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1

Vasculitis  

MedlinePLUS

Vasculitis is an inflammation of the blood vessels. It happens when the body's immune system attacks the ... or another disease. The cause is often unknown. Vasculitis can affect arteries, veins and capillaries. Arteries are ...

2

Allergic vasculitis  

MedlinePLUS

Vasculitis - allergic; Hypersensitivity vasculitis; Cutaneous leukocytoclastic vasculitis ... Allergic vasculitis is caused by an allergic reaction to a drug, an infection, or other foreign substance. It most ...

3

Hypersensitivity Vasculitis  

MedlinePLUS

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

4

Vasculitis Foundation  

MedlinePLUS

... letter from our founder, Marilyn Sampson. Vasculitis Events California Stanford University, 450 Serra Mall, Stanford, CA 94305, ... 7, 2015 at Stanford University in Palo Alto, California. The conference will feature speakers from both the ...

5

Necrotizing vasculitis  

MedlinePLUS

Stone JH. Classification and epidemiology of systemic vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology . 9th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap ...

6

Pustular vasculitis.  

PubMed

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

Central Nervous System Vasculitis  

MedlinePLUS

... Vasculitis? Reversible Cerebral Vasoconstriction Syndromes (RCVS) comprises a group of diverse conditions, all characterized by spasm of ... Vasculitis Clinical Research Consortium (VCRC) is an integrated group of academic medical centers, patient support organizations, and ...

9

Vasculitis in Antiphospholipid Syndrome.  

PubMed

The major manifestations of antiphospholipid syndrome (APS) are caused by thrombosis within the venous or arterial vasculature, whereas the vascular lesions in systemic vasculitis result from an inflammatory infiltrate in the vessel wall. There is an association between vascular thrombosis and inflammation, however, as vasculitis can occur in APS and thromboembolic complications are seen in systemic vasculitis. Although differentiating between vasculitis and antiphospholipid-associated thrombosis can be difficult, it may be crucial to do so given the different therapeutic implications for immunosuppression or anticoagulation. This article explores the relationship between thrombosis and inflammation as it relates to APS and systemic vasculitis. PMID:25399943

Lally, Lindsay; Sammaritano, Lisa R

2015-01-01

10

Vasculitis in Systemic Sclerosis  

PubMed Central

Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet's disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment. PMID:20976300

Kao, Lily; Weyand, Cornelia

2010-01-01

11

Patients with Retinal Vasculitis Rarely Suffer from Systemic Vasculitis  

PubMed Central

Objectives Systemic vasculitis is often mistakenly assumed to be a common cause of retinal vasculitis. We sought to determine the relationship between retinal vasculitis and systemic vasculitis. Methods A selected review was performed on 1390 charts of patients attending the uveitis clinic at the Oregon Health & Science University between 1985 and 2010. Included in the review were all patients with diagnoses commonly associated with retinal vasculitis and all patients who were diagnosed with a systemic vasculitis. Retinal vasculitis was identified by perivascular exudates, intraretinal hemorrhage, or cotton wool spots as seen on clinical examination or by vascular occlusion or leakage as identified by fluorescein angiogram. Results 207 or 14.9% of patients with uveitis had retinal vasculitis as a component of the intraocular inflammation. Thirty-five patients had retinal vasculitis which was primary, i.e. not associated with a systemic disease, and the dominant manifestation of the uveitis. Fourteen of the patients with retinal vasculitis had Behcet’s disease. Only 11 of the 1390 patients with uveitis had a systemic vasculitis. Of these 11, four had retinal vasculitis including one secondary to a CMV retinitis. Thus, systemic vasculitis was directly responsible for 1.4% or 3 of 207 cases of retinal vasculitis. No-vasculitic systemic diseases such as sarcoidosis (n=13), syndromes confined to the eye such as pars planitis (n=36), and intraocular infections (n=29) were far more common causes of retinal vasculitis. Conclusion Retinal vasculitis is a relatively common feature of uveitis. Patients with retinal vasculitis, however, rarely suffer from one of the classical systemic vasculitides. PMID:22177107

Rosenbaum, James T.; Ku, Jennifer; Ali, Amro; Choi, Dongseok; Suhler, Eric B.

2014-01-01

12

Biomarkers in Vasculitis  

PubMed Central

Purpose of review Better biomarkers are needed for guiding management of patients with vasculitis. Large cohorts and technological advances had led to an increase in pre-clinical studies of potential biomarkers. Recent findings The most interesting markers described recently include a gene expression signature in CD8+ T cells that predicts tendency to relapse or remain relapse-free in ANCA-associated vasculitis, and a pair of urinary proteins that are elevated in Kawasaki disease but not other febrile illnesses. Both of these studies used “omics” technologies to generate and then test hypotheses. More conventional hypothesis-based studies have indicated that the following circulating proteins have potential to improve upon clinically available tests: pentraxin-3 in giant cell arteritis and Takayasu’s arteritis; von Willebrand factor antigen in childhood central nervous system vasculitis; eotaxin-3 and other markers related to eosinophils or Th2 immune responses in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome); and MMP-3, TIMP-1, and CXCL13 in ANCA-associated vasculitis. Summary New markers testable in blood and urine have the potential to assist with diagnosis, staging, assessment of current disease activity, and prognosis. However, the standards for clinical usefulness, in particular the demonstration of either very high sensitivity or very high specificity, have yet to be met for clinically relevant outcomes. PMID:24257367

Monach, Paul A.

2014-01-01

13

Small vessel vasculitis  

Microsoft Academic Search

The pediatric small vessel vasculitides reviewed in this article are Henoch–Schönlein purpura (HSP) and the anti-neutrophil\\u000a cytoplasmic antibody-associated vasculitides (AAV). The new classification criteria for HSP and Wegener’s granulomatosis are\\u000a now validated and will facilitate the conduct of future epidemiological studies and clinical trials. The clinical manifestations\\u000a of small vessel vasculitis in children are described, and current therapies discussed. There

Paul Brogan; Despina Eleftheriou; Michael Dillon

2010-01-01

14

CMR in inflammatory vasculitis.  

PubMed

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

Raman, Subha V; Aneja, Ashish; Jarjour, Wael N

2012-01-01

15

Eosinophilic vasculitis in connective tissue disease  

Microsoft Academic Search

Background: Neutrophilic and lymphocytic vascular inflammation is common in vasculitis associated with connective tissue disease (CTD). We recently identified eight patients with CTD and eosinophilic vasculitis.Objective: The purpose of this study was to characterize a variant form of vasculitis in CTD with eosinophilic infiltration.Methods: Of 98 CTD patients with cutaneous necrotizing vasculitis, eight were found with predominantly eosinophilic vascular infiltration.

Ko-Ron Chen; W. P Daniel Su; Mark R Pittelkow; Doyt L Conn; Terry George; Kristin M Leiferman

1996-01-01

16

[Cerebral vasculitis associated with gemcitabine.  

PubMed

Neoadjuvant chemotherapy consisting of cisplatin and gemcitabine was given to a 50-year-old woman suffering from transitional cell carcinoma of the bladder. Whereas the first cycle was administered without major side effects, the patient experienced a generalized tonic-clonic seizure and a prolonged cognitive deficit with the second cycle. Magnetic resonance imaging of the brain was consistent with cerebral vasculitis. The short interval between the application of gemcitabine and the neurological deterioration suggests a causal relationship. Although recent reports have linked this drug with leukoencephalopathy and vasculitis in various localizations, this is the first case of cerebral vasculitis associated with gemcitabine. PMID:20063084

Schmorl, P; Heer-Sonderhoff, A; Vosshenrich, R; Conrad, S

2010-01-10

17

[Cerebral vasculitis associated with gemcitabine].  

PubMed

Neoadjuvant chemotherapy consisting of cisplatin and gemcitabine was given to a 50-year-old woman suffering from transitional cell carcinoma of the bladder. Whereas the first cycle was administered without major side effects, the patient experienced a generalized tonic-clonic seizure and a prolonged cognitive deficit with the second cycle. Magnetic resonance imaging of the brain was consistent with cerebral vasculitis. The short interval between the application of gemcitabine and the neurological deterioration suggests a causal relationship. Although recent reports have linked this drug with leukoencephalopathy and vasculitis in various localizations, this is the first case of cerebral vasculitis associated with gemcitabine. PMID:20213928

Schmorl, P; Heer-Sonderhoff, A; Vosshenrich, R; Conrad, S

2010-02-01

18

[Vasculitis and vasculopathy].  

PubMed

Many pathophysiological process components are known to be implicated in lower limb ulcerations, among which vascular lesions have a major role. Vasculitis denotes a heterogeneous group of clinical entities which all are characterized by the inflammatory process of arterial and venous walls of any size and in any organ, quite frequently in the skin. Vasculopathy, on the other hand, refers to vascular and capillary lesions caused by, for example, some medications. The classification of vasculitides according to the size of the blood vessels involved serves for proper understanding the issue among clinicians and researchers, and not as a diagnostic tool. According to histologic finding obtained by examination of blood vessel biopsy specimen, vasculitides are divided into three groups: lymphocytic, leukocytoclastic and granulomatous. Livedoid vasculitis (livedo reticularis) most commonly affects women and is generally localized on lower extremities. The etiology oflivedoid vasculitis may imply autoimmune diseases, capillary obstruction with cryoglobulins, or antiphospholipid syndrome. Livedoid vasculopathy is a hyalinization disease of the vasculature, with thromboses and ulcerations on lower extremities, and of unknown etiology. Livedoid vasculopathy has been singled out as a separate disease that usually does not occur consequentially to other primary diseases. Livedoid vasculopathy typically affects women (71%) at a mean age of 45 (range 10-85) years; bilateral involvement of both lower limbs is present in 80.8%, disease manifested with ulcerations in 68.9%, ulcerations followed by development of atrophie blanche in 71.1%, transcutaneous oximetry reduction is found in 74.1%, factor V mutation (Leiden heterozygotes) in 22.2%, reduced protein C activity in 13.3%, prothrombin gene mutation (G20210A) in 8.3%, positive lupus anticoagulant in 17.9%, positive anticardiolipin antibodies in 28.6%, and elevated homocysteine level in 14.3% cases; blood vessel histology shows intraluminal thrombosis in 97.8% of patients, while direct immunofluorescence of blood vessel specimen shows immunoglobulins and complement components in blood vessels on the surface, in the mid-dermis as well as deep in the dermis. The immunofluorescence pattern differs from that found in immune complex diseases. Some of the agents tried in the treatment of livedoid vasculopathy include pentoxifylline, low-molecular heparin, hyperbaric oxygen therapy, methylprednisolone i.v. with pentoxifylline, recombinant tissue plasminogen activator, intravenous immunoglobulins, phenformin (biguanide) and ethylestrenol (anabolic steroid) combination, warfarin, heparin, systemic photochemotherapy (PUVA with oral psoralen), and low-molecular dextran. Infected ulcerations are treated with antibiotics. Combined therapy with folic acid, vitamin B12 and vitamin B6 can also be used. PMID:23193816

Markovi?, Asjastipi?

2012-10-01

19

Pulmonary vasculitis: CT features.  

PubMed

The pulmonary vasculitides are a heterogeneous group of inflammatory disorders that may be primarily localized to the pulmonary vasculature or be systemic in nature. The primary pulmonary vasculitides usually affect small vessels, whereas the systemic vasculitides can involve any size pulmonary vessels. For all types of vasculitis, there is a high degree of overlap within groups in regard to their appearance at computed tomography (CT). The most common CT findings are the result of either diffuse or focal pulmonary hemorrhage, are nonspecific, and include ground-glass opacity, consolidation, and small centrilobular nodules. Therefore, the CT findings must be considered in concert with the history, physical examination, and laboratory examination when a specific diagnosis is sought. This review will discuss the typical CT features of both the systemic and the primary pulmonary vasculitides, drawing a distinction where CT is helpful in differentiating among the various causes. PMID:16088562

Ravenel, James G; McAdams, H Page

2003-08-01

20

Methotrexate use in systemic vasculitis.  

PubMed

Although GS and CYC have been important agents in improving the outcome and survival of patients with systemic vasculitis, they carry their own risk of drug-induced morbidity and mortality. It has also become apparent that these medications are not the final answer in disease management because some forms of vasculitis have the potential to relapse or be treatment resistant. For these reasons, the pursuit of effective, less toxic therapeutic alternatives is critical. Initial results from the use of MTX in systemic vasculitis have been encouraging. Although drug-related toxicity and disease relapse have still been found to occur, MTX appears to be a valuable addition in the treatment of vasculitis. Further studies will be necessary to determine the optimal way that this agent may be used to safely and effectively manage vasculitic disease. PMID:9361158

Langford, C A; Sneller, M C; Hoffman, G S

1997-11-01

21

Vasculitis of the nervous system  

Microsoft Academic Search

Vasculitis is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral\\u000a nervous system (PNS), or both. This involvement may be primary and restricted to the CNS, and rarely to the PNS. Vasculitis\\u000a is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral nervous\\u000a system

Aksel Siva

2001-01-01

22

Differential Diagnosis of Retinal Vasculitis  

PubMed Central

Retinal vaculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings. PMID:20404987

Abu El-Asrar, Ahmed M.; Herbort, Carl P.; Tabbara, Khalid F.

2009-01-01

23

Infections in vasculitis.  

PubMed

Infections, mainly viral, are the cause of some vasculitides, like polyarteritis nodosa (hepatitis B virus) or mixed cryoglobulinemia (hepatitis C virus), and it has been hypothesized that others might be due to infectious agents (HIV, EBV, parvovirus...). Among etiologies of vasculitis, the responsibility of a Burkholderia-like strain has been recently demonstrated as the cause of giant-cell arteritis. On the other hand, patients frequently develop infections, mainly as a consequence of steroids, immunosuppressants and most immunomodulating treatments prescribed to treat vasculitides. Infections occur when patients receive steroids and immunosuppressants, especially in the long term. They are more frequently observed in elderly patients or in patients with poor general condition. Infection risk is not reduced when biotherapies are prescribed to induce or maintain remission. Patients, considered at higher risk for infections, should be followed closely and their immunological status monitored periodically. We recommend especially to monitor neutrophiles, lymphocytes and if needed CD3-, CD4- and CD8-cell counts in patients receiving steroids and cyclophosphamide or other cytotoxic agents. In patients treated with rituximab, CD19 and gammaglobulins should be monitored regularly. Prophylaxis are needed in patients at risk to develop infections. PMID:23507054

Guillevin, Loďc

2013-02-01

24

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

25

[Vasculitis - Diagnostic and therapeutic advances].  

PubMed

Vasculitis is characterized by inflammation and necrosis of blood vessels walls. It represents a heterogeneous group of conditions, whose etiopathogenic mechanisms remain unclear. Although uncommon, with an annual incidence of 40-54 cases per 1.000.000 persons, this is an important cause of multiorganic dysfunction and premature mortality. Depending on the affected vessels, it can cause diverse clinical presentations, which makes difficult its recognition. It is therefore a challenge for any clinician. This paper reviews the diagnostic and therapeutic advances of the most common forms of vasculitis, in order to optimize the approach and management of this clinical entity. We have conducted a search in Medline database on articles written in English, published for the last 10 years using the keywords: vasculitis, epidemiology, classification, diagnosis and treatment. To minimize the impact of vasculitis it is essential an early diagnosis, allowing a timely institution of the appropriate treatment. The diagnosis depends on the integration of clinical, laboratory, imaging and histopathologic data. According to the clinical condition, it may be indicated the removal of the offending antigen, the treatment of the underlying disease or specific treatment of the primary vasculitis. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide has revolutionized the prognosis of these patients but, despite its efficacy, it is associated with frequent relapses and significant toxicity. The study of the pathogenesis has been providing more effective and safer diagnostic and therapeutic options, for example B-cell depleting agents, but additional studies are needed to confirm the potential of these alternatives. PMID:25596397

De Albuquerque, R; Machado, Filipa

2015-01-01

26

Circulating Immune Complexes in Cutaneous Vasculitis  

PubMed Central

To investigate the pathogeneic significance of immune complexes in cutaneous vasculitis, 107 patients with various forms of cutaneous vasculitis, including 59 patients with necrotizing (leukocytoclastic) vasculitis (group 1), and 48 patients with lymphocytic vasculitis, or a predominately lymphocytic perivascular infiltrate (group 2), were studied. Immunoglobulins or complement components in cutaneous blood vessels were detected by direct immunofluorescence in high frequency in both groups (91 and 88%, respectively). Using two radioassays for circulating immune complexes, Clq or monoclonal rheumatoid factor (mRF) reactive material was detected in 68% of the patients with necrotizing vasculitis but only 44% of the patients in the lymphocytic-perivascular group. The mRF radioassay was elevated in 58% of the first group of patients and 41% of the patients in group 2, although Clq binding activity was increased in 54% of the patients with necrotizing vasculitis but only in 9% of the patients with a lymphocytic vasculitis or lymphocytic perivascular infiltrate. By using both sucrose density gradient ultracentrifugation and Sepharose 6B gel filtration, the Clq and mRF reactive material detected in some patients with necrotizing vasculitis eluted in high molecular weight fractions that were also anticomplementary. In one patient with necrotizing vasculitis and hepatitis B antigenemia, these heavy molecular weight Clq and mRF reactive fractions contained a two- to three-fold increase in hepatitis B surface antigen when compared with lighter molecular weight fractions. Heavy and light molecular weight mRF reactive material could be detected in selected patients in the lymphocytic-perivascular group as well as in the necrotizing vasculitis group. These studies suggest that cutaneous vasculitis, including acute necrotizing (leukocytoclastic) vasculitis and some forms of lymphocytic vasculitis, and perhaps some diseases characterized by a lymphocytic perivascular infiltrate, may represent cutaneous expressions of immune complex disease. Images PMID:500831

Mackel, Susan E.; Tappeiner, Gerhard; Brumfield, Hilton; Jordon, Robert E.

1979-01-01

27

Vasculitis associated with connective tissue disorders  

Microsoft Academic Search

Vasculitis associated with connective tissue disorders is an important cause of secondary vasculitis about which little is\\u000a written. When vasculitis occurs in the setting of a preexisting connective tissue disorder, it often correlates with disease\\u000a severity and portends a poorer prognosis. It may involve virtually any organ system and present in a myriad of ways. Prompt\\u000a recognition and treatment of

Mittie K. Doyle

2006-01-01

28

Medium-size-vessel vasculitis  

PubMed Central

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

Eleftheriou, Despina; Brogan, Paul A.

