—Although this study was of only a small group of patients, it shows that BVAS and DEI as clinical measures and C3c and sCD30 as immunological markers may be useful in the follow up of disease activity of HCV associated CV. The data indicate that activity of the humoral (cryoglobulinaemia, RF, autoantibodies) and cellular (sIL2r, sICAM-1, sCD30) immune response and endothelial damage (sICAM-1) are found in HCV associated CV.?? PMID:11247870

Lamprecht, P; Moosig, F; Gause, A; Herlyn, K; Csernok, E; Hansen, H; Gross, W

2001-01-01

92

Abnormal plasma fibrinolysis in patients with rheumatoid arthritis and impaired endothelial fibrinolytic response in those complicated by vasculitis.  

PubMed Central

OBJECTIVES--(a) To assess plasma fibrinolytic parameters in patients with rheumatoid arthritis (RA) and to determine whether there are differences between patients with RA alone and those with RA complicated by vasculitis. (b) To determine if patients with RA respond differently to venous occlusion compared with normal subjects and to assess whether such a response differs in patients with RA alone and those with rheumatoid vasculitis. (c) To determine the extent of vascular damage in patients with rheumatoid vasculitis and if this correlates with the levels of plasma fibrinolytic parameters. METHODS--Sixty three patients with RA (38 had RA only and 25 had evidence of rheumatoid vasculitis) were recruited. Plasma levels of tissue plasminogen activator antigen (t-PA Ag), plasminogen activator inhibitor (PAI) activity, and factor VIII von Willebrand factor (vWF) were measured before and 10 minutes after venous occlusion. RESULTS--Patients with RA, with or without rheumatoid vasculitis, had higher baseline PAI levels than control subjects. The difference was statistically significant for patients with RA alone but was not statistically significant for patients with rheumatoid vasculitis. After venous occlusion, t-PA Ag levels increased significantly in normal subjects and patients with RA alone, but not in patients with rheumatoid vasculitis. Plasma levels of vWF were significantly higher in patients with rheumatoid vasculitis than in normal subjects and those with RA alone. In patients with RA alone, baseline vWF correlated positively with t-PA Ag levels, whereas a negative correlation was found between these two parameters in patients with rheumatoid vasculitis. A negative correlation between vWF and t-PA Ag levels after venous occlusion was also found in patients with rheumatoid vasculitis. CONCLUSIONS--Patients with rheumatoid vasculitis showed evidence of vascular damage with increased levels of vWF and impaired t-PA Ag release after venous occlusion, a useful measurement of endothelial reserve to remove fibrin. This may be of pathophysiological importance in the development of vasculitis in these patients. PMID:8239758

Lau, C S; McLaren, M; Hanslip, J; Kerr, M; Belch, J J

1993-01-01

93

p-ANCA-Associated Vasculitis Caused by Levamisole-Adulterated Cocaine: A Case Report  

PubMed Central

A rare case of a patient with an unusual skin rash after using cocaine is presented. A clinical diagnosis of levamisole-induced vasculitis was made based on initial presentation of purpuric skin lesions involving the ears and positive cocaine on urine toxicology screening. The diagnosis was confirmed after laboratory findings of neutropenia, positive serum levamisole, and a histopathologic test of the skin lesions. The pathophysiology of this condition is discussed. Treatment with methylprednisolone and prednisone led to the resolution of the neutropenia and complete recovery of the skin lesions. With the growing use of levamisole-tainted cocaine, clinicians should be aware of the symptoms of vasculitis and neutropenia induced by this combination of drugs to avoid unnecessary tests and delayed diagnosis. PMID:23476825

Carter, Michelle R.

2013-01-01

94

Metastatic Pulmonary Calcinosis and Leukocytoclastic Vasculitis in a Patient with Multiple Myeloma  

PubMed Central

Both leukocytoclastic vasculitis and metastatic pulmonary calcification are conditions that rarely occur during the course of multiple myeloma. We present a multiple myeloma patient that had severe dyspnea due to metastatic pulmonary calcinosis, and ulceronecrotic skin lesions caused by leukocytoclastic vasculitis. After 3 courses of standarddose chemotherapy all skin and pulmonary lesions disappeared. Autologous peripheral stem cell transplantation was performed and during 1 year of follow-up the patient was in complete remission; after 1 year, laboratory test results indicated disease relapse. Although the patient was treated with bortezomib and dexamethasone, the disease progressed. Non-myeloablative allogeneic stem cell transplantation was performed, but despite of all treatment the patient died due to disease progression. Conflict of interest:None declared. PMID:24385728

Ça??rgan, Seçkin; Soyer, Nur; Vural, Filiz; Saydam, Güray; Y?ld?r?m ?im?ir, Ilg?n; Dönmez, Ayhan; Akal?n, Taner; Biçero?lu, Selen; Tombulo?lu, Murat

2012-01-01

95

[Vasculitis 2013. What are the changes introduced in the 2012 Chapel Hill Consensus Conference?].  

PubMed

Vasculitis is a heterogeneous group of rare disorders in which inflammation of blood vessels is the common feature. Due to the increasing number of diseases as well as overlaps and gaps in the definition and nomenclature, the classification criteria were constantly changing in the past decades. The classifications were based essentially on the size of affected blood vessels and pathologic characteristics of inflamed vessel walls. The standard procedures and validated diagnostic criteria are missing from the diagnostics of vasculitis, thus in clinical practice the classification criteria are applicable. The 2012 Chapel Hill Consensus Conference brought a change in the definition, nomenclature and classification of previously uncategorized diseases. The definitions of subgroups accurately determine the diagnosis of the specific disease, and they are suitable for establishing homogeneous disease groups. By better understanding of the etiopathogenetic factors, further diseases and subgroups may be defined in the near future. PMID:24077160

Zeher, Margit

2013-10-01

96

Levamisole-induced myopathy and leukocytoclastic vasculitis: a case report and literature review.  

PubMed

Levamisole, an immunomodulator and anthelmintic medication, has been used in dermatology for years. Even though the adverse effects are usually mild and reversible, attention should be paid toward severe events such as vasculitis and neutropenia. To the best of our knowledge, this is the first case report on a patient presenting with myopathy caused by levamisole. Here, we report a 34-year-old woman with recalcitrant warts who received levamisole 100?mg daily for 5?days. Subsequently, bilateral lower limb weakness accompanied by multiple painful and non-blanchable purpura was noted. Levamisole-induced myopathy and leukocytoclastic vasculitis were diagnosed by skin histopathology, direct immunofluorescence, and electromyography. After discontinuing levamisole and giving a short course of systemic steroid, these symptoms demonstrated a resolving trend. PMID:24552411

Tsai, Meng-Hsuan; Yang, Jen-Hung; Kung, Sheng-Ling; Hsiao, Yu-Ping

2013-01-01

97

Silica and asbestos exposure in ANCA-associated vasculitis with pulmonary involvement.  

PubMed

Silica and asbestos exposure are thought to belong to the triggering factors of antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis. We carried out a study to find out whether patients with pulmonary involvement attributable to ANCA-associated vasculitis (AAV) have been exposed to silicon-containing materials. Thirty-one patients (12 women, 19 men, median age 51 years) were interviewed using a structured questionnaire. Occupational exposure to silicon-containing chemicals was reported by 22.6% of the patients (12.9% to SiO2, 9.7% to asbestos), compared with 0% of control subjects (p<0.05). Our findings support the pathophysiologic role of silica in AAV. PMID:16153001

Rihova, Zuzana; Maixnerova, Dita; Jancova, Eva; Pelclova, Daniela; Bartunkova, Jirina; Fenclova, Zdenka; Vankova, Zdenka; Reiterova, Jana; Merta, Miroslav; Rysava, Romana; Tesar, Vladimir

2005-01-01

98

A case of primary systemic necrotizing vasculitis presenting primarily with neurologic involvement.  

PubMed

Systemic necrotizing vasculitis (SNV) is a type of vasculitis that presents with necrosis, predominantly involving large, medium-sized and small arteries. Peripheral neuropathy is a major clinical feature of the primary and secondary systemic vasculitides, and is often observed during the early phases of the disease, causing axonal neuropathy. The prevalence of central nervous system (CNS) involvement ranges from 4% to 45%. Encephalopathy, focal neurological deficits, and seizures are the most common manifestations and usually occur late during the course of SNV. In this report, we describe a 61-year-old woman with SNV who had both CNS and peripheral nervous system vasculitic involvement. We also discuss the pathophysiology of nervous system involvement in patients with SNV. PMID:24854993

Liu, Jing-Yao; Zhang, Ren-Sheng; Zhou, Chun-Kui

2014-01-01

99

Acute Respiratory Failure as Primary Manifestation of Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis  

PubMed Central

The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a life-threatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.

Sourla, Evdokia; Bagalas, Vasilis; Tsioulis, Helias; Paspala, Asimina; Akritidou, Sofia; Pataka, Athanasia; Fekete, Katalin; Kioumis, Ioannis P.; Stanopoulos, Ioannis; Pitsiou, Georgia

2014-01-01

100

Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants  

PubMed Central

Background Secondary systemic vasculitis after posterior spinal fusion surgery is rare. It is usually related to over-reaction of immune-system, to genetic factors, toxicity, infection or metal allergies. Case Description A 14 year-old girl with a history of extended posterior spinal fusion due to idiopathic scoliosis presented to our department with diffuse erythema and nephritis (macroscopic hemuresis and proteinuria) 5 months post surgery. The surgical trauma had no signs of inflammation or infection. The blood markers ESR and CRP were increased. Skin tests were positive for nickel allergy, which is a content of titanium alloy. The patient received corticosteroids systematically (hydrocortisone 10 mg) for 6 months, leading to total recess of skin and systemic reaction. However, a palpable mass close to the surgical wound raised the suspicion of a late infection. The patient had a second surgery consisting of surgical debridement and one stage revision of posterior spinal instrumentation. Intraoperative cultures were positive to Staphylococcus aureus. Intravenous antibiotics were administered. The patient is now free of symptoms 24 months post revision surgery without any signs of recurrence of either vasculitis or infection. Literature Review Systemic vasculitis after spinal surgery is exceptionally rare. Causative factors are broad and sometimes controversial. In general, it is associated with allergy to metal ions. This is usually addressed with metal on metal total hip bearings. In spinal surgery, titanium implants are considered to be inert and only few reports have presented cases with systemic vasculitides. Therefore, other etiologies of immune over-reaction should always be considered, such as drug toxicity, infection, or genetic predisposition. Purposes and Clinical Relevance Our purpose was to highlight the difficulties during the diagnostic work-up for systemic vasculitis and management in cases of posterior spinal surgery. PMID:21914179

2011-01-01

101

Bioinspired engineering study of Plantae vascules for self-healing composite structures  

PubMed Central

This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a ‘lost-wax’ technique, orthogonal hollow vascules, inspired by the ‘ray cell’ structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122

Trask, R. S.; Bond, I. P.

2010-01-01

102

Blocking the NOTCH Pathway Inhibits Vascular Inflammation in Large Vessel Vasculitis  

PubMed Central

Background Giant cell arteritis (GCA) is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke and aortic aneurysm. CD4 T-cells are key pathogenic regulators, instructed by vessel wall dendritic cells (DC) to differentiate into vasculitic T cells. The unique pathways driving this DC-T-cell interaction are incompletely understood but may provide novel therapeutic targets for a disease in which the only established therapy is chronic treatment with high doses of corticosteroids. Methods and Results Immunohistochemical and gene expression analysis of GCA-affected temporal arteries revealed abundant expression of the NOTCH receptor and its ligands Jagged1 and Delta1. Cleavage of the NOTCH intracellular domain (NICD) in wall-infiltrating T cells indicated ongoing NOTCH pathway activation in large vessel vasculitis. NOTCH activation did not occur in small vessel vasculitis affecting branches of the vasa vasorum tree. We devised two strategies to block NOTCH pathway activation; ?-secretase inhibitor treatment, preventing nuclear translocation of the NICD, and competing for receptor-ligand interactions through excess soluble ligand, Jagged1-Fc. In humanized mice carrying human arteries NOTCH pathway disruption had strong immunosuppressive effects, inhibiting T-cell activation in the early and established phase of vascular inflammation. NOTCH inhibition was particularly effective in downregulating Th17 responses, but also markedly suppressed Th1 responses. Conclusions Blocking NOTCH signaling depleted T cells from the vascular infiltrates, implicating NOTCH-NOTCH ligand interactions in regulating T-cell retention and survival in vessel wall inflammation. Modulating the NOTCH signaling cascade emerges as a promising new strategy for immunosuppressive therapy of large vessel vasculitis. PMID:21220737

Piggott, Kisha; Deng, Jiusheng; Warrington, Kenneth; Younge, Brian; Kubo, Jessica T.; Desai, Manisha; Goronzy, Jorg J.; Weyand, Cornelia M.

2011-01-01

103

Percutaneous Management of Occlusive Arterial Disease Associated with Vasculitis: A Single Center Experience  

SciTech Connect

The purpose of this study was to evaluate the safety and effectiveness of percutaneous transluminal angioplasty for occlusive arterial disease associated with vasculitis. Eleven patients(10 women, 1 man; ages 35-82 years) with the diagnosis of vasculitis of the large vessels underwent interventional treatment during intraarterial angiography. The causes included giant cell arteritis(n = 8) and Takayasu arteritis (n = 3).Thirty-three occlusive lesions (including brachiocephalic and renalarteries, and arteries of upper and lower extremities) were treated with balloon angioplasty and/or stent placement. Follow-up included clinical examination, angiography, and color duplex ultrasound.Technical success was 100% (25/25) for stenoses and 50% (4/8) for occlusive lesions, representing all lesions combined from different anatomic locations. Dissection (n = 3) and arterial rupture with retroperitoneal hematoma (n = 1) was found in three patients. During follow-up (mean 12 months), restenoses(n = 8) and re-restenoses (n = 1)occurred in 8 vascular areas. Three of these lesions were treated with repeated PTA (n = 4). The cumulative primary clinical success rate was 67.6%, cumulative secondary success rate 74.4%, and cumulative tertiary success rate 75.9%. Interventional therapy in systemic vasculitis provides promising results in technical success rates and followup. Angioplasty may result in arterial injury, but the rate of complications is low.

Both, M.; Jahnke, T. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel(Germany); Reinhold-Keller, E. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Reuter, M.; Grimm, J.; Biederer, J.; Brossmann, J. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany); Gross, W.L. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Heller, M.; Mueller-Huelsbeck, S. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany)

2003-02-15

104

Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature.  

PubMed

Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40?? followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients. PMID:25107938

Medina, G; González-Pérez, D; Vázquez-Juárez, C; Sánchez-Uribe, M; Saavedra, M A; Jara, L J

2014-11-01

105

Antineutrophil cytoplasmic autoantibody-negative antiproteinase 3 syndrome presenting as vasculitis, endocarditis, polyneuropathy and Dupuytren's contracture.  

PubMed

Antiproteinase 3 antibodies (antiPR3) are assumed to be subtypes of antineutrophil cytoplasmic autoantibodies (ANCA), with a high specificity for active Wegener's granulomatosis and microscopic polyangiitis. Thus, antiPR3 positivity in ELISA, together with negativity in indirect immunofluorescence (IIF) is a rare finding. A 56-year-old man with Dupuytren's contracture and polyneuropathy was admitted for leukocytoclastic vasculitis. Echocardiography, performed because of fever and dyspnea, detected aortic valve endocarditis. Because of severe aortic insufficiency the valve was replaced. Blood cultures and bacteriologic investigations of the explanted valve were negative. AntiPR3 were elevated (123-163 U/ml; normal <6 U/ml), together with negativity in IIF. This case shows that antiPR3 elevation with negative ANCA may be associated with vasculitis, endocarditis, polyneuropathy and Dupuytren's contracture. A causal relationship between the clinical presentation and antiPR3 elevation is likely. In order not to miss such cases of vasculitis, combined screening by IIF and ELISA is recommended in selected cases. PMID:12918859

Stöllberger, Claudia; Finsterer, Josef; Zlabinger, Gerhard J; Weihsengruber, Felix; Redtenbacher, Susanne; Bonner, Gerhard; Herkner, Kurt; Deutsch, Manfred

2003-07-01

106

Surgical lung biopsy to diagnose Behcet's vasculitis with adult respiratory distress syndrome.  

PubMed

A 34-year-old female presented with fever and abdominal pain. Past medical history includes Crohn's and Behcet's disease. Examination revealed multiple skin ulcerations, oral aphthae, and bilateral coarse rales. She developed respiratory distress with diffuse bilateral alveolar infiltrates on chest radiograph requiring intubation. PaO2/FiO2 ratio was 132. The chest computed tomography revealed extensive nodular and patchy ground-glass opacities. Bronchoalveolar lavage demonstrated a predominance of neutrophils. Methylprednisolone 60 mg every 6 h and broad-spectrum antimicrobials were initiated. No infectious etiologies were identified. Surgical lung biopsy demonstrated diffuse alveolar damage (DAD) mixed with lymphocytic and necrotizing vasculitis with multiple small infarcts and thrombi consistent with Behcet's vasculitis. As she improved, steroids were tapered and discharged home on oral cyclophosphamide. Pulmonary involvement in Behcet's is unusual and commonly manifests as pulmonary artery aneurysms, thrombosis, infarction, and hemorrhage. Lung biopsy findings demonstrating DAD are consistent with the clinical diagnosis of adult respiratory distress syndrome. The additional findings of necrotizing vasculitis and infarcts may have led to DAD. PMID:25378849

Vydyula, Ravikanth; Allred, Charles; Huartado, Mariana; Mina, Bushra

2014-10-01

107

Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association  

PubMed Central

Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis. Materials and Methods. We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity. Case Report. Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis. Conclusion. This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture's syndrome and p-ANCA associated vasculitis.

Bal, Amanjit; Das, Ashim; Gupta, Dheeraj; Garg, Mandeep

2014-01-01

108

ANCA-associated Vasculitis Patients Have Defective Treg Function Exacerbated by Presence of a Suppression-Resistant Effector Population  

PubMed Central

Objective The development of pathogenic anti-neutrophil cytoplasmic autoantibodies (ANCAs) can result in systemic small vessel vasculitis. However, the breakdown in immune tolerance that results in the induction and persistence of ANCAs is not well-understood. We hypothesized that abnormal T cell regulation is central to disease pathogenesis and demonstrate here two separate abnormalities in T cell regulation in ANCA-associated vasculitis patients. Methods Peripheral blood samples were obtained from patients with ANCA-associated vasculitis (n=63) and healthy controls (n=19) for flow cytometric analysis of CD4+ T cell populations. Functional T cell studies were performed with FACS sorted CD4+ T cell populations stimulated with anti-CD3/28. Results First, we show that the Treg frequency in the peripheral blood of active disease patients is increased, but Tregs from patients with ANCA-associated vasculitis have decreased suppressive function. Tregs from active disease patients disproportionately utilize a FOXP3 isoform lacking exon 2, which may alter Treg function. Second, we identify a CD4+ T cell population with increased frequency that is resistant to Treg suppression, produces pro-inflammatory cytokines, and is antigen-experienced. Conclusion ANCA-associated vasculitis is associated with disruption of the suppressive Treg network and increased frequency of a distinct pro-inflammatory effector T cell subset which comprises the majority of peripheral CD4+ T cells. PMID:23553415

Free, Meghan E; Bunch, Donna O; McGregor, JulieAnne; Jones, Britta E; Berg, Elisabeth A; Hogan, Susan L; Hu, Yichun; Preston, Gloria A; Jennette, J. Charles; Falk, Ronald J; Su, Maureen A

2013-01-01

109

Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis  

PubMed Central

Objective To identify biomarkers that distinguish between active ANCA-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV. 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Serum levels of E-selectin, ICAM-3, MMP1, MMP3, MMP9, P-selectin, thrombomodulin, and VEGF were measured at study screening (time of active disease) and at month 6. ESR and CRP levels had been measured at the time of the clinical visit. The primary outcome was the difference in marker level between screening and month 6 among patients in remission (BVAS/WG score of 0) at month 6. Results All subjects had severe active vasculitis (mean BVAS/WG score 8.6 +/? 3.2 SD) at screening. Among the 123 subjects clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP3 levels were also higher among the 23 subjects with active disease at month 6 than among the 123 subjects in remission. MMP3 levels correlated weakly with ESR and CRP. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. PMID:21953143

Monach, Paul A; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Cuthbertson, David; Krischer, Jeffrey; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Ikle, David; Kallenberg, Cees GM; Langford, Carol A; Mueller, Mark; Seo, Philip; St.Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Gu, Yi-Zhong; Snyder, Ronald D; Merkel, Peter A

2011-01-01

110

Muscle restricted vasculitis causing dropped head syndrome: a case report and review of the literature.  

PubMed

A 52-year-old man presented with a severe head drop and proximal extremity weakness. Magnetic resonance imaging of the cervical spine showed T2 hyperintensity in cervical paraspinal muscles. Electrodiagnostic studies revealed an axial myopathy isolated to paraspinal muscles. A splenius capitis muscle biopsy confirmed an acute myopathy associated with nonsystemic vasculitis. The patient improved on steroids, intravenous immunoglobulin, and monthly pulse doses of cyclophosphamide. Our case emphasizes that a subgroup of patients with dropped head syndrome have treatable conditions. PMID:22538306

Nielsen, Amy Almaraz; Smith, Benn E; Engel, Andrew G; Bosch, Erich Peter

2012-03-01

111

Adenocarcinoma of the seminal vesicles complicated by antineutrophil cytoplasmic antibody vasculitis: a case report and review of the literature  

PubMed Central

Introduction Adenocarcinoma of the seminal vesicles is a very rare malignancy, with less than 100 cases reported worldwide. It is documented to have a poor prognosis, with the majority of patients developing metastatic disease, most commonly in the prostate, bladder and rectum. Currently there is no standard treatment for metastatic disease and the limited reports of treatment with radiotherapy, chemotherapy and hormonal (anti-androgenic) therapy show that they are generally of modest benefit. The association between malignancy and an increased risk of autoimmune vasculitis has been demonstrated in a number of malignancies, but to date there have been no documented cases of adenocarcinoma of the seminal vesicles associated with anti-neutrophil cytoplasmic antibody vasculitis. Case presentation In this report we describe the case of a 55-year-old Caucasian man with metastatic adenocarcinoma of the seminal vesicles. He previously had received chemotherapy treatment for advanced testicular cancer and later presented with hemospermia. He subsequently developed c-antineutrophil cytoplasmic antibody vasculitis requiring intensive immunosuppression and renal dialysis. Conclusion Adenocarcinoma of the seminal vesicles is a rare diagnosis and our case is more unusual in that our patient previously had chemotherapy treatment for advanced testicular cancer and went on to develop severe antineutrophil cytoplasmic antibody vasculitis when diagnosed with metastatic seminal vesicle cancer. This case illustrates that autoimmune vasculitis can occur in any patient with malignancy and an early referral to the renal team combined with renal biopsy can assist in the earlier diagnosis and more successful management of these rare events. This case should be of interest to oncologists, renal physicians, urologists and general physicians who encounter patients presenting with hemospermia or vasculitis. PMID:23452402

2013-01-01

112

A rare case of underlying pulmonary sequestration in a patient with recently diagnosed medium and large vessel vasculitis.  

PubMed

Vasculitis of medium- and large-sized arteries is an inflammatory and stenotic disease characterized by a strong predilection for the aortic arch and its branches. It presents with symptoms and signs as per the vessels and organs involved. Pulmonary sequestration is a rare abnormality characterized by a mass of nonfunctioning lung tissue that receives its vascular supply from a systemic artery and is separated from the normal tracheobronchial tree. The following is a rare case report showing the presence of pulmonary sequestration in a patient with recently diagnosed hypertension and intestinal angina due to medium and large vessel vasculitis. PMID:24778487

Malik, Sarthak; Khurana, Sakshi; Vasudevan, Vishnu; Gupta, Nikhil

2014-04-01

113

A rare case of underlying pulmonary sequestration in a patient with recently diagnosed medium and large vessel vasculitis  

PubMed Central

Vasculitis of medium- and large-sized arteries is an inflammatory and stenotic disease characterized by a strong predilection for the aortic arch and its branches. It presents with symptoms and signs as per the vessels and organs involved. Pulmonary sequestration is a rare abnormality characterized by a mass of nonfunctioning lung tissue that receives its vascular supply from a systemic artery and is separated from the normal tracheobronchial tree. The following is a rare case report showing the presence of pulmonary sequestration in a patient with recently diagnosed hypertension and intestinal angina due to medium and large vessel vasculitis. PMID:24778487

Malik, Sarthak; Khurana, Sakshi; Vasudevan, Vishnu; Gupta, Nikhil

2014-01-01

114

Experimental steroid-induced osteonecrosis in adult rabbits with hypersensitivity vasculitis.  

PubMed

Osteonecrosis (ON) was experimentally induced in rabbits by employing a combined protocol of hypersensitivity vasculitis and administration of high-dose corticosteroids. Thirty-five adult rabbits were used: five were injected twice with horse serum (Group A), five were injected three times with methylprednisolone acetate (Group B), 20 were treated with a combination of horse serum and methylprednisolone acetate (Group C), and five were used as a control (Group D). Both femurs of each rabbit were obtained one to five weeks after the final treatment and were histologically examined. There was no evidence of ON in Groups A, B, and D, whereas vasculitis was prominent in the femurs of Group A rabbits. In Group C, 14 of 20 specimens (70%) showed histologic evidence of ON in the femoral metaphysis: seven showed marrow necrosis and seven marrow and trabecular necrosis. Intramedullary hemorrhage was detected in eight animals. All specimens that showed ON or marrow necrosis revealed arteriopathy (i.e., severe damage to the vascular wall structure of arterioles). These findings were similar to those observed in early ON of clinical materials. The authors conclude that arteriopathy plays an important role in the pathogenesis of ON. PMID:1555358

Matsui, M; Saito, S; Ohzono, K; Sugano, N; Saito, M; Takaoka, K; Ono, K

1992-04-01

115

T cell receptor (TCR) V gene usage in patients with systemic necrotizing vasculitis.  

PubMed Central

Wegener's granulomatosis (WG) and polyarteritis nodosa (PAN) are systemic necrotizing vasculitides of unknown etiology. These disorders run a fatal course if untreated. T lymphocytes are implicated in the pathogenesis of WG, since they have been found to infiltrate affected organs, and sIL-2R correlates with disease activity. To elucidate further the role of T cells in necrotizing vasculitis, we have used a panel of 12 TCR V-specific MoAbs to investigate the number of cells expressing certain V alpha and V beta gene segments in the CD4+ and CD8+ subsets of altogether 11 patients with WG or PAN. In the group of patients, we found abnormal expansions of T cells using particular TCR V alpha or V beta gene products. These T cell expansions were more numerous, of a dramatically higher magnitude, and frequently more often found in the CD4 subset, compared with T cell expansions identified in healthy individuals. In long-term studies of the T cell expansions for up to 18 months, a heterogeneous pattern was revealed, with no obvious correlation to clinical features such as disease activity or treatment. Studies of TCR V gene usage in this group of patients may help in understanding the pathogenesis of necrotizing vasculitis, and in the identification of unknown antigens, and may open the possibility to a highly selective immunotherapy by targeting disease-mediating T cells. PMID:7648706

Giscombe, R; Grunewald, J; Nityanand, S; Lefvert, A K

1995-01-01

116

Antibodies to endothelial cells in systemic lupus erythematosus: a potential marker for nephritis and vasculitis  

PubMed Central

Using an ELISA, anti-endothelial cell antibodies (AECA) have been found in sera obtained at the time of renal biopsy in 46 out of 57 patients (81%) with systemic lupus erythematosus (SLE) and nephritis (mean binding index (BI)=84%±52.8) compared with 22 out of 50 SLE patients (44%) without nephritis (mean BI=45%±35.9). Seventy normal human sera had a mean BI of 10%±9.8. The highest levels were seen in patients with diffuse proliferative glomerulonephritis (WHO grade IV) and in patients with proteinuria and nephrotic syndrome. When the biopsies were assessed for activity and chronicity scores, AECA were associated with active renal lesions (P<0.001). AECA levels correlated with low complement levels but not with anti-DNA antibodies to extractable nuclear antigens (ENA), anti-cardiolipin or anti-neutrophil cytoplasmic antibodies. The presence of AECA conferred a positive predictive value of 0.68 for the presence of nephritis. Twenty-five patients had active vasculitis at the time of assay and the highest AECA values were seen in patients with both nephritis and vasculitis. No correlation was seen with serum immunoglobulin levels and immune complexes did not bind significantly to the endothelial surface. The possible role of these antibodies as a marker in lupus nephritis is discussed. PMID:1864005

D'Cruz, D. P.; Houssiau, F. A.; Ramirez, G.; Baguley, E.; McCutcheon, J.; Vianna, J.; Haga, H.-J.; Swana, G. T.; Khamashta, M. A.; Taylor, J. C.; Davies, D. R.; Hughes, G. R. V.

1991-01-01

117

Antibodies to endothelial cells in systemic lupus erythematosus: a potential marker for nephritis and vasculitis.  

PubMed

Using an ELISA, anti-endothelial cell antibodies (AECA) have been found in sera obtained at the time of renal biopsy in 46 out of 57 patients (81%) with systemic lupus erythematosus (SLE) and nephritis (mean binding index (BI) = 84% +/- 52.8) compared with 22 out of 50 SLE patients (44%) without nephritis (mean BI = 45% +/- 35.9). Seventy normal human sera had a mean BI of 10% +/- 9.8. The highest levels were seen in patients with diffuse proliferative glomerulonephritis (WHO grade IV) and in patients with proteinuria and nephrotic syndrome. When the biopsies were assessed for activity and chronicity scores, AECA were associated with active renal lesions (P less than 0.001). AECA levels correlated with low complement levels but not with anti-DNA antibodies to extractable nuclear antigens (ENA), anti-cardiolipin or anti-neutrophil cytoplasmic antibodies. The presence of AECA conferred a positive predictive value of 0.68 for the presence of nephritis. Twenty-five patients had active vasculitis at the time of assay and the highest AECA values were seen in patients with both nephritis and vasculitis. No correlation was seen with serum immunoglobulin levels and immune complexes did not bind significantly to the endothelial surface. The possible role of these antibodies as a marker in lupus nephritis is discussed. PMID:1864005

D'Cruz, D P; Houssiau, F A; Ramirez, G; Baguley, E; McCutcheon, J; Vianna, J; Haga, H J; Swana, G T; Khamashta, M A; Taylor, J C

1991-08-01

118

Wet Shape Memory Alloy Actuators for Active Vasculated Robotic Flesh Stephen A. Mascaro and H. Harry Asada  

E-print Network

Wet Shape Memory Alloy Actuators for Active Vasculated Robotic Flesh Stephen A. Mascaro and H is presented where Shape Memory Alloy (SMA) wires are embedded within artificial "blood vessels." Fluid flowing Memory Alloys, which are thin enough to fit inside of an artificial blood vessel while generating large

Mascaro, Stephen A.

119

Necrotizing vasculitis  

MedlinePLUS

... do not heal Muscles and joints: Joint pain Leg pain Muscle contractions Wasting away of muscles Brain and nervous system: Pain, numbness , tingling in an arm, leg, or other body area Weakness of an arm, ...

120

Rheumatoid Vasculitis  

MedlinePLUS

... medications. Figure: Digital ischemia – this image shows a blood flow deficiency in the tip of the finger caused ... who develops new constitutional symptoms, skin ulcerations, decreased blood flow to the fingers or toes, symptoms of a ...

121

Simultaneous Automated Screening and Confirmatory Testing for Vasculitis-Specific ANCA  

PubMed Central

Anti-neutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of small vessel vasculitis, so called ANCA-associated vasculitis. The international consensus requires testing by indirect immunofluorescence (IIF) on human ethanol-fixed neutrophils (ethN) as screening followed by confirmation with enzyme-linked immunosorbent assays (ELISAs). This study evaluates the combination of cell- and microbead-based digital IIF analysis of ANCA in one reaction environment by the novel multiplexing CytoBead technology for simultaneous screening and confirmatory ANCA testing. Sera of 592 individuals including 118 patients with ANCA-associated vasculitis, 133 with rheumatoid arthritis, 49 with infectious diseases, 77 with inflammatory bowel syndrome, 20 with autoimmune liver diseases, 70 with primary sclerosing cholangitis and 125 blood donors were tested for cytoplasmic ANCA (C-ANCA) and perinuclear ANCA (P-ANCA) by classical IIF and ANCA to proteinase 3 (PR3) and myeloperoxidase (MPO) by ELISA. These findings were compared to respective ANCA results determined by automated multiplex CytoBead technology using ethN and antigen-coated microbeads for microbead immunoassays. There was a good agreement for PR3- and MPO-ANCA and a very good one for P-ANCA and C-ANCA by classical and multiplex analysis (Cohen's kappa [?]?=?0.775, 0.720, 0.876, 0.820, respectively). The differences between classical testing and CytoBead analysis were not significant for PR3-ANCA, P-ANCA, and C-ANCA (p<0.05, respectively). The prevalence of confirmed positive ANCA findings by classical testing (IIF and ELISA) compared with multiplex CytoBead analysis (IIF and microbead immunoassay positive) resulted in a very good agreement (??=?0.831) with no significant difference of both methods (p?=?0.735). Automated endpoint-ANCA titer detection in one dilution demonstrated a very good agreement with classical analysis requiring dilution of samples (??=?0.985). Multiplexing by CytoBead technology can be employed for simultaneous screening and quantitative confirmation of ANCA. This novel technique provides fast and cost-effective ANCA analysis by automated digital IIF for the first time. PMID:25225805

Sowa, Mandy; Grossmann, Kai; Knutter, Ilka; Hiemann, Rico; Rober, Nadja; Anderer, Ursula; Csernok, Elena; Bogdanos, Dimitrios P.; Borghi, Maria Orietta; Meroni, Pier Luigi; Schierack, Peter; Reinhold, Dirk; Conrad, Karsten; Roggenbuck, Dirk

2014-01-01

122

Angioedema in a 47-year-old woman with hypocomplementemic urticarial vasculitis syndrome.  

PubMed

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a type III hypersensitivity reaction characterized by urticaria with persistent acquired hypocomplementemia. Although HUVS is uncommon, it is important for physicians to be familiar with this disease, as the initial presentation is often life-threatening angioedema. The author reports the case of a 47-year-old white woman with a history of HUVS. She presented to an outpatient clinic complaining of a rash and difficulty swallowing. Urticaria and angioedema were diagnosed. The patient was given epinephrine in the clinic and then transferred to the hospital. Laboratory testing confirmed urticaria, and the patient was given intravenous methylprednisolone sodium succinate and an additional dose of epinephrine. After 1 week, the patient's angioedema improved. PMID:22331803

Jones, Julie M; Reich, Keith A; Raval, Deena G

2012-02-01

123

Retinal vasculitis in two pediatric patients with systemic lupus erythematosus: a case report  

PubMed Central

We report two pediatric female patients with systemic lupus erythematosus (SLE) who presented with decreased vision. Both patients were found to have retinal vasculitis and occlusive disease. The first patient also presented with vitreous hemorrhage and later non-arteritic ischemic optic neuropathy. She was treated with panretinal photocoagulation and steroid therapy and later in her disease course was treated with rituximab and cyclophosphamide. Her vision remained decreased. The second patient was treated with rituximab and monthly cyclophosphamide infusions early in her disease course, and her vision improved dramatically. The difference in the presentations and outcomes of these two pediatric patients with SLE highlights the spectrum of severity of SLE retinopathy. We suggest that early recognition of disease and early intervention with B-cell depletion therapy in addition to a traditional cytotoxic agent should be considered in pediatric patients with SLE and occlusive retinopathy. PMID:23734963

2013-01-01

124

Necrotizing vasculitis in a case of disseminated neonatal herpes simplex infection.  

PubMed

A term newborn suffered disseminated herpes simplex virus (HSV) type II infection five days after cesarean section delivery for fetal distress. The mother had no history or evidence of herpetic lesions; the father had a history of genital herpetic lesions. The infant's terminal course was dominated by disseminated intravascular coagulation (DIC) with hepatic and renal failure. Microscopic examination revealed a necrotizing vasculitis of small and medium-sized lung and peripancreatic arteries. Nuclear inclusions characteristic of HSV were found in these arteries, as well as in the adrenal parenchyma, spleen, and lymph node; electron microscopy confirmed replication of virus within the arterial endothelial cells. The mechanism of arterial damage in severe herpetic infection contrasts with the immune-complex mechanism postulated for other viral vasculitides. Direct, virally induced arterial damage resulting in exposure of collagen may set the stage of DiC, a commonly fatal complication of this disease. PMID:6895828

Phinney, P R; Fligiel, S; Bryson, Y J; Porter, D D

1982-02-01

125

Magnetic resonance imaging of skeletal muscle involvement in limb restricted vasculitis  

PubMed Central

Objective: To describe the MRI findings in the legs of three patients with limb restricted vasculitides (two PAN, one isolated vasculitis of the skeletal muscle) with histologically established muscle involvement. Methods: MRI was carried out on calf muscles and T2 weighted images, unenhanced T1 weighted images, and STIR sequences were obtained. Results: Muscle damage resulted in oedema-like changes on MRI characterised by hyperintense signals in T2 weighted and slow tau inversion recovery (STIR) sequences and normal unenhanced T1 weighted sequences of one or several leg muscles. Conclusions: MRI should be considered a useful complementary examination that might facilitate the recognition of limb restricted vasculitides, and possibly indicate the site for muscle biopsy. It could also be useful in monitoring the course of the disease. Future studies should also evaluate MRI for systemic PAN or other systemic vasculitides with muscle involvement. PMID:12429545

Gallien, S; Mahr, A; Rety, F; Kambouchner, M; Lhote, F; Cohen, P; Guillevin, L

2002-01-01

126

Localized chronic fibrosing vasculitis in a tattoo: a unique adverse tattoo reaction.  

PubMed

Decorative tattoos are associated with a variety of adverse cutaneous reactions. We describe a unique fibrosing vasculitic reaction to red tattoo ink. The histopathology was similar to that in localized chronic fibrosing vasculitis (LCFV), but sharply limited to sites of red tattoo ink injection and associated with florid verrucoid epidermal hyperplasia. LCFV has been described in a broad variety of slowly progressive disorders with a firm consistency such as erythema elevatum diutinum, plasma cell granuloma, granuloma faciale, and IgG4-associated sclerosing diseases. It has been hypothesized that LCFV is the result of maladaptive immune reaction with failure to clear the causative antigen. To the best of our knowledge, this is the first case of LCFV associated with tattoo. We speculate on the implications our case holds for the pathogenesis of LCFV. PMID:24736671

Deeken, Audrey; Jefferson, Julie; Hawkinson, Dana; Fraga, Garth R

2014-04-01

127

Necrotizing vasculitis in Greece: clinical, immunological and immunogenetic aspects. A study of 66 patients.  

