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1

Vasculitis  

MedlinePLUS

... in children than adults. On the other hand, giant cell arteritis occurs only in adults over 50 ... test that often finds large-vessel vasculitis. Detecting giant cell arteritis often involves a biopsy of an ...

2

Vasculitis Foundation  

MedlinePLUS

... to attend the 2015 International Vasculitis Symposium. The scholarship program is in honor of Mercy LaVilla, a ... the 2015 International Symposium 2015 International Vasculitis Symposium Scholarship Application Education Articles Feeling Alone – The Impact of ...

3

Urticarial Vasculitis  

MedlinePLUS

... vasculitis that affects the skin, causing wheals or hives and/or red patches due to swelling of ... the disease. Symptoms The most common symptoms are hives that cause itching, pain and a burning feeling. ...

4

Pulmonary Vasculitis  

PubMed Central

Pulmonary vasculitis describes a number of distinct disorders that are pathologically characterized by the destruction of blood vessels. The clinical manifestations of each disorder are defined by the size, type, and location of the affected vasculature. The clinical approach to these disorders rests upon an astute clinician considering the diagnosis and identifying the specific patterns of clinical, radiologic, laboratory, and pathologic abnormalities. Lung involvement is most commonly seen with the primary, idiopathic, small-vessel, or antineutrophil cytoplasmic antibody–associated vasculitides; Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. However, primary, idiopathic medium and large-vessel vasculitis, primary immune complex–mediated vasculitis, and secondary vasculitis are all capable of presenting with lung involvement. In this article, we focus on the more common, antineutrophil cytoplasmic antibody–associated disorder, vasculitides. PMID:16493151

Brown, Kevin K.

2006-01-01

5

Urticarial vasculitis.  

PubMed

Urticarial vasculitis is a clinico-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. The cutaneous features may include painful, burning or pruritic skin lesions, the persistence of individual lesions greater than 24 hours, palpable purpura, pronounced central clearing of lesions, and residual hyperpigmentation following resolution. However, because clinical characteristics of urticarial vasculitis may overlap with those of allergic urticaria, confirmation of the diagnosis requires a lesional skin biopsy. This condition is idiopathic in many patients but can also occur in the context of autoimmune disorders, infections, drug reactions, or as a paraneoplastic syndrome. In idiopathic urticarial vasculitis common laboratory findings are an elevation of erythrocyte sedimentation rate and reduction of serum complement. An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized as some clinical manifestations of disease overlap and C1q autoantibodies may be present in both diseases. Normo-complementemic patients usually have minimal or no systemic involvement and often have a better prognosis. On-the-other-hand, hypocomplementemic patients have the propensity to have more severe multi-organ involvement. Response to treatment is variable and a wide variety of therapeutic agents may be efficacious. Initial recommendations for treatment of urticarial vasculitis manifest only as non-necrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required. With necrotizing skin lesions or visceral involvement, corticosteroids are regularly indicated. Cases of severe corticosteroid resistant urticarial vasculitis or where corticosteroid morbidity is evident [table: see text] may require treatment with other immunosuppressive agents such as azathioprine, cyclophosphamide, or cyclosporine. PMID:12221865

Venzor, Joe; Lee, Wai L; Huston, David P

2002-10-01

6

Pustular vasculitis  

PubMed Central

We present a rare case of pustular vasculitis secondary to streptococcal infection. The majority of cases described in the literature predominantly involve the dorsal hands, and may subsequently spread to involve the lower limbs. However, our case demonstrates a rare case where primary involvement of the lower limbs was present with secondary involvement at the dorsum of the hands. The patient responded well to intravenous methylprednisolone reflected both clinically and by his acute phase response. PMID:23505282

Selvan, Shilpa; Shakir, Rebecca; Chan, Antoni

2013-01-01

7

Pediatric Vasculitis Initiative  

ClinicalTrials.gov

Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

2014-12-02

8

CNS vasculitis.  

PubMed

Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV. Infectious, neoplastic, and autoimmune conditions should be carefully evaluated. Knowledge of CNSV mimics is crucial given the therapeutic and prognostic implications. Reversible cerebral vasoconstriction syndrome is now recognized as the most common mimicker of PACNS. Much progress has been made in the understanding of PACNS histopathology, and specific clinical subsets and their response to treatment. Contrary to historical reports, recent PACNS cohorts achieved favorable clinical outcomes when early diagnosis and prompt treatment was initiated. Successful treatment with newer drugs has also been reported in PACNS cases. Newer imaging modalities hold promise for distinguishing inflammatory from noninflammatory vascular lesions. In this review, the authors provide a broad overview with updates on the types of adult CNSV along with its clinical assessment, diagnosis, and treatment. PMID:25369436

John, Seby; Hajj-Ali, Rula A

2014-09-01

9

Types of Vasculitis  

MedlinePLUS

... to stop using tobacco of any kind. Central Nervous System Vasculitis Central nervous system (CNS) vasculitis usually occurs as a result of ... condition is called isolated vasculitis of the central nervous system or primary angiitis (an-jee-I-tis) of ...

10

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

11

Primary cutaneous small vessel vasculitis  

Microsoft Academic Search

Opinion statement  Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits\\u000a discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Schönlein\\u000a purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt\\u000a to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis

James P. Russell; Roger H. Weenig

2004-01-01

12

Managing mesenteric vasculitis.  

PubMed

Mesenteric vasculitis is a rare diagnosis, but it comprises a group of disorders that may have devastating manifestations. It is often difficult to diagnose using clinical symptoms and biomarkers. Vascular imaging often provides the best opportunity for the noninvasive diagnosis of vasculitis and obviates the need for performing a biopsy. The medical management of vasculitis involves controlling the inflammatory process with the use of steroids or other immunosuppressants, but medical therapy does not consistently provide regression of the vascular changes (ie, aneurysms or vascular occlusions) seen at the time of the initial diagnosis. Operative management remains the mainstay of therapy for focal occlusive or aneurysms, but the treatment options for multifocal disease remain challenging. Endovascular treatment is increasingly being used as a first line of treatment for symptomatic vasculitis. Interventionalists should be familiar with the indications and outcomes associated with the various therapeutic options for mesenteric vasculitis-associated occlusive disease and aneurysms. PMID:25814202

Angle, John Fritz; Nida, Berhanemeskel A; Matsumoto, Alan H

2015-03-01

13

How Is Vasculitis Treated?  

MedlinePLUS

... into a vein. Certain types of vasculitis may require surgery to remove aneurysms that have formed as a ... Trials Clinical trials are research studies that explore whether a medical strategy, treatment, ...

14

Central Nervous System Vasculitis  

MedlinePLUS

... CNS vasculitis is usually treated with steroids. High-dose steroids such as prednisone, in combination with cyclophosphamide (a medication that decreases the immune system’s response to autoimmune diseases), are generally used. In some ...

15

CMR in inflammatory vasculitis  

PubMed Central

Vasculitis, the inflammation of blood vessels, can produce devastating complications such as blindness, renal failure, aortic rupture and heart failure through a variety of end-organ effects. Noninvasive imaging with cardiovascular magnetic resonance (CMR) has contributed to improved and earlier diagnosis. CMR may also be used in serial evaluation of such patients as a marker of treatment response and as an indicator of subsequent complications. Unique strengths of CMR favoring its use in such conditions are its abilities to noninvasively visualize both lumen and vessel wall with high resolution. This case-based review focuses on the large- and medium-vessel vasculitides where MR angiography has the greatest utility. Because of increasing recognition of cardiac involvement in small-vessel vasculitides, this review also presents evidence supporting greater consideration of CMR to detect and quantify myocardial microvascular disease. CMR’s complementary role amidst traditional clinical, serological and other diagnostic techniques in personalized care for patients with vasculitis is emphasized. Specifically, the CMR laboratory can address questions related to extent and severity of vascular involvement. As ongoing basic and translational studies better elucidate poorly-defined underlying molecular mechanisms, this review concludes with a discussion of potential directions for the development of more targeted imaging approaches. PMID:23199343

2012-01-01

16

Cutaneous vasculitis complicating coeliac disease.  

PubMed Central

A 38 year old female, with chronic uncontrolled coeliac disease, presented with the rare complication of cutaneous leucocytoclastic vasculitis. Detailed study failed to identify any cause for the vasculitis, other than the underlying coeliac disease. Haematuria and proteinuria with mesangial nephritis were also demonstrated on renal biopsy with electron microscopic study. It is speculated that exogenous or endogenous antigens permeated the abnormal small bowel mucosa leading to formation of circulating immune complexes. Subsequent tissue deposition of these complexes then resulted in vasculitis and nephritis. The skin lesions cleared completely after treatment with a strict gluten free diet. Images Fig. 1 Fig. 2 PMID:7461477

Meyers, S; Dikman, S; Spiera, H; Schultz, N; Janowitz, H D

1981-01-01

17

Animal models of systemic vasculitis.  

PubMed

Necrotizing leucocytoclastic vasculitis is the histopathological hallmark of the small vessel systemic vasculitides (SV), a group of human diseases commonly associated with anti-neutrophil cytoplasm autoantibodies (ANCA). Necrotizing vasculitis is seen in a number of experimental systems, but none of these provide an ideal animal model for human SV. Vasculitis occurs in serum sickness reactions; in murine models of systemic lupus erythematosus; in association with infection, particularly chronic viral infections; and after treatment with certain drugs or inflammatory mediators. 'Spontaneous' vasculitis has been reported in specific mouse strains, especially with ageing, and in some larger species. The size of vessel involved and the type of inflammatory cells predominating are variable in these experimental situations, and none of these models feature antibodies analogous to ANCA. We have recently reported that Brown Norway rats treated with mercuric chloride (HgCl2) develop necrotizing leucocytoclastic vasculitis, especially in the gut, and also develop antibodies to myeloperoxidase (MPO) which recognize similar determinants on MPO to those bound by a subset of ANCA. Transfer of serum from HgCl2-treated rats to naive animals does not induce tissue injury. Preliminary experiments using pooled immunoglobulin or an anti-CD4 monoclonal antibody did not show useful therapeutic benefit from these treatments. HgCl2-induced vasculitis has weaknesses as an animal model of human SV, but is the only experimental model in which anti-MPO autoantibodies have so far been demonstrated, and therefore may be of particular relevance to ANCA-associated SV. PMID:8388693

Mathieson, P W; Qasim, F J; Esnault, V L; Oliveira, D B

1993-04-01

18

Imaging spectrum of CNS vasculitis.  

PubMed

Cerebral vasculitis is characterized by inflammation of the walls of blood vessels and may affect vessels of any size. The pathogenesis of vasculitis remains poorly understood. Vasculitis may affect large vessels (Takayasu arteritis, giant cell arteritis), medium-sized vessels (Kawasaki disease, polyarteritis nodosa), small vessels (immunoglobulin A vasculitis, microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis), or variable-sized vessels (Behçet disease, Cogan syndrome). Primary angiitis of the central nervous system (CNS) is an idiopathic disorder with no evidence of generalized inflammation that may simulate reversible cerebral vasoconstriction syndromes. Vasculitis may be secondary to systemic disease, infection, malignancy, drug use, or radiation therapy. Imaging findings vary from small ischemic changes to frank infarction, hemorrhage, and white matter edema and may show contrast material enhancement. The cerebral arteries may demonstrate a beaded appearance with variable degrees of stenosis, occlusion, and contrast enhancement of the vessel wall. Correlation of imaging findings with clinical presentation and laboratory test results helps establish the diagnosis of CNS vasculitis. PMID:25019429

Abdel Razek, Ahmed Abdel Khalek; Alvarez, Hortensia; Bagg, Stephen; Refaat, Sherif; Castillo, Mauricio

2014-01-01

19

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

20

DRESS syndrome with cerebral vasculitis.  

PubMed

DRESS (drug rash with eosinophilia and systemic symptoms) syndrome is a severe reaction triggered by drugs that manifests as pyrexia and eosinophilia with involvement of the skin and internal organs. We herein describe the case of a patient who developed hyperuricemia after receiving treatment for tuberculosis, then took allpurinol and developed DRESS syndrome with a contextual coma and right hemisyndrome. This report describes for the first time the presence of vasculitic cerebral involvement in a patient with DRESS syndrome. The cerebral vasculitis responded to treatment, showing clinical and instrumental remission. In cases such as this, allergic cerebral vasculitis should be considered in the differential diagnosis because it can be treated if recognized early, thus leading to remission without the development of any sequelae. PMID:23774556

Sola, Daniele; Rossi, Luca; Sainaghi, Pier Paolo; Pirisi, Mario

2013-01-01

21

Silica exposure and systemic vasculitis.  

PubMed

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

2003-12-01

22

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

23

New insights on biomarkers in systemic vasculitis.  

PubMed

The systemic vasculitis is a heterogeneous group of diseases characterized by the inflammation of blood vessels. The development of advanced diagnostic tests and genetic studies have resulted in greater improvement in our understanding of vasculitis pathogenesis and thus in the development of newer therapies. However, there is still an unmet need in the management of systemic vasculitis, focused on developing of new biomarkers that would enable distinction between active disease from damage or infection and predict treatment response and prognosis. PMID:25740705

Perez-Alamino, Rodolfo; Maldonado-Ficco, Hernán

2015-03-01

24

Pregnancy Outcomes Among Patients With Vasculitis  

PubMed Central

Objective Pregnancy outcomes of patients with vasculitis are unknown, but are of great concern to patients and physicians. Through an online survey, this study assessed pregnancy outcomes among patients with vasculitis. Methods Participants in the Vasculitis Clinical Research Consortium Patient Contact Registry were invited to respond to an anonymous, internet-based survey that included questions about pregnancy outcomes, the timing of pregnancy relative to a diagnosis of vasculitis, and medication use. Results A total of 350 women and 113 men completed the survey. After a diagnosis of vasculitis, 74 pregnancies were reported by women and 18 conceptions were reported by men. The rate of pregnancy loss was higher among women who conceived after a diagnosis of vasculitis compared to those who conceived prior to diagnosis (33.8% versus 22.4%; P = 0.04). Among women, the rate of preterm births increased significantly for pregnancies conceived after a diagnosis of vasculitis relative to those conceived before diagnosis (23.3% versus 11.4%; P = 0.03). Only 18% of women reported worsening of vasculitis during pregnancy, but those who experienced increased vasculitis activity were more likely to deliver preterm. Exposure to cyclophosphamide or prednisone did not appear to impact pregnancy outcomes; however, the number of pregnancies among women taking these medications was small. Among the pregnancies conceived by men with vasculitis, the timing of diagnosis had no significant effect on the rate of pregnancy loss. Conclusion Women who conceived after a diagnosis of vasculitis had a higher rate of pregnancy loss than those who conceived prior to diagnosis. Vasculitis did not worsen during the majority of pregnancies conceived after diagnosis. PMID:23401494

Clowse, Megan E. B.; Richeson, Rachel L.; Pieper, Carl; Merkel, Peter A.

2015-01-01

25

Vasculitis Syndromes of the Central and Peripheral Nervous Systems  

MedlinePLUS

NINDS Vasculitis Syndromes of the Central and Peripheral Nervous Systems Information Page Synonym(s): Temporal Arteritis, Cranial Arteritis, Giant ... is Vasculitis Syndromes of the Central and Peripheral Nervous Systems ? Vasculitis is an inflammation of blood vessels, which ...

26

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

27

[Vasculitis and hereditary small vessel diseases].  

PubMed

In younger patients with stroke, cerebral vasculitis and hereditary small vessel diseases should be considered as important differential diagnoses. Since the clinical course of cerebral vasculitis is highly variable, diagnostic workup, which includes laboratory tests, CSF analysis, cranial magnetic resonance imaging and biopsy, is often challenging. Therapy should be initiated on an interdisciplinary basis and includes immunosuppressive induction and maintenance regimes. Hereditary small vessel diseases, e.g. CADASIL or Fabry's disease, can mimic clinical features of cerebral vasculitis. Their diagnosis which is based on family history, typical clinical features and genetic analysis often has implications for treatment and genetic counselling. PMID:19838657

Opherk, C; Peters, N; Dichgans, M

2009-11-01

28

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

29

Neutrophilic dermatosis of the dorsal hands: Pustular vasculitis revisited  

Microsoft Academic Search

An entity termed “pustular vasculitis of the hands” was recently described. Patients with this condition presented with low-grade fevers and erythematous plaques, pustules, and bullae limited to the dorsal hands and fingers, which were characterized histologically by a dense neutrophilic infiltrate and leukocytoclastic vasculitis. We describe patients with a similar clinical presentation, but who lacked vasculitis on biopsy findings. We

Noreen A. Galaria; Jacqueline M. Junkins-Hopkins; Douglas Kligman; William D. James

2000-01-01

30

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

31

Red blood cell distribution width as a useful indicator to predict systemic vasculitis in patients with cutaneous vasculitis.  

PubMed

Cutaneous vasculitis can be limited to skin or a manifestation of primary systemic vasculitis. However, there are no definite markers to predict systemic involvements. Recent studies have shown that a higher red blood cell distribution width (RDW) is associated with disease activity in various disorders. We evaluated whether RDW can be used as an indicator for predicting systemic disease in patients with initial cutaneous involvements. We reviewed clinical and laboratory information of 143 patients with cutaneous vasculitis and 15 pigmented purpuric dermatosis patients seen at single academic hospital in Korea. Various parameters, including RDW, were evaluated in patients with primary cutaneous vasculitis and primary systemic vasculitis with initial cutaneous manifestations. The RDW value between cutaneous and systemic vasculitis patients was compared and RDW level was also investigated whether it can indicate systemic vasculitis in patients with cutaneous involvements. The mean age was 32.0 years, and 102 (64.6 %) patients were female. A total of 132 patients were patients with primary cutaneous vasculitis, and 11 were primary systemic vasculitis. Higher ratio of patients with high RDW was detected in systemic vasculitis group compared with cutaneous vasculitis group (36.4 vs. 7.6 %, P < 0.05). The mean RDW was significantly higher in systemic vasculitis patients (P < 0.05). RDW had the strongest association with systemic vasculitis (P < 0.05, OR 1.834). In conclusion, elevated level of RDW was significantly associated with systemic vasculitis. RDW can be used as one of the marker to predict systemic disease in patients with cutaneous vasculitis. PMID:25284376

Kim, Dae Suk; Shin, Dongyun; Kim, Tae-Gyun; Kim, Sung Hee; Kim, Do Young; Kim, Soo Min; Lee, Min-Geol

2015-04-01

32

Cryoglobulinemia and cutaneous vasculitis in human brucellosis  

Microsoft Academic Search

We report the occurrence of cryoglobulinemia and cutaneous vasculitis in three patients with brucellosis caused byBrucella melitensis. The isolated cryoglobulins were characterized as mixed polyclonal or type III. Brucella agglutinin activity was not detected in any of the cryoglobulins analyzed. However, the same agglutinin titer and the presence of precipitin lines of identity in immuno-diffusion gels were observed in the

Juan L. Yrivarren; Luis R. Lopez

1987-01-01

33

Isolated vasculitis of the peripheral nervous system.  

PubMed

Vasculitis restricted to the peripheral nervous system (PNS), referred to as nonsystemic vasculitic neuropathy (NSVN), has been described in many reports since 1985 but remains a poorly understood and perhaps under-recognized condition. There are no uniform diagnostic criteria. Classification is complicated by the occurrence of vasculitic neuropathies in many systemic vasculitides affecting small-to-medium-sized vessels and such clinical variants as nonsystemic skin/nerve vasculitis and diabetic/non-diabetic lumbosacral radiculoplexus neuropathy. Most patients present with painful, stepwise progressive, distal-predominant, asymmetric or multifocal, sensory-motor deficits evolving over months-to-years. NSVN is identical to but less severe than systemic vasculitis-associated neuropathies (SVNs). All vasculitic neuropathies are axonal by electrodiagnostic/pathologic criteria. Laboratory testing is unremarkable except for mildly elevated erythrocyte sedimentation rate (ESR) in 50%. Highly elevated ESRs, leukocytosis, rheumatoid factors, and anti-neutrophil cytoplasmic antibodies (ANCAs) raise concern for underlying systemic vasculitis. Without a specific clinical/laboratory marker, the condition depends on nerve biopsy for diagnosis. Biopsies showing necrotizing vasculitis are about 50% sensitive, mandating reliance on "suspicious" changes in many patients. Vasculitic lesions predominate in smaller epineurial vessels and are milder than those in SVNs. The disorder is often accompanied by subclinical involvement of adjacent muscles and skin. NSVN has the potential to spontaneously relapse and remit but neurologic deficits accumulate. No randomized controlled trials have been performed, but one retrospective cohort survey showed combination therapy to be more effective than prednisone alone. Although most patients have a good outcome, more than 30% relapse and 60% have residual pain. Many nosologic, pathogenic, diagnostic, and therapeutic questions remain unanswered. PMID:18799069

Collins, M P; Periquet, M I

2008-01-01

34

Vasculitis in childhood – a dermatological approach.  

PubMed

Vasculitis, an inflammatory condition affecting the blood vessels, may be restricted to a single organ or involve several organ systems. The size of the involved vessels is an important criterion for categorization of vasculitides, which is a prerequisite for rapid diagnosis and initiation of treatment. In pediatric patients, this particularly applies to Kawasaki disease. However, making the diagnosis can be challenging for dermatologists as skin involvement may be variable and non-specific. In contrast, Henoch-Schönlein purpura (IgA vasculitis) presents with the classic picture of palpable purpura. It predominantly affects postcapillary venules frequently following upper respiratory tract infections. Severe organ involvement is relatively rare in children and the prognosis is good. As renal involvement may occur during the course of disease, continuous monitoring is required. Acute hemorrhagic edema of infancy is considered as a distinct type of immune complex vasculitis and is characterized by a triad of fever, edema and rosette-shaped purpura. The clinical course of this rare disease is usually benign and self-limited. Due to the variability of clinical symptoms and manifestations, management of childhood vasculitides represents a special challenge requiring interdisciplinary collaboration. Dermatologists should be aware of their important role especially for making an early diagnosis. PMID:24494640

Sandrine, Benoit; Goebeler, Matthias

2014-02-01

35

Urticarial vasculitis and urticarial autoinflammatory syndromes.  

PubMed

Urticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1?, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications. PMID:25586657

Marzano, A V; Tavecchio, S; Venturini, M; Sala, R; Calzavara-Pinton, P; Gattorno, M

2015-02-01

36

Vasculitis Syndromes of the Central and Peripheral Nervous Systems  

MedlinePLUS

... or PNS vasculitis will gather a comprehensive medical history of the individual, perform a physical examination, order laboratory tests ( ... Johns Hopkins Vasculitis Center, and Mayo Clinic College of Medicine. The Consortium’s internet site provides information about clinical research and clinical ...

37

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

38

Limited Cutaneous Vasculitis Associated With Levamisole-Adulterated Cocaine  

PubMed Central

Levamisole is among the many contaminants that have been detected in seized cocaine throughout North America and Europe. Little is known about the association between levamisole-adulterated cocaine and vasculitis. Herein we describe a case of limited cutaneous vasculitis manifested as retiform purpura and skin necrosis in a user of cocaine contaminated with levamisole. PMID:23024742

Yachoui, Ralph; Kolasinski, Sharon L; Eid, Hala

2012-01-01

39

Limited cutaneous vasculitis associated with levamisole-adulterated cocaine.  

PubMed

Levamisole is among the many contaminants that have been detected in seized cocaine throughout North America and Europe. Little is known about the association between levamisole-adulterated cocaine and vasculitis. Herein we describe a case of limited cutaneous vasculitis manifested as retiform purpura and skin necrosis in a user of cocaine contaminated with levamisole. PMID:23024742

Yachoui, Ralph; Kolasinski, Sharon L; Eid, Hala

2012-10-01

40

Treatment of cystic fibrosis associated cutaneous vasculitis with chloroquine  

Microsoft Academic Search

Vasculitis is a well recognised complication of Cystic Fibrosis. Corticosteroids are the mainstay of treatment but some cases can be resistant and may require additional disease modifying agents.We describe a case of steroid resistant cutaneous vasculitis which was successfully treated with chloroquine in addition to corticosteroids and a subsequent relapse with chloroquine alone.

Ian D. Molyneux; Tanya Moon; A. Kevin Webb; Alyn H. Morice

2010-01-01

41

Renal paraneoplastic vasculitis complicating lung adenocarcinoma.  

PubMed

Renal paraneoplastic vasculitis (RNPV) is rare. It can be revealed by glomerulonephritis, microaneurysms or renal failure. RPNV may precede the onset of the primary tumor, and treatment and prognosis depend on the etiology (primary tumor). A 54-year-old man who had a primary lung adenocarcinoma was admitted for nephrotic syndrome. The investigations revealed RNPV. The patient was treated with corticosteroids at high dose and cyclophosphamide with improvement of the renal condition; however, the patient died from worsening of his pulmonary neoplasia. PMID:25193910

Dhaou, Besma Ben; Boussema, Fatma; Aydi, Zohra; Ketari, Sonia; Baili, Lilia; Moussa, Fatma Ben; Rokbani, Lilia

2014-09-01

42

Pathogenesis of ANCA-associated vasculitis.  

PubMed

Antineutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitides (AAV) are a group of systemic vasculitis syndromes characterized by inflammation and necrosis of blood vessel walls. Genetic, epigenetic, and environmental factors contribute to the etiology and pathogenesis of AAV. On the basis of currently available clinical and experimental evidence, it is reasonable to believe 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:22927039

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

2012-12-01

43

[Necrotizing cutaneous vasculitis with massive gastrointestinal bleeding following naproxen treatment].  

PubMed

Cutaneous necrotizing vasculitis is usually induced by an acute infection or exposure to a drug. Cutaneous vasculitis may precede severe systemic involvement, and may end in death. Accordingly, diagnosis of cutaneous vasculitis, identification of etiological factors, follow-up for systemic involvement and treatment are important. The authors present a case study of a 58-year-old male with fever and extensive eruption involving the trunk and extremities which appeared two days after initiation of treatment with oral naproxen. The clinical and histological findings were consistent with cutaneous necrotizing leukocytoclastic vasculitis. Two events of massive upper gastrointestinal bleeding occurred during treatment with systemic corticosteroids. The possible etiological factors and the mechanisms involved in the induction of the vasculitis and the gastrointestinal bleeding are discussed. PMID:20684166

Horev, Amir; Etzion, Ohad; Halevy, Sima

2010-03-01

44

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

45

pANCA-vasculitis associated with rectal adenocarcinoma.  

PubMed

We report the case of a 69-year-old male patient who was admitted for fever, dry cough, recurrent sinusitis with epistaxis, anorexia with weight loss of 20 kg over a 3-month period, myalgia, and mononeuritis multiplex. He was diagnosed with pANCA/anti-MPO associated vasculitis and rectal adenocarcinoma. The tumor was treated by surgical resection. Recurrence of vasculitis occurred during steroid tapering which prompted us to add Mycophenolate mofetyl. A complete remission was achieved. We conclude that in the present case the vasculitis was an independent disease, not a paraneoplastic phenomenon. We discuss the value of different ANCA serologies for diagnostics and follow-up, the epidemiology of vasculitis associated with malignancy, and the concept of vasculitis as a paraneoplastic syndrome. PMID:25113856

Hommel, C; Rihova, Z; Mokaddem, F; Libotte, B

2014-12-01

46

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

47

Diagnosis and treatment of cerebral vasculitis  

PubMed Central

Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved. PMID:21180634

Berlit, Peter

2010-01-01

48

Vasculitis and stroke due to Lyme neuroborreliosis - a review.  

PubMed

Abstract Lyme neuroborreliosis (LNB) is a rare cause of vasculitis and stroke. It may manifest as subarachnoid hemorrhage, intracerebral hemorrhage, and most often ischemic stroke due to cerebral vasculitis. The vast majority of reported cases have been described by European authors. A high index of suspicion is required in patients who live or have traveled to areas with high prevalence of tick-borne diseases, and in the case of stroke-like symptoms of unknown cause in patients without cardiovascular risk factors. In this review, we also present four illustrative cases of vasculitis and stroke-like manifestations of LNB. PMID:25342573

Zajkowska, Joanna; Garkowski, Adam; Moniuszko, Anna; Czupryna, Piotr; Ptaszy?ska-Sarosiek, Iwona; Tarasów, Eugeniusz; Ustymowicz, Andrzej; ?ebkowski, Wojciech; Pancewicz, S?awomir

2015-01-01

49

What Are the Signs and Symptoms of Vasculitis?  

MedlinePLUS

... you have. Gastrointestinal Tract If vasculitis affects your gastrointestinal tract, you may get ulcers (sores) in your mouth or have stomach pain. In severe cases, blood flow to the intestines can be blocked. ...

50

Historical perspective of vasculitis: Polyarteritis nodosa and microscopic polyangiitis  

Microsoft Academic Search

The original and early case reports of vasculitis provide a historical context and foundation for understanding current concepts\\u000a of these diseases. These early case reports are valuable as reference points for the current efforts in diagnosing, treating,\\u000a and classifying vasculitis. In addition, they emphasize the importance of careful clinical observation in these efforts and\\u000a the essential nature of medical science.

Eric L. Matteson

2002-01-01

51

Update on diffuse alveolar hemorrhage and pulmonary vasculitis.  

PubMed

Diffuse alveolar hemorrhage is a clinical syndrome that can be a manifestation of multiple different causes. Identification of the underlying etiology is of utmost importance and dictates treatment. Pulmonary vasculitis including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a common cause of diffuse alveolar hemorrhage. For AAV, treatment includes induction followed by maintenance therapy. Rituximab has an increasing role in the treatment of AAV. PMID:23102067

Krause, Megan L; Cartin-Ceba, Rodrigo; Specks, Ulrich; Peikert, Tobias

2012-11-01

52

Testicular Vasculitis – Literature Review and Case Report in Queensland  

PubMed Central

A 21-year-old male presented with acute onset, sharp right sided testicular pain. The testicle was removed with a histological diagnosis of testicular vasculitis. Anti-neutrophil cytoplasmic antibodies were negative. Although rare, males who present with acute onset pain should be screened for testicular vasculitis with a scrotal ultrasound and blood investigations including tumor markers and anti-neutrophil cytoplasmic antibodies. PMID:24917768

Lintern, Narelle; Johnson, Nigel R.; Mckenzie, Ian; Martin, Ben

2013-01-01

53

Preliminary classification criteria for the cryoglobulinaemic vasculitis  

PubMed Central

Background To develop preliminary classification criteria for the cryoglobulinaemic syndrome or cryoglobulinaemic vasculitis (CV). Methods Study part I developed a questionnaire for CV to be included in the formal, second part (study part II). Positivity of serum cryoglobulins was defined by experts as an essential condition for CV classification. In study part II, a core set of classification items (questionnaire, clinical and laboratory items, as agreed) was tested in three groups of patients and controls—that is, group A (new patients with the CV), group B (controls with serum cryoglobulins but lacking CV) and group C (controls without serum cryoglobulins but with features which can be observed in CV). Results In study part I (188 cases, 284 controls), a positive response to at least two of three selected questions showed a sensitivity of 81.9% and a specificity of 83.5% for CV. This questionnaire was employed and validated in study part II, which included 272 patients in group A and 228 controls in group B. The final classification criteria for CV, by pooling data from group A and group B, required the positivity of questionnaire plus clinical, questionnaire plus laboratory, or clinical plus laboratory items, or all the three, providing a sensitivity of 88.5% and a specificity of 93.6% for CV. By comparing data in group A versus group C (425 controls), the same classification criteria showed a sensitivity 88.5% and a specificity 97.0% for CV. Conclusion Classification criteria for CV were developed, and now need validation. PMID:21571735

De Vita, S; Soldano, F; Isola, M; Monti, G; Gabrielli, A; Tzioufas, A; Ferri, C; Ferraccioli, G F; Quartuccio, L; Corazza, L; De Marchi, G; Casals, M Ramos; Voulgarelis, M; Lenzi, M; Saccardo, F; Fraticelli, P; Mascia, M T; Sansonno, D; Cacoub, P; Tomsic, M; Tavoni, A; Pietrogrande, M; Zignego, A L; Scarpato, S; Mazzaro, C; Pioltelli, P; Steinfeld, S; Lamprecht, P; Bombardieri, S; Galli, M

2011-01-01

54

Systemic vasculitis: an annual critical digest of the most recent literature.  

PubMed

Herewith we provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia. PMID:24854379

Della Rossa, Alessandra; Cioffi, Elisa; Elefante, Elena; Ferro, Francesco; Parma, Alice; Vagelli, Roberta; Talarico, Rosaria

2014-01-01

55

Isolated lower limb medium-vessel vasculitis: a new entity?  

PubMed Central

A 47-year-old female was referred with bilateral ischaemic lesions affecting her toes with associated gangrene. Systemic examination was normal and her medical history was unremarkable. Close examination of the hands revealed splinter haemorrhages. Her feet were dusky in colour and the toes were dark and weeping, each becoming gangrenous, with erythema around the metatarsal phalangeal joint. Dorsalis pedis and posterior tibial pulses were intact with strong Doppler signals. Investigations for surgical pathology and malignancy were all negative as were routine blood tests and tests for autoimmune and viral markers. Vasculitis was diagnosed when her toes showed improvement with steroids, however, attempts to reduce the steroid dose were unsuccessful and azathioprine was introduced. Despite immunosuppression and immunomodulators, her toes deteriorated and were amputated. Histology revealed findings consistent with a medium-vessel vasculitis which antineutrophil cytoplasmic antibody was negative. A similar vasculitis has not been formally reported in the literature. PMID:22691585

Gore, David; Bukhari, Marwan

2011-01-01

56

Mycoplasmal Upper Respiratory Infection Presenting as Leukocytoclastic Vasculitis  

PubMed Central

Mycoplasma is a virulent organism that is known to primarily infect the respiratory tract; however, affection of the skin, nervous system, kidneys, heart and bloodstream has been observed in various forms, which include Stevens Johnson syndrome, erythema multiforme, toxic epidermal necrolysis, encephalitis, renal failure, conduction system abnormalities and hemolytic anemia. Small vessel vasculitis is a lesser-known complication of mycoplasma pneumonia infection. We report a case of mycoplasmal upper respiratory tract infection with striking cutaneous lesions as the presenting symptom. Mycoplasmal infection was confirmed by serology testing, skin biopsy was suggestive of leukocytoclastic vasculitis. This case brings forth an uncommon manifestation of mycoplasmal infection with extra-pulmonary affection, namely small vessel vasculitis.

Rao, Mana; Agrawal, Abhinav; Parikh, Manan; Banayat, Rikka; Thomas, Maria Joana; Guo, Tianhua; Lee, Andrew

2015-01-01

57

Secondary Syphilis with Nodular Vasculitis Mimicking Behçet'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

58

[Relation between vasculitis and hepatitis HBsAg].  

PubMed

The authors present the development of a generalized necrotic vasculitis accompanied by positive Virus B Cronic Hepatitis. A 31 years old female, affected by a multisystemic disease with jaundice, dark urine, and light stools, with biologic values of necrosis of hepatic cells and positive dosages of HBsAg. The biopsy of muscle revealed the presence of an alergic vasculitis with leucocitoclasia and the biopsy of the liver showed and Active Cronic Hepatitis. References are made about the different pathogenical hypothesis of these unusual association. PMID:747085

Parcero, S A; Orfus, S; Malamud, S W; Huberman, E D

1978-01-01

59

Leukocytoclastic Vasculitis Associated with Influenza A Virus Infection  

PubMed Central

Leukocytoclastic vasculitis (LCV) usually presents palpable purpura characterized by inflammation of vessel walls and fragmentation of nuclei. Various conditions can cause LCV, and it can be induced by influenza A virus infection. We report a 2-yr-old Korean girl who presented palpable purpuric and hemorrhagic lesions with fever. She was diagnosed as LCV by skin biopsy, and influenza A virus was isolated from nasopharyngeal swab. She was treated with oseltamivir (Tamiflu®) and prednisolone with dramatic effect of vasculitis and fever. PMID:23255867

Lee, Hyo Jin; Choi, Jong Soo; Kim, Ki Hong

2012-01-01

60

Leukocytoclastic vasculitis and acute allergic interstitial nephritis following ceftriaxone exposure.  

PubMed

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. PMID:25422573

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

2014-10-01

61

[Necrotizing glomerulonephritis in decursu vasculitis after vaccination against influenza].  

PubMed

Vaccinations against influenza are common, acceptable way of prevention of influenza, which develops as epidemic on huge areas of Europe. We described 17 years old girl, who has developed vasculitis after vaccination against influenza vaccine Vaxigrip produced by Aventis Pasteur. Aggressive immunosuppressive treatments caused regression majority of clinical symptoms and normalization of renal function tests. PMID:16194032

Hyla-Klekot, Lidia; Kucharska, Grazyna; Cieslak, Witold

2005-07-01

62

What’s new in the aetiopathogenesis of vasculitis?  

Microsoft Academic Search

The cause of the majority of childhood vasculitides is unknown although it is likely that a complex interaction between environmental\\u000a factors and inherited host responses trigger the disease and determine the vasculitis phenotype. Epidemiological clues continue\\u000a to implicate infectious triggers in Kawasaki syndrome (KS) and Henoch Sch?nlein purpura (HSP). Several genetic polymorphisms\\u000a have now been described in KS and HSP

Paul A. Brogan

2007-01-01

63

Central Nervous System Vasculitis: Still More Questions than Answers  

PubMed Central

The central nervous system (CNS) may be involved by a variety of inflammatory diseases of blood vessels. These include primary angiitis of the central nervous system (PACNS), a rare disorder specifically targeting the CNS vasculature, and the systemic vasculitides which may affect the CNS among other organs and systems. Both situations are severe and convey a guarded prognosis. PACNS usually presents with headache and cognitive impairment. Focal symptoms are infrequent at disease onset but are common in more advanced stages. The diagnosis of PACNS is difficult because, although magnetic resonance imaging is almost invariably abnormal, findings are non specific. Angiography has limited sensitivity and specificity. Brain and leptomeningeal biopsy may provide a definitive diagnosis when disclosing blood vessel inflammation and are also useful to exclude other conditions presenting with similar findings. However, since lesions are segmental, a normal biopsy does not completely exclude PACNS. Secondary CNS involvement by systemic vasculitis occurs in less than one fifth of patients but may be devastating. A prompt recognition and aggressive treatment is crucial to avoid permanent damage and dysfunction. Glucocorticoids and cyclophosphamide are recommended for patients with PACNS and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis. CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids (giant-cell arteritis) or glucocorticoids and immunosuppressive agents (Takayasu’s disease). However, in large vessel vasculitis, where CNS symptoms are usually due to involvement of extracranial arteries (Takayasu’s disease) or proximal portions of intracranial arteries (giant-cell arteritis), revascularization procedures may also have an important role. PMID:22379458

Alba, Marco A; Espígol-Frigolé, Georgina; Prieto-González, Sergio; Tavera-Bahillo, Itziar; García-Martínez, Ana; Butjosa, Montserrat; Hernández-Rodríguez, José; Cid, Maria C

2011-01-01

64

Mixed cryoglobulinemia as a model of systemic vasculitis  

Microsoft Academic Search

Summary  Leukocytoclastic vasculitis is the dominant lesion of mixed cryoglobulinemia (MC). The high prevalence of antibodies to hepatitis\\u000a C virus (HCV) in association with the higher concentration of HCV RNA genomic sequences in the cryoglobulins suggests a close\\u000a relationship between MC and HCV infection and strongly supports the view that this virus plays a key role in causing vascular\\u000a damage. Analysis

Franco Dammacco; Domenico Sansonno

1997-01-01

65

Cutaneous leukocytoclastic vasculitis associated with anti-tuberculosis drugs.  

PubMed

Leukocytoclastic vasculitis (LCV), a disease characterized by inflammation of the small vessels, presents with palpable purpura, especially in the lower extremities. Its etiology is known to include drugs, infection, collagen tissue disease, and malignancy, but LCV caused by anti-tuberculosis drugs is very rarely seen. This report describes the case of a 12-year-old girl who developed LCV with rifampicin and ethambutol while undergoing anti-tuberculosis treatment due to extensive pulmonary involvement. PMID:25711253

Avcu, Gülhadiye; Sensoy, Gülnar; Çeliksoy, Mehmet Halil; Sogut, Ayhan; Kefeli, Mehmet; Karli, Arzu; Belet, Nursen

2015-02-01

66

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

67

[Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine].  