2009-01-01

29

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

30

Ischemic Neuropathy Associated with Livedoid Vasculitis  

PubMed Central

Background Livedoid vasculitis is a chronic dermatological problem with an unclear etiology. Clinical findings are petechiae with painful ulcers in both lower extremities, which heal to become hyperpigmented and porcelain-white satellite lesions. There are only a few reported cases of livedoid vasculitis presenting in combination with peripheral neuropathy. Case Report We report the first case of a Korean patient presenting with mononeuritis multiplex combined with livedoid vasculitis, which was confirmed by electrophysiological and pathological studies. Conclusions Our report supports the possible vaso-occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy. PMID:22259622

Kim, Jee-Eun; Park, Su-Yeon; Sinn, Dong In; Kim, Sung-Min; Hong, Yoon-Ho; Park, Kyung Seok; Lee, Kwang-Woo

2011-01-01

31

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

32

Challenging Mimickers of Primary Systemic Vasculitis.  

PubMed

The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease. PMID:25399945

Miloslavsky, Eli M; Stone, John H; Unizony, Sebastian H

2015-01-01

33

Vasculitis  

MedlinePLUS

... Division of Intramural Research Research Resources Scientific Reports Technology Transfer Clinical Trials What Are Clinical Trials? Children & ... Inflammation” refers to the body’s response to injury, including injury to the blood vessels. Inflammation may involve ...

34

Vasculitis  

MedlinePLUS

... microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Other lab tests can show damage to organs, ... granulomatosis) Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) Primary angiitis of the central nervous system Small ...

35

Skin involvement in cutaneous and systemic vasculitis.  

PubMed

Cutaneous vasculitides are a heterogeneous group of inflammatory disorders affecting skin blood vessels. They may be triggered by several factors, such as infection or drug, or may be related to underlying disease, notably connective tissue or malignancies. However, vasculitis occurs without any demonstrable triggering agents in a relevant number of patients. On the other hand, vasculitic skin lesions may manifest as a component of vasculitis affecting also internal organs; in someone of these patients, skin involvement occurs initially as the sole sign of disease, leading to consider cutaneous vasculitis a diagnosis of exclusion. In this review, we have focused on the most common variants of cutaneous vasculitis, including cutaneous small vessel vasculitis and urticarial vasculitis as well as Henoch-Schönlein purpura, a systemic form in which however skin involvement often predominates. We have also argued on livedoid vasculopathy, a cutaneous entity which, although nonfrankly vasculitic in origin, is frequently associated with connective tissue disease. Finally, we have analyzed the variety of cutaneous manifestations that may develop during the course of the main systemic vasculitides, such as Wegener's granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa. PMID:22959234

Marzano, Angelo V; Vezzoli, Pamela; Berti, Emilio

2013-02-01

36

History of primary vasculitis in Latin America.  

PubMed

A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge. PMID:20190698

Iglesias Gammara, Antonio; Coral, Paola; Quintana, Gerardo; Toro, Carlos E; Flores, Luis Felipe; Matteson, Eric L; Restrepo, José Félix

2010-03-01

37

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

38

Small vessel vasculitis of the skin.  

PubMed

Small vessel vasculitis in the skin manifests with palpable purpura on the lower extremities. This clinical presentation prompts a complete physical examination, history, and review of systems, as well as biopsies for routine processing and direct immunofluorescence to confirm the diagnosis. The presence of vasculitis in other organs, associated underlying conditions, and the severity of cutaneous manifestations dictate management. The majority of cases are self-limited, and overall the prognosis is favorable. Still, a subset of patients can have serious complications and chronic or recurrent disease. PMID:25399937

Micheletti, Robert G; Werth, Victoria P

2015-01-01

39

Maintaining remission in a patient with vasculitis  

Microsoft Academic Search

Background A 40-year-old man was referred to a specialist vasculitis center 4 years after being diagnosed with Wegener's granulomatosis. At the time of diagnosis he had presented with skin, ear, nose and throat involvement, pulmonary hemorrhage, and microscopic hematuria. Remission was achieved with plasma exchange and with daily oral prednisolone and cyclophosphamide. The patient was switched to maintenance treatment with

David RW Jayne; Oliver Flossmann

2008-01-01

40

Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation.  

PubMed

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

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

2013-03-01

41

Vasculitis in the connective tissue diseases  

Microsoft Academic Search

Vasculitides in the setting of connective tissue diseases are generally thought to be infrequent and relatively little is\\u000a written about them. They are, however, important both because they may pose diagnostic and therapeutic challenges and affect\\u000a prognosis. In each of the connective tissue diseases, vasculitis can present in various clinical and pathologic forms adding\\u000a to their diagnostic and therapeutic difficulties.

Luis Felipe Flores-Suárez; Donato Alarcón-Segovia

2000-01-01

42

CHILDHOOD CUTANEOUS VASCULITIS: A COMPREHENSIVE APPRAISAL  

PubMed Central

Cutaneous vasculitides in childhood are rare and often present with clinical features distinct from adults. Diagnosis of cutaneous vasculitides in children was difficult because of lack of a satisfactory classification systems for this age group. A new international classification system for childhood vasculitis has been discussed in the following section along with important clinical features, diagnostic modalities, and recent therapeutic developments of important vasculitides in children. PMID:20101304

Palit, Aparna; Inamadar, Arun C

2009-01-01

43

Relapses in patients with a systemic vasculitis.  

PubMed

Treatment with cyclophosphamide and steroids has greatly improved survival in patients with systemic necrotizing vasculitis but does not always provide a complete cure. There are as yet few data on the incidence, pattern and outcome of relapses in these diseases. We studied relapses in 150 consecutive patients with an idiopathic necrotizing vasculitis: 12 with classical polyarteritis (CPAN); 95 with microscopic polyarteritis (MPA); 28 with Wegener's granulomatosis (WG); and 15 with limited Wegener's granulomatosis (LWG). The relapse rates and median time to relapse in months were: CPAN, 41.7%/33; MPA, 25.4%/24; WG, 44%/42; LWG, 52%/18. The clinical features of relapse were similar to or more aggressive than those of the original presentation in CPAN and LWG and included renal disease for the first time, but in MPA and WG, relapse involved less renal involvement in the majority of cases. Laboratory tests, although often positive at relapse, were unhelpful in its prediction. The considerable accumulative non-fatal relapse rate contrasts with the very good long-term survival rates, and confirms the importance of long-term follow-up in systemic vasculitis. PMID:7906421

Gordon, M; Luqmani, R A; Adu, D; Greaves, I; Richards, N; Michael, J; Emery, P; Howie, A J; Bacon, P A

1993-12-01

44

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

45

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

46

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

PubMed

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

Holl-Ulrich, K

2014-11-01

47

Childhood vasculitis hospitalizations in Spain, 1997-2011.  

PubMed

The aim of this study is to describe the childhood vasculitis hospital burden in Spain (1997-2011), considering type of disease, hospitalization rates and time trends. Data were obtained from the National Discharges Basic Minimum Data Set (National Patient Data Base). Inpatient events of children younger than 15 years of age were analyzed. Principal diagnosis of vasculitis were selected according Ninth Revision of the International Classification of Diseases: Takayasu arteritis, Polyarteritis nodosa, Kawasaki disease, Wegener`s granulomatosis, Churg-Strauss syndrome, and Henoch-Schönlein purpura. A total of 14518 children hospitalizations related to vasculitis were identified in Spain from 1997 to 2011. The average hospitalization rate for children was 13.33±1.71 per 100,000. Henoch-Schönlein purpura and Kawasaki disease were the most common type of vasculitis, hospitalization rates were 11.00 and 3.97 per 100,000 children, respectively. Other vasculitis hospitalizations are much rare in childhood. Average length of stay was 6.04 days and estimated cost per inpatient hospital care was 2,847€. Hospital case fatality rate was 0.05% for overall vasculitis. In conclusion, epidemiological data of childhood vasculitis are useful both to health decision-making and to identify research priorities. PMID:24940860

Villaverde-Hueso, A; Alonso, V; Morales-Piga, A; Hens-Pérez, M; Abaitua, I; Posada-de-la-Paz, M

2014-05-01

48

Evaluation, treatment, and prophylaxis of infections complicating systemic vasculitis.  

PubMed

Infection continues to be a major cause of morbidity and mortality in patients with systemic vasculitis. The recognition and treatment of infections in these patients is a particularly difficult task because the spectrum of potential pathogens is broad and the clinical manifestations of infection often mimic those of the underlying disease. This article provides 1) a general overview of the immunosuppressive properties of agents commonly used in the treatment of systemic vasculitis, and 2) a framework for the diagnostic evaluation and treatment of various infectious syndromes in patients with systemic vasculitis. PMID:9448988

Sneller, M C

1998-01-01

49

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

50

Lepra Reaction with Lucio Phenomenon Mimicking Cutaneous Vasculitis  

PubMed Central

Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis. PMID:25580317

Misra, Durga Prasanna; Parida, Jyoti Ranjan; Chowdhury, Abhra Chandra; Pani, Krushna Chandra; Kumari, Niraj; Krishnani, Narendra

2014-01-01

51

Skin vasculitis during Creutzfeldt-Jakob's disease.  

PubMed

We report the case of a 72-year-old woman with sporadic Creutzfeldt-Jakob's disease who presented a large purplish erythematous and edematous lesion, with subsequent bullous detachment on the anterior right thigh. The lesion rapidly evolved into an ulcer covered by a blackish necrotic eschar. Histological examination showed intense necrotizing leukocytoclastic vasculitis in the deep and middle dermis. Direct immunofluorescence revealed C3 and IgM deposits around vessels of the middle and deep dermis. The diagnosis of sporadic Creutzfeldt-Jacob's was confirmed post-mortem by immunoblotting on frozen brain tissue which showed pathologic proteinase-resistant prion-related protein isoform glycotype 2A. In the literature, only two cases of Creutzfeldt-Jakob's disease and cutaneous manifestations are reported. PMID:16436347

Satta, Rosanna; Pala, Antonello; Rosati, Giulio; Cottoni, Francesca

2006-01-01

52

Cerebral vasculitis and Cardiobacterium valvarum endocarditis.  

PubMed

We present a case of aortic and tricuspid native valve endocarditis in which Cardiobacterium valvarum was isolated from the blood culture of a 65-year-old man. Cardiobacterium valvarum is a fastidious, Gram-negative bacillus. The genus Cardiobacterium encompasses two species - Cardiobacterium valvarum and Cardiobacterium hominis. Although both species rarely feature as the aetiological agent of endocarditis, Cardiobacterium hominis has a higher incidence than Cardiobacterium valvarum. For this causative organism, we believe this is the first report of fatality prior to surgical intervention and the first clinical course to be complicated by cerebral vasculitis. Native valve endocarditis caused by Gram-negative bacilli is extremely rare and identification of isolates may require the use of reference laboratories with molecular identification techniques. PMID:22878249

Abraham, R; Irwin, R B; Kannappan, D; Isalska, B; Koroma, M; Younis, N

2012-11-01

53

Ultrastructural aspects in skin allergic vasculitis.  

PubMed

Twelve cases of skin allergic vasculitis were electronmicroscopically studied. The most striking changes were noticed at the level of capillaries, whose wall components were all affected, especially the endothelial cells. These presented at first adaptive changes, with swelling, microvillosities of plasmalemma protruding into the lumen, and many pinocytotic vesicles showing an intensely active transport. Followed different degrees of degenerative changes of cytoplasms and nuclei, the junctional complexes being interrupted, even discontinuous. The pericytes showed similar changes. The basement membrane was thickened, nonhomogeneous, with a spongy aspect, small discontinuities and some electron-dense depots. The endothelial cell damage and the discontinuity of basement membranes, as well as the alteration of pericytes allowed the migration of blood cells into interstitial spaces, followed by leucocytoclasia. As a consequence, in the perivascular tissue a polymorphous cellular infiltrate developed. The mechanisms and significance of these changes are discussed. PMID:6220209

Dobrescu, G; Dobrescu, A; Stoica, L

1983-01-01

54

Churg-Strauss syndrome and hemorrhagic vasculitis  

PubMed Central

Churg-Strauss syndrome (CSS) is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the left-hand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome. PMID:25386301

Marques, Rui Moreira; Cabral, Ana Rita; Monteiro, Antonio; Henriques, Pedro

2011-01-01

55

Current Therapies for ANCA-Associated Vasculitis.  

PubMed

The ANCA-associated vasculitides, granulomatosis with polyangiitis (GPA, formerly Wegener's), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss), are a group of multi-system autoimmune diseases characterized by necrotizing small- to medium-vessel vasculitis and the presence of anti-neutrophil cytoplasmic antibodies. Current therapeutic strategies consist of glucocorticoids in conjunction with either conventional or biologic agents for both induction of remission and remission maintenance. Treatment goals include reducing toxicity of induction therapy, preventing disease relapse, and limiting overall accrual of both disease-related damage and treatment-related morbidity. Future research directions include investigation of the optimal duration and frequency of maintenance therapy as well as development of targeted therapeutic agents, which is enhanced by emerging insights into disease pathogenesis. Expected final online publication date for the Annual Review of Medicine Volume 66 is January 14, 2015. Please see http://www.annualreviews.org/catalog/pubdates.aspx for revised estimates. PMID:25341007

Lally, Lindsay; Spiera, Robert

2014-10-17

56

Vasculitis Syndromes of the Central and Peripheral Nervous Systems  

MedlinePLUS

... Strauss syndrome, Wegener’s granulomatosis, systemic lupus erythematosis, scleroderma, rheumatoid arthritis, Sjogren’s syndrome, and Behcet’s disease. Is there any treatment? Treatment for a vasculitis syndrome depends upon the ...

57

What Are the Signs and Symptoms of Vasculitis?  

MedlinePLUS

... Weight loss Fatigue (tiredness) General aches and pains Organ- or Body System-Specific Signs and Symptoms Vasculitis can affect specific organs and body systems, causing a range of signs and symptoms. Skin ...

58

Rituximab induces remission in refractory HCV associated cryoglobulinaemic vasculitis  

PubMed Central

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

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

2003-01-01

59

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

60

[Disease activity assessment in large vessel vasculitis].  

PubMed

Disease activity assessment in large vessel vasculitis (LVV) is often challenging for physicians. In this study, we compared the assessment of disease activity based on inflammatory markers, clinical indices (Indian Takayasu Activity Score [ITAS] and the Kerr/National Institute of Health indices [Kerr/NIH]), and 18F-Fluorodesossiglucose (FGD) vascular uptake at positron emission tomography (Pet). We found that Pet results did not statistically correlate with the clinical indices ITAS and Kerr/NIH, because FDG uptake was increased (grade>2 on a 0-3 scale in at least one evaluated vascular segment) in many patients with inactive disease according to clinical and laboratory parameters (i.e., negative ITAS and Kerr/NIH indices as well as normal erythrocyte sedimentation rate (ESR) and C-reactive protein (PCR)). Similarly, interleukin- 6 and its soluble receptor did not statistically correlate with disease activity. In contrast, clinical indices showed a significant correlation between each other and with inflammatory markers (VES and PCR). These data suggest that while clinical indices and inflammatory markers may be useful to assess disease activity, Pet may be more sensitive. PMID:21776444

Magnani, L; Versari, A; Salvo, D; Casali, M; Germanň, G; Meliconi, R; Pulsatelli, L; Formisano, D; Bajocchi, G; Pipitone, N; Boiardi, L; Salvarani, C

2011-01-01

61

Vessel wall enhancement in herpes simplex virus central nervous system vasculitis.  

PubMed

Infection is a well-known cause of cerebral vasculopathy and vasculitis. We report a 36-year-old woman with cerebral vasculitis and ischemic stroke secondary to herpes simplex virus (HSV). MRI studies revealed a pontine stroke with basilar artery stenosis and vessel wall gadolinium enhancement. This case demonstrates the ability of HSV to cause a focal brainstem vasculitis and the utility of enhanced MRI in the diagnosis of stroke related to HSV central nervous system vasculitis. PMID:23517674

Guerrero, Waldo R; Dababneh, Haitham; Hedna, Shushrutha; Johnson, James A; Peters, Keith; Waters, Michael F

2013-09-01

62

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

63

Endoscopic and radiographic features of gastrointestinal involvement in vasculitis  

PubMed Central

Vasculitis is an inflammation of vessel walls, followed by alteration of the blood flow and damage to the dependent organ. Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract. The variety of GI lesions includes ulcer, submucosal edema, hemorrhage, paralytic ileus, mesenteric ischemia, bowel obstruction, and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typical cases, including Churg-Strauss syndrome, Henoch-Schönlein purpura, systemic lupus erythematosus, and Behçet’s disease. Important endoscopic features are ischemic enterocolitis and ulcer. Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign. Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy. PMID:22442741

Hokama, Akira; Kishimoto, Kazuto; Ihama, Yasushi; Kobashigawa, Chiharu; Nakamoto, Manabu; Hirata, Tetsuo; Kinjo, Nagisa; Higa, Futoshi; Tateyama, Masao; Kinjo, Fukunori; Iseki, Kunitoshi; Kato, Seiya; Fujita, Jiro

2012-01-01

64

Antineutrophil cytoplasmic antibody-associated vasculitis with oculomotor nerve palsy.  

PubMed

We report a patient with antineutrophil cytoplasmic antibody-associated vasculitis with oculomotor nerve palsy. The patient presented with a high fever, diplopia, blepharoptosis and impairment of ocular movement of the left eye except for lateral gaze. Multiple erythematous and livedoid lesions were observed on the forehead, both cheeks and both legs. Laboratory examination showed positive results for myeloperoxidase antineutrophil cytoplasmic antibodies. Skin biopsy revealed leucocytoclastic vasculitis of the small arteries in the lower dermis. The patient was successfully treated with systemic corticosteroids. PMID:19187297

Seishima, M; Mizutani, Y; Shibuya, Y; Arakawa, C

2009-03-01

65

Vasculitis: do we know more to classify better?  

PubMed

The systemic vasculitides are a heterogeneous group of disorders characterized by the inflammation of blood vessels. The development and implementation of advanced diagnostic tests and genetic studies have resulted in substantial improvement in our understanding of vasculitis pathogenesis, resulting in the revision of the nomenclature and classification for vasculitis. Multicenter, collaborative studies are currently underway to develop improved diagnostic criteria. In this review, the major nomenclature and classification systems for vasculitides are summarized, with special emphasis on those emerging from the recent 2012 Chapel Hill Consensus Conference (CHCC). PMID:25480731

Batu, Ezgi Deniz; Ozen, Seza

2014-12-01

66

Leukocytoclastic Vasculitis as an Onset Symptom of Crohn's Disease  

PubMed Central

We report the case of an octagenerian who presented with leukocytoclastic vasculitis as the first symptom of Crohn's disease. The patient was admitted with skin rash on the lower extremities and ankles and episodes of bloody diarrhea. Skin and colon biopsies revealed acute leukocytoclastic vasculitis and moderate Crohn's disease, respectively. The patient was treated with intravenous corticosteroids in conjunction with antibiotics and per os mesalazine. Symptoms resolved rapidly within 5 days, and the patient was still asymptomatic on follow-up 3 months later. PMID:21897791

Tsiamoulos, Z.; Karamanolis, G.; Polymeros, D.; Triantafyllou, K.; Oikonomopoulos, T.