PubMed

The clinical spectrum and outcome of necrotizing vasculitis were evaluated in a retrospective study in order to assess: (1) the clinical expression and evolution of the disease; (2) the response to several therapeutic approaches based on major events (organ involvement causing disability or death); (3) the immunogenetic background of patients. Sixty-six Greek patients fulfilling the ACR criteria for the diagnosis of vasculitis entered the study. Thirty-seven were diagnosed with Wegener's granulomatosis (WG), 22 with polyarteritis nodosa (PAN) and seven with Churg-Strauss syndrome (CSS). The demographic characteristics of patients with WG and PAN were similar. Cutaneous manifestations, gastrointestinal and peripheral nervous system involvement occurred more often in patients with PAN, whereas pulmonary and upper respiratory tract involvement, renal, ear abnormalities and fever were more frequent in patients with WG. Muscle weakness and asthma were found exclusively in patients with PAN and CSS, respectively, while the presence of classic-antineutrophil cytoplasmic antibodies (c-ANCA) characterized WG patients. Hepatitis B surface antigen (HBsAg) was found in 22% of PAN patients. No significant differences were detected when comparing the PAN and WG groups with respect to the first major event (log-rank P = 0.50) with and without potential confounders (age, gender, therapy or c-ANCA). For WG patients, a statistically significant difference was found on different routes of administration of cyclophosphamide (oral vs pulse) (P = 0.006). Regarding the HLA antigens, an increased frequency of DR1 (26.9% vs 10.3%, P = 0.057) in WG and the absence of DR3 in patients with PAN and CSS were noted. It appears that although the immunogenetic background and the clinical expression of the diseases differ, the response to treatment as well as the evolution and the survival rate of these patients are similar in the two groups. PMID:9374922

Boki, K A; Dafni, U; Karpouzas, G A; Papasteriades, C; Drosos, A A; Moutsopoulos, H M

1997-10-01

128

Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis*  

PubMed Central

BACKGROUND It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS Patients with active chronic urticaria had the highest serum levels of D-dimer (p<0.01), when compared to patients with CU under remission and the control group (patients with psoriasis). CONCLUSIONS Patients with active chronic urticaria have higher serum levels of D-dimer, when compared to patients with chronic urticaria under remission and patients with psoriasis. We found elevated serum levels of D-dimer among patients with urticarial vasculitis. PMID:23793207

Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva

2013-01-01

129

Coagulation and Fibrinolysis Index Profile in Patients with ANCA-Associated Vasculitis  

PubMed Central

Background Previous studies observed the high prevalence of venous thromboembolism in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The current study analyzed the coagulation and fibrinolysis index profile in AAV patients. Methods The current study recruited 321 AAV patients in active stage and 78 AAV patients in quiescent stage. Coagulation and fibrinolysis index profiles in these AAV patients were analysed, and their associations with various clinical and pathological parameters were further investigated. Results The circulating levels of D-dimer, fibrin degradation products and platelet count were significantly higher in AAV patients in active stage compared with those in remission [0.8 (0.4, 1.5) mg/L vs. 0.28 (0.2, 0.55) mg/L, P<0.05; 5.6 (5.0, 10.0) mg/L vs. 1.9 (1.2, 2.8) mg/L, P<0.05; 269±127×109/L vs. 227±80×109/L, P<0.05, respectively]. Among the 321 AAV patients in active stage, compared with patients with normal levels of D-dimer, patients with elevated D-dimer levels had significantly higher levels of initial serum creatinine, erythrocyte sedimentation rate, C reactive protein and the Birmingham Vasculitis Activity Scores (P?=?0.014, P<0.001, P<0.001, P?=?0.002, respectively). Moreover, correlation analysis showed that the levels of D-dimer correlated with erythrocyte sedimentation rate and C reactive protein levels (r?=?0.384, P<0.001; r?=?0.380, P<0.001, respectively). Conclusion Patients with active AAV are in hypercoagulable states, and circulating levels of D-dimer are associated with disease activity of AAV. PMID:24842719

Ma, Tian-Tian; Huang, Yi-Min; Wang, Chen; Zhao, Ming-Hui; Chen, Min

2014-01-01

130

PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature  

PubMed Central

PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis. PMID:25097791

Uchiyama, Robert C.

2014-01-01

131

Unusual cause of acute neurologic deficit in childhood: primary central nervous system vasculitis presenting with basilar arterial occlusion  

Microsoft Academic Search

Introduction  Primary central nervous system (CNS) vasculitis of childhood is a rare disorder. The most common signs and symptoms are acute\\u000a severe headache and focal neurologic deficit. It should be suspected in children who have an acquired neurologic deficit that\\u000a remains unexplained after an initial basic evaluation. Diagnosis usually depends on brain magnetic resonance imaging and conventional\\u000a angiography of cerebral vasculature.

Eren Kale Çekinmez; Nurcan Cengiz; ?lknur Erol; Osman K?z?lk?l?ç; Yasemin Uslu

2009-01-01

132

Antineutrophil cytoplasm autoantibodies from patients with systemic vasculitis activate neutrophils through distinct signaling cascades: comparison with conventional Fcg receptor ligation  

Microsoft Academic Search

In systemic vasculitis, interactions be- tween antineutrophil cytoplasm autoanti- bodies (ANCAs) and neutrophils initiate endothelial and vascular injury. ANCAs directed against either myeloperoxidase (MPO) or proteinase 3 (PR3) can activate cytokine-primed neutrophils by binding cell surface-expressed MPO or PR3, with the concurrent engagement of Fcg recep- tors (FcgR). Because roles for phospho- lipase D (PLD) and phosphatidylinositol 3 kinase (PI3K)

Anne Ben-Smith; Stephen K. Dove; Ashley Martin; Michael J. O. Wakelam; Caroline O. S. Savage

133

Long-term observation of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis treated with rituximab  

Microsoft Academic Search

Objective. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be quite effective in the treatment of immune disorders resulting from autoantibodies. We prospectively studied the long-term effects of rituximab in 10 patients with anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis refractory to conventional therapy (n^3) or in second or subsequent relapse (n^7). Methods. The median age of patients was 53

R. Stasi; E. Stipa; G. Del Poeta; S. Amadori; A. C. Newland; D. Provan

2006-01-01

134

Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist.  

PubMed

Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) are conditions classified under the general heading of antinuclear cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Lung lesion is a very common and important clinical feature in AAV. In MPA, diffuse alveolar hemorrhage and pulmonary fibrosis (PF) are the most frequent manifestations. High-resolution computed tomography (HRCT) chest findings associated with MPA in PF patients demonstrate a high frequency of usual interstitial pneumonia (UIP), fibrotic-nonspecific interstitial pneumonia (F-NSIP), and combined PF and emphysema (CPFE) pattern with honeycombing, traction bronchiectasis, ground-glass opacity, and emphysema. In most of these cases, the histologic pattern of PF has been classified as UIP and/or fibrotic NSIP. In addition, a high incidence of histological findings, such as extensive interstitial fibrosis, lymphoid hyperplasia, and bronchiolitis, are characteristics observed in PF associated with collagen vascular diseases and which are not observed in idiopathic PF (IPF). In some cases, PF precedes the development of MPA. Indeed, there are some cases of pulmonary-limited MPA in this group. Therefore, clinicians should be aware of MPA as an underlying feature of PF in order to avoid overlooking and misdiagnosing this condition as IPF. The median survival time (MST) in UIP pattern/MPA is comparable with that of IPF. In GPA, almost all patients have either upper airway or lower respiratory tract lesions. Solitary or multiple nodules (frequently cavitated) and masses are the most common findings on chest images. Asthma is a cardinal symptom of Churg-Straus syndrome, often preceded by allergic rhinitis. To induce remission, a severity-based regimen was given to patients according to the appropriate protocol of the Japanese patients with myeloperoxidase (MPO)-ANCA-associated vasculitis (JMAAV) study group: low-dose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; severe-form regimen plus plasmapheresis in those with the most severe form. PMID:23188194

Homma, Sakae; Suzuki, Aika; Sato, Keita

2013-10-01

135

Genetic Susceptibility to ANCA-Associated Vasculitis: State of the Art  

PubMed Central

ANCA-associated vasculitis (AAV) is a group of disorders that is caused by inflammation affecting small blood vessels. Both arteries and veins are affected. AAV includes microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) renamed from Wegener’s granulomatosis, and eosinophilic granulomatosis with polyangiitis (EGPA), renamed from Churg–Strauss syndrome. AAV is primarily due to leukocyte migration and resultant damage. Despite decades of research, the mechanisms behind AAV disease etiology are still not fully understood, although it is clear that genetic and environmental factors are involved. To improve the understanding of the disease, the genetic component has been extensively studied by candidate association studies and two genome-wide association studies. The majority of the identified genetic AAV risk factors are common variants. These have uncovered information that still needs further investigation to clarify its importance. In this review, we summarize and discuss the results of the genetic studies in AAV. We also present the novel approaches to identifying the causal variants in complex susceptibility loci and disease mechanisms. Finally, we discuss the limitations of current methods and the challenges that we still have to face in order to incorporate genomic and epigenomic data into clinical practice.

Bonatti, Francesco; Reina, Michele; Neri, Tauro Maria; Martorana, Davide

2014-01-01

136

Presumed reactive polyarthritis and granulomatous vasculitis in a Mississippi sandhill crane (Grus canadensis pulla).  

PubMed

A 1.5-year-old female Mississippi sandhill crane (Grus canadensis pulla) was presented and managed for a polyarthritis of the intertarsal and tarsophalangeal articulations. Results of aerobic bacterial cultures, Mycoplasma species culture, and polymerase chain reaction testing of articular fluid did not identify any causative organisms. Results of radiographs and cytologic examination of articular fluid were consistent with an inflammatory, nonerosive polyarthritis. The arthritis did not improve with systemic anti-inflammatory and antibiotic treatment and with joint lavage. A large necrotic granulomatous mass was detected on the right shoulder area from which Staphylococcus aureus and Enterococcus species were isolated as opportunistic pathogens. Two days after surgical resection of the mass, the distal polyarthritis resolved. Histopathologic examination of the mass was consistent with granulomatous vasculitis with abscess formation of unknown origin. In this crane, the unresponsiveness to standard therapy, the presence of an infected and inflammatory mass, and the resolution of the polyarthritis after the resection of the mass strongly supported a diagnosis of reactive immune-mediated nonerosive polyarthritis. Analysis of this case suggests that immune-mediated idiopathic arthritis should be a differential diagnosis of distal polyarthritis in cranes and that an inciting source remote from the joints should be investigated in case of lack of response to standard therapy. PMID:24640933

MacLean, Robert; Beaufrère, Hugues; Heggem-Perry, Brittany; Field, Cara; Garner, Michael

2013-12-01

137

Experience with rituximab in the treatment of antineutrophil cytoplasmic antibody associated vasculitis  

PubMed Central

Prior to the 1970s, severe cases of antineutrophil cytoplasmic antibody associated vasculitis (AAV) were thought to be invariably fatal. However, the use of cyclophosphamide-based treatment regimens fundamentally altered disease outcomes, transforming AAV into a manageable, chronic illness. Despite the tremendous success of cyclophosphamide in the treatment of AAV, there remained a need for alternative therapies, due to high rates of treatment failures and significant toxicities. In recent years, with the introduction of targeted biologic response modifiers into clinical practice, many have hoped that the treatment options for AAV could be expanded. Rituximab, a chimeric monoclonal antibody directed against the B-lymphocyte protein CD20, has been the most successful biologic response modifier to be used in AAV. Following the first report of its use in AAV in 2001, experience with rituximab for treatment of AAV has rapidly expanded. Rituximab, in combination with glucocorticosteroids, is now well established as a safe and effective alternative to cyclophosphamide for remission induction for severe manifestations of granulomatosis with polyangiitis and microscopic polyangiitis. In addition, initial experiences with rituximab for remission maintenance in these diseases have been favorable, as have experiences for remission induction in eosinophilic granulomatosis with polyangiitis. PMID:24688606

Clain, Jeremy M.; Cartin-Ceba, Rodrigo; Fervenza, Fernando C.

2014-01-01

138

Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment  

PubMed Central

Background The purpose of the study is to describe the clinical course and treatment of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. The study utilized non-randomized, retrospective and interventional case series. The eight eyes of six patients were analysed. Testing included wide fluorescein angiography, indocyanine green angiography and systemic evaluation. Treatment involved observation, panretinal laser photocoagulation (PRP) for peripheral retinal ischemia, grid laser for macular oedema and focal laser on the macroaneurysms. The main outcome measures were initial visual acuity (VA), initial stage at diagnosis, clinical course, surgical intervention, final VA, final stage and complications of disease. Results Five out of eight eyes with retinal ischemia in more than two quadrants that were treated with PRP and grid laser for macular oedema maintained excellent VA and demonstrated no progression of retinal ischemia during follow-up. The two eyes which exhibited retinal ischemia in less than two quadrants and macular oedema were treated with grid laser and focal laser on the macroaneurysms, but did not undergo PRP. VA improved by two lines of the Snellen chart, and there was no progression of retinal ischemia during the 3 and 4 years of follow-up. One eye with neither retinal ischemia nor macular oedema was not treated, and the clinical picture remained stable during the follow-up. Conclusion Early PRP may be considered in the presence of angiographic evidence of peripheral retinal non-perfusion. However, treatment could be withheld until the patient develops retinal ischemia in more than two quadrants. PMID:23514018

2013-01-01

139

Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis)  

PubMed Central

Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate a name may be, if it is not usable and used it is of no lasting value. This brief commentary focuses on the nomenclature of systemic vasculitides, and uses as a prime example Wegener's granulomatosis, which has been renamed recently ‘granulomatosis with polyangiitis’, in part because of concerns about the suitability of Friedrich Wegener as the source of an eponym. The most distinctive pathological feature of Wegener's granulomatosis is multi-focal necrotizing inflammation that has long been called granulomatosis. The systemic variant of Wegener's granulomatosis also is characterized by inflammation in many different vessels or different types, i.e. polyangiitis. Thus, granulomatosis with polyangiitis is a very appropriate alternative term for Wegener's granulomatosis. This term also is in accord with the name for a closely related vasculitis, i.e. microscopic polyangiitis. Terms that indicate aetiology and pathogenesis, when known, are useful to include in names for diseases (diagnoses). Anti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) are implicated in the cause of granulomatosis with polyangiitis and thus also should be specified in the diagnosis (e.g. PR3-ANCA-positive granulomatosis with polyangiitis or MPO-ANCA-positive microscopic polyangiitis). As our understanding of the clinical manifestations, pathogenesis and aetiology of vasculitides change over time, the names and approaches for diagnosing these diseases will change accordingly. PMID:21447122

Jennette, J C

2011-01-01

140

Caveolin-1 Single Nucleotide Polymorphism in Antineutrophil Cytoplasmic Antibody Associated Vasculitis  

PubMed Central

Objective Immunosuppression is cornerstone treatment of antineutrophil cytoplasmic antibody associated vasculitis (AAV) but is later complicated by infection, cancer, cardiovascular and chronic kidney disease. Caveolin-1 is an essential structural protein for small cell membrane invaginations known as caveolae. Its functional role has been associated with these complications. For the first time, caveolin-1 (CAV1) gene variation is studied in AAV. Methods CAV1 single nucleotide polymorphism rs4730751 was analysed in genomic DNA from 187 white patients with AAV from Birmingham, United Kingdom. The primary outcome measure was the composite endpoint of time to all-cause mortality or renal replacement therapy. Secondary endpoints included time to all-cause mortality, death from sepsis or vascular disease, cancer and renal replacement therapy. Validation of results was sought from 589 white AAV patients, from two European cohorts. Results The primary outcome occurred in 41.7% of Birmingham patients. In a multivariate model, non-CC genotype variation at the studied single nucleotide polymorphism was associated with increased risk from: the primary outcome measure [HR 1.86; 95% CI: 1.14-3.04; p=0.013], all-cause mortality [HR:1.83; 95% CI: 1.02-3.27; p=0.042], death from infection [HR:3.71; 95% CI: 1.28-10.77; p=0.016], death from vascular disease [HR:3.13; 95% CI: 1.07-9.10; p=0.037], and cancer [HR:5.55; 95% CI: 1.59-19.31; p=0.007]. In the validation cohort, the primary outcome rate was far lower (10.4%); no association between genotype and the studied endpoints was evident. Conclusions The presence of a CC genotype in Birmingham is associated with protection from adverse outcomes of immunosuppression treated AAV. Lack of replication in the European cohort may have resulted from low clinical event rates. These findings are worthy of further study in larger cohorts. PMID:23894397

Chand, Sourabh; Holle, Julia U.; Hilhorst, Marc; Simmonds, Matthew J.; Smith, Stuart; Kamesh, Lavanya; Hewins, Peter; McKnight, Amy Jayne; Maxwell, Alexander P.; Cohen Tervaert, Jan Willem; Wieczorek, Stefan; Harper, Lorraine; Borrows, Richard

2013-01-01

141

Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?  

PubMed Central

We present the case of an 11-year-old boy presenting with haemoptysis, dyspnoea and weight loss as a manifestation of isolated pulmonary vasculitis, leading to pulmonary hypertension. He also appeared to have a longstanding dural venous sinus thrombosis. This rare presentation, especially in childhood, might represent a case of the seldomly reported Hughes-Stovin syndrome. The patient achieved remission after therapy with cyclophosphamide pulses and high-dose steroids. Based on the presented case and review of the literature, we propose that this syndrome might be a variant of polyarteritis nodosa. This report highlights diagnostic issues and describes a successful treatment regimen. PMID:21816089

2011-01-01

142

The Role of 18F-FDG PET/CT in Large-Vessel Vasculitis: Appropriateness of Current Classification Criteria?  

PubMed Central

Patients with clinical suspicion of large-vessel vasculitis (LVV) may present with nonspecific signs and symptoms and increased inflammatory parameters and may remain without diagnosis after routine diagnostic procedures. Both the nonspecificity of the radiopharmaceutical 18F-FDG and the synergy of integrating functional and anatomical images with PET/CT offer substantial benefit in the diagnostic work-up of patients with clinical suspicion for LVV. A negative temporal artery biopsy, an ultrasonography without an arterial halo, or a MRI without aortic wall thickening or oedema do not exclude the presence of LVV and should therefore not exclude the use of 18F-FDG PET/CT when LVV is clinically suspected. This overview further discusses the notion that there is substantial underdiagnosis of LVV. Late diagnosis of LVV may lead to surgery or angioplasty in occlusive forms and is often accompanied by serious aortic complications and a fatal outcome. In contrast to the American College of Rheumatology 1990 criteria for vasculitis, based on late LVV effects like arterial stenosis and/or occlusion, 18F-FDG PET/CT sheds new light on the classification of giant cell arteritis (GCA) and Takayasu arteritis (TA). The combination of these observations makes the role of 18F-FDG PET/CT in the assessment of patients suspected for having LVV promising. PMID:25328890

Balink, H.; Bennink, R. J.; van Eck-Smit, B. L. F.; Verberne, H. J.

2014-01-01

143

Life-threatening allergic vasculitis after clipping an unruptured aneurysm: Case report, weighing the risk of nickel allergy  

PubMed Central

Background: This case report represents one of the estimated 17,000 aneurysms clipped annually in the United States, often with nickel-containing clips. The authors highlight the development of life-threatening allergic vasculitis in a 33-year-old woman after aneurysm clipping. Case Description: After suffering subarachnoid hemorrhage, the patient had coil embolization at another facility for rupture of a right internal carotid artery (ICA) aneurysm. An incidental finding, an unruptured left posterior communicating artery aneurysm unamenable to coiling, was then successfully clipped via a left pterional craniotomy. Arriving in our emergency department 11 days later, she progressively declined during the next weeks, facing deteriorating clinical status (i.e. seizures) and additional infarctions in the left frontal lobe, midline shift, and new infarctions in the bilateral frontal lobe, right sylvian, right insular regions, and posterior cerebral artery distribution. During decompressive surgery, biopsy findings raised the possibility of lymphocytic vasculitis; consultations with rheumatology, allergy, and immunology specialists identified that our patient had a nickel allergy. After reoperation to replace the nickel-containing clip with one of a titanium alloy, the patient had an uncomplicated postoperative course and was discharged 6 days later to a rehabilitation facility. Conclusions: Nickel-related allergies are more common than appreciated, affecting up to 10% of patients. Fortunately, severe reactions are rare; nevertheless, vascular neurosurgeons should be aware of this potential complication when using cobalt alloy aneurysms clips. The use of titanium alloy clips eliminates this risk. PMID:25071940

Grande, Andrew; Grewal, Sanjeet; Tackla, Ryan; Ringer, Andrew J.

2014-01-01

144

Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study  

PubMed Central

Introduction We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study. Methods In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated. Results Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n?=?61) had significantly lower BVAS (P?=?0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n?=?95). Conclusions MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA. Trial Registration The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009. PMID:24758294

2014-01-01

145

Is newer safer? Adverse events associated with first-line therapies for ANCA-associated vasculitis and lupus nephritis.  

PubMed

Clinical outcomes in ANCA-associated vasculitis (AAV) and lupus nephritis have improved greatly with treatment regimens containing high-dose glucocorticoids and cyclophosphamide. However, with the use of these medications come significant adverse events, most notably infections, cytopenias, malignancies, and reproductive abnormalities. Multiple recent randomized controlled trials in AAV and lupus nephritis have compared cyclophosphamide-based regimens with agents such as rituximab, mycophenolate mofetil, and azathioprine, with the hope of providing better clinical outcomes with improved safety profiles. Although some of these newer regimens are now considered first-line treatments of these diseases, their adverse event profiles have been disappointingly similar to those of cyclophosphamide-based protocols. Physicians and patients should consider the adverse event profiles generated by these trials in the context of their extensive use in other patient populations, as well as available measures to prevent such events, when choosing the ideal regimen for an individual patient. PMID:24832093

Hogan, Jonathan; Avasare, Rupali; Radhakrishnan, Jai

2014-09-01

146

ANCA-positive vasculitis as a secondary autoimmune disease after autologous stem cell transplantation for systemic sclerosis: a case report.  

PubMed

Autologous stem cell transplantation (SCT) is increasingly used to treat autoimmune diseases (AD), in particular systemic sclerosis (SSc). Secondary autoimmune diseases are a known complication after autologous stem cell transplantations for any cause. A 43-year-old man had received an autologous stem cell transplantation for an aggressive diffuse cutaneous SSc. After mobilisation with cyclophosphamide and Granulocyte-Colony-Stimulating Factor stem cells were CD34-selected. The patient received a conditioning regimen with cyclophosphamide and Antithymocyte globulin. He had an excellent response with the modified Rodnan Skin Score decreasing from 34 to 3. One year and 4 months after SCT mild erythrocyturia without acanthocytes and proteinuria were seen for the first time on routine urinalysis. During the following year erythrocyturia increased to 131 erythrocytes /?l and protein excretion to 628 mg/g creatinine. At that time, acanthocytes of 25% finally could be detected. Due to the clearly nephritic constellation in urinalysis a renal biopsy was performed, which revealed mild global and focal-segmental sclerosing and focal-segmental proliferative glomerulonephritis without any signs of a IgA-nephropathy. The result was compatible with a renal manifestation of a small-vessel vasculitis. During the following laboratory workup ANCA of a perinuclear pattern with specificity for myeloperoxidase in high titers could be detected. Therefore the diagnosis of a p-ANCA-positive glomerulonephritis was established. As treatment, the patient received Rituximab, which turned out to be effective. We provide the first report of a patient who developed a p-ANCA-associated vasculitis after autologous stem cell transplantation for an autoimmune disease, namely systemic sclerosis. PMID:25005330

Schmalzing, Marc; Amann, Kerstin; Tony, Hans-Peter

2014-01-01

147

Polymyalgia rheumatica as presenting manifestation of vasculitis involving the lower extremities in a patient with ulcerative colitis.  

PubMed

Extraintestinal features may be observed in patients with ulcerative colitis (UC). We describe a 69-year-old woman who was initially diagnosed as having polymyalgia rheumatica (PMR). Prednisone was progressively tapered to complete discontinuation a year and a half after PMR diagnosis. However, at that time, she started to complain of asthenia, abdominal cramping and pain on the left side, weight loss and bloody diarrhoea. A colonoscopy confirmed a diagnosis of left-sided UC. She experienced several flares of the disease that required admission and treatment with high-dose corticosteroids and azathioprine. Colectomy was performed as the disease became refractory to these therapies. Four months after surgery, when the patient was not receiving any corticosteroid therapy, she started to feel dull and achy pain in the thighs along with claudication of the lower limbs. An 18F-fluorodeoxyglucosepositron emission tomography with CT (FDG PET/CT) disclosed an inflammatory process with mild-moderate diffuse increased metabolism in the thoracic aorta and markedly increased FDG uptake in the in the femoral and posterior tibial arteries on both sides. Treatment with the anti-TNF-alpha monoclonal antibody-adalimumab (40 mg every 2 weeks subcutaneously) along with prednisone (initial dose 15 mg/day) yielded rapid improvement of symptoms. Also, a new FDG PET/CT performed 4 months later disclosed marked decrease of FDG uptake in the involved arteries.This report emphasises the importance of suspecting the presence of large- and medium-vessel vasculitis in a patient with UC presenting with musculoskeletal features. It also highlights the beneficial effect of TNF-antagonists in vasculitis associated to UC. PMID:22640654

Bejerano, Carmen; Blanco, Ricardo; González-Vela, Carmen; Pérez-Martín, Inés; Martinez-Rodriguez, Isabel; Jimenez-Bonilla, Julio; González-Gay, Miguel A

2012-01-01

148

Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease  

PubMed Central

Classically presenting with multiple or single peripheral cytopenias of variable severity, the myelodysplastic syndromes may occasionally present with bizarre manifestations that confuse the clinical picture and result in significant delays in making the correct diagnosis. We describe the case of an elderly male patient whose presentation with prolonged unexplained fever coupled with cutaneous, pulmonary and other systemic features of inflammation was finally diagnosed as having a primary myelodysplastic syndrome with associated vasculitis after a delay of 4 years. PMID:22031795

Hassan, Imad Salah; Dar, Javeed

2011-01-01

149

Intraglomerular and interstitial leukocyte infiltration, adhesion molecules, and interleukin-1? expression in 15 cases of antineutrophil cytoplasmic autoantibody—associated renal vasculitis  

Microsoft Academic Search

In renal biopsy specimens from 15 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated renal vasculitis, the infiltrating intraglomerular and interstitial leukocytes were localized and the adhesion molecules intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) and the cytokine interleukin-1? (IL-1?) were studied by an immunohistochemical method. Intraglomerular leukocytes were mainly macrophages (13.46 ± 9.29 cells\\/glomerular cross-section) and, to a

Maria Pia Rastaldi; Franco Ferrario; Stefania Tunesi; Lin Yang; Giuseppe D'Amico

1996-01-01

150

Levamisole-induced leukocytoclastic vasculitis and neutropenia in a patient with cocaine use: An extensive case with necrosis of skin, soft tissue, and cartilage  

PubMed Central

Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month’s duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography–mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390

2012-01-01

151

Levamisole-induced leukocytoclastic vasculitis and neutropenia in a patient with cocaine use: an extensive case with necrosis of skin, soft tissue, and cartilage.  

PubMed

Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month's duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography-mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390

Arora, Natasha Purai; Jain, Tania; Bhanot, Ravinder; Natesan, Suganthini Krishnan

2012-01-01

152

Analysis of immunoglobulin variable region genes of a human IgM anti-myeloperoxidase antibody derived from a patient with vasculitis.  

PubMed Central

Circulating antibodies to myeloperoxidase (MPO) are associated primarily with pauci-immune glomerulonephritis and systemic vasculitis. Anti-MPO antibodies belong to a group of autoantibodies, anti-neutrophil cytoplasmic antibodies, that may play a pathogenic role in vasculitis. We have generated a human monoclonal anti-MPO antibody (E3-MPO) using peripheral blood lymphocytes from a patient with microscopic polyarteritis. Variable region gene analysis of E3-MPO showed that the VH region had 90% homology with the germ line gene VH4-21. E3-MPO was also shown to carry the 9G4 idiotope, which so far has been associated only with human antibodies that utilize the VH4-21 gene. The 9G4 idiotope was also expressed on anti-MPO antibodies in sera from the donor patient and from 4/7 additional patients with active, untreated vasculitis. The nucleotide sequences of both the variable heavy and light chains of E3-MPO showed evidence of an antigen-driven response. PMID:8698399

Longhurst, C; Ehrenstein, M R; Leaker, B; Stevenson, F K; Spellerberg, M; Chapman, C; Latchmen, D; Isenberg, D A; Cambridge, G

1996-01-01

153

Palisaded neutrophilic granulomatous dermatitis with leukocytoclastic vasculitis in a patient without any underlying systemic disease detected to date.  

PubMed

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare dermatologic condition which shows various clinical and histopathological features. Although the PNGD lesions have been suggested to begin as leukocytoclastic vasculitis (LCV), there is still insufficient clinicopathological information in the reported cases of PNGD in acute stage with LCV. The relationship between PNGD and interstitial granumatous dermatis (IGD) also remains unclear. This report presents the case of a 60-year-old female patient with multiple erythematous nodules on the extremities. She had no underlying systemic disease detected to date, although transient, abnormal liver function tests were seen. The histopathological examination of an erythematous nodule revealed the features of PNGD in the acute stage. The patient presented the characteristic features of LCV including palisaded granulomatous pattern, and the interstitial granulomatous pattern was seen together, suggesting that PNGD with LCV can show an interstitial granulomatous pattern. The present case also suggested that PNGD in the acute stage with LCV tends to clinically manifest as erythematous nodules on the extremities and histopathologically shows a remarkable papillary edema and an extensive fibrin deposition in and around the vessel wall. PNGD may be associated with transient liver dysfunction. PMID:19903215

Misago, Noriyuki; Shinoda, Yohsuke; Tago, Masaki; Narisawa, Yutaka

2010-10-01

154

Hydralazine Induces Myeloperoxidase and Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody Vasculitis and Leads to Pulmonary Renal Syndrome  

PubMed Central

We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100?mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression. PMID:25210633

Sultan, Ghayyath; Werner, Sherry L.; Hura, Claudia

2014-01-01

155

Hydralazine induces myeloperoxidase and proteinase 3 anti-neutrophil cytoplasmic antibody vasculitis and leads to pulmonary renal syndrome.  

PubMed

We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100?mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression. PMID:25210633

Agarwal, Gaurav; Sultan, Ghayyath; Werner, Sherry L; Hura, Claudia

2014-01-01

156

Mycophenolate mofetil in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis: a prospective pharmacokinetics and clinical study.  

PubMed

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) treatment strategy is based on immunosuppressive agents. Little information is available concerning mycophenolic acid (MPA) and the area under the curve (AUC) in patients treated for AAV. We evaluated the variations in pharmacokinetics for MPA in patients with AAV and the relationship between MPA-AUC and markers of the disease. MPA blood concentrations were measured through the enzyme-multiplied immunotechnique (C(0), C(30), C(1), C(2), C(3), C(4), C(6) and C(9)) to determine the AUC. Eighteen patients were included in the study. The median (range) MPA AUC(0-12) was 50·55 (30·9-105·4) mg/h/l. The highest coefficient of determination between MPA AUC and single concentrations was observed with C(3) (P?

Chaigne, B; Gatault, P; Darrouzain, F; Barbet, C; Degenne, D; François, M; Szymanski, P; Rabot, N; Golea, G; Diot, E; Maillot, F; Lebranchu, Y; Nivet, H; Paintaud, G; Halimi, J-M; Guillevin, L; Büchler, M

2014-05-01

157

Lung involvement at presentation predicts disease activity and permanent organ damage at 6, 12 and 24 months follow?-?up in ANCA?-?associated vasculitis  

PubMed Central

Background Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may present with pulmonary involvement ranging from mild to life-threatening disease such as diffuse alveolar hemorrhage. There is a paucity of information regarding morbidity outcomes for AAV subjects presenting with lung involvement. This study determines the relationship between disease activity and damage in these subjects using the Birmingham Vasculitis Activity Score v 3 (BVAS 3) and Vasculitis Damage Index (VDI) respectively. Results 151 patients with AAV were included with 59 presenting initially with pulmonary involvement. The initial BVAS scores recorded at time of diagnosis were positively correlated with the final VDI scores at 24 months (p?

2014-01-01

158

Fibroblasts express OvHV-2 capsid protein in vasculitis lesions of American bison (Bison bison) with experimental sheep-associated malignant catarrhal fever.  

PubMed

American bison (Bison bison) are particularly susceptible to developing fatal sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2), a ?-herpesvirus in the Macavirus genus. This generally fatal disease is characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison, domestic cattle (Bos taurus), and other clinically susceptible species which are considered non-adapted, dead-end hosts. The pathogenesis and cellular tropism of OvHV-2 infection have not been fully defined. An earlier study detected OvHV-2 open reading frame 25 (ORF25) transcripts encoding the viral major capsid protein in tissues of bison with SA-MCF, and levels of viral transcript expression positively correlated with lesion severity. To further define the cellular tropism and replication of OvHV-2 infection in vascular lesions of bison, immunofluorescence studies were performed to identify cell type(s) expressing ORF25 protein within tissues. Cytoplasmic and not nuclear ORF25 protein was demonstrated in predominantly perivascular fibroblasts in six bison with experimentally-induced SA-MCF, and there was no evidence of immunoreactivity in vascular endothelium, smooth muscle, or infiltrating leukocytes. The cytoplasmic distribution of viral major capsid protein suggests that viral replication in perivascular fibroblasts may be abortive in this dead-end host. These findings provide a novel foundation for defining the pathogenesis of vasculitis in non-adapted hosts with SA-MCF. PMID:23953727

Nelson, Danielle D; Taus, Naomi S; Schneider, David A; Cunha, Cristina W; Davis, William C; Brown, Wendy C; Li, Hong; O'Toole, Donal; Oaks, J Lindsay

2013-10-25

159

How Is Vasculitis Diagnosed?  

MedlinePLUS

... as the aorta or the pulmonary arteries). Lung Function Tests Lung function tests measure how much air you can breathe ... your lungs deliver oxygen to your blood. Lung function tests can help your doctor find out whether ...

160

Central Nervous System Vasculitis  

MedlinePLUS

... headaches that last a long time strokes or transient ischemic attacks (“mini-strokes”) forgetfulness or confusion weakness ... cyclophosphamide (a medication that decreases the immune system’s response to autoimmune diseases), are generally used. In some ...

161

The expression of Toll-like receptors 2, 4 and 9 in kidneys of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis.  

PubMed

Increasing evidence suggested that Toll-like receptors (TLRs) were critically involved in immune responses of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The current study aimed to investigate the expression of TLR-2, TLR-4 and TLR-9 in kidneys of patients with ANCA-associated vasculitis. Renal biopsy specimens were collected from 24 patients with AAV. The expression of TLR-2, TLR-4 and TLR-9 in kidneys was detected by immunohistochemistry. Double immunofluorescence staining was performed to detect the expression of TLRs on various kinds of cells. In renal specimens, immunohistochemical examination revealed that expression of TLR-2 and TLR-4 could be detected in the glomeruli of AAV patients, while TLR-2 and TLR-4 were scarcely detected in the glomeruli of normal controls. Double immunofluorescence staining of TLR-2, TLR-4 and CD31 indicated that TLR-4 and TLR-2 were expressed on endothelial cells in the glomeruli. In the tubulointerstitial compartment, expression of TLR-2, TLR-4 and TLR-9 could be detected in both AAV patients and normal controls. The mean optical density of TLR-2 and TLR-4 in the tubulointerstitial compartment in AAV patients were significantly higher than that in normal controls. Among AAV patients, correlation analysis showed that the mean optical density of TLR-4 in the glomeruli correlated inversely with the initial serum creatinine, the proportion of total crescents and the proportion of cellular crescents in renal specimens (r?=?-0·419, P?=?0·041; r?=?-0·506, P?=?0·012; r?=?-0·505, P?=?0·012, respectively). The expression of TLR-2 and TLR-4 was dysregulated in kidneys of AAV patients. The expression of TLR-4 in glomeruli was associated with the severity of renal injury. PMID:24773611

Wang, H; Gou, S-J; Zhao, M-H; Chen, M

2014-09-01

162

Local and systemic activation of the whole complement cascade in human leukocytoclastic cutaneous vasculitis; C3d,g and terminal complement complex as sensitive markers.  

PubMed Central

We have studied complement activation both in plasma samples and in lesional skin from patients with leukocytoclastic cutaneous vasculitis (LCV). Enzyme immunoassay (EIA) quantification of the complement activation markers, C3d,g and the terminal complement complex (TCC) in plasma, showed that their levels were significantly increased in 66% and 55% of the patients, respectively (n = 29) compared with healthy controls, whereas the standard measurements of C3, factor B, C1q, C4 and C2 were generally within normal range. Elevations of C3d,g and TCC levels in plasma were significantly correlated. Importantly, a significant correlation was found between the severity of the vasculitis and both C3d,g and TCC plasma levels. Immunofluorescence studies of skin biopsy specimens demonstrated simultaneous presence of perivascular dermal deposits of C3d,g and TCC in lesional skin from 96% and 80% respectively of the patients (n = 25). There was a significant correlation between the intensity of the deposits of both markers. Clusterin, a TCC inhibitory protein, was always found at the same sites of perivascular TCC deposits. Immunofluorescence studies at the epidermal basement membrane zone (BMZ) revealed in each case deposits of C3d,g which were accompanied by TCC deposits in 52% of the biopsy specimens. These data demonstrate that there is a local and systemic activation of the whole complement cascade in human LCV. The presence of both C3d,g and clusterin-associated TCC perivascular deposits suggests an intervention of a regulatory mechanism of local complement activation in LCV. Finally, measurement of plasma C3d,g and TCC appears to be a sensitive indicator of systemic complement activation and disease severity in LCV. Images Fig. 1 Fig. 2 PMID:8485913

Dauchel, H; Joly, P; Delpech, A; Thomine, E; Sauger, F; Le Loet, X; Lauret, P; Tron, F; Fontaine, M; Ripoche, J

1993-01-01

163

Large vessel vasculitis in elderly patients: early diagnosis and steroid-response evaluation with FDG-PET/CT and contrast-enhanced CT.  

PubMed

Large vessel vasculitis (LVV) is an often-reported cause of inflammation of unknown origin (IUO) in elderly people. The objective of this study was to describe the usefulness of fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) and contrast-enhanced CT in early diagnosis and treatment follow-up of patients with LVV presenting as elderly onset IUO. We retrospectively compared contrast-enhanced CT findings and FDG-PET/CT findings of the patients diagnosed with LVV and 11 controls; all subjects were 50 years of age or older. We evaluated maximum standardised uptake value (SUVmax) and PET score of the aortic wall for quantitative comparison of FDG-PET/CT findings. We measured the aortic wall thickness (W) and its ratio against the radius (W/R) for quantitative comparison of aortic wall thickening by contrast-enhanced CT. After steroid treatment, we compared these values with those pre-treatment. Of 124 patients who were hospitalised due to advanced age and IUO, 88 underwent FDG-PET/CT and contrast-enhanced CT. Abnormal findings were observed on images from 78 patients. The findings were indicative of LVV in 13 patients (10.5 %), of whom more than half had only non-specific symptoms. Patients with LVV had significantly higher aortic wall SUVmax (3.85 vs. 1.95), PET scores by FDG-PET/CT, and aortic wall thicknesses by contrast-enhanced CT (3.8 vs. 2.6 mm) than controls. Significant improvement in aortic wall thickening was evidenced by reduced PET scores and by contrast-enhanced CT findings in patients who were followed up after treatment. LVV is an important cause of IUO with non-specific symptoms in elderly patients. Imaging examination comprising contrast-enhanced CT and FDG-PET/CT is useful for early diagnosis and early treatment evaluation of LVV, allowing for amelioration of reversible aortic wall thickening. PMID:24643395

Muto, Go; Yamashita, Hiroyuki; Takahashi, Yuko; Miyata, Yoko; Morooka, Miyako; Minamimoto, Ryogo; Kubota, Kazuo; Kaneko, Hiroshi; Kano, Toshikazu; Mimori, Akio

2014-11-01

164

Use of SNOMED CT to Represent Clinical Research Data: A Semantic Characterization of Data Items on Case Report Forms in Vasculitis Research  

PubMed Central

Objective To estimate the coverage provided by SNOMED CT for clinical research concepts represented by the items on case report forms (CRFs), as well as the semantic nature of those concepts relevant to post-coordination methods. Design Convenience samples from CRFs developed by rheumatologists conducting several longitudinal, observational studies of vasculitis were selected. A total of 17 CRFs were used as the basis of analysis for this study, from which a total set of 616 (unique) items were identified. Each unique data item was classified as either a clinical finding or procedure. The items were coded by the presence and nature of SNOMED CT coverage and classified into semantic types by 2 coders. Measurements Basic frequency analysis was conducted to determine levels of coverage provided by SNOMED CT. Estimates of coverage by various semantic characterizations were estimated. Results Most of the core clinical concepts (88%) from these clinical research data items were covered by SNOMED CT; however, far fewer of the concepts were fully covered (that is, where all aspects of the CRF item could be represented completely without post-coordination; 23%). In addition, a large majority of the concepts (83%) required post-coordination, either to clarify context (e.g., time) or to better capture complex clinical concepts (e.g., disease-related findings). For just over one third of the sampled CRF data items, both types of post-coordination were necessary to fully represent the meaning of the item. Conclusion SNOMED CT appears well-suited for representing a variety of clinical concepts, yet is less suited for representing the full amount of information collected on CRFs. PMID:16799121

Richesson, Rachel L.; Andrews, James E.; Krischer, Jeffrey P.

2006-01-01

165

Androgen deficiency in male patients diagnosed with ANCA-associated vasculitis: a cause of fatigue and reduced health-related quality of life?  