PubMed

In this article the diagnostics, differential diagnosis, laboratory findings, multimodal imaging and treatment of vasculitis of small, medium and large vessels as well as granulomatous and eosinophilic vascular diseases are described in the context of previous and current European League against Rheumatism (EULAR) and American College of Rheumatology (ACR) recommendations. Vasculitis is a syndrome which is part of various clinical disease entities, such as Wegener's granulomatosis, polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, cryoglobulinemia and other forms of vasculitis. PMID:25676009

Maisch, B

2015-02-01

68

Rhinologic and sinonasal changes in PR3 ANCA pulmonary vasculitis  

PubMed Central

Background Over 70-95% patients with PR3 ANCA pulmonary vasculitis present with upper respiratory tract symptoms or sings. Nasal cavity usually presents with obstruction and chronic refractory infections (rhinosinusitis) which commonly manifest as bloody discharge or crusting obstruction. Mucopurulent discharge may occur in the acute phase or remission, along with other symptoms suggesting sinusitis. Later on, saddle nose deformities can occur due to collapse of the nasal septum. Other common destruction areas are the maxillary ostia, erosion of the tubinates or damage of soft palate. Objective The aim of the study was to characterize pathologies of nasal and sinonasal CT scans in patients with PR3 pulmonary ANCA vasculitis and to establish the CT diagnostic criteria for WG. Between 2005-2009 sinonasal CT visualization was performed in 35 patients (19 female, 16 male) with PR3 ANCA positive WG. Results Bony destruction of the nasal cavity was revealed in 15 (42.8%), damage or distortion of the paranasal sinuses in 20 (57.1%), the mastoid cells in 7 (20%), and the orbits in 7 (20%) patients. Sclerosing osteitis of the nasal cavity and paranasal sinuses were observed in 11 (31.4%) and in 24 (68.5%), respectively. Bony thickening of the nasal cavity was shown in 5 (14.2%) patients and of the paranasal sinuses in 7 (20%) (unilateral in 2 and bilateral in 5 patients). Seven patients (20%) had orbital masses; all unilateral. Septal perforation was observed in 11 (31.4%) and saddle nose deformity in 7 (20%) patients. Conclusions Maxillary sinuses are regions which are most frequently affected during the course of PR3 ANCA pulmonary vasculitis. CT imagines may be a useful supplement to clinical and activity scoring of WG disease with pulmonary involvement. PMID:21147659

2010-01-01

69

Upregulated expression and function of integrin adhesive receptors in systemic lupus erythematosus patients with vasculitis.  

PubMed Central

Upregulation of integrin adhesive receptors has been implicated in various pathological conditions. We examined expression and function of integrin adhesive receptors on peripheral blood lymphocytes from patients with systemic lupus erythematosus (SLE), particularly those with the complication of vasculitis, and found that VLA-4 and LFA-1 expression was increased in SLE patients with vasculitis, while LFA-1 but not VLA-4 expression was increased in those without vasculitis. These results suggested a role of VLA-4 in the pathogenesis of vasculitis in SLE. Functional studies further demonstrated that adhesion to cytokine-activated human umbilical cord vein endothelial cells and to the CS-1 alternatively spliced domain of fibronectin was significantly increased in SLE patients with vasculitis. Analysis of the functional epitopes on the alpha 4 chain demonstrated that antigen densities of all the functional epitopes were increased in those with vasculitis, indicating that the increased expression of VLA-4 resulted from the increased number of VLA-4 molecules, and was not secondary to an increase in one particular functional epitope. Immunoprecipitation studies further support these results. Interestingly, high molecular weight bands associated with VLA-4 were observed in about half of the SLE patients with vasculitis. These results introduce a possibility that upregulation of integrin adhesive receptors has a potential role in the pathogenesis of vasculitis in SLE. Images PMID:8254055

Takeuchi, T; Amano, K; Sekine, H; Koide, J; Abe, T

1993-01-01

70

Disulfite-induced acute intermittent urticaria with vasculitis.  

PubMed

Recognition of sulfite sensitivity by the practicing dermatologist has become increasingly important. A wide spectrum of anaphylactoid reactions can occur after ingestion of sulfite additives in foods and medications. We report the case of a 47-year-old man with severe acute intermittent urticaria. A placebo-controlled oral challenge test with 50 mg sodium disulfite resulted in an acute urticaria attack. A biopsy taken 5 h after the appearance of the urticaria demonstrated a leukocytoclastic vasculitis with eosinophilia. Avoiding foods and drugs containing sulfites is often difficult due to its widespread use. Therefore, the patient should be equipped with a medical emergency kit. PMID:8274792

Wüthrich, B; Kägi, M K; Hafner, J

1993-01-01

71

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, Jörg J.; Weyand, Cornelia M.

2014-01-01

72

Case of disseminated cutaneous Mycobacterium chelonae infection mimicking cutaneous vasculitis.  

PubMed

Mycobacterium chelonae is a non-tuberculous, rapidly growing mycobacteria and is widely distributed in the natural environment. In the immunocompetent status, localized cutaneous infections such as cellulitis and subcutaneous abscesses commonly occur after traumatic injury. However, disseminated cutaneous infections occur on a background of immunosuppression. Cutaneous M. chelonae infection presents with a variety of skin eruptions. We report a case of disseminated M. chelonae infection mimicking cutaneous vasculitis. The patient was treated with long-term oral corticosteroids and injected etanercept for the treatment of rheumatoid arthritis and asthma. Because the skin eruptions were preceded by asthma and rheumatoid arthritis and the pathological findings showed fibrinoid necrosis around the vascular of dermis, cutaneous vasculitis was first suspected. The culture from the pus revealed the bacterium which grew within 5 days on Ogawa's culture medium suggesting a rapidly growing mycobacteria. This bacterium was identified as M. chelonae by the DNA-DNA hybridization method. We chose 800 mg/day clarithromycin and 500 mg/day levofloxacin as a result of the drug-sensitivity test. After 6 months of the treatment, infection symptoms disappeared. Rapidly growing mycobacteria should be considered in the differential diagnosis of infections in patients under immunosuppression caused by diseases or drugs such as corticosteroids and biologic agents. Repeated bacterial examinations are important and required for the diagnosis of rapidly growing mycobacteria. PMID:24801916

Ichihara, Asako; Jinnin, Masatoshi; Fukushima, Satoshi; Inoue, Yuji; Ihn, Hironobu

2014-05-01

73

Role of ultrasound in the understanding and management of vasculitis  

PubMed Central

Vasculitis is characterized by a circumferential vessel-wall thickening (‘halo’), which can be visualized by modern imaging techniques. In particular, the resolution of ultrasound has increased to 0.1 mm. Ultrasound detects abnormalities that are pathognomonic even in arteries with a diameter below 1 mm. It is particularly helpful in the diagnosis of large-vessel vasculitides, such as classic temporal arteritis, large-vessel giant-cell arteritis (GCA), Takayasu arteritis and idiopathic aortitis. Echocardiography is important for determining cardiac involvement in Takayasu arteritis and also for examining the coronary arteries of children with suspected Kawasaki disease, which is a medium-vessel vasculitis. In small vessel vasculitides ultrasound has only a role for determining the distribution or organ involvement. Fast-track clinics for the diagnosis of GCA help to initiate treatment before complications such as blindness occur; patients receive appointments within 24 h in these clinics. Clinical examination and ultrasound of temporal and axillary arteries are performed by an experienced rheumatologist. In most cases this is able to determine if GCA is present. Temporal artery biopsy can be still carried out in ambivalent cases. The wall swelling of temporal arteries disappears after 2–3 weeks of glucocorticoid treatment. After 3 days of treatment, diagnosis becomes more difficult with ultrasound in some cases. In larger arteries, such as the axillary arteries, wall thickening disappears within months. It tends to be darker (more hypoechoic) in acute disease because of oedema. PMID:24688604

2014-01-01

74

Role of ultrasound in the understanding and management of vasculitis.  

PubMed

Vasculitis is characterized by a circumferential vessel-wall thickening ('halo'), which can be visualized by modern imaging techniques. In particular, the resolution of ultrasound has increased to 0.1 mm. Ultrasound detects abnormalities that are pathognomonic even in arteries with a diameter below 1 mm. It is particularly helpful in the diagnosis of large-vessel vasculitides, such as classic temporal arteritis, large-vessel giant-cell arteritis (GCA), Takayasu arteritis and idiopathic aortitis. Echocardiography is important for determining cardiac involvement in Takayasu arteritis and also for examining the coronary arteries of children with suspected Kawasaki disease, which is a medium-vessel vasculitis. In small vessel vasculitides ultrasound has only a role for determining the distribution or organ involvement. Fast-track clinics for the diagnosis of GCA help to initiate treatment before complications such as blindness occur; patients receive appointments within 24 h in these clinics. Clinical examination and ultrasound of temporal and axillary arteries are performed by an experienced rheumatologist. In most cases this is able to determine if GCA is present. Temporal artery biopsy can be still carried out in ambivalent cases. The wall swelling of temporal arteries disappears after 2-3 weeks of glucocorticoid treatment. After 3 days of treatment, diagnosis becomes more difficult with ultrasound in some cases. In larger arteries, such as the axillary arteries, wall thickening disappears within months. It tends to be darker (more hypoechoic) in acute disease because of oedema. PMID:24688604

Schmidt, Wolfgang A

2014-04-01

75

Urticarial vasculitis in northern Spain: clinical study of 21 cases.  

PubMed

Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1-78 yr; interquartile range, 5-54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged <20 yr), human immunodeficiency virus (HIV) infection (n = 1), and malignancy (n = 1). Besides urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2-38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis is generally good, but depends on the underlying disease. PMID:24378743

Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A; Alvarez, Lino; González-Vela, M Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A; Blanco, Ricardo

2014-01-01

76

Diffuse alveolar hemorrhage secondary to ANCA-associated vasculitis in a patient with Down syndrome.  

PubMed

Diffuse alveolar hemorrhage (DAH) is a rare disease characterized by dyspnea, cough, hemoptysis, and new alveolar infiltrates. Among the various underlying disorders, vasculitis is believed to play a significant role in the pathogenesis of DAH. Here we report the first case of a patient with Down syndrome who developed DAH secondary to anti-neutrophil cytoplasmic antibody-associated vasculitis. This case highlights the significance of vasculitis as well as pulmonary hypoplasia and vulnerability associated with Down syndrome in the development of DAH. PMID:25868961

Hori, Ikumi; Nakaseko, Haruna; Kamei, Michi; Nomura, Takayasu; Iwata, Naomi; Ito, Yasuhiko; Saitoh, Shinji

2015-04-01

77

Cutaneous and systemic vasculitis: cellular players and pathogenetic aspects.  

PubMed

Vascular wall damage, inflammatory cell recruitment and subsequent structural remodelling define a vasculitic process. Histopathological classification of vasculitis is based on the caliber of the vessel involved and on the prevalent type of inflammatory cells (neutrophils in acute forms, lymphocytic for chronic, histiocytic for granulomatous). A large amount of information is emerging from the literature on the complex pathophysiology of the cellular components of vessel wall. For instance, endothelial cells not only have the task to cover the inner surface of the vascular system but they also play an active role in tuning the immunological response in a very sophisticated way. Neutrophils are not only terminally differentiated cells sacrificed for a valuable cause. Cellular types of the perivascular microenvironment play roles one time not expected. The spread of the inflammatory process into the vascular wall is not necessarily inside-out. These and other selected concepts will be discussed in this paper. PMID:25502539

Cassisa, A

2015-02-01

78

A case report: Multaq-induced leukocytoclastic vasculitis.  

PubMed

A 71-year-old white male presented to the emergency department complaining of a worsening lower extremity rash. Thirteen days before presentation, the patient was placed on Multaq (dronedarone) for his paroxysmal atrial fibrillation. Biopsy-proven leukocytoclastic vasculitis (LV) was diagnosed, and causes for the condition other than drug-induced were investigated and ruled out. Rash has been cited as a possible side effect of Multaq; however, a literature search has revealed this to be the first documented case of likely multaq-induced LV. Other patients on Multaq therapy should be monitored for signs and symptoms of LV and be referred to an acute care setting as indicated. PMID:23011162

Smith, Suzanne M; Al-Bataineh, Mohammed; Iorfido, Stephen B; Macfarlane, Jarrod

2014-01-01

79

Cutaneous vasculitis and immune complexes in severe bronchiectasis.  

PubMed Central

Four patients with severe bronchiectasis (chronic bronchial suppuration) are described who developed cutaneous lesions associated with exacerbations of their respiratory disease. The skin abnormalities consisted of purpuric lesions in three patients and an erythematous vasculitis in one. Circulating immune complexes were present in all patients and in three skin biopsy specimens showed deposition of C3, IgG, and IgA in dermal blood vessels. Haemophilus influenzae had been isolated from the sputum of all four patients and in two patients was present at the time the cutaneous lesions appeared. It is suggested that local immune complex deposition was responsible for the skin lesions which occurred during acute exacerbations of bronchiectasis. Images PMID:6710426

Hilton, A M; Hasleton, P S; Bradlow, A; Leahy, B C; Cooper, K M; Moore, M

1984-01-01

80

Eosinophilic/T-cell Chorionic Vasculitis: Histological and Clinical Correlations.  

PubMed

Eosinophilic T-cell chorionic vasculitis (E/TCV) is composed of eosinophils and T-lymphocytes originating within chorionic vessels, radiating toward the intervillous space and away from the amnion in a fashion different from the fetal vascular response seen in amnionitis. Clinical significance and risk factors are not well established. We report four pregnancies (five infants, one triplet was spared) with E/TCV, gestational ranging from 23 weeks to term. All had concurrent acute chorioamnionitis, three had the typical acute fetal inflammatory response. One had placental fetal obstructive vasculopathy and an upper extremity reduction defect (radio-ulnar synostosis), the mother had pre-eclampsia. A second case involved 2 of 3 23 week previable triplets. Our third case had a metatarsus varus resistant to casting, the mother had gestational diabetes. The last case was a normal infant. We review the literature, discuss the clinical findings and present the histologic characteristics of this infrequently recognized lesion. PMID:25338020

Cheek, Bradley; Heinrich, Stephen; Ward, Kenneth; Craver, Randall

2014-10-22

81

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

82

A case of vasculitis, retinitis and macular neurosensory detachment presenting post typhoid fever  

PubMed Central

Background Ocular and extraocular immune-mediated phenomena are known to occur following febrile illness. Vasculitis, retinitis and neurosensory detachment are not well-recognized sequelae of typhoid fever. Findings We report a case of vasculitis, retinitis and macular neurosensory detachment presenting post typhoid fever. A 27-year-old female presented with decreased vision in right eye with history of typhoid fever (treated adequately 6 weeks prior). Her best corrected visual acuity in right eye was 20/125, N36. Fundus showed a patch of vasculitis and retinitis superior to the disc associated with macular neurosensory detachment and disc pallor. With oral steroids, the inflammation resolved and visual acuity improved to 20/20 at 6 weeks. Conclusions Immune-mediated vasculitis and retinitis following typhoid fever may respond well to systemic steroids. PMID:25246983

2014-01-01

83

Isolated non-necrotising granulomatous vasculitis of the gall bladder- a rare entity.  

PubMed

Gall bladder diseases are a significant cause of morbidity and mortality worldwide. Gall bladder diseases comprise a wide spectrum of disease entities including non specific inflammatory diseases, acute and chronic cholecystitis, follicular cholecystitis, granulomatous cholecystitis, metaplasic and dysplastic diseases of the gall bladder mucosa, gall bladder polyps and carcinomas. Here, we describe an unusual and a rare case of granulomatous vasculitis of the gall bladder incidentally diagnosed in a 38-year-old female, in a routine cholecystectomy specimen. Granulomatous vasculitis has been reported as a part of localised vasculitis of the gastrointestinal tract in the literature. The case is presented here for the rarity of the diagnosis of an isolated non-necrotising granulomatous vasculitis of the gall bladder. PMID:25478351

Prasaad, Priavadhana Rajan; Shekhar, Shubhranshu; Priyadharshini, S Anu

2014-10-01

84

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

85

Neutrophil priming and apoptosis in anti-neutrophil cytoplasmic autoantibody-associated vasculitis1  

Microsoft Academic Search

Neutrophil priming and apoptosis in anti-neutrophil cytoplasmic autoantibody-associated vasculitis.BackgroundInteractions between anti-neutrophil cytoplasmic autoantibody (ANCA) and primed neutrophils (PMNs) may be central to the pathogenesis of primary small vessel vasculitis. PMNs from patients are primed, expressing proteinase 3 (PR3) on the cell surface, which permits interaction with ANCA. In vitro ANCA activates primed PMN to degranulate and generate a respiratory burst.

Lorraine Harper; Paul Cockwell; Dwoma Adu; Caroline O. S. Savage

2001-01-01

86

Spontaneous massive bilateral peri-renal hemorrhage as a complication of ANCAnegative granulomatous vasculitis.  

PubMed

Spontaneous retroperitoneal hemorrhage (SRH) is a rare, life-threatening clinical entity most commonly associated with renal cell cancers. Systemic vasculitis has also been described as a rare cause of SRH. The current report describes a patient with acute kidney failure complicated by massive SRH, which occurred in the setting of anti-neutrophil cytoplasmic antibody (ANCA)-negative systemic necrotizing angiocentric granulomatous vasculitis involving multiple organs with minimal constitutional symptoms and no respiratory tract involvement. PMID:25707453

Vo, Hieu; Showkat, Arif; Chikkalingaiah, K Mandya; Nguyen, Cuong; Wall, Barry M

2015-04-01

87

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

88

Mast Cells Contribute to Peripheral Tolerance and Attenuate Autoimmune Vasculitis  

PubMed Central

Mast cells contribute to the modulation of the immune response, but their role in autoimmune renal disease is not well understood. Here, we induced autoimmunity resulting in focal necrotizing GN by immunizing wild-type or mast cell-deficient (KitW-sh/W-sh) mice with myeloperoxidase. Mast cell-deficient mice exhibited more antimyeloperoxidase CD4+ T cells, enhanced dermal delayed-type hypersensitivity responses to myeloperoxidase, and more severe focal necrotizing GN. Furthermore, the lymph nodes draining the sites of immunization had fewer Tregs and reduced production of IL-10 in mice lacking mast cells. Reconstituting these mice with mast cells significantly increased the numbers of Tregs in the lymph nodes and attenuated both autoimmunity and severity of disease. After immunization with myeloperoxidase, mast cells migrated from the skin to the lymph nodes to contact Tregs. In an ex vivo assay, mast cells enhanced Treg suppression through IL-10. Reconstitution of mast cell-deficient mice with IL-10–deficient mast cells led to enhanced autoimmunity to myeloperoxidase and greater disease severity compared with reconstitution with IL-10–intact mast cells. Taken together, these studies establish a role for mast cells in mediating peripheral tolerance to myeloperoxidase, protecting them from the development of focal necrotizing GN in ANCA-associated vasculitis. PMID:23138486

Gan, Poh-Yi; Summers, Shaun A.; Ooi, Joshua D.; O’Sullivan, Kim M.; Tan, Diana S.Y.; Muljadi, Ruth C.M.; Odobasic, Dragana; Kitching, A. Richard

2012-01-01

89

Immune Mechanisms in Medium and Large Vessel Vasculitis  

PubMed Central

Summary Vasculitis of the medium and large arteries, most often presenting as giant cell arteritis (GCA), is an infrequent, but potentially fatal type of immune-mediated vascular disease. The site of the aberrant immune reaction, the mural layers of the artery, is strictly defined by vascular dendritic cells, endothelial cells, vascular smooth muscle cells and fibroblasts which engage in an interaction with T cells and macrophages to ultimately cause luminal stenosis or aneurysmal wall damage of the vessel. A multitude of effector cytokines, all known as critical mediators in host-protective immunity, has been identified in the vasculitic lesions. Two dominant cytokine clusters, one centering on the IL-6/IL-17 axis, the other on the IL-12/IFN-? axis, have been connected with disease activity. These two clusters appear to serve different roles in the vasculitic process. The IL-6/IL-17 cluster is highly responsive to standard corticosteroid therapy, whereas the IL-12/IFN-? cluster is resistant to steroid-mediated immunosuppression. The information exchange between vascular and immune cells and stabilization of the vasculitic process involves members of the NOTCH receptor and ligand family. Focusing on elements in the tissue context of GCA, instead of broadly suppressing host immunity, may allow for a more tailored therapeutic approach and spare patients the unwanted side-effects of aggressive immunosuppression. PMID:24189842

Weyand, Cornelia M.; Goronzy, Jörg J.

2014-01-01

90

[Takayasu arteritis: a chronic vasculitis that is rare in children].  

PubMed

Takayasu arteritis was diagnosed in two children, a 15-year-old girl and a 10-year-old boy. The girl had suffered from fatigue, malaise, abdominal pain and weight loss for several months, but no clear cause could be found. A few weeks later, when a blood pressure of 222/155 mmHg was measured, the possibility of renal artery stenosis was considered and imaging studies revealed indications for Takayasu's disease. The patient was given methylprednisolone followed by a combination of prednisone and, initially, cyclophosphamide, later methotrexate. This resulted in a clinical remission of the inflammatory process. The boy presented with increasing fatigue and variable episodes of fever. After 3 years, sarcoidosis or Castleman's disease were considered. Imaging studies revealed aortic stenosis. He underwent stenting of the involved vessel segment. Takayasu arteritis is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. Especially in the early phase, the symptoms often are non-specific. One should look for hypertension, blood pressure differences between the two arms, decreased peripheral pulsation or bruits over the aorta and its major branches. Radiological examination may consist ofangiography, magnetic resonance imaging or CT-scans. Treatment consists of corticosteroids and other immunosuppressants, such as cyclophosphamide, methotrexate, azathioprine, and antagonists of tumour-necrosis factor alpha. In addition, balloon dilatation or stenting is often necessary. PMID:17152333

Greidanus, P M; Benninga, M A; Groothoff, J W; van Delden, O M; Davin, J C M A; Kuijpers, T W

2006-11-18

91

[Rare forms of hypertension : From pheochromocytoma to vasculitis].  

PubMed

Secondary hypertension affects only 5-10?% of hypertensive patients. Screening is expensive and time-consuming and should be performed only in patients for whom there is a high clinical suspicion of secondary hypertension. Clinical signs of secondary forms of hypertension are new-onset hypertension in patients without other risk factors (i.e., family history, obesity, etc.), sudden increase of blood pressure (BP) in a previously stable patient, increased BP in prepubertal children, resistant hypertension, and severe hypertension or hypertensive emergencies. In adults, renal parenchymal and vascular diseases as well as obstructive sleep apnea are the most common causes of secondary hypertension. Medication-induced hypertension and non-adherence to medication have to be ruled out. Of the endocrine causes associated with hypertension, primary aldosteronism is the most common. Other endocrine causes of hypertension such as thyroid disease (hypo- or hyperthyroidism), hypercortisolism (Cushing's syndrome), hyperparathyroidism, and pheochromocytoma are rare. Monogenetic forms of hypertension are mostly of tubular origin and associated with alterations in mineralocorticoid handling or signaling. Rare causes of hypertension also include inflammatory vascular disease. Acute forms of vasculitis may present as "malignant" hypertension with associated thrombotic microangiopathy and organ damage/failure. It is important to diagnose these rare forms of hypertension in order to prevent acute organ damage in these patients or unnecessary invasive treatment strategies. PMID:25700646

Haller, H; Limbourg, F; Schmidt, B M; Menne, J

2015-03-01

92

An Interferon-Induced Digital Vasculitis-Like Syndrome: A Case Report.  

PubMed

This report describes a patient with chronic hepatitis C undergoing therapy with interferon (IFN) alpha who developed bilateral ischemia of his fingers. We present a 43-year-old man with a failed renal transplant and chronic hepatitis C. He was treated with 6 months of IFN therapy with good reduction of his viral load. He presented with 2 days of pain and swelling in the second digits of both hands. Workup for extrahepatic manifestations of hepatitis C was initiated including assessment for vasculitis because of cryoglobulin- and noncryoglobulin-related causes. Extensive assessment with invasive and noninvasive vascular testing was performed. His workup for vasculitis did not reveal any specific reasons for the ischemic changes. Angiography of his fingers showed mild stenotic changes but no evidence of systemic vasculitis. IFN therapy was stopped and over several weeks his symptoms resolved. The ischemic changes were attributed to IFN therapy. The patient in this report is unique because although IFN has been historically reported to cause a variety of vascular syndromes, the reported experience in hepatitis C patients is small. In addition, the likelihood of encountering vasculitis and vasculitis-like syndromes in patients with hepatitis C is significant, and the increasing use of IFN in this population makes drug-induced vascular changes an essential consideration in this subset of patients. PMID:24732907

Hamidi, Oksana; Reiser, Jochen; Hasler, Scott

2014-04-11

93

Genome-wide association study of hepatitis C virus- and cryoglobulin-related vasculitis.  

PubMed

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 hepatitis C virus (HCV) RNA-positive individuals with cryoglobulin-related vasculitis and 447 ethnically matched, HCV RNA-positive controls. All cases had both serum cryoglobulins and 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. 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 (odds ratio=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)). 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 mixed cryoglobulinemia vasculitis in this extended major histocompatibility complex region. PMID:25030430

Zignego, A L; Wojcik, G L; Cacoub, P; Visentini, M; Casato, M; Mangia, A; Latanich, R; Charles, E D; Gragnani, L; Terrier, B; Piazzola, V; Dustin, L B; Khakoo, S I; Busch, M P; Lauer, G M; Kim, A Y; Alric, L; Thomas, D L; Duggal, P

2014-10-01

94

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

95

Decreased Neutrophil Apoptosis in Quiescent ANCA-Associated Systemic Vasculitis  

PubMed Central

Background ANCA-Associated Systemic Vasculitis (AASV) is characterized by leukocytoclasis, accumulation of unscavenged apoptotic and necrotic neutrophils in perivascular tissues. Dysregulation of neutrophil cell death may contribute directly to the pathogenesis of AASV. Methods Neutrophils from Healthy Blood Donors (HBD), patients with AASV most in complete remission, Polycythemia Vera (PV), Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA) and renal transplant recipients (TP) were incubated in vitro, and the rate of spontaneous apoptosis was measured by FACS. Plasma levels of cytokines and sFAS were measured with cytometric bead array and ELISA. Expression of pro/anti-apoptotic factors, transcription factors C/EBP-?, C/EBP-? and PU.1 and inhibitors of survival/JAK2-pathway were measured by real-time-PCR. Results AASV, PV and RA neutrophils had a significantly lower rate of apoptosis compared to HBD neutrophils (AASV 50±14% vs. HBD 64±11%, p<0.0001). In RA but not in AASV and PV, low apoptosis rate correlated with increased plasma levels of GM-CSF and high mRNA levels of anti-apoptotic factors Bcl-2A1 and Mcl-1. AASV patients had normal levels of G-CSF, GM-CSF and IL-3. Both C/EBP-?, C/EBP-? were significantly higher in neutrophils from AASV patients than HBD. Levels of sFAS were significantly higher in AASV compared to HBD. Conclusion Neutrophil apoptosis rates in vitro are decreased in AASV, RA and PV but mechanisms seem to differ. Increased mRNA levels of granulopoiesis-associated transcription factors and increased levels of sFAS in plasma were observed in AASV. Additional studies are required to define the mechanisms behind the decreased apoptosis rates, and possible connections with accumulation of dying neutrophils in regions of vascular lesions in AASV patients. PMID:22403660

Abdgawad, Mohamed; Pettersson, Åsa; Gunnarsson, Lena; Bengtsson, Anders A.; Geborek, Pierre; Nilsson, Lars; Segelmark, Mårten; Hellmark, Thomas

2012-01-01

96

Effects of decomplementation with cobra venom factor on experimental vasculitis.  

PubMed Central

Mercuric chloride (HgCl2) induces autoimmunity in susceptible rat strains, with hyper-IgE, appearance of a number of autoantibodies, and widespread tissue injury, including necrotizing vasculitis in the gut. In the early phase of tissue injury there is granulocyte infiltration; later there is immunoglobulin deposition along basement membranes in vessels. We have analysed the role of complement in this model using cobra venom factor (CVF), which causes decomplementation lasting around 5 days. The characteristic time course when HgCl2 is given over 10 days is that tissue injury and autoantibody levels reach a peak at around day 15 (start of HgCl2 = day 0). We therefore gave CVF either early (day 0), intermediate (day 5) or late (day 10); a fourth group (controls) received HgCl2 but no CVF. At each time point, CVF caused complete decomplementation which lasted for at least 5 days. Serum IgE and autoantibody levels were similar in all four experimental groups. Tissue injury in the 'early' CVF group and in the 'late' CVF group was not significantly different from controls, but in the intermediate group tissue injury was significantly more severe than in controls. These data indicate that the complement system does not play a major role in the induction of autoantibodies by HgCl2, nor in the effector phase of tissue injury. We speculate that the exacerbation of tissue injury by CVF in the group given this agent at an intermediate stage of the model is explained by the presence of products of C3 activation which have proinflammatory effects during the phase of active granulocyte-mediated tissue injury. PMID:8082302

Mathieson, P W; Qasim, F J; Thiru, S; Oldroyd, R G; Oliveira, D B

1994-01-01

97

Intravascular immunity as a key to systemic vasculitis: a work in progress, gaining momentum  

PubMed Central

Vascular inflammation contributes to the defence against invading microbes and to the repair of injured tissues. In most cases it resolves before becoming apparent. Vasculitis comprises heterogeneous clinical entities that are characterized by the persistence of vascular inflammation after it has served its homeostatic function. Most underlying mechanisms have so far remained elusive. Intravascular immunity refers to the surveillance of the vasculature by leucocytes that sense microbial or sterile threats to vessel integrity and initiate protective responses that entail most events that determine the clinical manifestations of vasculitis, such as end-organ ischaemia, neutrophil extracellular traps generation and thrombosis, leucocyte extravasation and degranulation. Understanding how the resolution of vascular inflammation goes awry in patients with systemic vasculitis will facilitate the identification of novel pharmacological targets and bring us a step closer in each patient to the selection of more effective and less toxic treatments. PMID:24128276

Ramirez, G A; Maugeri, N; Sabbadini, M G; Rovere-Querini, P; Manfredi, A A

2014-01-01

98

Failure of Recombinant Activated Factor VII in Treatment of Diffuse Alveolar Hemorrhage due to Cryoglobulinemic Vasculitis.  

PubMed

Diffuse alveolar hemorrhage (DAH) is a serious complication of the small vessel vasculitis syndromes and carries a high mortality. Recombinant activated factor VII (rFVIIa) is used to treat bleeding in patients with hemophilia and antibodies to factor VIII or IX. It is increasingly being used in life-threatening hemorrhage in a variety of other settings in which conventional therapy is unsuccessful. Randomized controlled trials of rFVIIa in DAH are lacking. However, several case reports have described a complete or sustained control of DAH using rFVIIa after patients failed to respond to medical treatment. There are no case reports in the literature describing the use or the failure of rFVIIa in DAH associated with cryoglobulinemic vasculitis. We here report the failure of rFVIIa to control DAH in a patient with CD5+ B-cell non-Hodgkin's lymphoma and cryoglobulinemic vasculitis. PMID:25114814

Khoulani, Dania; Rao, Bharat; Khanshour, Ammar; Kuriakose, Philip; Yessayan, Lenar

2014-01-01

99

Failure of Recombinant Activated Factor VII in Treatment of Diffuse Alveolar Hemorrhage due to Cryoglobulinemic Vasculitis  

PubMed Central

Diffuse alveolar hemorrhage (DAH) is a serious complication of the small vessel vasculitis syndromes and carries a high mortality. Recombinant activated factor VII (rFVIIa) is used to treat bleeding in patients with hemophilia and antibodies to factor VIII or IX. It is increasingly being used in life-threatening hemorrhage in a variety of other settings in which conventional therapy is unsuccessful. Randomized controlled trials of rFVIIa in DAH are lacking. However, several case reports have described a complete or sustained control of DAH using rFVIIa after patients failed to respond to medical treatment. There are no case reports in the literature describing the use or the failure of rFVIIa in DAH associated with cryoglobulinemic vasculitis. We here report the failure of rFVIIa to control DAH in a patient with CD5+ B-cell non-Hodgkin's lymphoma and cryoglobulinemic vasculitis. PMID:25114814

Khoulani, Dania; Khanshour, Ammar; Kuriakose, Philip

2014-01-01

100

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

101

Finger necrosis due to cryoglobulinemic vasculitis in association with membranous nephropathy.  

PubMed

Cryoglobulinemic vasculitis is a small vessel vasculitis that has been associated with chronic infections and autoimmune, lymphoproliferative, and neoplastic disorders. When no significant etiological factors are identified, it is called essential mixed cryoglobulinemia. A detailed and thorough laboratory investigation is required to exclude all possible causes of cryoglobulin formation. Although cryoglobulin testing is simple, careful temperature regulation is needed to avoid false-negative results. Consensus diagnosis should be developed and implemented for appropriate cryoglobulin detection and accurate clinical diagnosis for cryoglobulinemic vasculitis. Here we present an interesting, first-ever case report of a 54-year-old Hispanic-American woman with essential mixed cryoglobulinemia presenting with significant digital necrosis in association with membranous nephropathy. PMID:25552807

Porres-Aguilar, Mateo; Rodriguez-Castro, Carlos E; Osvaldo, Padilla; Saifuddin, Fátima; Siddiqui, Tariq; Fan, Jerry; Mukherjee, Debabrata; Pema, Kanchan; Abbas, Aamer

2015-01-01

102

Large Vessel Vasculitis Occurring in Rheumatoid Arthritis Patient under Anti-TNF Therapy  

PubMed Central

Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic) or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept). PMID:25544845

Cestelli, Valentina; Spinella, Amelia; Campomori, Federica; Esposito, Carmela; Ciaffi, Sara; Sandri, Gilda; Ferri, Clodoveo

2014-01-01

103

Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls  

PubMed Central

Cerebral vasculitis is a rare cause of juvenile stroke. It may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation in the setting of systemic vasculitis. Clinical hints for vasculitis are headache, stroke, seizures, encephalopathy and signs of a systemic inflammatory disorder. Diagnostic work-up includes anamnesis, whole body examination, laboratory and cerebral spinal fluid (CSF) studies, magnetic resonance imaging (MRI), angiography and brain biopsy. Due to the rarity of the disease, exclusion of more frequent differential diagnoses is a key element of diagnostic work-up. This review summarizes the steps that lead to the diagnosis of cerebral vasculitis and describes the red flags and pitfalls. Despite considering the dilemma of angiography-negative vasculitis and false-negative brain biopsy in some cases, it is important to protect patients from ‘blind’ immunosuppressive therapy in unrecognized non-inflammatory differential diagnosis. PMID:24117125

Berlit, P; Kraemer, M

2014-01-01

104

Vasculitis associated with all trans retinoic acid (ATRA) in a case with acute promyelocytic leukemia.  

PubMed

All trans retinoic acid is the drug of choice in the treatment of acute promyelocytic leukemia. But this drug has some side effects, some of which may be life-threatening. Retinoic acid syndrome is the most frequent and dangerous side effect of this differentiation inducing agent. Other side effects include Sweet's syndrome, vasculitis, hypercalcemia, bone marrow necrosis and fibrosis, thromboembolic events, erythema nodosum, granulomatous proliferation and some pulmonary complications. Here, we report vasculitis in a case with APL treated with ATRA and review the literature. PMID:12688331

Paydas, Semra; Yavuz, Sinan; Disel, Umut; Sahin, Berksoy; Canbolat, Tugba; Tuncer, Ilhan

2003-03-01

105

Recurrent erythema nodosum: a red flag sign of hidden systemic vasculitis  

PubMed Central

Takayasu's arteritis is a rare, systemic vasculitis with varied presentations across multiple medical specialities. Here, we present a young woman who had recurrent episodes of erythema nodosum on the background of a low-grade fever and no vascular manifestations. The presence of a high erythrocyte sedimentation rate generated a high index of suspicion for underlying vasculitis, and radioimaging confirmed the suspicion of Takayasu's arteritis. The patient was found to have type III diseases in the vasculitic stage and was managed with systemic corticosteroids. PMID:23576669

Gupta, Monica; Singh, Kamal; Lehl, SS; Bhalla, Mala

2013-01-01

106

Appearance of ANCA – associated vasculitis under Tumor necrosis factor-alpha inhibitors treatment  

PubMed Central

Summary Background: Tumor necrosis factor-alpha inhibitors treatment is accosiated with several side effects. The most common are injection side reactions, headache, nausea and infections. The more rare are development of systemic autoimmune diseases. Case Report: We describe two patients, who developed ANCA associated vasculitis during Tumor necrosis factor alpha inhibitors treatment. The diagnosis was confirmed by appropriate tissue picture, CT scan and laboratory findings. Conclusions: Our case series are unique, because vasculitis appeared after many years of the treatment and during complete patient’s remission of there main illness. PMID:23569570

Reitblat, Tatiana; Reitblat, Olga

2013-01-01

107

Anti-neutrophil cytoplasmic antibody-associated vasculitis associated with infectious mononucleosis due to primary Epstein–Barr virus infection: report of three cases  

PubMed Central

Although the aetiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis remains unclear, it is generally believed that environmental factors such as infections contribute to its development of ANCA-associated vasculitis. Prior Epstein–Barr virus (EBV) infection is reported to be a trigger of systemic vasculitis. We herein report three cases of ANCA-associated vasculitis presenting with infectious mononucleosis due to primary EBV infection. The causal link between the two pathologies could not be proved, but primary EBV infection may play a role in the initiation or exacerbation of ANCA-associated vasculitis. Future studies are necessary to determine the interaction between these diseases conditions.

Yamaguchi, Makoto; Yoshioka, Tomoki; Yamakawa, Taishi; Maeda, Matsuyoshi; Shimizu, Hideaki; Fujita, Yoshiro; Maruyama, Shoichi; Ito, Yasuhiko; Matsuo, Seiichi

2014-01-01

108

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...

109

IgG rheumatoid factors and anti-nuclear antibodies in rheumatoid vasculitis.  

PubMed Central

We studied the distribution and characteristics of circulating rheumatoid factors (RF) and anti-nuclear antibodies (ANA) in 30 rheumatoid arthritis (RA) patients who had polyarthritis alone (group I), 28 RA patients with polyarthritis and extra-articular disease (group II), 28 RA patients with systemic vasculitis (group III) and 60 healthy matched controls. IgG RF occurred more frequently and in higher serum titres in group III (100%) than RA patients in group I (40%), or in group II (18%) or in normal controls (5.8%). The serum titre of IgM RF was higher in vasculitis patients than in other RA patients. ANA were found in 74% of all RA patients and although the frequency did not differ in the three patient groups, the serum titre was significantly higher in the vasculitis group. Antibodies to extractable nuclear antigen were found only in group III (18.7%). Antibodies to histones were also more prevalent in group III than in the other RA groups. The serological abnormalities in rheumatoid vasculitis differed quantitatively as well as qualitatively from other RA patients. PMID:6602676

Quismorio, F P; Beardmore, T; Kaufman, R L; Mongan, E S

1983-01-01

110

Nuove acquisizioni in tema di terapia delle vasculiti ANCA-associate  

Microsoft Academic Search

The prognosis of untreated ANCA-associated systemic vasculitis (AASV) is poor with up to 90% of patients dying within 2 years. The combination of prednisone and cyclophosphamide is now established as the treatment of choice and leads to con- trol of the disease in 80-90% of the patients. Such treatment has turned these acutely fatal diseases into chronic relapsing disorders with

R. A. Sinico; E. Sabadini

111

[Central nervous system vasculitis according to the 2012 revise international chapel hill consensus conference nomenclature of vasculitides].  

PubMed

Vasculitis can be primarily or secondarily to various underlying diseases. It frequently affects the nervous system, and neurological deficits may remain even after disease remission. Because the progression of vasculitides is usually acute to subacute, early initiation of treatment is important from the viewpoint of patients' functional status. However, early diagnosis may be difficult, particularly in patients with central nervous system vasculitis. Hence, it is important to understand the wide-ranging clinical manifestations of vasculitides. Here, we summarize the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides from the standpoint of central nervous system vasculitis. PMID:25846438

Koike, Haruki; Sobue, Gen

2015-03-01

112

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

113

[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

114

[Acute renal failure and vasculitis - when to consider it and what to do about it?].  

PubMed

Renal involvement in systemic autoimmune diseases such as ANCA-vasculitis is common. Acute kidney injury (AKI) limits the prognosis of the patient and needs to be detected early. However, AKI is in early phases clinically inapparent and at first contact with the patient, extra-renal symptoms may dominate the clinical presentation. Serum-Creatinine and urinary analysis provide useful information on the extent of renal involvement. A renal biopsy should confirm the diagnosis. Treatment needs to be adapted to the extent of ANCA-vasculitis and in cases with severe organ failure plasmapheresis should be performed. Maintenance immunosuppressive is necessary to prevent relapses and the patients should be monitored closely to detect renal flares early. PMID:25704521

Wilde, Benjamin; Bienholz, Anja; Witzke, Oliver; Kribben, Andreas

2015-02-01

115

Reversal of rejection-induced coronary vasculitis detected early after heart transplantation with increased immunosuppression.  

PubMed

Four patients who underwent heart transplantation, in whom coronary obstruction was seen early after transplantation, are described. Repeated acute rejection episodes were detected within the first 2 months in each patient. Coronary obstruction or ischemia was shown through a combination of T1-201 isotopic study findings, evidence of vasculitis of a small coronary arteriole seen at endomyocardial biopsy, or coronary angiographic results. Vigorous treatment for rejection (antithymocyte globulin and bolus methylprednisolone) was given, and coronary artery lesions or myocardial ischemia resolved after treatment. Rejection-induced coronary obstruction should be considered in patients with repeated acute rejection episodes who are predisposed to the development of vascular rejection. Early after transplantation such obstruction is caused by diffuse vasculitis of small and medium-sized vessels and may be reversed with increased immunosuppression. PMID:2795284

Ballester, M; Obrador, D; Carrió, I; Bordes, R; Augé, J M; Crexells, C; Oriol, A; Padró, J M; Arís, A; Caralps, J M

1989-01-01

116

Acute respiratory failure as primary manifestation of antineutrophil cytoplasmic antibodies-associated vasculitis.  