2008-01-01

67

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

PubMed Central

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

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

2001-01-01

68

Vasculitis Syndromes of the Central and Peripheral Nervous Systems  

MedlinePLUS

... how are they treated? The diagnosis of a CNS or PNS vasculitis disorder will depend upon the number of blood vessels ... diabetes, cataracts, and infection. Primary angiitis of the CNS ... rare disorder develop slowly. Symptoms include headache and encephalopathy-like ...

69

Progress in treatment of ANCA-associated vasculitis  

PubMed Central

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

2012-01-01

70

Diagnostic strategy for the assessment of rheumatoid vasculitis  

PubMed Central

Objective: To determine the clinical features associated with histologically proven rheumatoid vasculitis (HRV) and the additional diagnostic value of serological markers in an inception cohort of 81 patients with rheumatoid arthritis (RA) suspected of RV. Methods: The presence and number of recently developed extra-articular manifestations (EAMs) and a weighted EAM score, as well as the levels of serological markers, were compared between 31 patients with RA with histologically proven vasculitis and 50 patients with RA in whom vasculitis could not be documented histologically. The following markers were evaluated: circulating immune complexes, complement components C3 and C4, class-specific rheumatoid factors (IgM RF, IgG RF, IgA RF), antineutrophil cytoplasmic antibodies, antinuclear antibodies, antiendothelial antibodies, circulating intercellular adhesion molecule-1 and -3, circulating vascular cell adhesion molecule and E-selectin, cellular fibronectin, von Willebrand factor antigen, and C reactive protein. The diagnostic value of these markers, in addition to the clinical features, was evaluated with logistic regression analysis. Results: Peripheral neuropathy or purpura/petechiae, or both, were the most important clinical features to discriminate patients with RA with and without histologically proven RV. The presence of a high number of EAMs and a higher weighted EAM score in patients with RA suspected of vasculitis were also associated with an increased probability of histologically proven RV. After adjustment for EAMs, only the combination of an increased serum IgA RF level and a decreased serum C3 level appeared to make an additional contribution to the diagnosis histologically proven RV. Evidence of systemic vasculitis was found in a muscle biopsy of the rectus femoris in 9/14 (64%) patients with vasculitis with neuropathy and in 3/11 (27%) patients with purpura/petechiae and vasculitis of the skin. Conclusions: In the diagnostic process of RV the presence of peripheral neuropathy and/or purpura/petechiae or a high weighted EAM score will increase the probability of histologically proven RV. Of the circulating factors previously suggested to be markers for RV only IgA RF and C3 further increase the probability of histologically proven RV and may be useful to guide diagnostic decisions. PMID:12695150

Voskuyl, A; Hazes, J; Zwinderman, A; Paleolog, E; van der Meer, F J M; Daha, M; Breedveld, F

2003-01-01

71

Assessment of damage in vasculitis: expert ratings of damage  

PubMed Central

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

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

2009-01-01

72

Single-organ gallbladder vasculitis: characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 61 patients.  

PubMed

Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18-94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p?=?0.003) and exhibited higher erythrocyte sedimentation rate levels (80?±?28 vs 37?±?25?mm/h, respectively; p?=?0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was higher than in GB-SOV (35.5% vs 10%; p?=?0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n?=?10), hepatitis B virus-associated vasculitis (n?=?8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n?=?6), vasculitis associated with autoimmune diseases (n?=?6), microscopic polyangiitis (n?=?4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n?=?4), IgA vasculitis (Henoch-Schönlein) (n?=?2), and giant cell arteritis (n?=?1).GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory. PMID:25500710

Hernández-Rodríguez, José; Tan, Carmela D; Rodríguez, E René; Hoffman, Gary S

2014-11-01

73

Sudden death due to isolated segmentary coronary vasculitis.  

PubMed

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

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

2014-12-01

74

Advances in the Diagnosis of Large Vessel Vasculitis.  

PubMed

The diagnosis of large-vessel vasculitis has experienced substantial improvement in recent years. While Takayasu arteritis diagnosis relies on imaging, the involvement of epicranial arteries by giant-cell arteritis facilitates histopathological confirmation. When appropriately performed temporal artery biopsy has high sensitivity and specificity. However, an optimal biopsy is not always achievable and, occasionally, the superficial temporal artery may not be involved. Imaging in its various modalities including colour-duplex ultrasonography, computed tomography angiography, magnetic resonance angiography and positron emission tomography, are emerging as important procedures for the diagnosis and assessment of disease extent in large-vessel vasculitis. Recent contributions to the better performance and interpretation of temporal artery biopsies as well as advances in imaging are the focus of the present review. PMID:25399944

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

2015-01-01

75

Clofazimine-induced enteropathy in treatment-resistant nodular vasculitis.  

PubMed

We report a chronic case of nodular vasculitis that responded to oral clofazimine 300 mg daily. The condition had previously responded to moderate dose oral prednisolone, 50 mg daily, but would recur with weaning. Multiple corticosteroid-sparing agents were trialled, however these were either ineffective or poorly tolerated. The introduction of clofazimine enabled prednisolone dose reduction, not achieved with other agents, to 22.5 mg daily, and was associated with complete suppression of disease activity. Unfortunately the patient developed a clofazimine-induced enteropathy and the treatment was ceased after almost 2 years of therapy. Cessation of clofazimine was associated with a flare of the condition. Clofazimine should be considered as a corticosteroid-sparing agent in resistant cases of nodular vasculitis. Clinicians should be aware of clofazimine-induced enteropathy as a potentially serious complication of the therapy. PMID:22571566

Kieu, Violet; Williams, Richard; Hill, Prue; Kelly, Rob

2012-05-01

76

Chronic vasculitis and polyneuropathy due to infection with Bartonella henselae.  

PubMed

Bartonella henselae, the causative agent of cat scratch disease and bacillary angiomatosis, is associated with an expanding spectrum of diseases. Here, we report on a 40-year-old patient suffering from chronic recurrent painful ulcers of the toes, distal axonal sensomotor polyneuropathy and Raynaud's phenomenon. Biopsy of the sural nerve demonstrated an axonal neuropathy with a neurogenic muscular atrophy. Treatment with high dose corticosteroids had no beneficial effect. A biopsy taken from a recurring ulcer 7 years after the beginning of the disease revealed superficial ulcerated hyperkeratosis with subepithelial proliferation of small vessels compatible with a diagnosis of verruca peruana, however, without detection of microorganism. Serologic analysis revealed an elevated IFT titer of 1:1,024 against B. henselae. Treatment with erythromycin induced healing of the ulcer, remission of the vasculitis and the polyneuropathy, and a decline of the IFT titer. This case illustrates that B. henselae infection should be considered in patients with vasculitis and polyneuropathic syndromes. PMID:17401716

Stockmeyer, B; Schoerner, C; Frangou, P; Moriabadi, T; Heuss, D; Harrer, T

2007-04-01

77

Long-term patient survival in ANCA-associated vasculitis  

Microsoft Academic Search

BackgroundWegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.ObjectiveTo describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at

Oliver Flossmann; Annelies Berden; Kirsten de Groot; Chris Hagen; Lorraine Harper; Caroline Heijl; Peter Höglund; David Jayne; Raashid Luqmani; Alfred Mahr; Chetan Mukhtyar; Charles Pusey; Niels Rasmussen; Coen Stegeman; Michael Walsh; Kerstin Westman

2011-01-01

78

[Livedoid vasculitis in a patient with antiphospholipid syndrome].  

PubMed

The authors present a clinical case of a 30 year old male patient admitted to the hospital for recurrent cyanosis and feet pain lasting for one year. In addition he presented bilateral purpuric lesions in the lateral maleolar region, one of which with ulceration. The finding of anticardiolipin antibodies associated with intraluminal thrombosis in the dermal vessels, allowed to conclude for Antiphospholipid syndrome. The cutaneous changes identified are named livedoid vasculitis. PMID:20711098

Serra, Sara; Saavedra, M Joăo; Salvador, M Joăo; Reis, J Pedro; Malcata, A

2010-01-01

79

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

80

Von willebrand factor antigen in assessment of vasculitis in patients with connective tissue diseases  

Microsoft Academic Search

Von Willebrand factor antigen (vWF:Ag) is synthesized and secreted by endothelial cells. In the present study we tried to assess the relationship between plasma level of vWF:Ag and vascular damage in patients with vasculitis. The study was carried out on 59 patients with connective tissue diseases. Vasculitis was diagnosed by biopsies of the skin. The patients with vasculitis had a

J. Kloczko; A. Kuryliszyn-Moskal; K. Bernacka; M. Bielawiec; B. Cylwik; P. Radziwon

1994-01-01

81

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

82

Three cases of primary small vessel vasculitis of the skeletal muscle–an own entity  

PubMed Central

Whereas systemic vasculitis is the most common form of vasculitis, vasculitis restricted to a single organ system is rare. Primary vasculitis restricted to striated skeletal muscle has been described in few case reports for polyarteritis nodosa and leucocytoclastic vasculitis, but not for small vessel vasculitis, type microscopic polyangiitis. The authors describe three patients with primary small vessel vasculitis of the skeletal muscle without evidence of other major organ involvement. All three patients presented with myalgias and highly elevated acute phase reactants while muscle weakness and elevated creatine kinase levels were not consistently present. Diagnoses were established by muscle biopsy and extensive search for potential causes of secondary vasculitis. Complete remission could be accomplished by steroids alone in only one case, while additional immunosuppressants were needed in the other two cases. Primary small vessel vasculitis of the skeletal muscle should be considered in patients presenting with myalgia and signs of systemic inflammation in the absence of other organ manifestations. Once diagnosed, aggressive systemic immunosuppression is appropriate. PMID:22674106

Benz, Nadja; Daikeler, Thomas; Frank, Stephan; Mehling, Matthias; Tyndall, Alan; Trendelenburg, Marten

2011-01-01

83

Review article: Progress of treatment in ANCA-associated vasculitis.  

PubMed

This study reports on innovations in the field of, and current approaches to, the therapy of ANCA-associated vasculitis (AAV). Randomized clinical trials and prospective open label trial of newer therapies performed in the last 15 years in Wegener's granulomatosis and microscopic polyangiitis or both (AAV) were reviewed. Although cyclophosphamide remains the favoured immunosuppressive for remission induction, the use of alternative immunosuppressives and of intravenous pulsed administration have reduced cyclophosphamide exposure and are likely to increase the safety of treatment. Mycophenolate mofetil, leflunomide and deoxyspergualin are newer immunosuppressive drugs which have been evaluated in AAV, while tumpur necrosis factor, alemtuumab and rituximab are 'biologic' agents that have received attention. There is insufficient study of the dosing of glucocorticoids. Plasma exchange is indicated for severe renal vasculitis. Outcomes for elderly patients presenting with severe renal impairment are often poor despite optimal therapy. The development of collaborative networks in Europe and the USA has facilitated the conduct of larger randomized controlled trials. These have led to consensus recommendations on how AAV should be treated. Many newer agents are currently under evaluation which have the potential to improve AAV outcomes in the future. PMID:19335843

Jayne, David

2009-02-01

84

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

85

Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis  

PubMed Central

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

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

2014-01-01

86

Leukocytoclastic vasculitis in a patient with ankylosing spondylitis.  

PubMed

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

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

2014-01-01

87

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

88

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

89

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

90

Florid urticarial vasculitis heralding a flare up of ulcerative colitis.  

PubMed

A 75-year-old man with ulcerative colitis (UC) and diet controlled diabetes mellitus presented with a 3-week history of slightly itchy, red plaques on both lower limbs ascending gradually to cover the trunk and arms. One week later, he developed a flare up of his UC. Routine blood tests showed modest drop in haemoglobin (122?g/L) and C reactive protein (85?mg/L). Serology was remarkable for high antiproteinase 3 (c-ANCA). Serum electrophoresis showed a mildly positive paraprotein band (? region). Stool culture was negative. Urine analysis showed proteinuria. Skin biopsy showed features of urticarial vasculitis (UV). He underwent a flexible sigmoidoscopy after the flare up showed mildly active UC. The patient was given hydrocortisone for 7?days and then prednisolone. Both rash and UC subsided. Electrophoresis was repeated 4?weeks later showing normal pattern. Prednisolone has been gradually reduced. Although rare, UV can be considered as one of the skin manifestations of UC. PMID:25535230

Boules, Evon; Lyon, Calum

2014-01-01

91

Immune and ribosome related genes were associated with systemic vasculitis.  

PubMed

This study aimed to investigate the molecular mechanism of systemic vasculitis via bioinformatics analysis. Gene express profile of E-GEOD-16945 (13 Takayasu arteritis samples and 13 control samples) was downloaded from EBI (European Bioinformatics Institute) database. Differentially expressed genes (DEGs) were screened between Takayasu arteritis and normal controls (|log FC| >1). Basic local alignment search tool (BLASTX) was used for the COG (Clusters of Orthologous Groups) classification of DEGs. Gene ontology analysis was performed for the DEGs (p<0.05). A gene expression network was built with DEGs. Mcode in Cytoscape software was used to extract modules from the network (degree?2, K-core?2 and adjusted p-value <0.05) followed by pathway analysis using GenMAPP (false discovery rate <0.05). A total of 747 DEGs were identified. There were 16 significant GO function terms enriched with DEGs, out of which immune and defense response was the most significant GO term. Totally, 3 modules were extracted from gene expression network, including one module constituted with up-regulated genes and two modules constituted with down-regulated genes. Furthermore, human leukocyte antigen (HLA)-DRB1, HLA-DPA1, HLA-DPB1, HLA-DOA and HLA-DRA in the down-regulated modules were significantly linked to immune related pathways (Intestinal immune network for IgA production and Systemic lupus erythematosus pathways), while ribosomal protein L 31 (RPL31), RPS3A and RPL9 in the up-regulated module were enriched in ribosome pathway. The immune related pathways, ribosome pathway, immune-related genes including (HLA-DRB1, HLA-DPA1, HLA-DPB1, HLA-DOA and HLA-DRA) and ribosome related genes (RPL31, RPS3A and RPL9) might be involved in systemic vasculitis. This article is protected by copyright. All rights reserved. PMID:25410188

Gan, Shujie; Ye, Bo; Qian, Shuixian; Zhang, Ci; Mao, Jieqi; Li, Ke; Tang, Jingdong

2014-11-20

92

Mesenteric vasculitis in a juvenile systemic lupus erythematosus patient.  

PubMed

Lupus mesenteric vasculitis (LMV) is a rare cause of acute abdominal pain. Few cases of LMV have been reported in adults, children and adolescents. However, to our knowledge, the prevalence of that severe vasculitis in a pediatric population with lupus is yet to be studied. This study reviewed data from 28 consecutive years and included 5,508 patients being followed up at the hospital of the Faculdade de Medicina of the Univesidade de Săo Paulo (FMUSP). We identified 279 (5.1%) patients meeting the American College of Rheumatology classification criteria for the diagnosis of systemic lupus erythematosus (SLE), one of whom (0.4%) had LMV. That male patient was diagnosed with SLE at the age of 11 years. At the age of 13 years, he was hospitalized with diffuse and acute abdominal pain, nausea, bilious vomiting, abdominal distension, rebound tenderness, and abdominal muscle guarding. The patient underwent laparotomy immediately, and segmentary intestinal ischemia with intestinal wall edema and adhesions were identified. Partial small bowel resection with lysis of the adhesions was performed, as were pulses of intravenous methylprednisolone. The histopathologic analysis evidenced mesenteric arteritis. After 13 days, the diffuse and intense abdominal pain recurred, and the patient underwent a new laparotomy, during which adhesive small bowel obstruction with intestinal gangrene was identified. New intestinal resection was performed, and the patient received pulses of intravenous methylprednisolone and infusion of immunoglobulin. Thus, LMV is a rare and severe abdominal manifestation of the pediatric population with lupus, and can be the only manifestation of disease activity. In addition, this study stresses the importance of the early diagnosis and immediate treatment. PMID:23856801

Albuquerque-Netto, Adăo F; Cavalcante, Erica G; Sallum, Adriana M E; Aikawa, Nádia E; Tannuri, Uenis; Silva, Clovis Artur Almeida da

2013-04-01

93

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

94

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

95

Hepatitis C-associated mixed cryoglobulinemic vasculitis induces differential gene expression in peripheral mononuclear cells.  

PubMed

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

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

2014-01-01

96

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

PubMed Central

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

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

2014-01-01

97

Isolated vasculitis of the female genital tract: a case series and review of literature  

Microsoft Academic Search

To provide a clinicopathologic review of vasculitis confined to the female genital tract, we describe three cases, and we\\u000a searched PubMed from 1965 to 2006 with analysis of all relevant articles. We identified 118 additional cases in the literature\\u000a of whom 108 had isolated necrotizing vasculitis similar to classical polyarteritis nodosa (PAN-type), and 10 presented isolated\\u000a giant cell arteritis (GCA-type)

Emmanuel Hoppé; Louis-Rémi de Ybarlucéa; Jacky Collet; Jérome Dupont; Bettina Fabiani; Xavier Puéchal

2007-01-01

98

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

PubMed Central

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

2014-01-01

99

Endothelial nitric oxide synthase gene polymorphisms in Behçet's disease and rheumatic diseases with vasculitis  

PubMed Central

Objective: To assess potential associations between Korean Behçet's disease (BD) or other rheumatic diseases with vasculitis and two polymorphisms of the endothelial nitric oxide synthase (eNOS) gene, which include the Glu298Asp polymorphism in exon 7 and a variable number of tandem repeats (VNTR) polymorphism in intron 4. Methods: 65 patients with BD, 27 with rheumatic diseases with vasculitis, and 80 controls were studied. Analyses of the Glu298Asp polymorphism in exon 7 and VNTR polymorphism in intron 4 of the eNOS gene were made by the polymerase chain reaction (PCR)-restriction fragment length polymorphism technique and PCR genotyping, respectively. Additionally, HLA-B51 typing was performed in the BD group and controls by a two step PCR sequence-specific primers method. Results: Significant differences in Glu298Asp genotype frequencies were found between the BD or vasculitis groups and the controls (BD group v controls: pcorr=0.006; vasculitis group v controls: p<0.001). The Asp298 frequency was much higher in the BD and vasculitis groups than in the controls. Even after stratification of the BD group based on the results of HLA-B51 testing, a significant association of the Glu298Asp polymorphism was still found (p=0.002, Mantel-Haenszel weighted odds ratio 4.3, 95% confidence interval 1.7 to 10.9). Distribution of the genotype frequencies in two eNOS gene polymorphisms was similar in connective tissue diseases-associated vasculitis and primary vasculitic syndromes. In contrast, distribution of alleles and genotypes of VNTR polymorphism did not differ between BD or vasculitis groups and the controls. Conclusion: The Glu298Asp polymorphism in exon 7 of the eNOS gene seems to be a susceptibility gene for Korean BD and other rheumatic diseases. PMID:14583572

Kim, J; Chang, H; Lee, S; Kim, J; Kim, K; Lee, S; Chung, W

2003-01-01

100

New Features of Disease after Diagnosis in Six Forms of Systemic Vasculitis  

PubMed Central

Objective To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu's arteritis (TAK), was performed within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed “severe” manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results Out of 838 patients with vasculitis, 490 (59%) experienced ? 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA - 1.9, MPA - 1.2, EGPA - 1.5, PAN - 1.2, GCA - 0.7, TAK - 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA - 26%, MPA - 19%, EGPA - 21%, PAN - 23%, GCA - 24%, and TAK - 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. PMID:23908447

Grayson, Peter C.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.