PubMed Central

Introduction Low testosterone levels in men are associated with fatigue, limited physical performance and reduced health-related quality of life (HRQOL); however, this relationship has never been assessed in patients with anti-neutrophil cytoplasmic antibodies (ANCA) -associated vasculitides (AAV). The aim of this study was to assess the prevalence of androgen deficiency and to investigate the role of testosterone in fatigue, limited physical condition and reduced HRQOL in men with AAV. Methods Male patients with AAV in remission were included in this study. Fatigue and HRQOL were assessed by the multi-dimensional fatigue inventory (MFI)-20 and RAND-36 questionnaires. Results Seventy male patients with a mean age of 59 years (SD 12) were included. Scores of almost all subscales of both questionnaires were significantly worse in patients compared to controls. Mean total testosterone and free testosterone levels were 13.8 nmol/L (SD 5.6) and 256 pmol/L (SD 102), respectively. Androgen deficiency (defined according to Endocrine Society Clinical Practice Guidelines) was present in 47% of patients. Scores in the subscales of general health perception, physical functioning and reduced activity were significantly worse in patients with androgen deficiency compared to patients with normal androgen levels. Testosterone and age were predictors for the RAND-36 physical component summary in multiple linear regression analysis. Testosterone, age, vasculitis damage index (VDI) and C-reactive protein (CRP) were associated with the MFI-20 subscale of general fatigue. Conclusions This study showed that androgen deficiency was present in a substantial number of patients with AAV. Testosterone was one of the predictors for physical functioning and fatigue. Testosterone may play a role in fatigue, reduced physical performance and HRQOL in male patients with AAV. PMID:24028544

2013-01-01

166

Pulmonary lymphomatoid granulomatosis. Evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T-cell component and vasculitis.  

PubMed

Similarities have been noted in the histologic patterns of lymphomatoid granulomatosis and Epstein-Barr virus associated lymphoproliferative disease involving the lung. Epstein-Barr virus has also been identified by polymerase chain reaction in most cases of lymphomatoid granulomatosis; however, the precise cellular localization of Epstein-Barr virus sequences has not been extensively studied. We analyzed 10 cases of lymphomatoid granulomatosis involving the lung by immunohistochemistry and combined immunohistochemistry with in situ hybridization for Epstein-Barr virus, CD20, and CD45RO. All cases were selected from the files of the Armed Forces Institute of Pathology and met the clinical and histologic criteria for the diagnosis of lymphomatoid granulomatosis, grades 1 through 3. In all 10 cases, immunohistochemistry showed that most of the cells--small to medium-sized lymphocytes--were T cells (CD45RO+); however, a much smaller population of medium-sized to large atypical cells were B cells (CD20+). In each case, combined immunohistochemistry and in situ hybridization confirmed the presence of Epstein-Barr virus sequences within B (CD20+) cells and the absence of Epstein-Barr within T-cells (CD45RO+). Polymerase chain reaction analysis for immunoglobulin heavy-chain gene rearrangement identified a monoclonal pattern in six of nine cases tested, whereas analysis for T-cell receptor gamma-chain gene rearrangements was negative in three cases tested. On the basis of these findings, we hypothesize that most cases of lymphomatoid granulomatosis involving the lung represent a proliferation of Epstein-Barr virus infected B-cells with a prominent T-cell reaction and vasculitis, distinguishing these cases from angiocentric "T-cell lymphomas" in other sites, such as the head and neck. PMID:8037289

Guinee, D; Jaffe, E; Kingma, D; Fishback, N; Wallberg, K; Krishnan, J; Frizzera, G; Travis, W; Koss, M

1994-08-01

167

Clinical aspects of primary vasculitis  

Microsoft Academic Search

Conclusion  Vasculitic syndromes have now been recognized for some two centuries, and still pose diagnostic and other problems for clinicians.\\u000a In general, vasculitic syndromes cause substantial morbidity and mortality. Descriptions of larger series of patients, classification\\u000a efforts, and improved histological techniques have helped in dealing with these diseases. However, we still have to rely on\\u000a associations of certain clinical characteristics and

C. A. Stegeman; C. G. M. Kallenberg

2001-01-01

168

The “other” vasculitis syndromes and kidney involvement  

Microsoft Academic Search

There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and\\/or\\u000a are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of\\u000a this group as it involves vessels of any size on both the arterial and venous side. In addition to renal

Seza Ozen

2010-01-01

169

Use of imaging studies in the diagnosis of vasculitis.  

PubMed

Imaging studies are necessary to determine disease extension and disease activity in the small-vessel vasculitides. Computed tomography (CT) and magnetic resonance imaging (MRI) increase the number of pathologic findings compared with conventional radiography. MRI delineates mucosal inflammation and granulomas in the paranasal sinuses, whereas CT provides information about osseous lesions. CT is superior to MRI for the detection of pulmonary lesions. Radiograph angiography has been the gold standard for medium- and large-vessel vasculitides for decades. Echocardiography and MRI correspond well with conventional angiography to assess cardiac involvement in Kawasaki disease. MRI, CT, and CT angiography are alternative noninvasive techniques to delineate vasculitic lesions in polyarteritis nodosa, Takayasu's arteritis, and large-vessel giant cell arteritis. Duplex ultrasonography has the greatest resolution. It delineates typical artery wall swelling in temporal arteritis and Takayasu's arteritis. Positron emission tomography can assess inflammatory activity of large arteries. PMID:15134599

Schmidt, Wolfgang A

2004-06-01

170

Intradural vasculitis and hemorrhage in full sibling Welsh springer spaniels.  

PubMed

Two, full sibling, Welsh springer spaniel presented at 8 and 18 mo of age with rapidly progressive ataxia, recumbency, and pyrexia. The spinal cord contained extensive subdural hemorrhage and, in 1 dog, suppurative and necrotizing arteritis in the dura. The findings suggest a familial form of canine juvenile polyarteritis syndrome. PMID:12650042

Caswell, Jeff L; Nykamp, Stephanie G

2003-02-01

171

Intradural vasculitis and hemorrhage in full sibling Welsh springer spaniels  

PubMed Central

Two, full sibling, Welsh springer spaniels presented at 8 and 18 mo of age with rapidly progressive ataxia, recumbency, and pyrexia. The spinal cord contained extensive subdural hemorrhage and, in 1 dog, suppurative and necrotizing arteritis in the dura. The findings suggest a familial form of canine juvenile polyarteritis syndrome. PMID:12650042

Caswell, Jeff L.; Nykamp, Stephanie G.

2003-01-01

172

Therapy Insight: the recognition and treatment of retinal manifestations of systemic vasculitis  

Microsoft Academic Search

A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. The more prevalent of the systemic vasculitides are giant cell arteritis, polyarteritis nodosa, Wegener's granulomatosis,

Petros Aristodemou; Miles Stanford

2006-01-01

173

In vivo approaches to investigate ANCA-associated vasculitis: lessons and limitations  

Microsoft Academic Search

Anti-neutrophil cytoplasm autoantibody (ANCA)-associated diseases are autoimmune conditions characterized by necrotizing inflammation\\u000a of small blood vessels. The immunogenesis and etiology of these conditions are unknown, but our knowledge of the immunopathogenesis\\u000a has increased considerably in recent years. In this review, we discuss the animal models currently used to investigate the\\u000a mechanisms of vascular injury and to test novel therapies. We

Peter Heeringa; Mark A Little

2011-01-01

174

Circulating Angiopoietin-2 as a Biomarker in ANCA-Associated Vasculitis  

PubMed Central

The endothelial-specific Angiopoietin-Tie2 ligand-receptor system is an important regulator of endothelial activation. Binding of angiopoietin-2 (Ang-2) to Tie2 receptor renders the endothelial barrier responsive to pro-inflammatory cytokines. We previously showed that circulating Ang-2 correlated with disease severity in a small cohort of critically ill patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The current study reassessed Ang-2 as a biomarker of disease activity and relapse in AAV. Circulating Ang-2 was measured in 162 patients with severe AAV (BVAS/WG?3, with or without glomerulonephritis) in a clinical trial. Ang-2 levels during active AAV were compared to levels in the same patients during remission (BVAS/WG?=?0). Levels in clinical subsets of AAV were compared, and association with future disease course was assessed. Ang-2 levels were elevated in severe disease (median 3.0 ng/ml, interquartile range 1.9–4.4) compared to healthy controls (1.2, 0.9–1.5). However, they did not reliably decline with successful treatment (median 2.6 ng/ml, interquartile range 1.9–3.8, median change ?0.1). Ang-2 correlated weakly with BVAS/WG score (r?=?0.17), moderately with markers of systemic inflammation (r?=?0.25–0.41), and inversely with renal function (r?=??0.36). Levels were higher in patients with glomerulonephritis, but levels adjusted for renal dysfunction were no different in patients with or without glomerulonephritis. Levels were higher in patients with newly diagnosed AAV and lower in patients in whom treatment had recently been started. Ang-2 levels during active disease did not predict response to treatment, and Ang-2 levels in remission did not predict time to flare. Thus, Ang-2 appears to have limited practical value in AAV as a biomarker of disease activity at time of measurement or for predicting future activity. PMID:22279570

Monach, Paul A.; Kumpers, Philipp; Lukasz, Alexander; Tomasson, Gunnar; Specks, Ulrich; Stone, John H.; Cuthbertson, David; Krischer, Jeffrey; Carette, Simon; Ding, Linna; Hoffman, Gary S.; Ikle, David; Kallenberg, Cees G. M.; Khalidi, Nader A.; Langford, Carol A.; Seo, Philip; St. Clair, E. William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R.; Haubitz, Marion; Merkel, Peter A.

2012-01-01

175

Large vessel vasculitis without temporal artery involvement: Isolated form of giant cell arteritis?  

Microsoft Academic Search

Diffuse arterial involvement in giant cell arteritis (GCA) is well recognized. By contrast, GCA clinically isolated to large vessels without cephalic, rheumatologic or systemic symptoms represents a much rarer manifestation of the disease.

M. Lambert; A. Weber; B. Boland; J. F. Plaen; J. Donckier

1996-01-01

176

Recent Understanding on Diagnosis and Management of Central Nervous System Vasculitis in Children  

PubMed Central

Central nervous system vasculitides in children may develop as a primary condition or secondary to an underlying systemic disease. Many vasculitides affect both adults and children, while some others occur almost exclusively in childhood. Patients usually present with systemic symptoms with single or multiorgan dysfunction. The involvement of central nervous system in childhood is not frequent and it occurs more often as a feature of subtypes like childhood polyarteritis nodosa, Kawasaki disease, Henoch Schönlein purpura, and Bechet disease. Primary angiitis of the central nervous system of childhood is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures, and cognitive decline. The first line therapy of CNS vasculitides is mainly based on corticosteroids and immunosuppressor drugs. Other strategies include plasmapheresis, immunoglobulins, and biologic drugs. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitides in children including a tailored-diagnostic approach and new evidence regarding treatment. PMID:23008735

Iannetti, Ludovico; Zito, Roberta; Bruschi, Simone; Papetti, Laura; Ulgiati, Fiorenza; Nicita, Francesco; Del Balzo, Francesca; Spalice, Alberto

2012-01-01

177

occlusion or retinal ischemia commonly occurs in blood disorders, hypertension, retinal vasculitis, reti-  

E-print Network

right eye since childhood, and had had peripheral retinal vascular occlusion. Although the exact of night blindness, characterized by diffuse yellow spots and flecks in the fundus and by delayed dark adaptation.1 It is generally classed as one of the night-blinding disorders because the typical patient shows

Palczewski, Krzysztof

178

Vasculitis resulting from a superficial femoral artery angioplasty with a paclitaxel-eluting balloon.  

PubMed

Drug-eluting balloons (DEBs) coated with the antiproliferative agent paclitaxel may improve primary patency by reducing recurrent luminal stenosis. A proportion of the active drug and excipient coating are known to embolize distally, but until now, there have been no reports of adverse events resulting from their use. We report an unusual case of a painful nodular, biopsy specimen-proven vasculitic rash that afflicted the ipsilateral lower limb of a patient after superficial femoral artery treatment with a DEB. This adverse event may have implications for the use of DEB in this and other vascular territories. PMID:23642919

Thomas, Shannon D; McDonald, Robert R A; Varcoe, Ramon L

2014-02-01

179

Non-necrotizing Granulomatous Pulmonary Vasculitis Mimicking Lung Cancer on PET/CT  

PubMed Central

Fluorodeoxyglucose positron emission tomography (18FDG-PET) scan has become a valuable resource in the staging of lung cancer. Inflammation is known to cause false positives on 18FDG-PET scan. In the absence of symptoms suggesting a diagnosis of an inflammatory condition, 18FDG-avid lung masses on PET/CT scan is strongly suggestive of a diagnosis of lung cancer, rather than an inflammatory condition. We report the case of a 57-year-old man, with a history of heavy smoking and working in the sandblasting industry, with two suspicious 18FDG-avid nodules in the left lung. Surgical specimens of these nodules revealed findings suspecting giant cell arteritis rather than malignancy. PMID:25360406

Dixon, Matthew; Shaw, Jason; Rankin, Linda; Lazzaro, Richard

2013-01-01

180

Vasculitis reminds us that 'Ockham's razor' may still apply in the elderly.  

PubMed

A 76-year-old woman with a history of stage IV chronic kidney disease and hypertension was admitted to the hospital for progressive weakness and acute on chronic kidney injury. She was found to have anaemia requiring transfusion. On the evening of admission, she had worsening respiratory status with subsequent diagnosis of a non-ST-elevation myocardial infarction, pulmonary oedema on chest x-ray presumed to be a result of heart failure, and a possible transfusion reaction. A kidney biopsy performed as part of her ongoing nephrology evaluation revealed granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). Subsequent bronchoalveolar lavage confirmed diffuse alveolar haemorrhage as the explanation for her anaemia and respiratory decline. She underwent immunosuppressive therapies and initiated dialysis, but subsequently chose hospice care and died 2 months after her diagnosis. PMID:23505087

Mount, Hillary R

2013-01-01

181

Cutaneous granulomatous vasculitis associated with chronic lymphocytic leukemia: Case report and review of literature of a rare entity  

E-print Network

autoimmune diseases (rheumatoid arthritis, Wegener granulomatosis, Churg-Strauss syndrome/allergic granulomatosis), inflammatory/granulomatous disorders (sarcoidosis, inflammatory bowel disease), and infections (chronic hepatitis,

Gaertner, Erich M; Switlyk, Stephen A

2012-01-01

182

A man with small vessel vasculitis presenting with brachial diplegia, multiple cranial mononeuropathies and severe orthostatic hypotension in diabetes mellitus: a case report  

PubMed Central

Introduction We report a rare case of fulminant vasculitic mononeuropathy resulting in brachial diplegia, with suspected brainstem and autonomic nervous system involvement in a patient with diabetes mellitus. Case presentation A 58-year-old Hispanic Caucasian man with diabetes mellitus presented with a 1-year history of progressive bilateral upper extremity weakness, orthostatic intolerance and progressive memory decline. Diagnostic evaluation including laboratory tests for progressive encephalopathies, systemic inflammatory and non-inflammatory neuropathies, cerebrospinal fluid analyses, electrodiagnostic studies, and nerve biopsy were performed. Clinical examination revealed moderate cognitive deficits on the Montreal Cognitive Assessment scale, bilateral facial weakness and weakness of bilateral shoulder girdle and intrinsic hand muscles. Cerebrospinal fluid analyses revealed elevated protein and an elevated immunoglobulin G synthesis rate, suggesting an immune-mediated process. Further laboratory work up was non-diagnostic. Electrodiagnostic studies demonstrated chronic asymmetric axonal mononeuropathies with ongoing denervation. A superficial radial nerve biopsy showed a chronic vasculitic neuropathy. Glucocorticosteroid treatment, symptomatic pharmacologic and supportive non-pharmacologic therapies resulted in improved clinical outcomes despite challenges with glycemic control. Conclusions This case report emphasizes the importance of a thorough evaluation of atypical or uncommon neuromuscular presentations in diabetic patients without etiological presumptions. This is necessary in order to promptly establish a diagnosis, initiate appropriate therapies and prevent irreversible nerve injury. PMID:24083508

2013-01-01

183

Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial  

E-print Network

months. Both regimens are preceded by IV methyl- prednisolone of between 1 and 3 grams. Oral GC may be provided as prednisolone or prednisone according to local practice. Table 1 Glucocorticoid dosing in the standard and reduced-dose groups of PEXIVAS... in rheumatoid arthritis: an analysis of serious adverse events. Am J Med 1994, 96:115–123. 23. Wolfe F, Caplan L, Michaud K: Treatment for rheumatoid arthritis and the risk of hospitalization for pneumonia: associations with prednisone, disease...

Walsh, Michael; Merkel, Peter A; Peh, Chen Au; Szpirt, Wladimir; Guillevin, Loïc; Pusey, Charles D; deZoysa, Janak; Ives, Natalie; Clark, William F; Quillen, Karen; Winters, Jeffrey L; Wheatley, Keith; Jayne, David; on behalf of the PEXIVAS Investigators

2013-03-14

184

Interstitial granulomatous dermatitis  

E-print Network

interstitial granulomatous dermatitis with arthritis includes granuloma annulare, urticarial vasculitis, erythema chronicum migrans (Lyme borreliosis), rheumatoid neutrophilic dermatosis, mycosis fungoides (

Johnson, Hillary; Mengden, Stephanie; Brancaccio, Ronald R

2008-01-01

185

Palpable purpura and uveitis precipitated by splenectomy in an atypical case of Cogan's syndrome.  

PubMed

A patient with a baffling multisystem illness developed uveitis and palpable purpura shortly after a splenectomy, leading to the diagnosis of vasculitis. Clearance by the mononuclear phagocyte system is a key factor determining the pathogenicity of circulating immune complexes, which are known to be the cause of some forms of vasculitis. The sudden appearance of uveitis and cutaneous vasculitis in an illness of many years' duration was probably a direct result of removal of the spleen. PMID:1920316

Wohlgethan, J R; Stilmant, M M; Smith, H R

1991-07-01

186

Genetics Home Reference: Complement component 2 deficiency  

MedlinePLUS

... also associated with an increased risk of developing autoimmune disorders such as systemic lupus erythematosus (SLE) or vasculitis. Autoimmune disorders occur when the immune system malfunctions and attacks ...

187

Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases  

ClinicalTrials.gov

Other Specified Inflammatory Disorders of Skin or Subcutaneous Tissue; Pyoderma Gangrenosum; Erosive Pustular Dermatosis of the Scalp; Sweet's Syndrome; Behcet's Disease; Bowel-associated Dermatosis-arthritis Syndrome; Pustular Psoriasis; Acute Generalized Exanthematous Pustulosis; Keratoderma Blenorrhagicum; Sneddon-Wilkinson Disease; IgA Pemphigus; Amicrobial Pustulosis of the Folds; Infantile Acropustulosis; Transient Neonatal Pustulosis; Neutrophilic Eccrine Hidradenitis; Rheumatoid Neutrophilic Dermatitis; Neutrophilic Urticaria; Still's Disease; Erythema Marginatum; Unclassified Periodic Fever Syndromes / Autoinflammatory Syndromes; Dermatitis Herpetiformis; Linear IgA Bullous Dermatosis; Bullous Systemic Lupus Erythematosus; Inflammatory Epidermolysis Bullosa Aquisita; Neutrophilic Dermatosis of the Dorsal Hands (Pustular Vasculitis); Small Vessel Vasculitis Including Urticarial Vasculitis; Erythema Elevatum Diutinum; Medium Vessel Vasculitis

2014-06-11

188

Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients  

Microsoft Academic Search

BackgroundMixed cryoglobulinemia (MC) is a systemic vasculitis secondary to circulating immune complex deposition in the small vessels. In the overwhelming majority of patients, hepatitis C virus (HCV) infection represents the triggering factor of the disease. MC is characterized by multiple organ involvement, mainly skin, liver, renal, peripheral nerves, and less frequently by widespread vasculitis and cancer.

Clodoveo Ferri; Marco Sebastiani; Dilia Giuggioli; Massimiliano Cazzato; Giovanni Longombardo; Alessandro Antonelli; Rodolfo Puccini; Claudio Michelassi; Anna Linda Zignego

2004-01-01

189

Sweet's syndrome: histological and immunohistochemical study of 15 cases  

Microsoft Academic Search

Conventional histology and immunoperoxidase staining for fibrin, immunoglobulins, and complement components were used to look for evidence of cutaneous vasculitis and immune complex deposition in Sweet's syndrome. These features were not identified in any of the 15 cases studied. The lack of any vasculitis emphasises the distinctive character of Sweet's syndrome when compared with certain spontaneous and experimentally induced inflammatory

J J Going; S M Going; G W Beveridge

1987-01-01

190

Vasculitides in hairy cell leukemia  

Microsoft Academic Search

Forty-two cases of vasculitis coincident with hairy cell leukemia (HCL) havebeen reported, of which 17 had panarteritis nodosa (PAN), 21 had cutaneous leukocytoclastic vasculitis (LCV), and 4 had vessel wall infiltration by hairy cells. PAN generally occurred after the diagnosis of HCL, splenectomy, and infection. HBs antigen was detected in 3 of 12 patients tested, whereas immune complexes were positive

Paul Hasler; Hansjörg Kistler; Heini Gerber

1995-01-01

191

Hairy cell leukemia presenting initially with symptoms of Behçet's disease.  

PubMed

Vasculitis is relatively uncommon in lymphoproliferative disease and may predate the diagnosis of lymphoproliferative disease. Many vasculitides have been associated with hairy cell leukemia (HCL), including polyarteritis nodosa (PAN) and leukocytoclastic vasculitis. We herein report a case whose initial presentation was like Behçet's disease (BD) (arthritis, oral and genital ulcerations, papulopustular skin lesions) in addition to pancytopenia, but turned out to have HCL. Because of the overlap between their symptoms, like oral ulcerations, skin lesions, arthritis and constitutional findings, HCL and BD may mimic each other. We should keep in mind other reasons for vasculitis such as lymphoproliferative disease, especially whose who have hematological abnormalities such as pancytopenia. PMID:24762098

Oksuz, Mustafa Ferhat; Coskun, Belkis Nihan; Tufan, Ayse Nur; Orucoglu, Nurdan; Dalkilic, Ediz; Oztürk Nazl?o?lu, Hülya; Pehlivan, Yavuz

2014-07-01

192

Isolated Aortitis  

MedlinePLUS

... results will open in a new tab or window. Search MedlinePlus : Participation in Clinical Trials is one way you can help advance research into finding better treatments and perhaps even a cure for vasculitis! VCRC- ...

193

Cryoglobulinemia  

MedlinePLUS

... results will open in a new tab or window. Search MedlinePlus : Participation in Clinical Trials is one way you can help advance research into finding better treatments and perhaps even a cure for vasculitis! VCRC- ...

194

Histology Self-Study Presentation Ellen O'Dea  

E-print Network

. Systemic lupus erythematosus (SLE) 3. Scleroderma (Systemic sclerosis) 4. Crystal induced disease (Gout, Autoimmune disease Division of Rheumatology, Allergy,and Immunology Main diseases: 1. Rheumatoid arthritis 2: Rheumatoid Arthritis Systemic Lupus Erythematosus Sclerosis Gout Vasculitis #12;Lymphoid System Cells

Gleeson, Joseph G.

195

38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.  

Code of Federal Regulations, 2010 CFR

...coagulation with renal cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction....

2010-07-01

196

Spontaneous ureteral rupture in a patient with systemic lupus erythematosus  

SciTech Connect

A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.

Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.

1983-08-01

197

Giant Cell Arteritis  

MedlinePLUS

... polymyalgia rheumatica (also called PMR). What is giant cell arteritis? GCA is a type of vasculitis or ... right away to their doctors. What causes giant cell arteritis? As with PMR, the cause of GCA ...

198

38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.  

Code of Federal Regulations, 2011 CFR

...7540Disseminated intravascular coagulation with renal cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate...

2011-07-01

199

A Real World, Observational Registry of Chronic Wounds and Ulcers  

ClinicalTrials.gov

Diabetic Foot; Varicose Ulcer; Pressure Ulcer; Surgical Wound Dehiscence; Vasculitis; Skin Ulcer; Leg Ulcer; Wounds and Injuries; Pyoderma; Peripheral Arterial Disease; Diabetic Neuropathies; Lymphedema; Venous Insufficiency; Diabetes Complications; Amputation Stump

2014-10-29

200

Histological findings after brown recluse spider envenomation.  

PubMed

Histologic specimens from 41 rabbits were studied for changes resulting from the manual injection of brown recluse spider venom. Major findings included a mixed inflammatory cell infiltrate, coagulative tissue necrosis, and vasculitis. All specimens demonstrated a well-delineated zone of eosinophilic staining recognizable as "mummified" coagulative necrosis of the epidermis and dermis. A dense band of neutrophils bordered the zone of necrosis. Immediately adjacent to the neutrophilic band, small vessel vasculitis was a universal finding. Degranulated eosinophils and neutrophils and macrophages filled with eosinophilic granules were common. Inflammatory foci were often centered on groups of lipocytes within the dermis. Large vessel vasculitis resembling that seen in polyarteritis nodosa was present deep to 7 of the 40 eschars. Large vessel vasculitis may contribute to the large zones of necrosis seen after some brown recluse spider bites. Eosinophils may play a role in tissue damage after envenomation. PMID:10871067

Elston, D M; Eggers, J S; Schmidt, W E; Storrow, A B; Doe, R H; McGlasson, D; Fischer, J R

2000-06-01

201

Methotrexate  

MedlinePLUS

... Specific forms of vasculitis treated with MTX include Wegener’s granuloma t o s i s , Microscopic polyangiitis, Churg-Strauss ... 000/mm3. Do I need to take an antibiotic? Possibly. Depending on how much medication you take ...

202

Genetics Home Reference: Granulomatosis with polyangiitis  

MedlinePLUS

... granulomas grow, they can invade surrounding areas, causing tissue damage. The signs and symptoms of GPA vary based ... kidneys are commonly affected in people with GPA. Tissue damage caused by vasculitis in the kidneys can lead ...

203

Sequential strokes in a hyperacute stroke unit  

PubMed Central

Vasculitis is a rare, but treatable condition that can present to hyperacute stroke units. Thrombolysis does not treat the underlying pathology, and a rapidly evolving clinical picture drives clinical decision often before all the investigation results are available.

Ganesalingam, Jeban; Buddha, Sandeep; Carlton-Jones, Anoma L

2014-01-01

204

POLYNEUROPATHY + NEURONOPATHY: Points in Differential Diagnosis 1. Selective Functional Involvement  

E-print Network

; Horner's Treatable neuropathies Diabetes; Vasculitis; CIDP; Lymphoma; Infection; Toxic; Compression Motor Motor Neuropathy: Multifocal (MMN); Distal Motor Neuron Disorders Lower Motor Neuron (LMN): BVv Toxic: Pb; Dapsone; Botulism; Tick Acute: Porphyria; Axonal GBS (AMAN); Polio; West Nile Diabetic

Baloh, Bob

205

Cyclophosphamide (Cytoxan)  

MedlinePLUS

... forms of vasculitis that do not respond to steroids ( prednisone ) alone, cyclophosphamide may be a life–saving medicine. ... a fatal illness, even with high doses of steroids. Now, with the combination of cyclophosphamide and prednisone, ...

206

Popliteal artery thrombosis in a patient with Cogan syndrome: Treatment with thrombolysis and percutaneous transluminal angioplasty  

SciTech Connect

A 31-year-old woman with Cogan syndrome (a rare form of systemic vasculitis) was evaluated for a cold, painful left foot with diminished pulses. Arteriography demonstrated thrombosis of the left popliteal artery with evidence of vasculitis. Thrombolytic therapy was begun with initial success but eventual rethrombosis. After reinitiating thrombolytic therapy combined with intraarterial vasodilator therapy, successful angioplasty was performed with sustained results, at 6-month follow-up.

Bastug, Demir E.; Dominic, Anthony; Ortiz, Orlando [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); DiBartolomeo, Anthony G. [Robert C. Byrd Health Sciences Center, Department of Medicine, Rheumatology Section, West Virginia University (United States); Kotzan, Jeffrey M. [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); Abraham, F. Matthew [Robert C. Byrd Health Sciences Center, Department of Medicine, West Virginia University (United States)

1997-01-15

207

Facial heliotrope rash as the initial manifestation of acute myelomonocytic leukemia.  

PubMed

The association of leukocytoclastic vasculitis or dermatomyositis with malignancies has been reported. We describe a patient who developed a skin rash, histologically compatible with dermatomyositis, which during the course of the disease switched to leukocytoclastic vasculitis, which was accompanied with peripheral blood pancytopenia in the absence of any specific pathological manifestation from the bone marrow three years prior to the diagnosis of acute myelomonocytic leukemia (AMML). PMID:9168450

Tzavara, V; Stamoulis, K; Aroni, K G; Kordossis, T; Boki, K A

1997-04-01

208

Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis).  

PubMed

Granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis) is an autoimmune small vessel vasculitis which is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). The hallmarks of this condition are systemic necrotising vasculitis, necrotising granulomatous inflammation, and necrotising glomerulonephritis. The aetiology of granulomatosis with polyangiitis is linked to environmental and infectious triggers inciting onset of disease in genetically predisposed individuals. Anti-neutrophil cytoplasmic antibodies are pathogenic and play an important role in the pathogenesis of this disease, although ANCA positivity is not essential for a clinical diagnosis of granulomatosis with polyangiitis. Granulomatosis with polyangiitis is diagnosed based on clinical manifestations of systemic vasculitis and histological evidence of necrotising vasculitis or granulomatous inflammation. This small vessel vasculitis may present as limited disease of the ears, nose and upper airways or mild, moderate or severe systemic disease. Immunosuppression and adjuvant therapies have contributed to the improved prognosis of granulomatosis with polyangiitis over the past decades. Treatment strategies are tailored to the severity of the disease. They are based on published evidence of the efficacy and safety of the immunosuppressive drugs indicated to manage active vasculitis and maintain clinical remission. This review will summarise the history, aetiology, pathogenesis, classification, diagnosis and management of granulomatosis with polyangiitis. PMID:24485158

Lutalo, Pamela M K; D'Cruz, David P

2014-01-01

209

Bilateral renal ischemia after kyphoplasty and clodronate treatment: a case report  

PubMed Central

Introduction The most common adverse effects associated with bisphosphonates are renal toxicity, acute-phase reactions, gastrointestinal toxicity, osteonecrosis of the jaw, transitory fever and uveitis. We report a unique adverse case of vasculitis induced by clodronate. Case presentation A 61-year-old Caucasian woman developed bilateral renal ischemia after kyphoplasty and clodronate treatment for lumbar vertebral fracture. Tests revealed a vasculitis due to clodronate treatment. The antithrombotic and immunosuppressive drugs allowed us to reduce the extent of the renal ischemia. In the following months the increased auto-antibodies returned to the healthy physiological range, but the chronic renal failure persisted. Conclusions Drug-induced vasculitis is an inflammation of blood vessels caused by the use of various pharmaceutical agents. The spectrum of drug-induced vasculitis can range from cutaneous rashes to fatal multi-organ involvement. To the best of our knowledge this is the first documented case of drug-induced vasculitis caused by clodronate in the literature. Previously, it was verified that clodronate injection could increase the pro-apoptotic action on immune cells. Further studies are necessary to clarify the role of bisphosphonates on drug-inducing vasculitis. PMID:24571672

2014-01-01

210

A case of palpable purpura and nephropathy: Occam's Razor or Hickam's Dictum.  

PubMed

Vasculitis causing palpable purpura, nephropathy, and hematologic abnormalities is a well-known entity. However, sometimes, vasculitis may not be the primary cause but is part of a systemic disease. Literature suggests that infections like HIV can induce nephropathy and antineutrophilic cytoplasmic antibody-positive vasculitis, which is different from the well-known entity of "antineutrophilic cytoplasmic antibody-associated vasculitis." We present a 46-year-old female patient with a history of intravenous drug abuse who reported with a rash, swelling, and palpable purpura of the lower extremities. Peripheral smear showed no evidence of disseminated intravascular coagulation or thrombotic thrombocytopenic purpura; metabolic profile showed acute kidney injury. She was found to be HIV- and hepatitis C-positive. Immunologic workup was positive for both MPO and PR3 antineutrophilic cytoplasmic antibodies and negative for cryoglobulins; complement levels were low. Skin biopsy showed leukocytoclastic vasculitis but kidney biopsy was negative for any immunologic involvement; it showed only glomerulosclerosis. Thus, it was thought that nephropathy and vasculitis, in this case, are two distinct pathologic processes, both induced by infection (HIV and/or hepatitis C). The patient responded to low-dose steroid therapy. She was later started on the definitive therapy, the highly active antiretroviral therapy regimen. This case illustrates the fact that low-dose steroids can still be a good alternative in acute situations in patients at risk from immunosuppression. PMID:21248615

Mandhadi, Ranadeep; Kodumuri, Vamsi; Arora, Rohit; Puneet Singh, Param; Adigopula, Shashi; Chua, Serafin

2013-01-01

211

Current concept and epidemiology of systemic vasculitides.  

PubMed

Although a new classification algorithm for systemic vasculitides was proposed by Watts et al. and the Chapel Hill Consensus Conference (CHCC) was updated in 2012, there are currently no validated diagnostic criteria for systemic vasculitides. The Diagnostic and Classification Criteria for Vasculitis study (DCVAS) is a global study to develop and improve the diagnostic criteria for systemic vasculitides. The epidemiology of systemic vasculitides differs widely among countries. For example, in the case of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, patients with microscopic polyangiitis (MPA) and with positivity for MPO-ANCA are predominant in Asian countries, whereas patients with granulomatosis with polyangiitis (GPA) and with positivity for PR3-ANCA are predominant in northern Europe and the United States. Interstitial lung disease (ILD) occurs more frequently in Asian patients compared with patients in Europe. The incidence and the prevalence of large-vessel vasculitis also differ significantly. Giant cell arteritis (GCA) occurs frequently in northern Europe, unlike Takayasu arteritis (TAK). The ethnic and regional differences in the incidence, prevalence and clinical characteristics of patients with vasculitis should be recognized when we diagnose and treat patients with vasculitis using criteria, and should also be considered when interpreting the results from clinical studies. PMID:25339434

Katsuyama, Takayuki; Sada, Ken-Ei; Makino, Hirofumi

2014-12-01

212

Fever of unknown origin in a very old patient: beware of the kidney!  

PubMed

ANCA-associated vasculitis affects more than 20 per million of the population per year and prevails in the elderly. Renal involvement, either isolated or in the context of systemic vasculitis, is common. We report the case of an 86-year-old patient who presented with a histologically proven renal limited vasculitis and with fever and fatigue but with normal renal function and urine analysis. Serum creatinine increased and microscopic hematuria became evident only 3 weeks after symptoms onset, whereas ANCA positivity was the only early key to the diagnosis. This case shows that in the elderly an ANCA-related pauci-immune glomerulonephritis in its earliest stages should be suspected in the presence of a fever of unknown origin even if serum and urine analyses are normal. PMID:24569957

Giua, Renato; Pedone, Claudio; Onetti Muda, Andrea; Antonelli Incalzi, Raffaele

2014-10-01

213

An 11-year and 10-month-old girl with purpura and chest pain.  

PubMed

Mucosa-associated lymphoid tissue lymphoma (MALToma) is a type of B-cell lymphoma. Case reports of childhood thymic MALToma and its association with vasculitis are rarely found in the related literature. Herein, we present a report of an 11-year and 10-month-old girl who was initially diagnosed with cutaneous vasculitis characterized by nonthrombocytopenic palpable purpura, positive antinuclear antibody and anti-SSA (Ro) antibody. Eight months later, a thymic mediastinal mass was found. Surgical excision was performed and results of pathological analysis revealed an extranodal marginal zone CD20(+) B-cell MALToma. Benign response to the chemotherapeutic regimen of Berlin-Frankfurt-Münster group NHL-BFM 90 R2 without relapse was noted in 2 years of follow-up. For the first time, our case demonstrated some clinical evidence of the association between vasculitis and childhood MALToma. PMID:23006506

Chen, Pei-Hsuan; Chiang, Bor-Luen; Lu, Meng-Yao; Yang, Yao-Hsu

2014-10-01

214

What's wrong with this artery? A medical disease discovered by a surgeon.  

PubMed

A 50-year-old man presenting with chest pain had positive stress echocardiography; and angiogram showed single artery coronary stenosis, presumed to be atherosclerotic. He was started on optimal medical therapy with good compliance. Four months later, he had a myocardial infarction (MI) and cardiac catheterisation surprisingly showed interval development of severe three-vessel stenosis. He underwent coronary artery bypass grafting (CABG), during which the cardiothoracic surgeon noticed severely inflamed coronary arteries, concerning for vasculitis. Following CABG, the patient continued to have chest pain and was admitted again for MI within 4?months of surgery. Subsequent autoimmune workup was consistent with sarcoidosis. He was started on immunosuppressive therapy for presumed sarcoid-related coronary vasculitis, and 23?months later, the patient has not developed further ischaemic events. This is a rare case and extends the clinical spectrum of cardiac sarcoidosis, presenting with rapidly progressive coronary stenosis most likely due to vasculitis, mimicking atherosclerotic coronary artery disease. PMID:25246466

Singh, Vasvi; Luthra, Saurav; Kouides, Ruth; Gadir, Abdel K

2014-01-01

215

Cryoglobulins  

PubMed Central

Serum cryoglobulins are found in a wide spectrum of disorders but are often transient and without clinical implications. Monoclonal cryoglobulins are usually associated with haematological disorders, whereas mixed cryoglobulins are found in many infectious and systemic disorders. So called essential mixed cryoglobulinaemia shows a striking association with hepatitis C virus (HCV) infection (> 90%). It is a systemic vasculitis (leucocytoclastic vasculitis) with cutaneous and multiple visceral organ involvement. Chronic HCV infection can lead to a constellation of autoimmune and neoplastic disorders. In this review, the aetiology, diagnosis, disease heterogeneity, and treatment of cryoglobulinaemia are discussed. PMID:11825916

Ferri, C; Zignego, A L; Pileri, S A

2002-01-01

216

Takayasu Arteritis in a Young Woman  

PubMed Central

Takayasu arteritis is a chronic, progressive, autoimmune, idiopathic, large-vessel vasculitis that usually affects young adults. The disease has been reported to occur in all races and ethnicities. The diffuse nature of this vasculitis can affect multiple-organ systems to varying degrees. Herein, we report the case of a young woman whose exertional angina and claudication were the initial presentation of active Takayasu arteritis. During more than 4 years of ongoing treatment, therapy, and follow-up, she has displayed differing disease symptoms of varying intensity. We discuss the challenges of managing Takayasu arteritis in our patient and describe different treatments for this rare vasculitic disorder. PMID:19876432

Saab, Fadi; Giugliano, Robert P.; Giugliano, Gregory R.

2009-01-01

217

Allertic complications of meningococcal disease. I. Clinical aspects.  

PubMed

Out of 717 patients with meningococcal disease 53 showed one or more of the three allergic complications: 47 (6.6%) developed arthritis, 12 (1.7%) developed cutaneous vasculitis, and 6 developed episcleritis. These complications, which were often multiple, occurred six to nine days after the beginning of the illness and three to six days after the start of successful antibiotic therapy. Those patients with severe systemic disease were prone to the complications.Histological and bacteriological study of the arthritis and vasculitis showed that these lesions were probably not due to persisting infection and suggested that they might be due to immune complex disease. PMID:4268766

Whittle, H C; Abdullahi, M T; Fakunle, F A; Greenwood, B M; Bryceson, A D; Parry, E H; Turk, J L

1973-06-30

218

Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis  

PubMed Central

Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease. PMID:24124396

Shahedi, Kamyar; Hanna, Ramy Magdy; Melamed, Oleg; Wilson, James

2013-01-01

219

Lingual infarction in Wegener's Granulomatosis: A case report and review of the literature  

Microsoft Academic Search

Wegener's granulomatosis (WG) is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding

Lachlan M Carter; Eitan Brizman

2008-01-01

220

Superantigens and their Association with Dermatological Inflammatory Diseases: Facts and Hypotheses  

Microsoft Academic Search

Superantigens have been suggested to play an important role in the pathogenesis of several inammatory skin diseases as well as systemic diseases such as atopic dermatitis, psoriasis, vasculitis, T-cell lymphoma and autoimmune diseases. Infections often precede the onset and relapse of these diseases, and antibiotic treatment with or without additional glucocorticosteroids and immunoglobulins is occasionally successful. Superantigens are microbial proteins

Uta Jappe

2000-01-01

221

Behçet's disease in a patient with immunodeficiency virus infection  

Microsoft Academic Search

A patient with human immunodeficiency virus (HIV) infection who developed Behçet's disease is described. As various vasculitis syndromes have been encountered recently in association with HIV infection it is suggested that Behçet's disease may be related to the HIV infection in this patient.