PubMed

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. PMID:25332763

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

2014-06-18

117

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

PubMed

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

2010-06-01

118

Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis  

Microsoft Academic Search

Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. Anti-neutrophil cytoplasmic antibodies (ANCA) are widely used as diagnostic markers for Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and idiopathic rapidly progressive glomerulonephritis (iRPGN). The objective of this study was to evaluate the diagnostic value of ANCA measurement by the indirect immunofluorescence (IIF) test, and

E Christiaan Hagen; Mohamed R Daha; Jo Hermans; Konrad Andrassy; Elena Csernok; Gillian Gaskin; Phillippe Lesavre; Jens Lüdemann; Niels Rasmussen; R Alberto Sinico; Allan Wiik; Fokko J van der Woude

1998-01-01

119

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

120

A second case of Marburg's variant of multiple sclerosis with vasculitis and extensive demyelination.  

PubMed

Marburg's variant of multiple sclerosis is a rapidly progressive and malignant form of multiple sclerosis (MS) that usually leads to severe disability or death within weeks to months without remission. Few cases have been described in the literature since the original description by Marburg. The classic pathological findings usually include highly destructive zones of extensive demyelination, necrosis with dense cellular infiltrate, and giant reactive astrocytes. We report a case of a 31-year-old woman with Marburg's variant of MS who, over a period of eight months, became totally disabled, blind, and quadriplegic, with vocal cord paralysis, requiring a tracheostomy. The patient underwent diagnostic stereotactic brain biopsy. Clinical findings, magnetic resonance imaging (MRI), serologic and cerebrospinal fluid (CSF) findings, and neuropathology are discussed. MRI showed extensive white matter involvement in the brain and spinal cord that continuously progressed over time. A diagnostic stereotactic brain biopsy revealed extensive active demyelination with unexpected finding of active vasculitis and fibrinoid necrosis with a vascular inflammatory cell infiltrate, including polymorphonuclear neutrophils and rare eosinophils. Serologic work-up for vasculitis and neuromyelitis optica was unremarkable and the CSF showed only one oligoclonal band (OCB) not present in serum. This is the second case of Marburg's variant of MS that demonstrated both demyelination and vasculitis. In our case these features were demonstrated simultaneously, even though the demyelination was the predominant pathological finding. Since vasculitis is not a feature of classic MS, these findings pose the question as to whether Marburg's variant of MS is a true variant or different entity altogether. PMID:21816761

Elenein, Rania G A; Sharer, Leroy R; Cook, Stuart D; Pachner, Andrew R; Michaels, Jennifer; Hillen, Machteld E

2011-12-01

121

Radiation Retinopathy Caused by Low Dose Irradiation and Antithyroid Drug-Induced Systemic Vasculitis  

Microsoft Academic Search

Background: We report on a patient with Graves’ disease with radiation retinopathy caused by lowdose irradiation and antithyroid drug-induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. Case: A 38-year-old woman with Graves’ disease presented with bilateral blurred vision, microaneurysms, telangiectasia, and macular edema. The patient was examined by ophthalmoscopy and fluorescein angiography, and radiation retinopathy was diagnosed. Observations: The patient had been

Koh-Hei Sonoda; Masahiro Yamamoto; Tatsuro Ishibashi

2005-01-01

122

Contrasting genetic association of IL2RA with SLE and ANCA – associated vasculitis  

Microsoft Academic Search

BACKGROUND: Autoimmune diseases are complex and have genetic and environmental susceptibility factors. The objective was to test the genetic association of systemic lupus erythematosus (SLE) and anti-neutrophil cytoplasmic antibody (ANCA) – associated systemic vasculitis (AAV) with SNPs in the IL2RA region and to correlate genotype with serum levels of IL-2RA. METHODS: Using a cohort of over 700 AAV patients, two

Edward J Carr; Menna R Clatworthy; Christopher E Lowe; John A Todd; Andrew Wong; Timothy J Vyse; Lavanya Kamesh; Richard A Watts; Paul A Lyons; Kenneth GC Smith

2009-01-01

123

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°C 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

124

Immunomodulation with eicosapentaenoic acid supports the treatment of autoimmune small-vessel vasculitis  

PubMed Central

Small-vessel vasculitis is a life-threatening autoimmune disease that is frequently associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Conventional immunotherapy including steroids and cyclophosphamide can cause serious adverse events, limiting the efficacy and safety of treatment. Eicosapentaenoic acid (EPA), a key component of fish oil, is an omega-3 polyunsaturated fatty acid widely known to be cardioprotective and beneficial for vascular function. We report two elderly patients with systemic ANCA-associated vasculitis (AAV) in whom the administration of EPA in concert with steroids safely induced and maintained remission, without the use of additioal immunosuppressants. To explore the mechanisms by which EPA enhances the treatment of AAV, we employed SCG/Kj mice as a spontaneous murine model of AAV. Dietary enrichment with EPA significantly delayed the onset of crescentic glomerulonephritis and prolonged the overall survival. EPA-derived anti-inflammatory lipid mediators and their precursors were present in the kidney, plasma, spleen, and lungs in the EPA-treated mice. Furthermore, a decrease in ANCA production and CD4/CD8-double negative T cells, and an increase in Foxp3+ regulatory T cells in the lymph nodes of the kidney were observed in the EPA-treated mice. These clinical and experimental observations suggest that EPA can safely support and augment conventional therapy for treating autoimmune small-vessel vasculitis. PMID:25230773

Hirahashi, Junichi; Kawahata, Kimito; Arita, Makoto; Iwamoto, Ryo; Hishikawa, Keiichi; Honda, Mie; Hamasaki, Yoshifumi; Tanaka, Mototsugu; Okubo, Koshu; Kurosawa, Miho; Takase, Osamu; Nakakuki, Masanori; Saiga, Kan; Suzuki, Kazuo; Kawachi, Shoji; Tojo, Akihiro; Seki, George; Marumo, Takeshi; Hayashi, Matsuhiko; Fujita, Toshiro

2014-01-01

125

A role for mast cells in the development of adjuvant-induced vasculitis and arthritis.  

PubMed Central

The objective of this study was to characterize the role of mast cells in the development of vasculitis and joint swelling in adjuvant-immunized rats. Leukocyte trafficking within mesenteric venules (rolling and adhesion) and mast cell activation (ruthenium red uptake) were examined in vivo. Elevated leukocyte trafficking was observed by 4 days after immunization, whereas joint swelling developed between days 10 and 12. Perivascular mast cells took up ruthenium red and appeared activated by electron microscopy at 4 but not 12 days after immunization. Treatment with the mast cell stabilizer cromolyn on days 1 to 4 after immunization blocked ruthenium red uptake at day 4 and reduced leukocyte rolling and adhesion by approximately 50%. This treatment also reduced rolling, adhesion, and joint swelling at day 12 by approximately 50%. Cromolyn treatment over days 9 to 12 reduced joint swelling but increased leukocyte emigration into the mesentery. Peritoneal mast cells isolated 4 days after immunization elicited significant neutrophil chemotaxis in vitro, whereas day 12 mast cells did not. Mast cell activation and vasculitis were absent in adjuvant-resistant Fisher/344 rats. These data suggest that mast cells play an early role in the initiation of vasculitis and may function by day 12 to limit infiltration of leukocytes from the vasculature. In the joint, however, mast cells appear to contribute to inflammation at early as well as later time points. Images Figure 2 PMID:9466582

Johnston, B.; Burns, A. R.; Kubes, P.

1998-01-01

126

Systemic lupus erythematosus associated with ANCA-associated vasculitis: an overlapping syndrome?  

PubMed

Systemic lupus erythematosus (SLE) and small-sized vessel vasculitis are usually two distinguishable autoimmune diseases. However, a vasculitis may be found in the course SLE but rarely corresponds to an ANCA-associated vasculitis (AAV). We report four cases of de novo SLE associated with AAV, our aim being to discuss the clinical significance of this association. We included four patients fulfilling the criteria for both SLE and AAV and followed in two different university hospitals between 1996 and 2009. In light of a 20-year literature review (25 described clinical cases), we discussed the etiopathogeny of such an association. All patients presented a severe renal involvement (creatininemia ranging from 120 to 370 ?mol/l) and thrombopenia (ranging from 45,000 to 137,000 platelets/mm(3)). The other main clinical symptoms were arthritis (n = 3), serositis (n = 2) and intra-alveolar hemorrhage (n = 2). An inflammatory syndrome was noticed at diagnosis in all cases. ANCAs were MPO-ANCAs in all cases. Two out of these four patients were also diagnosed with antiphospholipid syndrome. The frequency of this association seems not fortuitous. Although the etiopathogenic mechanisms of such an association remain to be more precisely described, several clinical, histological and immunological features support the hypothesis of the existence of a SLE-AAV overlapping syndrome. Moreover, clinicians must be aware of such an overlapping syndrome, notably because its initial presentation can be very severe. PMID:21805174

Hervier, B; Hamidou, M; Haroche, J; Durant, C; Mathian, A; Amoura, Z

2012-10-01

127

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

128

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

129

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. PMID:25349763

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

2014-01-01

130

Septic vasculitis and vasculopathy in some infectious emergencies: the perspective of the histopathologist.  

PubMed

Sepsis is a potentially life-threatening complication of an infection where cutaneous lesions often represent one of the early signs. A myriad of microorganisms including bacteria, fungi, yeasts, viruses, protozoas, helminths and algae can be implicated. A broad spectrum of clinical and histopathologic findings can be observed in the skin and the common denominator is a thrombotic vasculopathy. The pathogenesis of cutaneous septic vasculitis (SV)/vasculopathy is complex and includes five main mechanisms: disseminated intravascular coagulation, direct invasion and occlusion of blood vessel walls by microorganisms, hypersensitivity reaction with immune complex deposition into blood vessel walls, embolism from a distant infectious site and vascular effects of toxins. Herein we describe the clinicopathologic findings of some selected cases of SV recently observed in our hospital, including purpura fulminans, necrotizing fasciitis, cutaneous meningococcemia, malignant syphilis and disseminated alternaria infection. Histopathologically, a wide spectrum of histopathologic changes was observed in skin specimens from the various entities, involving the intensity and composition of the inflammatory infiltrate, the degree of vascular changes and the presence of microorganisms, that ranged from a predominant not inflammatory, thrombotic-occlusive vasculopathy in purpura fulminans to leukocytoclastic vasculitis like changes in cutaneous meningococcemia to a dermal angiomatosis-like pattern in disseminated Alternaria infection. The different pathologic presentations may be related to the microorganism involved, the main pathogenetic mechanism that induced the vascular injury and the individual immunologic burden. Early skin biopsy for histopathologic examination and microbiologic culture is a cornerstone in the diagnosis of life-threatening diseases that present with cutaneous septic vasculitis. Ancillary techniques, such as immunohistochemistry and polymerase chain reaction are additional novel and helpful tools to identify pathogens, leading to definite diagnosis in cases with challenging or ambiguous clinical and/or pathologic findings. PMID:25592669

Tomasini, C

2015-02-01

131

Mycobacterium tuberculosis Infection Is Associated with the Development of Erythema Nodosum and Nodular Vasculitis  

PubMed Central

Background Mycobacterium tuberculosis (MTB) infection has been suggested to contribute to the pathogenesis of erythema nodosum (EN) and nodular vasculitis (NV), the classic forms of panniculitis. However, there is little evidence to demonstrate the presence of MTB in the skin lesions. This study is aimed at evaluating the association between MTB infection and the development of EN and NV in a Chinese population. Methods A total of 107 patients (36 EN, 27 NV, and 44 others) with vasculitis and 40 control cases with other skin diseases were recruited and their skin lesion samples were subjected to real time polymerase chain reaction (PCR) analysis of the IS6110 and mpt64 gene fragments of MTB. Their blood mononuclear cells were tested for MTB antigen-specific IFN-? responses by QuantiFERON®-TB Gold In-Tube (IT) assays. Results PCR analysis revealed that 7/23 (30.4%) and 7/18 (38.9%) of the EN and NV samples were positive for the IS6110 DNA, respectively, which were significantly higher than 3/34 (8.8%) of other vasculitis (OV) and 3/40 (7.5%) of the control samples (p<0.05). The nested Real-Time PCR assay indicated that 6/7 (86%) of the IS6110-positive EN samples, all of the IS6110-positive NV and control samples, but only 1/3 of the IS6110-positive OV samples, were positive for the mpt64 gene. Similarly, 19/32 (59.4%) of the EN patients, 20/26 (76.9%) of the NV patients, and 17/36 (47.2%) of the OV patients were positive for MTB antigen-specific IFN-? responses, which were significantly higher than 6/40 (15%) of the controls (p<0.05). Conclusion Our data strongly suggest that MTB infection and active TB are associated with the development of NV and EN in Chinese. PMID:23650522

Chen, Lianjun; Zhang, Qiao’an; Luo, Xiaoqun; Zhang, Wenhong

2013-01-01

132

Transformation from tubulointerstitial nephritis to crescentic glomerulonephritis: an unusual presentation of ANCA-associated renal vasculitis.  

PubMed

A 44-year-old man with acute renal failure and antineutrophil cytoplasmic antibodies (ANCA) positivity was described. The first renal biopsy specimen showed tubulointerstitial nephritis (TIN) with normal glomeruli. However, delayed recovery of renal function with low-dose steroid treatment for TIN prompted a second renal biopsy 1 month later; and the specimen demonstrated a dramatically different morphology, with necrotizing and crescentic glomerulonephritis. Improvement in renal function occurred, together with reduction of ANCA titers, following intensive immunosuppressive therapy. This case illustrates an unusual presentation of TIN in ANCA-associated renal vasculitis. The possible pathogenetic mechanism are discussed. PMID:16538980

Wen, Yao-Ko; Chen, Mei-Ling

2006-01-01

133

Generalized alopecia with vasculitis-like changes in a dog with babesiosis.  

PubMed

A locally bred, 12-year-old, intact female Satsuma dog presented with generalized alopecia. Erythema, crusts and desquamation were observed primarily on the truck. Papules and erosions were present in the pinnae, and there were multiple areas of skin necrosis on the right forelimb. The cutaneous lesions had not responded to treatment with systemic antibiotics and prednisolone. The dog also had progressive anemia. Babesia gibsoni was detected in the blood, and the dog was treated with antiprotozoal agents. The skin lesions and anemia improved, but relapsed after the treatment was discontinued. Histopathological examination of skin biopsies revealed findings suggestive of early leukocytoclastic vasculitis or ischemic vasculopathy. PMID:23676299

Tasaki, Yumi; Miura, Naoki; Iyori, Keita; Nishifuji, Koji; Endo, Yasuyuki; Momoi, Yasuyuki

2013-10-01

134

Therapy insight: The recognition and treatment of retinal manifestations of systemic vasculitis.  

PubMed

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, Churg-Strauss syndrome, relapsing polychondritis and systemic lupus erythematosus. Less frequently occurring vasculitides include Takayasu's arteritis, Goodpasture's disease, microscopic polyangiitis and Henoch-Schönlein purpura, as well as vasculitis secondary to scleroderma and rheumatoid arthritis. This article describes the pathogenesis, clinical features and treatment of retinal manifestations of systemic vasculitides. PMID:16932736

Aristodemou, Petros; Stanford, Miles

2006-08-01

135

Generalized Alopecia with Vasculitis-Like Changes in a Dog with Babesiosis  

PubMed Central

ABSTRACT A locally bred, 12-year-old, intact female Satsuma dog presented with generalized alopecia. Erythema, crusts and desquamation were observed primarily on the truck. Papules and erosions were present in the pinnae, and there were multiple areas of skin necrosis on the right forelimb. The cutaneous lesions had not responded to treatment with systemic antibiotics and prednisolone. The dog also had progressive anemia. Babesia gibsoni was detected in the blood, and the dog was treated with antiprotozoal agents. The skin lesions and anemia improved, but relapsed after the treatment was discontinued. Histopathological examination of skin biopsies revealed findings suggestive of early leukocytoclastic vasculitis or ischemic vasculopathy. PMID:23676299

TASAKI, Yumi; MIURA, Naoki; IYORI, Keita; NISHIFUJI, Koji; ENDO, Yasuyuki; MOMOI, Yasuyuki

2013-01-01

136

Vasculitis and long standing ankylosing spondylitis in a patient with familial Mediterranean fever  

PubMed Central

Coexistence of familial Mediterranean fever (FMF) and other inflammatory disorders has been frequently reported, but no specific underlying factor has been identified. We report a patient with FMF who is presented with long-standing ankylosing spondylitis (AS) and cutaneous leukocytoklastic vasculitis (LV) of the lower limbs. It is the first report on combination of FMF with AS and LV. The Mediterranean Fever (MEFV) gene mutation of heterozygote (R202Q/R726A) and HLA-B27 are detected in this case, and are believed to form genetic susceptibility to LV. PMID:25538787

Taylan, Ali; Yildiz, Yasar; Sari, Ismail; Ozkok, Guliz

2014-01-01

137

ANCA-positive vasculitis associated with simvastatin/ezetimibe: expanding the spectrum of statin-induced autoimmunity?  

PubMed

Although autoimmune syndromes such as systemic lupus erythematosus and dermatomyositis have been previously reported in association with statin use, vasculitis has not been well described. We present a patient with an antineutrophil cytoplasmic antibody-positive, predominantly cutaneous vasculitis, the temporal course of which was associated with simvastatin/ezetimibe use. The patient's serologic findings were consistent with drug-induced disease, with high titer antimyeloperoxidase, in addition to antinuclear and anti-Ro (SSA) antibodies. The patient demonstrated complete resolution of symptoms simply by withdrawing the drug. PMID:20704607

Sen, Deepali; Rosenstein, Elliot D; Kramer, Neil

2010-08-01

138

Vasculitis secondary to presumptive leptospirosis treated with long-term corticosteroids in a captive lesser kudu (Tragelaphus imberbis australis).  

PubMed

A 2-yr-old female lesser kudu (Tragelaphus imberbis australis) was presented for lethargy. Empirical antibiotic treatment appeared to improve its clinical signs, although no etiology for the symptoms was determined. The kudu again presented with lethargy, diffusely swollen limbs, and subcutaneous ecchymoses of 1 day's duration after completion of the initial therapy. Vasculitis secondary to presumptive leptospirosis infection was diagnosed based on a skin biopsy and decreasing paired serologic titers for Leptospira grippotyphosa. The vasculitis was responsive to intramuscular antibiotic therapy and dexamethasone treatment. This case provides evidence that corticosteroids can be used in ruminants at moderate doses for chronic treatment without clinically relevant detrimental effects. PMID:20945659

Fogelberg, Katherine; Ferrell, Shannon T

2010-09-01

139

Varicella zoster-associated retinal and central nervous system vasculitis in a patient with multiple sclerosis treated with natalizumab  

PubMed Central

We report the first case of combined retinal and CNS varicella zoster-associated vasculitis in a 49-year-old patient with multiple sclerosis who had been treated with natalizumab. He presented with a progressive bilateral visual loss. The diagnosis of a vasculitis was based on the fundoscopic examination and MRI findings. We confirmed the varicella zoster virus (VZV) infection of the CNS by PCR and increased intrathecal antibody indices in the cerebrospinal fluid. The patient was stabilized with antiviral treatment, methylprednisolone, plasmapheresis and cycophosphamide. Natalizumab was discontinued. This case illustrates the neuroimmunological and neuroinfectiological consequences of treatments with biologicals that influence the immune system. PMID:24479415

2014-01-01

140

Vasculitis-like hemorrhagic retinal angiopathy in Wegener’s granulomatosis  

PubMed Central

Background Granulomatosis with polyangiitis, also known as Wegener’s granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener’s granulomatosis). Case presentation A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener’s granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy. Conclusion Vasculitis-like retinal changes can occur in Wegener’s granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms. PMID:24021028

2013-01-01

141

Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis.  

PubMed

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA) positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this newly proposed concept of OMAAV. PMID:25339433

Yoshida, Naohiro; Iino, Yukiko

2014-12-01

142

Immune complex glomerulonephritis and dermal vasculitis following intestinal bypass for morbid obesity.  

PubMed

A kidney and skin biopsy were performed on a patient who developed cryoglobulinemia, polyarthritis, a purpuric skin rash, and acute renal failure four years following jejunoileal bypass for morbid obesity. Morphologic studies revealed a diffuse glomerulonephritis characterized by the presence of numerous subendothelial deposits containing IgG, IgA, C3, Clq, C4, and properdin, and an acute dermal vasculitis associated with similar immune complex deposits. Identical immunoglobulin and complement components were present in the cryoglobulin. In addition, both the cryoglobulin and a renal biopsy eluate containing anti-IgG antibody and antibody against Klebsiella pneumoniae which were present in the patient's stool in large numbers. Combined therapy with steroids and chloramphenicol resulted in marked improvement in the patient's arthritis, skin rash, and renal function. The findings indicate that glomerulonephritis and dermal vasculitis due to the deposition of bacterial antigen-antibacterial antibody complexes may occur as part of a systemic immune complex disease complicating small intestinal bypass. PMID:7041613

Gamble, C N; Kimchi, A; Depner, T A; Christensen, D

1982-03-01

143

The role of IgM rheumatoid factor in experimental immune vasculitis.  

PubMed Central

The effect of IgM rhematoid factor (RF) on reversepassive cutaneous Arthus reaction in rats was studied. The RF was obtained from the serum cryoglobulin of a patient with symptoms of purpura, arthralgia and digital gangrene. The cryoglobulins was of IgG-IgM type and when given i.v it induced a prompt hypocomplementaemia in experimental animals. The purified RF also induced low serum complement levels when injected i.v. along with complexes of non-complement-fixing, aggregated IgG. A reverse passive Arthus reaction was induced by intradermal injection of IgG anti-bovine serum albumin (BSA), followed by an i.v. dose of antigen (Ag). The cutaneous inflammatory reaction was aggravated by simultaneous administration of IgM RF intradermally, but not by IgM without antibody (Ab) properties. Intradermal injection of low concentrations of non-complement-fixing IgG anti-BSA, along with normal human IgM, followed by i.v. injection of BSA, resulted in a complete lack of cutaneous inflammation. At higher Ab concentrations there was only a mild inflammation. However, when IgM RF was substituted for normal IgM and injected with non-complement-fixing anti-BSA, an effective reverse passive cutaneous Arthus reaction and vasculitis was induced. The inflammatory response was greatly suppressed by decomplementation of animals by cobra venom factor. This study provides evidence favouring an inflammatory, complement-dependent role for RF in vasculitis. PMID:157238

Floyd, M; Tesar, J T

1979-01-01

144

Hypersensitivity Vasculitis  

MedlinePLUS

... with ongoing infections, there may be ongoing or ‘chronic’ symptoms of HV. Drugs used to manage the skin rash and joint pains associated with HV might include corticosteroids and/or nonsteroidal anti-inflammatory drugs. In patients with more severe or ongoing ...

145

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; Knütter, Ilka; Hiemann, Rico; Röber, 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

146

Lack of association of alpha-1 antichymotrypsin gene polymorphism with PR3-ANCA and MPO-ANCA associated vasculitis.  

PubMed

In patients with PR3-ANCA associated vasculitides the carrier frequency of alpha1-antitrypsin (AAT) deficiency allele PI*Z is increased and linkage disequilibrium between polymorphic markers within a cluster of serine protease inhibitor (serpin) genes, including AAT gene, at chromosome 14q32.1 has been described. A1-antichymotrypsin (AACT), part of an extended serpin gene cluster, was discussed to contribute to PR3-ANCA associated vasculitis formation. To analyse, if an AACT gene polymorphism within the signal peptide sequence is associatedwith antineutrophil cytoplasm autoantibodies (ANCA) vasculitis allelic frequencies of AACT polymorphism were analysed in 128 control persons, 79 PR3-ANCA, and 30 MPO-ANCA patients. In MPO-ANCA patients also phenotyping of AAT was performed as well as frequency and linkage analysis of simple tandem repeat polymorphisms in the genes of cortisol-binding globulin, AAT, protein C inhibitor, and three extragenic markers (S48, S55, S51) of the gene cluster. Allelic frequencies of the AACT polymorphism did not differ between controls and vasculitis patients. In the MPO-ANCA group no patient expressed the Pi*Z defective allele of AAT and the allelic frequencies of polymorphic markers within the serpin gene cluster did not differ from those of the controls. Strong linkage disequilibrium was detected in MPO-ANCA patients neither. Therefore, we can not support the hypothesis that AACT polymorphism contributes to the pathogenesis of PR3-ANCA vasculitis. Nor is it probable that any factor, coded by the serpin gene cluster, contributes to MPO-ANCA vasculitis. PMID:12685871

Borgmann, Stefan; Haubitz, Marion; Schwab, Sibylle G

2002-11-01

147

PF-1355, a Mechanism-Based Myeloperoxidase Inhibitor, Prevents Immune Complex Vasculitis and Anti-Glomerular Basement Membrane Glomerulonephritis.  

PubMed

Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences. MPO contributes to vascular injury by 1) catabolizing nitric oxide, impairing vasomotor function; 2) causing oxidative damage to lipoproteins and endothelial cells, leading to atherosclerosis; and 3) stimulating formation of neutrophil extracellular traps, resulting in vessel occlusion and thrombosis. Here we report a selective 2-thiouracil mechanism-based MPO inhibitor (PF-1355 [2-(6-(2,5-dimethoxyphenyl)-4-oxo-2-thioxo-3,4-dihydropyrimidin-1(2H)-yl)acetamide) and demonstrate that MPO is a critical mediator of vasculitis in mouse disease models. A pharmacokinetic/pharmacodynamic response model of PF-1355 exposure in relation with MPO activity was derived from mouse peritonitis. The contribution of MPO activity to vasculitis was then examined in an immune complex model of pulmonary disease. Oral administration of PF-1355 reduced plasma MPO activity, vascular edema, neutrophil recruitment, and elevated circulating cytokines. In a model of anti-glomerular basement membrane disease, formerly known as Goodpasture disease, albuminuria and chronic renal dysfunction were completely suppressed by PF-1355 treatment. This study shows that MPO activity is critical in driving immune complex vasculitis and provides confidence in testing the hypothesis that MPO inhibition will provide benefit in treating human vasculitic diseases. PMID:25698787

Zheng, Wei; Warner, Roscoe; Ruggeri, Roger; Su, Chunyan; Cortes, Christian; Skoura, Athanasia; Ward, Jessica; Ahn, Kay; Kalgutkar, Amit; Sun, Dexue; Maurer, Tristan S; Bonin, Paul D; Okerberg, Carlin; Bobrowski, Walter; Kawabe, Thomas; Zhang, Yanwei; Coskran, Timothy; Bell, Sammy; Kapoor, Bhupesh; Johnson, Kent; Buckbinder, Leonard

2015-05-01

148

Successful rechallenge with reduced dose of erlotinib in a patient with lung adenocarcinoma who developed erlotinib-associated leukocytoclastic vasculitis: A case report  

PubMed Central

The oral tyrosine kinase inhibitors of epidermal growth factor, erlotinib and gefitinib, are active in the treatment of non-small cell lung cancer (NSCLC). However, a number of skin manifestations have been found in patients receiving erlotinib therapy. Leukocytoclastic vasculitis is a rare side-effect of erlotinib therapy. However, whether or not erlotinib treatment should be continued when disseminated ulceration of leukocytoclastic vasculitis is encountered remains to be determined. In this study, we report a patient with NSCLC who remains responsive to erlotinib treatment following successful rechallenge with a reduced dose of erlonitib after presenting with severe degree of leukoclastic vasculitis. PMID:22783433

SU, BO-AN; SHEN, WAN-LIN; CHANG, SHENG-TSUNG; FENG, LI-YIA; WU, CHIA-JUNG; FENG, YIN-HSUN

2012-01-01

149

Non-infectious cryoglobulinemia vasculitis (CryoVas): update on clinical and therapeutic approach.  

PubMed

CryoVas is a small vessel vasculitis associated with the presence of circulating cryoglobulins. In the absence of HCV infection, several disorders have been identified in association of CryoVas. Although evidence is limited, a few studies have recently described the clinical presentation, prognosis, and therapeutic management of non-infectious CryoVas. Patients with type I CryoVas, especially associated with hematologic malignancies, have shown a worse clinical presentation. Recent studies have also identified prognostic factors in mixed CryoVas. Therapeutic management in non-infectious CryoVas remains to be defined. Overall, treatment options should be individualized based on severity of involvement. In this setting, new data have emerged regarding the role of biologic therapy in non-infectious CryoVas. Off-label use of rituximab should be highlighted, based on the assessment of benefits and risks, especially infections. PMID:24647999

Perez-Alamino, Rodolfo; Espinoza, Luis R

2014-05-01

150

[Goodpasture syndrome associated with p-ANCA microscopic vasculitis: a rare entity to recognize].  

PubMed

Some autoimmune diseases may be found presenting simultaneously antibodies (Abs) against basal membrane and anti-neutrophil cytoplasm (ANCA). The clinical picture is that of the Goodpasture syndrome with anti-basement membrane Abs associated with an ANCA-associated small-vessel vasculitis (micro-PAN). The arisen of these two pathological entities is nevertheless too frequent to be the fruit of the only fate. The pathophysiological hypothesis remains that of the micro-PAN initially creates lesions of the basement membrane facilitating the formation of Abs against some constituents of this latter. The prognosis remains controversial, leaving open this issue. This article aims to present a recent literature revue dealing with the simultaneous presence of these two auto-immune diseases. PMID:19626934

Pralong, G; Fournier, C; Dayer, E; Meier, P

2009-06-10

151

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

152

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis.  

PubMed

The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p?

Vera-Lastra, Olga; Sepúlveda-Delgado, Jesús; Cruz-Domínguez, María Del Pilar; Medina, Gabriela; Casarrubias-Ramírez, Moisés; Molina-Carrión, Luis E; Pineda-Galindo, Luis F; Olvera-Acevedo, Arturo; Hernández-Gonzalez, Claudia; Jara, Luis J

2015-04-01

153

Infliximab leads to a rapid but transient improvement in endothelial function in patients with primary systemic vasculitis  

PubMed Central

Objective To assess the immediate effects of tumour necrosis factor ? (TNF?) blockade on endothelial function in systemic vasculitis. Methods Endothelial function was assessed by laser Doppler flowmetry in patients with active vasculitis after 10 infusions of infliximab. For comparison endothelial responses were assessed after five infusions of cyclophosphamide plus methylprednisolone. Results Endothelial dependent vasodilatation (EDV) improved significantly within 24?hours of infliximab infusion. The median change in red blood cell flux (interquartile range) was 5.7 (4.3–8.2) before infusion v 8.4 (7.5–10.9) at 24?hours; p?=?0.027. This was not maintained at day 14. No improvement was seen in EDV after cyclophosphamide plus methylprednisolone infusion. Conclusion The rapid but transient improvement in EDV after TNF? inhibition suggests that TNF? may have a direct role in the impairment of endothelial function. PMID:16361277

Raza, K; Carruthers, D M; Stevens, R; Filer, A D; Townend, J N; Bacon, P A

2006-01-01

154

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

155

A case of necrotizing vasculitis with panniculitis, during sorafenib treatment for hepatocellular carcinoma, appeared in disease progression  

PubMed Central

Hepatocellular carcinoma (HCC) is the sixth most common cancer worldwide and is the third most common cause of death from cancer. Sorafenib is the only drug which improves survival in first line advanced HCC. Sorafenib has been associated with several dermatologic toxicities and toxic effects have been related to a better treatment response. We report the case of a well-circumscribed panniculitis and necrotizing vasculitis due to sorafenib, appeared in disease progression in a man affected by advanced HCC. PMID:25436135

Ragazzi, Moira; Asensio, Nuria Maria; Pagano, Maria; Gnoni, Roberta; Boni, Corrado

2014-01-01

156

Long-Term Maintenance Therapy Using Rituximab-Induced Continuous B-Cell Depletion in Patients with ANCA Vasculitis  

PubMed Central

Background and objectives Remission in the majority of ANCA vasculitis patients is not sustained after a single course of rituximab, and risk of relapse warrants development of a successful strategy to ensure durable remission. Design, setting, participants, & measurements A retrospective analysis of ANCA vasculitis patients who underwent maintenance therapy using rituximab-induced continuous B-cell depletion for up to 7 years was performed. Maintenance therapy with rituximab was initiated after achieving remission or converting from other prior maintenance therapy. Continuous B-cell depletion was achieved in all patients by scheduled rituximab administration every 4 months. Disease activity, serologic parameters, adverse events, and survival were examined. Results In the study, 172 patients (mean age=60 years, 55% women, 57% myeloperoxidase–ANCA) treated from April of 2006 to March of 2013 underwent continuous B-cell depletion with rituximab. Median remission maintenance follow-up time was 2.1 years. Complete remission (Birmingham Vasculitis Activity Score [BVAS]=0) was achieved in all patients. Major relapse (BVAS?3) occurred in 5% of patients and was associated with weaning of other immunosuppression drugs. Remission was reinduced in all patients. Survival mirrored survival of a general age-, sex-, and ethnicity-matched United States population. Conclusion This analysis provides evidence for long-term disease control using continuous B-cell depletion. This treatment strategy in ANCA vasculitis patients also seems to result in survival rates comparable with rates in a matched reference population. These findings suggest that prospective remission maintenance treatment trials using continuous B-cell depletion are warranted. PMID:24626432

Pendergraft, William F.; Cortazar, Frank B.; Wenger, Julia; Murphy, Andrew P.; Rhee, Eugene P.; Laliberte, Karen A.; Niles, John L.

2014-01-01

157

Renal cell carcinoma presenting as Henoch–Schönlein purpura with leukocytoclastic vasculitis, hematuria, proteinuria and abdominal pain  

Microsoft Academic Search

Some infectious, rheumatic, allergic diseases, and malignancies have been associated with leucocytoclastic vasculitis (LCV).\\u000a LCV and cancer occur most frequently in patients with hematological malignancies such as lymphomas and leukemias. There have\\u000a been a few prior cases reported of LCV associated with renal cell carcinoma (RCC). A 25-year-old male patient was referred\\u000a from the department of dermatology and nephrology because

Young Hoon Hong

2010-01-01

158

Acute dilated cardiomyopathy in a patient with beriberi and cryoglobulinaemic vasculitis: an unusual potential complication of two rare disorders.  

PubMed

We report the case of a 45-year-old patient who presented with acute dilated cardiomyopathy. During admission the patient was consecutively diagnosed with cryoglobulinaemic vasculitis and beriberi. In both diseases, cardiac involvement may occur as dilated cardiomyopathy. Thiamin deficiency was the final cause for the severe cardiac manifestations (cardiac acute beriberi or Shoshin syndrome), which returned to normal after thiamin supplementation. PMID:24429381

Tejedor, Ana; Solé, Manel; Prieto-González, Sergio; Alba, Marco Antonio; Grau, Josep Maria; Cid, Maria Cinta; Hernández-Rodríguez, José

2014-01-01

159

Cutaneous leukocytoclastic vasculitis due to Salmonella enteritidis in a child with interleukin-12 receptor beta-1 deficiency.  

PubMed

Defects in the interleukin 12 (IL-12)/interferon gamma (IFN-?) pathway result in Mendelian susceptibility to mycobacterial disease (MSMD). IL-12 receptor beta 1 (IL-12R?1) deficiency, the most common form of MSMD, is associated with weakly virulent mycobacteria and salmonella. Infections in patients with this deficiency are extraintestinal, or septicemic, recurrent infections with nontyphoid salmonellae. Here we report a case of an IL-12R?1 deficiency with cutaneous leukocytoclastic vasculitis due to Salmonella enteritidis. PMID:23004925

Filiz, Serkan; Kocacik Uygun, Dilara F; Verhard, Els M; van Dissel, Jaap T; Uygun, Vedat; Bassorgun, Cumhur; Bingol, Aysen; Yegin, Olcay; van de Vosse, Esther

2014-01-01

160

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

161

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis  

PubMed Central

Two hepatotropic viruses have been shown to have causal relationship with systemic vasculitis-hepatitis B with classical polyarteritis nodosa and hepatitis C with cryoglobulinemic vasculitis. The present paper provides an updated overview on the clinical presentations and management of these vasculitides. HBV associated PAN patients have higher weight loss, peripheral neuropathy, mononeuritis multiplex, abdominal pain, gastrointestinal manifestations requiring surgery, cardiomyopathy, orchitis, hypertension, and/or elevated transaminase levels. Microaneurysms are also more common in mesenteric artery. Skin manifestations, however are less common. These patients also have a severe disease as suggested by higher five factor score and higher BVAS. Though relapses are less common, mortality is higher in patients with HBV PAN as compared to non HBV PAN. Plasmapheresis has a role in treatment in clearing off immune complexes. The common clinical manifestations of HCV associated cryoglobulinemic vasculitis are skin lesions, peripheral neuropathy, glomerulonephritis, arthritis, and sicca symptoms. Though combination therapy comprising of pegylated interferon ? and ribavirin is the first line of management, immunotherapy is needed for severe or life threatening manifestations. Recent randomized trials have shown the efficacy of rituximab in such situations.

Sharma, Aman; Sharma, Kusum

2013-01-01

162

Mycobacterium avium-intracellulare pulmonary infection complicated by cutaneous leukocytoclastic vasculitis in a woman with anorexia nervosa.  

PubMed

A 53-year-old Caucasian woman with a history of anorexia nervosa developed a bilateral lower extremity rash comprised of palpable red to violaceous, sub-centimeter papular lesions that increased in quantity rapidly. She also noted a 2-month history of non-productive cough. Imaging modalities revealed a thin-walled cavitary lesion in the right lung apex and scattered nodular opacities. Acid fast bacilli (AFB) were found in sputum and subsequently identified by culture as Mycobacterium avium-intracellulare (MAI). Punch biopsies of her skin lesions yielded a histological diagnosis of small-to-medium vessel vasculitis. Stains and cultures for organisms were negative. Her skin lesions resolved quickly after the initiation of antimicrobial therapy for MAI. Hypersensitivity vasculitis associated with an atypical mycobacterial infection is unusual. The postulated underlying mechanism is the deposit of immune complexes and not the bacillus itself. While cutaneous leukocytoclastic vasculitis (CLV) due to MAI is certainly a rare entity, it should be entertained in patients with vasculitic skin lesions and a concomitant pulmonary disease. PMID:24363210

Walsh, T L; Baca, V; Stalling, S S; Natalie, A A; Veldkamp, P J

2014-06-01

163

Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?  

PubMed

Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease. PMID:25516870

File, Ibolya; Trinn, Csilla; Mátyus, Zsolt; Ujhelyi, László; Balla, József; Mátyus, János

2014-12-16

164

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

165

Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?  

PubMed Central

Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease. PMID:25516870

File, Ibolya; Trinn, Csilla; Mátyus, Zsolt; Ujhelyi, László; Balla, József; Mátyus, János

2014-01-01

166

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. PMID:25452756

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

2014-01-01

167

[A case with juvenile rheumatoid arthritis who developed cerebral vasculitis and venovascular hypertension].  

PubMed

On November, 1997, a 15-year-old boy visited our hospital because of headache, fever and arthralgia. He was treated with 5 mg/day of prednisolone thereafter. On October 21, 1998, he was admitted because of remittent fever and multiple arthralgia and diagnosis of juvenile rheumatoid arthritis (JRA) was made. He was also found to have hypertension of 210/110 mmHg, and soon developed ptosis of the eye, facial paresis and perceptive deafness of the right side. Cerebrospinal fluid showed protein of 98 mg/dl and mildly elevated IgG, IgA and IgM levels with normal cell count. Brain MRI examination revealed multiple cerebral lesions in the frontal, parietal and cerebellar areas on the right, whose cause was thought to be vasculitis. Renal angiography demonstrated a right renal artery stenosis, compatible with renovascular hypertension. He was treated with 60 mg of prednisolone per day, which brought about a satisfactory improvement of the above rheumatic and neurologic signs. On November 17, 1998, he received a follow-up study of MRI, which failed to show any cerebral lesions, supporting the effectiveness of prednisolone. An angiotensin converting enzyme inhibitor successfully normalized hypertension and renin activity in serum, although renal blood flow did not increase. PMID:11215160

Yamamoto, T; Abe, T; Mastuya, M; Kobayashi, K; Akaike, J; Ikeda, Y; Hosokawa, A; Shimoji, H; Sakai, H; Yonezawa, K; Tosaka, M; Imai, K; Matsumoto, H

2000-10-01

168

Leukocytoclastic vasculitis masking chronic vascular changes in previously undiagnosed erythropoietic protoporphyria.  

PubMed

A 31-year old man presented with swelling and purpura of the dorsum of the hands following sun exposure. There was a preceding lifelong history of photosensitivity, but this episode, which occurred after the recent commencement of oral iron therapy, and after recent alcohol ingestion, was much more severe than any preceding episode. Skin biopsy performed 48 h after the onset of symptoms showed features consistent with the early stages of leukocytoclastic vasculitis. Direct immunofluorescence showed homogeneous thick staining of the vessel walls with IgG, IgM and IgA, together with abundant perivascular fibrinogen. A subsequent periodic acid-Schiff (PAS) stain on the skin biopsy revealed thickening of the walls of dermal vessels, which was not discernible in routinely stained (hematoxylin/eosin) sections. The diagnosis of erythropoietic protoporphyria (EPP) was confirmed by significantly elevated erythrocyte protoporphyrin levels and positive plasma fluorimetry. The diagnosis of porphyria may have been missed by routine skin microscopy if not for the additional information provided by clinical history, direct immunofluorescence and PAS stain. The pathogenesis and histopathology of acute and chronic vascular changes in EPP are reviewed. PMID:24102632

Thom, Graham; Lam, Minh; Lucas, Michaela; Rossi, Enrico

2013-11-01

169

Clinical diagnosis and management of large vessel vasculitis: giant cell arteritis.  