2015-01-01

101

Acute brucellosis associated with leukocytoclastic vasculitis and splenic infarct.  

PubMed

Brucellosis is globally the most prevalent multisystem infection of zoonotic origin, while it is still one of the most important public health problems in Turkey as non-pasteurised milk and dairy products are consumed. Early diagnosis is vital to prevent the possibly lethal complications caused by the disease. However, diagnosis might be delayed as the disease does not have a single and typical manifestation and presents with various symptoms of different systems. Brucellosis and associated splenic infarct have rarely been studied, there being few cases in the literature. One of the rare involvements in this disease is dermatological involvement, which has been found in less than 10 percent of brucellosis cases. In this study, we discuss a 17 year old male patient who was admitted to our hospital due to fever, abdominal pain, arthralgia and rash on legs, diagnosed with brucellosis through brucellosis tube agglutination test and found to have splenic infarct upon examination and leukocytoclastic vasculitis according to the skin biopsies in the light of the present literature. PMID:25551851

Ucmak, Feyzullah; Ucmak, Derya; Bestas, Remzi; Anli, Ruken Azizoglu; Adanir, Haydar

2014-12-01

102

Tocilizumab improves systemic rheumatoid vasculitis with necrotizing crescentic glomerulonephritis.  

PubMed

Abstract We report a Japanese woman with systemic rheumatoid vasculitis (SRV) complicated by necrotizing crescentic glomerulonephritis (NCGN). Rheumatoid arthritis first occurred at the age of 19 years, followed by interstitial pneumonia, hepatitis, rheumatoid nodules, mononeuritis multiplex, and hypocomplementemia in chronological order. At the age of 51 years, rapidly progressive renal failure occurred with nephrotic proteinuria, and NCGN with subepithelial deposits was revealed by renal biopsy. Severe destructive changes of multiple joints and scleritis were detected, but anti-neutrophil cytoplasmic antibody was negative on enzyme-linked immunosorbent assays and indirect immunofluorescence. SRV was diagnosed due to involvement of multiple extra-articular organs. An anti-interleukin (IL)-6 receptor antibody (tocilizumab) was started at dosage of 280 mg (8 mg/kg) monthly. After 18 months, her serum creatinine decreased from 1.7 to 1.3 mg/dL, and urinary protein excretion declined from 5.2 to 1.2 g daily. Tocilizumab may be a therapeutic option for SRV associated with NCGN. PMID:24533557

Iijima, Takashi; Suwabe, Tatsuya; Sumida, Keiichi; Hayami, Noriko; Hiramatsu, Rikako; Hasegawa, Eiko; Yamanouchi, Masayuki; Hoshino, Junichi; Sawa, Naoki; Takaichi, Kenmei; Oohashi, Kenichi; Fujii, Takeshi; Ubara, Yoshifumi

2015-01-01

103

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

104

Clinical features and etiology of retinal vasculitis in Northern Thailand  

PubMed Central

Purpose: To report on the clinical features and etiology of patients with retinal vasculitis (RV). Materials and Methods: We reviewed medical records of 47 patients (75 affected eyes) diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered. Results: Etiology of RV included infectious causes in 10/47, (21%) while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%). Eales’ disease and Behcet's disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60%) among those with infectious disorders. RV was bilateral in 28/47 (60%) patients. Retinal veins were most commonly affected (72%, 34/47). Involvement of arteries was present in 12/47 (25%) and was associated with viral infections and Behcet's disease. Ocular complications developed in 60/75 (80%) eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%). Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%). Conclusions: RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet's disease. Tuberculosis was the most common infectious cause. PMID:24178403

Apinyawasisuk, Supanut; Rothova, Aniki; Kunavisarut, Paradee; Pathanapitoon, Kessara

2013-01-01

105

Propylthiouracil-induced lupus-like or vasculitis syndrome.  

PubMed

A 27?year old female with Graves' disease presented with fever, exertional dyspnea and polyarthralgia. Erythema nodosum had occured three months earlier. The patient declared irregular use of propylthiouracil (PTU) for the last 8?months. Neutropenia and microscopic hematuria developed in the second week of admission. Chest X-ray showed inhomogenous pulmonary opacities, left pleural effusion and cardiomegaly. Computed tomography (CT) revealed multiple subpleural nodules, left pleural effusion, pericardial effusion, enlarged mediastinal and axillary lymph nodes. Bronchoalveolar lavage (BAL) cytology demonstrated hemosiderin laden macrophages. Histopathologic examination of the transbronchial biopsy specimen revealed a nonspecific inflammation. Serum was positive for ANA, P-ANCA, MPO-ANCA, PR3-ANCA and negative for anti-ds-DNA, C-ANCA, C3, C4 and anti-histone antibody. All symptoms resolved in two months after PTU withdrawal and starting steroid treatment. The same clinical manifestations recurred when the patient used PTU erronously one month after discharge.This is a case of PTU induced-autoimmune disease in whom the accurate distinction between drug-induced-lupus (DIL) and vasculitis was not possible due to the significant overlap of clinical and laboratory findings causing a significant diagnostic challenge for the chest physician. PMID:22958435

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

2012-01-01

106

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

PubMed Central

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

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

2014-01-01

107

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

PubMed

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

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

2014-01-01

108

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

109

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

110

Urticarial vasculitis with papular lesions in a patient with type C hepatitis and cryoglobulinemia.  

PubMed

We describe a case of urticarial vasculitis accompanied by erythematous wheals, palpable pupura, and subsequent necrotic ulcerated papular lesions in a patient with type C chronic hepatitis and type II cryoglobulinemia (IgM-kappa and polyclonal IgG). A 56-year-old man developed recurrent urticarial lesions on his lower extremities and trunk. The histology revealed leukocytoclastic vasculitis with perivascular immunoglobulin deposits. Subsequently, multiple reddish papular lesions with necrotic ulcerations appeared on the extensor aspect of his extremities and buttocks. Histology of these lesions showed cryoglobulinemic vasculitis with prominent fibrinoid necrosis of the vascular walls and cryoprecipitate within the vasculature as well as increased hyalinized collagen bundles. These papular lesions have not previously been described as cutaneous necrotizing venulitis to the best of our knowledge. It is suggested that the immune response to hepatitis C virus infection and cryoglobulins may be responsible for severe necrotizing venulitis, resulting in unusual cutaneous lesions. PMID:8935345

Kuniyuki, S; Katoh, H

1996-04-01

111

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

NASA Astrophysics Data System (ADS)

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

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

1992-06-01

112

Central Retinal Vein Occlusion in a Patient with Retinal Vasculitis and Crohn's Disease  

PubMed Central

The authors report a rare case of a 47-year-old woman with Crohn's disease (CD) who presented with retinal vasculitis and central retinal vein occlusion (CRVO) during remission. The patient complained of sudden painless visual loss in her left eye (OS). Ophthalmologic evaluation revealed a best corrected visual acuity (BCVA) of 20/20 in the right eye and hand movements in OS. Ophthalmoscopy and fluorescein angiography of OS showed signs of nonischemic CRVO and extensive vasculitis. She was treated with oral prednisolone, mercaptopurine, and intravitreal bevacizumab in OS. After 1 month of treatment, VA of OS improved to 5/10 and after 1 year it was 10/10 with complete resolution of retinal vasculitis and nonischemic CRVO. PMID:25506451

Figueiredo, Lígia; Rothwell, Renata; Brandăo, Arnaldo

2014-01-01

113

Finger necrosis due to cryoglobulinemic vasculitis in association with membranous nephropathy  

PubMed Central

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

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

2015-01-01

114

Delayed diagnosis of ocular syphilis that manifested as retinal vasculitis and acute posterior multifocal placoid epitheliopathy  

PubMed Central

A 55-year-old female presented with bilateral progressive retinal vasculitis. She was on systemic and intravitreal steroids on the basis of uveitis work-up result (negative result including rapid plasma reagin), but her visual acuity continued to deteriorate to light perception only. Ocular examination showed retinal vasculitis, multiple yellow placoid lesions and severe macula edema in both eyes. Repeated work-up revealed positivity of fluorescent treponemal antibody-absorption in serum and subsequently in cerebrospinal fluid. Ocular syphilis was diagnosed. And intravenous penicillin G resulted in rapid resolution of vasculitis and macular edema. To avoid delay in the diagnosis of ocular syphilis, high index of suspicion and repeating serological tests (including both treponemal and non-treponemal tests) are warranted. PMID:24145569

Park, Jong Hoon; Joe, Soo Geun; Yoon, Young Hee

2013-01-01

115

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

116

An approach to the diagnosis and management of systemic vasculitis revised version with tracked changes removed  

PubMed Central

The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses. PMID:20070316

Miller, A; Chan, M; Wiik, A; Misbah, S A; Luqmani, R A

2010-01-01

117

Cryoimmunoglobulinemia in rheumatoid arthritis. Significance in serum of patients with rheumatoid vasculitis.  

PubMed Central

Cryogloculins were examined in a standardized manner in an unselected group of 35 patients with rheumatoid arthritis (RA) and 8 patients with RA complicated by cutaneous vasuclitis and neuropathy. Optimum conditions for detection and characterization of cryoglobulins were established; the proportion of resolubilized to total precipitable protein remained constant in an individual patient under these conditions. All 8 vascultis patients and 9 of 35 other patients with RA exhibited cryoglobulins; total protein and immunoglobin content were significantly higher in the cryoglobulins of patients with vasculitis. Immunoglobulins G and M constituted two-thirds and three-quarters of the total protein in the cryoglobulins from uncomplicated rheumatoid and vasculitis patients, respectively. Serum antiglobulin titers were higher, and serum C3 levels were lower, in vasculitis patients compared to rheumatoid patients without vasclitis. Anti-gamma globulin activity was detected in all cryoglobulins from vasculitis patients. Cryoglobulin IgG and IgM were polyclonal. Density gradient analyses demonstrated the majority of the cryoglobulin activity to reside in the 19S IgM fraction. There was no evidence of a light weight (8S) IgM. A monoclonal rheumatoid factor did not detect 7S-ANTI-7S complexes in the cryoprecipitates, but acid eluates from some cryoglobulins absorbed with insoluble IgG revealed an antiglobulin of the IgG class. Serial studies performed on vasculitis patients treated with cyclophosphamide disclosed a relationship between clinical evidence of vasculitis and the presence of cryoglobulins. The antigen (IgG) and antibody (largely IgM rheumatoid factor) nature of these cryglobulins is presented as evidence that the widespread vascular complications of RA are mediated, at least in part, by circulating immune complexes. PMID:169295

Weisman, M; Zvaifler, N

1975-01-01

118

Retinal arteriolar spasm during transient monocular visual loss in eosinophilic vasculitis.  

PubMed

A patient with eosinophilic vasculitis and acquired immunodeficiency syndrome (AIDS) developed episodic transient monocular visual loss. During or immediately after two visual loss episodes, we demonstrated narrowed retinal arterioles, delayed arterial filling time, and segmented retinal venous flow in the affected eye on fundus photography and fluorescein angiography (FA). Such findings have only rarely been reported in patients with transient monocular visual loss in other conditions, probably because the episodes have ended before fundus photography and FA could be performed. This is the first report to capture retinal vascular changes associated with transient monocular visual loss in a patient with eosinophilic vasculitis. PMID:19458579

Stasi, Kalliopi; Ramchandran, Rajeev S; Rao, Narsing A; Feldon, Steven E; DiLoreto, David A

2009-03-01

119

Bilateral retinal vasculitis in a patient with systemic lupus erythematosus and its remission with rituximab therapy.  

PubMed

Severe retinal vasculitis is a rare, but potentially blinding, complication of patients with systemic lupus erythematosus (SLE). We describe here the first reported case of treating severe bilateral SLE-associated retinal vasculitis with the anti-CD20 monoclonal antibody rituximab, a drug which has established its role in rheumatoid arthritis and has shown promise in case series for the treatment of severe SLE that is unresponsive to other therapies. This case suggests that rituximab-induced B-cell depletion may provide an important new therapeutic option for refractory cases of this devastating ocular complication. PMID:19900982

Hickman, R A; Denniston, A K; Yee, C-S; Toescu, V; Murray, P I; Gordon, C

2010-03-01

120

Value of anti-infective chemoprophylaxis in primary systemic vasculitis: what is the evidence?  

PubMed Central

Although infections are a major concern in patients with primary systemic vasculitis, actual knowledge about risk factors and evidence concerning the use of anti-infective prophylaxis from clinical trials are scarce. The use of high dose glucocorticoids and cyclophosphamide pose a definite risk for infections. Bacterial infections are among the most frequent causes of death, with Staphylococcus aureus being the most common isolate. Concerning viral infections, cytomegalovirus and varicella-zoster virus reactivation represent the most frequent complications. The only prophylactic measure that is widely accepted is trimethoprim/sulfamethoxazole to avoid Pneumocystis jiroveci pneumonia in small vessel vasculitis patients with generalised disease receiving therapy for induction of remission. PMID:19886977

2009-01-01

121

Ischemic retinal vasculitis in an 18-year-old man with chickenpox infection  

PubMed Central

Ocular involvement after primary infection with varicella zoster virus is very rare. We report a case of a healthy 18-year-old man who presented with unilateral ischemic retinal vasculitis 10 days after the onset of chickenpox. He developed acute severe visual loss and a relative afferent pupillary defect in his right eye. Fundus imaging, optical coherence tomography, fundus fluorescence angiography, and electrophysiologic studies confirmed the diagnosis of retinal vasculitis, which led to generalized retinal ischemia. Although aggressive treatment with systemic steroids and antiviral drugs was administered, a poor visual outcome still resulted. PMID:24591814

Poonyathalang, Anuchit; Sukavatcharin, Somsiri; Sujirakul, Tharikarn

2014-01-01

122

Increased hydroxyl radical generation by normal polymorphonuclear leukocytes incubated in sera from patients with leukocytoclastic vasculitis  

Microsoft Academic Search

The effect of sera from patients with untreated leukocytoclastic vasculitis was investigated on the generation of oxygen intermediates by normal polymorphonuclear leukocytes. Sera from untreated patients induced increased hydroxyl radical generation, which is one of the most potent oxidants capable of causing tissue damage. It is suggested that vascular injury may be mediated in part by enhanced production of hydroxyl

Yoshiki Miyachi; Keiko Yanase; Sadao Imamura; Yukie Niwa

1982-01-01

123

Recurrent cutaneous necrotizing vasculitis in a patient with Kikuchi's disease: case report.  

PubMed

Kikuchi's disease or histiocytic necrotizing lymphadenitis without granulocytic infilteration, is a clinico-pathologic entity well described in developed countries. We report a case of this disorder presenting with recurrent cutaneous necrotizing vasculitis, with the dual objective of highlighting this condition in developing countries and presenting an uncommon manifestation of this disorder. PMID:9557453

Belagavi, C S; Kubeyinje, E P

1997-11-01

124

Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy  

PubMed Central

Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor. PMID:22919534

Murray, Nigel P.; Ruíz, Amparo; Reyes, Eduardo

2012-01-01

125

Immune-mediated vasculitis in a shar-pei with swollen hock syndrome.  

PubMed Central

A castrated male shar-pei was presented for episodes of lethargy, swelling of the tarsal joints, and polydipsia with polyuria. Histological examination of biopsies from skin overlying the tarsi and direct immunoperoxidase immunohistochemical staining confirmed immune complex vasculitis, suggesting a role for immune complex deposition in the pathogenesis of shar-pei fever. PMID:11272460

Tellier, L A

2001-01-01

126

Double ANCA-positive vasculitis in a patient with infective endocarditis  

PubMed Central

The most common pattern of renal involvement in infective endocarditis is infection-associated glomerulonephritis. Due to clinical symptoms and signs that overlap with vasculitis, the diagnosis of infective endocarditis may be delayed. The unusual combination of reduced complement with positive antineutrophil cytoplasmic antibody should raise the suspicion of infections such as infective endocarditis. PMID:23439873

Veerappan, I.; Prabitha, E. N.; Abraham, A.; Theodore, S.; Abraham, G.

2012-01-01

127

eNOS inhibits the development of autoimmune-mediated vasculitis  

PubMed Central

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

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

2012-01-01

128

Leukocytoclastic vasculitis presenting as an erythema gyratum repens--like eruption on a patient with systemic lupus erythematosus.  

PubMed

Cutaneous leukocytoclastic vasculitis is a common finding among patients with systemic lupus erythematosus, although the clinical appearance of the lesions varies. We report the case of a 38-year-old woman with systemic lupus erythematosus who had leukocytoclastic vasculitis with peculiar clinical morphologic features of the cutaneous lesions. They consisted of figured erythema closely resembling erythema gyratum repens. We discuss the differential diagnosis with other annular eruptions. PMID:12399742

Piqué, Enric; Palacios, Santiago; Santana, Zaraida

2002-11-01

129

Cutaneous allergic vasculitis due to Solenopsis geminata (Hymenoptera: Formicidae) envenomation in Indonesia.  