D Buskila; D D Gladman; J Gilmore; I E Salit

1991-01-01

222

Polyarteritis nodosa presented as a dilatation of the intrahepatic bile duct.  

PubMed

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of small- and medium-sized arteries in multiorgan systems. PAN may affect the gastrointestinal tract in 14%-65% of patients, but rarely involves the biliary tract and liver. We describe a patient without underlying disease who was diagnosed with PAN during resection of the gallbladder and liver. PMID:25368854

Choi, Han Lim; Sung, Ro Hyun; Kang, Min Ho; Jeon, Hyun Jeong; Yun, Hyo Yung; Jang, Lee Chan; Choi, Jae Woon; Song, Young Jin; Ryu, Dong Hee

2014-11-01

223

Polyarteritis nodosa presented as a dilatation of the intrahepatic bile duct  

PubMed Central

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of small- and medium-sized arteries in multiorgan systems. PAN may affect the gastrointestinal tract in 14%-65% of patients, but rarely involves the biliary tract and liver. We describe a patient without underlying disease who was diagnosed with PAN during resection of the gallbladder and liver. PMID:25368854

Choi, Han Lim; Sung, Ro Hyun; Kang, Min Ho; Jeon, Hyun Jeong; Yun, Hyo Yung; Jang, Lee Chan; Choi, Jae Woon; Song, Young Jin

2014-01-01

224

Influences of Chloride and Hypochlorite on Neutrophil Extracellular Trap Formation  

E-print Network

such as small vessel vasculitis, lupus and cystic fibrosis lung disease. Reactive oxygen species (ROS at tissue foci of infection. NETs have also been implicated in the pathogenesis of non-infectious diseases patients with chronic granulomatous disease or myeloperoxidase (MPO) deficiency, produce fewer NETs

Nizet, Victor

225

Behcet's Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment  

PubMed Central

Behcet's disease (BD) is the only systemic vasculitis involving both arteries and vein in any sizes. It frequently encounters in rheumatology clinics. It has some major morbidities and even fatal outcomes in some cases. The aim of this paper is to analyze the main concepts on pathophysiology and treatment options in BD, focusing on cardiovascular aspects, thrombosis, and potential future treatment. PMID:22530146

Owlia, M. B.; Mehrpoor, G.

2012-01-01

226

Takayasu arteritis in children  

Microsoft Academic Search

Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation

Safia Al abrawi; Marine Fouillet-Desjonqueres; Louis David; Xavier Barral; Pierre Cochat; Rolando Cimaz

2008-01-01

227

Enfermedad de Adamantiades-Behçet: Un proceso enigmático con manifestaciones orales  

Microsoft Academic Search

Adamantiades-Behçet disease (ABD) is a chronic multisystemic vasculitis that is able to affect any human organ or system. Recurrent oral ulcers are a very important clinical sign. ABD is a worldwide pathology, which prevalence varies according to the population and geographic location. Although ABD has been known for ages, its aetiology remains an enigma. Genetic, immunological and microbiological factors have

Asier Eguia; Mariana Villarroel; Rafael Martínez-Conde; María Ángeles Echebarría; José Manuel Aguirre

228

La troponina I nella malattia di Kawasaki Troponin I in Kawasaki disease  

Microsoft Academic Search

Background. Kawasaki's disease is a multisystemic vasculitis affecting small- and medium-caliber arteries, and in 20-25% of patients coronary arteries, too. The diagnosis of the disease is clinical. In its incomplete form, not all clinical criteria are pre- sent, but heart involvement is evident. An early, sensitive and specific biochemical marker of myocardial damage could be useful in the diagnostic and

G. Cremaschini; R. Ferrari; T. Bignotti; F. Ricci; S. Fonte; L. D. Notarangelo

2007-01-01

229

Churg-strauss syndrome in childhood: a case report.  

PubMed

Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome. PMID:24170259

Albahri, Ziad; Minxová, Lenka; Lukeš, Antonín; Mawiri, Abdul Al; Stefá?ková, Sárka

2014-10-01

230

Granuloma annulare with a mycosis fungoides–like distribution and palisaded granulomas of CD68-positive histiocytes  

Microsoft Academic Search

We describe 3 unusual cases of granuloma annulare with multiple macular lesions in a distribution that simulated mycosis fungoides in patients with no associated underlying diseases. Repeated biopsies showed typical well-formed palisading granulomas and no evidence of an atypical lymphocytic infiltrate. There was no vasculitis, neutrophilic, eosinophilic, or interstitial infiltrate. The patients had no associated underlying diseases. Most of the

Hong Wu; Alan Barusevicius; Stuart R Lessin

2004-01-01

231

UniversityofConnecticutHealthCenter 263FarmingtonAvenue  

E-print Network

and #12;VASCUlAR DISEASE m ay 2011 | www.uchc.edu The UConn Health Center is the site of the first procedure performed in the Hartford area to repair an abdominal aneurysm without using a surgical incision. "Since there is no incision, recovery time is very short," says Dahn. "The infection rate is also lower

Oliver, Douglas L.

232

Small-Cell Carcinoma as a Cause of Superior Vena Cava Syndrome in a Patient with Behçet’s Disease  

Microsoft Academic Search

Behçet's disease (BD) is a multisystem disorder characterized by vasculitis. The first description consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. Three forms of vascular disease (venous occlusions, arterial aneurysms and\\/or arteri- al occlusions) are found in BD (1, 2). The coexistence of neoplasia and BD is not common, and there are

Tekin Akpolat; Levent Yildiz; Ilkser Akpolat; Mustafa Bekir Selcuk; Nilgün Ozbek; Idris Yücel

2000-01-01

233

A case of idiopathic tracheal stenosis  

PubMed Central

Acquired tracheal stenosis is a condition that often results from trauma, neoplasm, infection, vasculitis, inflammatory or infiltrative processes. Idiopathic tracheal stenosis is a rare entity and represents a diagnosis of exclusion. We report a case of severe localized extrathoracic tracheal stenosis in a 35 year old female who was initially suspected to have asthma as the cause of several months of chronic dyspnea.

Apostolova, M.; Zeidan, B.

2013-01-01

234

Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases  

ClinicalTrials.gov

Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

2005-06-23

235

[Vasculitic peripheral neuropathy].  

PubMed

The typical clinical manifestation of vasculitic peripheral neuropathy is sensory-dominant multiple mononeuropathy, although it can progress to distal-dominant sensorimotor polyneuropathy. It is painful in most cases. Peripheral nerves may be the most prone to produce symptoms of the vasculitis. Nerve conduction studies show reduced amplitude of M wave or sensory nerve action potential, which depends on the degree of injury of a nerve examined. Wallerian degeneration can cause pseudo-conduction block in the acute stage and temporal dispersion in the chronic stage. However, a definite diagnosis requires histological confirmation. Combined biopsy of the sural nerve and the peroneus brevis muscle can be performed by a single incision. Skin biopsy can also be performed. To increase the diagnostic yield, biopsy specimens are prepared in different manners to observe as many cross sections as possible: frozen unfixed, formalin-fixed paraffin-embedded, and glutaraldehyde-fixed epon embedded specimens, as well as teased fiber preparation of a nerve. Vasculitic peripheral neuropathy usually results from small-vessel vasculitis. There are still controversies regarding the classification of vasculitides. Differential diagnosis of vasculitis includes infection and lymphoma. Delayed diagnosis and treatment of neuropathy result in the impairment of ADL and QOL. Recovery from axonal degeneration usually takes time and is not always possible. Treatment includes corticosteroid, cyclophosphamide, and intravenous immunoglobulin administration; however, the intensity of treatment depends on the disease activity of vasculitis. PMID:24200608

Oya, Yasushi

2013-11-01

236

Colonic localization of indium-111 labeled leukocytes in active Behcet's disease  

SciTech Connect

A patient with known Behcet's disease demonstrated intense colonic localization of In-111 labeled leukocytes. Gastrointestinal involvement had not been previously manifested, but extensive colonic inflammation was documented by endoscopy. This case illustrates the utility of In-111 labeled leukocyte imaging for detecting active bowel disease in a debilitated patient with documented Behcet's vasculitis.

Harre, R.G.; Conrad, G.R.; Seabold, J.E.

1988-06-01

237

Alopecia areata and humpy-back syndrome in suckling piglets  

PubMed Central

This report describes an uncommon variant of humpy-back syndrome associated with multiple rib fractures and multisystemic vasculitis in several nursing piglets and, for the first time, a skin disease in swine consistent with alopecia areata. Both conditions were observed concurrently on the farm and occasionally in the same piglets. PMID:23372194

Drolet, Richard; Denicourt, Martine; D'Allaire, Sylvie

2012-01-01

238

Thrombotic thrombocytopenic purpura in adult Still's disease.  

PubMed

We describe 2 patients with adult Still's disease who developed thrombotic thrombocytopenic purpura (TTP) and were successfully treated. Although TTP has been associated with autoimmune diseases, usually with systemic lupus erythematosus or various forms of vasculitis, it has rarely been observed in patients with adult Still's disease. This uncommon coexistence of 2 clinical entities may indicate similar pathogenetic mechanisms. PMID:8882053

Boki, K A; Tsirantonaki, M J; Markakis, K; Moutsopoulos, H M

1996-02-01

239

Combination therapy with rituximab and cyclophosphamide in the treatment of anti-neutrophil cytoplasmic antibodies (ANCA) positive pulmonary hemorrhage: case report  

PubMed Central

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with pulmonary hemorrhage is rare in childhood. Standard treatment includes corticosteroids and cyclophosphamide (CYC), which is associated with a high level of toxicity. We report a white female with ANCA positive pulmonary hemorrhage who was treated with cyclophosphamide (CYC) and rituximab (RTX) combination therapy. PMID:22032441

2011-01-01

240

Experimental aerosol infection of cattle ( Bos taurus) with ovine herpesvirus 2 using nasal secretions from infected sheep  

Microsoft Academic Search

Infection of clinically susceptible ruminants, including domesticated cattle and American bison, with ovine herpesvirus 2 (OvHV-2) can result in the fatal lymphoproliferative and vasculitis syndrome known as malignant catarrhal fever (MCF). A reliable experimental infection model is needed to study the pathogenesis of MCF and to develop effective vaccination strategies to control the disease. An experimental aerosol infection model using

Naomi S. Taus; J. Lindsay Oaks; Katherine Gailbreath; Donald L. Traul; Donal O’Toole; Hong Li

2006-01-01

241

Erythema elevatum diutinum in association with dermatitis herpetiformis.  

PubMed

Erythema elevatum diutinum (EED) is a rare skin disease that initially presents as leucocytoclastic vasculitis and later resolves with fibrosis. Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. We report a rare association of these two disorders. PMID:24616856

Chandrasekaran, Shanmuga Sekar; Rai, Reena; Vedachalam, Sandhya; Dorairaj, Lathika; Palaniraman, Surendran

2014-01-01

242

Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges.  

PubMed Central

OBJECTIVES--To test the effectiveness of and tolerance to interferon-alpha 2b (INFa2b) in association with plasma exchanges for the treatment of polyarteritis nodosa (PAN) related to hepatitis B virus (HBV). METHODS--A prospective, non blinded, multicentre trial was carried out in which patients with multisystemic HBV-related PAN were included. Each patient received the association of INFa2b and plasma exchanges. The end point of the study was control of the disease (recovery or remission) or death. RESULTS--Six patients were included in the study. Each patient had histopathological or arteriographic evidence of vasculitis and was infected with actively replicating HBV. All patients were alive at the end of the study and no longer presented clinical or laboratory evidence of systemic vasculitis. HBeAg/anti-HBeAb seroconversion was observed in four patients (66.6%) and HBsAg/anti-HBsAb in 3/6 (50%). Two patients are still being treated with INFa2b because of chronic active hepatitis. CONCLUSIONS--It is considered that this new therapeutic approach to HBV-related PAN effectively cured systemic vasculitis and was associated with control of HBV infections. Antiviral therapy may have a role to play as the first line treatment regime of virus-induced vasculitis. PMID:7912504

Guillevin, L; Lhote, F; Sauvaget, F; Deblois, P; Rossi, F; Levallois, D; Pourrat, J; Christoforov, B; Trepo, C

1994-01-01

243

Eficacia del adalimumab en la enfermedad de Behçet: descripción de 6 casos  

Microsoft Academic Search

Behçet's disease (BD) is a systemic vasculitis, with a more aggressive course in young males. Orogenital ulcers, uveitis and cutaneous lesions are the most frequent manifestations.We analyzed the effects of adalimumab on six patients with BD pretreated with inmunosupressive therapy, two of whom had received infliximab. We observed a good clinical response in all patients. To date, after a mean

Javier Calvo Catalá; Cristina Campos Fernández; Amalia Rueda Cid; María Isabel González-Cruz Cervellera; Aurelio Baixauli Rubio; María Dolores Pastor Cubillo

2011-01-01

244

Allergic Complications of Meningococcal Disease I—Clinical Aspects  

Microsoft Academic Search

Out of 717 patients with meningococcal disease 53 showed one or more of the three allergic complications: 47 (6·6%) developed arthritis, 12 (1·7%) developed cutaneous vasculitis, and 6 developed episcleritis. These complications, which were often multiple, occurred six to nine days after the beginning of the illness and three to six days after the start of successful antibiotic therapy. Those

H. C. Whittle; M. T. Abdullahi; F. A. Fakunle; B. M. Greenwood; A. D. M. Bryceson; E. H. O. Parry; J. L. Turk

1973-01-01

245

Peripheral ulcerative keratitis in the setting of rheumatoid arthritis: Treatment with immunosuppressive therapy  

Microsoft Academic Search

Peripheral ulcerative keratitis (PUK) is a rare but serious inflammatory eyecondition that can complicate rheumatoid arthritis. PUK can be a warning sign of impending vasculitis, and cytotoxic therapy may be necessary to induce remission. We have encountered three patients with PUK in the past year. Two patients had long-standing quiescent rheumatoid arthritis who developed photophobia. Diagnosis was made by slit

Haitham Jifi-Bahlool; Constantine Saadeh; James O'Conner

1995-01-01

246

Cutaneous zygomycosis caused by Saksenaea vasiformis in a diabetic patient.  

PubMed Central

A 49-year-old woman with diabetes mellitus rapidly developed necrotizing cellulitis with fat necrosis and vasculitis after minor trauma to the right arm. Zygomycosis was diagnosed histologically. The lesion responded to aggressive debridement, amphotericin B, and normalization of blood glucose. Cultures yielded structures characteristic of Saksenaea vasiformis only after transfer to saline agar. Images PMID:7929783

Bearer, E A; Nelson, P R; Chowers, M Y; Davis, C E

1994-01-01

247

A Single-Base Deletion in the Thrombopoietin (TPO) Gene Causes Familial Essential Thrombocythemia Through a Mechanism of More Efficient Translation of TPO mRNA  

Microsoft Academic Search

37 months), we noticed the appearance of painful perimalleolar skin ulcers. Ulcers appeared with an erythematous border, whereas the epidermidis was replaced by a fibrinous exudate and the dermis scattered by necrotic areas. Skin biopsies of the ulcerated lesions showed in all cases an histologic picture compatible with small vessel vasculitis. Circulating immune complexes were not detectable and doppler-fluximetry was

Fabio Stagno; Patrizia Guglielmo; Ugo Consoli; Paolo Fiumara; Mario Russo; Rosario Giustolisi

248

Atrial myxoma masquerading as Takayasu’s arteritis  

PubMed Central

Lesson We describe the case of a 48-year-old woman whose atrial myxoma was mistaken for vasculitis. The case report highlights the reasons why these two disorders may become confused, the dangers of initiating the wrong treatment and a simple means of avoiding misdiagnosis.

Fung, Kenneth; Edmondson, Stephen

2014-01-01

249

Extrahepatic Manifestations of Chronic HCV Infection  

Microsoft Academic Search

Several extrahepatic manifestations have been reported in the natural history of hepatitis C virus infection (HCV). Up to 40-74% of patients infected with HCV might develop at least one extrahepatic manifestation during the course of their disease. Mixed Cryoglobulinemia (MC) is the most known and studied syndrome associated with HCV infection. It is a systemic vasculitis that may involve the

Alessandra Galossi; Riccardo Guarisco; Lia Bellis; Claudio Puoti

250

Cardiovascular and thrombophilic risk factors for central retinal vein occlusion  

Microsoft Academic Search

Retinal vein occlusion (RVO) is a relatively common disease that is often associated with a variety of systemic disorders including arterial hypertension, diabetes mellitus, dyslipidemia, and systemic vasculitis. There are various types of RVO, categorized on the basis of the site of occlusion and on the type of consequent vascular damage. Central retinal vein occlusion (CRVO) is the most frequently

Domenico Prisco; Rossella Marcucci; Laura Bertini; Anna Maria Gori

2002-01-01

251

A Case of Henoch-Sch?nlein Purpura with P369S Mutation in MEFV Gene  

PubMed Central

Background Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. HSP can affect multiple organs presenting with a characteristic rash in most of the patients. Familial Mediterranean Fever (FMF) is an inherited inflammatory disease common in mediterranean populations. HSP is the most common vasculitis seen in children with FMF. Case Presentation A 16 year old boy was referred with history of abdominal pain lasting for 20 days. He was hospitalized and had appendectomy. Due to the persistence of his abdominal pain after surgery he was admitted to our hospital. His physical examination showed palpable purpuric rashes symmetrically distributed on lower extremities. Abdominal examination revealed periumbilical tenderness. Laboratory tests showed elevated erythrocyte sedimentation rate, Creactive protein and fibrinogen. Urinalysis revealed microscopic hematuria and severe proteinuria. The fecal occult blood testing was positive. Based on these clinic findings, the patient was diagnosed as HSP with renal, gastrointestinal tract and skin involvement. We performed DNA analysis in our patient because he had diagnosis of vasculitis with severe symptoms and found that he was carrying heterozygote P369S mutation. Conclusion Our case is noteworthy as it indicates that it may be important not to overlook presence of FMF mutations in patients with a diagnosis of severe vasculitis. PMID:23056796

Ertan, Pelin; Tekin, Gokhan; Sahin, Gulseren Evirgen; Kas?rga, Erhun; Taneli, Fatma; Kandioglu, Ali Riza; Sozeri, Betul

2011-01-01

252

CD40 ligand gene and Kawasaki disease  

Microsoft Academic Search

Kawasaki disease (KD) is an acute systemic vasculitis syndrome of infants and young children. Although its etiology is largely unknown, epidemiological findings suggest that genetic factors play a role in the pathogenesis of KD. To identify genetic factors, affected sib-pair analysis has been performed. One of the identified peaks was located on the Xq26 region. A recent report of elevated

Yoshihiro Onouchi; Sakura Onoue; Mayumi Tamari; Keiko Wakui; Yoshimitsu Fukushima; Mayumi Yashiro; Yoshikazu Nakamura; Hiroshi Yanagawa; Fumio Kishi; Kazunobu Ouchi; Masaru Terai; Kunihiro Hamamoto; Fumiyo Kudo; Hiroyuki Aotsuka; Yoshitake Sato; Akiyoshi Nariai; Yoichi Kaburagi; Masaru Miura; Tsutomu Saji; Tomisaku Kawasaki; Yusuke Nakamura; Akira Hata

2004-01-01

253

A case of ulcerative colitis presenting as pyoderma gangrenosum and lung nodule  

PubMed Central

Pyoderma gangrenosum is a phenomenon of cutaneous ulceration where etiology is not well understood. About half of the cases have an associated extracutanoeus manifestation or associated systemic diseases. Most commonly associated systemic disorders include inflammatory bowel disease, hematologic malignancies, autoimmune arthritis, and vasculitis. We are reporting a case where pyoderma gangrenosum has presenting features for ulcerative colitis. PMID:24596646

Li, Xin; Chandra, Subhash

2014-01-01

254

IgA nephropathy  

MedlinePLUS

... glomerulonephritis). Risk factors include: A personal or family history of IgA nephropathy or Henoch Schonlein purpura , a form of vasculitis that affects many parts of the body Caucasian or Asian ethnicity IgA nephropathy can occur in people of ...

255

Detection and Multigenic Characterization of a Herpesvirus Associated with Malignant Catarrhal Fever in White-Tailed Deer (Odocoileus virginianus) from Missouri  

Microsoft Academic Search

Between 1998 and 2001, tissues from four captive white-tailed deer were observed to have histologic lesions of systemic lymphocytic vasculitis. These lesions suggested malignant catarrhal fever, although epizootic hemorrhagic disease and bluetongue were included in the differential diagnosis. Initial diagnostic efforts, including virus isolation and reverse transcription-PCR for epizootic hemorrhagic disease virus and blue- tongue virus, failed to identify an

Steven B. Kleiboeker; Margaret A. Miller; Susan K. Schommer; Jose A. Ramos-Vara; Magalie Boucher; Susan E. Turnquist

2002-01-01

256

State of the Art in the Treatment of Systemic Vasculitides  

PubMed Central

Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. In milder forms of ANCA-associated vasculitis, methotrexate, leflunomide, or mycophenolate mofetil are effective. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30–40% at 5?years. Mortality from GPA is four times higher than the background population. Early deaths are due to active vasculitis and infection. Subsequent deaths are more often due to cardiovascular events, infection, and cancer. We need to improve the long-term outcome, by controlling disease activity but also preventing damage and drug toxicity. By contrast, in large vessel vasculitis where mortality is much less but morbidity potentially greater, such as giant cell arteritis (GCA) and Takayasu arteritis, therapeutic options are limited. High dose glucocorticoid results in significant toxicity in over 80%. Advances in understanding the biology of the vasculitides are improving therapies. Novel, mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases. PMID:25352843

Luqmani, Raashid Ahmed

2014-01-01

257

Differential diagnosis of multiple sclerosis: contribution of magnetic resonance techniques.  

PubMed

It is widely accepted that magnetic resonance imaging (MRI) findings are not totally specific for the diagnosis of multiple sclerosis. White matter lesions that mimic those of multiple sclerosis may be detected in both normal volunteers and patients harbouring different diseases. Virtually all the characteristic features of multiple sclerosis are sometimes encountered in other conditions affecting predominantly the white matter. Different conditions such as vasculitis, subcortical atherosclerotic leukoencephalopathy, Lyme disease, or acute disseminated encephalomyelitis can be virtually indistinguishable from multiple sclerosis on conventional MR images. Also the FLAIR technique adds little to the differential diagnosis. The calculation of magnetisation transfer ratio (MT ratio) may be useful to better characterise some entities, such as vasculitis, from multiple sclerosis. PMID:9647278

Triulzi, F; Scotti, G

1998-05-01

258

A Case of Polyarteritis Nodosa Presenting Initially as Peripheral Vascular Disease  

PubMed Central

Polyarteritis nodosa is a rare necrotizing vasculitis that can be progressive and fatal, and its initial presenting symptom may be leg claudication due to peripheral vascular ischemia. To date, there have been fewer than ten case reports of polyarteritis nodosa presenting as peripheral vascular disease. We report a case of a 38-year-old man initially diagnosed to have premature peripheral vascular disease who presented 1 year later with symptoms consistent with giant cell arteritis and subsequently developed bowel ischemia leading to a fatal outcome. Based on the autopsy and the patient’s clinical course, the final diagnosis was polyarteritis nodosa. This case illustrates the challenges in diagnosing polyarteritis nodosa and the importance of considering vasculitis in young patients presenting with atypical presentations of diseases such as peripheral vascular disease or giant cell arteritis. PMID:18560943

Parikh, Sameer; Lu, Lee

2008-01-01

259

Polyangiitis overlap syndrome of granulomatosis with polyangiitis (Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).  

PubMed

Polyangiitis overlap syndrome is defined as systemic vasculitis that cannot be classified into one of the well-defined vasculitic syndromes. In this report, a female patient who presented with vasculitis-like and asthmatic symptoms was diagnosed as having polyangiitis overlap syndrome of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). The patient fulfilled the American College of Rheumatology diagnostic criteria for GPA and EGPA. She was successfully treated with immunosuppressants and steroids and has been in remission for 20 months. It is important to establish a proper diagnosis and introduce an appropriate treatment modality in patients with this rare and serious pathology to prevent irreversible organ damage. PMID:24577169

Uematsu, Hironori; Takata, Shohei; Sueishi, Katsuo; Inoue, Hiromasa

2014-01-01

260

Managing Behçet's disease: An update on current and emerging treatment options.  

PubMed

Behçet's disease is an autoinflammatory vasculitis of unknown origin characterized by recurrent oral and genital ulcers, uveitis, arthritis and skin lesions. Additionally, involvement of the gastrointestinal tract, central nervous system and large vessels may occur. The disease is prevalent in countries along the ancient Silk Road from Eastern Asia to the Mediterranean Basin. Many treatment modalities are currently available. The choice of treatment depends on organ involvement and severity of disease. Topical treatment with corticosteroids is often sufficient for mucocutaneous involvement, however for more severe disease with vasculitis or neurological involvement a more aggressive approach is warranted. Newer drugs (biologicals) influencing cytokines and thereby T-cell function are promising with an acceptable side effect profile. Unfortunately, reimbursement of the costs of biologicals for rare disease is still a problem in various countries. In this report we discuss the current treatment modalities for Behçet's disease. PMID:19536320

van Daele, P La; Kappen, J H; van Hagen, P M; van Laar, J Am

2009-04-01

261

Adult Dermatomyositis with Bleeding Ulcer in the Pharynx  

PubMed Central

Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding. PMID:25328739

Kusano, Junko; Takahashi, Yuka

2014-01-01

262

ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms  

PubMed Central

Kawasaki disease is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected. We identified a functional SNP (itpkc_3) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene on chromosome 19q13.2 that is significantly associated with Kawasaki disease susceptibility and also with an increased risk of coronary artery lesions in both Japanese and US children. Transfection experiments showed that the C allele of itpkc_3 reduces splicing efficiency of the ITPKC mRNA. ITPKC acts as a negative regulator of T-cell activation through the Ca2+/NFAT signaling pathway, and the C allele may contribute to immune hyper-reactivity in Kawasaki disease. This finding provides new insights into the mechanisms of immune activation in Kawasaki disease and emphasizes the importance of activated T cells in the pathogenesis of this vasculitis. PMID:18084290

Onouchi, Yoshihiro; Gunji, Tomohiko; Burns, Jane C; Shimizu, Chisato; Newburger, Jane W; Yashiro, Mayumi; Nakamura, Yoshikazu; Yanagawa, Hiroshi; Wakui, Keiko; Fukushima, Yoshimitsu; Kishi, Fumio; Hamamoto, Kunihiro; Terai, Masaru; Sato, Yoshitake; Ouchi, Kazunobu; Saji, Tsutomu; Nariai, Akiyoshi; Kaburagi, Yoichi; Yoshikawa, Tetsushi; Suzuki, Kyoko; Tanaka, Takeo; Nagai, Toshiro; Cho, Hideo; Fujino, Akihiro; Sekine, Akihiro; Nakamichi, Reiichiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Nakamura, Yusuke; Hata, Akira

2010-01-01

263

Lateral medullary stroke in patient with granulomatous polyangiitis.  

PubMed

Granulomatous polyangiitis (GPA), also known as Wegener granulomatosis, is a systemic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that infrequently affects the central nervous system. We report a 41-year-old man with lateral medullary infarction who developed rapidly progressive renal failure. He was diagnosed with GPA based on positive serum c-ANCA and antiproteinase 3 antibodies and demonstration of pauci-immune crescentic glomerulonephritis on kidney biopsy. He was treated with Coumadin, pulse steroids, cyclophosphamide, and plasmapheresis. He had resolution of his neurologic deficits and improvement in renal function. This case report highlights the importance to consider GPA vasculitis in the differential diagnosis of stroke in patients with development of acute kidney injury. PMID:24128976

Taraschenko, Olga D; Amory, Colum F; Waldman, Jonathan; Hanspal, Era K; Bernardini, Gary L

2014-01-01

264

Dermatologic complications from levamisole-contaminated cocaine: a case report and review of the literature.  

PubMed

Levamisole is a veterinary anthelmintic drug with immunomodulatory properties in humans. It has become increasingly common as a contaminant in cocaine and is now detected in the majority of cocaine seized in the United States. A variety of adverse reactions have been reported in association with levamisole, the most severe being agranulocytosis, vascular occlusive disease, and thrombotic vasculopathy, with or without vasculitis. The combination of rapidly progressive cutaneous ecchymosis and purpura leading to necrosis, often affecting the ears and cheeks; neutropenia or agranulocytosis; serologic autoantibodies; and thrombotic vasculopathy, with or without associated vasculitis, in a patient who has recently used cocaine is characteristic of exposure to contaminant levamisole. We report the case of a 54-year-old man who presented with the clinical findings of levamisole-contaminated cocaine use and review the literature regarding cutaneous reactions associated with levamisole. Our case highlights this important public health issue and represents a clinical course that is unusually severe. PMID:24605347

Gaertner, Erich M; Switlyk, Stephen A

2014-02-01

265

[Therapy of peripheral vessel stenosis and occlusion in patients with thromboangiitis obliterans].  

PubMed

Vasculitis consists of a group of diseases characterized by an inflammatory process of the vessel wall. There is a wide variation in symptoms and almost any organ or tissue can be affected. Thromboangiitis obliterans (TAO; also known as Buerger's disease) is a special form of vasculitis with recurring inflammation and thrombosis of small and medium size arteries and veins of the hands and feet. To date the etiology still remains unclear but there is a strong association with the use of tobacco products. Ulcerations and gangrene of the extremities are common complications often resulting in the need for amputation of the extremity involved. Treatment of TAO includes both surgical and non-surgical methods but there is still no agreement concerning the optimal treatment strategy. In this contribution the advantages and disadvantages of different treatment options will be addressed and representative cases will be discussed. PMID:20717643

Sandner, T A; Degenhart, C; Becker-Lienau, J; Reiser, M F; Treitl, M

2010-10-01

266

Polyarteritis nodosa and mixed cryoglobulinaemia related to hepatitis B and C virus coinfection  

PubMed Central

OBJECTIVE—To determine the responsibility of hepatitis B virus (HBV) and hepatitis C virus (HCV) and therapeutic implications in a patient who developed systemic vasculitis.?CASE REPORT—The case of a 38 year old woman who had a past history of addiction to intravenous drugs and developed systemic vasculitis after infection by HBV and HCV is described. The clinical and laboratory findings substantiated not only the diagnosis of polyarteritis nodosa (PAN) but also that of mixed cryoglobulinaemia with a monoclonal IgM? component.?CONCLUSION—Because cryoglobulins are rarely found in HBV related PAN but often associated with HCV infection, and in light of the histological findings, cryoglobulinaemia was interpreted as being secondary to HCV infection. This example of a highly complex situation emphasises the need to gather all relevant clinical, biological, histological, and complementary data so that the best treatment for overlapping of distinct vasculitides can be selected.?? PMID:11602482

Lefebvre, P; Mouthon, L; Cohen, P; Lhote, F; Guillevin, L

2001-01-01

267

Allergic Complications of Meningococcal Disease I—Clinical Aspects  

PubMed Central

Out of 717 patients with meningococcal disease 53 showed one or more of the three allergic complications: 47 (6·6%) developed arthritis, 12 (1·7%) developed cutaneous vasculitis, and 6 developed episcleritis. These complications, which were often multiple, occurred six to nine days after the beginning of the illness and three to six days after the start of successful antibiotic therapy. Those patients with severe systemic disease were prone to the complications. Histological and bacteriological study of the arthritis and vasculitis showed that these lesions were probably not due to persisting infection and suggested that they might be due to immune complex disease. ImagesFIG. 3FIG. 4FIG. 5FIG. 6 PMID:4268766

Whittle, H. C.; Abdullahi, M. T.; Fakunle, F. A.; Greenwood, B. M.; Bryceson, A. D. M.; Parry, E. H. O.; Turk, J. L.

1973-01-01

268

Neutrophilic dermatosis of the dorsal hands associated with hypopharyngeal carcinoma.  

PubMed

Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare and recently described disorder regarded as a subset of neutrophilic dermatosis, similar to superficial pyoderma gangrenosum and pustular vasculitis. Many currently consider it to be a localized variant of Sweet Syndrome. We describe the case of a 63-year-old male patient with NDDH associated with hypopharyngeal carcinoma. A biopsy demonstrated changes consistent with those seen in Sweet Syndrome, with leukocytoclasia but no signs of true vasculitis. Treatment with oral prednisolone resulted in complete resolution of the lesions in 1 month, with no recurrences. The clinical presentation, the histological features, and the excellent response to oral corticosteroid therapy allowed us to classify this case as NDDH. We emphasize the rare association of this entity with a solid tumor. PMID:18718189

Cravo, Mariana; Cardoso, José C; Tellechea, Oscar; Cordeiro, Margarida R; Reis, José Pedro; Figueiredo, Américo

2008-01-01

269

Dengue retinochoroiditis.  

PubMed

Dengue is a mosquito-borne infection caused by a flavivirus. I describe the ocular findings observed in two patients infected with dengue virus who presented with acute onset of loss of vision preceded by febrile illness, malaise, generalized fatigue headache, and maculopapular rash. Ophthalmologic evaluation in each patient revealed a normal anterior segment. Vitreous cells were noted in one patient. Ophthalmoscopy revealed multiple foci of retinochoroiditis, vasculitis, cotton-wool spots, and retinal hemorrhages. The healing of the lesion showed discrete atrophic and pigmented retinochoroiditic scars. Fluorescein angiography displayed early hypofluorescence and late hyperfluorescence suggestive of leakage. The healed scars showed late staining. The serologic testing showed elevated IgG antibodies, and one had high IgM antibodies to dengue virus. Ocular findings of dengue fever consist of multifocal areas of retinochoroiditis and may lead to loss of vision. In Saudi Arabia, dengue fever should be considered in the differential diagnosis of multifocal chorioretinal lesions and retinal vasculitis. PMID:22871625

Tabbara, Khalid

2012-01-01

270

Progressive pigmentary purpura.  

PubMed

A 58-year-old man presented for evaluation and treatment of non-tender, non-pruritic, annular patches on the right temple and frontal aspect of the scalp that reddened with exercise. A biopsy specimen showed a purpuric dermatitis with features of lymphocytic vasculitis; a diagnosis of exercise-induced progressive pigmentary purpura was made. Whereas progressive pigmentary purpura is purported to be caused by exercise, other similar appearing entities are associated with exercise, namely exercise-induced vasculitis (EIV). EIV may be considered as an acute microcirculatory deficiency and thermoregulation decompensation that occurs after episodes of exhaustive major muscular activity or after unusual or excessive exercise. The combination of age greater than 50 years, heat, and prolonged exercise are the most potent contributing factors. This is the first report of exercise-induced progressive pigmentary purpura. PMID:22031640

Brauer, Jeremy A; Mundi, Jyoti; Chu, Julie; Patel, Rishi; Meehan, Shane; Greenspan, Alan H; Stein, Jennifer

2011-01-01

271

Grading the severity of soft tissue changes associated with metal-on-metal hip replacements: reliability of an MR grading system  

Microsoft Academic Search

Introduction  Metal-on-metal (MoM) soft tissue reactions or aseptic lymphocytic vasculitis-associated lesions (ALVAL) are being recognised\\u000a using metal artefact reduction (MAR) MR with increasing frequency following the advent of second generation metal-on-metal\\u000a bearings, but there is no standardised technique for reporting of MR appearances in this disease. The aim of this study was\\u000a to measure the reliability of a grading system designed

Helen Anderson; Andoni Paul Toms; John G. Cahir; Richard W. Goodwin; James Wimhurst; John F. Nolan

2011-01-01

272

Identification of a Gammaherpesvirus Selective Chemokine Binding Protein That Inhibits Chemokine Action  

Microsoft Academic Search

Chemokines are involved in recruitment and activation of hematopoietic cells at sites of infection and inflammation. The M3 gene of gHV68, a gamma-2 herpesvirus that infects and establishes a lifelong latent infection and chronic vasculitis in mice, encodes an abundant secreted protein during productive infection. The M3 gene is located in a region of the genome that is transcribed during

VICTOR VAN BERKEL; JOHN BARRETT; H. LEE TIFFANY; DAVED H. FREMONT; PHILIP M. MURPHY; GRANT MCFADDEN; SAMUEL H. SPECK; HERBERT W. VIRGIN

2000-01-01

273

Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre  

Microsoft Academic Search

Objective. Churg-Strauss syndrome is a rare multisystem vasculitis of unknown aetiology. Due to the rarity of the disease, few single-centre case series have been described. The aim of this study was to evaluate a small series from a single Italian centre in order to describe the clinical features of the disease, the treatment and long-term follow-up. Methods. Nineteen Churg-Strauss syndrome

A. Della Rossa; C. Baldini; A. Tavoni; A. Tognetti; D. Neglia; G. Sambuceti; R. Puccini; C. Colangelo; S. Bombardieri

2002-01-01

274

Massive Proteinuria and Acute Glomerulonephritis Picture in a Patient With Familial Mediterranean Fever and E148Q Mutation.  

PubMed

Familial Mediterranean fever (FMF) is an inherited auto-inflammatory disorder. Secondary AA amyloidosis is the most devastating complication of FMF. Nonamyloid renal involvements have also been reported in association with FMF, including vasculitis, focal and diffuse glomerulonephritis, and IgA nephropathy. We describe a patient with FMF and E148Q mutation who presented with massive proteinuria, elevated serum creatinine level, and acute glomerulonephritis picture. Disease remission was achieved after treatment with corticosteroids and colchicine. PMID:25362225

Ardalan, Mohammadreza; Nasri, Hamid

2014-11-01

275

[Arthritis, erythema nodosum and genital ulcerations. Behçet disease].  

PubMed

Behcet's disease is a disease of unknown etiology resting in between vasculitis, spondyloarthropathy and autoinflammatory diseases. If his predilection for the population originating from the Silk Road is well known, as are its cutaneous, ocular and vascular manifestations, this case illustrates the non-specificity of those manifestations, the diagnostic difficulties and the importance of routinely assessing for eyes and bowel diseases in this type of patient. PMID:21484713

Arnold, C; Revaz, S; Dudler, J

2011-04-13

276

A Rare Case of Beh?et Disease Presenting with Pyrexia of Unknown Origin, Pulmonary Embolism, and Right Ventricular Thrombus  

PubMed Central

Behçet disease is a systemic vasculitis characterized by recurrent oral and genital ulcers and uveitis. We describe a rare case of a 43-year-old woman with Behçet disease who was admitted for pyrexia of unknown origin, cough, dyspnea, and chest pain. Her computerized tomography scan revealed pulmonary embolism and right ventricular thrombus. She was treated with anticoagulation for pulmonary embolism and right ventricular thrombus. She was well during her last follow-up. PMID:24436611

Xing, Weili; Swaminathan, Girider; Appadorai, Dorai Raj; Sule, Ashish Anil

2013-01-01

277

Computerized information-gathering in specialist rheumatology clinics: an initial evaluation of an electronic version of the Short Form 36  

Microsoft Academic Search

Objectives. Longitudinal outcome data are important for research and are becoming part of routine clinical practice. We assessed an initial version of an electronic Short Form 36 (SF-36), a well-established health assessment questionnaire, in comparison with standard paper forms, in two specialist rheumatology clinics. Methods. Out-patients (20 with systemic lupus erythematosus and 31 with vasculitis) were randomly selected to complete

A. S. Wilson; G. D. Kitas; D. M. Carruthers; C. Reay; J. Skan; S. Harris; G. J. Treharne; S. P. Young; P. A. Bacon

2002-01-01

278

Infection or Inflammation and ICU Seizures  

Microsoft Academic Search

Effective treatment of seizures associated with central nervous system (CNS) infection and inflammation depends on rapid diagnosis\\u000a and early attainment of bactericidal activity in the cerebrospinal fluid with appropriate antimicrobial agents, or appropriate\\u000a management of vasculitis-induced cerebral complications. Despite the rarity of these disorders, there is nothing specific\\u000a regarding the management in the intensive care unit of seizures in these

Wendy C. Ziai

279

Juvenile rheumatoid arthritis and bronchiolitis obliterans organized pneumonia.  