PubMed

Large vessel vasculitis (LVV) covers a spectrum of primary vasculitides predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA) and Takayasu arteritis (TA). Less commonly LVV occurs in various other diseases. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Occasionally LVV is discovered unexpectedly on pathological examination of a resected aortic aneurysm. Clinical evaluation is often unreliable in determining disease activity. Moreover, the diagnostic tools are imperfect. Acute phase reactants can be normal at presentation and available imaging modalities are more reliable in delineating vascular anatomy than in providing reliable information on degree of vascular inflammation. Glucocorticoids are the mainstay of therapy of LVV. Patients may develop predictable adverse effects from long-term glucocorticoid use. Several steroid-sparing agents have also shown some promise and are currently in use. Endovascular revascularization procedures and open surgical treatment for aneurysms and dissections are sometimes necessary, but results are not always favorable and relapses are common. This article, the first in a series of two, will be devoted to GCA and isolated (idiopathic) aortitis, while TA will be covered in detail in the next article. PMID:24893935

Chatterjee, Soumya; Flamm, Scott D; Tan, Carmela D; Rodriguez, E Rene

2014-07-01

170

Acute abdominal pain in systemic lupus erythematosus: focus on lupus enteritis (gastrointestinal vasculitis)  

PubMed Central

Methods: A retrospective study was carried out for all patients admitted with SLE from 1993 to March 2001. The SLEDAI and laboratory data were collected at the time of diagnosis of SLE and at the time of acute abdominal pain. Lupus enteritis (gastrointestinal vasculitis) was diagnosed by clinical investigation and abdominal computed tomographic findings. Results: Chart review identified 175 patients (20 male, 155 female) who had been admitted with SLE. Of these patients, 38 (22%) presented with acute abdominal pain. Lupus enteritis was the most common cause of acute abdominal pain. Patients were divided into three groups: group 1: lupus enteritis (n=17), group 2: acute abdominal pain without lupus enteritis (n=21), and group 3: SLE without acute abdominal pain (n=137). There was no difference in age and sex among the three groups. Antiphospholipid, anti-RNP, anti-Sm, anti-Ro, and anti-La antibodies did not differ among the three groups. There was no difference in the SLEDAI at the time of diagnosis and at the time of acute abdominal pain between groups 1 and 2. Complement, erythrocyte sedimentation rate, C reactive protein, and anti-dsDNA measured at the time of acute abdominal pain did not differ between groups 1 and 2. A drop in the white blood cell count at the time of abdominal pain was more prominent in group 1 than group 2. In lupus enteritis, the jejunum and ileum were the sites most commonly affected. Rectal involvement was rare. Even though four patients relapsed, all the patients with lupus enteritis, including those who relapsed, responded well to corticosteroid. Conclusion: Lupus enteritis is the most common cause of acute abdominal pain in SLE. All patients with lupus enteritis responded well to a high dose of a corticosteroid without surgical intervention. The SLEDAI and laboratory data, except leucopenia, do not correlate with the occurrence of lupus enteritis. PMID:12006332

Lee, C; Ahn, M; Lee, E; Shin, J; Cho, Y; Ha, H; Yoo, B; Moon, H

2002-01-01

171

ANCA Associated Vasculitis and Renal Failure Related to Propylthiouracil and Hyperthyroidism Induced Cholestasis in the Same Case  

PubMed Central

Introduction. Liver involvement due to hyperthyroidism and also ANCA positive vasculitis related renal failure cases were reported separately several times before. However, to our knowledge, these two complications together in the same case had never been observed before. Case Presentation. The case of an ANCA positive 71-year-old Caucasian male with renal failure and lung involvement, subclinical hyperthyroidism, and intrahepatic cholestatic jaundice was presented in this paper. After exclusion of all of the other possibilities, cholestatic hepatitis was explained by subclinical hyperthyroidism; renal failure and lung involvement were interpreted as ANCA related vasculitis which might be a side effect of propylthiouracil use. Conclusion. The coexistence of these rare conditions in the same patient deserves emphasis and it is worth reporting. This case demonstrates that following the clinical course of the patient is essential after prescribing any medications to see whether any complication occurs or not. If the complications of this case were noticed earlier, it would be possible to treat and to prevent the permanent damages. PMID:25506446

Tuncay, Mehmet; Kivrakoglu, Emine; Yegenaga, Itir; Dervisoglu, Erkan

2014-01-01

172

Successful outcome of pregnancy in patients with anti-neutrophil cytoplasm antibody-associated small vessel vasculitis.  

PubMed

Pregnancy in patients with anti-neutrophil cytoplasm antibody-associated vasculitis is reportedly associated with a high risk of fetal and maternal complications. Here we describe the outcome of pregnancies in patients with granulomatosis with polyangiitis and microscopic polyangiitis at five centers in the United Kingdom using a retrospective case review of all women who became pregnant following diagnosis. We report 15 pregnancies in 13 women resulting in 15 live births including one twin pregnancy and 13 singleton pregnancies. One patient had an unplanned pregnancy and a first trimester miscarriage while taking methotrexate. All other pregnancies were planned following a minimum of 6 months clinical remission. Eleven successful pregnancies were delivered vaginally at full term, whereas three were delivered by cesarean section. All infants were healthy with no neonatal complications on their initial health check within the first 24?h of delivery and no evidence of neonatal vasculitis. One relapse occurred during pregnancy and was successfully treated with an increased dose of azathioprine and corticosteroids, intravenous immunoglobulin, and plasma exchange therapy. One patient developed tracheal crusting and subglottic stenosis of infective etiology in the third trimester requiring tracheal debridement post delivery. No patient had a relapse in the first 12 months postpartum. Thus, successful pregnancy outcomes can occur following planned pregnancy in women in sustained remission on non-teratogenic therapies. PMID:25272233

Croft, Adam P; Smith, Stuart W; Carr, Sue; Youssouf, Sajeda; Salama, Alan D; Burns, Aine; Pusey, Charles D; Hamilton, Patrick; Brown, Nina; Venning, Michael; Harper, Lorraine; Morgan, Matthew D

2015-04-01

173

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

174

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

Technology Transfer Automated Retrieval System (TEKTRAN)

Sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2), a '-herpesvirus, is an often fatal disease characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison (Bison bison), cattle (Bos taurus), and other clinically susceptible speci...

175

Urinary Levels of High Mobility Group Box-1 Are Associated with Disease Activity in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis  

PubMed Central

Background High mobility group box-1 (HMGB1), a kind of pro-inflammatory mediator, is associated with inflammatory conditions and tissue damage. Our previous study demonstrated that the circulating levels of HMGB1 correlated with disease activity of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). In the current study, we aimed to measure urinary levels of HMGB1 in AAV patients, correlated them to clinical activity index and analysed the immunohistochemical HMGB1 staining in kidney specimens. Methods 50 patients with AAV in active stage and 56 patients with AAV in remission were recruited. The urinary levels of HMGB1 were determined by enzyme-linked immunosorbent assay. Moreover, renal biopsy specimens from 27 patients with active AAV were randomly collected to evaluate the deposition of HMGB1. Results Urinary HMGB1 levels in AAV patients in active stage were significantly higher than those in AAV patients in remission and healthy controls (1.46 [0.56-3.43] versus 0.38 [0.10-1.35] mg/?molCr, P=0.001; 1.46 [0.56-3.43] versus 0.48 [0.40-0.60] mg/?molCr, P=0.000, respectively). Further analysis found that urinary levels of HMGB1 correlated with erythrocyte sedimentation rate (r=0.354, p=0.012), C-reactive protein (r=0.289, p=0.042), and Birmingham Vasculitis Activity Score (r=0.350, p=0.013). Renal tissue of active AAV patients showed HMGB1 was mainly expressed in the cytoplasm and the extracellular space. The percentage of HMGB1-negative nuclei in renal tissue of patients with active AAV was significantly higher than that in normal controls (60.6±20.2 % versus 2.7±0.6 %, p<0.01). Conclusion Urinary levels of HMGB1 may be associated with the disease activity in AAV patients. PMID:25884225

Wang, Chen; Chang, Dong-Yuan; Zhao, Ming-Hui; Chen, Min

2015-01-01

176

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

177

Cutaneous adverse effects of hormonal adjuvant therapy for breast cancer: a case of localised urticarial vasculitis following anastrozole therapy and a review of the literature.  

PubMed

Hormonal therapy with either tamoxifen or aromatase inhibitors is commonly used to treat women with breast cancer in both the adjuvant and recurrent disease setting. Cutaneous adverse reactions to these drugs have been rarely reported in the literature. We report an unusual case of urticarial vasculitis following the aromatase inhibitor anastrozole that localised to the unilateral trunk and mastectomy scar, and review the literature on the cutaneous adverse effects of hormonal therapy for breast cancer. PMID:24575835

Bock, Vanessa L; Friedlander, Michael; Waring, Dale; Kossard, Steven; Wood, Glenda K

2014-11-01

178

Tissue microscopic changes and artifacts in multi-phase post-mortem computed tomography angiography in a hospital setting: a fatal case of systemic vasculitis.  

PubMed

A 27-year-old man suddenly died in hospital of acute respiratory distress syndrome secondary to severe systemic vasculitis. Multi-phase post-mortem computed tomography angiography followed by scientific autopsy of the thoracic and abdominal cavity and histology was performed, illustrating the advantages and drawbacks of such techniques. Imaging enabled us to examine the cranium, as the family refused cerebral dissection. MPMCTA revealed absence of opacification of the left middle cerebral artery. But parenchymal findings of thoracic and abdominal organs were still difficult to interpret after both imaging and macroscopic examination during the autopsy. Microscopic examination provided the definitive diagnosis of cause of death. Analysis revealed systemic vasculitis of the lung complicated by diffuse alveolar, mediastinal, splenic and retroperitoneal lesions. We were unable to determine the type of vasculitis, whether polyarteritis nodosa or microscopic polyangiitis, because of artifactual glomerular collapse. We observed some structural changes in tissue secondary to contrast agent injection, affecting the vascular system and renal parenchyma in particular. Such artifacts must be known in order to avoid misinterpreting them as pathological findings. MPMCTA and conventional autopsy are two complementary techniques showing both their specific advantages and limits which have to be known in order to choose the appropriate technique. One limit of both techniques is the detection of microscopic findings which can only be obtained by additional histological examination. This case report underlines this fact and demonstrates that caution is required in some cases if microscopic analyses are carried out after contrast agent injection. PMID:25085763

Capuani, Caroline; Guilbeau-Frugier, Céline; Mokrane, Fatima-Zohra; Delisle, Marie-Bernadette; Marcheix, Bertrand; Rousseau, Hervé; Telmon, Norbert; Rougé, Daniel; Dedouit, Fabrice

2014-09-01

179

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

180

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

181

Anti-idiotypes against anti-neutrophil cytoplasmic antigen autoantibodies in normal human polyspecific IgG for therapeutic use and in the remission sera of patients with systemic vasculitis.  

PubMed Central

Anti-neutrophil cytoplasmic antigen (ANCA) activity was inhibited in 15 out of 21 sera from patients with acute systemic vasculitis following incubation with normal polyspecific IgG for therapeutic use (IVIg). ANCA antibodies reacted with IVIg through idiotypic-anti-idiotypic interactions, as shown in competitive binding assays using F(ab')2 fragments from IVIg and affinity chromatography of ANCA IgG on Sepharose-bound F(ab')2 fragments from IVIg. Co-incubation of sera from patients with acute systemic vasculitis with paired autologous remission stage sera also resulted in inhibition of ANCA activity in acute sera. Remission sera contain IgM and IgG capable of interacting with beta and or alpha idiotypes of ANCA IgG from acute sera. Anti-idiotypic IgM may account for the lack of expression of ANCA activity in whole serum from patients in remission from systemic vasculitis, which were found to contain high titres of ANCA IgG. These observations suggest that remission of systemic vasculitis is associated with the generation of anti-idiotypes against autoantibodies rather than the suppression of production of ANCA autoantibodies. IVIg may modulate the activity of systemic vasculitis in vivo. PMID:1993362

Rossi, F; Jayne, D R; Lockwood, C M; Kazatchkine, M D

1991-01-01

182

Establishment of a vascular endothelial cell-reactive type II NKT cell clone from a rat model of autoimmune vasculitis.  

PubMed

We previously generated a rat model that spontaneously developed small vessel vasculitis (SVV). In this study, a T cell clone reactive with rat vascular endothelial cells (REC) was established and named VASC-1. Intravenous injection of VASC-1 induced SVV in normal recipients. VASC-1 was a TCR??/CD3-positive CD4/CD8 double-negative T cell clone with expression of NKG2D. The cytokine mRNA profile under unstimulated condition was positive for IL-4 and IFN-? but negative for IL-2 and IL-10. After interaction with REC, the mRNA expression of IL-2, IL-5 and IL-6 was induced in VASC-1, which was inhibited by blocking of CD1d on the REC surface. Although the protein levels of these cytokines seemed to be lower than the detection limit in the culture medium, IFN-? was detectable. The production of IFN-? from the VASC-1 stimulated with LPS-pre-treated REC was inhibited by the CD1d blockade on the REC. These findings indicated VASC-1 as an NKT cell clone. The NKT cell pool includes two major subsets, namely types I and II. Type I NKT cells are characterized by expression of semi-invariant TCRs and the potential to bind to marine sponge-derived ?-galactosylceramide (?-GalCer) loaded on CD1d; whereas, type II NKT cells do not manifest these characteristics. VASC-1 exhibited a usage of TCR other than the type I invariant TCR ? chain and did not bind to ?-GalCer-loaded CD1d; therefore, it was determined as a type II NKT cell clone. The collective evidence suggested that REC-reactive type II NKT cells could be involved in the pathogenesis of SVV in rats. PMID:25239132

Iinuma, Chihiro; Waki, Masashi; Kawakami, Ai; Yamaguchi, Madoka; Tomaru, Utano; Sasaki, Naomi; Masuda, Sakiko; Matsui, Yuki; Iwasaki, Sari; Baba, Tomohisa; Kasahara, Masanori; Yoshiki, Takashi; Paletta, Daniel; Herrmann, Thomas; Ishizu, Akihiro

2015-02-01

183

Clozapine-Induced Late Agranulocytosis and Severe Neutropenia Complicated with Streptococcus pneumonia, Venous Thromboembolism, and Allergic Vasculitis in Treatment-Resistant Female Psychosis.  

PubMed

Clozapine is a second-generation antipsychotic agent from the benzodiazepine group indicated for treatment-resistant schizophrenia and other psychotic conditions. Using clozapine earlier on once a case appears to be refractory limits both social and personal morbidity of chronic psychosis. However treatment with second-generation antipsychotics is often complicated by adverse effects. We present a case of a 33-year-old Caucasian woman with a 25-year history of refractory psychotic mania after switching to a 2-year clozapine therapy. She presented clozapine-induced absolute neutropenia, agranulocytosis, which were complicated by Streptococcus pneumonia and sepsis. Clozapine-induced thromboembolism of the common femoral and right proximal iliac vein, as well as allergic vasculitis, was diagnosed. She achieved full remission on granulocyte-colony stimulating factor and specific antibiotic treatment. Early detection of severe clozapine-induced absolute neutropenia and agranulocytosis enabled the effective treatment of two among its most severe complications. Additional evidence to the previously reported possible causal relation between clozapine and venous thromboembolism is offered. Finally, clozapine-induced allergic vasculitis is confirmed as a late adverse effect of clozapine therapy. PMID:25755670

Voulgari, Christina; Giannas, Raphael; Paterakis, Georgios; Kanellou, Anna; Anagnostopoulos, Nikolaos; Pagoni, Stamata

2015-01-01

184

Clozapine-Induced Late Agranulocytosis and Severe Neutropenia Complicated with Streptococcus pneumonia, Venous Thromboembolism, and Allergic Vasculitis in Treatment-Resistant Female Psychosis  

PubMed Central

Clozapine is a second-generation antipsychotic agent from the benzodiazepine group indicated for treatment-resistant schizophrenia and other psychotic conditions. Using clozapine earlier on once a case appears to be refractory limits both social and personal morbidity of chronic psychosis. However treatment with second-generation antipsychotics is often complicated by adverse effects. We present a case of a 33-year-old Caucasian woman with a 25-year history of refractory psychotic mania after switching to a 2-year clozapine therapy. She presented clozapine-induced absolute neutropenia, agranulocytosis, which were complicated by Streptococcus pneumonia and sepsis. Clozapine-induced thromboembolism of the common femoral and right proximal iliac vein, as well as allergic vasculitis, was diagnosed. She achieved full remission on granulocyte-colony stimulating factor and specific antibiotic treatment. Early detection of severe clozapine-induced absolute neutropenia and agranulocytosis enabled the effective treatment of two among its most severe complications. Additional evidence to the previously reported possible causal relation between clozapine and venous thromboembolism is offered. Finally, clozapine-induced allergic vasculitis is confirmed as a late adverse effect of clozapine therapy. PMID:25755670

Voulgari, Christina; Giannas, Raphael; Paterakis, Georgios; Kanellou, Anna; Anagnostopoulos, Nikolaos; Pagoni, Stamata

2015-01-01

185

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

186

Large vessel vasculitis  

Microsoft Academic Search

Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during\\u000a the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological\\u000a and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children.\\u000a The recent definition

Ashima Gulati; Arvind Bagga

2010-01-01

187

How Is Vasculitis Diagnosed?  

MedlinePLUS

... blood vessel with a color image of the blood flow through that vein or artery. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) identifies areas that show higher glucose metabolism leading to problems in the blood vessels. Angiography ...

188

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

189

C-antineutrophil cytoplasmic antibody positivity in vasculitis patients is associated with the Z allele of alpha-1-antitrypsin, and P-antineutrophil cytoplasmic antibody positivity with the S allele  

Microsoft Academic Search

Background. Antineutrophil cytoplasmic antibodies (ANCA) in vasculitis have either cANCA or pANCA patterns as defined by immunofluorescence. The target autoantigen of cANCA is usually proteinase 3 (PR3), whereas that of pANCA is usually myeloperoxidase (MPO). Alpha-1-antitrypsin (alAT) is the major physiological inhibitor of PR3, while MPO is an inhib- itor of a 1 AT. Methods. To determine whether there was

M. E. Griffith; J. U. Lovegrove; G. Gaskin; D. B. Whitehouse; C. D. Pusey

1996-01-01

190

Emergency testing for antineutrophil cytoplasmic antibodies combined with a dialog-based policy between clinician and biologist: effectiveness for the diagnosis of ANCA-associated vasculitis.  

PubMed

A prompt immunosuppressive treatment initiation is crucial in ANCA-associated vasculitis (AAV) to minimize organ injury. The aim of the present work was to analyze the accuracy of emergency ANCA screening to identify rapidly patients with AAV. In our Institution, emergency ANCA screening is based on a telephone call between a Clinician and a Biologist. Indirect immunofluorescence (IIF) for ANCA detection was performed using a commercial kit (Euroimmun(®) Granulocyte Mosaic 12). Positive serums for c- or p-ANCA at IIF are subsequently screened for antigenic specificity (MPO or PR3) by an immunodot technique (immunodot, D-Tek(®).) Positive samples with atypical c- or p-ANCA pattern at IIF are subsequently screened for antigenic specificity by ELISA. Data were retrieved from patients' medical records and confronted to emergency ANCA screening results. Between 2005 and 2012, 114 patients were screened. IIF was positive in 27.2 % of patients, but c-/p-ANCA anti-MPO/-PR3 was detected in 13.2 % of patients. The sensibility and specificity of IIF combined with immunodot for newly diagnosed AAV were 83.3 and 100 %, respectively. Ten patients were newly diagnosed with AAV. In these patients, a specific AAV treatment was initiated less than 24 h following ANCA screening. Emergency ANCA screening based on a clinical gating policy was relevant to identify patients with AAV diagnosis, and was associated with a rapid treatment initiation. PMID:25343851

Sayegh, Johnny; Poli, Caroline; Chevailler, Alain; Subra, Jean-François; Beloncle, François; Deguigne, Pierre Antoine; Beauvillain, Céline; Augusto, Jean-François

2015-04-01

191

Giant Cell Arteritis: A Systematic Review of the Qualitative and Semiquantitative Methods to Assess Vasculitis with 18F-Fluorodeoxyglucose Positron Emission Tomography  

PubMed Central

Giant cell arteritis (GCA) is the most common vasculitis affecting medium and large vessels. It shows a close clinical association with polymyalgia rheumatica (PMR), a musculoskeletal inflammatory disorder, which is clinically characterized by girdles pain and stiffness. 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) is an effective tool for the diagnosis, grading, and follow-up of patients affected by GCA involving the aorta and its proximal branches, but the lack of a standardized method for the assessment of vascular inflammation remains a critical issue, potentially leading to misclassification. In our systematic review, including 19 original articles for a total of 442 GCA patients (with or without PMR symptoms) and 535 healthy controls, we described the different qualitative, semiquantitative and combined methods that have been proposed throughout the literature for assessing the presence and grading the severity of GCA-related vascular inflammation on 18F-FDG PET scans, focusing on the diagnostic performance and examining their respective advantages and limitations. The majority of the included studies adopted qualitative methods of PET image analysis, which are less sensitive but more specific than semiquantitative ones. Among the semiquantitative approaches, the aortic-to-blood pool uptake ratio of the aortic arch seems to be the most accurate method. PMID:25254211

Puppo, Cristina; Massollo, Michela; Paparo, Francesco; Camellino, Dario; Piccardo, Arnoldo; Shoushtari Zadeh Naseri, Mehrdad; Villavecchia, Giampiero; Rollandi, Gian Andrea; Cimmino, Marco Amedeo

2014-01-01

192

Myocarditis, pancreatitis, polyarthritis, mononeuritis multiplex and vasculitis with symmetrical peripheral gangrene of the lower extremities as a rare presentation of leptospirosis: a case report and review of the literature  

PubMed Central

Introduction Leptospirosis is a zoonosis caused by the spirochete, Leptospira interrogans. While most cases of leptospirosis are mild to moderate, the course may be complicated by multiorgan dysfunction. We present a rare case of leptospirosis with acute myocarditis, pancreatitis, polyarthritis, mononeuritis multiplex and severe vasculitis with necrosis of the extremities. Case presentation A 32-year-old man from Congo presented with high-grade fever, confusion and headache. He developed tachycardia and hypotension followed by electrocardiogram changes and elevation of troponin I levels suggesting myocarditis. A physical examination revealed conjunctival suffusion, polyarthritis of his lower extremities and cutaneous necrosis of his feet due to vasculitis. Laboratory findings included amylase levels 10-fold the upper normal serum levels and thrombocytopenia. The diagnosis was confirmed by a positive leptospira immunoglobulin M, negative immunoglobulin G and a positive rapid agglutination test. Our patient recovered progressively with antimicrobials and supportive care. Conclusions Because the clinical features and diagnostic findings of leptospirosis are not specific, a high index of suspicion must be maintained for the diagnosis. Serology is the most important tool for accurate and quick diagnosis in order to administer the appropriate therapy. PMID:24885926

2014-01-01

193

Autologous stem cell therapy for systemic vasculitis  

Microsoft Academic Search

\\u000a Stem cell transplantation (SCT) has been proposed as a potentially highly effective therapy for a variety of autoimmune diseases.\\u000a In this group of diseases a disturbed immune response results in an attack by the immune system on host antigens with B- and\\u000a T-cell components involved in the process. The hypothesis behind SCT in autoimmune disease is that a vigorous immuno-ablative

David M. Carruthers; Paul A. Bacon

194

Small vessel vasculitis of the lung  

Microsoft Academic Search

Interstitial Lung Disease Program, National Jewish Medical and Research Center, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado, USA The diagnosis and management of SVV remains one of the most challenging clinical scenarios encountered by a clinician. Careful attention to detail and a thorough knowledge of the specific disorders, their therapies, and

S K Frankel; G P Cosgrove; K K Brown

2005-01-01

195

Assessment of disease activity in systemic vasculitis.  

PubMed Central

The systemic vasculitides are a group of inflammatory disorders characterised by relapses and remission. Before the introduction of immunosuppressive drugs, mortality was unacceptably high. Immunosuppressive therapy has had a therapeutic impact, but at the cost of increased risk of infection and other adverse effects. Differentiating infection from active disease can be difficult, and the inappropriate prescription of immunosuppressive drugs can be fatal. Hence disease indices which can aid physicians to identify the active phase of disease and enable early treatment, will be valuable in the management of this group of disorders. PMID:9538478

Tse, W. Y.; Cockwell, P.; Savage, C. O.

1998-01-01

196

Bullous reactions to bed bug bites reflect cutaneous vasculitis  

Technology Transfer Automated Retrieval System (TEKTRAN)

This study evaluates bullous cutaneous reactions and sequential histopathology in an individual sensitized to bed bug bites in an effort to better understand the allergic response and histology associated with these bites. There was a progression of the inflammatory response across time ranging from...

197

Forty years’ experience treating septic arteritis and vasculitis  

PubMed Central

By their natures, septic arteritis and infected grafts present a high morbidity and mortality situation for both the patient and the treating physician. In the experience of the authors, therapy frequently includes removing the previous infected graft, use of long-term antibiotics, repeat grafting and omental wraps. When possible, aortic endografting followed by future elective primary repair may be considered an option. Leaving wounds open to granulate from the ‘bottom up’ has been successful in the extremities and the groin. Again, avoidance of infection is primary. Antibiotics are frequently used at the time of vascular grafting, both intravenously and as irrigation. Examples of the authors’ techniques and results demonstrate the variety of procedures available. PMID:22477474

Dieter, Raymond A; Kuzycz, George B; Dieter, Raymond A; Dieter, Robert S

2009-01-01

198

Forty years' experience treating septic arteritis and vasculitis.  

PubMed

By their natures, septic arteritis and infected grafts present a high morbidity and mortality situation for both the patient and the treating physician. In the experience of the authors, therapy frequently includes removing the previous infected graft, use of long-term antibiotics, repeat grafting and omental wraps. When possible, aortic endografting followed by future elective primary repair may be considered an option. Leaving wounds open to granulate from the 'bottom up' has been successful in the extremities and the groin. Again, avoidance of infection is primary. Antibiotics are frequently used at the time of vascular grafting, both intravenously and as irrigation. Examples of the authors' techniques and results demonstrate the variety of procedures available. PMID:22477474

Dieter, Raymond A; Kuzycz, George B; Dieter, Raymond A; Dieter, Robert S

2009-01-01

199

Diffuse alveolar haemorrhage secondary to propylthiouracil-induced vasculitis.  

PubMed

Propylthiouracil is a drug used to treat hyperthyroidism. It can cause several side effects including pulmonary disorders that, although rare, can be severe. The authors describe the case of a woman treated with propylthiouracil who developed diffuse alveolar haemorrhage with severe respiratory failure and anaemia, which improved with discontinuation of the antithyroid drug and on starting systemic corticosteroid therapy. PMID:25661751

Ferreira, Catarina; Costa, Teresa; Marques, Ana Vieira

2015-01-01

200

Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis  

Microsoft Academic Search

OBJECTIVESReversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS.CASE REPORTSAll patients had headache, seizures, visual

A Primavera; D Audenino; N Mavilio; L Cocito

2001-01-01

201

Treatment of autoimmune diseases and systemic vasculitis with pooled human intravenous immune globulin.  

PubMed Central

Treatment of autoimmune and vasculitic disorders with intravenous immune globulin (IVIG) has shown great promise. IVIG appears to provide large amounts of immunoregulatory substances that have the capacity to regulate the immune system in various ways. The ability of IVIG to regulate deleterious autoimmune responses and disease is largely directly related to its ability to stimulate the production of anti-idiotypic autoantibodies after infusion. In this respect, IVIG represents a novel immunoregulatory agent with the ability to control autoimmune and vasculitic disorders without subsequent predisposition to infectious complications. Future work and controlled clinical trials will be necessary to prove the efficacy of this therapy for specific autoimmune and vasculitic disorders. Images Fig. 1 PMID:8033432

Jordan, S C; Toyoda, M

1994-01-01

202

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

E-print Network

, reti- nopathy of prematurity, and diabetic retinopathy.7 The retinal lesions in our patient could and Reconstructive Surgery. Baltimore: Williams & Wilkins, 1990:849­858. ALBIPUNCTATE RETINOPATHY WITH CONE

Palczewski, Krzysztof

203

Increased Levels of Soluble ST2 in Patients with Active Newly Diagnosed ANCA-Associated Vasculitis  

PubMed Central

Objective. ST2, a member of the interleukin-1 receptor family, is selectively expressed on Th2 cells and mediates important Th2 functions. IL-33 is a specific ligand of ST2. The aim of the study was to determine whether serum levels of soluble ST2 (sST2) or IL-33 predict activity of the disease in patients with ANCA-associated vasculitides (AAV). Methods. 139 AAV patients and 62 controls were studied. IL-33 and sST2 in the blood were measured with a commercially available ELISA. Results. Newly diagnosed AAV patients had higher sST2 levels than controls (P < 0.01). Levels of sST2 were significantly higher in active newly diagnosed AAV patients than in patients with remission (P < 0.001). IL-33 levels were higher in AAV patients than in the control groups (P = 0.002). However, serum IL-33 levels were not increased in patients with active AAV compared to patients in remission. IL-33 levels were higher in patients with granulomatosis with polyangiitis than in patients with microscopic polyangiitis (P = 0.012). Conclusions. Serum sST2, but not serum IL-33, may be a marker of activity in AAV patients.

Hladinova, Z.; Hruskova, Z.; Svobodova, B.; Malickova, K.; Lanska, V.; Konopásek, P.; Jancova, E.; Rysava, R.; Edelstein, C. L.; Tesar, V.

2015-01-01

204

Intraoperative recognition of retinal vasculitis in a patient with early lens-induced uveitis.  

PubMed

The purpose of this article is to describe a clinical finding not previously reported in lens-induced uveitis. An 86-year-old woman was seen by the authors 2 weeks after she had undergone phacoemulsification surgery complicated by retained lens material. A pars plana vitrectomy was indicated. Intraoperatively, retinal arteritis and phlebitis were documented in the retina immediately adjacent to the lens material. This resolved after removal of the material by vitrectomy. This previously unreported finding demonstrates the focal inflammatory effect of the retained lens material on the retinal vasculature. PMID:9031309

Besen, G; Freeman, W R

1997-01-01

205

Confirmation of the genetic association of CTLA4 and PTPN22 with ANCA-associated vasculitis  

E-print Network

allele of rs3087243 might thus contribute to T cell activation and autoimmune disease susceptibility. Taken together these data suggest that gen- otype at rs3087243 could impact upon both the expres- PTPN22 encodes a second negative regulator of T cell... the clinical syndromes Wegener's granulomato- sis (WG), microscopic polyangiitis (MPA) and Churg- Strauss Syndrome (CSS). AAV is a complex disease with both genetic and environmental factors involved in pathogenesis [1]. The magnitude of the increased familial...

Carr, Edward J; Niederer, Heather A; Williams, Julie; Harper, Lorraine; Watts, Richard A; Lyons, Paul A; Smith, Kenneth G C

2009-12-01

206

Large-Vessel Vasculitis: Interobserver Agreement and Diagnostic Accuracy of 18F-FDG-PET/CT  

PubMed Central

Introduction. 18F-FDG-PET visualises inflammation. Both atherosclerosis and giant cell arteritis cause vascular inflammation, but distinguishing the two may be difficult. The goal of this study was to assess interobserver agreement and diagnostic accuracy of 18F-FDG-PET for the detection of large artery involvement in giant cell arteritis (GCA). Methods. 31 18F-FDG-PET/CT scans were selected from 2 databases. Four observers assessed vascular wall 18F-FDG uptake, initially without and subsequently with predefined observer criteria (i.e., vascular wall 18F-FDG uptake compared to liver or femoral artery 18F-FDG uptake). External validation was performed by two additional observers. Sensitivity and specificity of 18F-FDG-PET were determined by comparing scan results to a consensus diagnosis. Results. The highest interobserver agreement (kappa: 0.96 in initial study and 0.79 in external validation) was observed when vascular wall 18F-FDG uptake higher than liver uptake was used as a diagnostic criterion, although agreement was also good without predefined criteria (kappa: 0.68 and 0.85). Sensitivity and specificity were comparable for these methods. The criterion of vascular wall 18F-FDG uptake equal to liver 18F-FDG uptake had low specificity. Conclusion. Standardization of image assessment for vascular wall 18F-FDG uptake promotes observer agreement, enables comparative studies, and does not appear to result in loss of diagnostic accuracy compared to nonstandardized assessment. PMID:25695092

Lensen, K. D. F.; Comans, E. F. I.; Voskuyl, A. E.; van der Laken, C. J.; Brouwer, E.; Zwijnenburg, A. T.; Pereira Arias-Bouda, L. M.; Glaudemans, A. W. J. M.; Slart, R. H. J. A.; Smulders, Y. M.

2015-01-01

207

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

PubMed Central

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

208

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

209

Vasculitis with renal involvement in essential mixed cryoglobulinemia: Case report and mini-review.  

PubMed

The discovery of a strong association between hepatitis C virus (HCV) infection and mixed cryoglobulinemia (MC) has led to an increasingly rare diagnosis of idiopathic essential MC (EMC). The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence, including HCV. The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation. In addition, although cryoglobulin testing is simple, improper testing conditions will result in false negative results. Here, we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations, highlighting the importance of extensive investigation to reach a proper diagnosis. We review the need for appropriate laboratory testing, which is often neglected in clinical practice and which can result in false negative results. This review also emphasizes the significance of an extended testing repertoire necessary for better patient management. Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation, EMC remains a separate entity. Correct diagnosis requires proper temperature regulation during sample handling, as well as characterization and quantification of the cryoprecipitate. Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring. Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome, which will reduce variability in inter-laboratory reporting. PMID:24868518

Anis, Sabiha; Abbas, Khawar; Mubarak, Mohammad; Ahmed, Ejaz; Bhatti, Sajid; Muzaffar, Rana

2014-05-16

210

Disseminated Herpes Zoster Mimicking Rheumatoid Vasculitis in a Rheumatoid Arthritis Patient on Etanercept  

Microsoft Academic Search

Tumor necrosis factor-? (TNF?)-blocking agents are immunomodulating agents introduced for treatment of a variety of chronic inflammatory disease conditions. Adverse effects include an increased incidence of infections. Clinically, these infections often have atypical presentations that may hamper prompt diagnosis. In our report of a patient on etanercept therapy for rheumatoid arthritis, the correct diagnosis was delayed because disseminated herpes zoster

S. Tresch; R. M. Trüeb; J. Kamarachev; L. E. French; G. F. L. Hofbauer

2009-01-01

211

Vasculitis with renal involvement in essential mixed cryoglobulinemia: Case report and mini-review  

PubMed Central

The discovery of a strong association between hepatitis C virus (HCV) infection and mixed cryoglobulinemia (MC) has led to an increasingly rare diagnosis of idiopathic essential MC (EMC). The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence, including HCV. The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation. In addition, although cryoglobulin testing is simple, improper testing conditions will result in false negative results. Here, we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations, highlighting the importance of extensive investigation to reach a proper diagnosis. We review the need for appropriate laboratory testing, which is often neglected in clinical practice and which can result in false negative results. This review also emphasizes the significance of an extended testing repertoire necessary for better patient management. Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation, EMC remains a separate entity. Correct diagnosis requires proper temperature regulation during sample handling, as well as characterization and quantification of the cryoprecipitate. Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring. Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome, which will reduce variability in inter-laboratory reporting. PMID:24868518

Anis, Sabiha; Abbas, Khawar; Mubarak, Mohammad; Ahmed, Ejaz; Bhatti, Sajid; Muzaffar, Rana

2014-01-01

212

Contrasting genetic association of IL2RA with SLE and ANCA - associated vasculitis  

E-print Network

of Rheumatol- ogy revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997, 40:1725. 13. Graham DS, Graham RR, Manku H, Wong AK, Whittaker JC, Gaffney PM, Moser KL, Rioux JD, Altshuler D, Behrens TW, Vyse TJ: Poly- 14... , Timothy J Vyse4, Lavanya Kamesh5, Richard A Watts6, Paul A Lyons1,2 and Kenneth GC Smith*1,2 Address: 1Cambridge Institute for Medical Research, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 0XY...

Carr, Edward J; Clatworthy, Menna R; Lowe, Christopher E; Todd, John A; Wong, Andrew; Vyse, Timothy J; Kamesh, Lavanya; Watts, Richard A; Lyons, Paul A; Smith, Kenneth G C

2009-03-05

213

Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study.  

PubMed

Recognizing giant cell arteritis (GCA) in patients with stroke may be challenging. We aimed to highlight the clinical spectrum and long-term follow-up of GCA-specific cerebrovascular accidents. Medical charts of all patients followed in a French Department of Internal Medicine for GCA between January 2008 and January 2014 were retrospectively reviewed. Patients with cerebrovascular accidents at GCA diagnosis were included. Diagnosis of GCA was based on American College of Rheumatology criteria. Transient ischemic attacks and stroke resulting from an atherosclerotic or cardioembolic mechanism were excluded. Clinical features, GCA-diagnosis workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data were analyzed. From January 2008 to January 2014, 97 patients have been followed for GCA. Among them, 8 biopsy-proven GCA patients (mean age 70±7.8 years, M/F sex ratio 3/1) had stroke at GCA diagnosis. Six patients reported headache and visual impairment. Brain MR angiography showed involvement of vertebral and/or basilar arteries in all cases with multiple or unique ischemic lesions in the infratentorial region of the brain in all but one case. Intracranial cerebral arteries involvement was observed in 4 cases including 2 cases with cerebral angiitis. Long lasting lesions on diffusion-weight brain MRI sequences were observed in 1 case. All patients received steroids for a mean of 28.1±12.8 months. Side effects associated with long-term steroid therapy occurred in 6 patients. Relapses occurred in 4 patients and required immunosuppressive drugs in 3 cases. After a mean follow-up duration of 36.4±16.4 months, all but 1 patient achieved complete remission without major sequelae. The conjunction of headache with vertebral and basilar arteries involvement in elderly is highly suggestive of stroke associated with GCA. Intracranial cerebral arteries involvement with cerebral angiitis associated with long lasting brain lesions on diffusion-weight brain MRI sequences may occur in GCA. Both frequent relapses and steroid-induced side effects argue for the use of immunosuppressive agents combined with steroids as first-line therapy. PMID:25526454

Larivière, Delphine; Sacre, Karim; Klein, Isabelle; Hyafil, Fabien; Choudat, Laurence; Chauveheid, Marie-Paule; Papo, Thomas

2014-12-01

214

The Role of Biological Agents in the Management of Large Vessel Vasculitis (LVV): A Systematic Review and Meta-Analysis  

PubMed Central

Background Giant cell arteritis (GCA) and Takayasu's arteritis (TAA) are large vessel vasculitides (LVV) for which corticosteroids (CS) are the mainstay for treatment. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness. Objective To systematically review the effectiveness and safety of biological agents in patients with LVV. Methods We searched 5 electronic databases (inception to October 2012) and conference abstracts with no language restrictions. Two reviewers independently selected studies, extracted data and assessed methodological quality. Our protocol was registered in PROSPERO. Results We included 25 studies (3 RCTs and 22 case series with ?2 cases). 95 GCA and 98 TAA patients received biological agents. The RCTs using anti-TNF agents (infliximab, etanercept and adalimumab) did not suggest a benefit in GCA. GCA patients receiving tocilizumab, in case series, achieved remission (19 patients) and reduction of corticosteroid dose (mean difference, –16.55 mg/day (95% CI: –26.24, –6.86)). In case series, 75 patients with refractory TAA treated with infliximab discontinued CS 32% of the time. Remission was variably defined and the studies were clinically heterogeneous which precluded further analysis. Conclusion This systematic review demonstrated a weak evidence base on which to assess the effectiveness of biological treatment in LVV. Evidence from RCTs suggests that anti-TNF agents are not effective for remission or reduction of CS use. Tocilizumab and infliximab may be effective in the management of LVV and refractory TAA, respectively, although the evidence comes from case series. Future analytical studies are needed to confirm these findings. PMID:25517966

Osman, Mohammed; Pagnoux, Christian; Dryden, Donna M.; Storie, Dale; Yacyshyn, Elaine

2014-01-01

215

Limited Cutaneous Systemic Sclerosis Associated With MPO-ANCA Positive Renal Small Vessel Vasculitis of the Microscopic Polyangiitis Type  

Microsoft Academic Search

Renal disease in systemic sclerosis may present in various patterns. A 66-year-old woman with a history of longstanding limited cutaneous systemic sclerosis of the CREST syndrome variant presented with a sudden left foot drop and rapidly progressive renal insufficiency associated with mild proteinuria, a nephritic urine sediment, and a urinary output of 900 mL\\/d. There was no history of intake

Bart Maes; An Van Mieghem; Thierry Messiaen; Dirk Kuypers; Boudewijn Van Damme; Yves Vanrenterghem

2000-01-01

216

ANCA-associated renal vasculitis at the end of the twentieth century--a disease of older patients  

Microsoft Academic Search

Objective. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are increasingly recognized in older patients. However, it is unknown whether disease presentation and response to treatment differs from younger patients. We aimed to examine the presentation, response to treatment and outcome of patients over 65 yr of age compared with a younger cohort. Methods. This retrospective, single centre, sequential cohort study reports presenting

L. Harper; C. O. Savage

2005-01-01

217

Acute blast crisis with EBV-infected blasts, in a patient with chronic myeloid leukemia, and vasculitis.  