PubMed

Severe cutaneous allergic vasculitis in a 60 year-old Caucasian male following the bite of the tropical fire ant, Solenopsis geminata (F.) is reported. Over the course of 8 weeks, the pathology progressed from an extensive red papular urticaria to vasculitis with peri-vascular inflammation and ulceration of the skin on the feet, ankles and lower limbs. Many of the affected areas of the skin eventually became covered with black eschar associated with further tissue breakdown and ulcer formation. After debridement, compression dressings, antimicrobial ointment and corticosteroids, complete healing eventually took place with only residual scarring. An awareness of the severe dermatologic reactions caused by a bite of S. geminata, albeit rare, is clinically important. Recognizing the characteristic skin lesions caused by the bite of S. geminata, treated with prompt administration of appropriate chemotherapy will speed recuperation of the patient and reduce possible secondary complications. PMID:18041296

Knight, David; Bangs, Michael J

2007-09-01

130

Evidence for the role of platelet-activating factor in immune complex vasculitis in the rat.  

PubMed Central

These studies were designed to determine the role of platelet-activating factor (PAF) in the pathogenesis of immune complex (IgG) induced dermal vasculitis in the rat. In vitro, very low (pM and nM) concentrations of PAF "primed" rat neutrophils for enhanced O2-. responses to IgG immune complexes while higher concentrations were directly stimulatory. The PAF receptor antagonist, L-652,731, blocked responses (O2-. production and enzyme release) of rat neutrophils stimulated with PAF but did not block responses triggered by immune complexes, formyl chemotactic peptide or opsonized zymosan particles. When L-652,731 was added to the antibody employed in the reversed passive Arthus reaction, the injury resulting from immune complex-induced vasculitis was significantly attenuated. In order to determine if in vivo protection provided by L-652,731 was related to neutrophils, we developed a new model in which rats are systemically depleted of neutrophils by cyclophosphamide and then locally reconstituted with intact neutrophils in a manner that allows restoration of immune complex-induced vascular injury. With this model, we demonstrated that the effects of neutrophil reconstitution are substantially diminished if the cells are pretreated with L-652,731 and then washed. By priming neutrophils with substimulatory concentrations of PAF, we have also provided in vivo evidence that neutrophil priming can increase the magnitude of vascular injury. These data provide evidence that vascular injury associated with immune complex dermal vasculitis is related to availability of PAF receptors on neutrophils, suggesting a mechanism through which PAF may function as a mediator in the pathogenesis of immune complex vasculitis. Images PMID:2536403

Warren, J S; Mandel, D M; Johnson, K J; Ward, P A

1989-01-01

131

CAF-elektrophoretische und immunelektrophoretische Untersuchungen bei Patienten mit Vasculitis allergica Ruiter  

Microsoft Academic Search

CAF-electrophoretic investigations were performed on 33 patients with allergic vasculitis. Compared with a control group of healthy individuals highly significant increases of alpha-1-, alpha-2- und gamma-globulins were found, whereas increases of beta-globulins were just significant. 20 immunelectrophoretic investigations showed the same acute inflammatory pattern of serum-protein-changes. Beta-1-C-globulin was normal in 14 patients and increased in 6 patients.

P. Wang; N. Hofmann; O. P. Hornstein

1973-01-01

132

Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants  

PubMed Central

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

2011-01-01

133

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

PubMed Central

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

2014-01-01

134

Isoniazid Induced Cutaneous Leukocytoclastic Vasculitis in Extra Pulmonary Tuberculosis (Pott’s Spine): A Case Report  

PubMed Central

Anti-tuberculosis drugs frequently result in cutaneous adverse reactions but Isoniazid is known to have least toxic potential for cutaneous reactions. We report a rare case of Isoniazid induced cutaneous leucocytoclastic vasculitis. A 64-year-old male was diagnosed to have Pott’s spine with multiple vertebral body involvement (D8-12 vertebrae). Subsequently, he was treated with first line anti-TB drugs i.e., Isoniazid, Rifampicin, Pyrazinamide and Ethambutol. On the fourth day of treatment with Anti Tuberculosis Treatment (ATT), the patient developed an erythematosus rash over right upper limb not associated with itching or pain, non-blanchable macules and papules over bilateral shins on lower limbs, petechiae on both forearms and hyper pigmented, scaly rash over right axilla and buttocks. The skin biopsy report was consistent with cutaneous leukocytoclastic vasculitis. Although rare, Isoniazid among anti-tuberculosis drugs should be considered as potential cause of drug-induced cutaneous leukocytoclastic vasculitis in the differential diagnosis of erythematosus rash with petechiae. PMID:25302231

V, Dharma Rao; Rampure, Dilip; S, Rama Rao

2014-01-01

135

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

136

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

137

Recurrent ALK-negative anaplastic large T-cell lymphoma presenting as necrotizing vasculitis.  

PubMed

Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma histologically characterized by expression of CD30, a cell surface receptor present on activated T cells and B cells. ALCL may occur in a primary cutaneous form or as systemic ALCL with lymph node involvement. Anaplastic lymphoma kinase (ALK) is a tyrosine kinase that induces neoplastic transformation as a result of translocational fusion with an activating promoter. The presence of ALK can be used to distinguish between primary cutaneous ALCL and systemic nodal ALCL in certain cases. Primary cutaneous and systemic ALCL metastatic to the skin are histologically indistinguishable. "Leukemic vasculitis"--an uncommon finding in cases of cutaneous leukemia and even more exceptional in cutaneous lymphoma--refers to a pattern of vasculitis occurring as a direct result of infiltrating neoplastic cells. We report a fatal case of recurrent ALK-negative ALCL presenting as ulcerating skin lesions in a patient previously treated with the new anti-CD30 agent brentuximab vedotin. Biopsy revealed a necrotizing vasculitis resulting from the infiltration of neoplastic cells reminiscent of the patient's primary malignancy. We review the clinical and pathological findings of ALCL and present this case to highlight a subtle diagnostic clue in assessing recurrence of cutaneous lymphoma. PMID:23291583

Nambudiri, Vinod E; Aboutalebi, Amir; Granter, Scott R; Saavedra, Arturo

2013-06-01

138

Increased serum levels of soluble vascular endothelial-cadherin in patients with systemic vasculitis.  

PubMed

Henoch-Schönlein purpura (HSP) is a commonest systemic vasculitis (SV) in childhood characterized by an inflammatory reaction directed at vessels. Endothelial damage and perivascular leukocyte infiltrates are vital in the development of HSP. Vascular endothelial (VE)-cadherin is an endothelial cell-specific adhesion molecule, which plays critical roles in angiogenesis and endothelial integrity. Herein, we investigated the serum levels of soluble VE-cadherin (sVE-cadherin) in patients with HSP and other forms of SV. The serum levels of sVE-cadherin in 30 patients with HSP, together with patients with urticarial vasculitis, allergic vasculitis, Behcet disease, psoriasis vulgaris (PV) and atopic dermatitis (AD) and 26 health controls were measured by enzyme-linked immunosorbent assay. Serum levels of sVE-cadherin were significantly increased in patients with HSP in acute stage and patients with other forms of SV but not in patients with PV or AD. Moreover, Serum sVE-cadherin levels in HSP patients were correlated with the severity of this disease and serum concentrations of IgA anticardiolipin antibodies and vascular endothelial growth factor. Taken together, we show firstly that serum sVE-cadherin is abnormally increased in HSP patients. Increased serum levels of sVE-cadherin might be a novel biomarker for evaluating the severity of HSP and useful for identifying the presence of SV in inflammatory skin conditions. PMID:24469639

Chen, Tao; Guo, Zai-Pei; Cao, Na; Qin, Sha; Li, Meng-Meng; Jia, Rui-Zhen

2014-08-01

139

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

140

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

141

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

142

Transduction of a foreign histocompatibility gene into the arterial wall induces vasculitis.  

PubMed Central

Autoimmune vasculitis represents a disease characterized by focal inflammation within arteries at multiple sites in the vasculature. Therapeutic interventions in this disease are empirical and often unsuccessful, and the mechanisms of immune injury are not well-defined. The direct transfer of recombinant genes and their expression in the arterial wall provides an opportunity to explore the pathogenesis and treatment of vascular disease. In this report, an animal model for vasculitis has been developed. Inflammation has been elicited by direct gene transfer of a foreign class I major histocompatibility complex gene, HLA-B7, to specific sites in porcine arteries. Transfer and expression of this recombinant gene was confirmed by a polymerase chain reaction and immunohistochemistry, and cytolytic T cells specific for HLA-B7 were detected. These findings demonstrate that expression of a recombinant gene in the vessel wall can induce a focal immune response and suggest that vessel damage induced by cell-mediated immune injury can initiate vasculitis. Images PMID:1594626

Nabel, E G; Plautz, G; Nabel, G J

1992-01-01

143

Key advances in the clinical approach to ANCA-associated vasculitis.  

PubMed

The updated nomenclature for vasculitis defines this varied group of disorders by aetiology, specific features of pathogenesis and clinical symptoms; diagnostic and classification criteria for clinical practice are in development. Here, I review some important advances in the management of vasculitis within the category of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which encompasses microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The clinical approach to the management of the patient with AAV should include testing for ANCA specificity; proteinase 3 (PR3)-specific ANCAs are most often associated with GPA, whereas myeloperoxidase (MPO)-ANCAs are usually associated with MPA. Also important to the management of AAV is an assessment of the disease stage and severity, to enable tailored treatment based on an algorithm derived from controlled-trial data. Remaining questions pertain to the dosage and duration of corticosteroid treatment, the selection of patients for, and duration of, maintenance treatment after induction of remission, and the identification of safer and more effective therapies than are currently in use. Outcome measures should assess not only disease activity, but also damage and quality of life. Infections, cardiovascular events and malignancies also contribute to outcome, and their prevention should therefore be part of the clinical approach to managing patients with AAV. PMID:24980139

Kallenberg, Cees G M

2014-08-01

144

Correlation of blood levels of soluble vascular cell adhesion molecule-1 with disease activity in systemic lupus erythematosus and vasculitis.  

PubMed

Levels of soluble adhesion molecules have been shown to reflect their cell surface expression in vitro, and thus may provide a useful surrogate marker of surface expression at inflammatory sites. In patients with SLE and vasculitis, serum levels of intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1) and E-Selection were determined by ELISA during different stages of disease activity. Levels of soluble(s) VCAM-1 correlated with disease activity in patients with SLE, being significantly higher during active compared with inactive disease (P = 0.003), and normalizing with clinical remission. By contrast, in patients with vasculitis, although sVCAM-1 levels were elevated in active disease, they fell but did not normalize in inactive disease, suggesting that treatment may be suppressing the clinical manifestations rather than targeting the underlying pathogenic mechanism. Soluble ICAM-1 and E-Selectin levels did not relfect disease activity in either SLE or vasculitis. PMID:7528085

Janssen, B A; Luqmani, R A; Gordon, C; Hemingway, I H; Bacon, P A; Gearing, A J; Emery, P

1994-12-01

145

[Autopsy case of PR3-ANCA-associated vasculitis complicated with rectus muscle hematoma].  

PubMed

A 80-year-old man was admitted to our hospital because of coughing, hemosputum and dyspnea. As a chest X-ray showed infiltrates of the right lung, he was diagnosed as bacterial pneumonia and treated with antibiotics. However, after a few days, he exhibited hemoptysis and developed severe dyspnea, while laboratory findings showed rapid elevation of the serum creatinine level (5.55 mg dL). Computed tomography (CT) revealed large areas of ground glass opacity in the right lung, hence the hemoptysis was considered to be due to alveolar hemorrhage. As he had been diagnosed as chronic renal failure a few years before this admission and we also noticed that interstitial pneumonia with a slightly elevated level of C-reactive protein had existed from that time, ANCA-associated vasculitis was suspected to be the underlying pathogenesis. Accordingly, he was started on methylprednisolone pulse therapy and temporary hemodialysis resulted in improvement of dyspnea and renal function. PR3-ANCA was 12.4 EU, so he was diagnosed as PR3-ANCA-associated vasculitis. After a few days, he suddenly complained of abdominal pain, developing hypotension and anemia. Abdominal CT showed an irregular low-density mass in the right muscle, so he was diagnosed as rectus muscle hematoma. Surgery was performed and a massive hematoma was found in the rectus muscle without any ruptures of macroscopic vessels in the abdomen. Bleeding could not be stopped followed by multiple organ failure and the patient died four days postoperatively. Rectus muscle hematoma is an uncommon cause of acute abdomen, and has been reported in about 100 cases in Japan. It occurs because of a tear in epigastric vessels and is usually managed conservatively with a good prognosis, although hemodynamically unstable cases require surgery. To the best of the authors' knowledge, this is the first case of rectus muscle hematoma complicated with ANCA-associated vasculitis. PMID:19715163

Sakaguchi, Yusuke; Niihata, Kakuya; Yasuda, Keiko; Shimomura, Akihiro; Uehata, Takuya; Inoue, Kazunori; Kaneko, Tetsuya; Shoji, Tatsuya; Tsubakihara, Yoshiharu; Okada, Noriyuki

2009-01-01

146

Dysregulation of Autoantigen Genes in ANCA-Associated Vasculitis Involves Alternative Transcripts and New Protein Synthesis.  

PubMed

Proteinase 3 (PR3) and myeloperoxidase (MPO) are two major autoantigens in patients with vasculitis with ANCA. The genes encoding these autoantigens are abnormally expressed in peripheral granulocytes of patients with active ANCA-associated vasculitis. This study provides evidence that this transcriptional dysregulation results in a variety of mRNA processing events from the PRTN3 gene locus. In addition to elevated levels of PR3 message, leukocyte RNA from patients contained PR3 transcripts with an alternative 3' untranslated region. Furthermore, we detected usage of an alternative transcription start site within intron 1 of the PRTN3 gene locus that coincided with active disease (odds ratio, 3.3; 95% confidence interval, 1.3 to 8.4; P=0.01). This promoter may be developmentally regulated, because it was active in normal human bone marrow, multiple leukemia cell lines, MCF-7 cells, and subjects after GM-CSF treatment but not subjects with a neutrophil left shift. This transcript, which lacks exon 1 of PRTN3, encodes a 24-kD protein (p24(PR3/MBN)) with a sequence similar to that previously described for myeloblastin. Notably, PR3, p24(PR3/MBN), and MPO were synthesized in cultured neutrophils from patients with active ANCA-associated vasculitis, indicating that increased transcription results in newly synthesized autoantigens in peripheral neutrophils of patients. The synthesis of p24(PR3/MBN) seems to expand the autoantigen repertoire, because immunoblots showed that sera from patients recognized p24(PR3/MBN). These findings emphasize the importance of transcriptional dysregulation of the autoantigen in autoimmune disease. PMID:25060059

McInnis, Elizabeth A; Badhwar, Anshul K; Muthigi, Akhil; Lardinois, Olivier M; Allred, S Colby; Yang, Jiajin; Free, Meghan E; Jennette, J Charles; Preston, Gloria A; Falk, Ronald J; Ciavatta, Dominic J

2015-02-01

147

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

PubMed Central

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

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

2011-01-01

148

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

149

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

PubMed

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

2014-10-01

150

Systemic lupus erythematosus presenting with a fatal intestinal vasculitis: a case report.  

PubMed

This case report demonstrates fatal gastrointestinal vasculitis as a rare presentation of systemic lupus erythematosus. A 34-year-old woman presented with abdominal pain and diarrhea. Anti nuclear antibody was positive and high titre of anti-ds DNA antibody was also reported. Treatment with corticosteroid and supportive cares were started; however, her condition worsened. Eventually, she was considered as a candidate for diagnostic laparoscopy. Immediately after laparoscopy, she developed respiratory distress along with upper gastrointestinal bleeding. Soon after, the patient died because of disseminated intravascular coagulation . PMID:25093065

Nozari, Neda; Divsalar, Parisa

2014-07-01

151

Segmental testicular infarction due to minocycline-induced antineutrophil cytoplasmic antibody--positive vasculitis.  

PubMed

Segmental testicular infarction is an uncommon clinical entity marked by acute scrotal pain and swelling. Classically, these appear as wedge-shaped, avascular, hypoechoic lesions on a testicular ultrasound. We present a unique case of testicular infarct caused by an antineutrophil cytoplasmic antibody-positive vasculitis secondary to the use of the antibiotic minocycline. The patient's symptoms resolved with cessation of minocycline. We suggest that patients who present with otherwise unexplained testicular infarction undergo a careful review of medications to uncover a potential cause. PMID:24793001

Lyon, Timothy D; Ferroni, Matthew C; Casella, Daniel P; D'Agostino, Louis A; Jackman, Stephen V

2014-07-01

152

Hepatic vasculitis mimicking liver abscesses in a patient with systemic lupus erythematosus  

PubMed Central

Clinical and radiological liver diseases are uncommon in patients with systemic lupus erythematosus (SLE). We report a 29-year-old female with SLE who presented with right upper quadrant abdominal pain, thrombocytopenia, elevated liver enzymes and multiple hypodense lesions in the liver on a computed tomography (CT) study that mimicked multiple liver abscesses. A liver biopsy showed mild chronic inflammation. Culture results were negative. With steroid therapy the patient improved clinically, the platelet count returned to the normal range and the multiple liver lesions disappeared radiologicaly. This patient represents a rare case of SLE that had hepatic vasculitis mimicking multiple liver abscesses. PMID:19847087

Alanazi, Thari; Alqahtani, Mohammad; Al Duraihim, Huda; Al Khathlan, Khalid; Al Ahmari, Bader; Makanjuola, Dorothy; Afzal, Mohammad

2009-01-01

153

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

154

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.

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

2014-01-01

155

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

156

Occurrence of relapsing polychondritis with a rising cANCA titre in a cANCA-positive systemic and cerebral vasculitis patient  

PubMed Central

Relapsing polychondritis (RP) is a relatively rare disorder, with a high death rate that affects cartilaginous structures. RP can be either primary or secondary as part of autoimmune syndromes. We present a case of RP in a 49-year-old man suffering from cytoplasmic antineurophil cytoplasmic antibodies (cANCA) positive vasculitis, admitted to our hospital with red swollen left ear and painful sternoclavicular joint and larynx. The patient was in remission from the vasculitis but manifested a high cANCA titre indicating vasculitis activity. With his high cANCA titre vasculitis, full manifestation of RP concomitantly occurred. After a successful cortisone treatment for RP, the patient received cyclophosphamide treatment for his vasculitis that resulted in a decrease in cANCA titre and full remission of his RP symptoms. PMID:23417970

Mattiassich, Georg; Egger, Markus; Semlitsch, Georg; Rainer, Franz

2013-01-01

157

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

PubMed Central

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

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

2014-01-01

158

[A rapidly progressive orbital tumor in a seven-year-old girl revealing a primitive systemic vasculitis].  