PubMed

Diverse pleuropulmonary manifestations, including pleural effusion, rheumatoid nodulosis, fibrosis, obliterans brochiolitis, bronchiectasias, vasculitis, drug-induced lung disease, and obliterans bronchiolitis with organized pneumonia, have been described in patients with rheumatoid arthritis (RA). Bronchiolitis obliterans organized pneumonia (BOOP) is an uncommon condition described in patients with RA but not in juvenile RA (JRA). We described a patient with JRA who developed a BOOP. PMID:16362444

Sohn, Debora Ingrid; Laborde, Hugo Armando; Bellotti, Marta; Seijo, Luis

2007-02-01

280

Identification of masqueraders of autoimmune disease in the office.  

PubMed

There are several rheumatologic and autoimmune disorders that can masquerade as allergic disease. Identification of these conditions in an office setting can be a challenge for the practicing allergist-immunologist. These conditions include rheumatoid and juvenile arthritis, Sjogren's syndrome, systemic lupus erythematosus, Behcet's and antiphospholipid syndromes, systemic sclerosis, vasculitis, sarcoidosis, chronic fatigue syndrome, and fibromyalgia. The article will address these topics and include clinical uses of immunologic tests for diagnosis. PMID:14763244

Frieri, Marianne

2003-01-01

281

Acral gangrene as a presentation of non-uremic calciphylaxis  

PubMed Central

We are describing a case of 55-year-old obese female with significant history of uncontrolled rheumatoid arthritis, who recently had decreased her immune-suppression medications. She presented with extensive acral gangrene involving multiple fingers and toes. Clinical picture and laboratory findings were suggestive of vasculitis; however, skin biopsy established diagnosis of calciphylaxis, in settings of normal kidney function. Patient was treated with sodium thiosulfate with gradual improvement in her skin lesions. PMID:24327971

Hammadah, Muhammad; Chaturvedi, Shruti; Jue, Jennifer; Buletko, Andrew Blake; Qintar, Mohammed; Madmani, Mohammed Eid; Sharma, Prashant

2013-01-01

282

Immunopathogenesis of Behçet’s disease with special emphasize on the possible role of antigen presenting cells  

Microsoft Academic Search

Behçet’s disease (BD) is a systemic vasculitis with unclear etiology and pathogenesis. Although several viral and bacterial\\u000a causes have been investigated in detail for several years, of late it is widely accepted that microorganisms may play a role\\u000a as a trigger, or as a cross-reactive antigens that interfere with self-antigens, such as heat-shock proteins. Genes such as\\u000a HLA-B51, MICA and

Salih Pay; ?smail ?im?ek; Hakan Erdem; Ayhan Dinç

2007-01-01

283

Cutaneous manifestations associated with rheumatoid arthritis  

Microsoft Academic Search

Rheumatoid arthritis presents various cutaneous manifestations, either specific or nonspecific skin features, which are induced\\u000a by the activation of inflammatory cells (neutrophils, lymphocytes, macrophages), vasculopathy, vasculitis, acral deformity,\\u000a drugs, and so on. These include (1) specific findings, (2) findings due to vascular impairment, (3) findings due to immune\\u000a dysfunction, (4) characteristic neutrophilic conditions, and (5) miscellaneous conditions. On the other

T. Yamamoto

2009-01-01

284

Cutaneous nodules in patients with rheumatoid arthritis: a case report and review of literatures  

Microsoft Academic Search

We report a case of 57-year-old Japanese woman with an overlap syndrome of both rheumatoid arthritis (RA) and autoimmune hepatitis,\\u000a who developed multiple skin nodules. An extensive biopsies of the nodules revealed rheumatoid neutrophilic dermatitis, showing\\u000a panniculitis without vasculitis, combining with granulomatous formation histopathologically. Since cutaneous nodules in patients\\u000a with RA are very complex, differential diagnosis should be done according

Tadashi Nakamura; Syu-ichi Higashi; Kunihiko Tomoda; Michishi Tsukano; Ken-ichi Iyama

2011-01-01

285

Elastic Liposomal Formulation for Sustained Delivery of Colchicine: In Vitro Characterization and In Vivo Evaluation of Anti-gout Activity  

Microsoft Academic Search

Colchicine, an alkaloid found in extracts of the plants Colchicum autumnale and Gloriosa superb, is effective in the treatment of acute gout and dermatological conditions like leuko-cytoclastic vasculitis, psoriasis,\\u000a and Sweet’s syndrome. Oral administration of colchicine is associated with gastrointestinal side effects and its accumulation\\u000a in the body leads to bone marrow suppression. In the present study, an attempt has

Hardevinder Pal Singh; Puneet Utreja; Ashok Kumar Tiwary; Subheet Jain

2009-01-01

286

Atherosclerosis associated with vasculopathic lesions in a golden retriever with hypercholesterolemia  

PubMed Central

A 2-year-old neutered male golden retriever dog presented for lameness secondary to ulcerations of multiple digital paw pads was diagnosed with vasculitis and hypercholesterolemia. Despite treatment, ischemic necrosis progressed to include all distal extremities and the dog eventually expired due to myocardial infarction secondary to severe atherosclerosis. The rapid demise and the dermatologic lesions may have been secondary to cholesterol embolism syndrome which has never before been reported in a dog. PMID:24790237

Boynosky, Nicole A.; Stokking, Laura

2014-01-01

287

A Rare Cause of Pericardial Effusion: Giant Cell Arteritis  

PubMed Central

Giant cell arteritis is a granulomatous vasculitis characterized by medium or large sized vessel involvement. Although extracranial branches of the carotid artery are typically involved, involvement of aorta and its major branches can also be seen. Cardiac involvement has been encountered less frequently and pericardial effusion is rarely encountered. In this paper, a case has been presented in which pericardial effusion was determined during the examination and diagnosis was giant cell arteritis. PMID:24523978

Tasliyurt, Turker; Sivgin, Hakan; Bekar, Lutfu; Sahin, Safak; Uzun Kaya, Suheyla; Koseoglu, Resit Dogan; Kutluturk, Faruk; Yilmaz, Abdulkerim

2014-01-01

288

Kawasaki disease  

Microsoft Academic Search

Opinion statement  Kawasaki disease is an acute vasculitis of childhood, complicated by coronary artery aneurysms in up to 25% of affected children.\\u000a High-dose intravenous immunoglobulin (IVIG) administered in the acute phase of Kawasaki disease leads to a fivefold reduction\\u000a in the prevalence of coronary aneurysms. When fever persists, a second IVIG infusion should be administered. Rescue therapies\\u000a for IVIG-resistant Kawasaki disease

Jane W. Newburger; David R. Fulton

2007-01-01

289

Methylprednisolone pulse therapy for massive lymphadenopathy in a child with intravenous immunoglobulin-resistant Kawasaki disease  

Microsoft Academic Search

Kawasaki disease (KD) is an acute febrile multi-system vasculitis of unknown etiology. The diagnosis is based on clinical features. We describe a case of intravenous immunoglobulins (IVIG)-resistant KD presenting with persistent fever and massive cervical lymphadenopathy associated with mild respiratory distress. The symptoms resolved after methylprednisolone pulse therapy. High-dose pulse steroid may be an alternative therapeutic option in KD which

Hsin-Hsu Chen; Po-Mai Liu; Chin-Nam Bong; Yu-Tsun Wu; Kuender D. Yang; Chih-Lu Wang

290

Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.  

PubMed

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis that usually occurs in children younger than 2 years of age. It is a rare disease characterized by mild fever, a violent onset of hemorrhagic skin lesions, and edema usually followed by a spontaneous and complete recovery. Although the etiology is unknown, AHEI often follows infections, drug treatment, or vaccination. In the present report, the authors describe 4 cases of AHEI and review the relevant literature. PMID:18772356

Karremann, Michael; Jordan, Alexander J; Bell, Nellie; Witsch, Michael; Dürken, Matthias

2009-04-01

291

Epstein-Barr virus genome-positive T lymphocytes in a boy with chronic active EBV infection associated with Kawasaki-like disease  

Microsoft Academic Search

Epstein-Barr virus (EBV), a ubiquitous human herpesvirus and an aetiological agent of infectious mononucleosis, has a unique trop-ism for B lymphocytes1. Clinical and laboratory features of chronic active EBV infections are chronic or persistent infectious mononu-cleosis-like symptoms and high antibody titre against early anti-gens (EA)2-4 . Kawasaki disease (KD), aetiology unknown, is thought to be self-limited immunologically mediated vasculitis. Clinical

Hideaki Kikuta; Yuichi Taguchi; Kazuhiro Tomizawa; Kimikazu Kojima; Nobuaki Kawamura; Akihito Ishizaka; Yukio Sakiyama; Shuzo Matsumoto; Shosuke Imai; Toshifumi Kinoshita; Shigeki Koizumi; Toyoro Osato; Ichiro Kobayashi; Isamu Hamada; Kanji Hirai

1988-01-01

292

Primary Systemic Al Amyloidosis Presenting as Temporal Arteritis  

PubMed Central

Temporal arteritis is most common vasculitis in elderly and imitated by miscellaneous disorders. Temporal artery biopsy is the gold standard test in the diagnosis of giant cell arteritis (GCA). Hereby, we describe a case of a 67-year-old man who presented initially with temporal arteritis; however, a lip biopsy then revealed AL amyloidosis. In this respect, temporal artery biopsy should be performed for definitive diagnosis of GCA particularly patients with systemic symptoms and treatment resistant. PMID:24551471

Emmungil, Hakan; Kalfa, Melike; Basar?k, Burcu; Kahraman, Hasip; Tanhan, Ferhat; Yaman, Banu; Ozturk, Atiye; Erdemir, Zehra; Kandiloglu, Gulsen; Inal, Vedat; Kabasakal, Yasemin

2014-01-01

293

Takayasu arteritis: a review  

PubMed Central

Takayasu arteritis is a well known yet rare form of large vessel vasculitis. This review details the history, clinical features, differential diagnoses, classification, and immunology of the disorder. Suppression of inflammation and preservation of vascular competence are the aims of treatment. As with any rare disease, randomised controlled treatment trials are either lacking or based on small patient numbers, making management decisions difficult. Current evidence based treatments are presented and discussed. PMID:12101189

Johnston, S L; Lock, R J; Gompels, M M

2002-01-01

294

Immunoglobulin Heavy Chain Constant Region Determines the Pathogenicity and the Antigen-Binding Activity of Rheumatoid Factor  

Microsoft Academic Search

An IgG3 monoclonal antibody, 6-19, derived from unmanipulated MRL\\/MpJ-lpr\\/lpr mice, exhibiting cryoglobulin and anti-IgG2a rheumatoid factor activities, induces skin leukocytoclastic vasculitis and glomerulonephritis when injected into normal mice. To determine the role of the gamma 3 heavy chain constant region in the generation of cryoglobulins and associated tissue lesions, we have established an IgG1 class switch variant, clone SS2F8, from

Thierry Fulpius; Francois Spertini; Luc Reininger; Shozo Izui

1993-01-01

295

Ulcerative colitis complicating seronegative HLA-A2B27 rheumatoid arthritis with sacroiliitis  

Microsoft Academic Search

The case is reported of a 50 year old man with longstanding seronegative rheumatoid arthritis who developed ulcerative colitis. The patient also had sacroiliitis and his tissue was typed as HLA-A2-B27 several years before the bowel disease began. A possible overlap between primary inflammatory bowel disease, complications to the treatment of rheumatoid arthritis with drugs, and gastrointestinal rheumatoid vasculitis is

T Klausen; K Amris; P Helin

1992-01-01

296

Acute myocardial infarction and cerebrovascular accident in a young girl after a viper bite.  

PubMed Central

A 17-year-old girl developed an acute myocardial infarction immediately after being bitten by a viper and four days later she had a cerebrovascular accident. The close clinical and laboratory follow-up of this case suggested that myocardial damage could be attributed to a direct cardiotoxic effect of the venom, while the brain injury that subsequently appeared was probably the result of a disseminated intravascular coagulopathy, possibly in conjunction with vasculitis. Images PMID:7073914

Aravanis, C; Ioannidis, P J; Ktenas, J

1982-01-01

297

Recurrent Intracranial Hemorrhage Due to Postpartum Cerebral Angiopathy Implications for Management  

Microsoft Academic Search

Background—Postpartum cerebral angiopathy as a cause of hemorrhagic stroke in young women is not well recognized. It is unknown whether this disorder represents a true inflammatory vasculitis or transient vasoconstriction related to the hormonal events of pregnancy and the postpartum period. Case Description—A 39-year-old woman presented with postpartum intracranial hemorrhage and, 32 months later, with subarachnoid hemorrhage, following normal pregnancies.

Melanie R. Ursell; Connie L. Marras; Richard Farb; David W. Rowed; Sandra E. Black; James R. Perry

298

EULAR\\/PReS endorsed consensus criteria* for the classification of childhood vasculitides  

Microsoft Academic Search

Background: There has been a lack of appropriate classification criteria for vasculitis in children.Objective: To develop a widely accepted general classification for the vasculitides observed in children and specific and realistic classification criteria for common childhood vasculitides (Henoch-Scho?nlein purpura (HSP), Kawasaki disease (KD), childhood polyarteritis nodosa (PAN), Wegener’s granulomatosis (WG), and Takayasu arteritis (TA)).Methods: The project was divided into two

S. Ozen; N. Ruperto; M. J. Dillon; A. Bagga; K. Barron; J. C. Davin; T. Kawasaki; C. Lindsley; R. E. Petty; A M Prieur; A Ravelli; P Woo

2006-01-01

299

[Vascular involvement in Cogan's syndrome. A case report].  

PubMed

Vascular manifestations of Cogan's syndrome are rarely reported. We report the case of a young woman followed for typical Cogan's disease. Serious vascular involvement was found only during work-up for arterial hypertension. This case highlights potentially asymptomatic nature of extensive vasculitis affecting large and medium-sized vessels in Cogan's disease. Careful screening is required to prevent life-threatening complications. PMID:22197586

Luong Nguyen, L-B; Warzocha, U; Brillet, P-Y; Abad, S; Larroche, C; Amar, L; Frachet, B; Dhote, R

2012-02-01

300

Gastrointestinal Manifestations of Behçet’s Disease  

Microsoft Academic Search

Behçet’s disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital\\u000a ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along\\u000a the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of\\u000a life

Ellen C. Ebert

2009-01-01

301

Rapidly Progressive Glomerulonephritis in a Patient with Rheumatoid Arthritis during Treatment with High-Dosage D-Penicillamine  

Microsoft Academic Search

A patient with advanced rheumatoid arthritis and severe clinical manifestations of rheumatoid vasculitis died of acute renal failure after 30 months of treatment with high-dosage D-penicillamine. She had had no signs of adverse drug reactions until the terminal illness. Although streptococcal pharyngitis was diagnosed late in her disease, penicillamine-induced immune complex glomerular damage is considered more likely than poststreptococcal glomerulonephritis,

Seyed A. Sadjadi; Mildred S. Seelig; Adolph R. Berger; Mayer Milstoc

1985-01-01

302

Peripheral neuropathy in two patients with rheumatoid arthritis receiving infliximab treatment  

Microsoft Academic Search

Antitumor necrosis alpha agents have been successfully used for the treatment of rheumatoid and seronegative arthritis, Crohn’s\\u000a disease, psoriasis, and severe cases of vasculitis. Several side effects have been observed in patients receiving these agents\\u000a including hypersensitivity reactions, infections, drug-induced lupus, or demyelinating syndromes. The presence of peripheral\\u000a neuropathy has been reported only in isolated cases. We describe two cases

Maria G. Tektonidou; John Serelis; Fotini N. Skopouli

2007-01-01

303

An Unusual Presentation of Giant Cell Arteritis  

PubMed Central

Giant cell arteritis (GCA) is a chronic vasculitis that typically presents with headache, fever and polymyalgia although atypical presentations are known. We present a case of GCA with nonproductive cough and pyrexia of unknown origin emphasizing this atypical nature of presentation. We report a rare association of GCA with granulomatous hepatitis. We also support the use of PET scanning in diagnosing and monitoring this condition. PMID:22811725

Prabhavalkar, Siddhesh; Bogusz, Pawel; Merard, Reena; Gormley, Mark

2012-01-01

304

Churg-Strauss Syndrome: An Update  

Microsoft Academic Search

Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though\\u000a it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show\\u000a that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody–associated\\u000a vasculitis. The presence of

Andy Abril

305

A Proinflammatory Role of IL18 in the Development of Spontaneous Autoimmune Disease1  

Microsoft Academic Search

Serum from patients with systemic lupus erythematosus (SLE) contained significantly higher concentrations of IL-18 than normal individuals. MRL\\/lpr mice, which develop spontaneous lupus-like autoimmune disease, also had higher serum levels of IL-18 than wild-type MRL\\/ mice. Daily injections of IL-18 or IL-18 plus IL-12 resulted in accelerated proteinuria, glomerulonephritis, vasculitis, and raised levels of proinflammatory cytokines in MRL\\/lpr mice. IL-18-treated

Ehsanollah Esfandiari; Iain B. McInnes; George Lindop; Fang-Ping Huang; Max Field; Mousa Komai-Koma; Xiao-qing Wei; Foo Y. Liew

306

Low-Dose Cyclosporin for Multiple Colonic Ulcers Associated with Mixed Connective Tissue Disease  

Microsoft Academic Search

:   This report describes a patient with multiple colonic ulcers and mixed connective tissue disease. The histological findings\\u000a of the colonic lesions showed vasculitis with T-cell infiltration, and the peripheral T cells were frequently in the activated\\u000a phase of the cell cycle. In this patient, low-dose cyclosporin treatment (2.5 mg\\/kg\\/day) inhibited the T-cell activation in\\u000a the peripheral lymphocytes and was

N. Maeda; S. Abe; T. Yoshizawa; K. Ogawa; I. Sekigawa; N. Iida; M. Eguchi; M. Matsumoto; H. Hashimoto; S. Hirose

1999-01-01

307

Two Cases of Frosted Branch Angiitis with Central Retinal Vein Occlusion  

Microsoft Academic Search

Background: Frosted branch angiitis usually occurs in children, and has a good prognosis. We report two cases of unilateral frosted branch angiitis in adults. Both had poor visual outcomes because of associated central retinal vein occlusion and neovascular glaucoma.Cases: Case 1 was a 36-year-old woman. Almost all retinal veins and some retinal arteries showed vasculitis in her right eye, and

Toshikatsu Kaburaki; Makoto Nakamura; Kazuhiro Nagasawa; Miyuki Nagahara; Satoru Joko; Yujiro Fujino

2001-01-01

308

Anti-neutrophil cytoplasmic autoantibodies (ANCA) to bactericidal\\/permeability-increasing (BPI) protein recognize the carboxyl terminal domain  

Microsoft Academic Search

Objectives: to identify the region of bactericidal\\/permeability-increasing protein (BPI) recognized by anti-BPI AN CA.Methods: sera from 140 patients with a variety of clinical diagnoses (20 systemic vasculitis, 12 cystic fibrosis, 22 bronchiectasis\\/chronic obstructive airways disease, three diabetes mellitus, 13 chronic renal failure, 12 primary sclerosing cholangitis, eight ulcerative colitis, three Crohn's disease, seven cancer, and 40 other or unknown diagnoses)

Austin C. Dunn; Russell S. Walmsley; Russell L. Dedrick; Andrew J. Wakefield; Christopher M. Lockwood

1999-01-01

309

Use of drug therapy to manage acute cutaneous necrosis of the skin.  

PubMed

Acute cutaneous necrosis is defined as a sudden onset of gangrenous skin changes in the skin, associated with significant morbidity and mortality. The following diseases are included in this discussion: coumadin necrosis, heparin necrosis, brown recluse spider bite, necrotizing fasciitis, vasculitis, pyoderma gangrenosum, calciphylaxis, clotting abnormalities and embolic phenomena. The importance of early diagnosis, early distinction and early drug therapy or drug withdrawal must match the diagnosis for maximal preservation of the skin and underlying tissue. PMID:20514791

Wallace, Jill S; Hall, John C

2010-04-01

310

Recurrent Meningitis and Subarachnoid Hemorrhage Due to Salmonella in an HIV+ Patient: Case Report and Mini-Review of the Literature  

PubMed Central

Meningitis due to non-typhi salmonella is infrequent in HIV-positive adults. We report a case of a patient with >300 CD4+ cells/mm3 who presented with five episodes of recurrent meningitis, focal subarachnoid hemorrhage and cerebral vasculitis ultimately attributed to Salmonella choleraesuis infection. Even within the cART era invasive salmonellosis can occur in unusual ways in HIV-infected patients. PMID:21772932

Belloso, Waldo H; Romano, Marina; Greco, Graciela S; Davey, Richard T; Perelsztein, Ariel G; Sánchez, Marisa L; Ajzenszlos, Martín R; Otegui, Inés M

2011-01-01

311

Fluorodeoxyglucose (FDG) uptake in pulmonary rheumatoid nodules diagnosed by video-assisted thoracic surgery lung biopsy: two case reports and a review of the literature.  

PubMed

Two cases of rheumatoid nodules evaluated by fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) and video-assisted thoracic surgery (VATS) biopsy are reported. The first case was that of a 44-year-old woman who presented with a cavitated nodule with intense standardized uptake values (SUVs) both in the early (max 3.4) and delayed (max 4.4) phases, suggesting malignancy. However, after VATS biopsy, she was diagnosed as having a rheumatoid nodule with vasculitis. The second case was that of a 74-year-old woman admitted with bilateral lung nodules, two of which showed intense early (max 2.2) and delayed (max 6.0) phase SUVs, and mild early (max 0.6) and delayed (max 0.9) phase SUVs. These two nodules were finally proven to be a lung cancer and rheumatoid nodule without vasculitis, respectively. These cases show that rheumatoid nodules with an enhanced inflammatory process, such as vasculitis, can appear false-positive for malignancy on FDG-PET/CT scan images. PMID:22669597

Saraya, Takeshi; Tanaka, Ryota; Fujiwara, Masachika; Koji, Hitoshi; Oda, Miku; Ogawa, Yukari; Nagatomo, Tomoko; Watanabe, Masato; Yokoyama, Takuma; Ishii, Haruyuki; Takei, Hidefumi; Goya, Tomoyuki; Takizawa, Hajime; Goto, Hajime

2013-03-01

312

Diagnosis and classification of autoimmune orchitis.  

PubMed

Autoimmune orchitis is characterized by testis inflammation and the presence of specific antisperm antibodies (ASA). It is classified in two categories. Primary autoimmune orchitis is defined by infertility and asymptomatic orchitis associated with ASA (100%) directed to the basement membrane or seminiferous tubules in infertile men, without any systemic disease and usually asymptomatic. Secondary autoimmune orchitis is characterized by symptomatic orchitis and/or testicular vasculiti`s associated with a systemic autoimmune disease, particularly vasculitis. These patients typically demonstrate testicular pain, erythema and/or swelling. ASA in secondary autoimmune orchitis have been reported in up to 50% of patients, especially in systemic lupus erythematosus patients. The pathogenesis of primary as well as secondary autoimmune orchitis is still unknown. Although the etiology is likely to be multifactorial, testicular inflammation, infection or trauma may induce T cell response with pro-inflammatory cytokine production with a consequent blood-testis-barrier permeability alteration, ASA production and apoptosis of spermatocytes and spermatids. ASA is known to cause immobilization and/or agglutination of spermatozoa, which may block sperm-egg interaction resulting in infertility. Assisted reproduction has been used as an efficient option in primary cases and immunosuppressive therapy for secondary autoimmune orchitis, although there is no double-blind, randomized trial to confirm the efficacy of any treatment regimens for these conditions. PMID:24424181

Silva, C A; Cocuzza, M; Carvalho, J F; Bonfá, E

2014-01-01

313

Pathology of Experimental Encephalitozoon cuniculi Infection in Immunocompetent and Immunosuppressed Mice in Iraq.  

PubMed

This study was performed to evaluate pathology of experimental Encephalitozoon cuniculi (Iraqi isolate) infection in normal and immunosuppressed mice. Pathological changes were not seen in negative control mice while secondary bacterial infections were noted in the lungs, kidneys, and heart of mice given dexamethasone. Typical E. cuniculi infection lesions were found in brain, livers, lungs, and kidneys of mice given 10(7)??E. cuniculi spores/mouse orally. These lesions were in the form of nonsuppurative meningoencephalitis with vasculitis in brain, interstitial inflammation with infiltration of both lymphocytes and plasma cells in lung tissue, and nonsuppurative interstitial (focal and diffuse) nephritis, presence of vacuole containing mature and immature spores in enterocytes within the tips of villi, and lymphoiod hyperplasia of the white pulp and vasculitis of the intratrabecular vessels. Mice that were given 10(7)??E. cuniculi spores/mouse orally showed lesions similar to those observed in the previous group (vasculitis and granulomas) but the lesions were more severe and widespread. In conclusion, this is the first report of experimental E. cuniculi infection induced by E. cuniculi isolated from a naturally infected rabbit in Iraq and that infection became more severe and widespread upon the administration of dexaethasone. PMID:24772366

Al-Sadi, Hafidh I; Al-Mahmood, Saevan S

2014-01-01

314

Spontaneous and antiviral-induced cutaneous lesions in chronic hepatitis B virus infection  

PubMed Central

AIM: To describe spontaneous, or interferon (IFN)- or immunization-induced skin lesions in hepatitis B virus (HBV) infection. METHODS: A comprehensive literature search of all the papers presenting case reports of dermatological lesions in patients with chronic HBV infection was carried out. We included only patients with histologically proven skin lesions that appeared in the normal course of hepatitis B infection, or after immunization for hepatitis B or antiviral treatment. RESULTS: We found 44 papers on this topic, reporting 151 cases. About 2% of patients with hepatitis B infection, mainly men, presented with skin lesions. Among patients with chronic hepatitis B, vasculitis and essential mixed cryoglobulinemia seemed to be the most frequent skin lesion (53.3%), followed by papular changes, rashes and Gianotti-Crosti syndrome, skin carcinoma and Henoch-Schönlein purpura were rare. IFN treatment seemed to be effective against HBV-associated and immunoglobulin-complex-mediated disease (vasculitis). Two cutaneous lesions (lichen planus and granuloma annulare) were described after hepatitis B vaccination. Systemic lupus and lupus-like lesions were the most frequently encountered lesions after antiviral treatment. Immunosuppressive and steroid therapy ameliorates lichen planus lesions in 50% of cases. CONCLUSION: Vasculitis was the most frequent spontaneous skin lesion found in chronic hepatitis B. Lichen planus was most frequent after immunization and lupus/lupus-like lesions after IFN.

Grigorescu, Ioana; Dumitrascu, Dan Lucian

2014-01-01

315

Pathology of Experimental Encephalitozoon cuniculi Infection in Immunocompetent and Immunosuppressed Mice in Iraq  

PubMed Central

This study was performed to evaluate pathology of experimental Encephalitozoon cuniculi (Iraqi isolate) infection in normal and immunosuppressed mice. Pathological changes were not seen in negative control mice while secondary bacterial infections were noted in the lungs, kidneys, and heart of mice given dexamethasone. Typical E. cuniculi infection lesions were found in brain, livers, lungs, and kidneys of mice given 107??E. cuniculi spores/mouse orally. These lesions were in the form of nonsuppurative meningoencephalitis with vasculitis in brain, interstitial inflammation with infiltration of both lymphocytes and plasma cells in lung tissue, and nonsuppurative interstitial (focal and diffuse) nephritis, presence of vacuole containing mature and immature spores in enterocytes within the tips of villi, and lymphoiod hyperplasia of the white pulp and vasculitis of the intratrabecular vessels. Mice that were given 107??E. cuniculi spores/mouse orally showed lesions similar to those observed in the previous group (vasculitis and granulomas) but the lesions were more severe and widespread. In conclusion, this is the first report of experimental E. cuniculi infection induced by E. cuniculi isolated from a naturally infected rabbit in Iraq and that infection became more severe and widespread upon the administration of dexaethasone. PMID:24772366

Al-Sadi, Hafidh I.; Al-Mahmood, Saevan S.

2014-01-01

316

Acute Retinal Necrosis in Childhood  

PubMed Central

Background Acute retinal necrosis (ARN) is a viral syndrome consisting of uveitis/vitritis, occlusive vasculitis and peripheral necrosis. Few incidents are reported in children. The etiology is reactivated herpes simplex virus (HSV) or varicella-zoster virus (VZV). Treatment with acyclovir is often used. The administration of oral glucocorticosteroids is of unproven benefit. Prognosis is variable but poor. Methods Three weeks after contracting mild chickenpox, a healthy 4-year-old girl developed blurred vision in her right eye. Severely reduced visual acuity was noted, together with anterior uveitis, ‘mutton-fat’ precipitates and vitral flare. Retinal vasculitis with necrosis was present. Serology for toxoplasma, cytomegalovirus and HIV was negative, while HSV and VZV IgG antibodies were positive. She was treated with 30 mg/kg of intravenous methylprednisolone (3 days), 30 mg of oral prednisone (3 days), and tapering for 8 weeks. Intravenous acyclovir was given for 10 days, followed by oral acyclovir for 4 months. Aspirin (100 mg/day) was given for 4 months. Results At 12 months, the girl felt good. Her right eye acuity was 6/9, with an intraocular pressure of 17 mm Hg. The peripheral retina showed scarring but no detachment. Conclusions This is the first report of a once-daily high-dose methylprednisolone pulse therapy in one of the youngest known ARN cases. Pulsed steroid therapy was based on its known effectiveness in vasculitis, which is the main pathophysiology in ARN. There was no evidence of steroid-related viral over-replication. Our case achieved an excellent clinical and ophthalmic recovery in spite of the poor prognosis. The positive result of this case report provides a basis for further evaluation of high-dose steroid pulse therapy in ARN. PMID:24932179

Pikkel, Yoav Y.; Pikkel, Joseph

2014-01-01

317

Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment.  

PubMed

Central nervous system (CNS) manifestations are not rare in pediatric rheumatic diseases. They may be a relatively common feature of the disease, as in systemic lupus erythematosus (SLE) and Behçet's disease. Direct CNS involvement of a systemic rheumatic disease, primary CNS vasculitis, indirect involvement secondary to hypertension, hypoxia and metabolic changes, and drug associated adverse events may all result in CNS involvement. We have reviewed the CNS manifestations of SLE, Behçet's disease, Henoch-Schönlein purpura, polyarteritis nodosa, juvenile idiopathic arthritis, juvenile ankylosing spondylitis, familial Mediterranean fever, scleroderma, sarcoidosis, Wegener's granulomatosis, Takayasu's arteritis, CINCA syndrome, Kawasaki disease, and primary CNS vasculitis; and adverse CNS effects of anti-rheumatic drugs in pediatric patients. The manifestations are diverse; ranging from headache, seizures, chorea, changes in personality, depression, memory and concentration problems, cognitive impairment, cerebrovascular accidents to coma, and death. The value of cerebrospinal fluid (CSF) examination (pleocytosis, high level of protein), auto-antibodies in serum and CSF, electroencephalography, neuroimaging with computerized tomography, magnetic resonance imaging, SPECT, PET, and angiography depends on the disease. Brain biopsy is gold standard for the diagnosis of CNS vasculitis, however it may be inconclusive in 25% of cases. A thorough knowledge of the rheumatic diseases and therapy-related adverse events is mandatory for the management of a patient with rheumatic disease and CNS involvement. Severe CNS involvement is associated with poor prognosis, and high mortality rate. High dose steroid and cyclophosphamide (oral or intravenous) are first choice drugs in the treatment; plasmapheresis, IVIG, thalidomide, and intratechal treatment may be valuable in treatment-resistant, and serious cases. PMID:18537653

Duzova, Ali; Bakkaloglu, Aysin

2008-01-01

318

Wegener granulomatosis (granulomatosis with polyangiitis): evolving concepts in treatment.  

PubMed

Wegener granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. THE TERM GRANULOMATOSIS WITH POLYANGIITIS (WEGENER) WAS RECENTLY PROPOSED TO REPLACE THE OLDER TERM, WG. THE TERM GRANULOMATOSIS WITH POLYANGIITIS CAN BE ABBREVIATED TO GPA, WITH THE IDEA THAT THE EPONYM WEGENER WOULD BE OMITTED OVER TIME. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. The spectrum and severity of the disease are heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antibodies against cytoplasmic components of neutrophils [anti-neutrophil cytoplasmic antibodies (c-ANCAs)] likely play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CSs) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3 to 6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, methotrexate combined with CS may be adequate for limited, non-life-threatening WG. Recent studies suggest that rituximab may be useful for induction therapy or CYC-refractory WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored. PMID:21674414

Lynch, Joseph P; Tazelaar, Henry

2011-06-01

319

Early-Onset Stroke and Vasculopathy Associated with Mutations in ADA2  

PubMed Central

BACKGROUND We observed a syndrome of intermittent fevers, early-onset lacunar strokes and other neurovascular manifestations, livedoid rash, hepatosplenomegaly, and systemic vasculopathy in three unrelated patients. We suspected a genetic cause because the disorder presented in early childhood. METHODS We performed whole-exome sequencing in the initial three patients and their unaffected parents and candidate-gene sequencing in three patients with a similar phenotype, as well as two young siblings with polyarteritis nodosa and one patient with small-vessel vasculitis. Enzyme assays, immunoblotting, immunohistochemical testing, flow cytometry, and cytokine profiling were performed on samples from the patients. To study protein function, we used morpholino-mediated knockdowns in zebrafish and short hairpin RNA knockdowns in U937 cells cultured with human dermal endothelial cells. RESULTS All nine patients carried recessively inherited mutations in CECR1 (cat eye syndrome chromosome region, candidate 1), encoding adenosine deaminase 2 (ADA2), that were predicted to be deleterious; these mutations were rare or absent in healthy controls. Six patients were compound heterozygous for eight CECR1 mutations, whereas the three patients with polyarteritis nodosa or small-vessel vasculitis were homozygous for the p.Gly47Arg mutation. Patients had a marked reduction in the levels of ADA2 and ADA2-specific enzyme activity in the blood. Skin, liver, and brain biopsies revealed vasculopathic changes characterized by compromised endothelial integrity, endothelial cellular activation, and inflammation. Knockdown of a zebrafish ADA2 homologue caused intracranial hemorrhages and neutropenia — phenotypes that were prevented by coinjection with nonmutated (but not with mutated) human CECR1. Monocytes from patients induced damage in cocultured endothelial-cell layers. CONCLUSIONS Loss-of-function mutations in CECR1 were associated with a spectrum of vascular and inflammatory phenotypes, ranging from early-onset recurrent stroke to systemic vasculopathy or vasculitis. (Funded by the National Institutes of Health Intramural Research Programs and others.) PMID:24552284

Zhou, Q.; Yang, D.; Ombrello, A.K.; Zavialov, Andrey V.; Toro, C.; Zavialov, Anton V.; Stone, D.L.; Chae, J.J.; Rosenzweig, S.D.; Bishop, K.; Barron, K.S.; Kuehn, H.S.; Hoffmann, P.; Negro, A.; Tsai, W.L.; Cowen, E.W.; Pei, W.; Milner, J.D.; Silvin, C.; Heller, T.; Chin, D.T.; Patronas, N.J.; Barber, J.S.; Lee, C.-C.R.; Wood, G.M.; Ling, A.; Kelly, S.J.; Kleiner, D.E.; Mullikin, J.C.; Ganson, N.J.; Kong, H.H.; Hambleton, S.; Candotti, F.; Quezado, M.M.; Calvo, K.R.; Alao, H.; Barham, B.K.; Jones, A.; Meschia, J.F.; Worrall, B.B.; Kasner, S.E.; Rich, S.S.; Goldbach-Mansky, R.; Abinun, M.; Chalom, E.; Gotte, A.C.; Punaro, M.; Pascual, V.; Verbsky, J.W.; Torgerson, T.R.; Singer, N.G.; Gershon, T.R.; Ozen, S.; Karadag, O.; Fleisher, T.A.; Remmers, E.F.; Burgess, S.M.; Moir, S.L.; Gadina, M.; Sood, R.; Hershfield, M.S.; Boehm, M.; Kastner, D.L.; Aksentijevich, I.

2014-01-01

320

Malignant catarrhal fever associated with ovine herpesvirus-2 in free-ranging mule deer in Colorado.  

PubMed

Malignant catarrhal fever (MCF) was diagnosed in four free-ranging mule deer (Odocoileus hemionus) in January and February of 2003. Diagnosis was based on typical histologic lesions of lymphocytic vasculitis and PCR identification of ovine herpesvirus-2 (OHV-2) viral genetic sequences in formalin-fixed tissues. The animals were from the Uncompahgre Plateau of southwestern Colorado. Deer from these herds occasionally resided in close proximity to domestic sheep (Ovis aries), the reservoir host of OHV-2, in agricultural valleys adjacent to their winter range. These cases indicate that fatal OHV-2 associated MCF can occur in free-ranging mule deer exposed to domestic sheep that overlap their range. PMID:17699095

Schultheiss, Patricia C; Van Campen, Hana; Spraker, Terry R; Bishop, Chad; Wolfe, Lisa; Podell, Brendan

2007-07-01

321

Odocoileus hemionus deer adenovirus is related to the members of Atadenovirus genus  

Microsoft Academic Search

Summary.  ?The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize\\u000a the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames\\u000a that mapped to pVIII hexon-associated protein precursor and fiber protein of several other adenoviruses. The highest amino\\u000a acid homology for

A. Zakhartchouk; A. Bout; L. W. Woods; H. D. Lehmkuhl; M. J. E. Havenga

2002-01-01

322

[Behçet's disease revealed by deep vein thrombosis in an HIV-1-infected patient].  

PubMed

Behçet's disease is a multisystemic vasculitis characterized by the association of recurrent oral and genital ulcers with systemic involvements, particularly ocular, nervous and vascular manifestations. The association of Behçet's disease and aids/HIV infection is rare. The role of HIV infection in the pathomechanism of both diseases is unclear. We report a 28-year-old HIV-positive patient who presented Behçet's disease complicated by deep vein thrombosis and left lateral sinus thrombosis. This rare association raises questions about the etiopathogenic process and therapeutic management. PMID:23068309

Marih, L; Sodqi, M; Marhoum El Filali, K; Chakib, A

2012-12-01

323

Endovascular Treatment of a Superior Mesenteric Artery Aneurysm Secondary to Behcet's Disease with Onyx (Ethylene Vinyl Alcohol Copolymer)  

SciTech Connect

Behcet's disease is a complex multisystemic chronic inflammatory disease that is characterized by oral and genital aphtous ulcers and vasculitis. Aneurysms of major arteries are the most important cause of mortality in Behcet's disease. Four patients with superior mesenteric artery (SMA) aneurysms related to Behcet's disease have been reported in the literature. We report here the first successful endovascular treatment of a giant, wide-necked SMA aneurysm secondary to Behcet's disease. We performed a balloon-assisted embolization technique using ethylene vinyl alcohol copolymer (Onyx, ev3, Irvine, CA, USA). There were no signs of recurrence during 2-year follow-up.

Gueven, Koray, E-mail: korayguven@yahoo.com; Rozanes, Izzet, E-mail: rozanes@superonline.co [Istanbul University, Department of Radiology, Istanbul Faculty of Medicine (Turkey); Kayabali, Murat, E-mail: murat.kayabali@veezy.co [Istanbul University, Section of Vascular Surgery, Department of General Surgery, Istanbul Faculty of Medicine (Turkey); Minareci, Ozenc, E-mail: minareci@doctor.co [Istanbul University, Department of Radiology, Istanbul Faculty of Medicine (Turkey)

2009-01-15

324

Hepatitis C-associated rheumatic disorders.  

PubMed

Hepatitis C virus (HCV) is an important causative agent of liver diseases. However, HCV infection is also associated with numerous hematologic, renal, dermatologic, rheumatic, and autoimmune disorders. These include arthralgia, arthritis, vasculitis, sicca syndrome, myalgia, and fibromyalgia. The purpose of this article is to review the prevalence and spectrum of rheumatic disorders and autoimmune phenomena in HCV-infected patients. It evaluates and current treatment options including nonsteroidal anti-inflammatory drugs, low-dose corticosteroids, hydroxychloroquine, methotrexate, penicillamine, combined antiviral therapy, cyclosporin A, anti-TNF-a agents, and rituximab. It concludes that larger, controlled studies are needed to establish further the treatment indications, efficacy, and safety of these agents. PMID:19481000

Buskila, Dan

2009-02-01

325

Leprosy with Guillain Barre Syndrome: a new neurologic manifestation?  