PubMed

Unless they undergo transplantation, all patients with chronic myeloid leukemia (CML) will eventually develop a late phase of acute blast crisis (ABC). Although additional chromosomal abnormalities to the Philadelphia (Ph) chromosome may herald ABC in many CML cases, the mechanisms leading to this fatal event are obscure. Viral etiology, including the Epstein-Barr virus (EBV) has never been implicated in the pathogenesis of ABC in CML. Iloprost is an analogue of epoprostenol (prostacyclin; PGI2) commonly used for the treatment of peripheral vascular diseases and acts via inhibition of platelet activation, and by vasodilation. A case of ABC with blasts of undetermined lineage showing EBV infection in a male patient with Ph positive CML is described here. This unusual event developed during a course of treatment with the prostacyclin analogue, iloprost administered for vasculopathic leg ulcers. The proliferating blasts stained positively by immunohistochemistry only for the leukocyte common antigen (LCA/CD-45), and the EBV-latent membrane protein 1 (LMP-1). The only chromosomal abnormality detected by cytogenetic analysis was the conventional Ph-chromosome. It is suggested that ABC in this case of CML, was associated with EBV-activated blasts of undetermined lineage. PMID:10752995

Ariad, S; Argov, S; Manor, E; Yermiahu, T; Kedar, I

2000-04-01

218

Cryoglobulinemia Induced by a Murine IgG3 Rheumatoid Factor: Skin Vasculitis and Glomerulonephritis Arise from Distinct Pathogenic Mechanisms  

Microsoft Academic Search

MRL-lpr\\/lpr mice spontaneously develop a lupus-like syndrome characterized by immunopathological manifestations such as necrotizing vascular lesions of ear tips and severe glomerulonephritis. Similar skin vascular and glomerular lesions associated with cryoglobulinemia can be induced in normal mice by injection of a monoclonal antibody (mAb)-6.19 (gamma 3 heavy chain and kappa light chain), exhibiting both cryoglobulin and anti-IgG2a rheumatoid factor (RF)

Luc Reininger; Thierry Berney; Takanori Shibata; Francois Spertini; Ramon Merino; Shozo Izui

1990-01-01

219

CD4CD25 regulatory T-cell deficiency in patients with hepatitis C-mixed cryoglobulinemia vasculitis  

Microsoft Academic Search

Patients who are chronically infected with hepatitis C virus (HCV) often develop mixed cryoglobulinemia (MC), a B-cell proliferative disorder with polyclonal acti- vation and autoantibody production. We investigated if MC is associated with a deficit of CD4CD25 immunoregulatory T (Treg) cells, which have been shown to control autoimmunity. Because Treg cells express higher amounts of CD25 than activated CD4 T

Olivier Boyer; David Saadoun; Julien Abriol; Melanie Dodille; Jean-Charles Piette; Patrice Cacoub; David Klatzmann

2004-01-01

220

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

100,000 [1]. The most common severe AAV manifestations are glomerulonephritis, leading to renal failure and alveolar capillaritis causing lung hemorrhage. The current standards of care for initial treatment are either combination cyclophosphamide (CYC... ) and glu- cocorticoid (GC) therapy or rituximab and GC therapy. Although these treatments have significantly improved survival compared to untreated AAV, overall survival is still poor and many patients suffer from chronic morbi- dity including end...

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

221

Chronic Urticaria: Recent Advances  

Microsoft Academic Search

Chronic urticaria is an umbrella term, which encompasses physical urticarias, chronic “idiopathic” urticaria and urticarial\\u000a vasculitis. It is important to recognize patients with physical urticarias as the investigation and treatment differs in important\\u000a ways from patients with idiopathic chronic urticaria or urticarial vasculitis. Although relatively uncommon, urticarial vasculitis\\u000a is an important diagnosis to make and requires histological confirmation by biopsy.

Malcolm W. Greaves; Kian Teo Tan

2007-01-01

222

Anti-endothelial cell auto-antibodies in hepatitis C virus mixed cryoglobulinemia  

Microsoft Academic Search

Background\\/Aim: Hepatitis C virus (HCV) infection is often associated with mixed cryoglobulins (MC) and may manifest as small-vessel vasculitis. It has been suggested that antibody (Ab) or sensitized T cells to HCV-containing endothelial cells may initiate the vasculitis process. Anti-endothelial cell antibodies (AECA) have been found in various connective tissue disorders, with a high prevalence in systemic vasculitis. The aim

Patrice Cacoub; Pascale Ghillani; Ronan Revelen; Vincent Thibault; Vincent Cálvez; Frédéric Charlotte; Lucile Musset; Pierre Youinou; Jean-Charles Piette

1999-01-01

223

Vasculitides associated with HIV infection  

PubMed Central

The manifestations of human immunodeficiency virus (HIV) infection are protean and vasculitides are one of the less common but nonetheless important consequences. A wide range of vasculitides can be encountered, ranging from vasculitis resulting from specific infective agents to a non-specific vasculitis. Among the infective causes, cytomegalovirus and tuberculosis are probably the most common. A polyarteritis nodosa-like vasculitis with important differences to classic polyarteritis nodosa is also described. Hypersensitivity vasculitis resulting in several patterns of vasculitis and angiocentric immunoproliferative vasculitis are well recognised. As part of the immunocompromise caused by HIV, a granulomatous inflammation involving small arteries and veins of the brain surface and leptomeninges, termed a primary angiitis of the central nervous system, is a rare vasculitis associated with high mortality. A recently described large vessel (aorta, femorals, carotids) vasculopathy resulting in either multiple aneurysm formation or occlusive disease is seen in young adults. An infective agent is not found but aetiologically some of these lesions might be the result of a leucocytoclastic vasculitis of vasa vasora or periadventitial vessels. A final group of non-specific vasculitides not fitting into any of the characteristic patterns described accounts for the residue of vasculitides associated with HIV. Key Words: human immunodeficiency virus • vasculitis • immunocompromise PMID:11304843

Chetty, R

2001-01-01

224

Immunologic aspects of cardiovascular disease.  

PubMed

The spectrum of vasculitis is diverse, and numerous entities do not fit the aforementioned broad categories. Examples of these include Buerger's disease; vaso-occlusive vasculitis of the lower extremities associated with cigarette smoking; Behçet's disease, which is prevalent in the Orient and Middle East and is characterized by recurrent aphthous stomatitis, genital ulcerations, uveitis, meningoencephalitis, and phlebitis; and isolated central nervous system vasculitis, a rare disease with a poor prognosis that primarily affects intracranial arteries without a systemic acute-phase response. Improvement in the classification and definitive therapy of vasculitis awaits identification of etiologic agents and definition of host factors and the immune response responsible for the pathology. PMID:1359164

Ledford, D K

1992-11-25

225

Compassionate Use Protocol for the Treatment of Autoinflammatory Syndromes  

ClinicalTrials.gov

Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature (CANDLE); Juvenile Dermatomyositis (JDM); Stimulator of Interferon Genes (STING)-Associated Vasculitis With Onset in Infancy (SAVI)

2015-02-17

226

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

227

Diagnosis and therapeutic options for peripheral vasculitic neuropathy  

PubMed Central

Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types. PMID:25829955

2015-01-01

228

Endothelin 1 levels in relation to clinical presentation and outcome of Henoch Schonlein purpura  

Microsoft Academic Search

BACKGROUND: Henoch Schonlein purpura (HSP) is a common vasculitis of small vessels whereas endothelin-1 (ET-1) is usually reported elevated in vasculities and systematic inflammation. The aim of the present study was to investigate whether ET-1 levels are correlated with the clinical presentation and the outcome of HSP. METHODS: The study sample consisted of thirty consecutive patients with HSP. An equal

S Fessatou; P Nicolaidou; D Gourgiotis; H Georgouli; K Douros; M Moustaki; A Fretzayas

2008-01-01

229

Microscopic polyangiitis (microscopic polyarteritis) with late emergence of generalised Wegener’s granulomatosis  

Microsoft Academic Search

OBJECTIVESRecent proposals for the nomenclature of systemic vasculitis have focused on a distinction between (classic) polyarteritis nodosa (PAN) and microscopic polyangiitis or polyarteritis (MPA). Thus, MPA may cause necrotising vasculitis of medium sized or small arteries but, unlike PAN, involvement of “microscopic” vessels must always be present in the former. This study aimed to show that the term “MPA” may

Xavier Bosch

1999-01-01

230

Intravenous Immunoglobulin (IVIg) Therapy in MPO-ANCA Related Polyangiitis with Rapidly Progressive Glomerulonephritis in Japan  

Microsoft Academic Search

Jpn. J. Infect. Dis., 57, 2004 scarring is more evident in MPO-ANCA vasculitis than PR3-ANCA disease and indicates chronicity and a poorer outcome. A similar pathogenesis is responsible for other organ manifestations, for example, alveolar capillaritis in the lung is the cause of the lung hemorrhage. Serology: The association of ANCA with vasculitis has provided both a diagnostic tool and

Eri Muso; Toshiko Ito-Ihara; Takahiko Ono; Enyu Imai; Kunihiro Yamagata; Akira Akamatsu; Kazuo Suzuki

231

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

232

Characterization and analysis of carbon fibre-reinforced polymer composite laminates with embedded circular vasculature  

PubMed Central

A study of the influence of embedded circular hollow vascules on structural performance of a fibre-reinforced polymer (FRP) composite laminate is presented. Incorporating such vascules will lead to multi-functional composites by bestowing functions such as self-healing and active thermal management. However, the presence of off-axis vascules leads to localized disruption to the fibre architecture, i.e. resin-rich pockets, which are regarded as internal defects and may cause stress concentrations within the structure. Engineering approaches for creating these simple vascule geometries in conventional FRP laminates are proposed and demonstrated. This study includes development of a manufacturing method for forming vascules, microscopic characterization of their effect on the laminate, finite element (FE) analysis of crack initiation and failure under load, and validation of the FE results via mechanical testing observed using high-speed photography. The failure behaviour predicted by FE modelling is in good agreement with experimental results. The reduction in compressive strength owing to the embedding of circular vascules ranges from 13 to 70 per cent, which correlates with vascule dimension. PMID:20150337

Huang, C.-Y.; Trask, R. S.; Bond, I. P.

2010-01-01

233

Pulmonary and central nervous system involvement in Sweet's syndrome: a very rare case report.  

PubMed

Sweet's syndrome is a multisystem inflammatory disorder characterized by painful, erythematous plaques and aseptic neutrophilic infiltration of various organs. The absence of vasculitis is a histological criterion for diagnosis, but recent reports suggest that vasculitis can occur in Sweet's syndrome. Involvement of the central nervous system and the pulmonary system is very rare. In this case study we describe a chronic alcoholic man with Sweet's syndrome associated with acute-onset encephalitis and severe pulmonary involvement. The patient's symptoms responded dramatically to steroid treatment, and notably, a skin biopsy of his lesions showed vasculitis. PMID:18703859

Aydemir, Hande; Oztoprak, Nefise; Celebi, Güven; Altinyazar, Cevdet; Barut, Figen; Piskin, Nihal; Akduman, Deniz

2008-01-01

234

Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances.  

PubMed

Cryoglobulins are immunoglobulins that precipitate at temperatures less than 37°C. They occur secondary to infectious, autoimmune, and malignant processes. In the Brouet classification, type I cryoglobulinemia is caused by hyperviscosity, whereas type II and III manifestations are caused by vasculitis in target organs (primarily skin, peripheral nerves, and kidney). New classification criteria were recently proposed that may help with study and treatment of cryoglobulinemic vasculitis (CryoVas). Hepatitis C virus is the most common cause of CryoVas and treatment with antivirals can be curative in mild cases, whereas rituximab is highly effective in treating active vasculitis in more severe cases. PMID:25399942

Ghetie, Daniela; Mehraban, Navid; Sibley, Cailin H

2015-01-01

235

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 ...

236

Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases  

ClinicalTrials.gov

Rheumatoid Arthritis; Spondyloarthritis; Systemic Lupus Erythematosus (SLE); Dermatomyositis (DM); DMixed Connective Tissue Disease; Systemic Vasculitis; Systemic Sclerosis (SSc); Sjögren's Syndrome; Antiphospholipid Syndrome; Juvenile Idiopathic Arthritis; Juvenile SLE; Juvenile DM

2010-06-25

237

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

238

Chemical Sensitivities  

MedlinePLUS

... inflammation of the blood vessels (vasculitis-related disorder). Ozone and Nitrogen Dioxide Temporary or perhaps permanent bronchial hypersensitivity has been connected to inhaled ozone and nitrogen dioxide. Long-term exposure to nitrogen ...

239

Buerger's Disease  

MedlinePLUS

... below ). This decreased blood flow (“ischemia”) led to ulcers of the fingers and severe pain. An abnormal ... unlike many other forms of vasculitis. Even as ulcers and gangrene develop in the digits, organs such ...

240

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

241

The CARRA Registry  

ClinicalTrials.gov

Juvenile Idiopathic Arthritis; Systemic Lupus Erythematosus; Mixed Connective Tissue Disease; Juvenile Ankylosing Spondylitis; Juvenile Dermatomyositis; Localized Scleroderma; Systemic Sclerosis; Vasculitis; Sarcoid; Fibromyalgia, Primary; Auto-inflammatory Disease; Idiopathic Uveitis Idiopathic

2014-03-17

242

Isolated Aortitis  

MedlinePLUS

... are forms of large vessel vasculitis, most commonly giant cell arteritis and Takayasu arteritis. Other autoimmune diseases ... cases are due to a rheumatic cause like giant cell arteritis and Takayasu arteritis. Who gets Isolated ...

243

Kawasaki disease: Current therapeutic perspectives  

Microsoft Academic Search

Opinion statement  Kawasaki disease is an acute systemic vasculitis of unknown etiology that has become the most common form of acquired heart\\u000a disease in young children in developing countries. The most serious threat from Kawasaki disease is the development of coronary\\u000a vasculitis, with subsequent development of aneurysms, thrombosis, and coronary compromise. Standard treatment during the acute\\u000a phase of Kawasaki disease now

Erik C. Michelfelder; David Shim

2002-01-01

244

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

245

Are circulating neutrophils intravascularly activated in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides?  

PubMed Central

Vascular injury in vasculitis may be due to activation of circulating neutrophils resulting in their increased adhesiveness to locally activated endothelium (Shwartzman phenomenon). Previously, we demonstrated up-regulation of endothelial intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) in biopsies from patients with ANCA-associated vasculitis. In the present study, we investigated the expression of adhesion molecules (CD11b, ICAM-1, VLA-4, l-selectin) and activation markers (CD66b, CD64, CD63) on circulating neutrophils from patients with ANCA-associated vasculitis in comparison with their expression on cells from healthy volunteers and patients with sepsis. We related these findings to parameters of disease activity. Surface marker expression was determined by using a non-activating whole blood flow cytometric assay. The expression of activation markers, but not the expression of adhesion molecules, was increased on neutrophils from patients with active vasculitis. The expression of CD63 and CD66b on neutrophils correlated with disease activity as determined by the Birmingham Vasculitis Activity Score (BVAS). In contrast to patients with active vasculitis, patients with sepsis showed up-regulation of all markers, including adhesion molecules, suggesting that circulating neutrophils are fully activated in sepsis. We conclude that in ANCA-associated vasculitis, circulating neutrophils are not fully activated, since they do not express increased levels of adhesion molecules as sepsis or in the Shwartzman reaction. These findings are compatible with the concept that in vivo vascular damage in ANCA-associated vasculitides does not occur due to a Shwarzman-like reaction but only after ANCA-induced neutrophil activation at the endothelial cell surface. PMID:9844062

MULLER KOBOLD, A C; MESANDER, G; STEGEMAN, C A; KALLENBERG, C G M; COHEN TERVAERT, J W

1998-01-01

246

[Cerebral vasculitides].  

PubMed

Cerebral vasculitides can be secondary to a broad variety of conditions such as systemic disease, infections, malignancies and toxic exposures, or can be isolated to the central nervous system. When cerebral vasculitis is suspected, the diagnostic work up must be exhaustive to rule out all these potential causes of secondary vasculitis. Most often, the diagnosis of cerebral vasculitis relies on the combination of clinical, biological and radiological findings. It is definite only when based on a positive leptomeningeal biopsy. This latter should be done when the diagnostic uncertainty remains high, especially in patients with underlying systemic disease and/or infrequent or rapidly-progressive symptoms. One can probably rule out the diagnostic of cerebral vasculitis in patients in whom both MRI and cerebrospinal fluid examination are normal. For secondary cerebral vasculitis, treatment depends on the identified underlying etiology. However, corticosteroids combined with immunosuppressive drugs are usually used. Actually, there is no consensual treatment for primary angiitis of the central nervous system. The prognosis is better when diagnosis is made early and treatment started promptly. PMID:22326667

de Boysson, Hubert; Faivre, Anthony; Pagnoux, Christian

2012-11-01

247

Acute antibody-mediated rejection of skin grafts without involvement of granulocytes or complement  

SciTech Connect

In immunosuppressed mice that carry rat skin xeno-grafts, acute antibody-mediated graft rejection (AAR) can be induced by intravenous administration of mouse anti-rat globulin. Dependent on the amount of antibody injected and on the complement status of the recipient, an Arthus-like or a Shwartzman-like pattern of vasculitis occurs. The role of polymorphonuclear granulocytes (PMNs) in either type of vasculitis was tested by inducing AAR in recipients depleted of PMNs by total body irradiation. Despite the absence of PMNs in the graft vessels, AAR occurred both in the Arthus-like and in the Shwartzman-like type. Moreover, AAR could be elicited in PMN-depleted recipients that were complement-depleted by cobra venom factor treatment or were congenitally C5-deficient. We conclude that neither the PMN nor complement is an essential mediator the PMN nor complement is an essential mediator in this form of antibody-mediated vasculitis.

Bogman, M.J.; Cornelissen, I.M.; Koene, R.A.

1984-05-01

248

Idiopathic Nonviral Cryoglobulinemia Treated Successfully With Rituximab.  

PubMed

Cryoglobulinemia is a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. The therapeutic management of idiopathic cryoglobulinemic vasculitis has yet to be defined because no study has evaluated the best strategies. However, treatment of severe vasculitis is traditionally based on a combination of corticosteroids and immunosuppressants or plasmapheresis, and more recently rituximab. We report a case of 77-year-old female patient diagnosed with idiopathic cryoglobulinemia, treated successfully with 6 months prednisone tapering and 2 doses of rituximab (1 g each dose). After receiving the above-mentioned treatment, her creatinine went back to normal with resolution of proteinuria and hematuria, normalization of serum complements, and significant improvement in her clinical picture. We conclude that rituximab could be an effective treatment for idiopathic cryoglobulnemia. PMID:24914502

Kamel, Mahmoud; Thajudeen, Bijin; Bracamonte, Erika; Madhrira, Machaiah

2014-06-01

249

[Thrombotic vasculopathy probably associated with cocaine contaminated with levamisole: report of 2 cases].  

PubMed

The vasculities are complex diseases. Their cutaneous manifestations are very important and often mirror several pathologies. Cocaine use has been related to both, vasculitis and thrombotic vasculopathy and pseudovasculitis. A new syndrome has been described in association with its adulteration with levamisole. It can be very serious, leading patients to death. This is relevant as levamisole-adultered cocaine seems to be increasingly offered to consumers. Our goal is to report the first two cases in Mexico, which faces an important raise in cocaine use, emphasizing that a high suspicion based on certain characteristics allows for early recognition and adequate treatment. PMID:25680649

Martínez-Velasco, María Abril; Flores-Suárez, Luis Felipe; Toussaint-Caire, Sonia; Rodríguez-Carreón, Angélica; Díaz-Lozano, Marisol; Sánchez-Armendáriz, Karen

2015-01-01

250

Takayasu arteritis in a young woman: a 4-year case history.  

PubMed

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

251

Kawasaki disease.  

PubMed

Kawasaki disease (KD) is the archetypal pediatric vasculitis, exemplifying the unique aspects and challenges of vascular inflammation in children. The condition is almost unheard of in adults, is closely associated with infections, and is self-limited, with fever resolving after an average of 12 days even without treatment. Yet KD is also a potentially fatal disease and the most common cause of acquired heart disease in the developed world. Unraveling of the developmental, immunologic, and genetic secrets of Kawasaki disease promises to improve our understanding of vasculitis in particular, and perhaps also to provide a window on the fundamental mysteries of inflammatory diseases in general. PMID:25399940

Sundel, Robert P

2015-01-01

252

Behçet's disease diagnosed after acute HIV infection: viral replication activating underlying autoimmunity?  

PubMed

Behçet's disease is an autoimmune systemic vasculitis that can occur after exposure to infectious agents. Behçet's disease also has been associated with HIV infection, including de novo development of this condition during chronic HIV infection and resolution of Behçet's disease symptoms following initiation of antiretroviral therapy. We describe a patient who presented with systemic vasculitis with skin and mucous membrane ulcerations in the setting of acute HIV infection, who was eventually diagnosed with Behçet's disease, demonstrating a possible link between acute HIV infection, immune activation and development of autoimmunity. PMID:24912539

Roscoe, Clay; Kinney, Rebecca; Gilles, Ryan; Blue, Sky

2015-05-01

253

Management of IRVAN syndrome with observation.  

PubMed

A 7-year-old girl with IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome was monitored for 9 years. The patient had symmetric multiple aneurysmal dilations, retinal vasculitis, and severe hard exudation in the macula bilaterally. Long-term visual acuity in her untreated right eye was 20/25. In the left eye, treated with laser panretinal photocoagulation and intravitreal vascular endothelial growth factor inhibitor therapy, visual acuity decreased to 20/200 from macular hard exudates and central fibrovascular tissue. Observation is a consideration in IRVAN syndrome if the vision remains good with hard exudation. PMID:24766157

Basha, Madhi; Brown, Gary C; Palombaro, Geremie; Shields, Carol L; Shields, Jerry A

2014-01-01

254

Marked Retroperitoneal Lymphadenopathy in Hairy Cell Leukemia: A Case Report  

PubMed Central

Hairy cell leukemia (HCL) is uncommonly associated with lymphadenopathy, while retroperitoneal lymphadenopathy is extremely uncommon. We report on a patient with a 12-year history of HCL who developed painless jaundice and ascites, accompanied by positional discomfort with persistent nausea. Computed tomography examination revealed 2 large retroperitoneal masses, which at autopsy consisted of HCL with focally intermixed pancreatic and peripancreatic tissue. Lymphadenopathy was not identified above the diaphragm or below the aortic bifurcation. No vasculitis or an unusual HCL histology was identified. As previous reports, our findings suggest that HCL with massive lymphadenopathy has a specific site predilection, but it is not necessarily accompanied by vasculitis or an unusual histology. PMID:24163667

Shackelford, R.E.; Heldmann, M.; Eskandari, F.; Joshi, N.; Browning, J.; Maxwell, N.; Coteligam, J.

2013-01-01

255

Bench-to-bedside review: Pulmonary–renal syndromes – an update for the intensivist  

Microsoft Academic Search

The term Pulmonary–renal syndrome refers to the combination of diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis. A variety of mechanisms such as those involving antiglomerular basement membrane antibodies, antineutrophil cytoplasm antibodies or immunocomplexes and thrombotic microangiopathy are implicated in the pathogenesis of this syndrome. The underlying pulmonary pathology is small-vessel vasculitis involving arterioles, venules and, frequently, alveolar capillaries. The underlying

Spyros A Papiris; Effrosyni D Manali; Ioannis Kalomenidis; Giorgios E Kapotsis; Anna Karakatsani; Charis Roussos

2007-01-01

256

Pancreatic mass as an initial manifestation of polyarteritis nodosa: A case report and review of the literature  

PubMed Central

Classic polyarteritis nodosa (PAN) that targets medium-sized muscular arteries and microscopic polyangiitis (MPA), characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA), are distinct clinicopathological entities of systemic vasculitis. A 66-year-old woman presented with fever, cholestasis and positive MPO-ANCA. Radiological examination showed a pancreatic mass compressing the bile duct. Therefore, we performed pancreatoduodenectomy. Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels, spared arterioles or capillaries in the pancreas, a finding consistent with PAN. Unexpectedly, renal biopsy revealed small-caliber vasculitis and glomerulonephritis, supporting MPA. The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles. Its diagnosis is challenging because no reliable clinico-radiological findings have been observed. Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory. Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA. PMID:25624739

Yokoi, Yoshihiro; Nakamura, Ippei; Kaneko, Takeshi; Sawayanagi, Tomoki; Watahiki, Youichi; Kuroda, Makoto

2015-01-01

257

Coeliac disease in patients with Kawasaki disease. Is there a link?  

Microsoft Academic Search

Objective. Kawasaki disease (KD) is an acute febrile systemic vasculitis, mainly affecting infants and young children. Immunological abnormalities during the acute phase of KD have been described extensively. However, the occurrence of a second immunological disorder in a patient with a history of KD is rarely reported. We evaluated the presence of autoimmune thyroiditis and coeliac disease (CD) in patients

S. Stagi; G. Simonini; L. Ricci; M. de Martino; F. Falcini

2006-01-01

258

Increased nitric oxide production in patients with Behçet's disease: Is it a new activity marker?  

Microsoft Academic Search

Background: The origin of Behçet's disease (BD) is unclear. One of the prominent features of BD is vasculitis and thrombosis as a result of endothelial dysfunction. Because nitric oxide (NO) is synthesized by endothelium, we considered it as an interesting target of investigation in BD. Objective: Our purpose was to define the level of NO in the serum of patients

Cem Evereklioglu; Yusuf Turkoz; Hamdi Er; H. Serhat Inaloz; Emin Ozbek; Mustafa Cekmen

2002-01-01

259

REVIEW Open Access Lupus enteritis: from clinical findings to  

E-print Network

REVIEW Open Access Lupus enteritis: from clinical findings to therapeutic management Peter Janssens steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare [8]. In the BILAG 2004, lupus enteritis is de- fined as either vasculitis or inflammation

Paris-Sud XI, Université de

260

Microscopic Polyangiitis in a Pediatric Patient  

Microsoft Academic Search

icroscopic polyangiitis (MPA), previously called hypersensitivity angiitis, is a sys- temic necrotizing vasculitis that involves many organ systems including the skin, joints, kidneys, and lungs. Microscopic polyangiitis most commonly affects adults in the fourth and fifth decades of life, with only a few cases reported in children. We describe a pediatric patient with microscopic polyangiitis. Arch Fam Med. 2000;9:1189-1192 REPORT

Sujatha Ramesh; Yongxin Chen; Cazibe Celik; Edwin Jenis; Julian L. Ambrus

261

Oral lesions as the first clinical sign of microscopic polyangiitis: A case report  

Microsoft Academic Search

Microscopic polyangiitis (MPA) is defined as a systemic necrotizing vasculitis that clinically and histologically affects small-sized vessels without granulomata. The main clinical feature of MPA is renal involvement characterized by rapidly progressing glomerulonephritis. We report a case of oral lesions, initially thought to be associated with Wegener's granulomatosis, as the first sign of MPA in an otherwise healthy man. To

Caroline H. Shiboski; Joseph A. Regezi; Henry C. Sanchez; Sol Silverman

2002-01-01

262

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. PMID:25408917

Fung, Kenneth; Edmondson, Stephen

2014-01-01

263

Advances in the Etiology and Management of Immune-mediated Glomerulonephritides  

Microsoft Academic Search

Immune-mediated inflammatory disease of the glomerulus remains a particular chal - lenge for the nephrologist, whether it is limited to the kidney or a part of a multisystem disease. Studies on animal models and the development of new viral detection assays have led to notable advances in our understanding of disease mechanisms in antineutrophil cyto - plasmic antibody (ANCA)-associated vasculitis

JEREMY S. DUFFIELD; AMIR QAMAR; Joseph V. Bonventre; Barry M. Brenner; Jessamyn Bagley; D. Sangeeta Bhattia; Steven Brunelli; Anil K. Chandraker; David M. Charytan; Mary Choi; Kenneth B. Christopher; Gary C. Curhan; Bradley M. Denker; John P. Forman; Markus H. Frank; Monica Grafals; Indira Guleria; Dirk M. Hentschel; Andreas Herrlich; Li-Li Hsiao; Benjamin D. Humphreys; John J. Iacomini; D. Vicki Rubin Kelley; Julie Lin; M. P. H. Edgar; L. Milford; David B. Mount; Nader Najafian; Shona Pendse; Martin R. Pollak; Mohamed H. Sayegh; Julian L. Seifter; Jagesh V. Shah; Alice M. Sheridan; Ajay K. Singh; Theodore I. Steinman; Eric N. Taylor; John K. Tucker; Takuya Ueno; Sushrut S. Waikar; Wolfgang C. Winkelmayer; D. Xueli Yuan

264

Anthrax vaccine associated deaths in miniature horses.  

PubMed

During a widespread anthrax outbreak in Canada, miniature horses were vaccinated using a live spore anthrax vaccine. Several of these horses died from an apparent immune-mediated vasculitis temporally associated with this vaccination. During the course of the outbreak, other miniature horses from different regions with a similar vaccination history, clinical signs, and necropsy findings were found. PMID:25829553

Wobeser, Bruce K

2015-04-01

265

[Wegener's granulomatosis diagnosed by orbital-meningeal presentation: a case report].  

PubMed

Wegener's granulomatosis is a necrotizing granulomatous vasculitis with a strong affinity for the upper respiratory tract, lung and kidney. The ophthalmologic manifestation most often presents as inflammatory orbital pseudotumor or scleritis. We report a case of a 27-year-old woman with an orbital-meningeal presentation leading to a diagnosis of Wegener's granulomatosis. PMID:22975270

Louati, H; Chebbi, A; Ben Hassen, I; Douira, W; Ben Hassine, L; Lahmar, L; Ayed, S; Bellagha, I

2012-10-01

266

Henoch–Schönlein purpura: a clinical case with dramatic presentation  

PubMed Central

A case study involving a 55-year-old Caucasian male with end-stage glomerulosclerosis is presented here. Kidney biopsies showed no deposits on imunofluorescent microscopy. Relapsing massive haemoptysis and suspected bronchovascular malformation required lung lobectomy which revealed malformative and tortuous small blood vessels, with no vasculitis. Blood antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were undetectable. Seric immunoglobulins and complement levels were normal. Three months later, arthralgia and joint oedema first appeared. Skin biopsy revealed vasculitis immune-reactive to immunoglobulin A. Systemic corticotherapy was then started. Two weeks later, the patient presented with abdominal pain melena and rectal bleeding (haematoquesia). Endoscopic study showed diffuse gastrointestinal haemorrhage. Angiographic study revealed diffuse lesions compatible with vasculitis and haemorrhage from multiple spots. Cyclophosphamide and then intravenous immunoglobulin were associated to treatment without response. Increasing blood loss occurred, with massive gastrointestinal haemorrhage and haemorrhagic ascitis. Death occurred due to uncontrolled diffuse bleeding. Necropsy findings showed generalised small vessels vasculitis compatible with Henoch–Schönlein purpura. PMID:22778211

Bento, João; Magalhães, Adriana; Moura, Conceição Souto; Hespanhol, Venceslau

2010-01-01

267

Comparison of PR3-ANCA and MPO-ANCA Epitope Specificity upon Disease Relapse  

EPA Science Inventory

BACKGROUND Relapse is a major clinical problem in ANCA vasculitis that causes increased morbidity and mortality. Compared to MPO-ANCA patients, patients with PR3-ANCA run a significantly increased risk of experiencing relapses. We hypothesized that a relapsing patient is produ...

268

zentrum chirurgie ABTEIlUNG THORAX-, HERZ-UND GEFSSCHIRURGIE centre for Surgery DEPARTMENT OF THORACIC AND CARDIOVASCUlAR SURGERy  

E-print Network

OF THORACIC AND CARDIOVASCUlAR SURGERy Abteilungsdirektor/in | Head of Department Prof. Dr. med. DiplHirurGie Centre for Surgery depArtment of tHorAcic And cArdioVAsculAr surGery Einleitung Die Abteilung vertrittzentrum chirurgie ABTEIlUNG THORAX-, HERZ- UND GEFäSSCHIRURGIE centre for Surgery DEPARTMENT

Gollisch, Tim

269

Endothelial progenitor cell dysfunction in rheumatic disease  

Microsoft Academic Search

Rheumatic disease is characterized by inflammation and endothelial dysfunction, which contribute to accelerated atherosclerosis. Circulating endothelial progenitor cells (EPCs) can restore dysfunctional endothelium and thereby protect against atherosclerotic vascular disease. The number and function of EPCs are, however, affected in rheumatic diseases such as psoriatic arthritis, rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and antineutrophil cytoplasmic autoantibody-associated vasculitis. Rheumatic disease

Marianne C. Verhaar; Peter E. Westerweel

2009-01-01

270

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

271

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

272

[What's new in internal medicine?].  

PubMed

In this paper based on a review of medical articles from September 2012 to September 2013, new data were selected about IgG4-related disease, connections between vitamin D and systemic lupus erythematosus, revised nomenclature of vasculitis, effects of salt on autoimmunity, new autoinflammatory syndromes and some diseases as systemic sclerosis and thrombangiitis obliterans. PMID:24365498

Doutre, M-S

2013-11-01

273

Granulomatosis with Polyangiitis Presenting as a Parotid Gland Abscess  

PubMed Central

Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess.

Dias, Blenda; Soares, Daniela; Sampaio, Patrick; Santiago, Mittermayer

2015-01-01

274

The relevance of large-vessel vascular disease and restricted ankle movement to the aetiology of leg ulceration in rheumatoid arthritis  

Microsoft Academic Search

SUMMARY Leg ulceration in rheumatoid arthritis (RA) without systemic vasculitis is a diYcult clinical problem and a common cause of morbidity. We have assessed venous function, arterial pressures and range of ankle movement in 23 RA patients with a leg ulcer and compared the results with those in the non-ulcerated contralateral limb and in 25 RA patients matched for age

E. R. MCRORIE; C. V. RUCKLEY; G. NUKI

1998-01-01

275

Renal Abnormalities in Psoriatic Patients: A Review  

Microsoft Academic Search

Numerous systemic diseases affect both skin and kidneys: autoimmune diseases (e.g. lupus, vasculitis), hematologic abnormalities, genetic disorders and others have been described (table 1). Although psoriasis is considered an immunomediated disease, it is assumed to affect the skin and joints exclusively. Psoriasis [1] is a common chronic inflammatory disorder of the skin, which affects more than 2% of people with

J. Heuvels; A. Maximus; J. L. Bosmans; J. Lambert; M. E. De Broe

1999-01-01

276

Myeloperoxidase-antineutrophil cytoplasmic antibody-positive crescentic glomerulonephritis complicating the course of Graves' disease: Report of three adult cases  

Microsoft Academic Search

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been recently recognized in Graves' disease patients treated with propylthiouracil. We have experienced three adult cases of Graves' disease with main features being renal derangements. All three patients, who were between the ages of 22 and 82 years, had been treated with propylthiouracil for 2 to 5 years after a diagnosis of Graves' disease.

Masayuki Tanemoto; Hiroshi Miyakawa; Junichi Hanai; Masako Yago; Masafumi Kitaoka; Shunya Uchida

1995-01-01

277

Textbook of rheumatology  

SciTech Connect

This book contains 23 papers. Some of the titles are: Diagnostic Radiology in the Rheumatic Diseases; Laboratory Testing in Rheumatology; Arthritis Nursing and the Team Approach in the Management of Rheumatic Disease; The Surgical Management of Arthritis; Vasculities; Neoplasms of Bone and Joints; and Rheumatic Disease of Childhood.

Turner, R.A.; Wise, C.M.

1986-01-01

278

Oligoclonal IgA Response in the Vascular Wall in Acute Kawasaki Disease  

Microsoft Academic Search

Kawasaki Disease (KD) is a potentially fatal acute vasculitis of childhood. Although KD is the leading cause of acquired heart disease in children in developed nations, its pathogenesis remains unknown. We previously reported the novel observation that IgA plasma cells infiltrate the vascular wall in acute KD. We have now examined the clonality of this IgA response in vascular tissue

Anne H. Rowley; Stanford T. Shulman; Benjamin T. Spike; Carrie A. Mask; Susan C. Baker

2001-01-01

279

Use and Perceived Credibility of Medication Information Sources for Patients with a Rare Illness: Differences by Gender  

Microsoft Academic Search

Patients with rare illnesses may use medication information sources that are appreciably different from those used by patients with more common illnesses. This article's purpose is to describe vasculitis patients' most frequently used medication information sources, determine which sources patients perceive as credible, and explore gender differences in source use and perceived credibility. Using an online questionnaire, patients (n = 232) indicated

Delesha M. Carpenter; Robert F. DeVellis; Susan L. Hogan; Edwin B. Fisher; Brenda M. DeVellis; Joanne M. Jordan

2011-01-01

280

[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

281

Coil embolization of an inferior pancreaticoduodenal artery aneurysm associated with celiac artery occlusion  

Microsoft Academic Search

Pancreaticoduodenal arcade aneurysms are rare. Untreated, these lesions enlarge progressively and have the potential for spontaneous rupture. Aneurysmal degeneration of pancreaticoduodenal arcade vessels is known to be associated with celiac artery occlusion, vasculitis, and certain connective tissue disorders. Given their precarious location, surgical expiration is a challenging endeavor. Innovations in endovascular techniques offer a possible alternative.We report a case of

Brian G Peterson; Scott A Resnick; Mark K Eskandari

2003-01-01

282

Confusing presentation of chaetomium brain abscess.  

PubMed

We put forth the case of a young immunocompetent female who presented with fever and left basal ganglia bleed secondary to vasculitis. After 2 weeks of prednisolone therapy, she continued to deteriorate. MRI scan revealed enhancement of previous hematoma, pus was aspirated surgically and fungal culture grew Chaetomium. PMID:24977715

Waqas, Muhammad; Waheed, Shahan; Mangrio, Salman Ahmed; Rashid, Sana; Qadeer, Mohsin; Bari, Ehsan

2014-12-01

283

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

284

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

285

Suspected Sarcocystis infections of the bovine placenta and foetus  

Microsoft Academic Search

Summary Protozoan parasites resembling Sarcocystis schizonts were found in the brains of 2 bovine foetuses and the placentas of another 4. As typical of Sarcocystis, the organisms were located mainly within vascular endothelial cells, and frequently the zoites within the schizonts were arranged in a rosette or palisade fashion. Pathological changes noted were placentitis, myocarditis, pulmonary vasculitis and encephalitis. Attempts

B. L. Munday; H. Black

1976-01-01

286

OVINE HERPESVIRUS 2 MRNA EXPRESSION IN CATTLE AND BISON WITH MALIGNANT CATARRHAL FEVER  

Technology Transfer Automated Retrieval System (TEKTRAN)

Malignant catarrhal fever (MCF) is a fulminant disease of certain susceptible ruminants caused by ovine herpesvirus 2 (OvHV-2). The characteristic lesions of MCF include lymphoproliferation, vasculitis and mucosal ulceration. The pathogenesis of these lesions is very poorly understood, but is most ...

287

A novel bocavirus in canine liver  

E-print Network

gastroenteritis, necrotizing vasculitis, granulomatous lymphadenitis and anuric renal failure, we identified and characterized a novel bocavirus we named Canine bocavirus 3 (CnBoV3). The three major ORFs of CnBoV3 (NS1, NP1 and VP1) shared less than 60% aa...

Li, Linlin; Pesavento, Patricia A.; Leutenegger, Christian M.; Estrada, Marko; Coffey, Lark L.; Naccache, Samia N.; Samayoa, Erik; Chiu, Charles; Qiu, Jianming; Wang, Chunlin; Deng, Xutao; Delwart, Eric

2013-02-13

288

Rituximab Induced Left Bundle Branch Block  

PubMed Central

Rituximab (a monoclonal antibody directed against CD 20) therapy can be acutely complicated by infusion reactions and cardiac arrhythmia on rare occasions. We report the first case of a new onset left bundle branch block (LBBB) after rituximab therapy for Wegener's vasculitis.

Sheikh, Mujeeb; Moza, Ankush; Grubb, Blair P.

2015-01-01

289

A 27-year-old woman presenting with refractory hypoxaemic respiratory failure, haemoptysis and thyrotoxicosis: a rare manifestation of propylthiouracil therapy.  

PubMed

Pulmonary manifestations of hyperthyroidism not only include pulmonary hypertension and hydrostatic pulmonary oedema, but also treatment/drug-associated pulmonary diseases have to be considered as an exclusion diagnosis. A 27-year-old woman with hypoxaemic respiratory failure under an arterial-venous extra-corporeal membrane oxygenator (AV-ECMO) was admitted to the intensive care unit (ICU). The patient had progressive dyspnoea with haemoptysis, palpitations and failure to thrive. The patient had Graves' disease treated previously with propylthiouracil (PTU). Diffuse alveolar haemorrhage is a non-specific syndrome characterised by evidence of diffuse alveolar damage, exclusion of infectious aetiology and progressively bloodier bronchoalveolar lavage (and/or 20% hemosiderin laden macrophages on cytological examination). PTU associated perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) vasculitis appears to be more common in younger female patients presenting with leukocytoclastic vasculitis, myalgias and arthralgias. The latter compared to non-drug associated ANCA vasculitis which are more common in older males with visceral involvement. PTU-induced ANCA vasculitis prognosis appears to be better compared to primary ANCA syndromes. PMID:25150234

Ortiz-Diaz, Enrique O

2014-01-01

290

BRIEF REPORT: ODOCOILEUS HEMIONUS DEER ADENOVIRUS IS RELATED TO THE MEMBERS OF ATADENOVIRUS GENUS  

Technology Transfer Automated Retrieval System (TEKTRAN)

The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames that mapped to pVIII hexon...