PubMed

Orbital tumor lesions in pediatric population encompass a wide range of pathological processes, which are very different in etiology and prognosis. They usually require an emergency in histological diagnosis because of their quick growth. Beside malignant and benign tumors, the inflammatory pseudo-tumors group included dysimmune orbital involvement secondary to systemic vasculitis such as granulomatosis with polyangiitis (ex-Wegener's granulomatosis). We report the case of a seven-year-old girl suffering from a severe primitive ANCA vasculitis, revealed by an orbital mass. PMID:25499862

Picard, Cécile; Borgnat, Florent; Chastang, Marie-Caroline; Fleury, Aude; Guibaud, Laurent; Dijoud, Frédérique

2014-12-01

159

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

160

Retinal vasculitis and ocular vitreous metastasis following complete response to PD-1 inhibition in a patient with metastatic cutaneous melanoma.  

PubMed

We report on a 36-year-old woman treated with the anti PD-1 antibody Pembrolizumab for metastatic cutaneous melanoma in the first line setting. She achieved a complete response and then relapsed with metastases to the vitreous cavity with an associated angiographically determined retinal vasculitis. Vitreous metastasis without choroidal involvement is unusual and may be due to individual cell extravasation, vitreous hemorrhage containing malignant cells, or direct spread through the optic nerve. This finding highlights the need for immune sanctuary sites to be monitored in the presence of PD-1 inhibition and we hypothesize that the use of PD-1 inhibitor potentiated the patient's angiographically determined retinal vasculitis. PMID:25516805

Manusow, Joshua S; Khoja, Leila; Pesin, Nataly; Joshua, Anthony M; Mandelcorn, Efrem D

2014-01-01

161

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

PubMed Central

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

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

1995-01-01

162

Myocardial Ischemia in Wegener’s Granulomatosis: Coronary Atherosclerosis Versus Vasculitis  

PubMed Central

Wegener’s granulomatosis (WG) is one of the most common small- and medium-sized necrotizing vasculitides that mainly affects the upper and lower respiratory tract and the kidneys. Cardiac manifestations in WG are relatively rare, and their role and place among different causes of mortality remain largely unknown. Substantially increased number of reports describing involvement of all structures of the heart, which underlie conduction disturbances, valvular disease, ischemic heart disease and other potentially serious conditions, underscores importance of comprehensive cardiovascular investigations and monitoring of patients with WG. The majority of previous reports and our current observation distinguish coronary vasculitis and thrombosis as a cause of myocardial ischemia and cardiovascular co-morbidities in WG. It seems plausible that inflammatory processes in this disease, like in some other systemic vasculitidies, do not predispose to accelerated atherogenesis. However, characteristic small- and medium-sized vasculitis still can manifest as myocardial ischemia and infarction. We overview diverse cardiac manifestations and present our own rare case of angina in the oligosymptomatic debut of WG. Importantly, in this case, coronarography failed to reveal atherosclerotic disease or thrombotic occlusion. However, magnetic resonance imaging (MRI) with adenosine test revealed subendocardial ischemia. As a result of immunosuppressive therapy with a steroid and cyclophosphamide, myocardial ischemia disappeared. PMID:20360980

Cocco, Giuseppe; Gasparyan, Armen Yuri

2010-01-01

163

The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of 57 patients.  

PubMed

Background: Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology, rarely described in the literature. Patients and methods: To analyze the clinical spectrum and the therapeutic management of patients with HUV, we conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histological leukocytoclastic vasculitis and hypocomplementemia. Results: Urticarial lesions were typically erythematous papules more pruritic than painful, associated with angioedema in 51%, purpura in 35% and livedo reticularis in 14%. Extra-cutaneous manifestations included constitutional symptoms (56%), musculoskeletal (82%), ocular (56%), pulmonary (19%), gastrointestinal (18%) and kidney involvement (14%). Patients with HUV typically presented with low C1q complement level and normal C1 inhibitor level, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine (HCQ) or colchicine seemed to be as effective as corticosteroids in 1(st) -line therapy. In patients with relapsing and/or refractory disease, cutaneous and immunological response rates seemed to be higher using immunosuppressive agents, in particular azathioprine (AZA), mycophenolate mofetil (MMF) or cyclophosphamide, while rituximab (RTX)-based regimen may have a higher efficacy. Finally, cutaneous response was strongly associated with immunological response after therapy. Conclusion: HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly musculoskeletal and ocular involvement associated with anti-C1q antibodies found in half of patients. The therapeutic strategy has yet to be defined. © 2014 American College of Rheumatology. PMID:25385679

Jachiet, Marie; Flageul, Béatrice; Deroux, Alban; Le Quellec, Alain; Maurier, François; Cordoliani, Florence; Godmer, Pascal; Abasq, Claire; Astudillo, Leonardo; Belenotti, Pauline; Bessis, Didier; Bigot, Adrien; Doutre, Marie-Sylvie; Ebbo, Mikaël; Guichard, Isabelle; Hachulla, Eric; Heron, Emmanuel; Jeudy, Géraldine; Jourde-Chiche, Noémie; Jullien, Denis; Lavigne, Christian; Machet, Laurent; Macher, Marie-Alice; Martel, Clotilde; Melboucy-Belkhir, Sara; Morice, Cécile; Petit, Antoine; Simorre, Bernard; Zenone, Thierry; Bouillet, Laurence; Bagot, Martine; Frémeaux-Bacchi, Véronique; Guillevin, Loďc; Mouthon, Luc; Dupin, Nicolas; Aractingi, Selim; Terrier, Benjamin

2014-11-10

164

Randomized Trial of Plasma Exchange or High-Dosage Methylprednisolone as Adjunctive Therapy for Severe Renal Vasculitis  

Microsoft Academic Search

Systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA) is the most frequent cause of rapidly progressive glomerulonephritis. Renal failure at presentation carries an increased risk for ESRD and death despite immunosuppressive therapy. This study investigated whether the addition of plasma exchange was more effective than intravenous methylprednisolone in the achievement of renal recovery in those who presented with

David R. W. Jayne; Gill Gaskin; Niels Rasmussen; Daniel Abramowicz; Franco Ferrario; Loic Guillevin; Eduardo Mirapeix; Caroline O. S. Savage; Renato A. Sinico; Coen A. Stegeman; Kerstin W. Westman; Fokko J. van der Woude; Charles D. Pusey

2007-01-01

165

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

166

Rheumatoid Vasculitis  

MedlinePLUS

... in RV – for example, elevations in the erythrocyte sedimentation rate or C-reactive protein – are non-specific, ... in RV – for example, elevations in the erythrocyte sedimentation rate or C-reactive protein are non-specific, ...

167

Urticarial Vasculitis  

MedlinePLUS

... Founder – October 1995 VF Board of Directors VF Annual Report FY 2013-2014 Staff Contact Us Donate Membership ... Living Our Dreams VF Board of Directors VF Annual Report FY 2013-2014 VF Board of Directors Staff ...

168

Circulating cytokine profile in anti-neutrophilic cytoplasmatic autoantibody-associated vasculitis: prediction of outcome?  

PubMed Central

AIMS: The anti-neutrophilic cytoplasmatic autoantibody-associated vasculitides (AASV) are diseases of relapsing-remitting inflammation. Here we explore the cytokine profile in different phases of disease, looking for pathogenic clues of possible prognostic value. RESULTS: Interleukin (IL)-6, IL-8 and IL-10 were significantly elevated in plasma. Patients in the stable phase who subsequently developed adverse events had higher IL-8 values. Patients in the stable phase who relapsed within 3 months had lower IL-10 values and higher IL-6 levels. CONCLUSIONS: Patients with AASV have raised circulating cytokine levels compared with healthy controls, even during remission. Raised IL-8 seems associated with poor prognosis. Lower levels of IL-10 and higher levels of IL-6 herald a greater risk of relapse. Patients with systemic vasculitis in clinical remission have persistent disease activity, kept under control by inhibitory cytokines. PMID:15545059

Ohlsson, Sophie; Wieslander, Jörgen; Segelmark, Mĺrten

2004-01-01

169

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

PubMed

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

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

2014-04-01

170

Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement.  

PubMed

Granuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brown-purple papules, plaques and/or nodules, localized mostly on the face, although extrafacial lesions can also occur. Eosinophilic angiocentric fibrosis (EAF) is regarded as the mucosal counterpart of GF. Histologically, it has been described as a persistent leukocytoclastic vasculitis, with a dense polymorphous inflammatory infiltrate in the superficial and mid dermis, typically sparing the subpapillary dermis, the so called grenz zone. The presence of eosinophils is considered a characteristic feature of the disease. All the cases of GF seen at the Dermatology Unit from 2002 to 2013 were considered and reviewed, both clinically and histopathologically. Only cases with consistent clinical findings of GF, and accurate patient's history were considered. Ten cases of GF were reviewed for both histological specificity and clinico-pathological correlation. Two patients presented extrafacial lesions. One patient had involvement of nasal mucosa. Two patients suffered from associated rheumatological diseases. The most frequent histopathologic features were the presence of a grenz zone and eosinophils in the infiltrate, but also adnexal involvement was often present; vascular changes were constant, yet leukocytoclastic vasculitis could be recorded only in four cases. Fibrosis or sclerosis were always absent. Clinical pictures of the patients treated demonstrated a complete remission of the lesions, without scarring. However, a complete enduring healing was observed only in two patients, and relapse or incomplete remission of the disease was the rule. In conclusion a review of clinicopathological findings of ten patients affected by GF was made and new details of the disease presented. PMID:25592670

Vassallo, C; Derlino, F; Croci, G A; Brazzelli, V; Borroni, G

2015-02-01

171

Eosinophilic/T-cell chorionic vasculitis: a clinicopathologic and immunohistochemical study of 51 cases.  

PubMed

We report 51 placentas diagnosed with eosinophilic/T-cell chorionic vasculitis (E/TCV), an unusual form of chorionic vasculitis characterized by an infiltrate composed predominantly of CD3+ T cells and eosinophils. The placentas were all 3rd trimester, with 48 (94.1%) being term. Forty-seven (92.2%) were singleton placentas, and the remaining 4 were twins. The E/TCV was limited to 1 chorionic surface vessel in 40 (78.4%) and involved 50% or less of the vessel circumference in 30 (58.8%) placentas. The inflammation faced the intervillous space in 12 (23.5%) and the amniotic cavity in 8 (15.7%) and had no distinct predominant direction in the remaining 31 (60.8%) placentas. Twelve (25.5%) placentas showed mural thrombi or intramural fibrin in association with the E/TCV. One hundred six term singleton placentas were selected as the control group, and the 47 singleton placentas with E/TCV made up the study group for comparison of demographic and histopathologic features. Villitis of unknown etiology was identified more frequently in study group placentas (20 [42.6%]) compared with control group placentas (14 [13.2%]) (P < 0.001). Vascular changes of fetal vascular thrombo-occlusive disease were identified away from the E/TCV more frequently in study group placentas (8 [17.0%]) compared with control group placentas (4 [3.8%]) (P ?=? 0.008). There were no significant differences in the frequencies of other placental lesions studied, including acute inflammatory lesions and lesions related to maternal underperfusion. There were no significant differences in maternal age, race, parity, birth weight, allergy history, blood type, or medication use. PMID:21050080

Jacques, Suzanne M; Qureshi, Faisal; Kim, Chong Jai; Lee, Joon Ho; Giorgadze, Tamar; Mittal, Pooja; Hassan, Sonia S; Romero, Roberto

2011-01-01

172

Epigenetic basis for aberrant upregulation of autoantigen genes in humans with ANCA vasculitis.  

PubMed

Antineutrophil cytoplasmic autoantibody (ANCA) causes vascular injury that leads to small-vessel vasculitis. Patients with ANCA aberrantly express neutrophil granule-encoding genes, including 2 that encode autoantigens: proteinase 3 (PR3) and myeloperoxidase (MPO). To uncover a potential transcriptional regulatory mechanism for PR3 and MPO disrupted in patients with ANCA vasculitis, we examined the PR3 and MPO loci in neutrophils from ANCA patients and healthy control individuals for epigenetic modifications associated with gene silencing. We found that levels of the chromatin modification H3K27me3, which is associated with gene silencing, were depleted at PR3 and MPO loci in ANCA patients compared with healthy controls. Interestingly, in both patients and controls, DNA was unmethylated at a CpG island in PR3, whereas in healthy controls, DNA was methylated at a CpG island in MPO. Consistent with decreased levels of H3K27me3, JMJD3, the demethylase specific for H3K27me3, was preferentially expressed in ANCA patients versus healthy controls. In addition, we describe a mechanism for recruiting the H3K27 methyltransferase enhancer of zeste homolog 2 (EZH2) to PR3 and MPO loci mediated by RUNX3. RUNX3 message was decreased in patients compared with healthy controls, and may also be under epigenetic control. DNA methylation was increased at the RUNX3 promoter in ANCA patients. These data indicate that epigenetic modifications associated with gene silencing are perturbed at ANCA autoantigen-encoding genes, potentially contributing to inappropriate expression of PR3 and MPO in ANCA patients. PMID:20714105

Ciavatta, Dominic J; Yang, Jiajin; Preston, Gloria A; Badhwar, Anshul K; Xiao, Hong; Hewins, Peter; Nester, Carla M; Pendergraft, William F; Magnuson, Terry R; Jennette, J Charles; Falk, Ronald J

2010-09-01

173

Crohns disease with central nervous system vasculitis causing subarachnoid hemorrhage due to aneurysm and cerebral ischemic stroke.  

PubMed

Cerebral vasculitis secondary to Crohn's disease (CD) seems to be a very rare phenomenon. We report a 39-year-old male who presented with headache, vomiting, and left-sided weakness in the known case of CD. Cross-sectional imaging (computed tomography and magnetic resonance imaging,) showed right gangliocapsular acute infarct with supraclinoid cistern subarachnoid hemorrhage (SAH). Cerebral digital substraction angiography (DSA) showed dilatation and narrowing of right distal internal carotid artery (ICA). Left ICA was chronically occluded. His inflammatory markers were significantly raised. Imaging features are suggestive of cerebral vasculitis. Arterial and venous infarcts due to thrombosis are known in CD. Our case presented with acute subarachnoid hemorrhage in supraclinoid cistern due to rupture of tiny aneurysm of perforator arteries causing SAH and infarction in right basal ganglia. Patient was treated conservatively with immunosuppression along with medical management of SAH. PMID:25506170

Garge, Shaileshkumar S; Vyas, Pooja D; Modi, Pranav D; Ghatge, Sharad

2014-10-01

174

Cerebral vasculitis and encephalitis due to Epstein-Barr virus in a patient with newly diagnosed HIV infection.  

PubMed

Epstein-Barr virus (EBV) is a common infection which usually produces mild or no symptoms in immunocompetent individuals. In human immunodeficiency virus (HIV) associated immunosuppression it is most commonly associated with malignancy which usually occurs at very low CD4+ cell counts. We describe a newly diagnosed HIV-positive patient who presented with headaches and cerebellar signs. She was incorrectly diagnosed with cerebral tuberculosis (TB) infection based on the histology report from a cerebellar biopsy specimen. After extensive investigation including cerebrospinal fluid sampling and reanalysis of the brain biopsy specimens she was found to have EBV-associated cerebral vasculitis and encephalitis and was successfully treated with valganciclovir and steroids. Whilst there are a few reports of EBV-associated encephalitis, cerebral vasculitis secondary to EBV in the context of HIV infection has not previously been described in the literature. PMID:24568965

Raman, Lavanya; Nelson, Mark

2014-04-01

175

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

176

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

177

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

178

A Randomized Controlled Trial of Rituximab Following Failure of Antiviral Therapy for Hepatitis C-Associated Cryoglobulinemic Vasculitis  

PubMed Central

OBJECTIVES To report on the results of a randomized controlled trial of rituximab in hepatitis C virus (HCV)-associated mixed cryoglobulinemic vasculitis. METHODS We conducted an open-label single center randomized controlled trial of rituximab (375 mg/m2 per week for 4 weeks) compared to best available therapy for treatment of patients with HCV-associated cryoglobulinemic vasculitis in whom antiviral therapy failed to induce remission. The primary endpoint was remission at 6 months from study entry. RESULTS A total of 24 patients were enrolled. Baseline disease activity and organ involvement were similar in the two groups. Ten patients in the rituximab group (83%) were in remission at study month 6, compared with 1 patient in the control group (8%), a result that met criterion for stopping the study (P<0.001). The median duration of remission for rituximab-treated patients reaching the primary endpoint was 7 months. No adverse effect of rituximab on HCV plasma viremia or hepatic transaminase levels was observed. CONCLUSIONS Therapy with rituximab was well tolerated and effective treatment for patients with HCV-associated cryoglobulinemic vasculitis in whom antiviral therapy fails to induce remission. PMID:22147444

Sneller, Michael C.; Hu, Zonghui; Langford, Carol A.

2011-01-01

179

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

PubMed

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

Homma, Sakae; Suzuki, Aika; Sato, Keita

2013-10-01

180

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

PubMed Central

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

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

2014-01-01

181

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; Beaufrčre, Hugues; Heggem-Perry, Brittany; Field, Cara; Garner, Michael

2013-12-01

182

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

183

Necrotic, haemorrhagic, hepatomegalic hepatitis associated with vasculitis and amyloidosis in commercial laying hens.  

PubMed

A 60,000 layer flock in a multi-age commercial operation experienced high weekly mortality ranging from 0.17% to 0.36% starting at 40 weeks of age. The most prominent post-mortem findings were enlargement of the liver and spleen (up to 3 times normal) and liquid blood in the abdominal cavity. Microscopic examination of the liver revealed large or small areas of coagulative necrosis and occasional granulomas. Small pools of amorphous eosinophilic material were found around some areas of necrosis and cords of hepatocytes were separated by pink-staining material. There was generalized lymphoid depletion and multifocal areas of interwoven, light pink-staining material in the spleen. The pink material was shown to contain amyloid by Congo red staining. A vasculitis, characterized by the proliferation of inflammatory cells within and around the portal veins, was also observed. These findings tend to strengthen a theory that 'hepatitis-liver hemorrhage syndrome' could be precipitated by a continuous immune response to an infectious agent such as would occur by vaccination with a live vaccine that continues to replicate in the body or with an inactivated vaccine containing a slow-release adjuvant. PMID:18671138

Tablante, N L; Vaillancourt, J P; Julian, R J

1994-12-01

184

Necrotizing villitis and decidual vasculitis in the placentas of mothers with Behçet disease.  