PubMed

A 19-year-old female patient of lepromatous leprosy with Type II reaction, on multidrug therapy and prednisolone, presented with acute onset flaccid quadriparesis. The cerebrospinal fluid examination revealed albumino-cytologic dissociation. Nerve biopsy showed infiltration with lepra bacilli, features of vasculitis, and demyelination. There were no other identifiable precipitating factors for Guillain Barre Syndrome in this patient. Her condition improved without any steroid therapy. This case emphasizes the hypothesis that cell injury caused by Type II reaction can expose neural antigens and incite an autoimmune reaction in the form of Guillain Barre Syndrome. PMID:15160867

Grover, Chander; Kubba, Samir; Nanda, Soni; Kumar, Vijay; Reddy, Belum Siva Nagi

2004-02-01

326

Familial Behçet's disease.  

PubMed

Behçet's disease (BD) is a multisystemic vasculitis syndrome characterized by a course of remissions and exacerbations of unpredictable frequency and duration. The disease has a worldwide distribution, but the majority of cases cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. The etiopathogenesis of BD is still unknown, but familial aggregation and peculiar geographical distribution have been regarded as evidence supporting genetic influence on the pathogenesis of BD. In this article, we describe a patient with BD, who had four members of his family associated with BD. PMID:19575202

Yilmaz, Sema; Cimen, Kadriye Akar

2010-06-01

327

[Neuro-Behçet in a Caucasian Danish patient].  

PubMed

Behçet's disease (BD) is a chronic, relapsing multisystem inflammatory disorder, vasculitis being its major pathological feature. It is prevalent in the Orient and areas along the ancient Silk Road, but very rare among North European Caucasians. Neurological involvement is relatively uncommon. The diagnosis of neuro-Behçet can be difficult to establish if the mucocutaneous involvement is incomplete, absent, or unattended. We present an exceptional case of neuro-Behçet in a 38-year-old Caucasian Danish woman. On account of the risk of serious complication, it is important to make the diagnosis as soon as possible. PMID:20184824

Karshena, Ali; Koch-Hendriksen, Nils J

2010-02-22

328

[Necrotizing fasciitis after varicella].  

PubMed

Necrotizing fasciitis is a rare and severe infection characterised by extremely rapid progressive involvement of the superficial fascias and deep dermal layers of the skin, with resultant vasculitis and necrosis. The authors present three clinical cases of necrotizing fasciitis; all three patients previously had varicella rash, rapid progressive spreading erythema with severe pain and toxic shock syndrome. Two patients had positive cultures of b-haemolytic streptococcus. Early stage differential diagnosis with celulitis, aggressive antibiotic treatment and pediatric intensive care support are essential. However, the main therapy is early extensive surgical approach involving all indurate areas, down to and including the muscle fascia. PMID:11552336

Gonçalves, E; Furtado, F; Estrada, J; Vale, M C; Pinto, M; Santos, M; Moura, G; Vasconcelos, C

2001-01-01

329

[Chlamydial infection of the central nervous system. Laboratory diagnosis and clinic and morphological features].  

PubMed

The paper presents data on the diagnosis, clinical and pathomorphological changes in the central nervous system (CNS) in neurochlamydiasis according to clinical, autoptic, and experimental evidence. It discusses the possible implication of Ch. trachomatis, Ch. pneumoniae, and Ch. psittaci in the development and course of different diseases with CNS involvement: atherosclerosis, vasculitis, multiple sclerosis, Alzheimer's disease, schizophrenia, autism, vegetative state, sequels of perinatal lesions in childhood and adolescence, HIV infection, etc. Considerable attention is paid to the specific features of diagnosis of Chlamydia-induced CNS lesions. Purposeful pathomorphological investigations are shown to be needed. PMID:24745195

Va?nshenker, Iu I; Nuralova, I V; Onishenko, L S

2014-01-01

330

Role of neutrophils in murine cryoglobulinemia  

Microsoft Academic Search

Murine IgG3 anti-IgG2a rheumatoid factor (RF) monoclonal antibodies (mAb) with cryoglobulin activity, are able to induce, in normal mice, skin leukocytoclastic vasculitis and lupus-like glomerulonephritis resembling 'wire-loop' lesions (subendothelial immune deposits). The development of glomerular, but not skin, lesions in immunoglobulin-deficient mice (lacking the corresponding IgG2a autoantigen) receiving IgG3 RF cryoglobulins indicates that the RF activity of IgG3 monoclonal cryoglobulins

S. Izui; T. Fulpius; L. Reininger; Y. Pastore; T. Kobayakawa

1998-01-01

331

Giant Cell Arteritis in a 12-Year-Old Girl Presenting with Nephrotic Syndrome  

PubMed Central

Giant cell arteritis (GCA) is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities.

El-Sayed, Zeinab A.; El-Awady, Hanaa M.; Hassan, Zeinab E.; Adham, Tamer M. H.; Mostafa, Hossam M.; Elhefnawy, Nadia G.

2014-01-01

332

Extracorporeal removal of circulating immune complexes: from non-selective to patient-specific.  

PubMed

The classical immune complex-mediated disease, termed serum sickness, developed a short time after the injection of horse anti-tetanus toxin. Antibodies against circulating horse plasma proteins lead to the formation of immune complexes within the blood circulation (CIC). The inflammatory response, including systemic complement activation and vasculitis, seriously affected the function of all organs, including the most susceptible kidney. Meanwhile CIC have been detected in almost every systemic disease, including autoimmune disorders and also cancer and infections. This brief review will focus on the rationale and the equipment for extracorporeal elimination of CIC. PMID:10838476

Matic, G; Schütt, W; Winkler, R E; Tiess, M; Ramlow, W

2000-01-01

333

Clinical presentation of Churg-Strauss syndrome in children  

PubMed Central

Churg–Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg–Strauss syndrome. The propositus included, 50 cases of childhood Churg–Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg–Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome.

Razenberg, Femke G.E.M.; Heynens, Jan W.C.M.; Jan de Vries, Geeuwke; Duijts, Liesbeth; de Jongste, Johan C.; de Blic, Jacques; Rosias, Philippe P.R.

2012-01-01

334

Granuloma annulare with a mycosis fungoides-like distribution and palisaded granulomas of CD68-positive histiocytes.  

PubMed

We describe 3 unusual cases of granuloma annulare with multiple macular lesions in a distribution that simulated mycosis fungoides in patients with no associated underlying diseases. Repeated biopsies showed typical well-formed palisading granulomas and no evidence of an atypical lymphocytic infiltrate. There was no vasculitis, neutrophilic, eosinophilic, or interstitial infiltrate. The patients had no associated underlying diseases. Most of the histiocytes in the palisading granulomas were strongly positive for CD68. The lymphocytes were a minor component of the granulomatous inflammation and were predominantly CD8(+) T-cells. The findings in these cases add to the spectrum of previously defined granulomatous eruptions of the skin. PMID:15243522

Wu, Hong; Barusevicius, Alan; Lessin, Stuart R

2004-07-01

335

Central venous catheter infection-induced Henoch-Schönlein purpura in a patient on hemodialysis.  

PubMed

A 69-year-old man, who had been dialyzed using a permanent central venous catheter for 2 years, presented with Henoch-Schönlein purpura and positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). He was diagnosed with catheter-related infection by Staphylococcus aureus. After administration of antibiotic and steroid therapy, purpura disappeared and p-ANCA gradually became negative. This case supports the conclusion that infection can be pathogenesis of the vasculitis, including ANCA-positive HSP. Additionally, impregnation of catheters with antibiotics can be an effective treatment for catheter infections. PMID:24845224

Gao, Jian Jun; Wei, Jia Mei; Gao, Yue Hua; Li, Shuang; Na, Yu

2014-08-01

336

Cardiac denervation procedure to treat refractory angina in a patient with Churg-Strauss syndrome and non-obstructive coronary lesions  

PubMed Central

Cardiac involvement in Churg–Strauss syndrome is common and represents the main cause of mortality. We report the case of a patient with Churg–Strauss vasculitis, mitral regurgitation with left ventricular dysfunction, paroxysmal atrial fibrillation and refractory angina with non-significant coronary lesions. Cardiac denervation was proposed as an associated procedure to treat angina. The total removal of peri-adventitial and adventitial tissue around the superior vena cava, ascending aorta and main pulmonary trunk was performed. After 3 months of follow-up, the patient was angina-free and could resume his normal lifestyle. PMID:22493151

Cura, Leandro; Dayan, Victor; Cristar, Florencia; Soca, Gerardo

2012-01-01

337

Unfamiliar Manifestations of Anti-tubercular Therapy  

PubMed Central

Cutaneous drug reactions are frequent in hospitalized patients and vary from simple manifestations like rash and erythema to severe life threatening conditions like angio-oedema, erythroderma, Stevens-Johnson syndrome and toxic epidermal necrolysis. However drug eruptions with antitubercular drugs are largely unknown except few case reports. We highlight here one similar case which presented with pleomorphic cutaneous manifestations after taking anti tubercular therapy and closely mimicked vasculitis. But when the offending drugs were stopped the lesions disappeared and the patient improved. PMID:24791242

Aggarwal, Ramesh; Dwivedi, Shridhar; Aggarwal, Meenakshi

2014-01-01

338

A clinicopathological classification of granulomatous disorders  

PubMed Central

Granulomatous disorders comprise a large family sharing the histological denominator of granuloma formation. A granuloma is a focal compact collection of inflammatory cells, mononuclear cells predominating, usually as a result of the persistence of a non-degradable product and of active cell mediated hypersensitivity. There is a complex interplay between invading organism or prolonged antigenaemia, macrophage activity, a Th1 cell response, B cell overactivity and a vast array of biological mediators. Differential diagnosis and management demand a skilful interpretation of clinical findings and pathological evidence. They are classified into infections, vasculitis, immunological aberration, leucocyte oxidase deficiency, hypersensitivity, chemicals, and neoplasia.???Keywords: granuloma; Th1 cell; cytokines; neoplasia PMID:10908370

James, D

2000-01-01

339

A young patient with history of Kawasaki disease presenting with triple vessel disease.  

PubMed

Kawasaki disease is primarily a condition that affects young children and it is associated with cardiac morbidity and mortality. This disease has been known to cause coronary artery aneurysms which occurs as a sequelae of vasculitis. The progression of triple vessel disease in adult which results from cardiac complications from Kawasaki disease is rare. We report a case of a young man with history of Kawasaki disease at infancy presenting with triple vessel disease requiring cardiac bypass surgery at the age of 20 years old. PMID:22822650

Lim, A L; Lam, H Y; Kareem, B A; Kamarulzaman, M H

2012-04-01

340

Beh?et's disease  

PubMed Central

Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.???Keywords: Behçet's disease; oral ulcers; uveitis; immunosuppressants PMID:11009577

Kontogiannis, V; Powell, R

2000-01-01

341

How anti-neutrophil cytoplasmic autoantibodies activate neutrophils.  

PubMed

Neutrophils are pivotal to host defence during infectious diseases. However, activated neutrophils may also cause undesired tissue damage. Ample examples include small-vessel inflammatory diseases (vasculitis) that are associated with anti-neutrophil cytoplasmic autoantibodies (ANCA) residing in the patients' plasma. In addition to being an important diagnostic tool, convincing evidence shows that ANCA are pathogenic. ANCA-neutrophil interactions induce important cellular responses that result in highly inflammatory necrotizing vascular damage. The interaction begins with ANCA binding to their target antigens on primed neutrophils, proceeds by recruiting transmembrane molecules to initiate intracellular signal transduction and culminates in activation of effector functions that ultimately mediate the tissue damage. PMID:22861361

Kettritz, R

2012-09-01

342

Splenic Infarct Due to Scrub Typhus  

PubMed Central

Scrub typhus is a mite borne infectious disease that has the potential to involve multiple organs and can be fatal. Involvement of the abdomen in the form of hepatitis, gastric ulcerations and pancreatitis are well-documented, the pathology being disseminated vasculitis. However involvement of the spleen in scrub typhus is extremely rare and is reported only in a few autopsy studies. We report the case of a 50-year-old lady who presented with fever and left upper quadrant abdominal pain due to a splenic infarct due to scrub typhus. PMID:24926171

Raj, Selvin Sundar; Krishnamoorthy, Adhiti; Jagannati, Manjeera; Abhilash, Kundavaram PP

2014-01-01

343

Diffuse Alveolar Hemorrhage in a 39-year-old Woman: Unusual Initial Presentation of Microscopic Polyangiitis  

PubMed Central

Microscopic polyangiitis (MPA) is a necrotizing vasculitis involving the small vessels without granulomatous inflammation. Most MPA initially presents with renal involvement without pulmonary involvement. Isolated and initially presenting alveolar hemorrhage is very rare. The patient was a 39-year-old female with a progressive cough, dyspnea, and blood-tinged sputum for the previous 5 days. We determined that her condition was MPA though VATS lung biopsy and renal biopsy. After 2 months of steroid therapy, the chest lesions had improved. We report here a rare case of MPA with isolated and initial involvement of the lung with a review of the literature. PMID:22324034

Kim, Jae Jun; Wang, Young-Pil; Park, Hyung Joo; Sung, Sook-Whan; Kim, Do-Yeon

2011-01-01

344

Hemorrhagic colitis with unusual colonoscopy features, complicated with chronic graft-versus-host disease after allogeneic bone marrow transplantation.  

PubMed

A 32-year-old man was admitted after bone marrow transplantation because of hematochezia. He had history of chronic graft-versus-host disease (GVHD) of the skin and the liver, and cytomegaloviral pneumonia. Barium enema and colonoscopy showed multiple colon ulcers in the ascending and transverse colon. This feature is very rare in chronic GVHD and resembles the feature in autoimmune disease such as periarteritis nodosa. Thus, this ulceration is thought to be caused by vasculitis due to an autoimmune reaction in chronic GVHD. PMID:7742748

Murayama, T; Nakagawa, T; Matsushita, K; Matozaki, S; Yasutake, K; Kizaki, T; Sashikata, T

1995-01-01

345

Lymphocytic thrombophilic arteritis: an enigma.  

PubMed

A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report. PMID:25284860

Kalegowda, Inchara Yeliur; Tirumalae, Rajalakshmi; Murthy, K Srinivasa; Rout, Pritilata

2014-09-01

346

Comparative study of group A and group C meningococcal infection.  

PubMed Central

114 patients with meningococcal infection were studied; 72 had group C infection and 42 group A infection. 14 patients had acute meningococcaemia, all of whom had group C infection and 9 of whom died. Clinical and laboratory findings were similar in patients with meningitis due to a group A and C organisms, but arthritis and cutaneous vasculitis were more common in patients with group C infection. The overall mortality was 22% in patients with group C infection, and 12% in patients with group A infection, but was the same in both groups when cases of acute meningococcaemia are excluded. PMID:860875

Evans-Jones, L G; Whittle, H C; Onyewotu, I I; Egler, L J; Greenwood, B M

1977-01-01

347

Zygomycotic necrotizing fasciitis caused by Apophysomyces elegans.  

PubMed Central

A case of necrotizing fasciitis of the anterior abdominal wall caused by the zygomycete Apophysomyces elegans in a healthy male following inguinal herniorrhaphy is reported. The portal of entry of the fungus into the incised skin and subcutaneous tissues was probably through either contaminated surgical sutures or postoperative surgical dressings. Broad, aseptate fungal hyphae were seen in the necrosed tissues with an associated necrotizing vasculitis. Extensive tissue debridements and a low dose of amphotericin B were not successful in controlling the rapid invasion of the tissues by the fungus. Images PMID:8501244

Lakshmi, V; Rani, T S; Sharma, S; Mohan, V S; Sundaram, C; Rao, R R; Satyanarayana, G

1993-01-01

348

Zygomycotic necrotizing fasciitis caused by Apophysomyces elegans.  

PubMed

A case of necrotizing fasciitis of the anterior abdominal wall caused by the zygomycete Apophysomyces elegans in a healthy male following inguinal herniorrhaphy is reported. The portal of entry of the fungus into the incised skin and subcutaneous tissues was probably through either contaminated surgical sutures or postoperative surgical dressings. Broad, aseptate fungal hyphae were seen in the necrosed tissues with an associated necrotizing vasculitis. Extensive tissue debridements and a low dose of amphotericin B were not successful in controlling the rapid invasion of the tissues by the fungus. PMID:8501244

Lakshmi, V; Rani, T S; Sharma, S; Mohan, V S; Sundaram, C; Rao, R R; Satyanarayana, G

1993-05-01

349

HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy.  

PubMed

Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity. PMID:22852104

Cernea, Daniela; Dragoescu, Alice; Novac, Marius

2012-01-01

350

HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy  

PubMed Central

Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity. PMID:22852104

Cernea, Daniela; Dragoescu, Alice; Novac, Marius

2012-01-01

351

Behcet's disease and cardiovascular involvement: our experience of asymptomatic Behcet's patients  

PubMed Central

Summary Abstract Behcet’s syndrome is a systemic inflammatory disease associated with vasculitis, and arterial, venous and cardiac disorders. Thirty-eight Behcet’s disease patients were examined prospectively with echocardiography, ultrasonography and computed tomography, and coagulation parameters were determined. Deep venous insufficiency was found in 16 patients, venous thrombosis in seven, one patient had iliac artery stenosis, three had carotid arterial intimal proliferation, two patients had aortic annulus dilatation, six had aortic valve insufficiency, and three had mitral valve insufficiency. None had coagulation defects. To decrease morbidity and mortality rates, a multidisciplinary approach is important for early diagnosis of cardiovascular involvement in Behcet’s disease. PMID:24844550

Ulusan, Zeynep; Karadag, Ayse Serap; Tasar, Mehmet; Kalender, Mehmet; Tansel Darcin, Osman

2014-01-01

352

Imaging Characteristics of Takayasu Arteritis  

SciTech Connect

Takayasu arteritis is a rare, large-vessel vasculitis in which the nonspecific systemic inflammatory symptoms are followed by inflammation of the aorta and its major branches. The inflammation of this vessel leads to progressive luminal stenosis or aneurysm formation resulting in limb or organ ischemia. Although conventional angiography is still accepted as the gold standard modality, the information obtained is limited to the vessel lumen. Multidetector computed tomographic angiography and magnetic resonance angiography can provide valuable information not only regarding intraluminal pathologies but also concerning the thickening of the vessel wall, which may be the earliest manifestation of the disease.

Canyigit, Murat, E-mail: mcanyigit@yahoo.com; Peynircioglu, Bora; Hazirolan, Tuncay; Dagoglu, Merve Gulbiz; Cil, Barbaros Erhan; Haliloglu, Mithat; Balkanci, Ferhun; Besim, Aytekin [Faculty of Medicine, Hacettepe University, Department of Radiology (Turkey)

2007-07-15

353

Repeated surgeries in a patient with Beh?et's disease, what changes to expect?  

PubMed Central

Behçet's disease is a multisystem inflammatory disorder of unknown etiology characterized by vasculitis of large and smaller vessels. Most common features are appearance of oral ulcers which later heal by scarring and ocular involvement leading to glaucoma requiring surgical treatment. Here, we report a case of child suffering from Behçet's disease undergoing repeated ocular surgery for uncontrolled glaucoma and found that with the usual risk involved in such patients there are important changes in the airway when the patient is exposed to repeated surgery under general anesthesia.

Gupta, Anurag; Banerjee, Neerja; Sharma, Jyoti; Sood, Rajesh

2013-01-01

354

In silico design, synthesis, and assays of specific substrates for proteinase 3: influence of fluorogenic and charged groups.  

PubMed

Neutrophil serine proteases are specific regulators of the immune response, and proteinase 3 is a major target antigen in antineutrophil cytoplasmic antibody-associated vasculitis. FRET peptides containing 2-aminobenzoic acid (Abz) and N-(2,4-dinitrophenyl)ethylenediamine (EDDnp) as fluorophore and quencher groups, respectively, have been widely used to probe proteases specificity. Using in silico design followed by enzymatic assays, we show that Abz and EDDnp significantly contribute to substrate hydrolysis by PR3. We also propose a new substrate specific for PR3. PMID:24397704

Narawane, Shailesh; Budnjo, Adnan; Grauffel, Cédric; Haug, Bengt Erik; Reuter, Nathalie

2014-02-13

355

[A case of early recognized Wegener's granulomatosis with renal involvement. Insights for early diagnosis].  

PubMed

Systemic necrotic and granulomatous vasculitis presents with vascular involvement in almost every organ, manifesting with stenosis, ischemia, infarction, or hemorrhage. The clinical picture depends on the kind and size of the involved vessels and on the activity of the inflammatory process. Wegener's granulomatosis is a necrotic, granulomatous inflammation of small vessels of the upper and lower airways and kidneys. We present the case of a 25-year-old male in whom the definitive diagnosis of Wegener's granulomatosis was achieved within five weeks from onset. Among the first symptoms were microinfarcts localized in the fingertips and the nail matrix; their nature was investigated with capillaroscopy. PMID:21427813

Matuszewska, Agnieszka; Misterska-Skóra, Maria; Wiland, Piotr

2010-01-01

356

Fistulization of rheumatoid joints. Spectrum of identifiable syndromes.  

PubMed Central

Eight patients with rheumatoid arthritis developed cutaneous fistulae adjacent to affected joints. Rheumatoid factor was positive in eight patients; subcutaneous nodules were noted in seven. Two patients had features of rheumatoid vasculitis. A spectrum of syndrome characterized by cutaneous fistulae was observed. Three patients showed classical fistulous rheumatism. Four patients developed septic arthritis which subsequently fistulized; in two, infection was associated with total joint replacement. One patient showed a cutaneous sinus accompanying a large calf cyst. A variety of diagnoses must be considered when cutaneous fistulae appear near joints in patients with rheumatoid arthritis. Images PMID:1221937

Shapiro, R F; Resnick, D; Castles, J J; D'Ambrosia, R D; Lipscomb, P R

1975-01-01

357

Lymphocytic Thrombophilic Arteritis: An Enigma  

PubMed Central

A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report.

Kalegowda, Inchara Yeliur; Tirumalae, Rajalakshmi; Murthy, K Srinivasa; Rout, Pritilata

2014-01-01

358

Generalized vasculitic exanthem following Loxosceles reclusa envenomation.  

PubMed

Brown recluse spider bites (BRSB) cause a myriad of reactions ranging from local necrosis to potentially lethal systemic involvement. Envenomation may induce a generalized exanthem known clinically but not described histologically. We report a 49-year-old female who developed a generalized exanthem 24 hours after BRSB. The histopathology demonstrated a necrotizing vasculitis similar to that seen at an envenomation site but without epidermal necrosis. Loxoscelism should be considered in patients residing in endemic areas who present with a vasculitic exanthem. PMID:17518783

Robb, Christopher W; Hayes, Benjamin B; Boyd, Alan S

2007-06-01

359

Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature  

PubMed Central

About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents. PMID:22811887

Palejwala, Neal V.; Walia, Harpreet S.; Yeh, Steven

2012-01-01

360

Giant aortic arch aneurysm complicating Kawasaki's disease  

PubMed Central

Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD. This complication was managed by steroids therapy in the beginning. Because of mechanical complication and potential risk of rupture, surgery was undertaken.

Hakim, Kaouthar; Boussada, Rafik; Chaker, Lilia; Ouarda, Fatma

2014-01-01

361

Marked Acceleration of Atherosclerosis following Lactobacillus casei induced Coronary Arteritis in a Mouse Model of Kawasaki Disease  

PubMed Central

Objective To investigate if Lactobacillus casei cell wall extract (LCWE)-induced Kawasaki Disease (KD) accelerates atherosclerosis in hypercholesterolemic mice. Method and Resuslts Apoe?/? or Ldlr?/? mice were injected with LCWE (KD mice) or PBS, fed high fat diet for 8 weeks, and atherosclerotic lesions in aortic sinuses (AS), arch (AC) and whole aorta were assessed. KD mice had larger, more complex aortic lesions with abundant collagen, and both extracellular and intracellular lipid and foam cells, compared to lesions in control mice despite similar cholesterol levels. Both Apoe?/? KD and Ldlr?/? KD mice showed dramatic acceleration in atherosclerosis vs. controls, with increases in en face aortic atherosclerosis and plaque size in both the AS and AC plaques. Accelerated atherosclerosis was associated with increased circulating IL-12p40, IFN-?, TNF-?, and increased macrophage, DC, and T cell recruitment in lesions. Furthermore, daily injections of the IL-1Ra, which inhibits LCWE induced KD vasculitis, prevented the acceleration of atherosclerosis. Conclusions Our results suggest an important pathophysiologic link between coronary arteritis/vasculitis in the KD mouse model and subsequent atherosclerotic acceleration, supporting the concept that a similar relation may also be present in KD patients. These results also suggest that KD in childhood may predispose to accelerated and early atherosclerosis as adults. PMID:22628430

Chen, Shuang; Lee, Young Ho; Crother, Timothy R.; Fishbein, Michael; Zhang, Wenxuan; Yilmaz, Atilla; Shimada, Kenichi; Schulte, Danica J; Lehman, Thomas J.A.; Shah, Prediman K.; Arditi, Moshe

2012-01-01

362

Sclerosing Cholangitis in Beh?et's Disease  

PubMed Central

Introduction. Sclerosing cholangitis is characterized by an inflammatory and fibrotic lesion of intra- and/or extrahepatic bile ducts. When a causal mechanism of a bile duct lesion is identified, the sclerosing cholangitis is considered secondary. The vasculitis, including the Behçet disease, is cited as a probable cause of the ischemia and the sclerosing cholangitis. No cases of extrahepatic secondary sclerosing cholangitis have been reported to date. Case Report. We report the first case of secondary sclerosing cholangitis of the extrahepatic bile ducts associated with Behçet disease in a male who is aged 43, with a previous history of the angio-Behçet followed by complications of thrombophlebitis and a cerebral thrombophlebitis, and who has a cholestatic jaundice. The diagnosis has been carried out by the MR cholangiopancreatography which has objectified a moderate distension of the intrahepatic bile ducts upstream of regular stacked parietal thickening of the main bile duct. The patient has been treated successfully with the ursodeoxycholic acid and the placement of a plastic stent. Conclusion. This diagnosis should be mentioned to any patient with vasculitis and who has a cholestatic jaundice. PMID:24198838

Ben Slama Trabelsi, Aida; Issaoui, Dhilel; Ksiaa, Mehdi; Souguir, Ahlem; Mama, Nadia; Brahem, Ahlem; Jmaa, Ali; Ajmi, Salem

2013-01-01

363

In Vitro Global Gene Expression Analyses Support the Ethnopharmacological Use of Achyranthes aspera  

PubMed Central

Achyranthes aspera (family Amaranthaceae) is known for its anticancer properties. We have systematically validated the in vitro and in vivo anticancer properties of this plant. However, we do not know its mode of action. Global gene expression analyses may help decipher its mode of action. In the absence of identified active molecules, we believe this is the best approach to discover the mode of action of natural products with known medicinal properties. We exposed human pancreatic cancer cell line MiaPaCa-2 (CRL-1420) to 34??g/mL of LE for 24, 48, and 72 hours. Gene expression analyses were performed using whole human genome microarrays (Agilent Technologies, USA). In our analyses, 82 (54/28) genes passed the quality control parameter, set at FDR ? 0.01 and FC of ?±2. LE predominantly affected pathways of immune response, metabolism, development, gene expression regulation, cell adhesion, cystic fibrosis transmembrane conductance regulation (CFTR), and chemotaxis (MetaCore tool (Thomson Reuters, NY)). Disease biomarker enrichment analysis identified LE regulated genes involved in Vasculitis—inflammation of blood vessels. Arthritis and pancreatitis are two of many etiologies for vasculitis. The outcome of disease network analysis supports the medicinal use of A. aspera, viz, to stop bleeding, as a cure for pancreatic cancer, as an antiarthritic medication, and so forth. PMID:24454496

Subbarayan, Pochi R.; Sarkar, Malancha; Lokeshwar, Balakrishna L.; Ardalan, Bach

2013-01-01

364

Lupus Enteritis as an Initial Presentation of Systemic Lupus Erythematosus  

PubMed Central

Systemic lupus erythematosus (SLE) is an autoimmune disorder which can affect multiple organs and clinical presentation is often a myriad of symptoms; therefore, the index of suspicion should rise when evaluating patients with multiorgan symptomatology. Lupus enteritis is a distinct subset of SLE, defined as either vasculitis or inflammation of the small bowel, with supportive image and/or biopsy findings. The clinical picture of lupus enteritis is often nonspecific, with mild to severe abdominal pain, diarrhea, and vomiting being the cardinal manifestations. Although considered a form of visceral or serosal vasculitis, lupus enteritis is seldom confirmed on histology, making computerized tomography (CT) the gold standard for diagnosis. Lupus enteritis is generally steroid-responsive, and the route of administration is based on clinical status and organ involvement, with preference for intravenous (IV) route in flares with significant tissue edema. The following case describes a young woman presenting with lupus enteritis and lupus panniculitis as an initial manifestation of SLE, the utilization of abdominal CT in diagnosis, and current treatment protocols used for lupus enteritis. PMID:25295199

Sran, Manpreet; Patel, Narmisha

2014-01-01

365

Fluorescence endoscopic imaging study of anastomotic recurrence of Crohn's disease after right ileocolonic resection  

NASA Astrophysics Data System (ADS)

Crohn's disease is an inflammatory bowel disease of unknown etiology. Vasculitis is hypothesized but it was never demonstrated in vivo. This study aimed to evaluate the vascular mucosa perfusion using fluorescence imaging in 13 patients who had previously undergone eileocolonic resection and who agreed to participate in a prospective endoscopic study of anastomotic recurrence. This anastomotic recurrence rate is known to be high (73% after 1 year follow-up) and is characterized by ulcerations. The fluorescence study was started with an I.V. bolus injection of sodium fluorescein. The pre-anastomotic mucosa was endoscopically examined with blue light that stimulates fluorescein fluorescence. Fluorescence emission was recorded with an ultra-high-sensitivity camera connected to the endoscope via an interference filter (520 - 560 nm). A uniform fluorescence was observed a few seconds after the injection and lasted for 15 min in healthy subjects. In case of recurrence, the centers of the ulcerations displayed a very low fluorescence indicating localized ischemia. In contrast, the rims of the ulcers revealed brighter fluorescent images than those of normal mucosa. The anastomotic ulcerations of Crohn's disease recurrence exhibit a high fluorescence intensity at their margins indicating an increased mucosal blood flow and/or enhanced transcapillary diffusion. These findings support the hypothesis of a primary vasculitis in Crohn's disease.

Mordon, Serge R.; Maunoury, Vincent; Klein, Olivier; Colombel, Jean-Frederic

1995-12-01

366

Gastrointestinal manifestations of Behçet's disease.  

PubMed

Behçet's disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of life are mainly affected, with abdominal pain being the most common symptom. The ileocecal region is most commonly affected, with ulcerations that may penetrate or perforate. Rarely, the esophagus and stomach may have ulcerations. Bowel wall thickening is the most common finding on computed tomography (CT) scan. Pathology shows a vasculitis mainly involving the small veins or, alternatively, nonspecific inflammation. Corticosteroids, with or without other immunosuppressive drugs, are used for severe eye disease. Their use in intestinal disease is largely empirical. Surgery may be required for perforation. Behçet's disease runs a chronic, unpredictable course with exacerbations and remissions which decrease in frequency and severity over time. Death is mainly due to major vessel disease and neurological involvement. PMID:18594975

Ebert, Ellen C

2009-02-01

367

Branch retinal artery occlusion associated with posterior uveitis  

PubMed Central

Background The purpose of this study is to report the clinical features and visual outcome of branch retinal artery occlusion (BRAO) associated with posterior uveitis. This is a retrospective study including the 18 eyes of 18 patients. All patients underwent a complete ophthalmic evaluation. Fundus photography, fluorescein angiography, and visual field testing were performed in all cases. Results Diseases associated with BRAO included active ocular toxoplasmosis in 7 patients, rickettsiosis in 4, Behçet’s uveitis in 2, West Nile virus infection in 1, idiopathic retinal vasculitis in 1, Crohn’s disease in 1, ocular tuberculosis in 1, and idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome in 1 patient. The mean initial visual acuity was 20/50. BRAO involved the first order retinal artery in 33.3% of the eyes, the second order retinal artery in 33.3%, an arteriole in 27.8%, and a cilioretinal artery in 5.5%. The macula was involved in 44.4% of the eyes and an acute focus of retinitis or retinochoroiditis was associated to BRAO in 55.5%. Repermeabilization of the occluded artery occurred in all patients with permanent scotomas in the corresponding visual field. The mean visual acuity at last visit was 20/32. Conclusions BRAO, with subsequent visual impairment, may occur in the eyes with posterior uveitis. Physicians should be aware of such vision-threatening complication of infectious and inflammatory eye diseases. PMID:23514435

2013-01-01

368

Adamantiades-Behcet's disease-complicated gastroenteropathy.  

PubMed

Adamantiades-Behcet's disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and most frequently affects young adults between the second and fourth decades of life. ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality, with abdominal pain as the most common symptom. The ileocecal region is affected predominantly, with ulcerations that may lead to penetration and/or perforation, whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected. Endoscopy is useful to locate the site and extent of the lesions, and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or, alternatively in some cases, nonspecific inflammation. Bowel wall thickening is the most common finding on computed tomography scan. Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease, and the unpredictable course with exacerbation and remission. Corticosteroids with or without other immunosuppressive drugs, such as cyclophosphamide, azathioprine, sulfasalazine, tumor necrosis factor ? antagonist or thalidomide should be applied before surgery, except in emergency. PMID:22363131

Wu, Qing-Jun; Zhang, Feng-Chun; Zhang, Xuan

2012-02-21

369

Behçet's Uveitis.  

PubMed

Behçet's disease is a multisystem inflammatory disorder that is most common in countries along the ancient "Silk Road". The eye is the most commonly involved vital organ in Behçet's patients and the typical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Uveitis is the initial manifestation of the disease in 10-15% of the patients. Anterior uveitis is always nongranulomatous. Diffuse vitritis, retinal infiltrates, sheathing of predominantly retinal veins, and occlusive vasculitis are the typical signs of posterior segment inflammation. Spontaneous resolution of acute inflammatory signs is a diagnostic feature. Fundus fluorescein angiography is the gold standard in monitoring inflammatory activity. Laser flare photometry is a useful noninvasive tool since flare readings correlate with fluorescein angiographic leakage. The most common complications are cataract, maculopathy, and optic atrophy. Male patients have a more severe disease course and worse visual prognosis. Immunomodulatory therapy is indicated in all patients with posterior segment involvement. Corticosteroids combined with azathioprine and/or cyclosporine is used initially. Biologic agents, including interferon alfa and infliximab, are used in resistant cases. Visual prognosis has improved in recent years with an earlier and more aggressive use of immunomodulatory therapy and the use of biologic agents in resistant cases. PMID:20404988

Tugal-Tutkun, Ilknur

2009-10-01

370

A review on the vascular features of the hyperimmunoglobulin E syndrome.  

PubMed

Autosomal recessive, autosomal dominant and the sporadic forms of hyperimmunoglobulin E syndrome (HIES) are multi-system disorders. Although HIES patients may present with cold abscesses, the vascular features of HIES are not well recognized. The objective of this review is to characterize the nature and spectrum of vascular abnormalities in HIES patients. Vascular abnormalities in HIES patients were reviewed with Medline and Google Scholar-based searches. In brief, the searches combined terms related to HIES with the terms related to vasculature. Furthermore, reference lists from the original studies and review papers identified were screened. There were vascular abnormalities in 25 patients with HIES. These abnormalities were identified as aneurysms (coronary, aortic, carotid and cerebral), pseudoaneurysms, congenital patent ductus venosus, superior vena cava syndrome, vasculitides, vascular ectasia, thrombosis and others. They may be congenital or acquired, in the veins and arteries, affecting both sexes. These abnormalities can be seen in all subtypes of HIES. They could be also fatal in children and adults. Limited pathological investigations revealed the presence of vasculitis. Three of the patients were found to have overlap diseases. In this review, the spectrum of vascular abnormalities in HIES are documented and discussed in detail for the first time. They highlight a previously under-recognized and potentially devastating complication of these disorders. These vascular abnormalities constitute one of the major clinical characteristics in HIES. The presence of hypereosinophilia, vasculitis and defective angiogenesis in HIES may contribute to the formation of vascular abnormalities in HIES. PMID:19912258

Yavuz, H; Chee, R

2010-03-01

371

Asymmetric dimethylarginine levels in patients with cutaneous anthrax: a laboratory analysis  

PubMed Central

Background Asymmetric dimethylarginine (ADMA), the main endogenous inhibitor of nitric oxide synthase, is considered to be associated with endothelial dysfunction. High ADMA levels have been shown to be related with disorders causing vascular inflammation such as hypertension, hypercholesterolemia, atherosclerosis, chronic heart failure, stroke and sepsis. Cutaneous anthrax (CA) is a serious infectious disease which may cause vasculitis. The aim of the study was to investigate the serum ADMA levels in patients with CA. Methods A total of 35 serum samples of the patients with CA and 18 control sera were tested for ADMA levels using ADMA ELISA kit (Immunodiagnostik AG, Bensheim, Germany). Results ADMA levels were found to be significantly higher in the patients group than the controls (p?vasculitis in CA. PMID:24669818

2014-01-01

372

Rituximab for refractory granulomatous eye disease  

PubMed Central

Objective To determine the effectiveness of rituximab therapy for patients with granulomatous disease of the eye. Methods Retrospective review was undertaken of cases seen at a single institution for ocular antineutrophil cytoplasmic antibody-associated vasculitis or sarcoidosis with persistent ocular disease despite systemic therapy. All patients were treated with rituximab and followed for at least 6 months. Results Nine patients were identified (five with antineutrophil cytoplasmic antibody-associated vasculitis, four with sarcoidosis), and all were treated for at least 6 months. Eight experienced improvement of eye disease and were able to reduce prednisone and other drug therapies. One patient remained stable, but still required high dosages of prednisone. All five patients with lung disease improved with rituximab therapy. Rituximab treatment was well tolerated. Two patients discontinued the drug due to leukopenia; however, both patients reinstituted rituximab at modified doses. Conclusion Rituximab therapy was effective in controlling granulomatous ocular disease in most cases. The drug was corticosteroid-sparing and effective in refractory cases, with no severe adverse events encountered. PMID:23055686

Lower, Elyse E; Baughman, Robert P; Kaufman, Adam H

2012-01-01

373

Beh?et's Syndrome and Thrombosis  

PubMed Central

Behçet syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatening panuveitis are the hallmark of the disease. Males are more severely affected than females. Vascular involvement can occur in up to 40% of cases. BS is unique among the vasculitides in that it may involve all sizes and types of vessels. It affects the veins more than the arteries. Lower extremity vein thrombosis is the most frequent manifestation of vascular involvement, followed by vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms. Vascular involvement is frequently associated with constitut onal symptoms and increased acute phase response and is the major cause of increased mortality. A predominantly neutrophilic vasculitis around the vaso vasorum is typical of BS. The thrombus is tightly adherent to the vessel wall which probably explains why thromboembolism is so rare despite the high frequency of venous disease. Thrombophilic factors do not seem to explain thrombotic tendency in BS. Immunosuppressive treatment is essential in suppression and preventing the attacks. PMID:21869912

Seyahi, Emire; Yurdakul, Sebahattin

2011-01-01

374

Rheumatoid arthritis, spondyloarthropathies, and relapsing polychondritis.  