291

Cryoglobulinemia presenting after liver transplantation  

Microsoft Academic Search

Essential mixed cryoglobulinemia is frequently associated with chronic hepatitis C. Three patients undergoing transplantation for end-stage chronic hepatitis C in whom cryoglobulinemia with vasculitis developed after transplantation are described. Hepatitis C virus (HCV) infection was confirmed in the 3 patients by the presence of HCV RNA detected by polymerase chain reaction. The time interval between transplantation and the first expression

J Gournay; LD Ferrell; JP Roberts; NL Ascher; TL Wright; JR Lake

1996-01-01

292

Infection of Peripheral Blood Mononuclear Cells by Hepatitis C Virus in Mixed Cryoglobulinemia  

Microsoft Academic Search

IXED CRYOGLOBULINEMIA (MC) is a lympho- M proliferative disorder characterized clinically by purpura, arthralgias, weakness, and various organ involve- ments such as peripheral neuropathy, chronic hepatitis, glo- merulonephritis, and generalized vasculitis.' The typical pathologic lesions involving small- and medium-sized ves- sels are due to deposition of circulating immune-complexes (CIC) and complement.',' In MC patients, a monoclonal rheumatoid factor in the

Clodoveo Ferri; Monica Monti; Luca La Civita; Giovanni Longombardo; Francesco Greco; Giampiero Pasero

1993-01-01

293

Efficacy and safety of rituximab in type II mixed cryoglobulinemia  

Microsoft Academic Search

ready administered at the time of recruit- ment. All patients had active disease, poorly controlled or difficult to manage with previous treatments, including corti- costeroids in all. Efficacy and safety of rituximab therapy were evaluated in the following 6 months. The overall follow-up after rituximab treatment ranged from 9 to 31 months. Rituximab proved effective on skin vasculitis manifestations (ulcers,

Francesco Zaja; Salvatore De Vita; Cesare Mazzaro; Stefania Sacco; Daniela Damiani; Ginevra De Marchi; Angela Michelutti; Michele Baccarani; Renato Fanin; Gianfranco Ferraccioli

294

Multiorgan dysfunction syndrome secondary to joint supplement overdosage in a dog.  

PubMed

A 5-year-old spayed female Bernese mountain dog, with a chief complaint of vomiting and melena ingested approximately 200 nutritional joint supplement tablets. Despite aggressive therapy, the patient developed a coagulopathy, pancreatitis, peritonitis, acute kidney injury, and was euthanized. Postmortem examination revealed myocardial necrosis, pneumonia, centrilobular hemorrhage and necrosis of the liver, vasculitis, and acute tubular necrosis. PMID:25829554

Nobles, Irma J; Khan, Safdar

2015-04-01

295

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; Štefá?ková, Šárka

2014-10-01

296

Factors influencing immune complex localisation  

Microsoft Academic Search

In systemic immune complex (IC) diseases such as SLE and rheumatoid vasculitis, IC accumulate in a number of tissues, either after deposition from the circulation or from in situ formation. The tissue localisation of IC depends on a delicate balance between the production of IC and the ability of the mononuclear phagocytic system (MPS) to remove them from blood. At

L. Schrieber; R. Penny

1984-01-01

297

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

298

BULLOUS PRESENTATION OF HENOCH-SCHÖNLEIN PURPURA IN AN ADULT  

Microsoft Academic Search

Henoch-Schönlein purpura is a small vessel vasculitis occurring mainly in childhood and rarely in adulthood. Typical cutaneous eruption may begin as macular or urticarial erythematous lesions progressing to a palpable purpura. In adults, the dis - ease has a propensity to be more severe and chronic and affects mainly the ankles and feet. Bullae, vesi - cles and ulcers are

Fouad EL SAYED; Rola DHAYBI; Alfred AMMOURY

299

Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): A concomitant disease or a feature of FMF?  

Microsoft Academic Search

Background: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. Objectives: To determine the special characteristics and the prognosis of PAN

Seza Ozen; Eldad Ben-Chetrit; Aysin Bakkaloglu; Hanan Gur; Keriman Tinaztepe; Meral Calguneri; Cetin Turgan; Aydin Turkmen; Ilkser Akpolat; Murat Danaci; Nesrin Besbas; Tekin Akpolat

2001-01-01

300

Anticardiolipin Antibodies in Myelodysplastic Syndromes  

Microsoft Academic Search

The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic stem cell disorders characterized by bone marrow dysplasia, chronic cytopenias and a high propensy for evolution into acute leukemia. A spectrum of immunological abnormalities has been reported in MDS, including cutaneous vasculitis, peripheral neuropathy and lupus-like syndromes [1]. Laboratory studies may reveal raised or reduced levels of serum immunoglobulins,

Izidore S. Lossos; Yaacov Matzner

1999-01-01

301

Therapy for vasculitic neuropathies.  

PubMed

The term vasculitis refers to a pathologic condition defined by inflammatory cell infiltration and destruction of blood vessels. Systemic vasculitis is classified as primary (eg, polyarteritis nodosa, Churg-Strauss syndrome) or secondary, the latter associated with connective tissue disorders, infections, medications, and rarely, as a paraneoplastic phenomenon. Neuropathy is a common complication of systemic vasculitis and is related to ischemic nerve fiber damage with axon loss. Peripheral neuropathy may be the sole manifestation of vasculitis, a condition termed nonsystemic vasculitic neuropathy (NSVN). Treatment of vasculitic neuropathy requires long-term immunosuppressive therapies with potential side effects. The diagnosis of vasculitis should be established by tissue (preferably nerve) biopsy. High-dose prednisone is the standard platform therapy for patients with systemic and NSVN; for those with systemic vasculitis, at least 3 to 12 months of treatment with cyclophosphamide (monthly intravenous pulse or daily oral therapy) is also necessary to sustain remission and allow successful prednisone tapering. The use of cyclophosphamide in patients with NSVN is controversial, but recent retrospective data suggest that those treated with prednisone and cyclophosphamide from the outset fare better than those initially treated only with prednisone. If prednisone is administered as monotherapy, cyclophosphamide should be added after several months if there is no improvement or relapse occurs with tapering of prednisone. Intravenous pulse and daily oral cyclophosphamide probably offer similar efficacy, although the risk of complications is greater with oral therapy. Azathioprine can be safely substituted for cyclophosphamide after 3 months without an increased relapse rate. Azathioprine, methotrexate, intravenous immune globulin, mycophenolate mofetil, plasma exchange, and rituximab can be offered to patients who are intolerant or have a contraindication to cyclophosphamide. However, efficacy is unproven for any of these therapies. Interferon-alpha, sometimes combined with plasma exchange, is used to treat vasculitis associated with hepatitis B infection. Some patients also may improve with corticosteroids. The classification of diabetic lumbosacral radiculoplexus neuropathy as a vasculitic disorder remains controversial. However, there is compelling pathological evidence that this condition represents a T-cell-mediated microvasculitis. Some patients treated with intravenous corticosteroids may have greater recovery and improved pain control. PMID:16464407

Gorson, Kenneth C

2006-03-01

302

Epidemiology: Most common in children with over half less than 5 yrs old. Disease is more severe in older children and adults, especially with regards to renal involvement. Often follows an upper respiratory infection but a recent study suggests that an occult malignancy may be the cause. Pathophysiology: Correlation with recent illness suggests an antigenic precipitant to the ensuing vasculitis. Very similar to IgA nephropathy and will see IgA deposits in skin and kidneys on biopsy. Interestingly, pts with renal involvement also have IgG antibodies against mesangial cells  

Microsoft Academic Search

Treatment: Most recover on their own without treatment. Dapsone may help articular, GI, and skin disease. In those with renal disease, particularly if it is severe, steroids (methylprednisolone followed by 3 months of oral prednisone at 1mg\\/kg\\/d) are often given. Other therapies (usually in conjuction with steroids) that have been tried are cyclophosphamide, azathioprine, plamapheresis, and IVIG; none have been

Henoch-Schonlein Purpura

303

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

304

Host modifier genes affect mouse autoimmunity induced by the lpr gene.  

PubMed Central

A defect in apoptotic signal transmission through CD95 is an essential genetic mechanism for lymphoproliferation and autoimmunities in lpr or gld mice. However, disease manifestations are largely affected by the host genetic background. To identify and map such host genes modifying lpr gene effect, ie, the lpr modifier (Lprm) genes, 82 MRL/lpr x (MRL/lpr x C3H/lpr) F1 mice were subjected to immunopathological and genetical analyses. High-grade vasculitis and glomerulonephritis among backcross mice were observed in separate groups of mice. Microsatellite analysis revealed that there were two host genes affecting the occurrence of vasculitis, Lprm1 (chromosome 4) and Lprm2 (chromosome 3). A recessive MRL allele at Lprm1 enhanced vasculitis to occur in both sexes, whereas that of Lprm2 inhibited its development selectively in females. Genotype combinations of these two genes explained the severity of vasculitis in crosses of MRL/lpr and C3H/lpr mice and also the vasculitis-prone recombinant inbred strain McH5/lpr. A recessive MRL allele at Lprm3 (chromosome 14) suppressed glomerulonephritis. The weight of the spleen was increased by a recessive MRL allele at Lprm4 (chromosome 5) yielding a logarithm of odds score of 2.02 in a quantitative trait locus analysis. In contrast, the weight of axillary lymph nodes was increased by a recessive MRL allele at a locus on chromosome 2, but its presence was not supported by the quantitative trait locus analysis. The titer of anti-dsDNA autoantibody was controlled by the locus Lprm5 on chromosome 16, which had an logarithm of odds score of 3.41. Possible candidate genes for Lprm genes deduced from their map locations are discussed and compared with the autoimmunity genes reported thus far. In conclusion, autoimmune disease manifestations by the lpr mutation are affected by multiple host genes separately. PMID:9403730

Wang, Y.; Nose, M.; Kamoto, T.; Nishimura, M.; Hiai, H.

1997-01-01

305

Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil.  

PubMed

Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis. Renal involvement in HSP is one of the major causes of chronic renal failure in children. It is important to start effective and relatively safe medication to prevent end-stage renal disease (ESRD). Mycophenolate mofetil (MMF) appears to be a promising therapeutic agent in many autoimmune diseases such as lupus nephritis and vasculitis. Herein, we describe the treatment with MMF of three patients with HSP nephritis. In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed. In another patient with HSP nephritic-nephrotic syndrome who showed resistance to steroid, MMF offered a favorable effect. MMF seems to be a promising therapeutic agent in the treatment of the severe HSP nephritis. PMID:24969202

Nikibakhsh, Ahmad Ali; Mahmoodzadeh, Hashem; Karamyyar, Mohamad; Hejazi, Sasan; Noroozi, Mehran; Macooie, Ali Agayar

2014-07-01

306

[Treatment of Behçet's disease].  

PubMed

Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, vascular, neurological, articular, and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, non-steroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapses, sight threatening eye disease, or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD. PMID:24438860

Comarmond, C; Wechsler, B; Cacoub, P; Saadoun, D

2014-02-01

307

Churg-Strauss Syndrome Presented With Hearing Impairment and Facial Palsy  

PubMed Central

Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis. Cranial nerve involvement is very rare in CSS. A 59-year-old woman had complained of both hearing impairments for eight months and left facial palsy for three months. Left facial and cochlear neuropathies were detected in electrodiagnostic studies. Paranasal sinus computed tomography (CT) showed chronic pansinusitis. Chest CT revealed eosinophilic infiltration in the right upper lobe. Tissue biopsy of the right inferior turbinate displayed necrotizing vasculitis with eosinophilic infiltration. She was diagnosed as CSS, based on the presence of eosinophilia, pulmonary infiltration, paranasal sinusitis, and biopsy containing blood vessels with extravascular eosinophils. She was treated with intravenous and oral steroids and azathioprine, showing relatively good prognosis on facial palsy and hearing impairment. We report a very rare case of CSS presented with hearing impairment and facial palsy. PMID:25566487

Byun, Jeong-Hyun; Lee, Jong-Hoo

2014-01-01

308

Experimental posterior uveitis. I: A clinical, angiographic, and pathological study.  

PubMed Central

The clinical, angiographic, and histopathological features of experimental posterior uveitis in the black hooded Lister rat are described. This mild form of experimental allergic uveoretinitis (EAU) is induced by sensitisation with retinal S antigen in Freund's complete adjuvant, and the inflammation produced is confined to the posterior segment of the eye. This allows for the first time precise photographic and angiographic documentation of the evolution of clinical signs, because there is minimal clouding of the vitreous by inflammatory cells. Clinically the disease is characterised by the appearance of disc oedema and periphlebitis, followed by focal infiltrates in the deep retinal layers, with eventual atrophy of the pigment epithelium. Histologically, retinal vasculitis is associated with focal mononuclear cell infiltration and necrosis of the photoreceptor layers. This model closely resembles the clinical features of idiopathic retinal vasculitis seen in man. Images PMID:3651374

Stanford, M R; Brown, E C; Kasp, E; Graham, E M; Sanders, M D; Dumonde, D C

1987-01-01

309

Neurorickettsioses: A Rare Presentation with Stroke in a Young Adult  

PubMed Central

Acute stroke-like presentations due to rickettsial infections have been sparsely reported in literature. We report a young patient who presented with high grade fever and acute encephalopathy with right hemiplegia. CT head showed left cerebral, bilateral thalamic and midbrain infarcts. The stroke in young work-up for conventional and non-conventional risk factors including few infections known to cause cerebral vasculitis was negative. He did not respond to empirical antimalarials and antibiotics. With a high index of suspicion of his ‘febrile cerebrovasculitis’ like presentation, serological test for rickettsia was done and found to be positive. He responded to doxycycline. This case expands the spectrum of presentation of neurorickettsioses and highlights the importance of considering rickettsial vasculitis as one of the potentially treatable causes of infections causing stroke. PMID:25478386

K, Pramod

2014-01-01

310

[Systemic vasculitides: novel nomenclature and novel therapeutic approaches].  

PubMed

A novel nomenclature of systemic vasculitides is proposed by the 2012 Chapel Hill Consensus Conference. It aims at substituting established eponyms and introducing new terms and groups closer to our current understanding of vasculitis pathophysiology. In parallel, a therapeutic revolution is taking place partially based on the concept of re-induction of immune tolerance for ANCA-associated vasculitis (AAV). Two major multicentric randomized studies have shown that rituximab (RTX), monoclonal antibody capable of selectively killing B lymphocytes, is not inferior when compared to cyclophosphamide (CYC) to induce remission in AAV, and superior in the case of disease relapse. Thus, a hot debate is taking place whether or not to maintain CYC or use RTX in AAV. An individual-based choice may be wise for the moment being. PMID:24834643

Allali, D; Chizzolini, C

2014-04-16

311

Necrolytic acral erythema: an expanding spectrum.  

PubMed

Hepatitis C virus (HCV) infection is the most common chronic blood-borne viral infection in the United States. Well-described cutaneous manifestations of HCV infection include polyarteritis nodosa, porphyria cutanea tarda, type II cryoglobulinemia-associated vasculitis, pruritus, erythema nodosum, urticaria and urticarial vasculitis, lichen planus, and erythema multiforme. First described in 1996, necrolytic acral erythema (NAE) is now recognized as a cutaneous acral eruption uniquely associated with HCV infection. Most patients present with chronic, acral, erythematous, and psoriasiform lesions. Acute presentations of NAE are rare and patients may present with atypical clinical features; in these cases, suspicion for HCV infection may be delayed for weeks to months until more classic chronic lesions develop. In many cases, NAE presents before the patient has been diagnosed with HCV infection, which allows dermatologists the unique opportunity to suspect and diagnose HCV infection based on skin findings alone. PMID:20166571

Halpern, Analisa V; Peikin, Steven R; Ferzli, Pascal; Heymann, Warren R

2009-12-01

312

Cerebral aneurysms associated with Behçet's disease: a case report  

PubMed Central

Cerebral aneurysms in Behçet's disease are very rare. The role of vasculitis in the aetiology of these aneurysms has not been clarified. A 57 year old man with Behçet's disease is described, who had a subarachnoid hemorrhage due to rupture of a peripheral middle cerebral artery aneurysm. He underwent a successful aneurysmal clipping. Three years later he had seizures and was found to have a new aneurysm on the contralateral peripheral middle cerebral artery as well as some radiological features of vasculitis. After 3 months of steroid therapy, the aneurysm disappeared. Although surgical treatment is the first choice for ruptured aneurysms, steroid therapy may be effective for unruptured small aneurysms in patients with Behçet's disease.?? PMID:11309468

Nakasu, S; Kaneko, M; Matsuda, M

2001-01-01

313

A presentation of massive hemoptysis in a patient with Churg-Strauss syndrome.  

PubMed

Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis. When involving the lungs, small-vessel vasculitides typically cause capillaritis, leading to diffuse alveolar hemorrhage and submassive hemoptysis. In contrast, massive hemoptysis primarily originates from the bronchial arteries; therefore, small-vessel vasculitis is not considered when a patient presents with massive hemoptysis. The authors describe a patient with CSS who presented with the novel finding of massive hemoptysis. Computed tomography scans lacked alveolar infiltrates and bronchoalveolar lavage lacked hemosiderin-laden macrophages. Bronchoscopy demonstrated a raised mucosal lesion in the right mainstem bronchus and computed tomography angiogram revealed aberrant dilated bronchial arteries underlying the same region, suggesting this as the source of the hemoptysis. To the authors' knowledge, the present report describes the first reported case of CSS to present with massive hemoptysis with likely involvement of the bronchial arterial circulation. CSS should be considered in patients with unexplained massive hemoptysis. PMID:25255461

Hikmat, Fadi; Pearse, David B; Damarla, Mahendra

314

Coronary involvement in Churg-Strauss syndrome: a case report with CT findings.  

PubMed

We report a case of Churg-Strauss syndrome (CSS) associated with coronary artery involvement, as demonstrated on coronary CT angiography (CCTA), without specific cardiac symptoms. A 69-year-old male had an 8-year history of bronchial asthma and chronic sinusitis with hypereosinophilia (35 %), polyneuropathy, and a positive antineutrophil cytoplasmic antibody titer, so he was diagnosed with CSS. The patient had no specific cardiac symptoms, but CCTA showed vasculitis and a saccular aneurysm involving the proximal coronary arteries. The 3-year follow-up CCTA demonstrated an increase in the extent of soft-tissue wall thickening and infiltration involving the coronary arteries. Although vasculitis of the major coronary arteries is not a prominent feature of CSS, our case suggests that the coronary arteries may also be targeted in this syndrome. PMID:24155026

Doo, Kyung Won; Yong, Hwan Seok; Kang, Eun-Young

2013-12-01

315

Bilateral Facial Palsy in Rapidly Progressive Course of Wegener's Granulomatosis: A Case Report  

PubMed Central

Introduction. Wegener's granulomatosis belongs to a group of systemic vasculitis diseases, which is characterized by necrotizing vasculitis and presence of granulomas. In a lot of cases, the first symptoms of the disease are observed in the head and neck region, but the bilateral facial nerve palsy occurs very rarely. Objective. The objective of our report was to describe the unusual course of Wegener's granulomatosis with the bilateral facial nerve paralysis, which subsided after application of steroids and immunosuppressive therapy in combination with surgical treatment. Results and Conclusions. Hearing loss may precede other symptoms in Wegener's granulomatosis. Ear pain and otorrhea may suggest the diagnosis of bacterial purulent otitis media and delay the proper diagnosis. In the presented case, considering the clinical course, it was necessary to apply both pharmacological and surgical treatments. PMID:24187641

Roszkowska, Anna; Morawska-Kochman, Monika; Temporale, Hanna; Sikorska-?uk, Ma?gorzata; Kr?cicki, Tomasz

2013-01-01

316

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

317

Extrahepatic manifestations of HCV infection: A brief review and update  

Microsoft Academic Search

Hepatitis C affects approximately 3% of the population worldwide and extrahepatic manifestations may occur in nearly 40% of\\u000a all patients. Recent efforts have focused on incidence, prevalence, clinical significance, mechanism of disease, and the role\\u000a of antiviral therapy in treatment. Cryoglobulinemia, cryoglobulinemic vasculitis, membranoproliferative glomerulonephritis,\\u000a and lymphoproliferative disorders have a well-established link to hepatitis C, and many other endocrine, autoimmune,

Nidhi Singh; Nancy Reau

2006-01-01

318

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

319

Serum homocysteine level is increased and correlated with endothelin-1 and nitric oxide in Behc?et's disease  

Microsoft Academic Search

Background\\/aims: Behc?et's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown aetiology, characterised by endothelial dysfunction and occlusion in both deep venous and retinal circulation. Ocular involvement occurs in 70% of cases and is characterised by periphlebitis, periarteritis, vascular occlusion, and thrombosis leading to blindness despite vigorous treatment. Endothelin-1 (ET-1) is a vasoconstricting peptide while nitric oxide

H Er; C Evereklioglu; T Cumurcu; Y Tu?rko?z; E O?zerol; K S?ahin; S Doganay

2002-01-01

320

Proteinase3 as the major autoantigen of c-ANCA is strongly expressed in lung tissue of patients with Wegener's granulomatosis  

Microsoft Academic Search

Proteinase-3 (PR-3) is a neutral serine proteinase present in azurophil granules of human polymorphonuclear leukocytes and serves as the major target antigen of antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA) in Wegener's granulomatosis (WG). The WG disease appears as severe vasculitis in different organs (e.g. kidney, nose and lung). Little is known about the expression and distribution of

Holger Brockmann; Andreas Schwarting; Jörg Kriegsmann; Peter Petrow; Andreas Gaumann; Klaus-Michael Müller; Peter Robert Galle; Werner Mayet

2002-01-01

321

Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome  

PubMed Central

The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

Saatci, Ali Osman; Ayhan, Ziya; Take?, Ömer; Yaman, Aylin; Bajin, F. Meltem Söylev

2015-01-01

322

[Rare complication in a patient with giant cell arteritis].  

PubMed

Giant cell arteritis (temporal arteritis) is a chronic vasculitis that usually affects older people. Although this is a systemic disease, it most often affects the cranial arteries. The most frequent complication of this disorder is visual loss. We report the case of a patient who suffered several rare complications, including tongue necrosis, as a result of being diagnosed with giant cell arteritis following the start of treatment. PMID:21233870

Olivera, S; Gonzalez, S Olivera; Amores, B; Arriaga, B Amores; Torralba, M A; Cabeza, M A Torralba; Pérez Calvo, J I; Calvo, J I Pérez

2010-01-01

323

A new antibody in rheumatoid arthritis targeting glycated IgG: IgM anti- IgG-AGE  

Microsoft Academic Search

SUMMARY Hyperglycaemia and\\/or oxidative stress can cause IgG to be modified by advanced glycation end products (AGE). Three patients with aggressive rheumatoid arthritis (RA) and vasculitis are described who have high titres of IgM antibodies against AGE-modified IgG (IgM anti-IgG-AGE ). Diabetics and randomly selected patients with rheumatic diseases, including 50 additional RA patients, were tested for IgM and IgA

S. LIGIER; P. R. FORTIN; M. M. NEWKIRK

1998-01-01

324

Palpable purpura with foot drop: Common presentations in an uncommon disease.  

PubMed

Churg-Strauss syndrome is a rare disease manifested by hypereosinophilia, vasculitis and tissue infiltration. This report describes the case of a 45-year-old man who presented with a history of fever, difficulty in breathing, reddish lesions over the extremities and inability to walk since two weeks. The cutaneous features prompted us to conduct serial lab investigations which led to an early, potentially life saving diagnosis. PMID:25821734

Mathanda, Teena Ramesh; Bhat, Ramesh; Alva, Jayaprakash

2015-01-01

325

Anti-MPO-ANCA-Positive Microscopic Polyangiitis following Subacute Bacterial Endocarditis  

Microsoft Academic Search

:   Although infectious agents such as Staphylococcus aureus have been implicated in the pathogenesis of Wegener’s granulomatosis, the role of bacterial infections in the pathogenesis\\u000a of other types of small-vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) is less clear. We\\u000a describe a patient who developed a non-granulomatous necrotising small vessel vasculitis and perinuclear ANCA (p-ANCA) directed\\u000a against myeloperoxidase (MPO)

B. Hellmich; M. Ehren; M. Lindstaedt; M. Meyer; M. Pfohl; H. Schatz

2001-01-01

326

Association of HLA-DRB1*0901-DQB1*0303 haplotype with microscopic polyangiitis in Japanese  

Microsoft Academic Search

Microscopic polyangiitis (MPA) is a rare and severe form of systemic necrotizing vasculitis associated with myeloperoxidase (MPO)-specific antineutrophil cytoplasmic antibody (ANCA). We previously reported significant association of HLA-DRB1*0901 with MPA. To define the susceptibility loci within the HLA region, we determined the genotypes of HLA-DQB1, DPB1, B and C in 50 patients with MPA and 77 unrelated Japanese controls. In

N Tsuchiya; S Kobayashi; H Hashimoto; S Ozaki; K Tokunaga

2006-01-01

327

Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis  

Microsoft Academic Search

Microscopic polyangiitis is a vasculitis which primarily affects capillaries, venules or arterioles. Involvement of small\\u000a and medium-sized arteries may also occur. A 70-year-old Japanese female with a fever and cough was diagnosed with pneumonia\\u000a and antibiotics were administered. Her symptoms initially improved, but her fever recurred and she experienced malaise and\\u000a loss of appetite. Her renal function gradually worsened and

Kensei Yahata; Chinatsu Okamoto; Hirotaka Imamaki; Koichi Seta; Tsuyoshi Terashima; Sachiko Minamiguchi; Akira Sugawara

2009-01-01

328

Polyarteritis nodosa and microscopic polyangiitis: Etiologic and diagnostic considerations  

Microsoft Academic Search

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized vessels with clinical manifestations resulting\\u000a from ischemia and infarction of affected tissues and organs. Although the cause of most cases of PAN and the related disorder\\u000a microscopic polyangiitis (MPA) remains largely unknown, there has been significant progress in understanding the pathogenesis\\u000a of vascular inflammation. The diagnostic approach to PAN and

Laura B. Hughes; S. Louis Bridges

2002-01-01

329

Spontaneous Duodenal Perforation as a Complication of Kawasaki Disease  

PubMed Central

Kawasaki disease is generally known as a systemic vasculitis that often concerns doctors due to its serious cardiac complications; however, other visceral organs may get involved as well. Surgical manifestations of the intestinal tract in Kawasaki disease are rare. In this report, we describe the case of a 2.5-year-old boy with typical Kawasaki disease who presented with GI bleeding and surgical abdomen. The diagnosis of duodenal perforation was confirmed.

Forouzan, Arash; Saidi, Hossein; Javaherizadeh, Hazhir; Khavanin, Ali; Bahadoram, Mohammad

2015-01-01

330

The current status of neutrophil cytoplasmic antibodies.  

PubMed Central

Several studies in the past 10 years have demonstrated the occurrence of autoantibodies against cytoplasmic constituents in patients with vasculitis and glomerulonephritis. In this review the nomenclature of these antibodies is discussed and assays and clinical associations are summarized. Although the antigens involved are not completely identified, antibodies and T cells reactive with myeloid lysozomal enzymes may both play a significant role in pathogenesis. PMID:12412739

van der Woude, F J; Daha, M R; van Es, L A

1989-01-01

331

Indium-111 leukocyte scintigraphy in Wegener's granulomatosis involving the spleen  

SciTech Connect

Indium-111-labeled leukocyte scintigraphy was performed on a 44-yr-old man to exclude an occult abscess. Four- and twenty-four-hour images of the abdomen revealed splenic photopenia except for a rim of activity medially. A subsequent computed tomography (CT) study demonstrated necrosis or hemorrhage of the spleen except for a medial rim. Exploratory laparotomy demonstrated necrotizing vasculitis with granuloma formation consistent with Wegener's granulomatosis and a rim of viable splenic tissue corresponding to the radionuclide and CT studies.

Morayati, S.J.; Fink-Bennett, D.

1986-12-01

332

Evaluation of current therapeutic strategies in Behçet’s disease  

Microsoft Academic Search

Behçet’s disease (BD) is a chronic relapsing vasculitis with multifunctional pathogenesis. The mucocutaneous and ocular lesions\\u000a are the commonest manifestations, but BD also affects the musculoskeletal, intestinal, cardiac, and central nervous system.\\u000a BD therapy is based on the suppression of the inflammatory process, using immunomodulating and immunosuppressive agents. In\\u000a selected cases, invasive procedures may be required.

Iliana Alexoudi; Violetta Kapsimali; Aristides Vaiopoulos; Meletios Kanakis; George Vaiopoulos

2011-01-01

333

Henoch–Schönlein purpura: a clinical case with dramatic presentation  

Microsoft Academic Search

A case study involving a 55-year-old Caucasian male with end-stage glomerulosclerosis is presented here. Kidney biopsies showed no deposits on imunofluorescent microscopy. Relapsing massive haemoptysis and suspected bronchovascular malformation required lung lobectomy which revealed malformative and tortuous small blood vessels, with no vasculitis. Blood antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were undetectable. Seric immunoglobulins and complement

João Bento; Adriana Magalhães; Conceição Souto Moura; Venceslau Hespanhol

2010-01-01

334

Bullous Fixed Drug Eruption to Ciprofloxacin: A Case Report  

PubMed Central

Adverse reactions to medications are extremely common and display a characteristic clinical morphology such as fixed drug eruption (FDE), Stevens-Johnson syndrome, urticaria, morbilliform exanthem, hypersensitivity syndrome, pigmentary changes, lichenoid, dermatitis, acute generalized exanthematous pustulosis, photosensitivity, vasculitis etc. Here we report a case of a 60 year old male who presented to us with multiple bullous eruptions over both the hands and feet after oral ingestion of ciprofloxacin. PMID:23730666

Jain, Sonia Pramod; Jain, Pramod Ajit

2013-01-01

335

Role of antineutrophil cytoplasmic antibodies and glomerular basement membrane antibodies in the diagnosis and monitoring of systemic vasculitides.  

PubMed

Systemic vasculitis, although rare, is often diagnosed late and long after the onset of symptoms. The small vessel vasculitides are recognized clinically by their multisystem presentation, markers of inflammation and evidence for an acute glomerulonephritis (GN), with the most apparent organ involved directing referral to secondary care. Routine laboratory tests are usually non-specific in systemic vasculitis but the use of anti-neutrophil cytoplasmic antibodies (ANCAs) and glomerular basement membrane (GBM) antibodies can aid diagnosis, treatment and monitoring decisions. These antibodies are detected and quantified by indirect immunofluorescence (IIF) and antigen-specific enzyme-linked immunosorbent assay (ELISA), usually in combination for ANCA, and ELISA systems (or direct IIF on kidney biopsy) for GBM antibodies. The presence or absence of ANCA does not confirm or exclude the diagnosis of systemic vasculitis but negative and positive predictive values will be strongly influenced by clinical presentation. Various large studies have been unable to conclude that following serial ANCA titres has great clinical utility in each case but each patient must be considered on its own merits; for example the reappearance of ANCA in a patient who was rendered ANCA negative following treatment is more likely to indicate relapse. The adoption of consensus guidelines that direct testing towards patients with rapidly progressive GN, pulmonary haemorrhage, persistent and destructive ear, nose and upper airways problems, such as subglottic tracheal stenosis, a retro-orbital mass and cutaneous vasculitis with systemic features or peripheral neuropathy, will greatly increase the clinical utility and positive predictive value of these tests. PMID:17761028

Sinclair, David; Stevens, Judith M

2007-09-01

336

Prevalence and Functional Role of Anti-IgE Autoantibodies in Urticarial Syndromes  

Microsoft Academic Search

The prevalence of autoantibodies of immunoglobulin G (IgG) and immunoglobulin M (IgM) classes directed against myeloma immunoglobulin E (IgE) were determined in distinct subsets of urticaria, using an enzyme immunoassay. IgG anti-IgE antibodies were found in five of nine patients (55%) with cold urticaria, four of eight patients (50%) with urticarial vasculitis, and tree of six patients (50%) with chronic

Barry L. Gruber; Maria L. Baeza; Mary J. Marchese; Vincent Agnello; Allen P. Kaplan

1988-01-01

337

Takayasu’s arteritis associated with Crohn’s disease  

Microsoft Academic Search

Takayasu’s arteritis (TA), also known as the “pulseless disease,” is a chronic vasculitis of the aorta and aortic branches.\\u000a TA with Crohn’s disease is rare and has not been documented in China before. In this paper we report on a case of Takayasu’s\\u000a arteritis associated with concurrent Crohn’s disease. A 17-year-old Chinese male developed upper limb sourness and a sensation

You-shi Liu; You-hong Fang; Ling-xiang Ruan; You-ming Li; Lin Li; Ling-ling Jiang

2009-01-01

338

Role of Advanced MRI Brain Sequences in Diagnosing Neurological Complications of Scrub Typhus  

PubMed Central

Scrub typhus is a rare disease affecting many organs and causing vasculitis by affecting the endothelium of blood vessels. Review of literature shows that there are only a few case reports describing the neuroradiological manifestations of scrub typhus. This case report describes how newer and advanced MRI sequences are able to diagnose neurological complications of scrub typhus, such as hemorrhages, meningoencephalitis, infarctions, cranial nerve involvement, thrombosis, and hypoperfusion, that are not picked up on routine magnetic resonance imaging (MRI) sequences.

Sood, Shikha; Sharma, Sanjeev; Khanna, Shweta

2015-01-01

339

Henoch - Schönlein Purpura, a Rare Disease in Pregnancy  

Microsoft Academic Search

Introduction: Henoch-Schönlein purpura is an IgA-mediated small vessel vasculitis involving mainly skin, gastrointestinal system and kidneys. It is predominantly a disease of young children. Most common symptoms are rash (95-100%), sometimes necrotizing involving specially the legs, subcutaneous oedema (20-50%), abdominal pain and vomiting(85%), bloody stool and joint pain (60-80%) involving mainly the knees and ankles. Diagnosis is clinical and not

TABASSUM PARVEEN; FIROZA BEGUM

2008-01-01

340

Vascularite restreinte au système nerveux périphérique : présentation clinique atypique  

Microsoft Academic Search

Introduction. – Peripheral neuropathy is a common feature of many vasculitic syndromes. In some patients, the neuropathy may be the sole manifestation of vasculitis.Exegesis. – A 74-year-old lady complained of pain and weakness of the lower limbs. In her history, we noted right optic neuritis, monoclonal gammopathy and dyslipidemia treated by fenofibrate. Clinical examination showed proximal muscle strength deficit of lower limbs,

V Marcaud; C Gauthier; T Maisonobe; J Hogenhuis; R Morizot-Koutlidis; C. F Degos

2002-01-01

341

Detection of inflammatory processes during various diseases by the method of flow cytofluorometry.  

PubMed

Oxidative (respiratory) burst is an important manifestation of inflammation. Precise quantitative assessment of this reaction by flow cytometry made it possible to record and evaluate the severity of the inflammatory processes in a wide spectrum of diseases including diphtheria, hepatitis, pneumonia, bronchial asthma, arthritis, vasculitis, postoperative complications, tuberculosis, psoriasis, rheumatoid arthritis, systemic lupus erythematosus, and myocardial infarction. This approach can be employed as a highly sensitive method of detection of inflammatory reactions and monitoring of their course in various pathological processes. PMID:21234449

Varfolomeeva, E Yu; Ivanov, E I; Drobchenko, E A; Semenova, E V; Filatov, M V

2010-10-01

342

Detection of Inflammatory Processes during Various Diseases by the Method of Flow Cytofluorometry  

Microsoft Academic Search

Oxidative (respiratory) burst is an important manifestation of inflammation. Precise quantitative assessment of this reaction\\u000a by flow cytometry made it possible to record and evaluate the severity of the inflammatory processes in a wide spectrum of\\u000a diseases including diphtheria, hepatitis, pneumonia, bronchial asthma, arthritis, vasculitis, postoperative complications,\\u000a tuberculosis, psoriasis, rheumatoid arthritis, systemic lupus erythematosus, and myocardial infarction. This approach can

E. Yu. Varfolomeeva; E. I. Ivanov; E. A. Drobchenko; E. V. Semenova; M. V. Filatov

2010-01-01

343

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

344

[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

345

Palpable purpura with foot drop: Common presentations in an uncommon disease  

PubMed Central

Churg–Strauss syndrome is a rare disease manifested by hypereosinophilia, vasculitis and tissue infiltration. This report describes the case of a 45-year-old man who presented with a history of fever, difficulty in breathing, reddish lesions over the extremities and inability to walk since two weeks. The cutaneous features prompted us to conduct serial lab investigations which led to an early, potentially life saving diagnosis.

Mathanda, Teena Ramesh; Bhat, Ramesh; Alva, Jayaprakash

2015-01-01

346

Assessment of alveolar epithelial permeability in Behçet’s disease with 99m Tc-DTPA aerosol scintigraphy  

Microsoft Academic Search

Objective  Behçet’s disease (BD) is a multisystem disorder characterized by vasculitis, and consists of a triad of recurrent ulcers of\\u000a the oral and genital mucosa with relapsing uveitis. The prevalance of pulmonary involvement varies in the range of 1–10% in\\u000a various studies and its complications are severe and life threatening. In this study, we investigated the changes of pulmonary\\u000a epithelial permeability

Fikriye G. Gumuser; Timur Pirildar; Dilek Batok; Ays?n Sakar; Ebru Ruksen; Elvan Sayit

2008-01-01

347

Microarray Studies of Gene Expression in Circulating Leukocytes in Kidney Diseases  

Microsoft Academic Search

The molecular characterization of changes in mRNA expression in renal tissue during disease is hampered by the acquisition of sufficient mRNA to do genomewide expression profiling. In many renal diseases, such as systemic lupus erythematosus, IgA nephropathy, antineutrophil cytoplasmic autoantibody (ANCA) associated glomerulonephritis, and small-vessel vasculitis (ANCA disease), circulating leukocytes play a role in onset, progression, and severity of the

David Alcorta; Gloria Preston; William Munger; Pamela Sullivan; Jia Jin Yang; Iwao Waga; J. Charles Jennette; Ronald Falk

2002-01-01

348

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

349

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

350

Churg-Strauss syndrome associated with montelukast therapy  

Microsoft Academic Search

Churg-Strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. There have been several recent case reports of the condition in association with leukotriene antagonists and it has been speculated that the Churg-Strauss syndrome was unmasked when oral corticosteroids were withdrawn. We report a case of Churg-Strauss syndrome associated with montelukast therapy in an asthmatic patient in whom

J M Tuggey; H S R Hosker

2000-01-01

351

Evaluation of the ameliorative effects of immunosuppressants on crescentic glomerulonephritis in SCG\\/Kj mice  

Microsoft Academic Search

The therapeutic efficacy of immunosuppressants for treating rapidly progressive glomerulonephritis (RPGN) with crescent formation remains controversial. SCG\\/Kj mice spontaneously develop RPGN-like symptoms, characteristic of crescentic glomerulonephritis and systemic small vessel vasculitis, associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). We evaluated the “ameliorative”, not prophylactic, effects of immunosuppressive agents, deoxyspergualin (DSG), cyclophosphamide (CYC) and prednisolone (PDN), on RPGN in

Kan Saiga; Minako Yoshida; Iwao Nakamura; Eriko Toyoda; Kazuhiro Tokunaka; Hirohisa Morohashi; Fuminori Abe; Kyuichi Nemoto; Masato Nose

2008-01-01

352

Clinical and surgical therapeutic approach in erithema elevatum diutinum--case report.  