PubMed

Behçet disease is an inflammatory disorder characterized by recurrent aphthous stomatitis, genital ulcers, uveitis, and skin lesions. There are occasional reports of neonatal Behçet disease suggesting a possible prenatal transmission. However, no placental Behçet disease lesion has been reported. Here, we report characteristic necrotizing inflammation in placentas and decidua from 2 patients with Behçet disease. The first patient had full-term delivery after uneventful medical therapy. The second patient underwent therapeutic abortion in the first trimester because of the fetal exposure to a potentially harmful medication for Behçet disease. Both placentas showed focal necrotizing villitis with neutrophil-dominant infiltration, similar to the characteristic Behçet disease lesions in other organs. Intervillous fibrinous exudates with neutrophilic infiltrates and granuloma-like histiocytic aggregates were present. The decidua also showed neutrophilic vasculitis. No evidence of infection was present. Our observation suggests that Behçet disease may involve placentas from the early stage of pregnancy. It may be implicated in potential fetal compromise and/or intrauterine transmission of the disease. PMID:18715615

Hwang, Ilseon; Lee, Chang-Keun; Yoo, Bin; Lee, Inchul

2009-01-01

185

Biomarkers of endothelial cell activation: candidate markers for drug-induced vasculitis in patients or drug-induced vascular injury in animals.  

PubMed

There is a pressing need for vascular biomarkers for studies of drug-induced vasculitis in patients and drug-induced vascular injury (DIVI) in animals. We previously reviewed a variety of candidate biomarkers of endothelial cell (EC) activation (Zhang et al., 2010). Now we update information on EC activation biomarkers from animal data on DIVI and clinical data of vasculitic patients, particularly patients with primary antineutrophil cytoplasmic autoantibody (ANCA)-associated small vessel vasculitis (primary AAVs), including Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and necrotizing crescentic glomerulonephritis. Drug-associated ANCA-positive small vessel vasculitis (drug-AAVs) can closely resemble primary AAVs, suggesting the large overlap between primary idiopathic systemic vasculitis and drug-induced vasculitis. AAVs in patients and DIVI in animals vary considerably; however, there is close resemblance between AAVs and DIVI in some respects: (1) the immunopathogenetic mechanisms (activation of primed neutrophils, ECs and T cells by ANCA in patients and activation of ECs, mast cells, and macrophages by drugs in animals); (2) the morphologic changes (fibrinoid necrosis of the vessel wall and neutrophilic infiltration); (3) the preferable sites (small arteries, arterioles, capillaries and venules); and (4) elevation of vascular biomarkers suggestive of an endothelial origin. The present review discusses soluble and cell component biomarkers and provides a rationale for the potential utility of EC activation biomarkers in nonclinical and clinical studies during new drug development. Further investigation, however, is needed to assess their potential utility. PMID:21968053

Zhang, Jun; Hanig, Joseph P; De Felice, Albert F

2012-01-01

186

Rituximab use in patients with ANCA-associated vasculitis: clinical efficacy and impact on immunological parameters.  

PubMed

Rituximab (RTX) was reported effective in ANCA-associated vasculitis (AAV). We aimed to evaluate clinical efficacy of RTX in AAV along with its impact on immunological parameters. Eighteen RTX-treated AAV patients (M/F 11/7; median age 37.5; 15× PR3-ANCA, 3× MPO-ANCA; 16× relapsing/refractory, 2× first-line therapy) were enrolled. Clinical response, ANCA, total serum IgG levels and cellular immunity parameters were examined. The patients were followed up (FU) for a median of 26 months (range 3-82, 15 for ?6 months). All patients achieved B cell depletion (lasting 3-24 months). No significant increase was noted in T cell or NK cell subpopulations. At 6 months, partial remission was achieved in 5/15 patients (33 %) and complete in 8 (53 %). The median prednisone dose (30..10 mg/d) and ANCA levels (17.2..2.7 IU/mL) decreased (p?

Chocova, Z; Hruskova, Z; Mareckova, H; Svobodova, B; Duskova, D; Bednarova, V; Jancova, E; Rysava, R; Tesar, V

2015-01-01

187

Intravenous Cyclophosphamide and Plasmapheresis in Dialysis-Dependent ANCA-Associated Vasculitis  

PubMed Central

Summary Background and objectives Induction therapy with oral cyclophosphamide (CYP) has been a mainstay of treatment in patients with severe renal failure secondary to ANCA-associated vasculitis (AAV). Recent evidence proposes using pulsed intravenous CYP in less severe disease to minimize adverse events. It is unclear if this can be translated to those with dialysis-dependent renal insufficiency. Design, setting, participants, & methods All AAV patients presenting between 2005 and 2010 requiring dialysis at presentation were retrospectively analyzed. Patients were treated with plasma exchange, corticosteroids, and intravenous CYP. Rate of dialysis independence at 3 and 12 months and adverse effects were assessed and compared with the outcome of the plasmapheresis, prednisolone, and oral CYP arm of the randomized MEPEX (methylprednisolone versus plasma exchange) trial. Results Forty-one patients were included. At 3 months, 3 (7.3%) patients had died on dialysis, 12 (29.3%) remained dialysis dependent, and 26 (63.4%) were dialysis independent (creatinine, 2.5 mg/dl; GFR, 26 ml/min per 1.73 m2). Four patients subsequently reached ESRD at a median time of 83 days. Thirty-seven (90%) patients reached 1 year follow-up, 13 (35%) remained dialysis dependent, and 24 (65%) had independent renal function. Eleven patients (27%) had episodes of leukopenia (white cell count <4×109/L) during CYP therapy and 17 (41%) experienced infectious complications. This compares favorably with the dialysis-dependent cohort treated with plasmapheresis in the MEPEX study in which 51% were alive with independent renal function at 1 year. Conclusions Intravenous CYP used with corticosteroids and plasmapheresis may be an effective alternative to oral CYP in patients with dialysis-dependent AAV. PMID:23160261

Pepper, Ruth J.; Chanouzas, Dimitrios; Tarzi, Ruth; Little, Mark A.; Casian, Alina; Walsh, Michael; Pusey, Charles D.; Harper, Lorraine

2013-01-01

188

Cutaneous Vasculitis, Interstitial Pneumonia with Crazy-Paving Appearance, and Positive pANCA in a Patient with Severe Crohn's Disease  

PubMed Central

Cutaneous vasculitis, interstitial pneumonia with crazy-paving appearance on high-resolution computed tomography, and repeated positive perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) are rarely found together in patients with inflammatory bowel disease in the existing literature. We report the case of a Chinese patient previously diagnosed with cutaneous vasculitis and interstitial pneumonia, who presented with acute pain and mass in his right lower quadrant a couple of years later. The terminal ileum biopsy and postoperative pathology confirmed Crohn's disease (CD). PMID:25371834

Chen, Guang-liang; Wang, Juan; Li, Li-mei; Mo, Han-you; Ye, Shuang

2014-01-01

189

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

PubMed Central

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

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

2014-01-01

190

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

191

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.Kidney International advance online publication, 1 October 2014; doi:10.1038/ki.2014.329. 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

2014-10-01

192

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

PubMed Central

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

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

2014-01-01

193

Aggressive cutaneous vasculitis in a patient with chronic lymphatic leukemia following granulocyte colony stimulating factor injection: a case report  

PubMed Central

Introduction Vasculitis has been reported in a few cases of chronic lymphatic leukemia and with granulocytic colony-stimulating factor therapy. Those with granulocytic colony-stimulating factor occurred after prolonged therapy and there was a rise in total leukocyte count unlike that in our patient who received just a single injection for the first time. Case presentation We report the case of a 64-year-old Egyptian man with chronic lymphatic leukemia who developed progressive cutaneous vasculitic lesions following injection of a single dose of a granulocytic colony stimulating factor before a third cycle of chemotherapy to improve neutropenia. This is an unusual case and the pathogenesis is not fully understood. Our patient was not on any medical treatment except for bisoprolol for ischemic heart disease. Although aggressive management with steroids, anticoagulation and plasmapheresis had been carried out, the condition was aggressive and the patient's consciousness deteriorated. A magnetic resonance imaging scan of his brain revealed multiple ischemic foci that could be attributed to vasculitis of the brain. Conclusion The aim of this case report is to highlight the importance of monitoring patients on granulocytic colony-stimulating factor therapy, especially in the context of other conditions (such as a hematological malignancy) that may lead to an adverse outcome. PMID:21362198

2011-01-01

194

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

195

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...

196

Current and emerging treatment options for ANCA-associated vasculitis: potential role of belimumab and other BAFF/APRIL targeting agents  

PubMed Central

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises several clinical entities with diverse clinical presentations, outcomes, and nonunifying pathogenesis. AAV has a clear potential for relapses, and shows unpredictable response to treatment. Cyclophosphamide-based therapies have remained the hallmark of induction therapy protocols for more than four decades. Recently, B-cell depleting therapy with the anti-CD20 antibody rituximab has proved beneficial in AAV, leading to Food and Drug Administration approval of rituximab in combination with corticosteroids for the treatment of AAV in adults. Rituximab for ANCA-associated vasculitis and other clinical trials provided clear evidence that rituximab was not inferior to cyclophosphamide for remission induction, and rituximab appeared even more beneficial in patients with relapsing disease. This raised hopes that other B-cell-targeted therapies directed either against CD19, CD20, CD22, or B-cell survival factors, B-cell activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand could also be beneficial for the management of AAV. BAFF neutralization with the fully humanized monoclonal antibody belimumab has already shown success in human systemic lupus erythematosus and, along with another anti-BAFF reagent blisibimod, is currently undergoing Phase II and III clinical trials in AAV. Local production of BAFF in granulomatous lesions and elevated levels of serum BAFF in AAV provide a rationale for BAFF-targeted therapies not only in AAV but also in other forms of vasculitis such as Behcet’s disease, large-vessel vasculitis, or cryoglobulinemic vasculitis secondary to chronic hepatitis C infection. BAFF-targeted therapies have a very solid safety profile, and may have an additional benefit of preferentially targeting newly arising autoreactive B cells over non-self-reactive B cells. PMID:25609919

Lenert, Aleksander; Lenert, Petar

2015-01-01

197

Primary vasculitis of the central nervous system in patients infected with HIV-1 in the HAART era.  

PubMed

Angiitis of the central nervous system (CNS) in patients infected with HIV-1-is often associated with concomitant infection or lymphoproliferative disease of the CNS. Four HAART naďve patients infected with HIV-1 with severe stroke are described. Evidence of vasculitis was found by magnetic resonance angiography. Extensive investigations excluded concomitant opportunistic, lymphoproliferative or autoimmune disorders leading to the diagnosis of primary angiitis of the CNS. Despite initiation of HAART and prolonged suppression of viral replication, these patients remained severely immunosuppressed. The addition of corticosteroids led to a significant improvement of clinical symptoms. Primary angiitis of the CNS should be considered in patients with HIV and stroke. The prognosis of these patients remain poor despite HAART. These observations suggest that the vascular inflammatory process persists despite the control of viral load under HAART in patients with persistent immunosuppression. PMID:19235861

Melica, Giovanna; Brugieres, Pierre; Lascaux, Anne-Sophie; Levy, Yves; Leličvre, Jean-Daniel

2009-04-01

198

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

PubMed

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

Schmalzing, M; Amann, K; Tony, H-P

2014-01-01

199

Meta-analysis of myeloperoxidase G-463/A polymorphism in anti-neutrophil cytoplasmic autoantibody-positive vasculitis  

PubMed Central

Wegener's granulomatosis, microscopic polyangiitis and Churg Strauss syndrome are small-vessel vasculitides associated with anti-neutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) and myeloperoxidase (MPO). A G to A polymorphism at position 463 in the promoter region of the MPO gene, which leads to the loss of a SP1 transcription binding site in an Alu hormone responsive element, reduces MPO expression. We hypothesized that MPO alleles may play a role in determining disease susceptibility or severity in ANCA-associated vasculitis (AASV). MPO genotypes were determined by restriction fragment length polymorphism polymerase chain reaction (RFLP/PCR) in 134 Caucasian patients (Wegener's granulomatosis, n = 69; microscopic polyangiitis, n = 65; PR3–ANCA n = 91; MPO–ANCA, n = 43) and 150 matched healthy controls. There was no difference in survival to renal failure or death in patients with the different MPO alleles (?2 = 0·904, P = 0·6362) or in presenting serum creatinine concentration based on MPO genotype (?2 = 0·389, P = 0·8232). There was no significant difference in genotype frequencies between controls (13AA, 102GG, 35GA) and patients (14AA, 97GG, 23GA: ?2 = 1·75, P = 0·417), patients with Wegener's granulomatosis (5AA, 53GG, 11GA: ?2 = 1·864, P = 0·3938) or patients with microscopic polyangiitis (9AA, 44GG, 12GA: ?2 = 1·682, P = 0·4317). A meta-analysis of our study and two previous studies showed that there was no association between the myeloperoxidase G-463/A polymorphism and the risk of developing ANCA-associated vasculitis; GG versus GA plus AA (odds ratio 1·14; 95% confidence interval 0·86–1·50). The MPO G-463/A polymorphism is not a risk factor for the development or severity of AASV. PMID:17521322

Rajp, A; Adu, D; Savage, C O

2007-01-01

200

Bactericidal/permeability-increasing protein (BPI) is an important antigen for anti-neutrophil cytoplasmic autoantibodies (ANCA) in vasculitis.  

PubMed Central

Indirect immunofluorescence (IIF) techniques have shown that ANCA are useful serological markers for some small vessel vasculitides, and ELISA assays, using purified molecules as solid-phase ligand, have helped to identify proteinase 3 (PR3) and myeloperoxidase (MPO) as two of the major ANCA antigens. There remain a substantial number of serum samples, which are positive by IIF, yet recognize neither PR3 nor MPO (double-negative samples). We found, by Western blot analysis of soluble neutrophil granule proteins, that certain of these double-negative samples recognized a 55-kD doublet of which the first eight residues shared N-terminal amino acid sequence homology with BPI, a potent antibiotic towards Gram-negative bacteria. We developed a simple, quick and robust two-step immunobiochemical method to purify BPI. This was then employed to detect anti-BPI autoantibodies by ELISA and Western blot analysis. We tested 100 double-negative samples and 400 consecutive new samples sent for routine ANCA testing in the anti-BPI ELISA. We found that 45 of the 100 double-negative and 44 of the 400 new routine samples recognized BPI. By Western blot analysis 20/20 positive anti-BPI samples blotted the 55-kD protein. Inhibition assays confirmed the specificity of binding. Review of the 89 anti-BPI-positive patients showed a male dominance (M:F ratio 55:34), a mean age of 60.4 years and clinical diagnoses ranging from organ limited vasculitis to widespread systemic vasculitis. Images Fig. 2 Fig. 5 PMID:7813109

Zhao, M H; Jones, S J; Lockwood, C M

1995-01-01

201

Macrophage Migration Inhibitory Factor (MIF) and Thyroid Hormone Alterations in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV)  

PubMed Central

Macrophage migration inhibitory factor (MIF) is a proinflammatory cytokine known to be released from lymphocytes, macrophages and endothelial cells and also in animal models shown to be inducible with glucocorticoids (GC). In contrast, thyroxine seems to antagonize MIF activity. To investigate whether MIF is increased in active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and possible correlations with GC dosing and thyroid hormone levels, 27 consecutive patients with active AAV were studied and followed prospectively. Disease activity was assessed using Birmingham Vasculitis Activity Score 2003 (BVAS) at baseline and at follow-up at 3 and 6 months, along with MIF, thyroid hormones free triiodothyronine (fT3) and free thyroxine (fT4), C-reactive protein (CRP) and creatinine. MIF was elevated significantly at baseline compared with follow-up at 3 and 6 months (8,618 pg/mL versus 5,696 and 6,212 respectively; P < 0.002) but did not correlate to CRP, GC dose, creatinine or organ involvement. fT3 was depressed significantly at baseline compared with follow-up (1.99 pg/mL versus 2.31 and 2.67 respectively; P = 0.01) and correlated inversely to the BVAS score at baseline. We found a significant correlation between the MIF/fT4 ratio at baseline versus MIF/fT4 ratio at 6 months (? = 0.52, P < 0.005) and a trend between the baseline MIF/fT3 ratio versus MIF/fT3 ratio at 6 months (? = 0.39, P = 0.05). These results suggest a possible role for MIF and thyroid status in AAV. Further studies could reveal whether the association between AAV and thyroid hormone levels in the context of elevated MIF may present a link as well as a target of treatment. PMID:23552723

Wendt, Mĺrten; Börjesson, Ola; Avik, Aune; Bratt, Johan; Anderstam, Björn; Qureshi, Abdul R; Miller, Edmund J; Gunnarsson, Iva; Bruchfeld, Annette

2013-01-01

202

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

203

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

204

TWEAK Enhances E-selectin and ICAM-1 Expression, and May Contribute to the Development of Cutaneous Vasculitis  

PubMed Central

Our previous work indicated that TWEAK is associated with various types of cutaneous vasculitis (CV). Herein, we investigate the effects of TWEAK on vascular injury and adhesion molecule expression in CV mice. We showed that TWEAK priming in mice induced a local CV. Furthermore, TWEAK priming also increased the extravasation of FITC-BSA, myeloperoxidase activity and the expression of E-selectin and ICAM-1. Conversely, TWEAK blockade ameliorated the LPS-induced vascular damage, leukocyte infiltrates and adhesion molecules expression in LPS-induced CV. In addition, TWEAK treatment of HDMECs up-regulated E-selectin and ICAM-1 expression at both mRNA and protein levels. TWEAK also enhanced the adhesion of PMNs to HDMECs. Finally, western blot data revealed that TWEAK can induce phosphorylation of p38, JNK and ERK in HDMECs. These data suggest that TWEAK acted as an inducer of E-selectin and ICAM-1 expression in CV mice and HDMECs, may contribute to the development of CV. PMID:23457623

Chen, Tao; Guo, Zai-pei; Li, Li; Li, Meng-meng; Wang, Ting-ting; Jia, Rui-zhen; Cao, Na; Li, Jing-yi

2013-01-01

205

Cutaneous leukocytoclastic vasculitis due to erlotinib: just an adverse event or also a putative marker of drug efficacy?  

PubMed

Erlotinib is a targeted anticancer therapy with selective inhibitory activity for tyrosine kinase of the epidermal growth factor receptor (EGFR). Different skin reactions have been described linked to these drugs. There are no other reports about erlotinib-induced leukocytoclastic vasculitis (LV) in the erlotinib-bevacizumab regimen for bone metastasis, from a relapsed hepatocellular carcinoma (HCC) in liver-transplanted patients. In our patient a dose reduction and then the suspension of erlotinib was required. After a 2 week withdrawal, the drug was re-challenged at a lower dose. The patient continued it without any skin recurrence, and resulted progression free for 16 months. Thus, we underline the possibility to avoid a permanent withdrawal of erlotinib and to rechallenge with it without any cutaneous toxicity, particularly in patients benefiting from this drug. Moreover, the median overall survival from the initial treatment of bone relapsed patients after liver transplant for HCC is found to be less than 5 months, while our patient died 5 years later. This longer survival encourages further investigations to assess also whether LV, even if rare, might be used as a marker of antitumor efficacy of EGFR inhibitors. PMID:23534992

Brandi, Giovanni; Venturi, Michela; Dika, Emi; Maibach, Howard; Patrizi, Annalisa; Biasco, Guido

2013-10-01

206

Successful use of interleukin 6 antagonist tocilizumab in a patient with refractory cutaneous lupus and urticarial vasculitis.  