PubMed

The neurologic complications of rheumatic disease are highly variable and their manifestations are linked to the pathogenesis and clinical phenotype of the specific rheumatologic syndrome. In active rheumatoid arthritis (RA), the peripheral nervous system is most commonly involved and mononeuritis multiplex, nerve entrapment and vascultitic sensorimotor neuropathy are not uncommon. Central nervous system complications such as pachymeningitis and cerebral vasculitis are rare. TNF blockade therapy of RA is rarely associated with demyelinating syndromes. In the spondyloarthropathies, especially ankylosing spondylitis (AS), neurologic complications are more frequent in long-standing, advanced disease and include atlantoaxial subluxation, cauda equina syndrome, spinal stenosis, and acute vertebral fractures. Peripheral nervous system involvement in any of the spondyloarthropaties is rare. Relapsing polychondritis (RP) is characterized by recurring bouts of inflammation, destruction of cartilaginous structures, and systemic and rarely central nervous system vasculitis. Visual-oculo and auditory complications are common. Definitive treatment of the neurologic complications and prevention of subsequent ones is dependent upon effective treatment of RA, AS or RP. PMID:24365312

Ostrowski, Rochella A; Takagishi, Troy; Robinson, John

2014-01-01

375

Cavia porcellus as a Model for Experimental Infection by Trypanosoma cruzi  

PubMed Central

The guinea pig (Cavia porcellus) is a natural reservoir for Trypanosoma cruzi but has seldom been used as an experimental infection model. We developed a guinea pig infection model for acute and chronic Chagas disease. Seventy-two guinea pigs were inoculated intradermally with 104 trypomastigotes of T. cruzi strain Y (experimental group); 18 guinea pigs were used as control group. Eight animals from the experimental group and two from the control group were sacrificed 5, 15, 20, 25, 40, 55, 115, 165, and 365 days after inoculation. During the acute phase (15 to 55 days), we observed parasitemia (with a peak on day 20) and positive IgM and IgG Western blots with anti-shed acute-phase antigen bands. The cardiac tissue showed vasculitis, necrosis (on days 40 to 55), moderate to severe inflammation, and abundant amastigote nests. Smaller numbers of amastigote nests were also present in kidney, brain, and other organs. In the early chronic phase (115 to 165 days), parasitemia disappeared and anti–T. cruzi IgG antibodies were still detectable. In cardiac tissue, the number of amastigote nests and the grade of inflammation decreased. In the chronic phase (365 days), the cardiac tissue showed vasculitis and fibrosis; detectable parasite DNA was associated with higher grades of inflammation. The experimental T. cruzi infection model in guinea pigs shows kinetics and pathologic changes similar to those of the human disease. PMID:21703410

Castro-Sesquen, Yagahira E.; Gilman, Robert H.; Yauri, Verónica; Angulo, Noelia; Verastegui, Manuela; Velásquez, Daniel E.; Sterling, Charles R.; Martin, Diana; Bern, Caryn

2011-01-01

376

Histopathological diagnosis of atopic eruption of pregnancy and polymorphic eruption of pregnancy: a study on 41 cases.  

PubMed

: The specific dermatoses of pregnancy represent a recently reclassified heterogeneous group of pruritic inflammatory skin diseases unique to pregnancy that include pemphigoid gestationis, polymorphic eruption of pregnancy (PEP), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy (AEP). Among them, PEP and AEP are the most frequent ones. We performed a histopathological study of a series of PEP and AEP patients (n = 41). Twenty-two patients had PEP that started in the third trimester in 16 (73%) patients and postpartum in 6 (27%) patients. Histopathology revealed a superficial or superficial and deep perivascular dermatitis with eosinophils in all biopsies and signs of a lymphocytic vasculitis in 5 (23%) cases. Epidermal changes, including epidermal hyperplasia, spongiosis, and parakeratosis, occurred in 8 cases, in particular in elder lesions. Nineteen patients had AEP that started earlier [less than third trimester, 14 (74%) patients; third trimester, 5 (26%) patients]. Clinically, 5 (26%) patients showed eczematous lesions, 7 (37%) papular lesions, 3 (16%) presented both eczematous and prurigo lesions, and 4 (21%) experienced exacerbation of preexisting atopic dermatitis. Histopathologically, AEP was characterized by a perivascular lymphohistiocytic infiltrate with frequent eosinophils (74%) and epidermal changes in all but most of P-type biopsies. No definitive differential histopathological criteria between PEP and AEP were found. Only lymphocytic vasculitis with a mixed infiltrate with eosinophils was more frequent in PEP patients. Timing of onset, morphology of skin lesions, and a detailed clinicopathologic correlation are essential for diagnosis. PMID:25243396

Massone, Cesare; Cerroni, Lorenzo; Heidrun, Neureiter; Brunasso, Alexandra M G; Nunzi, Enrico; Gulia, Andrea; Ambros-Rudolph, Christina M

2014-10-01

377

Dermatopathology: LC07-1 PANNICULITIS.  

PubMed

The subcutaneous fat is a metabolic depot and also provides insulation from trauma. The subcutis is divided into lobules by fibrous septa. Within the fibrous septae course the small arteries, arterioles, veins and small nerve fibres. The nutrient artery supplies the centre of the lobule and drains to venules within the septum. Inflammatory disorders of the subcutaneous fat can be grouped into three main categories: 1) Septal panniculitis. 2) Lobular panniculitis. 3) Panniculitis associated with large vessel vasculitis.Most panniculitis are strictly speaking mixed but the classification is based on the predominant area of involvement.In septal panniculitis the inflammation is centred upon the connective tissue septa. There is often some spill over of the inflammatory cells into the periphery of the lobule. Erythema nodosum (EN) is the prototype of septal panniculitis. Other disorders associated with a septal panniculitis include necrobiosis lipoidica, factitial panniculitis, nephrogenic systemic fibrosis, cellulitis and microscopic polyangiitis.In the lobular panniculitides the inflammation is centered mainly within the lobule. Examples of lobular panniculitis include nodular vasculitis, lupus panniculitis, subcutaneous fat necrosis of the newborn, sclerema neonatorum, cold panniculitis, a1 anti-trypsin deficiency and pancreatic panniculitis. PMID:25188048

Calonje, Eduardo

2014-10-01

378

Clinical aspects of posterior uveitis in ocular sarcoidosis.  

PubMed

Two clinical forms of the "white spot" syndrome in patients with posterior uveitis in definitive and presumable ocular sarcoidosis were analyzed. Group 1 was characterized by periphlebitis and discrete white spots around the vein of the retina, so-called "candle-wax", whereas group 2 showed yellow-orange solitary nodules located at the choroid, i.e. multifocal choroiditis. Visual acuity and the severity of clinical presentation were assessed in both groups. Visual acuity, Snellen equivalent was 0.52 +/- 0.36 in group 1 and 0.82 +/- 0.39 in group 2 with lesions at the level of choroid. One-way analysis of variance ANOVA showed a statistically significant between-group difference in visual acuity (p = 0.03). The mean severity of clinical presentation was 11.80 +/- 2.04 points in group 1 and 5.80 +/- 4.18 points in group 2. T-test for independent samples yielded a statistically significant difference between the groups (p = 0.02). A statistically significant difference in visual acuity was the result of vasculitis in the group with the "candle-wax" phenomenon, which is associated with retinal vasculitis and causes cystoid macular edema and reduction of visual acuity. Complications such as cataract, glaucoma and neovascularization, which also decrease visual acuity, were more frequent in group 1. PMID:23115950

Jovanovi?, Svetlana V; Jovanovi?, Zorica D; Radoti?, Filip M; Sre?kovi?, Suncica B; Paunovi?, Svetlana S; Stojanovi?, Jasmina D

2012-06-01

379

Churg-Strauss syndrome and persistent heart failure: active disease or damage?  

PubMed

Churg-Strauss syndrome (CSS) is a rare small-vessel vasculitis typically associated with adult-onset asthma, peripheral and tissue hypereosinophilia, migratory pulmonary infiltrates, upper respiratory tract symptoms, and clinical evidence of systemic vasculitis. Cardiac involvement is a well-recognized complication with an estimated prevalence of 60%. Heart disease is associated with poor prognosis, accounting for almost 50% mortality in CSS. We present a case of a 48-year-old woman with CSS complicated by congestive heart failure with left ventricular ejection fraction of 25%, who was initially treated with long course of high-dose steroids without any clinical or echocardiographic improvement. She was referred to our hospital 1 year later and was initiated with cyclophosphamide 2 mg/kg per day and prednisone 60 mg/d followed by slow taper. Subsequently, the patient had remarkable improvement. Patient was then transitioned to azathioprine for 1.5 years with sustained disease remission. It may be difficult to determine myocardial disease activity status versus tissue damage in CSS with prolonged duration of heart failure symptoms. This is the first case report demonstrating that CSS cardiac disease may remain active despite 1 year of corticosteroid therapy, and significant improvement or remission can still be achieved by administering more aggressive cytotoxic immunosuppressive therapy. PMID:24048112

Lin, Yih Chang; Oliveira, Guilherme H M; Villa-Forte, Alexandra

2013-10-01

380

Severe congestive heart failure as the main symptom of eosinophilic granulomatosis and polyangiitis (Churg-Strauss syndrome).  

PubMed

Patients with cardiovascular symptoms are mainly diagnosed in cardiological wards. However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3. High eosinophilia is connected with production of cytotoxic eosinophilic proteins which can cause eosinophilic vasculitis or eosinophilic myocarditis. One of the better known hypereosinophilic syndromes is EGPA described by the pathomorphologists Churg and Strauss. The further research works allowed for the clinical characteristics of patients with EGPA. In the course of this disease the following three phases were recognized : prodromal-allergic, eosinophilic, vasculitic. The definitive diagnosis can be established only in the third phase, when vasculitis causes organ involvement. Besides symptoms of the respiratory tract (asthma, nasal polyps, eosinophilic lung infiltrations) also cardiovascular symptoms, gastrointestinal tract symptoms, as well as skin lesions and kidneys involvement can appear. The most dangerous for patients is involvement of the nervous and cardiovascular systems. We present a patient with asthma and eosinophilia in whom EGPA was diagnosed in the course of acute recurrent substernal chest pain, with subsequent signs of cardiac insufficiency. PMID:25339570

Za??ska, Jolanta; Wiatr, El?bieta; Zych, Jacek; Szopi?ski, Janusz; Oniszh, Karina; Kober, Jaros?aw; Piotrowska-Kownacka, Dorota; Roszkowski-?li?, Kazimierz

2014-01-01

381

Polyarteritis Nodosa Presenting with Bilateral Testicular Swelling and Complicated by Unilateral Facial Nerve Palsy  

PubMed Central

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that is generally restricted to medium-sized vessels. Here we describe the first case of a patient in which a bilateral testicular mass was a presenting symptom and the diagnosis was made on the basis of testicular histopathology. A 53-year-old Asian man presented with a history of constitutional symptoms and testicular swelling. Scrotal ultrasound revealed two avascular, bilateral, intratesticular lesions. The bilateral testicular abscess was treated without improvement. The patient developed left seventh cranial nerve palsy during his admission. The clinical changes made vasculitis or a related disorder more likely and the patient underwent a right testicular biopsy. Histopathology demonstrated features of transmural inflammation and fibrinoid necrosis of medium-sized vessel walls, consistent with PAN. This case illustrates the difficulty in diagnosing polyarteritis nodosa with isolated bilateral testicular swelling and the delay in the diagnosis. After 9 months of follow-up, no relapse had occurred and the patient's testosterone level was on the lower side of normal. PMID:21687399

Richards, Bethan; Rasiah, Kris; Thanigasalam, Ruban; Atmore, Bryn B; Laurent, Roger

2011-01-01

382

Advances and challenges in the diagnosis and treatment of polymyalgia rheumatica  

PubMed Central

Polymyalgia rheumatica (PMR) is a common inflammatory condition that often affects people over the age of 50 years. Characteristic symptoms are shoulder and hip girdle pain and prolonged morning stiffness. Markers of inflammation are often elevated. Clinicians are often faced with the challenge of distinguishing PMR from other conditions, particularly rheumatoid arthritis and spondyloarthropathy that can mimic symptoms of PMR in older people. Additionally, there is an association between PMR and giant cell arteritis, a common large-vessel vasculitis which also affects people over the age of 50 years. Imaging of the large vessels in asymptomatic patients with PMR often reveals findings of subclinical vasculitis. Presently, there are no tests that are specific for the diagnosis of PMR and clinicians rely on a combination of history, physical examination, laboratory tests and imaging studies to make a diagnosis. A recent undertaking by the European League Against Rheumatism/American College of Rheumatology has led to the publication of provisional classification criteria of PMR. Ultrasonography, which is being increasingly used by rheumatologists, can greatly aid in the diagnosis of PMR and often shows changes of synovitis and tenosynovitis. Treatment consists of low doses of glucocorticoids which are associated with morbidity. Evaluation of newer biologic therapies targeting inflammatory cytokines is underway. Despite treatment, relapses are common. PMID:24489611

Warrington, Kenneth J.

2014-01-01

383

Rheumatologic manifestations of pediatric HIV infection.  

PubMed

In order to assess the frequency of rheumatologic manifestations at different stages of pediatric human immunodeficiency virus (HIV) infection, 26 HIV-infected children at any stage of infection, seen at the Children's AIDS Clinic of "La Raza" National Medical Center from January 1997 to December 1998, were studied. Rheumatologic manifestations were assessed following the criteria established by the American College of Rheumatology. Blood samples were taken for measuring CD4+ and CD8+ T cells, antinuclear antibodies (ANA), anticardiolipin (ACL) antibodies, and rheumatoid factor (RF). The results were compared to those of 25 HIV-negative children of similar ages. Rheumatologic manifestations were identified in 5 (19.2%) of 26 children. Two of whom were twin sisters with biphasic Raynaud's syndrome, and one had necrosing vasculitis of a finger, as well as lip necrosis and livedo reticularis. These patients were positive for ANA and ACL. One case each of knee arthalgias, vasculitis, and septic arthritis of the ankle were also seen. All of the rheumatologic manifestations were in advanced stages of HIV disease. These rheumatologic changes are similar to those reported for HIV-positive adults, and should be considered as part of the HIV acquired immune deficiency syndrome (AIDS) clinical spectrum in the pediatric population. PMID:11689139

Martínez-Rojano, H; Juárez Hernández, E; Ladrón De Guevara, G; del Carmen Gorbea-Robles, M

2001-10-01

384

A suspected case of coronary periarteritis due to IgG4-related disease as a cause of ischemic heart disease.  

PubMed

IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by marked infiltration of IgG4-positive (+) plasma cells into affected organs, but the concept of this disease has only recently been established. Coronary vasculitis is a rare disease that can cause sudden death, and it has recently been reported that IgG4-RD may be associated with vasculitis, including periarteritis and coronary disease. In this paper we report an autopsy case of sudden death of a man in his thirties, in which coronary periarteritis with features of IgG4-related periarteritis was detected. IgG4-RD was suspected from the presence of the following histopathological features: (1) markedly thickened adventitia and marked infiltration of the adventitia and periarterial fat by lymphocytes and plasma cells; and (2) infiltration of IgG4-positive plasma cells (ratio of IgG4+ cells to IgG4+ cells of >40 %, 50 IgG4+ plasma cells per high-power field) on immunostaining. The etiology and pathophysiology of IgG4-RD and IgG4-related periarteritis are still unclear, and further investigation of these conditions and their association with coronary lesions is needed. Careful consideration should be given to the possible presence of IgG4-RD when forensic pathologists encounter cases of sudden death accompanied by coronary periarteritis. PMID:24337948

Inokuchi, Go; Hayakawa, Mutsumi; Kishimoto, Takashi; Makino, Yohsuke; Iwase, Hirotaro

2014-03-01

385

Adverse immunologic effects of antithyroid drugs.  

PubMed Central

Propylthiouracil and methimazole are frequently used in the management of hyperthyroidism. Two patients in whom adverse immunologic effects other than isolated agranulocytosis developed during treatment with propylthiouracil are described. A review of the literature revealed 53 similar cases over a 35-year period. Rash, fever, arthralgias and granulocytopenia were the most common manifestations. Vasculitis, particularly with cutaneous manifestations, occurs and may be fatal. The clinical evidence suggests that an immunologic mechanism is involved. A number of different autoantibodies were reported, but antinuclear antibodies were infrequent, and none of the cases met the criteria for a diagnosis of systemic lupus erythematosus. Thus, the reactions do not represent a true drug-induced lupus syndrome. Current hypotheses and experimental data regarding the cause of the reactions are reviewed. No specific clinical subgroup at high risk can be identified, and manifestations may occur at any dosage and at any time during therapy. Cross-reactivity between the two antithyroid drugs can be expected. Except for minor symptoms (e.g., mild arthralgias or transient rash), such reactions are an indication for withdrawal of the drug and the use of alternative methods to control the hyperthyroidism. In rare cases of severe vasculitis a short course of high-dose glucocorticoid therapy may be helpful. PMID:3539299

Wing, S S; Fantus, I G

1987-01-01

386

[A case of miliary tuberculosis showing diffuse alveolar hemorrhage].  

PubMed

A 43-year-old diabetic man had a productive cough and high fever and was admitted to another hospital. His condition did not improve despite treatment with Cefepime, and he was transferred to our hospital. Chest X-ray films and CT findings showed pulmonary infiltration and diffuse ground-glass opacities in bilateral lung fields, but disseminated nodules were not identified. Since his bronchial lavage fluid (BALF) was bloody, we suspected diffuse alveolar hemorrhage due to vasculitis. Steroid pulse therapy was given, and his fever and chest X-ray findings completely improved. However, 1 week later, he again suffered a high fever and bloody sputum, and a chest X-ray film showed granular shadows in bilateral lung fields. He died of respiratory failure on the 18th hospital day despite treatment and mechanical ventilation. An autopsy revealed many necrotizing epithelioid granulomas in both lungs, the liver, the spleen, both kidneys and both adrenal glands. These findings indicated miliary tuberculosis, and a culture of his sputum and BALF finally revealed mycobacterium tuberculosis. Marked alveolar hemorrhage and a hyaline membrane were also found in both lungs, but vasculitis was not recognized in any organ. We report this case, because to the best of our knowledge diffuse alveolar hemorrhage has not been reported as the primary symptom of miliary tuberculosis. PMID:21842695

Nakamura, Sukeyuki; Kamioka, Eiko; Tokuda, Atsuko; Tabeta, Hiroshi

2011-07-01

387

Cutaneous manifestations of Churg-Strauss syndrome: report of two cases and review of the literature.  

PubMed

Churg-Strauss syndrome (CSS) is a small-vessel vasculitis characterized by severe asthma, lung/tissues infiltrates, extravascular necrotizing granulomas, and eosinophilia. Cutaneous involvement is common but may not be highly suggestive. Two typical cases of CSS with cutaneous involvement are herein reported, both females, 37 and 54 years old, presenting with lower limbs palpable purpura and urticarial lesions of the neck, respectively. A comprehensive review of the literature showed that cutaneous manifestations occurred in 40-81% of CSS patients and were the presenting sign in 14% of the patients. Moreover, a total of 68 cases of CSS with a detailed description of the cutaneous lesions have been published. In the majority of these patients, skin lesions allowed for the histopathological diagnosis of CSS. The most common clinical features were papulo-nodules with the histological picture of extravascular Churg-Strauss granuloma followed by purpuric and/or necrotic lesions in the lower limbs corresponding to small-vessel vasculitis with eosinophils. Less common lesions included urticarial lesions and livedo reticularis. Therefore, a high index of suspicion on skin lesions and the proper lesion selection for histological examination may be very important for early diagnosis of CSS. Clinical-pathological correlation is essential, as both clinical and histological features are not pathognomonic. PMID:20949297

Bosco, Laura; Peroni, Anna; Schena, Donatella; Colato, Chiara; Girolomoni, Giampiero

2011-04-01

388

Immune complex associated complications in the subacute phase of meningococcal disease: incidence and literature review  

PubMed Central

Aim: To determine the incidence of immune complex associated complications (IAC) after severe meningococcal disease (SMD) in a group of Dutch children admitted to a paediatric intensive care unit (PICU). Methods: Retrospective chart analysis and follow up of 130 survivors of SMD admitted to PICU. Signs of IAC, inflammatory parameters, and temperature profile were reviewed. Results: Of 130 children with SMD, 20 (15.3%) showed one or more of the three manifestations of IAC: 18 (13.8%) developed arthritis (effusion, with or without erythema/arthralgia), 11 (8.4%) vasculitis, and five (3.8%) pleuritis. Eighteen of 20 (90%) patients with IAC had a secondary rise in temperature; in patients with no IAC this was 48 of 110 (43.6%). IAC was associated with leucocytosis in 82.3% versus 47.7% in patients without IAC, and with increased CRP in 86.6% versus 47.2% in patients without IAC. Leucocytes on admission were significantly lower in patients who would later develop IAC (mean 8.6 versus 13.8x109/l). Conclusion: IAC is a common complication of SMD, mainly occurring 4–10 days after systemic disease. IAC presents clinically as arthritis or vasculitis, mostly accompanied by secondary fever and raised inflammatory parameters. PMID:14500317

Goedvolk, C; von Rosenstiel, I A; Bos, A

2003-01-01

389

[Ultrasound diagnostic techniques in dermatologic angiology and phlebology].  

PubMed

The diagnostic uses of high-resolution ultrasonography (7.5-20 MHZ) have dramatically advanced in the past 20 years. We present representative examples of ultrasound diagnosis, based on our experience with over 15,000 examinations. For example, peripheral arterial disease was identified in 30% of older patients with venous diseases, while peripheral emboli and vasculitis can lead to widespread skin disease. Ultrasonographic diagnosis is required for appropriate interdisciplinary treatment. In addition, regular evaluation of the supra-aortic vessels and coronary arteries as well as arteries and veins of the limbs is needed in some forms of vasculitis. The causes of peripheral venous disease can be subdivided into four groups based on sonographic findings: Primary or secondary epi- and/or subfascial venous insufficiency, thrombotic venous occlusion, venous compression syndrome and arteriovenous-lymphatic malformations. The diagnosis and therapeutic approach to lymphedema and soft tissue diseases, with or without lymphadenopathy, requires careful sonographic evaluation. Ultrasonography is an essential part of dermatologic oncology, both for planning primary therapy and for follow-up. A sound command of ultrasonography is today an essential aspect of dermatologic diagnosis and therapy. PMID:12835863

Hesse, G; Stiegler, H

2003-07-01

390

FDG PET of infection and inflammation.  

PubMed

Nuclear medicine plays an important role in the evaluation of infection and inflammation. Fluorine 18 fluorodeoxyglucose (FDG) is a readily available radiotracer that offers rapid, exquisitely sensitive high-resolution tomography. In patients with acquired immunodeficiency syndrome, FDG positron emission tomography (PET) accurately helps localize foci of infection and is particularly useful for differentiating central nervous system lymphoma from toxoplasmosis. FDG PET can also help localize the source of fever of undetermined origin (FUO), thereby guiding additional testing. In the musculoskeletal system, FDG PET accurately helps diagnose spinal osteomyelitis, and in inflammatory conditions such as sarcoidosis and vasculitis, it appears to be useful for defining the extent of disease and monitoring response to treatment. FDG PET may be of limited usefulness in postoperative patients and in patients with a failed joint prosthesis or a tumor. Nevertheless, this relatively new imaging technique promises to be helpful in the diagnosis of infection and inflammation. FDG PET will likely assume increasing importance in assessing FUO, spinal osteomyelitis, vasculitis, and sarcoidosis and may even become the radionuclide imaging procedure of choice in the evaluation of some or all of these pathologic conditions. PMID:16160116

Love, Charito; Tomas, Maria B; Tronco, Gene G; Palestro, Christopher J

2005-01-01

391

Avian simuliotoxicosis: outbreak in Louisiana.  

PubMed

From April 20 to May 10, 2010, multiple species of birds were seen at the Louisiana State University (LSU) Zoological Medicine Department for a disease syndrome characterized by acute lethargy, generalized subcutaneous petechiae, vasculitis, and death caused by a black fly identified as Simulium meridionale. Twenty psittacine birds presented with severe depression and multifocal subcutaneous hemorrhages over the body and especially noted in the featherless areas of the head and neck. Ten out of 20 clinical cases seen survived on a treatment regimen consisting of intravenous fluid therapy, anti-inflammatories, antihistaminic medications, and supportive care. The 10 other birds likely died of cardiopulmonary collapse and anaphylactoid reactions. In all post mortem examinations multifocal to coalescing dermal hemorrhage, eosinophilic dermatitis, severe edema, and vasculitis were observed. The LSU School of Animal Science conducted a brief survey that included 34 of the 64 Louisiana parishes and showed that 17 parishes, at least, were affected by this outbreak. A total of at least 225 poultry bird deaths could be attributed to black fly strikes. Simuliotoxicosis outbreaks have previously been reported in North America, and this report documents the clinical and post mortem findings, treatment response, and extent of such an outbreak in pet and farm birds in Louisiana. PMID:23050485

Schnellbacher, Rodney W; Holder, Kali; Morgan, Tim; Foil, Lane; Beaufrère, Hugues; Nevarez, Javier; Tully, Thomas N

2012-09-01

392

Feline infectious peritonitis in a mountain lion (Puma concolor), California, USA.  

PubMed

Feline infectious peritonitis (FIP) is a fatal immune-mediated vasculitis of felids caused by a mutant form of a common feline enteric virus, feline enteric coronavirus. The virus can attack many organ systems and causes a broad range of signs, commonly including weight loss and fever. Regardless of presentation, FIP is ultimately fatal and often presents a diagnostic challenge. In May 2010, a malnourished young adult male mountain lion (Puma concolor) from Kern County, California, USA was euthanized because of concern for public safety, and a postmortem examination was performed. Gross necropsy and histopathologic examination revealed necrotizing, multifocal myocarditis; necrotizing, neutrophilic, and histiocytic myositis and vasculitis of the tunica muscularis layer of the small and large intestines; and embolic, multifocal, interstitial pneumonia. Feline coronavirus antigen was detected in both the heart and intestinal tissue by immunohistochemistry. A PCR for coronavirus performed on kidney tissue was positive, confirming a diagnosis of FIP. Although coronavirus infection has been documented in mountain lions by serology, this is the first confirmed report of FIP. PMID:23568918

Stephenson, Nicole; Swift, Pamela; Moeller, Robert B; Worth, S Joy; Foley, Janet

2013-04-01

393

Proapoptotic Bak and Bax guard against fatal systemic and organ-specific autoimmune disease  

PubMed Central

Dysregulation of the “intrinsic” apoptotic pathway is associated with the development of cancer and autoimmune disease. Bak and Bax are two proapoptotic members of the Bcl-2 protein family with overlapping, essential roles in the intrinsic apoptotic pathway. Their activity is critical for the control of cell survival during lymphocyte development and homeostasis, best demonstrated by defects in thymic T-cell differentiation and peripheral lymphoid homeostasis caused by their combined loss. Because most bak?/?bax?/? mice die perinatally, the roles of Bax and Bak in immunological tolerance and prevention of autoimmune disease remain unclear. We show that mice reconstituted with a Bak/Bax doubly deficient hematopoietic compartment develop a fatal systemic lupus erythematosus-like autoimmune disease characterized by hypergammaglobulinemia, autoantibodies, lymphadenopathy, glomerulonephritis, and vasculitis. Importantly, these mice also develop a multiorgan autoimmune disease with autoantibodies against most solid glandular structures and evidence of glandular atrophy and necrotizing vasculitis. Interestingly, similar albeit less severe pathology was observed in mice containing a hematopoietic compartment deficient for only Bak, a phenotype reminiscent of the disease seen in patients with point mutations in BAK. These studies demonstrate a critical role for Bak and an ancillary role for Bax in safeguarding immunological tolerance and prevention of autoimmune disease. This suggests that direct activators of the intrinsic apoptotic pathway, such as BH3 mimetics, may be useful for treatment of diverse autoimmune diseases. PMID:23349374

Mason, Kylie D.; Lin, Ann; Robb, Lorraine; Josefsson, Emma C.; Henley, Katya J.; Gray, Daniel H. D.; Kile, Benjamin T.; Roberts, Andrew W.; Strasser, Andreas; Huang, David C. S.; Waring, Paul; O'Reilly, Lorraine A.

2013-01-01

394

[Isolated neuritis of the oculomotor nerve in infectious mononucleosis].  

PubMed

A 19-year-old immune-competent patient developed right-sided headache and, subsequently, subacute diplopia. On clinical examination he had incomplete right oculomotor palsy. Cranial MRI showed pathologic contrast enhancement of the right oculomotor nerve at its exit point from the mesencephalon, and the CSF displayed slight pleocytosis. The following relevant differential diagnoses were not supported by additional examinations: neurosarcoidosis, Lyme neuroborreliosis, neurosyphilis, tuberculous meningitis, viral meningitis (HIV, VZV, CMV), CNS lymphoma, vasculitis associated with rheumatic disease, Tolosa-Hunt syndrome, and diabetic neuropathy. However, on the basis of blood lymphocytosis, positive heterophile antibody test (Paul-Bunnell test), the presence of IgM antibodies against Epstein-Barr virus capsid antigen, and elevated transaminases, infectious mononucleosis was diagnosed. Isolated neuritis of the oculomotor nerve is a rare parainfectious manifestation of infectious mononucleosis. PMID:18058080

Erben, Y; Gonzalez Hofmann, C; Steinmetz, H; Ziemann, U

2008-04-01

395

Indian tick typhus presenting as Purpura fulminans.  

PubMed

Seriously ill patients presenting with purpura fulminans, sepsis and multi-organ failure often require extensive diagnostic workup for proper diagnosis and management. Host of common infections prevalent in the tropics, e.g. malaria, dengue; other septicemic infections e.g. meningococcemia, typhoid, leptospirosis, toxic shock syndrome, scarlet fever, viral exanthems like measles, infectious mononucleosis, collagen vascular diseases (Kawasaki disease, other vasculitis) diseases, and adverse drug reactions are often kept in mind, and the index of suspicion for rickettsial illness is quite low. We present a case of Indian tick typhus presenting with purpura fulminans (retiform purpura all over the body), sepsis and multiorgan failure without lymphadenopathy and eschar, successfully treated with doxycycline and discharged home. Hence, a high index clinical suspicion and prompt administration of a simple therapy has led to successful recovery of the patient. PMID:25097365

Tirumala, Suhasini; Behera, Bijayini; Jawalkar, Srikanth; Mishra, Pradeep Kumar; Patalay, Pavithra Vani; Ayyagari, Sudha; Nimmala, Pavani

2014-07-01

396

Ataxia.  

PubMed

The approach to the child with ataxia requires a detailed history and careful general and neurological examination as well as selected blood work and brain imaging and increasingly available genetic testing for inherited ataxias that usually have an episodic or progressive presentation. The differential of acute and recurring ataxia covered in this chapter includes intoxication (e.g., antiepileptics, lead, alcohol), postinfectious cerebellitis, hemorrhage, ischemic stroke, tumor (posterior fossa or cerebellum), brainstem encephalitis, occult neuroblastoma, Miller Fisher syndrome, conversion reaction, multiple sclerosis, epileptic pseudoataxia, vasculitis (e.g., Kawasaki), metabolic etiologies (e.g., maple syrup urine disease, pyruvate dehydrogenase deficiency, ornithine transcarbamylase deficiency, biotinidase deficiency, Hartnup disease, and argininosuccinic aciduria), migraine, migraine equivalents (benign paroxysmal positional vertigo), autosomal dominant episodic ataxias (with seven types currently identified), and hypothyroidism. Cooperation with therapists and providers from other specialties including ophthalmology and genetics and metabolism is essential to caring for these children and their families. PMID:23622331

Winchester, Sara; Singh, Piyush K; Mikati, Mohamad A

2013-01-01

397

Severe Eosinophilic Syndrome Associated with the Use of Probiotic Supplements: A New Entity?  

PubMed Central

Severe eosinophilic syndromes related to the administration or use of unsuspected immunogenic substances have been described previously. Many of these diseases presented initially as clusters or isolated cases. The spanish toxic oil syndrome, the eosinophilia myalgia syndrome, and nephrogenic systemic fibrosis are examples of such diseases. We describe 2 cases of a severe eosinophilic syndrome characterized by marked peripheral blood eosinophilia (>15,000?cells/ml), mononeuritis multiplex, and necrotizing vasculitis which developed in a close temporal association with the recent onset use of nonprescription probiotics. There was no history of a prior autoimmune disease. Although both cases had prompt response to immunosuppression with rapid resolution of peripheral blood eosinophilia and accompanying constitutional symptoms, they remained with permanent neurological deficits. PMID:23259129

Mendoza, Fabian A.; Purohit, Shivani; Kenyon, Lawrence; Jimenez, Sergio A.

2012-01-01

398

Chronic urticaria.  

PubMed Central

Urticaria affects 15% to 20% of the population once or more during a lifetime. Chronic urticaria is a frequent recurrent eruption over a period greater than 6 weeks; the cause remains a mystery in more than 75% of cases. Urticaria and angioedema may be produced by immunologic or nonimmunologic means. Urticarial vasculitis, contact urticaria, mastocytosis, physical urticarias, dermatographism, cholinergic urticaria, localized heat urticaria, cold urticaria, aquagenic urticaria, and vibratory angioedema all require specific evaluation and treatment. Chronic idiopathic urticaria is usually controlled by antihistamines; depending on the circadian rhythm of the eruption, sedative or nonsedative antihistamines are prescribed. Some patients will require a combination of H1 and H2 antagonists, or even parenteral corticosteroids. PMID:1970697

Burrall, B. A.; Halpern, G. M.; Huntley, A. C.

1990-01-01

399

Recurrent lip swelling as a late presentation of Kawasaki disease: Case report and review of literature?  

PubMed Central

Kawasaki Disease (KD) is an acute systemic vasculitis of unknown etiology. In many developed countries, KD has replaced rheumatic heart disease as the leading cause of acquired heart disease in children. Among the classical criteria for a diagnosis of KD are oral manifestations such as strawberry tongue, erythematous cracked lip, and oropharyngeal mucositis. We report the case of a 24-year-old Saudi female with a history of Kawasaki disease who presented to our oral medicine clinic with recurrent painless swelling of the upper lip. As lip swelling has not previously been reported as an oral manifestation of KD, this case represents a novel presentation of recurrent Kawasaki disease in an adult female. PMID:23960555

Faden, Asmaa

2012-01-01

400

Thiabendazole-induced acute liver failure requiring transplantation and subsequent diagnosis of polyarteritis nodosa.  

PubMed

Polyarteritis nodosa (PAN), a systemic necrotising vasculitis that affects medium- and small-sized arteries, has visceral involvement in 40-60% of the patients. According to the Five-Factor Score (FFS), it is associated with poor outcome. We describe a patient who underwent orthotopic liver transplantation (OLT) for severe ductopenia induced by thiabendazole that was empirically prescribed for chronic hypereosinophilia. Eleven years later, despite immunosuppressive treatment to prevent graft rejection, he developed mononeuritis multiplex; PAN was diagnosed. He also had severe recurrent ischaemic cholangitides because of post-OLT hepatic artery ligation to treat a postoperative severe haematemesis. His outcome was favourable after second OLT, under steroids, cyclophosphamide pulses and tacrolimus. In retrospect, his initial symptoms and hypereosinophilia were probably attributable to PAN. PMID:22640653

Groh, Matthieu; Blanche, Philippe; Calmus, Yvon; Guillevin, Loïc

2012-01-01

401

Contracturing granulomatous myositis: a separate entity.  

PubMed Central

Granulomatous muscle disease is most commonly seen in sarcoidosis, but may be seen in association with a wide variety of other disorders or in isolation. Patients with granulomatous myositis usually present with slowly progressive muscle pain and weakness affecting mainly proximal muscles. There are, however, a few reports of granulomatous muscle disease presenting with flexion contractures of the limbs. Two further patients with granulomatous muscle disease and flexion contractures of the limbs, but with no evidence of systemic granulomatous disease, is presented. It is suggested that such patients represent a separate clinical entity that is distinct from idiopathic granulomatous myositis presenting with muscle pain and weakness. The association of contracturing granulomatous myositis with a long-standing vasculitis in one patient suggests that the two conditions may be related. Images PMID:2283532

Simmonds, N J; Hoffbrand, B I

1990-01-01

402

Multi-system mitochondrial disorder with recurrent steroid-responsive eosinophilia.  

PubMed

Though mitochondrial disorders due to a respiratory chain defect may manifest with haematological abnormalities such as anaemia, neutropenia, or thrombocytopenia, recurrent steroid-responsive eosinophilia has not been reported as a manifestation of a mitochondrial disorder. In a polymorbid 65-year old female recurrent erythema, asthma, chronic bronchitis, gastritis with eosinophilic granuloma, recurrent episodes of hypereosinophilia, and polyarthralgia suggested Churg–Strauss syndrome, which was excluded upon absence of eosinophilic vasculitis on any of the biopsies. However, corticosteroids were effective for hypereosinophilia. In addition to the hypereosinophilic syndrome a mitochondrial disorder with multi-system affection was diagnosed upon the clinical presentation and a muscle biopsy indicative of a mitochondrial disorder. Hypereosinophilia was interpreted as a manifestation of the mitochondrial disorder after exclusion of various differentials. Mitochondrial disease may go along with marked eosinophilia mimicking Churg–Strauss syndrome. Steroids may be useful to resolve episodic eosinophilia but may be ineffective for other features of mitochondrial disorders. PMID:19370352

Finsterer, Josef; Höger, Franz

2009-11-01

403

Churg-Strauss syndrome in a patient previously diagnosed with multiple sclerosis  

PubMed Central

A lady in her 70s with a background of multiple sclerosis (MS) and late-onset asthma was admitted with a 2-week history of cough and shortness of breath, progressive right-sided weakness and functional decline. Investigation revealed eosinophilia, elevated myeloperoxidase antineutrophil cytoplasmic antibody, CT sinuses showed long-standing inflammatory changes consistent with sinonasal polyposis and MRI head showed lesions consistent with vasculitis. She then developed left-sided weakness and increased wheeze. Review of her case notes demonstrated that, the eosinophilia was long-standing, her asthma was severe and steroid-dependent, and her neurologic syndrome was atypical for MS. Intravenous methylprednisolone then cyclophosphamide were administered. She demonstrated remarkable improvement, becoming more alert, with improvement in left-sided weakness. A diagnosis of Churg–Strauss syndrome was established. She was discharged to a nursing home with outpatient rheumatology follow-up. The diagnosis of MS was revisited. PMID:22679315

Sarkar, Pamela; Ibitoye, Richard Tolulope; Promnitz, Douglas Anthony

2011-01-01

404

Cellular Targeting in Autoimmunity  

PubMed Central

Many biologic agents that were first approved for the treatment of malignancies are now being actively investigated and used in a variety of autoimmune diseases such as rheumatoid arthritis (RA), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic lupus erythematosus (SLE), and Sjogren’s syndrome. The relatively recent advance of selective immune targeting has significantly changed the management of autoimmune disorders, and in part, can be attributed to the progress made in understanding effector cell function and their signaling pathways. In this review, we will discuss the recent FDA approved biologic therapies that directly target immune cells as well as the most promising investigational drugs affecting immune cell function and signaling for the treatment of autoimmune disease. PMID:23054625

Rogers, Jennifer L.; Serafin, Donald S.; Timoshchenko, Roman G.; Tarrant, Teresa K.

2012-01-01

405

Fatal cytauxzoonosis in a free-ranging bobcat (Lynx rufus).  

PubMed

In September 2000, a free-ranging bobcat (Lynx rufus) cub was presented to the Kansas State University Veterinary Teaching Hospital (Manhattan, Kansas, USA) in a moribund state with signs of severe anemia and respiratory difficulty. The cub was euthanized. Gross necropsy findings included multifocal atelectasis, splenomegaly, and pericardial effusion. Microscopic examination revealed subacute pulmonary thrombosis, mild vasculitis in the brain, and large schizont-filled macrophages within blood vessels of all tissues examined. The organisms were typical of the developmental stages of Cytauxzoon felis. Cytauxzoonosis is considered to be a persistent, subclinical infection in the bobcat; however, this cub had lesions consistent with those seen in fatal infections in domestic cats. This case of fatal C. felis infection indicates that some free-ranging bobcats may die of cytauxzoonosis. PMID:12238380

Nietfeld, Jerome C; Pollock, Christal

2002-07-01

406

Pathology Case Study: Bilateral Pneumonia  

NSDL National Science Digital Library

This is a case study presented by the University of Pittsburgh Department of Pathology which describes a 75 year old woman "with a past medical history of significant for a 6 month history of cryoglobulinemia with vasculitis, congestive heart failure, hypertension, cryptogenic cirrhosis, peripheral vascular disease, chronic renal insufficiency, anemia, degenerative joint disease, and diverticulitis." Visitors are provided with patient history, admission data, and microscopic findings (lung), including images, and are given the opportunity to diagnose the patient. A "Final Diagnosis" section provides a discussion of the findings as well as references. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose disease. It is also a helpful site for educators to use to introduce or test student learning in clinical microbiology.

Davie, James; Graur, Octavia; Sheaffer, John

2008-12-12

407

Epidemiology of Behçet disease.  