PubMed

Erythema elevatum diutinum is a rare chronic cutaneous vasculitis which usually affects adults. It is characterized by symmetrical and persistent papules, plaques, and nodules. These lesions are usually located on the extensor surfaces of the extremities. We report a case of erythema elevatum diutinum in which the association of dapsone and surgical excision promoted complete remission of skin lesions, providing a new approach to the treatments described in the literature. PMID:24346869

Zacaron, Luciana Helena; Gonçalves, Júlia Costa de Faria; Curty, Vanessa Maria Amoreira; D'Acri, Antonio Macedo; Lima, Ricardo Barbosa; Martins, Carlos José

2013-01-01

353

Giant cell arteritis  

PubMed Central

Giant cell arteritis (GCA), temporal arteritis or Horton's arteritis, is a systemic vasculitis which involves large and medium sized vessels, especially the extracranial branches of the carotid arteries, in persons usually older than 50 years. Permanent visual loss, ischaemic strokes, and thoracic and abdominal aortic aneurysms are feared complications of GCA. The treatment consists of high dose steroids. Mortality, with a correct treatment, in patients with GCA seems to be similar that of controls. PMID:13679546

Calvo-Romero, J

2003-01-01

354

Interstitial Lung Disease Induced by Drugs and Radiation  

Microsoft Academic Search

An ever-increasing number of drugs can reproduce variegated patterns of naturally occurring interstitial lung disease (ILD), including most forms of interstitial pneumonias, alveolar involvement and, rarely, vasculitis. Drugs in one therapeutic class may collectively produce the same pattern of involvement. A few drugs can produce more than one pattern of ILD. The diagnosis of drug-induced ILD (DI-ILD) essentially rests on

Philippe Camus; Annlyse Fanton; Philippe Bonniaud; Clio Camus; Pascal Foucher

2004-01-01

355

Clinical utility of peripheral nerve biopsy  

Microsoft Academic Search

Histopathologic evaluation of nerve biopsy specimens provides important diagnostic information in some patients with peripheral\\u000a neuropathy. The role of nerve biopsy is more restricted than that of muscle biopsy. Nerve biopsy is utilized mainly for diagnosis\\u000a of vasculitis and infiltrative neuropathies. It is also utilized in diagnosis of atypical inflammatory demyelinating neuropathies\\u000a in which the clinical, electrodiagnostic, and laboratory features

David Lacomis

2005-01-01

356

Influence of inherited and acquired thrombophilic defects on the clinical manifestations of mixed cryoglobulinaemia  

Microsoft Academic Search

Objective. To investigate the contribution of inherited and acquired thrombophilic defects to the clinical manifestations of mixed cryoglobulinaemia vasculitis. Methods. The following thrombophilic defects were investigated in 64 consecutive patients with HCV-associated mixed cryoglobulinaemia: aPLs, lupus anti-coagulant, homocysteinaemia, protein C and protein S concentrations, activated protein C resistance, plasminogen activator inhibitor-1 4G4G and 5G5G genotypes, and the presence of mutations

M. Casato; M. Carlesimo; A. Francia; C. Timarco; A. Antenucci; M. Bove; H. Martini; M. Visentini; M. Fiorilli; L. Conti

2008-01-01

357

Induction of the Gene Encoding Macrophage Chemoattractant Protein 1 by Orientia tsutsugamushi in Human Endothelial Cells Involves Activation of Transcription Factor Activator Protein 1  

Microsoft Academic Search

Human macrophage chemoattractant protein 1 (MCP-1) is a potent mediator of macrophage migration and therefore plays an essential role in early events of inflammation. In endothelial cells, at least three independent pathways regulate MCP-1 expression by NF-B and AP-1. Orientia tsutsugamushi causes vasculitis in humans by replicating inside macrophages and endothelial cells. In the present study, we investigated the cis-acting

Nam-Hyuk Cho; Seung-Yong Seong; Myung-Sook Huh; Na-Hyun Kim; Myung-sik Choi; Ik-sang Kim

2002-01-01

358

Peripheral neuropathy with essential mixed cryoglobulinemia: biopsies from 5 cases  

Microsoft Academic Search

Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy and IgG polyclonal gammopathy, and the other four had cryoglobulinemia with polyclonal gammopathy. Biopsies showed perivascular infiltration by mononuclaer cells around medium, and mainly small-sized

C. Vital; C. Deminière; A. Lagueny; F. X. Bergouignan; J. L. Pellegrin; M. S. Doutre; A. Clement; J. Beylot

1988-01-01

359

Localization of hepatitis c virus antigens in liver and skin tissues of chronic hepatitis c virus—Infected patients with mixed cryoglobulinemia  

Microsoft Academic Search

Skin and\\/or liver biopsy specimens were obtained from the following patients: 15 anti-hepatitis C virus (HCV), HCV RNA-positive patients and 3 anti-HCV, HCV RNA-negative patients with type II mixed cryoglobulinemia (MC); 7 anti-HCV, HCV RNA-positive patients with chronic active liver disease (CALD); 5 anti-HCV, HCV RNA-negative patients with noncryoglobulinemic vasculitis; and 7 anti-HCV, HCV RNA-negative patients with lichen ruber planus.

Domenico Sansonno; Vito Cornacchiulo; Anna Rina Jacobelli; Rita Di Stefano; Mario Lospalluti; Franco Dammacco

1995-01-01

360

Rupture of Abdominal Aortic Aneurysms in Behçet’s Disease  

Microsoft Academic Search

Behçet’s disease is a systemic disease of unknown etiology with a chronic relapsing course, characterized by oral aphtous, genital ulcers, ocular lesions, and occasionally vasculitis. Major asymptomatic vascular complications should always be considered in patients with Behçet’s disease. We present the surgical treatment of two male Behçet’s patients of 41 and 30 years of age with ruptured infrarenal abdominal aortic

Vedat Erentug; Nilgün Bozbuga; Suat Nail Ömeroglu; Hasan Ardal; Ercan Eren; Murat Güçlü Elevli; Füsun Güzelmeriç; Kaan Kirali; Esat Akinci; Cevat Yakut

2003-01-01

361

Pulmonary-Renal vasculitic disorders: Differential diagnosis and management  

Microsoft Academic Search

Pulmonary-renal syndrome (PRS) is a combination of diffuse pulmonary hemorrhage and glomerulonephritis. Pulmonary-renal syndrome\\u000a is not a single entity and is caused by a variety of conditions, including Goodpasture’s syndrome associated with autoantibodies\\u000a to the glomerular and alveolar basement membranes, various forms of primary systemic vasculitis associated with serum positivity\\u000a for antineutrophil cytoplasmic antibodies (ANCA), cryoglobulinemia, systemic lupus erythematosus, systemic

Luis J. Jara; Olga Vera-Lastra; Maria C. Calleja

2003-01-01

362

Aortic root dilation secondary to giant cell aortitis in a human immunodeficiency virus-positive patient  

Microsoft Academic Search

HIV-associated vasculitis rarely involves the aorta. There is no well-established association of HIV and giant cell arteritis. We present the case of a 31-year-old HIV positive Indian woman who was referred to us with complaints of dyspnea and chest pain. Physical examination revealed a diastolic murmur in the aortic area and echocardiography showed a dilated aortic root causing severe aortic

Muhammad A. Javed; Mary N. Sheppard; John Pepper

2006-01-01

363

Aortic root dilation secondary to giant cell aortitis in a human immunodeficiency virus-positive patient  

Microsoft Academic Search

HIV-associated vasculitis rarely involvestheaorta. Thereis no well-establishedassociationof HIVand giantcell arteritis.We present thecaseof a 31-year-old HIV positive Indian woman who was referred to us with complaints of dyspnea and chest pain. Physical examination revealed a diastolic murmur in the aortic area and echocardiography showed a dilated aortic root causing severe aortic regurgitation. She was being adequately treated with anti-HIV therapy. She

Muhammad A. Javed; Mary N. Sheppard; John Pepper

2010-01-01

364

Schönlein-Henoch purpura during pregnancy with successful outcome for mother and newborn  

Microsoft Academic Search

ABSTRAC: BACKGROUND: Schönlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. CASE PRESENTATION: We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy

Robert Feldmann; Walter Rieger; Paul G Sator; Friedrich Gschnait; Friedrich Breier

2002-01-01

365

Perineurial cells filled with collagen in ‘atypical’ Cogan’s syndrome  

Microsoft Academic Search

Cogan’s syndrome is a rare clinical entity characterized by non-infectious interstitial keratitis with vestibuloauditory dysfunction.\\u000a The clinical course is extremely variable. In the majority of patients, there appears to be an underlying systemic process,\\u000a often a “vasculitis”. We were able to study for the first time a sural nerve biopsy of a 38-year-old female with clinically\\u000a suggested Cogan’s syndrome associated

Kay W. Nolte; Volkmar J. Hans; Christoph Schattenfroh; Joachim Weis; J. Michael Schröder

2008-01-01

366

Ataxia and peripheral neuropathy: rare manifestations in Henoch-Schönlein purpura  

Microsoft Academic Search

.   Henoch-Schönlein purpura (HSP) is a multisystemic vasculitis. Nervous system involvement is usually underestimated. Headaches,\\u000a mental status changes and seizures are the most frequent neurologic symptoms. Ataxia and mononeuropathy are both very rare.\\u000a We present an 11-year-old boy with HSP who suffered from ataxia during the initial presentation and peripheral neuropathy\\u000a at the time of a relapse. Brainstem vasculitic involvement

A. Bulun; R. Topaloglu; A. Duzova; I. Saatci; N. Besbas; A. Bakkaloglu

2001-01-01

367

A Challenging Twist in Pulmonary Renal Syndrome  

PubMed Central

Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09?mg/dL (baseline 0.9). Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed. PMID:25525550

Namas, Rajaie; Rubin, Bernard

2014-01-01

368

Granulomatosis with polyangiitis (Wegener's granulomatosis) in children: report of three cases with cutaneous manifestations and literature review.  

PubMed

Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is a rare disease in childhood. Of 39 GPA patients that we diagnosed during a 20-year period, only 3 (7.7%) were younger than 18 years. We report the course of GPA in three girls whose disease started at the ages of 16, 11, and 6 years. All had cutaneous manifestations: the first had necrotizing vasculitis, the second had palpable purpura, and the third had right upper-eyelid edema and infiltration and proptosis caused by extraocular pseudotumor, initially histologically misdiagnosed as orbital immunoglobulin G4 (IgG4)-related disease. Unlike with skin vasculitis and glomerulonephritis, upper-airway and orbital inflammation were resistant to immunosuppressive therapy. Our report emphasizes that children presenting with cutaneous vasculitis, chronic eyelid swelling, sinusitis, or hoarseness should be tested for antineutrophil cytoplasmic antibodies. We emphasize that the upper-eyelid edema and infiltration, with histologic characteristics of orbital IgG4-related disease, may be the initial presentation of localized GPA in children, a feature that, until now, has been described only in adults. PMID:23145989

Gajic-Veljic, Mirjana; Nikolic, Milos; Peco-Antic, Amira; Bogdanovic, Radovan; Andrejevic, Sladjana; Bonaci-Nikolic, Branka

2013-01-01

369

Development of Canadian Recommendations for the Management of ANCA-Associated Vasculitides: Results of the National Needs Assessment Questionnaire  

PubMed Central

Objectives : To study variations in Canadian clinical practice patterns for the management of ANCA-associated vasculitis (AAV) and identify points to consider for the development of national recommendations. Material and Methodology : A 30-item needs assessment questionnaire was sent to all members of the Canadian Vasculitis network (CanVasc), Canadian Rheumatology Association (CRA), Canadian Thoracic Society (CTS) and Canadian Society of Nephrology (CSN). Respondent characteristics, practice patterns, concerns and expectations were analyzed. Results : Among 132 physicians who followed at least 1 vasculitis patient and responded to the survey, 39% stated that they felt confident in their management of AAV. Several variations in practice were observed regarding diagnostic procedure, induction and maintenance treatments and use of biologics; some were due to logistic constraints (difficulties in access to some specific tests, drugs or care; lack of health care coverage for the costs). The top 5 topics for which recommendations are expected involve treatment for remission induction, maintenance, refractory disease, and relapse as well as biologics. Conclusion : Practice variations identified in this needs assessment survey will serve to formulate key questions for the development of CanVasc recommendations.

Famorca, Leilani; Twilt, Marinka; Barra, Lillian; Bakowsky, Volodko; Benseler, Susanne; Cabral, David; Carette, Simon; Dhindsa, Navjot; Fifi-Mah, Aurore; Goulet, Michelle; Khalidi, Nader; Khraishi, Majed; McGeoch, Lucy; Milman, Nataliya; Pineau, Christian; Shojania, Kam; Taylor-Gjevre, Regina; Towheed, Tanveer; Trudeau, Judith; Yacyshyn, Elaine; Liang, Patrick; Pagnoux, Christian

2015-01-01

370

[An autopsy case of subarachnoid and intracerebral hemorrhage and necrotizing angitis associated with methamphetamine abuse].  

PubMed

We report an autopsy case of methamphetamine-related intracranial hemorrhage and vasculitis. The possible relationship between drug usage and the occurrence of intracranial bleeding and cerebral vasculitis in such patients is discussed. A 22-year-old woman died after an intravenous injection of unknown dose of methamphetamine. A computed tomography head scan demonstrated massive subarachnoid hemorrhage and hematoma in corpus callosum. Cerebral angiography revealed nonfilling of bilateral intracranial carotid arteries and extravasation of contrast medium from right pericallosal artery which was visualized retrogradely via vertebral artery. Postmortem studies showed cerebral edema, subarachnoid and intracerebral hemorrhage, and intracranial vasculitis in the absence of aneurysm, arteriovenous malformation or chronic hypertension. Histological findings of necrosis of blood vessel walls with destruction of the elastica and smooth muscle layer, and without leukocytotic infiltration of the blood vessel walls were observed in order of anterior cerebral, middle cerebral, vertebral, posterior cerebral and basilar arteries. These angiographic and histological evidence suggests that such hemorrhage results from the development of fibrinonecrosis in the large intracerebral vessels, in addition to a sudden rise in blood pressure. PMID:3219243

Shibata, S; Mori, K; Sekine, I; Suyama, H

1988-11-01

371

Schönlein-Henoch purpura during pregnancy with successful outcome for mother and newborn  

PubMed Central

Background Schönlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. Case presentation We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy showed leukocytoclastic vasculitis with intravascular fibrin thrombi. The direct immunofluorescence analysis evidenced vascular deposits of IgA and C3 in the upper and mid-dermis. These findings were consistent with Schönlein-Henoch purpura. There was no evidence of renal involvement or placental dysfunction. The patient was treated with low-dose oral corticosteroids and a healthy infant was delivered by cesarean section. Examination of the placenta and the navel string disclosed no signs of vasculitis or infarction. Conclusions Schönlein-Henoch purpura is rarely reported in pregnancy. Treatment with orally administred corticosteroids may lead to a beneficial outcome for mother and newborn. PMID:11866865

Feldmann, Robert; Rieger, Walter; Sator, Paul G; Gschnait, Friedrich; Breier, Friedrich

2002-01-01

372

Acute onset distal symmetrical vasculitic polyneuropathy associated with acute hepatitis B.  

PubMed

Hepatitis B can have varied extrahepatic manifestations involving the skin, renal, haematological and nervous systems. Neurological manifestations in hepatitis B may take the form of Guillain-Barré syndrome and secondary systemic vasculitis-related mononeuritis multiplex. The clinical course of hepatitis B-related, vasculitis-related neuropathy is usually subacute to chronic and clinical evolution is relatively benign. To our knowledge, acute hepatitis B-associated vasculitis manifesting as acute distal symmetric polyneuropathy has not been reported. We report a 60-year-old man who presented with fever, mild hepatomegaly, skin lesions in the form of non-palpable purpura and acute onset distal symmetric sensorimotor polyneuropathy. Serum transaminase levels were raised and viral serological markers revealed acute hepatitis B. The patient remained anicteric throughout his clinical course. Nerve conduction studies showed severe axonal sensorimotor polyneuropathy and histopathological examination of sural nerve biopsy was suggestive of vasculitic neuropathy. The patient was first given a course of intravenous immunoglobulin with the antiviral drug entecavir. The fever subsided after 1 week of treatment. The patient was started on prednisolone in addition to the entecavir, and showed significant improvement in motor power and marked resolution in paresthesia after 2 weeks of treatment. Thus, acute onset distal symmetric sensorimotor polyneuropathy of vasculitic etiology can be a manifestation of acute hepatitis B. PMID:23164826

Mehndiratta, Manmohan; Pandey, Sanjay; Nayak, Rajeev; Saran, Ravindra K

2013-02-01

373

The Usefulness of Interferon-gamma Release Assay for Diagnosis of Tuberculosis-related Uveitis in Korea  

PubMed Central

Purpose To evaluate the usefulness of the interferon-gamma release assay (IGRA) for diagnosing tuberculosis (TB)-related uveitis (TRU). Methods Records from 181 patients with ocular signs and symptoms suggestive of TRU and intraocular inflammation of unknown etiology were reviewed. All subjects underwent clinical and laboratory testing, including IGRA, to rule out presence of underlying disease. A diagnosis of presumed TRU was made based on an internist's TB diagnosis and a patient's response to anti-TB therapy. Sensitivity, specificity, and positive predictive values of IGRA for TRU diagnosis were calculated. Clinical characteristics were compared between patients with positive and negative IGRA results. Results The sensitivity and specificity of IGRA for TRU were 100% and 72.0%, respectively. Mean age, percentage of patients with retinal vasculitis, and the anatomic type of uveitis were significantly different between patients with positive and negative IGRA results (all p ? 0.001). Positive IGRA rates and false-positive rates were significantly different between age and anatomic type groups (both p = 0.001). The positive predictive value of the IGRA among patients with intraocular inflammation was high (70%) when all of younger age (?40 years), posterior uveitis, and retinal vasculitis were present. Conclusions The IGRA is useful for diagnosing TRU in the Korean population, especially when it is used as a screening test. Clinical characteristics, including younger age (?40 years), posterior uveitis, and retinal vasculitis in IGRA-positive patients, increase the likelihood of the patient having TRU. PMID:24882956

Ahn, Seong Joon; Kim, Ko Eun; Park, Kyu Hyung

2014-01-01

374

Unusual urticarias.  

PubMed

Typical urticarial lesions are transient cutaneous swellings of sudden onset, often itchy, persisting for less than 24 hours and resolving to leave normal appearing skin. Angioedema lesions are similar subcutaneous lesions. Atypical urticarias persist for longer than 24 hours, may be painful and bruised in appearance and accompanied with severe systemic symptoms. Conditions where prolonged weals are present include delayed pressure urticaria and urticarial vasculitis. These conditions do not respond well to antihistamine therapy. In delayed pressure urticaria, weals appear after a delay of hours at sites of sustained pressure on the skin and occur in association with ordinary chronic 'idiopathic' urticaria. Weals of urticarial vasculitis show histological features of venulitis, and can be accompanied by arthralgia and abdominal pain. Rarely, the condition is due to infective or autoimmune disease. Urticarial diseases, sometimes with features of urticarial vasculitis, and with associated systemic features include Schnitzler's Syndrome, Still's disease and Muckle-Wells syndrome. The latter syndrome is linked with chromosome 1q44, as is autosomal dominant cold urticaria, an unusual physical urticaria. Persistent cholinergic erythema, a variant of cholinergic urticaria, has been mistaken for a drug eruption or cutaneous mastocytosis. Rarely, food and exercise induced urticaria and anaphylaxis occur when exercise follows a specific food or any meal within a few hours. The early stages of inflammatory disease may be mistaken for urticaria and angioedema, but lesions usually persist for longer than 48 hours and are accompanied by epidermal changes. PMID:11770721

Black, A K

2001-11-01

375

Effectiveness of Heparin during Long-Term Tocolysis  

PubMed Central

Objective. Drip infusion during long-term tocolysis causes mechanical and infectious vasculitis and increases the frequency of peripheral venous catheter exchange (PVC), thereby placing a burden on patients. Our study aim is to confirm whether heparin ameliorates pain due to vasculitis during long-term tocolysis and reduces the frequency of peripheral venous catheter exchange. Design. Prospective study. Setting and Sample. All the patients requiring admission because of the presence of uterine contraction or progressive cervical dilatation from August 2009 to June 2011 at Juntendo University in Japan. Methods. Heparin was used for patients at the time the total number of peripheral venous catheter exchanges exceeded 5 in two weeks, and we evaluated whether heparin reduced the frequency of peripheral venous catheter exchange and improved the visual analog scale (VAS) for patients. The main outcome measures frequency of PVC exchange and VAS. Results. This study demonstrated that heparin reduced the frequency of peripheral venous catheter exchange (P = 0.0069) and VAS (P = 0.042). No side effects were noted. Conclusion. Heparin could satisfy patients during long-term tocolysis in terms of ameliorating pain due to vasculitis and reducing the PVC exchange frequency. PMID:23589778

Inagaki, Tetsunori; Makino, Shintaro; Yorifuji, Takashi; Sugimura, Motoi; Takeda, Satoru

2013-01-01

376

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. PMID:25400473

Grigorescu, Ioana; Dumitrascu, Dan Lucian

2014-01-01

377

Clinical Course, Prognosis, and Cause of Death in Primary Sjögren's Syndrome  

PubMed Central

The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4 ± 6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk. PMID:24963499

Horvath, Ildiko Fanny; Szanto, Antonia; Papp, Gabor; Zeher, Margit

2014-01-01

378

Monocyte activation in patients with Wegener's granulomatosis  

PubMed Central

OBJECTIVE—Wegener's granulomatosis (WG) is an inflammatory disorder characterised by granulomatous inflammation, vasculitis, and necrotising vasculitis and is strongly associated with anti-neutrophil cytoplasmic antibodies (ANCA). Activated monocytes/macrophages are present in renal biopsy specimens and participate in granuloma formation by synthesising and secreting a variety of chemoattractants, growth factors, and cytokines. In view of these findings, in vivo monocyte activation was evaluated in patients with WG and the findings related to parameters of clinical disease activity.?METHODS—Monocyte activation was analysed by measuring plasma concentrations of soluble products of monocyte activation, that is neopterin and interleukin 6 (IL6), by ELISA, and by quantitating the surface expression of activation markers on circulating monocytes by flow cytometry.?RESULTS—Twenty four patients with active WG were included in this study. Ten of these patients were also analysed at the time of remission. Twelve patients with sepsis served as positive controls, and 10 healthy volunteers as negative controls for monocyte activation. Patients with active disease had increased monocyte activation compared with healthy controls as shown by increased concentrations of neopterin (p <0.0001) and increased surface expression of CD11b (p < 0.05) and CD64 (p < 0.05). In those patients with increased concentrations of IL6 during active disease plasma concentrations of IL6 decreased during follow up when patients went into remission (p < 0.0001). In addition, neopterin (r = 0.37, r = 0.44), IL6 (r = 0.37, r = 0.60) and CD63 expression (r = 0.39, r = 0.45) correlated significantly with disease activity as measured by the Birmingham Vasculitis Activity Score and C reactive protein values, respectively. Compared with patients with sepsis, all markers of monocyte activation in patients with vasculitis were lower.?CONCLUSION—It is concluded that disease activity in WG correlates with the extent of activation of monocytes, compatible with their role in the pathophysiology of this disease.?? Keywords: anti-neutrophil cytoplasmic antibody; monocyte activation; vasculitis; flow cytometry PMID:10364903

Kobold, A.; Kallenberg, C.; Tervaert, J. W.

1999-01-01

379

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

380

Modulation of MMP-2 and MMP-9 in Churg-Strauss syndrome respiratory mucosa: potential monitoring parameters.  

PubMed

Churg-Strauss (CSS) syndrome is rare and of unknown etiology. It is associated with vasculitis, blood eosinophilia and granulomatosis, and affects multiple organs and systems at various stages of the disease. Specific diagnostic and monitoring tests are not yet available. This study aims to assess the changes in MMP-2 and MMP-9 along with the histopathological alterations in two cases of CSS, as possible potential diagnostic and monitoring criteria. Two adult male patients were diagnosed with CSS in the otorhinolaryngology clinic in the University of Palermo, based on multiple clinical and histopathologic criteria. Biopsies of respiratory mucosa were taken after the consent of the patients, processed for routine histopathology and immunohistochemistry as well as quantitative polymerase chain reaction (qPCR). Similar biopsies were also taken from a non- CSS patient. The Assessment of MMP-2 and MMP-9 was performed using both immunohistochemistry and qPCR techniques. Histopathological alterations in the respiratory mucosa were consistent with vasculitis and granulomatous tissue formation, in addition to inflammatory cell infiltration with abundance of eosinophils. Immunohistochemistry assay performed on the samples derived from the two CSS patients showed a relative and remarkable increase of both MMP-2 and MMP-9 compared to controls. Such an increase was consistent with the qPCR results which depicted a significant increase between 20 and 30% for both MMP-2 and MMP-9, respectively. Since the secretion of MMPs is an essential step in angiogenesis, could these enzymatic factors be used as parameters to diagnose or monitor the evolution of CSS? The small number of samples analyzed in this study does not allow us to suggest a general statement correlating the increase in expression of MMP-2 and MMP-9 to the appearance or evolution of vasculitis; it is only speculative. PMID:25004843

Leone, A; Uzzo, M L; Gerbino, A; Tortorici, S; Tralongo, P; Cappello, F; Incandela, S; Spatola, G F; Jurjus, A R

2014-01-01

381

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

382

Clinical Significance of Antiproteinase 3 Antibody Positivity in cANCA-Positive Patients  

Microsoft Academic Search

:   We addressed the clinical significance of antiproteinase 3 (anti-PR3) antibody (Ab) positivity by reviewing the files of\\u000a 79 patients whose serum contained antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (cANCA) and had\\u000a been tested for anti-PR3 reactivity. Vasculitis was present in most (22\\/35) cANCA+ PR3+ patients but in only a few (5\\/44) cANCA+ PR3+ patients, thereby suggesting that

V. van Pesch; M. Jadoul; C. Lefèbvre; B. R. Lauwerys; J.-P. Tomasi; J.-P. Devogelaer; F. A. Houssiau

1999-01-01

383

A case of multiple extrahepatic manifestations in a patient with untreated, chronic hepatitis C virus infection.  

PubMed

Chronic hepatitis C virus (HCV) infection is a complex, multi-organ disorder, not just limited to the liver. Mixed cryoglobulinemia (MC) type 2 is a common extrahepatic complication, in which immunoglobulin complexes deposit in vascular endothelium. This in turn creates a diffuse inflammatory reaction, leading to a variety of disorders involving multiple systems. We report the rare case of a patient with cryoglobulinemia, cutaneous vasculitis, membroproliferative glomerulonephritis, and B cell lymphoma with a variant t(6;10) translocation in the setting of an untreated, chronic HCV infection. This case highlights the challenge associated with diagnosing and managing such a complex presentation. PMID:24315985

Meillier, Andrew; McGee, Jean; Kartan, Saritha; Baskin, Stuart

2014-02-01

384

Nodular Erythema Elevatum Diutinum Mimicking Kaposi's Sarcoma in a Human Immunodeficiency Virus Infected Patient  

PubMed Central

Erythema elevatum diutinum (EED) has been emerging as a specific Human Immunodeficiency Virus (HIV) associated dermatosis in recent times. It is an extremely rare chronic disease of unknown origin and part of the spectrum of leukocytoclastic vasculitis. We describe a case of EED simulating Kaposi's sarcoma in a 52-year-old HIV infected female patient with no previous opportunistic infections and CD4+ count of 164/mm3. Therapy with oral dapsone (100 mg/day) for two weeks resulted in resolution of some lesions. PMID:25484391

Rao, G Raghurama; Joshi, Rajiv; Phaneendra Prasad, A Krishna; Amareswar, A; Sandhya, S; Sridevi, M

2014-01-01

385

[Acute hemiparesis revealing a neuroborreliosis in a child].  

PubMed

We report on a 11-year-old boy who had 2 acute hemiparesis episodes over a period of 1 month. He suffered from headache and fatigue since 1 year. He could not remember neither a tick bite nor a local erythematous skin lesion. The diagnosis of neuroborreliosis was based on intrathecal production of specifics antibodies. Furthermore, the CSF/blood glucose ratio was decreased (0.14), which was rarely described. Cranial MRI showed left capsulothalamic inflammation and a vasculitis. The patient was successfully treated by ceftriaxone. Neuroborreliosis should be considered in all children with stroke-like episode, even in the absence of a history of a tick bite. PMID:18155890

Rénard, C; Marignier, S; Gillet, Y; Roure-Sobas, C; Guibaud, L; Des Portes, V; Lion-François, L

2008-01-01

386

Percutaneous Transcatheter Embolization of Gastrointestinal Bleeding in a Child with Polyarteritis Nodosa  

PubMed Central

Summary Background Polyarteritis nodosa is a form of necrotizing vasculitis of small and medium-sized arteries. Major gastrointestinal complications are ulceration, perforation, hemorrhage, and obstruction. Case Report We report on a clinical case of a 16-year-old female patient with massive hematemesis, who was successfully treated with embolization with a 1:2 dilution of N-butyl cyanoacrylate glue. Conclusions To the best of our knowledge, this is the youngest child reported on with massive GI bleeding secondary to PAN, treated with successful percutaneous transcatheter embolization under emergency conditions. PMID:25512765

Bas, Ahmet; Samanci, Cesur; Numan, Furuzan

2014-01-01

387

NOAH-New York Online Access to Health: Arthritis and Rheumatoid Disorders  

NSDL National Science Digital Library

From New York Online Access to Health (NOAH), this website offers links to numerous Internet resources for Arthritis and Rheumatoid Disorders. Care and Treatment resource categories include Diet; Medications; Total Hip Replacement; and Alternative Therapies-to name a few. Websites relating to diagnosis and symptoms, as well as specific concerns like arthritis in children, and rheumatoid diseases and pregnancy are available as well. In addition, the site links to websites for a wide range of Rheumatoid Disorders such as Avascular Necrosis, Gout, Osteoarthritis, Vasculitis, and more. Site visitors can also link to Internet resources for other health issues on the NOAH website including Heart Disease, Infections, Cancer, Asthma, and Dental Care.

388

Hemorrhagic stroke after naphazoline exposition: case report.  

PubMed

Ten percent of all strokes are due to spontaneous cerebral hemorrhages. They are associated to drugs (licit and illicit) in 9.5% of all cases in young adults. This is a case report of a 44-year-old man, without previous morbidities, who presented a sudden onset headache and arterial hypertension 24 hours after use of naphazoline as nasal decongestant. Cranial tomography showed right thalamus hemorrhage. Cerebral angiography showed no aneurisms, vascular malformations or vasculitis. No other risk factors were found during investigation in this patient and the stroke was attributed to naphazoline exposition. PMID:15476091

Zavala, Jorge A A; Pereira, Eduardo R; Zétola, Viviane H F; Teive, Hélio A G; Nóvak, Edison M; Werneck, Lineu C

2004-09-01

389

Overlap between dermatomyositis and ANCA vasculitides  

PubMed Central

We present the second report of the association between antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis with dermatomyositis (DM). A 47-year-old woman suddenly developed rapidly progressive renal failure in the context of (DM). The kidney biopsy showed focal and segmental necrotizing glomerulonephritis with crescent formation. Cyclophosphamide treatment was commenced resulting in a significant recovery of kidney function and maintenance of recovery at 6 months. Although the pathophysiology is unknown, we hypothesize that CD8-T-deficient cells and MPO+ neutrophils in the DM lesions play an important role in the disease process.

Yuste, Claudia; Rapalai, Molefe; Pritchard, Benjamin A.; Jones, Terence J.; Amoasii, Constanza; Al-Ansari, Atheer; Ramakrishna, Satish B.

2014-01-01

390

Recurrent Febrile Neutropenia and Thrombocytopenia in a Chronic Cocaine User: A Case of Levamisole Induced Complications  

PubMed Central

Cocaine is used by approximately 1.5 million Americans each month and up to 69% of the cocaine seized contains levamisole. The real incidence of cocaine-levamisole induced neutropenia is unclear but probably underestimated. Associated complications include fever, thrombocytopenia, skin-vasculitis disorders, and rarely kidney injury. We present a young male, with chronic active cocaine use presenting with recurrent episodes of febrile neutropenia and thrombocytopenia. He underwent extensive work-up and was treated with many antibiotics and we suspect that his neutropenia and thrombocytopenia were caused by recurrent cocaine-levamisole use.

Martinez, Eduardo; Alvi, Raza; Venkatram, Sindhaghatta; Diaz-Fuentes, Gilda

2015-01-01

391

[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

392

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

393

Hyperostosis frontalis interna in a patient with giant cell arteritis.  

PubMed

Hyperostosis frontalis interna (HFI) is a disorder characterized by progressive symmetric thickening of the inner table of the frontal bone of the human skull. HFI may be accompanied by headache and some neuropsychiatric diseases such as epilepsy and dementia. Giant cell arteritis (GCA), also called temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries. It affects elderly people and may result in a wide variety of systemic, neurologic and ophthalmologic complications. As no association of HFI and GCA was encountered in the literature, we found it interesting to report a case with both of these clinical entities. PMID:18250961

Kocabas, Hilal; Sezer, Ilhan; Melikoglu, Meltem Alkan; Gurbuz, Ulku; Illeez, Ozge; Ozbudak, Irem Hicran; Butun, Bulent

2008-01-01

394

Granulomatous orchitis in a pre-pubertal school-aged child: differential diagnosis dilemmas.  

PubMed

A 6-year-old male presented with testicular growth and persistent chronic orchiepididymitis with high inflammatory markers (C reactive protein and erythrocyte sedimentation rate). Biopsies of the testes and epididymides showed bilateral epididymal and testicular granulomata, testicular fibrosis and chronic inflammatory infiltration, and the histological diagnosis was granulomatous orchitis. The symptoms receded with oral corticosteroids. Although rare, granulomatous orchitis is a possible diagnosis in children presenting testicular enlargement. It is important to differentiate it from testicular tumors (if necessary with testicular biopsy) and to investigate its association with systemic vasculitis and infectious diseases. PMID:22575712

Jesus, Lisieux E; Rocha, Kátia L M; Caldas, Maria L R; Fonseca, Elissa

2012-10-01

395

Diagnostic exercise: suppurative rhombencephalitis and meningitis in a goat.  

PubMed

A 1-year-old female Boer goat was presented for necropsy following spontaneous death and history of acute recumbency, nystagmus, and sialorrhea. A swollen area was grossly observed in the brainstem at the level of the pons. On cut surface, the right cerebellar peduncles were expanded by a focal, pale, poorly demarcated, slightly depressed, and soft area of malacia. Microscopically, this area contained diffuse edema and necrosis, with microabscesses, neuronal necrosis, neuronophagia, axonal spheroids, vasculitis, and perivascular accumulations of lymphocytes, plasma cells, macrophages, and neutrophils. The diagnosis was based on the morphologic findings, fluorescent antibody test results, and special staining. PMID:23576239

Dill, J A; Rissi, D R

2014-07-01

396

Extreme distal bypass to improve wound healing in Buerger's disease.  

PubMed

Thromboangiitis obliterans or Buerger's disease is a rare non-atherosclerotic segmental inflammatory vasculitis that most commonly involves small and medium-sized arteries, veins and nerves of the extremities, and generally affects young tobacco smokers. A 53-year-old man was found to have critical ischaemia of his left lower limb with foot gangrene. He underwent extremely distal surgical revascularisation using a great saphenous vein bypass graft. The choice of a very distal artery as run-off vessel promoted a faster wound healing and pain relief, with improvement in quality of life. PMID:24612761

De Caridi, Giovanni; Massara, Mafalda; Villari, Simona; Martelli, Eugenio; Spinelli, Francesco; Grande, Raffaele; Butrico, Lucia; de Franciscis, Stefano; Serra, Raffaele

2014-02-25

397

Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement  

PubMed Central

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI) symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder. PMID:25473392

Franco, Diana L.; Ruff, Kevin; Mertz, Lester; Lam-Himlin, Dora M.; Heigh, Russell

2014-01-01

398

Sarcoidosis presenting as severe renin-dependent hypertension due to kidney vascular injury  

PubMed Central

Renal sarcoidosis embraces a wide variety of clinical patterns. Renal vascular involvement has seldom been reported and usually in the setting of systemic vasculitis. We report the case of a 22-year-old patient in whom inaugural manifestation of renal sarcoidosis consisted of severe hypertension associated with bilateral perfusion defects and tumour-like nodules. In the setting of renal sarcoidosis, our case suggests that renin-dependant hypertension may arise from renal ischaemia as a result of extrinsic compression of kidney blood vessels due to severe granulomatous inflammation. PMID:25852913

Rafat, Cedric; Bobrie, Guillaume; Chedid, Antoine; Nochy, Dominique; Hernigou, Anne; Plouin, Pierre-François

2014-01-01

399

Multiple cerebral infarctions with severe multi-organ dysfunction following multiple wasp stings  

PubMed Central

Wasp and bee sting are commonly encountered worldwide. Local reactions are more common, generally are self-limiting and settle within a few hours. Multiple stings can lead to various clinical manifestations like vomiting, diarrhea, dyspnea, generalized edema, hypotension, syncope, acute renal failure, and even death. Rarely, they can cause vasculitis, serum sickness, neuritis, and encephalitis. We are reporting a case of 40-year-old male who presented with stroke, right hemiparesis with severe multi-organ dysfunction due to multiple wasp stings. PMID:24753680

Wani, Mushtaq; Saleem, Sheikh; Verma, Sawan; Yousuf, Irfan; Wani, Maqbool; Asimi, Ravouf; Daga, Riyaz Ahmed; Shah, Irfan; Aejaz

2014-01-01

400

The intestinal bypass: arthritis-dermatitis syndrome.  

PubMed

Of the 31 patients who developed polyarthritis following jejunoileal bypass for obesity, 24 had cutaneous vasculitis (urticarial, pustular, and nodular), 11 paresthesias, 10 Raynaud's phenomenon, and 1 pericarditis. Blind loop symptoms (14 of 26 patients), cryoglobulinemia (10 of 28), and immune deposits in biopsied skin lesions (5 of 7) support the theory of a relationship between bowel bacteria and immune complexes. Treating the blind loop with antibiotics and sphincteroplasty to prevent bacterial reflux into the blind loop helped 5 of 10 and 6 of 9 patients, respectively. A comparison is made to other bowel associated arthritides. PMID:7236324

Stein, H B; Schlappner, O L; Boyko, W; Gourlay, R H; Reeve, C E

1981-05-01

401

[Spontaneous regression of sclerosing mesenteritis presenting as a huge mass].  

PubMed

Sclerosing mesenteritis is a rare benign disease originated from the mesenteries. It can be related to autoimmune disease, vasculitis, ischemia, infection, trauma and operation, but most of cases are idiopathic. The overall prognosis of sclerosing mesenteritis is usually good with benign, course. However, no consensus of treatment has yet been established. We report a case of spontaneous partial regression of sclerosing mesenteritis presented as a huge mass and diagnosed by finding of contrast enhanced abdominal computed tomography and percutaneous ultrasonography guided needle biopsy. PMID:22544031

Lee, Hyun Jeong; Kim, Jin Il; Ahn, Ji Won; Kim, Jeong Ho; Cheung, Mo Eun; Park, Soo Heon; Kim, Jae Kwang; Im, Soyoung

2012-04-01

402

Notes on the kidney and its diseases for the neurologist  

PubMed Central

To save their patients from dialysis and transplantation, neurologists need simply remain alert to the possibility of renal failure, particularly in the context of systemic disease, diabetes, sepsis and drugs. Of the numerous territories shared by our respective specialities, we outline a pragmatic approach to the diagnosis and treatment of the vasculitides, underpinned by knowing which questions to ask, equally importantly when to ask them, and in the art of obtaining a tissue diagnosis. We consider the current evolving trial evidence that directs the usage of a growing arsenal of therapies in the induction and maintenance stages of vasculitis treatment, and extend this consideration to Lupus and Sjogren's. PMID:17435183

Zandi, Michael S; Coles, Alasdair J

2007-01-01

403

Giant aortic arch aneurysm complicating Kawasaki's disease.  

PubMed

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. PMID:25298695

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

2014-09-01

404

Indications for biotherapy in systemic vasculitides  

Microsoft Academic Search

Biotherapy now holds a specific place in the therapeutic armamentarium for systemic vasculitides. Such therapy includes cytokines,\\u000a such as (pegylated)?-interferon for hepatitis B virus-related polyarteritis nodosa and hepatitis C virus-related cryoglobulinemic\\u000a vasculitis, and polyvalent immunoglobulin (IVIg), with well-defined indications and pending positive results. More specifically\\u000a targeted monoclonal antibodies include antitumor necrosis factor-? or anti-CD20 for antineutrophil cytoplasmic antibody-associated\\u000a vasculitides or

Loïc Guillevin; Christian Pagnoux; Philippe Guilpain; Boris Bienvenu; Valérie Martinez; Luc Mouthon

2007-01-01

405

Acute acalculous cholecystitis complicating systemic lupus erythematosus: case report and review.  

PubMed Central

A case of acalculous cholecystitis presented as an acute abdominal emergency in a 22 year old woman with severe systemic lupus erythematosus. At the time of presentation the patient was receiving high doses of prednisone and cyclophosphamide to control her underlying disease. Histological examination of the biopsy specimen from the gall bladder showed lupus vasculitis. This complication of systemic lupus erythematosus has not been reported before. Laboratory studies and changes in lupus activity may fail to predict the onset of cholecystitis. Images p252-a PMID:6402058

Swanepoel, C R; Floyd, A; Allison, H; Learmonth, G M; Cassidy, M J; Pascoe, M D

1983-01-01

406

Severe haemoptysis due to subclavian arteritis.  

PubMed

Severe haemoptysis due to infective subclavian arteritis has, to our knowledge, never been documented. We report a case of subclavian arterial vasculitis that eroded into the left lung apex, causing a large intraparenchymal mycotic pseudoaneurysm. The patient presented with high fever and blood expectoration. An emergent left lateral thoracotomy was performed. The inflamed segment of the subclavian artery was resected and continuity was restored with a reversed saphenous vein graft. The postoperative course was uneventful and the patient was discharged on the 10th postoperative day. PMID:22732928

Lioulias, A; Misthos, P; Kokotsakis, J; Drosos, O; Karagiannidis, N; Pavlopoulos, D; Mitselou, M

2012-06-01

407

Technetium scanning in Kaposi's sarcoma and its simulators.  

PubMed

The clinical picture of ulcerated purple plaques on the legs often suggests several diagnoses: Kaposi's sarcoma, stasis dermatitis, atrophie blanche (livedoid vasculitis), and a poorly understood condition called acroangiodermatitis of Favre-Chaix (pseudo-Kaposi's sarcoma). Even the skin biopsy may not always be conclusive. We describe our experience with three patients with pseudo-Kaposi's sarcoma, one with "true" Kaposi's sarcoma and two with atrophie blanche. Clinical and histopathologic similarities among these three conditions pointed up the need for additional confirmatory studies, i.e., isotope scanning. The technetium scan was positive in both Kaposi's sarcoma and pseudo-Kaposi's sarcoma but negative in atrophie blanche. PMID:6281316

Gunnoe, R; Kalivas, J

1982-04-01

408

Unfamiliar Manifestations of Anti-tubercular Therapy.  