PubMed

Behçet disease (BD) is a multisystem inflammatory disorder that is an important cause of morbidity worldwide. BD is most common along the ancient "Silk Road" route in the Far East and Mediterranean basin. The eye is the most commonly involved organ in BD patients.The prototypical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Less commonly, BD may present in the form of conjunctivitis, conjunctival ulcers, keratitis, episcleritis, scleritis, and extraocular muscle paralysis. Uveitis in BD carries significant implications for the patient, because it is a chronic recurrent disease characterized by explosive attacks of severe inflammation that may cause significant, cumulative damage to the intraocular structures. This review summarizes the epidemiology of systemic and ocular clinical features of BD with particular focus on risk factors, clinical characteristics, complications, and prognosis of BD-associated uveitis. PMID:23030353

Khairallah, Moncef; Accorinti, Massimo; Muccioli, Cristina; Kahloun, Rim; Kempen, John H

2012-10-01

408

Skin gangrene as an extraintestinal manifestation of inflammatory bowel disease*  

PubMed Central

Inflammatory bowel diseases can commonly present many cutaneous lesions which can contribute to the diagnosis of the disease or its activity. The most frequent cutaneous or mucocutaneous manifestations suggesting ulcerative rectocolitis activity are erythema nodosum (3-10%), pyoderma gangrenosum (5-12%) and aphthous stomatitis (4%). Other reactive skin manifestations related to immunological mechanisms associated with the inflammatory bowel disease are: Sweet's syndrome, arthritis-dermatitis syndrome associated with inflammatory bowel disease and leukocytoclastic vasculitis. We describe the case of a young man with diagnosis of ulcerative rectocolitis, which presented an extensive cutaneous gangrene secondary to microvascular thrombosis. The case represents a dermatologic rarity and should be recognized as a cutaneous manifestation related to the hypercoagulability state observed in the disease's activity. PMID:25387503

Komatsu, Yumi Cristina; Capareli, Gabriela Cunha; Boin, Maria Fernanda Feitosa de Camargo; Lellis, Rute; de Freitas, Thais Helena Proenca; Simone, Karine

2014-01-01

409

Life-threatening rupture of an external iliac artery pseudoaneurysm caused by necrotizing fasciitis following laparoscopic radical cystectomy: a case report  

PubMed Central

Background Pseudoaneurysms are caused by trauma, tumors, infections, vasculitis, atherosclerosis and iatrogenic complications. In this paper, we report about a patient with rupture of an external iliac artery pseudoaneurysm, which lead to hemorrhagic shock, after undergoing laparoscopic radical cystectomy and extended pelvic lymphadenectomy. Case presentation The patient was a 68-year-old Japanese male diagnosed with invasive bladder cancer. Laparoscopic radical cystectomy and extended pelvic lymphadenectomy were performed. On postoperative day 12, he developed a high fever and an acute inflammatory response with redness and swelling in the right inguinal region. He was diagnosed with necrotizing fasciitis and underwent debridement. On postoperative day 42, a sudden hemorrhage developed from the open wound in the right inguinal region. He was diagnosed with external iliac artery pseudoaneurysm rupture by computed tomography. Conclusion These complications occur extremely rarely after cystectomy with pelvic lymphadenectomy. There are no reports to date on these complications following laparoscopic cystectomy with pelvic lymphadenectomy. PMID:24885366

2014-01-01

410

Protracted superficial Wegener granulomatosis in a child.  

PubMed

Wegener granulomatosis is a rare, chronic, multisystemic vasculitis affecting mainly the upper and lower respiratory tracts together with glomerulonephritis, but the disease may involve any other organ. Protracted superficial form is a rare variant of the disease. We report a case of protracted superficial Wegener granulomatosis in a 16-year-old boy in whom the disease started with recurrent digital ulcers at age 7 years. Later, he developed nodules and papules associated with upper airway involvement and ocular keratitis without lung or renal involvement. C-ANCA was positive. The patient was treated with oral prednisone. Similar cutaneous and mucosal lesions developed during two relapses of the disease without renal or respiratory involvement. PMID:18319011

Ben Ghorbel, Imed; Dhrif, Asma Sioud; Miled, Mohamed; Houman, Mohamed Habib

2007-01-01

411

Current options for the treatment of optic neuritis  

PubMed Central

Optic neuritis can be defined as typical (associated with multiple sclerosis, improving independent of steroid treatment), or atypical (not associated with multiple sclerosis, steroid-dependent improvement). Causes of atypical optic neuritis include connective tissue diseases (eg, lupus), vasculitis, sarcoidosis, or neuromyelitis optica. In this manuscript, updated treatment options for both typical and atypical optic neuritis are reviewed. Conventional treatments, such as corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin therapy are all discussed with commentary regarding evidence-based outcomes. Less commonly used treatments and novel purported therapies for optic neuritis are also reviewed. Special scenarios in the treatment of optic neuritis – pediatric optic neuritis, acute demyelinating encephalomyelitis, and optic neuritis occurring during pregnancy – are specifically examined. PMID:22927730

Pula, John H; MacDonald, Christopher J

2012-01-01

412

Pediatric Inflammatory Diseases. Part IV: Miscellaneous, Reye, PRES, Sarcoidosis.  

PubMed

Some rare neurological diseases affecting children have no well defined etiology and pathogenetic mechanisms. In this article diseases like Reye syndrome, Behçet disease, pediatric neurosarcoidosis, Posterior Reversible Encephalopathy Syndrome are described. Some of the main neuroradiological differential aspects are also critically considered. Reye syndrome is characterized by symmetric thalamic, white matter and basal ganglia lesions, in children with recent history of salycilates or immunosuppressive drugs intake. The most typical MRI feature of neurosarcoidosis is basilar meningeal thickening and enhancement with intraparenchymal enhancing nodules and white matter focal abnormalities. The classical distribution of lesions helps differential diagnosis with infectious meningoencephalitis. Differential diagnosis with relapsing-remitting multiple sclerosis his helped by the evidence of meningeal abnormalities. Neuro-Behçet is characterized by mesodiencephalic lesions in children with encephalopathy and coexistence of oral and genital ulcers and ocular abnormalities. PRES can be differentiated from vasculitis for the typical posterior white matter involvement and the different clinical features. PMID:24029186

Gallucci, M; Smith, J D; Limbucci, N; Rossi, A; Demaerel, P; Krings, T; D'Amico, A; Micheli, C

2012-12-20

413

Portal vein and aortic thromboses in a Siberian husky with ehrlichiosis and hypothyroidism.  

PubMed

A six-year-old, neutered male Siberian husky was presented for euthanasia for end-stage liver disease. Examination of the dog raised questions regarding the severity of the condition. It had presented to the referring veterinarian with polyuria, polydipsia and weight loss. Blood tests at that time revealed elevated liver enzymes and hypoalbuminaemia. Cirrhosis was presumptively diagnosed, based on an ultrasound examination, which showed ascites with a normal liver. The dog had a history of hypothyroidism, which was controlled with levothyroxine. Physical examination revealed cachexia. A second abdominal ultrasound examination was performed and revealed portal vein and aortic thromboses. Tick titres showed a positive Ehrlichia canis titre of 1:640. Skin biopsies showed lymphoplasmacytic vasculitis. Doxycycline and aspirin treatment was initiated, and the clinical signs resolved. The authors concluded that the thrombi were primarily caused by chronic ehrlichiosis, based on the clinical findings and the response to treatment. PMID:14510331

Bressler, C; Himes, L C; Moreau, R E

2003-09-01

414

Xenotransplantation of human neural progenitor cells to the subretinal space of nonimmunosuppressed pigs.  

PubMed

To investigate the feasibility of transplanting human neural progenitor cells (hNPCs) to the retina of nonimmunosuppressed pigs, cultured hNPCs were injected into the subretinal space of 5 adult pigs after laser burns were applied to promote donor cell integration. Postoperatively, the retinal vessels appeared normal without signs of exudation, bleeding, or subretinal elevation. Eyes were harvested at 10-28 days. H&E consistently showed mild retinal vasculitis, depigmentation of the RPE, and marked mononuclear cell infiltrate in the choroid adjacent to the site of transplantation. Human-specific antibodies revealed donor cells in the subretinal space at 10-13 days and smaller numbers within the retina on days 12 and 13, with evidence suggesting a limited degree of morphological integration; however, no cells remained at 4 weeks. The strong mononuclear cell reaction and loss of donor cells indicate that modulation of host immunity is likely necessary for prolonged xenograft survival in this model. PMID:21766011

Warfvinge, Karin; Schwartz, Philip H; Kiilgaard, Jens Folke; la Cour, Morten; Young, Michael J; Scherfig, Erik; Klassen, Henry

2011-01-01

415

[Uveitis: diagnostic approach].  

PubMed

A 32 year-old woman was referred from the Ophthalmology Department to rule out a possible systemic disease. Her only past medical history of relevance was a tuberculosis contact during childhood. She complained of floaters and progressive blurring of vision in both eyes for some months, as well as arthralgia and cough. Her visual acuity was 0.3 in the right eye and 0.4 in the left eye. Biomicroscopy showed bilateral anterior granulomatous uveitis (1+ cells). Funduscopy showed bilateral vitritis 3+, snow banking and peripheral phlebitis. Fluorescein angiography did not show central vasculitis, and optical coherence tomography showed bilateral cystoid macular oedema. Fundus autofluorescence was normal. How would you initially assess this patient in order to decide which systemic examination should be performed, bearing in mind the ophthalmological manifestations? PMID:22296724

Martínez-Berriotxoa, A; Fonollosa, A; Artaraz, J

2012-10-01

416

Tumor necrosis factor blockade in the management of children with orphan diseases.  

PubMed

Tumor necrosis factor (TNF) blockade has been used successfully to treat a number of rheumatic disorders that have a substantial burden of illness. In children, the TNF antagonists are used mainly for the treatment of juvenile idiopathic arthritis (JIA). There are, however, a variety of rare systemic inflammatory diseases, in which TNF blockade appears promising. Preliminary data in adults suggest that several forms of vasculitis appear to be responsive to TNF antagonists-Behcet's disease, polyarteritis nodosa, Wegener granulomatosis, among others. Some of them respond better to infliximab, a chimeric monoclonal anti-TNF antibody, than to etanercept, a recombinant p75 TNF receptor. We describe our limited experience with infliximab in the treatment of three children with rare vasculitic conditions. PMID:17219021

Brik, Riva; Gepstein, Vardit; Shahar, Eli; Goldsher, Dorit; Berkovitz, Drora

2007-10-01

417

Drug-induced granulomatous interstitial nephritis in a patient with ankylosing spondylitis during therapy with adalimumab.  

PubMed

Tumor necrosis factor ? (TNF-?) inhibitors are used in the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn disease, ankylosing spondylitis, and juvenile idiopathic arthritis. Use of TNF inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, psoriasis, and sarcoidosis/sarcoid-like granulomas). We report a case of interstitial granulomatous nephritis in a patient with ankylosing spondylitis after 18 months of treatment with adalimumab. Previously reported cases of sarcoid-like reactions secondary to the use of TNF-? inhibitors involved the liver, lung, lymph nodes, central nervous system, and skin. Granulomatous nephritis after adalimumab treatment has not been described. Close observation of patients undergoing treatment with TNF inhibitors for evolving signs and symptoms of autoimmunity is required. Organ involvement is unpredictable, which makes correct diagnosis and management extremely challenging. PMID:20974510

Korsten, Peter; Sweiss, Nadera J; Nagorsnik, Ulf; Niewold, Timothy B; Gröne, Hermann-Josef; Gross, Oliver; Müller, Gerhard A

2010-12-01

418

[Symptomatic intermittent fever of inflammatory diseases].  

PubMed

Intermittent fever has a wide variety of causes such as infectious, cancers, or inflammatory disease. Intermittent fever is sometimes a diagnostic challenge when fever appears as the first and isolated sign of the disease. Adult onset Still disease and juvenile chronic arthritis are mainly the most common cause of intermittent inflammatory fever. Some frequent diseases gives intermittent fever in few cases like ankylosing spondylitis, pulmonary embolism, sarcoidosis or Crohn's disease. Some rare inflammatory disease gives typical intermittent fever like genetic periodic fever. Other rare diseases give sometimes intermittent fever like vasculitis, polychondritis, Castleman disease, etc. Drug fever and factitious fever are other classical causes of intermittent fever. Diagnosis of inflammatory intermittent fever is frequently based on the clinical course but some biological tests and computerized tomographic scans are worthwhile tools. Follow-up of undiagnosed cases is needed. PMID:11915560

Hachulla, Eric

2002-01-15

419

Role of biological agents in immune-mediated inflammatory diseases.  

PubMed

A new era in the treatment of immune-mediated inflammatory disorders has begun with the clinical availability of anticytokine therapy. Biological agents that are currently available include 3 agents that decrease the activity of tumor necrosis factor-alpha (infliximab, adalimumab, etanercept) and an interleukin-1 receptor antagonist (anakinra), with many more in development. Those extraordinarily effective medications are an important addition to our therapeutic armamentarium, and, although originally developed for rheumatoid arthritis and Crohn disease, have been found to be efficacious in the treatment of seronegative spondyloarthropathies (psoriatic arthritis, ankylosing spondylitis) and juvenile rheumatoid arthritis. Their role is currently being defined in other autoimmune disorders such as uveitis, sarcoidosis, interstitial lung disease, vasculitis, inflammatory myopathies, graft-versus-host disease, and Sjögren syndrome. PMID:15759950

Efthimiou, Petros; Markenson, Joseph A

2005-02-01

420

Differentiating the efficacy of tumor necrosis factor inhibitors.  

PubMed

Blockade of tumor necrosis factor (TNF) has emerged as one of the most promising therapies in rheumatoid arthritis (RA). Three agents are currently available as specific TNF antagonists, etanercept (Enbrel), infliximab (Remicade), and adalimumab (Humira). Data from noncomparative trials suggest that all 3 agents have comparable therapeutic activity in RA. Etanercept and infliximab have also demonstrated beneficial activity in other inflammatory arthritides [i.e., psoriatic arthritis and ankylosing spondylitis (both agents) and juvenile rheumatoid arthritis (etanercept only)] and inflammatory diseases (i.e., psoriasis and uveitis). Their effects in granulomatous diseases are more variable, with only infliximab demonstrating clear efficacy in the treatment of Crohn's disease, sarcoidosis, and Wegener's vasculitis. In this brief review current efficacy data are summarized and possible explanations for observed clinical differences are explored. PMID:15742457

Haraoui, Boulos

2005-03-01

421

Anti-inflammatory actions of acupuncture.  

PubMed Central

Acupuncture has a beneficial effect when treating many diseases and painful conditions, and therefore is thought to be useful as a complementary therapy or to replace generally accepted pharmacological intervention. The attributive effect of acupuncture has been investigated in inflammatory diseases, including asthma, rhinitis, inflammatory bowel disease, rheumatoid arthritis, epicondylitis, complex regional pain syndrome type 1 and vasculitis. Large randomised trials demonstrating the immediate and sustained effect of acupuncture are missing. Mechanisms underlying the ascribed immunosuppressive actions of acupuncture are reviewed in this communication. The acupuncture-controlled release of neuropeptides from nerve endings and subsequent vasodilative and anti-inflammatory effects through calcitonine gene-related peptide is hypothesised. The complex interactions with substance P, the analgesic contribution of beta-endorphin and the balance between cell-specific pro-inflammatory and anti-inflammatory cytokines tumour necrosis factor-alpha and interleukin-10 are discussed. PMID:12775355

Zijlstra, Freek J; van den Berg-de Lange, Ineke; Huygen, Frank J P M; Klein, Jan

2003-01-01

422

Severe cutaneous adverse drug reactions  

PubMed Central

Severe cutaneous drug reactions are one of the commonest medical challenges presenting to an emergency room in any hospital. The manifestations range from maculopapular rash to severe systemic symptoms like renal failure and cardiovascular compromise. Toxic epidermal necrolysis, erythroderma, drug rash with eosinophilia and systemic symptoms, acute generalised exanthematous pustulosis and drug induced vasculitis are the common cutaneous drug reactions which can have severe morbidity and even mortality. Careful history taking of the lag period after drug intake and associated symptoms, along with detailed examination of the skin, mucosa and various systems, help in early diagnosis of these reactions. Early stoppage of the incriminating drug, specific therapy including corticosteroids, cyclosporine and intravenous immunoglobulin depending on the case along with supportive therapy and local measures help in salvaging most patients. An overview of these important cutaneous drug reactions along with their management is being reviewed in this article. PMID:24600147

Verma, Rajesh; Vasudevan, Biju; Pragasam, Vijendran

2013-01-01

423

Autoimmune thyroiditis presenting as interstitial granulomatous dermatitis.  

PubMed

A 54-year-old female presented with recurrent, widespread, erythematous, painful plaques, over a 3-month period. Skin biopsy was compatible with interstitial granulomatous dermatitis. Additional investigation revealed hypothyroidism and positive anti-thyroid antibodies. Normalization of thyroid function and high-potency topical corticosteroids provided only transitory improvement of the dermatosis. Interstitial granulomatous dermatitis is a histologic inflammatory reaction, with variable cutaneous expression. It has been reported in association with several drugs, lymphoproliferative diseases and autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus and vasculitis, but association with autoimmune thyroiditis is rare. Optimal therapy for this condition is yet to be established, but topical corticosteroids have been a mainstay of treatment. In most cases, this disease is characterized by flares and remissions. PMID:23044569

Antunes, Joana; Pacheco, David; Travassos, Ana Rita; Soares-Almeida, Luís Miguel; Filipe, Paulo; Sacramento-Marques, Manuel

2012-01-01

424

Wells' Syndrome Associated with Churg-Strauss Syndrome  

PubMed Central

Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features. PMID:22148019

Lee, Soo Hyun; Roh, Mi Ryung; Jee, Hyunjoong; Chung, Kee Yang

2011-01-01

425

[Neurologic complications in inflammatory bowel diseases].  

PubMed

Some inflammatory diseases of the gastrointestinal system are associated with neurological symptoms which, in rare cases, may precede the onset of the gastrointestinal manifestation of the disease. Celiac sprue is characterized by an intolerance to the wheat protein gluten. The typical neurological manifestation of celiac sprue is cerebellar ataxia. The ataxia as well as the gastrointestinal symptoms can be treated with a strictly gluten-free diet. The neurological symptoms of Whipple's disease, a disorder caused by gram-positive bacilli, are variable. Typical symptoms of cerebral Whipple's disease include dementia, ophthalmoplegia, movement disorders, and signs of hypothalamic dysfunction. Nowadays, the diagnosis of cerebral Whipple's disease is made by PCR detection of the bacilli in the CSF. Crohn's disease and ulcerative colitis are associated with neurological symptoms to a similar proportion. Various forms of polyneuropathies have been observed. The CNS manifestations of inflammatory bowel diseases include thromboembolic stroke, cerebral venous thrombosis, and cerebral vasculitis. PMID:12242995

Töpper, R; Gartung, C; Block, F

2002-06-01

426

Churg-strauss syndrome.  

PubMed

Churg-Strauss syndrome (CSS) is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%). We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010). The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS). PMID:22345778

Ghosh, Subhasish; Bhattacharya, Maitreyee; Dhar, Sandipan

2011-11-01

427

Spontaneous whole-lung torsion after massive pleural effusion and atelectasis.  

PubMed

We present a case of whole-lung torsion after massive pleural effusion and atelectasis. A 79-year-old woman with a history of recent pneumonia and pleurisy presented to our hospital and complained of left leg edema and pain that was considered to be vasculitis. A sagittal computed tomography (CT) scan showed that her whole right lung had a 120-degree counterclockwise torsion toward the hilum. We obtained and compared a CT image from the previous doctor. By comparing the CT scans, we determined that lung torsion had progressed gradually. To our knowledge, this is the first report that confirms the progress of whole-lung torsion with CT images. PMID:24384188

Irie, Masahiro; Okumura, Norihito; Nakano, Jun; Fujiwara, Atsushi; Noguchi, Misa; Kayawake, Hidenao; Yamashina, Akihiko; Matsuoka, Tomoaki; Kameyama, Kotaro

2014-01-01

428

Angiostrongylus vasorum infection in a coyote (Canis latrans) from Newfoundland and Labrador, Canada.  

PubMed

Tissue samples and feces were collected from a dead, adult female coyote (Canis latrans) found at the side of the road in late March 2003 in the Avalon Peninsula region of Newfoundland, Canada. The coyote apparently died of vehicular-related trauma. Samples of lung, brain, heart, liver, and kidney were fixed in formalin and submitted for histologic examination. The entire remaining lung and heart also were submitted for examination. The coyote was diagnosed with moderate, multifocal, granulomatous interstitial pneumonia with eosinophilic vasculitis and many intralesional nematode eggs, larvae, and occasional intravascular adult worms. Adult nematodes recovered from the pulmonary arteries were identified as Angiostrongylus vasorum. Small foci of granulomatous inflammation, often containing nematode eggs and larvae, were scattered in the brain and kidney. To our knowledge, this is the first report of A. vasorum infection in a coyote from the only endemic area of infection in North America. PMID:16456176

Bourque, Andrea; Whitney, Hugh; Conboy, Gary

2005-10-01

429

Rosacea-like facial rash related to metformin administration in a young woman  

PubMed Central

Background Since the skin represents a common site of adverse drug reactions, few data are reported at this time regarding the development of skin rash during the treatment with antidiabetic drugs. Case presentation We report a 29-year old woman that developed a facial skin rash during the treatment with metformin. Clinical and laboratory findings excluded the presence of systemic diseases, but several diagnosis and many drugs were administered without clinical improvement. The self-dismission of metformin induced an improvement of symptoms, while the re-challenge documented an impairments of skin rash. The Naranjo probability scale suggested a probable association between metformin and skin rash and metformin was definitively dismissed. Conclusion We report for the first time a non vasculitis facial skin manifestation related to metformin in a young woman. However, this case may emphasizes the need to consider the ADRs as a differential diagnosis in order to reduce medical errors and the related medical costs. PMID:24507578

2014-01-01

430

Interferon-? induced remission in three patients with eosinophilic granulomatosis and polyangiitis. A case study  

PubMed Central

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small vessel vasculitis associated with asthma and eosinophilia. Optimal therapy for maintenance of remission is yet to be defined. We present a case-series of three patients with EGPA in whom IFN-?, an immunomodulatory cytokine induced remission, which was maintained even after discontinuation of the drug. In all patients (ages 60, 51, and 50 years), remission was associated with normalisation of eosinophil counts and IgE-levels. Moreover, the patients remained in remission for one to four years. Two patients did not need further immunosuppression, one patient required low dose maintenance therapy. Although reversible side effects occur, IFN-?-therapy induces long-term remission of EGPA even after discontinuation of treatment.

Seeliger, B.; Foerster, M.; Neumann, T.; Moeser, A.; Happe, J.; Kehler, N.; Kroegel, C.

2013-01-01

431

Thoracoscopic cervicodorsal sympathectomy with diathermy.  

PubMed

This study reports our experience of using thoracoscopic cervicodorsal sympathectomy with diathermy. From December 1994 to September 1998, we performed 53 thoracoscopic sympathectomies in 35 patients. There were 15 men and 20 women, ages 18 to 61 years. Ten surgeries were performed on the right side, 7 were the left, and 18 were bilateral. Indications for surgery were causalgia/reflex sympathetic dystrophy in 8 patients, Raynaud's/vasculitis in 6, intractable Raynaud's disease in 4, and hyperhydrosis in 17 (bilateral procedure). Operating time ranged from 10 to 50 min for unilateral procedures and from 45 to 80 min for bilateral procedures. Patients stayed in the hospital 1 to 4 days. From favorable immediate and follow-up results we conclude that thoracoscopic cervicodorsal sympathectomy using diathermy is feasible, safe, and effective. PMID:10541610

Cartier, B; Cartier, P

1999-11-01

432

Ranavirus-associated morbidity and mortality in a group of captive eastern box turtles (Terrapene carolina carolina).  

PubMed

Seven captive eastern box turtles (Terrapene carolina carolina) from a large collection of North American chelonians in North Carolina became acutely ill in the fall of 2002. Five of the turtles died. Clinical signs included cutaneous abscessation, oral ulceration or abscessation (or both), respiratory distress, anorexia, and lethargy. The predominant postmortem lesion was fibrinoid vasculitis of various organs, including skin, mucous membranes, lungs, and liver. No inclusion bodies were detected by histopathology or electron microscopy of formalin-fixed tissue. An iridovirus was isolated from tissues obtained postmortem from two of the box turtles that died. The virus was characterized by electron microscopy, polymerase chain reaction, and sequence analysis of a portion of the major capsid protein as a member of the genus Ranavirus. PMID:15732597

De Voe, Ryan; Geissler, Kyleigh; Elmore, Susan; Rotstein, David; Lewbart, Greg; Guy, James

2004-12-01

433

Experimental malignant catarrhal fever (African form) in white-tailed deer.  

PubMed

White-tailed deer (Odocoileus virginianus) were experimentally infected with the African form of malignant catarrhal fever (AMCF) virus by inoculation of whole blood from experimentally infected cattle, from whole blood obtained from a greater kudu (Tragelaphus strepsiceros) and from virus isolated in cell culture. The incubation period from AMCF in experimentally infected deer ranged from 13 to 18 days. Clinical disease was characterized by lacrimation, an elevated body temperature, conjunctivitis and swelling of the external lymph nodes. Histologic lesions were primarily characterized by widespread vasculitis and lymphadenopathy. The organs most severely affected were liver, lymphoid tissue, brain and lungs. Successful recovery and identification of AMCF virus was accomplished from one experimentally infected deer. PMID:7310953

Whitenack, D L; Castro, A E; Kocan, A A

1981-07-01

434

[Systemic vasculitides: revised nomenclature, new therapeutic strategies].  

PubMed

Vasculitides comprise a heterogeneous group of inflammatory diseases of the blood vessels, which are characterized both by their diversity and an overlap of clinical and pathological findings and manifestations. The etiology of most vasculitides is not yet clear. Their nomenclature and classification is therefore a challenge for internists, immunologists, and pathologists. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature includes an update of the names and definitions of the CHCC 1994 nomenclature. In addition, four new categories were introduced to describe the spectrum of vasculitis comprehensively. Because of the side effects of cytotoxic immunosuppressive agents there is a need for safer and more effective targeted therapies. The first results from studies using biologicals in the treatment of vasculitides are promising. PMID:23512366

Lamprecht, P

2013-03-01

435

Juvenile Churg-Strauss Syndrome as an Etiology of Myocarditis and Ischemic Stroke in Adolescents; a Case Report  

PubMed Central

Background Churg-Strauss syndrome (CSS), a systemic vasculitis accompanied by asthma and eosinophilia, almost invariably affects the lung and is frequently associated with cutaneous involvement. It rarely has cardiac involvement. We report an unusual case of CSS with myocardial involvement and stroke. Case Presentation A 16-year old female suffered of allergic asthma for 4 years. She was under treatment with oral prednisolone and seretide inhalation. After CSS diagnosis, she developed paroxysmal atrial tachycardia. Serum levels of Troponin I and Troponin T were increased indicating massive myocardial damage probably due to myocarditis. After 5 months she developed acute hemiparesis without any evidence of ischemic or hemorrhagic event. She was treated with IVIg, intravenous pulses of methylprednisone and cyclophosphamide for each complication. Conclusion Myocarditis and stroke may also complicate CSS which should be taken in consideration for better management. PMID:23056844

Moradinejad, Mohammad-Hassan; Rezaei, Amir; Ziaee, Vahid

2011-01-01

436

The diagnosis and classification of the cryoglobulinemic syndrome.  

PubMed

Cryoglobulinemia refers to the presence of reversible precipitation of immunoglobulins in the blood upon exposure to reduced (body) temperature. In clinical practice, the detection and typing of cryoglobulins appear to be poorly standardized. A consensus protocol for detection and typing of cryoglobulins, as extracted from the literature, is presented. Items that require further standardization are discussed. Cryoglobulins may cause clinical symptoms due to either obstruction of the small blood vessels, in particular in the extremities that are more exposed to the cold, or vascular inflammation due to the deposition of immune complexes. Only for the latter situation, i.e. cryoglobulinemic vasculitis in the presence of mixed-type cryoglobulinemia, preliminary classification criteria have been defined. Besides the presence of cryoglobulins, at least two of three items have to be present. These items include a restricted number of self-reported clinical history, typical clinical manifestations, and specified laboratory findings. These classification criteria await further validation in an independent patient cohort. PMID:24424176

Damoiseaux, Jan

2014-01-01

437

T2-Weighted Cardiac Magnetic Resonance Imaging of Edema in Myocardial Diseases  

PubMed Central

The purpose of this paper is to describe imaging techniques and findings of T2-weighted magnetic resonance imaging (MRI) of edema in myocardial diseases. T2-weighted cardiac MRI is acquired by combining acceleration techniques with motion and signal suppression techniques. The MRI findings should be interpreted based on coronary artery supply, intramural distribution, and comparison with delayed-enhancement MRI. In acute myocardial diseases, such as acute myocardial infarction and myocarditis, the edema is larger than myocardial scarring, whereas the edema can be smaller than the scarring in some types of nonischemic cardiomyopathy, including hypertrophic cardiomyopathy. T2-weighted MRI of edema identifies myocardial edema associated with ischemia, inflammation, vasculitis, or intervention in the myocardium and provides information complementary to delayed-enhancement MRI. PMID:22973170

Amano, Yasuo; Tachi, Masaki; Tani, Hitomi; Mizuno, Kyoichi; Kobayashi, Yasuhiro; Kumita, Shinichiro

2012-01-01

438

Gender Differences in Beh?et's Disease Associated Uveitis  

PubMed Central

Behçet's disease is a systemic vasculitis of unknown etiology, characterized by oral and genital ulceration, skin lesions, and uveitis as well as vascular, central nervous system, and gastrointestinal system involvement. It is prevalent in the Middle East, Mediterranean, and Eastern Asia. The aim of this review is to evaluate the gender differences in clinical manifestations of Behçet's disease, treatment responses, mortality, and morbidity. Behçet's disease has been reported to be more prevalent in males from certain geographic regions and particular ethnic groups; however, recent reports indicate more even gender distribution across the world. There are gender differences in clinical manifestations and severity of the disease. Ocular manifestations, vascular involvement, and neurologic symptoms are more frequently reported in male patients whereas oral and genital ulcers, skin lesions, and arthritis occur more frequently in female patients. The disease can have a more severe course in males, and overall mortality rate is significantly higher among young male patients. PMID:24864195

Ucar-Comlekoglu, Didar; Sen, H. Nida

2014-01-01

439

A new infant case of Nakajo-Nishimura syndrome with a genetic mutation in the immunoproteasome subunit: an overlapping entity with JMP and CANDLE syndrome related to PSMB8 mutations.  

PubMed

Nakajo-Nishimura syndrome (NNS) is a very rare hereditary autoinflammatory disorder that generally has its onset in infancy with pernio-like rashes and gradually develops into partial lipodystrophy. A distinct homozygous PSMB8 mutation encoding an immunoproteasome subunit has recently been identified as its genetic cause. Here, we report a new case of a patient with NNS who developed exudative erythemas on his face and extremities at 2 months of age, along with high fever, elevated serum hepatic aminotransferase levels and hepatosplenomegaly. Massive infiltration of inflammatory cells was observed histologically in the dermis and subcutis without apparent leukocytoclastic vasculitis. These symptoms improved with oral corticosteroids but recurred periodically, and a thin angular face with long clubbed fingers gradually developed. Identification of the PSMB8 mutation finalized the diagnosis of NNS at 5 years of age. Understanding a variety of clinicopathological features at the developmental stages is necessary to make an early diagnosis of NNS. PMID:23942189

Kunimoto, Kayo; Kimura, Ayako; Uede, Koji; Okuda, Masumi; Aoyagi, Noriyuki; Furukawa, Fukumi; Kanazawa, Nobuo

2013-01-01

440

2007 Popper et Volume al. 8, Issue 12, Article R261 Open Access Research Gene-expression patterns reveal underlying biological processes in  

E-print Network

Gene Analysis expression expression programs of patterns in Kawasaki involving of gene disease neutrophil expression activation in peripheral and blood apoptosis. from children with Kawasaki disease revealed dynamic and variable gene Background: Kawasaki disease (KD) is an acute self-limited vasculitis and the leading cause of acquired heart disease in children in developed countries. No etiologic agent(s) has been identified, and the processes that mediate formation of coronary artery aneurysms and abatement of fever following treatment with intravenous immunoglobulin (IVIG) remain poorly understood. Results: In an initial survey, we used DNA microarrays to examine patterns of gene expression in peripheral whole blood from 20 children with KD; each was sampled during the acute, subacute, and convalescent phases of the illness. Acute KD was characterized by increased relative abundance of gene transcripts associated with innate immune and proinflammatory responses and decreased abundance of transcripts associated with natural killer cells and CD8 + lymphocytes.

Kawasaki Disease; Chisato Shimizu; Hiroko Shike; John T Kanegaye; Jane W Newburger; Robert P Sundel; Patrick O Brown; Jane C Burns; David A Relman

2007-01-01

441

Case of scrub typhus complicated by severe disseminated intravascular coagulation and death.  

PubMed

Scrub typhus is an infectious disease that is caused by Orientia tsutsugamushi. The authors describe an autopsied case of scrub typhus complicated with severe disseminated intravascular coagulation (DIC). An 82-year-old man complained of fever 4?days after climbing a mountain. The patient was admitted to an urban hospital, and meropenem and ceftriaxone were administered. The patient's condition deteriorated and he was transferred to a second hospital. On physical examination, a black scab was found and scrub typhus was suspected. Despite intensive treatment, the patient died on the fifth day. High levels of O.?tsutsugamushi IgM antibody were confirmed. An autopsy revealed systemic vasculitis and perivasculitis. The endothelial tissue of the white pulp of the spleen was markedly infiltrated by plasma cells. The authors speculated that a severe immune reaction against O.?tsutsugamushi enhanced an inflammatory response, leading to DIC. This case is a warning to doctors who are not familiar with scrub typhus. PMID:23039302

Ono, Yuko; Ikegami, Yukihiro; Tasaki, Kazuhiro; Abe, Masahumi; Tase, Choichiro

2012-10-01

442

Moyamoya syndrome in a known case of pulmonary tuberculosis  

PubMed Central

We report an unusual association of pulmonary tuberculosis with moyamoya syndrome in a 30-year-old Filipino female who was admitted to our hospital with a 1-week history of fever and cough. Chest X-ray showed widespread bilateral consolidation with cavity, whereas sputum was positive for acid fast bacilli (AFB). Two weeks after starting antituberculous treatment, the patient developed two episodes of loss of consciousness, which were unwitnessed. Urgent brain computed tomography (CT) showed multiple infarctions, suggesting vasculitis. The electroencephalogram showed epileptic discharges. Magnetic resonance angiography showed a picture consistent with moyamoya disease. Brain CT angiography was performed and it showed the same pictures. The patient was diagnosed with pulmonary tuberculosis-associated moyamoya syndrome. On the following days, she was discharged on antituberculous medications, antiepileptic and oral hypoglycemic treatment. After 1 year, the patient was seen in the clinic, she was well and seizure-free. PMID:21808515

Khan, Fahmi Yousef; Kamal, Hussain; Musa, Rania; Hayati, Ahmed

2010-01-01

443

Neutrophilic dermatoses: an update.  

PubMed

Neutrophilic dermatoses constitute a heterogeneous group of dermatologic diseases, which are unified by the predominance of neutrophils within the inflammatory infiltrate on histopathology. The aims of this review were to provide an update on the clinical and histologic presentation of the main neutrophilic dermatoses and to develop a guide for clinical practice. A structured literature search of PubMed, Medline, and Embase was performed, using the key words "neutrophilic disorders", "cutaneous small vessel vasculitis", "Sweet's syndrome", "bowel associated dermatosis arthritis syndrome", "Behcet's", "palisaded neutrophilic and granulomatous dermatosis", "rheumatoid neutrophilic dermatitis", and "pyoderma gangrenosum". Related articles were screened for key terms and were included if appropriate. This group contains a wide spectrum of unique disorders, each with its own histologic and clinical subtleties, making specific diagnosis of a given entity within the group diagnostically challenging. The fact that overlapping forms of neutrophilic dermatoses, which share features of multiple neutrophilic dermatoses, are not uncommon makes the diagnoses more challenging. PMID:25154386

Alavi, Afsaneh; Sajic, Dusan; Cerci, Felipe B; Ghazarian, Danny; Rosenbach, Misha; Jorizzo, Joseph

2014-10-01

444

Common dermatologic manifestations of primary immune deficiencies.  

PubMed

The skin is the largest organ of our body; it consists of the epidermis, dermis, hair follicles, sweat glands, blood vessels, and connective tissue matrix. Its main function is to act as a barrier to the outside world and protect us from infections. Any component of the skin is subject to insults from the environment and/or from within the body. Primary immune deficiency patients present with recurrent or prolonged infections not frequently seen in healthy individuals. Oftentimes, these infections involve the skin. Primary immune deficiency may also present with noninfectious cutaneous signs, such as eczema; erythroderma; granulomas; dysplasia of the skin, hair, nails, or teeth; pigmentary changes; angioedema; urticaria; vasculitis; or autoimmune skin disease due to immune dysregulation. Prompt recognition of the underlying diagnosis and initiation of treatment decrease morbidity. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of primary immune deficiency diseases. PMID:25269404

Relan, Manisha; Lehman, Heather K

2014-12-01

445

Cerebral tumor or pseudotumor?  

PubMed

Pseudotumoral lesions are uncommon but important to identity lesions. They can occur during inflammatory diseases (systemic diseases, vasculitis, demyelinating diseases), infectious, and vascular diseases. Also, in a patient with a treated tumor, pseudo-progression and radionecrosis must be differentiated from the tumoral development. Diagnosis can be difficult on an MRI scan, but some MRI aspects in conventional sequences, diffusion, perfusion and spectroscopy can suggest the pseudotumoral origin of a lesion. Imaging must be interpreted according to the context, the clinic and the biology. The presence of associated intracranial lesions can orientate towards a systemic or infectious disease. A T2 hyposignal lesion suggests granulomatosis or histiocytosis, especially if a meningeal or hypothalamic-pituitary involvement is associated. Non-tumoral lesions are generally not hyperperfused. In the absence of a definitive diagnosis, the evolution of these lesions, whether under treatment or spontaneous, is fundamental. PMID:25260711

Leclercq, D; Trunet, S; Bertrand, A; Galanaud, D; Lehéricy, S; Dormont, D; Drier, A

2014-10-01

446

Wegener's Granulomatosis: A Model of Auto-antibodies in Mucosal Autoimmunity  

PubMed Central

Wegener's granulomatosis (WG) is an autoimmune condition marked by vasculitis of small and medium sized vessels particularly affecting the upper respiratory tract and kidneys. There is a strong mucosal component similar to other autoimmune conditions such as systemic lupus erythematosus and Behçet's disease. While the pathogenesis of WG is not completely known, auto-antibodies such as IgG ANCAs have been implicated in endovascular damage and modulation of neutrophil / monocyte responses by Fc receptor (FcR) signaling. Due to the substantial mucosal involvement in WG (oral, nasal, and upper respiratory tract involvement), it is probable that IgA antibodies (perhaps IgA ANCAs) play a role in disease. Given discrepancies in associating ANCA levels with disease activity, future work should determine if IgA ANCAs are present in WG patients and examine the biology underlying the ANCAs' signaling partners - the FcRs. PMID:19482554

Kelley, James M.; Edberg, Jeffrey C.; Kimberly, Robert P.

2009-01-01

447

AL amyloidosis with temporal artery involvement simulates giant-cell arteritis.  

PubMed

Light-chain (AL) amyloidosis may present with features suggesting vasculitis, including giant-cell arteritis (GCA). We describe a case of an 80-year-old man, who initially presented with bilateral jaw claudication, bi-temporal headache and arthralgia, however a temporal-artery biopsy then revealed AL amyloidosis. A diagnosis of AL amyloidosis complicating multiple myelome simulates GCA and polymyalgia rheumatica was established. The patient was successfully treated with melphalan and dexamethasone: the free kappa light chains decreased, the patient's jaw claudication and headache disappeared. Then we discuss similarities between GCA and AL amyloidosis and potential confusion in diagnosis. We suggest that, in patients with clinical features of GCA without any temporal-artery typical findings, specimens are stained with Congo red, which then results in a different diagnosis and treatment. PMID:22209315

Audemard, Alexandra; Boutemy, Jonathan; Galateau-Salle, Françoise; Macro, Margaret; Bienvenu, Boris

2012-03-01