PubMed

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

409

[Ocular manifestations of Churg-Strauss syndrome: review article and case report].  

PubMed

This paper summarizes the possible ocular manifestations of Churg-Straus syndrome (CCS) from the literature and presents an unusual case report from routine clinical practice with conjunctival granuloma, orbital pseudotumor and choroidal folds. The CSS is an ANCA-associated granulomatous vasculitis which can be manifested in various organs and represents a life-threatening situation for the patient. Ocular manifestations are rare and can spread to all segments of the eye and orbit. The most frequent forms of ocular involvement described in the literature are retinal occlusion and orbital pseudotumor with various degrees of expression. PMID:23765370

Atili, A; Richter, C; Bahn, E; Rustenbeck, H H; Schittkowski, M

2013-11-01

410

Indications for /sup 99m/technetium dimercapto-succinic acid scan in children  

SciTech Connect

The /sup 99m/technetium dimercapto-succinic acid scan provides an image of functional renal parenchyma. This static scan has specific indications and cannot be used simply in place of a /sup 99m/technetium diethylenetriaminepentaacetic acid scan. The major clinical indications for this investigation are the detection and/or evaluation of a renal scar, the small or absent kidney, an occult duplex system, certain renal masses, systemic hypertension or suspected vasculitis. The physiology of the /sup 99m/technetium dimercapto-succinic acid scan is reviewed briefly.

Gordon, I.

1987-03-01

411

Lessons learned from an animal model of Kawasaki disease.  

PubMed

Kawasaki disease is the most common cause of multisystem vasculitis in childhood. Kawasaki disease has been reported throughout the world and affects children of all ethnicity. Coronary artery damage from Kawasaki disease is the leading cause of acquired heart disease in children in the developed world. Diagnostic tests and prognostic markers are lacking, and questions remain unanswered in our understanding of the etiopathogenesis of the disease, thus limiting our ability to improve therapy and coronary outcome. In this article I will review advances made in an animal model of disease, which has helped advance our understanding of the etiology and pathogenesis of this fascinating clinical syndrome. PMID:17428374

Yeung, R S M

2007-01-01

412

Hypereosinophilic syndrome as a cause of fatal thrombosis: two case reports with histological study.  

PubMed

Herein we present two cases of hypereosinophilic syndrome with a unique clinical presentation. One patient showed severe systemic thrombosis with splenic rupture and the other patient showed finger gangrene with various systemic symptoms. Both patients were examined histologically, and several characteristics were noted. First, fresh or organized thrombosis with marked eosinophilic infiltration was observed in the cavity and walls of the thrombosed vessels. Second, many eosinophils showed degranulation and were positive for eosinophilic cationic protein on immunohistological examination. Third, the structures of thrombosed vessels were well preserved, which is not observed in systemic vasculitis. These patients exhibited no neoplastic features and were treated with prednisolone with excellent therapeutic results. PMID:25388084

Fujita, Kumi; Ishimaru, Hiroyasu; Hatta, Kazuhiro; Kobashi, Yoichiro

2014-11-12

413

Systemic lupus erythematosus presenting with hyporeninemic hypoaldosteronism in a 10-year-old girl.  

PubMed

Hyperkalemia has been noted to occur spontaneously in patients with long-standing systemic lupus erythematosus who did not have advanced renal insufficiency. The patients previously described all had relatively normal renin-aldosterone systems, and the hyperkalemia was thus presumed to be secondary to a primary defect in renal tubular potassium secretion. We describe at 10-year-old girl with lupus nephritis, without significant renal insufficiency, who had hyperkalemia from hyporeninemic hypoaldosteronism postulated to be due to vasculitis involving the afferent/efferent arterioles and juxtaglomerular apparatus. PMID:3535507

Kozeny, G A; Hurley, R M; Fresco, R; Vertuno, L L; Bansal, V K; Hano, J E

1986-01-01

414

Amyloidosis in Behcet's disease.  

PubMed

Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy. PMID:25579733

Ghorbel, I Ben; Feki, N Bel; Salem, T Ben; Lamloum, M; Houman, M H

2015-01-01

415

[An abdominal aortic aneurysm revealing Behçet's disease].  

PubMed

Behçet's disease is a vasculitis of unknown origin. Vascular lesions predominantly affect veins. Arterial involvement is rare but usually associated with poor prognosis. Aortic syndromes are usually aneurysmal and occasionally reveal Behçet's disease. We report the case of a 46-year-old man whose Behçet's disease was revealed by a sub-renal abdominal aortic aneurysm in pre-rupture state. The diagnosis of this disease was retained based on clinical, biological and radiological criteria. The surgical procedure consisted in the resection of the aneurysmal sac and the interposition of a prosthetic PTFE tube. PMID:25457357

Naouli, H; Zrihni, Y; Jiber, H; Bouarhroum, A

2014-12-01

416

Cerebral complications in juvenile rheumatoid arthritis  

PubMed Central

A study of 170 patients with juvenile rheumatoid arthritis and a review of the literature indicate that this disease can significantly affect the central nervous system. Signs of CNS dysfunction were observed in 13 children. During the acute toxic stages the EEG is abnormal in many cases. Other manifestations of toxic encephalopathy such as irritability, drowsiness, stupor, convulsions and marked meningismus may be evident in severe cases. Meningitis is often suspected but ruled out by the finding of normal CSF. Steroids can rapidly improve the condition of these children. If `unexplained' seizures occur during the chronic stage, the diagnosis of cerebral vasculitis should be entertained. PMID:4665094

Jan, James E.; Hill, Robert H.; Low, Morton D.

1972-01-01

417

Severe Raynaud's Phenomenon in a Patient With Antinuclear Antibody-Negative Systemic Lupus Erythematosus  

PubMed Central

There is a well-recognized subset of patients with clinical findings consistent with systemic lupus erythematosus (SLE) but with negative antinuclear antibodies (ANA). Most of these patients have significant cutaneous involvement with little central nervous system or renal pathology. The following case report describes such a patient whose presentation was suggestive of SLE but who was ANA negative despite repeated testing. Additionally, the patient was found to have severe Raynaud's phenomenon and cutaneous vasculitis. This case is noteworthy because of the prominence of vascular insufficiency as a presenting feature of ANA-negative SLE. PMID:3262169

Maxwell, Celia J.; Potter, Edger; Townsend, John

1988-01-01

418

Atypical presentation of acute-onset endophthalmitis after uncomplicated pars plana vitrectomy.  

PubMed

A 70-year-old woman presented with signs of acute-onset endophthalmitis within 24 hours of surgery, with retinal hemorrhages and vascular changes in the absence of pain or significant inflammation. She had undergone uncomplicated 25-gauge pars plana vitrectomy for epiretinal membrane with presenting visual acuity of 20/40- and significant distortion. Vitreous culture revealed Staphylococcus epidermidis. Final visual acuity was no light perception with persistent pain, and the patient ultimately underwent enucleation. Although rarely, acute-onset postoperative endophthalmitis can present as retinal vasculitis with intraretinal hemorrhages and mild inflammation. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:393-395.]. PMID:25856829

Reilly, Gayatri S; Garfinkel, Richard A; Melamud, Alexander

2015-03-01

419

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

420

Kawasaki Disease has so much to teach us!  

PubMed

Kawasaki Disease (KD) is primarily a childhood vasculitis of mid-size muscular arteries, of which the coronary arteries (CA) are most clinically significant. Although timely treatment with pooled intravenous immunoglobulin (IVIG) has significantly reduced CA pathology, as determined by ECHO cardiology, about 30% of children still develop potentially fatal aneurysms, thrombi, or stenosis. This paper describes several additional pathologies and phenomena of undetermined significance, e.g. myocarditis, endocarditis with fibroelastosis, adrenal medullary necrosis, arterial smooth muscle cell transition into proliferating myofibroblasts, pneumonia in patients dying from KD, and calcification of organizing thrombi. PMID:24460693

Orenstein, Jan M

2014-04-01

421

Systemic amyloidosis of beta 2-microglobulin type: a complication of long-term haemodialysis.  

PubMed Central

A patient receiving long-term haemodialysis developed systemic amyloidosis, which was shown immunohistochemically to be of beta 2-microglobulin type, a previously unrecognised form of systemic amyloidosis. Histologically, the amyloid deposits were closely associated with foci of acute and granulomatous inflammation and vasculitis, although it was not clear if the amyloid deposits directly caused the inflammatory process, or if amyloid was deposited preferentially in areas of inflammation of uncertain aetiology. Images Fig 1 Fig 2 Fig 3 Fig 4 Fig 5 PMID:3119676

Theaker, J M; Raine, A E; Rainey, A J; Heryet, A; Clark, A; Oliver, D O

1987-01-01

422

Chronic inflammatory lesions of the placenta.  

PubMed

The chronic inflammatory lesions of the placenta often run in the shadows of the better-known acute inflammatory processes of the placenta, such as acute chorioamnionitis and acute funisitis. A heterogeneous population of T-cell lymphocytes, plasma cells, and macrophages is the primary player in chronic villitis, chronic chorioamnionitis, chronic deciduitis, and chronic intervillositis, and eosinophils are an added component of eosinophilic/T-cell chorionic vasculitis. The histologic appearance, sites of occurrence in the placenta, and pathogeneses of these entities are reviewed. PMID:25455621

Katzman, Philip J

2015-02-01

423

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

424

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

425

[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

426

Tuberculosis infection causing intestinal perforations in 2 patients with systemic lupus erythematosus.  

PubMed

Patients with systemic lupus erythematosus (SLE) have a higher incidence rate of tuberculosis and a more frequent extrapulmonary involvement than the general population. We present 2 SLE patients who developed gastrointestinal tuberculosis complicated with intestinal perforation, a rare but serious complication that could be confused with lupus-associated intestinal vasculitis. Opportunistic infections such as tuberculosis must be suspected in SLE patients with abdominal symptoms on immunosuppressive therapy because its early recognition could prevent catastrophic complications such as intestinal perforation and subsequent peritonitis. PMID:25036568

González, Luis A; Muñoz, Carolina; Restrepo, Mauricio; Vanegas, Adriana Lucía; Vásquez, Gloria

2014-08-01

427

Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases  

PubMed Central

Henoch-Schonlein purpura (HSP) is a small vessel vasculitis mediated by type III hypersensitivity with deposition of IgA immune complex in the walls of vessels. It is a multi-system disorder characterized by palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP.

Prathiba Rajalakshmi, Parameswaran; Srinivasan, Kalyanasundaram

2015-01-01

428

A 29-year-old man with hospital-acquired cavitary pneumonia  

PubMed Central

Cavitary lung processes pose a formidable diagnostic challenge. Causes vary widely and include cavitary pneumonia, vasculitis and malignancy. In some cases, patient history and basic work-up may yield a diagnosis, but in others, an extensive work-up, including tissue biopsy, may be necessary to establish the cause. The authors present a case of cavitary pneumonia that complicated an open lung biopsy. It developed in the hospital and was caused by mucormycosis, a potential emerging infection causing health care-associated infections. PMID:24712011

Mehmood, Mansoor; Halloush, Ruba A; Khasawneh, Faisal A

2014-01-01

429

Mechanical Thrombectomy with Rotarex System in Buerger's Disease  

PubMed Central

We report the case of a patient with 2-month history of chronic thromboembolism of the distal superficial femoral and popliteal arteries with diagnostic features of thromboangiitis obliterans disease. The occlusion could not be crossed by antegrade approach and was achieved retrogradely via dorsalis pedis artery puncture followed by mechanical removal of the thrombus with Rotarex system (Straub Medical AG, Wangs, Switzerland). Subsequent ballooon angioplasties achieved exclusion of the thrombus, and straight-line flow established to the foot through the anterior tibial Artery. The present case report demonstrates the success of mechanical thrombectomy in a patient with Buerger's vasculitis.

Kilickesmez, Ozgur; Oguzkurt, Levent

2015-01-01

430

Pathology of the heart and the cardiac conduction system in Wegener's granulomatosis.  

PubMed Central

Two cases of Wegener's granulomatosis occurred in two women aged 53 and 67 years. The clinical course in both was of rapid deterioration resulting from renal failure and leading to death. At necropsy both cases showed severe and disseminated necrotising granulomatosis in the lungs, kidneys, and spleen but with variable degrees of cardiac and systemic vasculitis. One patient, with appreciable cardiac damage, developed a P wave abnormality due to atrial disease. In patients with Wegener's granulomatosis damage to the heart should be suspected and signs of conduction system abnormalities looked for on the electrocardiogram. Images PMID:6508968

Allen, D C; Doherty, C C; O'Reilly, D P

1984-01-01

431

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

432

[Acute abdomen at onset of incomplete and atypical Kawasaki disease: Case report].  

PubMed

Kawasaki disease is one of the most common systemic vasculitis in children and the most common cause of acquired heart disease in developed countries. Diagnosis relies on clinical findings and there is no specific test. Diagnosis and treatment decisions are very difficult in incomplete or atypical cases and questions inevitably arise during the care of these patients. In children with atypical or incomplete disease, the option in favor of therapy with gamma globulin seems currently to be the most suitable one. A patient with acute abdomen is presented as the initial symptom of Kawasaki disease. PMID:25727840

García Munitis, Pablo; Ves Losada, Juan; Montali, César

2015-04-01

433

Kawasaki disease: pathophysiology, clinical manifestations, and management.  

PubMed

Kawasaki Disease, a systemic vasculitis of unknown origin with specific predilection for the coronary arteries, is the most common cause of childhood-acquired heart disease in western countries. Despite its world-wide incidence, the pathophysiology of this enigmatic disease is still under investigation. Diagnosis is made on a clinical basis, with supportive laboratory evidence and imaging. Once identified, timely initiation of treatment is imperative in order to quell the inflammatory response and decrease the incidence of long-term sequelae, specifically coronary artery aneurysms. Finally, longitudinal follow-up should be implemented based on risk stratification and individualized to each patient. PMID:24744086

Dimitriades, Victoria R; Brown, Amanda G; Gedalia, Abraham

2014-06-01

434

Sacral myeloradiculitis (Elsberg syndrome) secondary to eosinophilic meningitis caused by Angiostrongylus cantonensis  

PubMed Central

Elsberg syndrome secondary to eosinophilic meningitis caused by Angiostrongylus cantonensis is uncommon. Clinicians should consider a wide differential diagnosis including tumour, spinal cord infarction, necrosis, vasculitis, drug induced or other sources of infection. In addition, acute urinary retention is a urological emergency and clinicians should keep in mind the prevention of bladder overdistension. The intervention of rehabilitation programmes and clean intermittent catheterisation education for bladder management, in accordance with the patient’s condition, is also important. Earlier rehabilitation is important to ensure a speedy recovery and to prevent further complications. PMID:21811516

Hsu, Jui-Jen; Chuang, Shin-Hung; Chen, Chia-Hsin; Huang, Mao-Hsiung

2009-01-01

435

Clinical Examination and Treatment of a Leg Ulcer Caused by a Stingray Puncture.  

PubMed

The most frequent causes of leg ulcers include venous incompetency (superficial and deep), arterial insufficiency, trauma, vasculitis, and neoplasm. Leg ulcers from injury by stingrays are quite rare. In this case report, we describe a leg ulcer caused by a stingray sting complicated by infection, which healed completely without surgery. In addition, since there few cases in the literature describing such traumas, we performed a comprehensive review of the literature. Important is the fact that the wound healing was complete without resorting to surgery, but only with a correct targeted antibiotic therapy and the use of a collagenase, which has the particularity of having hyaluronic acid as a component. PMID:24879461

Fino, Pasquale; Onesti, Maria Giuseppina; Felli, Antonina; Scuderi, Nicolò

2014-05-30

436

Acute penile pain and swelling in a 4-year-old child with Henoch-Schönlein purpura.  

PubMed

Henoch-Schönlein purpura (HSP) is the most common vasculitis found in children. It usually affects the small vessels of the skin, joints, gastrointestinal tract and, more rarely, kidneys, brain, lungs and genitalia. Apart from classical presentation with purpuric rashes around buttocks and lower extremities, features such as arthralgia, abdominal pain, haematuria and proteinuria as well as scrotal swelling have been described in the literature. Penile involvement is rare and is not commonly described. We describe a child with HSP who developed penile involvement. We review the literature of all the cases reported in detail, in order to highlight useful clinical presentation, management and prognosis of this rare manifestation. PMID:25858918

Tewary, Kishor Kumar; Khodaghalian, Bernadette; Narchi, Hassib

2015-01-01

437

Myocardial infarction following bee sting.  

PubMed

Bee stings are commonly encountered worldwide. Various manifestations after bee sting have been described. Local reactions are common. Unusually, manifestations like vomiting, diarrhoea, dyspnoea, generalised oedema, acute renal failure, hypotension and collapse may occur. Rarely vasculitis, serum sickness, neuritis and encephalitis have been described which generally develop days to weeks after a sting. Acute coronary syndromes after hymenoptera stings and other environmental exposures are referred to as the Kounis syndrome or allergic myocardial ischaemia and infarction. We report a 60 year old male who developed myocardial infarction after multiple bee stings over his body. PMID:25856951

Puvanalingam, A; Karpagam, P; Sundar, C; Venkatesan, S; Ragunanthanan

2014-08-01

438

Giant cell arteritis--a changing entity.  

PubMed

Giant cell arteritis (GCA) is the most common of the vasculitis syndromes and, being a disease of the elderly, its incidence is increasing with the general ageing of the population. GCA is most feared for its early complications, namely blindness and stroke, resulting from inflammation and subsequent occlusion of ocular and extra cranial arteries, respectively. More recently, however, GCA has been recognised to also affect limb arteries and the aorta with a high prevalence. These newly recognised features of GCA pose diagnostic, therapeutic and prognostic challenges to treating physicians. Here, recent developments in the field of GCA are summarised and discussed. PMID:21956650

Kesten, F; Aschwanden, M; Gubser, P; Glatz, K; Daikeler, T; Hess, C

2011-01-01

439

Acute generalized exanthematous pustulosis due to clindamycin.  

PubMed

Acute generalized exanthematous pustulosis (AGEP) is a rare skin eruption most commonly caused by medications. It is characterized by fever and the acute eruption of non-follicular pustules overlying erythrodermic skin. Histopathology shows subcorneal pustules with a background of dermal edema and spongiosis, leukocytoclastic vasculitis, perivascular eosinophils, and focal necrosis of keratinocytes. Three cases of clindamycin induced AGEP have been reported in the literature. A case of AGEP due to clindamycin is reported in a patient with numerous other drug allergies and without history of psoriasis. Presentation and treatment of AGEP are reviewed. PMID:18718198

Sulewski, Ronald J; Blyumin, Marianna; Kerdel, Francisco A

2008-01-01

440

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

441

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

442

Call-Fleming syndrome  

PubMed Central

Call–Fleming syndrome is a part of reversible cerebral vasoconstriction syndrome (RCVS) group and is thought to be of idiopathic origin. It is classically described to be having multisegmental, focal vasospasms in the cerebral arteries. It is characterized clinically by the sudden onset of severe headache, classically described as thunderclap headache, with or without associated neurological deficits. The importance of it lies in that it is a potentially reversible cause of this clinical presentation, unlike its other counterparts, aneurysmal subarachnoid hemorrhage (SAH) or vasculitis. PMID:24347846

Skandhan, Avni Kalangott Padmanabhan; Ramakrishnan, Kollengode Gopalakrishnan; Anand, Rajeev

2013-01-01

443

Atypical Cogan's syndrome: A case report and summary of current treatment options.  

PubMed

Cogan's syndrome is a rare chronic vasculitis, characterized in its typical form by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. Only a few cases have been reported in children, most of whom have been diagnosed with the typical form. Early diagnosis and treatment are crucial to ensure a favorable prognosis. Systemic treatment usually begins with high dosage corticosteroids. In case the initial treatment fails, other immunosuppressive drugs are used (cyclophosphamide, methotrexate, cyclosporine A and azathioprine). Additional treatment possibilities, such as plasmapheresis, TNF-alpha blockers (etanercept and infliximab), rituximab, tocilizumab and mycophenolate mofetil have been described over the past few years. PMID:25600284

Jan?atová, Debora; Zeleník, Karol; Komínek, Pavel; Matoušek, Petr

2015-03-01

444

Recent Advances in the Immunopathogenesis of Systemic Lupus Erythematosus  

PubMed Central

Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disease having definite etiologic associations with ethnic, genetic, viral and immunologic factors. Its pathologic hallmark, vasculitis, is currently felt to be the end result of an immune-complex mechanism. Several clinical and serologic variants of SLE are recognized including discoid lupus erythematosus (DLE), mixed connective tissue disease (MCTD) and drug-induced equivalents—such as procainamide-induced lupus (PIL). The distinguishing features of these variants as well as their prognosis and therapy are discussed in relation to recent developments in the immunopathogenesis of SLE. PMID:46657

Bardana, Emil J.; Pirofsky, Bernard

1975-01-01

445

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

446

Extrahepatic manifestations in chronic hepatitis C virus carriers.  

PubMed

Patients with chronic hepatitis C virus (HCV) infection frequently present with extrahepatic manifestations covering a large spectrum, involving different organ systems leading to the concept of systemic HCV infection. These manifestations include autoimmune phenomena and frank autoimmune and/or rheumatic diseases and may dominate the course of chronic HCV infection. Chronic HCV infection causes liver inflammation affecting the development of hepatic diseases. HCV is also a lymphotropic virus that triggers B cells and promotes favorable conditions for B lymphocyte proliferation, including mixed cryoglobulinemia (MC) and MC vasculitis, which is the most prominent extrahepatic manifestation of chronic HCV infection. HCV may also promote a low-grade chronic systemic inflammation that may affect the development of some extrahepatic manifestations, particularly cardiovascular and cerebral vascular diseases. Recognition of extrahepatic symptoms of HCV infection could facilitate early diagnosis and treatment. The development of direct-acting antiviral agents (DDAs) has revolutionized HCV treatment. DDAs, as well as new B-cell-depleting or B-cell-modulating monoclonal antibodies, will expand the panorama of treatment options for HCV-related extrahepatic manifestations including cryoglobulinemic vasculitis. In this context, a proactive, integrated approach to HCV therapy should maximize the benefits of HCV therapy, even when liver disease is mild. PMID:25801890

Rosenthal, E; Cacoub, P

2015-04-01

447

Locked-in Syndrome and Blue Toe Syndrome Caused by Cardiopulmonary Bypass  

PubMed Central

Severe inflammation after cardiopulmonary bypass with the vasculitis of the acral extremity and vertebro-basilar arterial system leads to the locked-in syndrome and blue toe syndrome. In broad terms, systemic, idiopathic, and environmental factors provoke syndromes that present with digital discoloration or the blue toe syndrome. Painful digital discoloration, accompanied by ulceration, suggests vasculitis, involving small blood vessels. Definitive diagnosis usually requires histological documentation because vasculitic syndromes have no pathognomonic clinical features or laboratory test results. The case introduced herein is that of a woman who developed the locked-in syndrome in conjunction with quadriplegia, loss of facial movement, speech loss, and loss of horizontal eye movements. She had initially presented with severe mitral stenosis and left atrial clot and undergone mitral valve replacement and clot extraction. The patient expired from multiple organ failure despite prolonged ventilatory support, including tracheotomy, and meticulous nursing care and antibiotic prophylaxis. Given the previously reported partial recovery from this syndrome with the use of steroids, we would advocate the use of such pharmacological agents. PMID:23074585

Sabzi, Feridoun; Moloudi, Abdolrasoul

2010-01-01

448

A Systematic Literature Review of the Association of Lipoprotein(a) and Autoimmune Diseases and Atherosclerosis  

PubMed Central

Objective. To investigate the association of lipoprotein(a) and atherosclerosis-related autoimmune diseases, to provide information on possible pathophysiologic mechanisms, and to give recommendations for Lp(a) determination and therapeutic options. Methods. We performed a systematic review of English language citations referring to the keywords “Lp(a)” AND “autoimmune disease” AND “atherosclerosis,” “Lp(a)” AND “immune system” OR “antiphospholipid (Hughes) syndrome (APS)” OR “rheumatoid arthritis” OR “Sjögren's syndrome” OR “systemic lupus erythematosus” OR “systemic sclerosis” OR “systemic vasculitis” published between 1991 and 2011 using Medline database. Results. 22 out of 65 found articles were identified as relevant. Lp(a) association was highest in rheumatoid arthritis (RA), followed by systemic lupus erythematosus (SLE), moderate in APS and lowest in systemic sclerosis (SSc). There was no association found between Lp(a) and systemic vasculitis or Sjögren's syndrome. Conclusion. Immune reactions are highly relevant in the pathophysiology of atherosclerosis, and patients with specific autoimmune diseases are at high risk for CVD. Elevated Lp(a) is an important risk factor for premature atherosclerosis and high Lp(a) levels are also associated with autoimmune diseases. Anti-Lp(a)-antibodies might be a possible explanation. Therapeutic approaches thus far include niacin, Lp(a)-apheresis, farnesoid x-receptor-agonists, and CETP-inhibitors being currently under investigation. PMID:23304154

Missala, I.; Kassner, U.; Steinhagen-Thiessen, E.

2012-01-01

449

Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis.  

PubMed

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions. PMID:22641596

Stahelin, Letícia; Fialho, Sonia Cristina de Magalhães Souza; Neves, Fabrício Souza; Junckes, Larissa; Werner de Castro, Gláucio Ricardo; Pereira, Ivânio Alves

2012-01-01

450

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

451

The renin–angiotensin system as a primary cause of polyarteritis nodosa in rats  

PubMed Central

Abstract Polyarteritis nodosa is a necrotizing vasculitis of medium-sized arteries of unknown origin. Hypertension is present in 30% of patients with polyarteritis nodosa. In those cases, high renin levels are thought to be secondary to renal involvement. The present study was performed to identify causal factors of polyarteritis nodosa. In cyp1a1ren-2 transgenic rats, vasculitis of medium-sized arteries resembling classical polyarteritis nodosa can be induced. In this model, oral administration of indole-3-carbinol (I3C) activates the liver-specific cyp1a1 promoter, leading to prorenin expression in a dose-dependent manner. After the first 6 weeks of chronic induction with 0.125% I3C, the mean arterial pressure reached a plateau of about 170 mmHg. Ten out of 11 I3C-treated rats, which were chronically instrumented with a telemetric device to measure blood pressure, developed polyarteritis nodosa within 10 weeks of I3C treatment. I3C alone or instrumentation alone did not cause polyarteritis nodosa. The angiotensin-converting enzyme inhibitor captopril completely prevented the development of polyarteritis nodosa, indicating that local angiotensin II generation is a pathogenetic factor in this model. The renin–angiotensin system can play a primary role in the development of polyarteritis nodosa in rats. PMID:19432815

Peters, Barbara S; Kuttler, Beate; Beineke, Andreas; Lorenz, Gerd; Thiele, Andrea; Nicolai, Oliver; Rettig, Rainer; Mullins, John J; Peters, Jörg

2010-01-01

452

Case of Behçet's disease complicated by oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm.  

PubMed

Behçet's disease (BD) is a relapsing systemic inflammatory disorder of unknown etiology involving systemic vasculitis. Vasculitis in BD results from the involvement of arteries, veins and blood vessels of all sizes, which leads to the three major manifestations of this condition: venous occlusion, arterial occlusion and aneurysm formation. Therefore, whole-body vascular involvement should always be considered in BD patients. Here, we describe the first appearance of an internal carotid-posterior communicating artery aneurysm, resulting in complete oculomotor nerve palsy in a BD patient. A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. His condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Ten years after his initial clinical presentation, he had manifestations of right-sided ptosis and cyclic dull pain in his right temporal region. Magnetic resonance imaging and angiography revealed a right internal carotid artery-posterior communicating artery aneurysm. Although oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm in a BD patient has not been reported previously, our report highlights the fact that this abnormal manifestation should be considered in those with vasculo-BD. PMID:25573207

Yamaoka, Toshifumi; Murota, Hiroyuki; Katayama, Ichiro

2015-03-01

453

Cells perpetuating the inflammatory response in scleritis.  

PubMed Central

Scleritis can be a destructive disease frequently associated with autoimmune disorders. It is believed that primary vasculitis plays an important role in its pathogenesis, but little is known about the cellular effector mechanisms. The purpose of this study was to analyse the inflammatory cellular infiltrate in scleritis. Six episcleral biopsies and two enucleated eyes were studied. The episcleral biopsies were taken from patients with nodular scleritis. In one patient enucleation was done after perforation in anterior necrotising scleritis and, in the other after misdiagnosis of posterior scleritis as intraocular tumour. Morphological criteria and immunohistochemical methods were used to characterise the inflammatory cellular infiltrate. The inflammatory cells infiltrating the episcleral tissue were mainly T lymphocytes and macrophages. There was a predominance of CD4 positive cells, but only few lymphocytes were activated (expressed IL-2 receptor). The cells infiltrating the scleral fibres in the enucleated eyes consisted in both cases predominantly of T cells. Clusters of B cells were found in perivascular areas. In circumscribed areas neutrophils, macrophages, and plasma cells were part of the scleral infiltrate. Signs of a granulomatous process with activated macrophages (epithelioid and giant cells) were present in necrotising scleritis. Expression of major histocompatibility class II molecules (MHC II) was found on lymphocytes and rarely on macrophages. Signs of primary vasculitis were not found in any of the specimens. The cellular infiltrate in scleritis shows, at least at certain stages, features compatible with a T cell mediated (autoimmune) disorder, which may have major therapeutic implications. Images PMID:8025072

Bernauer, W; Watson, P G; Daicker, B; Lightman, S

1994-01-01

454

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

455

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

456

Outbreak of swine erysipelas in a semi-intensive wild boar farm in Spain.  

PubMed

Swine erysipelas (SE) is a disease caused by the bacterium Erysipelothrix rhusiopathiae and is one of the best-known and most serious diseases affecting domestic pigs. However, few studies exist concerning the susceptibility of wild boars to this disease and the role of this species as a reservoir. This study investigates and describes an outbreak of SE that occurred on a semi-intensive wild boar breeding farm housing 40 boars in Extremadura (SW Spain) on 11-18 February 2010. Seven animals died, of which four were examined post-mortem. Of these, three (two females and one male) were approximately 3 months old, and one was 1 year old (male). Lesions were consistent with acute septicaemia, consisting of cutaneous erythema/cyanosis and petechial haemorrhages in kidneys, urinary bladder, lungs and meninges. The 1-year-old male also had proliferative polyarthritis. Histopathology confirmed the presence of disseminated intravascular coagulation and vasculitis. Additionally, a bilateral acute panuveitis with concurrent necrotizing vasculitis and diffuse corneal oedema, neither of which have been described before in this disease, were found in the 3-month-old male boar. E. rhusiopathiae was isolated from all four animals in pure cultures from several tissues. Of these four animals, antibodies against E. rhusiopathiae, using an indirect ELISA test, were only detected in the 1-year-old male boar with polyarthritis. Posteriorly, of nine live adults tested for antibodies, four (including an adult male with polyarthritis) were positive. PMID:21649879

Risco, D; Llario, P F; Velarde, R; García, W L; Benítez, J M; García, A; Bermejo, F; Cortés, M; Rey, J; de Mendoza, J H; Gómez, L

2011-10-01

457

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

458

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

459

Levamisole-adulterated cocaine induced skin necrosis of nose, ears, and extremities: Case report  

PubMed Central

Levamisole is an immunomodulatory and antihelminthic drug, previously removed from the United States market, and now estimated to be present in the vast majority of cocaine distributed in the United States. Levamisole-adulterated cocaine (LAC) exposure can result in neutropenia, thrombocytopenia, and vasculitis with a predilection for subsites of the face. The objective of this review is to increase awareness among otolaryngologists of the manifestations of LAC exposure. We present the case of a 33-year-old woman with a history of cocaine use, consulted for purpuric, necrotic lesions of the nose, cheeks, and ears, with accompanying leukopenia, thrombocytopenia, and positive antineutrophil cytoplasmic antibodies (ANCA). The effects of levamisole are immune mediated, with antibodies directed against neutrophils causing neutropenia, and vasculitis caused by antibody deposition or secondary to induction of antiphospholipid antibodies causing thrombosis. LAC exposure can be differentiated from other similar appearing pathologies by evaluating serology for specific ANCA. The most important treatment is cessation of cocaine use, which most often results in complete resolution of symptoms. Awareness of the presentation, complications, and treatment of LAC exposure may be especially important for otolaryngologists, who may be one of the firsts to evaluate an affected patient. PMID:25565048

Jiron, Jose L.; Lin, Ho-Sheng; Folbe, Adam J.

2014-01-01

460

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

461

Hydrophilic polymer embolism and associated vasculopathy of the lung: prevalence in a retrospective autopsy study.  

PubMed

Hydrophilic polymers are commonly applied as surface coatings on vascular devices and have been shown to dissociate during endovascular use, causing hydrophilic polymer embolism (HPE). Adverse effects related to this phenomenon have been recognized and reported. The prevalence of this complication is unknown. We conducted a retrospective study to determine the prevalence of HPE among hospital autopsies over a 29-month period. Postmortem tissue was histologically evaluated for the presence, location(s) and extent of HPE. HPE findings were correlated with documented clinical and laboratory data and patient outcome. Of 136 hospital autopsies examined, 18 (13%) showed evidence of HPE involving the lungs (n = 18), heart (n = 1) or central nervous system (n = 1). Localized pulmonary HPE was seen in 12 patients (9%). Multifocal pulmonary HPE was found in 6 patients (4%) and was associated with clinical vasculitis (33%; P < .0001), suspected pulmonary ischemia (50%; P = .008), coagulopathy (67%; P = .002), and constitutional disease (83%; P = .01). Within affected lung, associated histopathologic changes included occlusive intravascular or perivascular inflammation (89%), intravascular fibrous response (56%), microthrombus formation (44%), vasculitis (28%), and/or pulmonary microinfarction (28%). Statistically significant differences in hospital days (P = .008) and number of vascular interventions (P = .01) were noted between affected and unaffected patients. We conclude that HPE is an underdiagnosed phenomenon with primary involvement of the lungs, where secondary vascular changes are common. Additional studies may be needed to clarify risks and to identify preventative strategies for this iatrogenic complication of catheterizations and "minimally invasive" endovascular techniques. PMID:25543660

Mehta, Rupal I; Mehta, Rashi I; Choi, Julia M; Mukherjee, Arnob; Castellani, Rudy J

2015-02-01

462

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

463

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

464

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

465

Microscopic polyangiitis associated with pulmonary fibrosis.  

PubMed

Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis characterised by inflammation of the small blood vessels, the absence of granulommas on histopathological specimens, with few or no immune deposits and the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCAs). The classic pulmonary manifestation is diffuse alveolar haemorrhage (DAH), but its association with pulmonary fibrosis (PF) has been increasingly reported and may be the first manifestation of MPA. Our aim was to evaluate MPA patients with PF and compare their characteristics and evolution to those of MPA patients without PF. We conducted a retrospective review of MPA patients followed in our hospital over a 15-year period. They were divided into two subgroups, with PF (MPA-PF) and without PF (MPA-non PF), and their clinical and functional features were compared. Nine of the 28 patients were classified as MPA-PF (32 %). This subgroup showed significantly more respiratory symptoms and higher mortality than MPA-non PF subgroup. The most frequent chest computed tomographic pattern of PF was usual interstitial pneumonia. PF preceded other manifestations of vasculitis in five patients and occurred simultaneously in the remaining four. During the follow-up period, four deaths were reported in the MPA-PF subgroup. No deaths were registered in the MPA-non PF subgroup. We found a high prevalence of MPA-PF patients (32 %), most of whom had a poor outcome and PF was often the first manifestation of the disease. PMID:24863847

Fernandez Casares, Marcelo; Gonzalez, Alejandra; Fielli, Mariano; Caputo, Flavia; Bottinelli, Yanina; Zamboni, Marcelo

2014-05-27

466

Pneumatosis intestinalis associated with Henoch-Schönlein purpura.  

PubMed

Henoch-Schönlein purpura (HSP) is the most common vasculitis in children. It is a disorder of the inflammatory cascade leading to immunoglobulin A deposition and leukocytoclastic vasculitis of small vessels of skin, kidneys, joints, and gastrointestinal (GI) tract. A wide variety of GI manifestations are seen in ?50% to 75% of patients with HSP. Diffuse colicky abdominal pain is the most common GI symptom. The small bowel is the most frequently involved GI site. Intussusception is rare but is the most common surgical complication. We report the case of a 2-year-old girl with a 5-day history of abdominal pain followed by a palpable purpuric rash. Her urinalysis, complete blood cell count, and tests of renal function were normal. An acute abdominal series was unremarkable initially, and abdominal ultrasound imaging showed ascites and thickened small bowel loops. She was diagnosed with HSP. The abdominal pain worsened, and an abdominal computed tomography scan demonstrated distal small bowel wall thickening and pneumatosis intestinalis in the descending colon. She was started on total parenteral nutrition and antibiotics and placed on bowel rest. She was given 2 mg/kg of intravenous immunoglobulin. Her abdominal pain gradually improved over the next week, and a repeat computed tomography scan showed significant improvement of the small bowel wall thickening and pneumatosis. The purpuric rash improved, and her abdominal pain resolved. We report a case of HSP and pneumatosis intestinalis, an association that has not been reported previously. PMID:25157006

Fatima, Ayesha; Gibson, Donald Paul

2014-09-01

467

Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis  

PubMed Central

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions. PMID:24955278

Memarpour, Roya; Johnston, Yasmin

2014-01-01

468

Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.  

PubMed

The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3-20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2-38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement. PMID:24646467

Calvo-Río, Vanesa; Loricera, Javier; Mata, Cristina; Martín, Luis; Ortiz-Sanjuán, Francisco; Alvarez, Lino; González-Vela, M Carmen; González-Lamuño, Domingo; Rueda-Gotor, Javier; Fernández-Llaca, Héctor; González-López, Marcos A; Armesto, Susana; Peiró, Enriqueta; Arias, Manuel; González-Gay, Miguel A; Blanco, Ricardo

2014-03-01

469

Nonsystemic vasculitic neuropathy: update on diagnosis, classification, pathogenesis, and treatment.  

PubMed

The primary systemic vasculitides are autoimmune disorders characterized by chronic immune responses directed against vascular structures. They commonly affect small or medium-sized vessels in the peripheral nervous system (PNS), producing vasculitic neuropathies. Some patients develop vasculitis clinically restricted to the PNS, known as nonsystemic vasculitic neuropathy (NSVN), the most commonly encountered vasculitic neuropathy in pathologically based series. Diabetic and nondiabetic radiculoplexus neuropathies are clinical variants of NSVN. NSVN is clinically similar to systemic vasculitis-associated neuropathies except for reduced severity. Patients most commonly present with progressive, stepwise pain, weakness, and numbness over multiple months. Almost all exhibit a multifocal or asymmetric, distally accentuated pattern of involvement. The most commonly affected nerves are the common peroneal nerve in the leg and the ulnar nerve in the arm. Sedimentation rate is mildly to moderately elevated in 50%; other markers of systemic inflammation are generally normal. Electrodiagnostic studies reveal a predominantly axonal, asymmetric, sensorimotor polyneuropathy, but pseudo-conduction blocks may occur. Definite diagnosis requires biopsy evidence of vascular inflammation and signs of active or remote vascular damage. In biopsies lacking definite vasculitis, the diagnosis is suspected if axonal alterations are accompanied by perivascular inflammation and such supportive features as Wallerian-like degeneration, asymmetric fiber loss, hemosiderin, vascular immune deposits, neovascularization, myofiber necrosis/regeneration, focal perineurial damage, and endoneurial purpura. NSVN preferentially affects larger epineurial arterioles. Epineurial infiltrates are composed primarily of T cells and macrophages, suggesting that cellular cytotoxicity is the primary effector mechanism. Systemic vasculitides with progressive neuropathy are usually treated with cyclophosphamide and prednisone. No randomized controlled trial of therapy has been performed in NSVN, but data from retrospective cohorts suggest that combination therapy is more effective than steroid monotherapy. Once remission has been induced, cyclophosphamide should be replaced with azathioprine or methotrexate. Refractory patients can be treated with intravenous immunoglobulin, mycophenolate, rituximab, infliximab, or alemtuzumab. Although long-term outcome is reasonably good, more than one third of patients relapse, infrequent patients die from the disease or its treatment, and still others develop chronic pain. PMID:19349704

Collins, Michael P; Periquet-Collins, Isabel

2009-01-01

470

Successful induction of granulomatosis with polyangiitis with tacrolimus  

PubMed Central

We report a 50-year-old female who presented with inflammatory arthritis, upper respiratory tract symptoms, and microscopic hematuria with nephrotic range proteinuria. Antineutrophil cytoplasmic antibodies (ANCA) were detectable and kidney biopsy showed pauci-immune focal necrotizing crescentic glomerulonephritis. She was treated with pulse intravenous cyclophosphamide (CYC) and prednisolone. Patient developed severe leucopenia after the first dose and subsequently had leucopenia to low dose CYC, mycophenolate mofetil and azathioprine were also tried. However, patient developed leukopenia with all the above agents. Initiation of tacrolimus (TAC) was followed by dramatic response: Proteinuria decreased, serum albumin normalized and C-ANCA and anti-PR3 ANCA assays became negative. This is the first successful case of TAC as an indu