PubMed

Tocilizumab (Actemra; Genentech, Inc) is the first biologic therapy targeting the cytokine interleukin 6 (IL-6). It is a humanized monoclonal immunoglobulin G1 antibody against the ?-chain of the IL-6 receptor that prevents the binding of IL-6 to membrane-bound and -soluble IL-6 receptor. It was approved by the US Food and Drug Administration in January 2010 for rheumatoid arthritis refractory to other approved therapies and in April 2011 for systemic juvenile idiopathic arthritis. It has been used as an off-label treatment in many autoimmune diseases, where IL-6 plays a major role in pathogenesis. We report a case of refractory systemic lupus erythematosus in a 22-year-old woman with recurrent high-grade fever, polyarthritis, diffuse rash with urticarial vasculitis, and tumid lupus who did not respond to topical corticosteroids, photoprotection, antimalarials, methotrexate, anakinra, mycophenolate mofetil, etanercept, and intravenous immunoglobulin therapy. Symptoms recurred after corticosteroid tapers below 10 mg. She was noted to have an elevated IL-6 level, and tocilizumab was started. She responded favorably with remission of fever, arthritis, and skin manifestations and was able to taper corticosteroid therapy successfully. PMID:22334272

Makol, Ashima; Gibson, Lawrence E; Michet, Clement J

2012-03-01

207

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

208

Circulating anti-neutrophil cytoplasmic autoantibodies in subglottic stenosis: a useful aid in diagnosing vasculitis in this condition?  

PubMed

Five patients with subglottic stenosis, occurring either as a presenting symptom or as a manifestation in the course of a systemic disease, are described. Indirect immunofluorescence revealed the presence of circulating autoantibodies against both cytoplasmic and perinuclear constituents of neutrophils in all five. Antibodies directed against a 29 kDa antigen of the azurophilic granules (two patients), against myeloperoxidase (one patient), and against both the 29 kDa antigen and myeloperoxidase (one patient) were found by enzyme-linked immunosorbent assay. These autoantibodies have previously been found in patients with Wegener's granulomatosis, microscopic polyarteritis, (idiopathic) glomerulonephritis and Churg-Strauss syndrome. However, only one of these five patients fulfilled the criteria for these conditions. Since these autoantibodies are seldom observed in other conditions, and other diseases had been excluded by careful evaluation, we suggest that their presence places subglottic stenosis within the spectrum of necrotizing (granulomatous) vasculitis. Whether immunosuppressive therapy is always warranted in patients with subglottic stenosis and circulating anti-neutrophil cytoplasmic antibodies is a matter of debate. PMID:1946937

Gans, R; de Vries, N; Donker, A J; Goldschmeding, R; Hoorntje, S J

1991-07-01

209

ANCA-positive vasculitis induced by levamisole-adulterated cocaine and nephrotic syndrome: The kidney as an unusual target  

PubMed Central

Patient: Male, 36 Final Diagnosis: Levamisole-induced vasculopathy Symptoms: Purpuric skin lesions Medication: Levamisole Clinical Procedure: — Specialty: Internal Medicine Objective: Unusual clinical course Background: Levamisole has been detected in seized cocaine samples and a levamisole-induced vasculopathy (LIV) has been described, mainly focused on skin. Case Report: A 36-year-old Caucasian man with history of antibodies to hepatitis C infection (negative hepatitis C virus RNA and negative HIV serology), smoking, and intravenous use of cocaine and brown heroin, presented to the hospital with purpuric skin lesions on extremities and earlobes. One month before the current presentation, a skin punch biopsy of one of these lesions was performed, showing histopathologic findings suggestive of mixed cryoglobulinemia. Laboratory testing revealed leukopenia, renal failure, and nephrotic syndrome. Antimyeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) were positive. The previous skin punch biopsy was revised and demonstrated pathologic findings consistent with leukocytoclastic vasculitis. An analysis of a cocaine sample for personal use, provided by the patient, was performed using mass spectrometry-gas chromatography and levamisole was detected. Three boluses of intravenous methylprednisolone were administered, followed by oral prednisone 1 mg/Kg per day. Skin lesions and renal function improved. Conclusions: To our knowledge, this is the first report of nephrotic syndrome induced by levamisole-adulterated cocaine, proven by cocaine sample toxicology. Lack of renal biopsy is a limitation of this report. PMID:24478818

Álvarez Díaz, Hortensia; Mari?o Callejo, Ana Isabel; García Rodríguez, José Francisco; Rodríguez Pazos, Laura; Gómez Buela, Inmaculada; Bermejo Barrera, Ana María

2013-01-01

210

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

211

Eosinophilic/T-cell chorionic vasculitis and chronic villitis involve regulatory T cells and often occur together.  

PubMed

Eosinophilic/T-cell chorionic vasculitis (ETCV) is characterized by mixed T-cell, eosinophilic, and histiocytic infiltrates within the chorionic vessel wall. We sought to better characterize this lesion with respect to other pathologic correlates and the T-cell populations involved. Epidemiologic data and other pathologic diagnoses, including concurrent chronic villitis (CV), were tabulated for each case of ETCV diagnosed at our institution over a 6-year period. CD3, CD25, FOXP3, and dual FOXP3-CD3 immunostains were used to identify regulatory T-cell populations in ETCV and CV. Cells positive for CD3, FOXP3, and CD25 were quantitated by manual counts of ×40 fields at the sites of ETCV and CV, and FOXP3?CD3 and CD25?CD3 ratios were calculated. Digital analysis of ETCV and CV using the dual FOXP3-CD3 immunostain was also performed on select cases. Of 31 ETCV cases, 10 (32%) were accompanied by CV and 13 (42%) by a thrombus in the vessel affected by ETCV. The mean Treg cell marker?CD3 ratios in ETCV ranged from 0.18 to 0.26 by manual count and digital analysis, but the counts did not statistically differ by method. The mean Treg cell marker?CD3 ratios in CV ranged from 0.37 to 0.39 by manual count and 0.19 by digital analysis, but these counts also did not statistically differ by method. Chronic villitis was seen in one-third of ETCV cases. FOXP3+ and CD25+ regulatory T cells represent a significant subpopulation of T cells in ETCV and CV, suggesting that they may play a role in these entities. PMID:23600955

Katzman, Philip J; Oble, Darryl A

2013-01-01

212

Standardised work-up programme for fever of unknown origin and contribution of magnetic resonance imaging for the diagnosis of hidden systemic vasculitis  

PubMed Central

Background: Fever of unknown origin (FUO) is a diagnostic challenge. Rheumatologists are often in charge of patients with FUO because the vasculitides, especially, are potential and common causes of FUO. Objective: To evaluate the value of a standardised investigation to identify the cause of FUO. Methods: A standardised work-up programme for patients with FUO was started at the beginning of September 1999. The rate of identified causes of FUO was compared between all patients with FUO admitted to a tertiary care centre of rheumatology between January 1996 and August 1999 (control group) and September 1999 and January 2003 (work-up group). In January 2002 magnetic resonance imaging (MRI) was added to the investigation. Results: 67 patients with FUO were identified—32 before and 35 after institution of the work-up programme. Before implementation 25% of all patients with FUO remained undiagnosed, after implementation 37%. After institution of the investigation the percentage of patients with vasculitides increased significantly from 6% (n = 2) to 26% (n = 9, p = 0.047, Fisher's exact test). This increase could be attributed to the addition of MRI in 2002. When all patients with FUO before 2002 (n = 55) and thereafter (n = 12) were compared the prevalence of systemic vasculitis increased from 11% (n = 6) to 42% (n = 5, p = 0.021). Conclusion: Implementation of a standardised work-up programme for FUO did not improve the overall rate of diagnosis. Addition of MRI significantly increased the diagnosis of systemic vasculitis as the underlying cause of FUO. MRI should be included in the investigation of patients with FUO when vasculitis is suspected. PMID:15608307

Wagner, A; Andresen, J; Raum, E; Lotz, J; Zeidler, H; Kuipers, J; Jendro, M

2005-01-01

213

Mycobacteria other than Mycobacterium tuberculosis are not present in erythema induratum/nodular vasculitis: a case series and literature review of the clinical and histologic findings.  

PubMed

Erythema induratum (EI)/nodular vasculitis (NV) is characterized by recurrent crops of tender oedematous nodules on the lower legs. A lobular panniculitis with granulomatous inflammation, vasculitis, focal necrosis and septal fibrosis is present. Mycobacterium tuberculosis DNA has been detected in some lesions by means of polymerase chain reaction (PCR). Ten cases of EI/NV were found. H&E slides were reviewed. PCR assays for M. tuberculosis and mycobacteria other than M. tuberculosis (MOTT) were performed. PCR did not reveal M. tuberculosis (0%) or MOTT (0%) DNA, with positive controls, indicating the reliability of the assays. Among the MOTT, cutaneous infections are most commonly caused by M. marinum. Subcutaneous tuberculoid granulomas may be seen with M. kansasii, M. marinum, M. scrofulaceum and M. avium complex. M. gordonae, M. szulgai and M. malmoense rarely cause cutaneous infections. M. simiae, M. gastri and M. asiaticum are probably not cutaneous pathogens. M. tuberculosis and MOTT DNA was not found in EI/NV. EI/NV has diverse aetiologies with varying pathogeneses leading to similar histologic changes. The cases analysed may not have had an infectious aetiology. However, in EI/NV, performance of PCR for MOTT as well as M. tuberculosis complex may still be beneficial, particularly in cases from immunocompromised hosts. PMID:15701084

Bayer-Garner, Ilene B; Cox, Mark D; Scott, Margie A; Smoller, Bruce R

2005-03-01

214

CD8+/perforin+/WC1- gammadelta T cells, CD8+ alphabeta T cells, infiltrate 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) is a fatal disease associated with lymphoproliferation, lymphocytic vasculitis, and mucosal ulceration in clinically susceptible species. SA-MCF is an important threat to American bison (Bison bison) due to th...

215

Epidemiology of systemic vasculitis  

Microsoft Academic Search

The systemic vasculitides are heterogeneous conditions of unknown etiology characterized by inflammation and necrosis of different\\u000a sized blood vessels. Wegener’s Granulomatosis, microscopic polyangiitis, and Churg Strauss syndrome are associated with anti-neutrophil\\u000a cytoplasmic antibodies and affect small and medium blood vessels. They are very rare in childhood and peak in the 65 to 70\\u000a year old age group. Wegener’s Granulomatosis appears

Richard Watts; David G. I. Scott

2005-01-01

216

Types of Vasculitis  

MedlinePLUS

... not always, affect the body’s larger blood vessels. Behçet's Disease Behçet's (beh-CHET'S) disease can cause recurrent, painful ulcers ( ... get it, but it also can affect women. Behçet's disease is more common in people of Mediterranean, ...

217

Systemic Sclerosis Presenting with Simultaneous Retinal Vasculitis in One Eye and Optic Neuritis in the other along with Severe Immune Haemolytic Anaemia  

PubMed Central

Systemic Sclerosis (SSc) is a connective tissue disorder which involves multiple systems in a chronic progressive manner. Micro–angiopathic haemolytic anaemia is a distinguished feature of “scleroderma renal crisis”, which is manifested by severe hypertension, a rapidly progressing renal dysfunction and hyperreninaemia and is seen in patients with an early, diffuse form of the disease. A nervous system involvement is rare, though entrapment neuropathies have been reported. Who presented with a sequential loss of vision in both eyes; due to retinal vasculitis in right eye and optic nerve demyelination in the left eye. She also had severe Coombs’ negative haemolytic anaemia in absence of any renal dysfunction or hypertension. Both the ophthalmologic and the haematologic manifestations are very rare and both responded well to oral prednisolone therapy. PMID:24551698

Moulick, Avijit; Sarkar, Biswanath Sharma; Jana, Anirban; Guha, Pradipta; Das, Anjan

2013-01-01

218

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

219

Induction of remission in active anti?neutrophil cytoplasmic antibody?associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide  

PubMed Central

Background Active anti?neutrophil cytoplasmic antibody?associated vasculitis (AAV) is commonly treated with cyclophosphamide, a drug with serious side effects, and with corticosteroids. Objective To determine the efficacy of a possible alternative drug for cyclophosphamide, oral mycophenolate mofetil (MMF) 1000?mg twice daily and oral prednisolone 1?mg/kg once daily as remission induction treatment. Methods 32 consecutive patients with 34 episodes of active vasculitis who could not be treated with cyclophosphamide were diagnosed for a median (range) of 6.0 (0.3–22)?years and experienced 4 (0–14) relapses prior to the current episode. Treatment response and relapse?free survival were analysed. Results Complete remission (CR) was obtained in 25 (78%) patients, partial remission (PR) in 6 (19%), whereas 1 (3%) patient did not respond. 19 patients relapsed, 13 (52%) after CR, 14 (3–58)?months after starting the treatment and 6 (100%) after PR, 6 (2–10)?months after starting the treatment. The median relapse?free survival was 16?months, comparable with the interval between the previous relapse and the current MMF?treated relapse (17 (3–134)?months). Relapse?free survival at 1, 3, and 5?years was 63%, 38% and 27%, respectively. Patients who had been treated successfully with cyclophosphamide before responded better (CR 84%, relapse 50%) than those who had not (CR 50%, relapse 100%). Minor gastrointestinal side effects and infections occurred frequently. MMF was prematurely discontinued due to adverse effects in two patients. Conclusion MMF, in combination with prednisolone, can induce remission in patients with relapses of AAV intolerant to cyclophosphamide. PMID:17179175

Stassen, Patricia M; Tervaert, Jan Willem Cohen; Stegeman, Coen A

2007-01-01

220

Importance of checking anti-glomerular basement membrane antibody status in patients with anti-neutrophil cytoplasmic antibody-positive vasculitis.  

PubMed

The case is reported of a 68-year-old man with perinuclear anti-neutrophil cytoplasmic antibody (pANCA)-associated glomerulonephritis who developed antibodies to glomerular basement membrane (anti-GBM) resulting in end stage renal failure. His pANCA titre on admission was 1:1024 IgG and he was anti-myeloperoxidase positive. A renal biopsy showed advanced sclerosing necrotising glomerulonephritis consistent with a pauci-immune ANCA-positive glomerulonephritis. He was treated with steroids and cyclophosphamide. His serum creatinine profile improved. He had a relapse of disease 16 months later, which was successfully treated. After a further 16 months, he presented with acute renal failure (creatinine 1060 micromol/l). His pANCA titre on admission was 1:64 IgG. This was treated as a further relapse of ANCA-positive vasculitis. He became oliguric and his haemoglobin concentration fell. Eight days after admission, he was found to be strongly positive for anti-GBM (138 U/ml). Despite receiving cyclophosphamide, steroids and plasma exchange, he remained dialysis-dependent. PMID:18424581

Gallagher, J L; Sinha, S; Reeve, R; Kalra, P A

2008-04-01

221

Use of cytotoxic agents and cyclosporine in the treatment of autoimmune disease. Part 2: Inflammatory bowel disease, systemic vasculitis, and therapeutic toxicity.  

PubMed

When cytotoxic agents were introduced, their ability to disrupt nucleic acid and protein synthesis led to their effective use for the treatment of neoplastic disease. During the course of this use, however, it became apparent that these agents also suppress the immune system. This usually unwelcome effect was subsequently studied and beneficially directed toward the treatment of non-neoplastic diseases in which autoimmune mechanisms were considered important to pathogenesis. As a result of these investigations, cytotoxic agents and, more recently, cyclosporine have emerged to become an important part of the therapeutic regimen for many autoimmune diseases. Nonetheless, these medications may still cause treatment-induced illness or even death. It is therefore particularly important to weigh the benefits and risks of cytotoxic therapy when treating a non-neoplastic disease. This two-part Clinical Staff Conference reviews data on the efficacy and toxicity of cytotoxic drugs and cyclosporine in selected autoimmune diseases. In part 2, we focus on the role of these agents in treating inflammatory bowel disease and systemic vasculitis and review the toxic effects of these agents. PMID:9653000

Langford, C A; Klippel, J H; Balow, J E; James, S P; Sneller, M C

1998-07-01

222

Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear.  

PubMed

Patients with antineutrophil cytoplasm antibody-associated vasculitis (AAV) requiring dialysis at diagnosis are at risk for developing end-stage renal disease (ESRD) or dying. Short-term results of a trial comparing plasma exchange (PLEX) to intravenous methylprednisolone (IV MeP) suggested PLEX improved renal recovery. Here we conducted long-term follow-up to see if this trend persisted. A total of 137 patients with newly diagnosed AAV and a serum creatinine over 500 ?mol/l or requiring dialysis were randomized such that 69 received PLEX and 68 received IV MeP in addition to cyclophosphamide and oral glucocorticoids. The patients were followed for a median of 3.95 years. In each group there were 35 deaths, while 23 PLEX and 33 IV MeP patients developed ESRD. The hazard ratio for PLEX compared to IV MeP for the primary composite outcome of death or ESRD was 0.81 (95% confidence interval 0.53-1.23). The hazard ratio for all-cause death was 1.08 with a subhazard ratio for ESRD of 0.64 (95% confidence interval 0.40-1.05). Thus, although short-term results with PLEX are encouraging, the long-term benefits remain unclear. Further research is required to determine the role of PLEX in AAV. Given the poor outcomes of patients with severe AAV, improved treatment is urgently needed. PMID:23615499

Walsh, Michael; Casian, Alina; Flossmann, Oliver; Westman, Kerstin; Höglund, Peter; Pusey, Charles; Jayne, David R W

2013-08-01

223

An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy.  

PubMed

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old boy, with abdominal distention, pretibial edema, arthritis and petechia on bilateral ankles. He had deteriorated renal functions (creatinine 1.65 mg/dl), hypoalbuminemia (1.6 g/dl) and hypocomplementemia with nephrotic range proteinuria and hematuria. He developed pleural effusion and peritonitis. Serum ANA, anti dsDNA, p ANCA, c ANCA, anticardiolipin IgM and IgG titers were negative. A renal biopsy was performed which revealed diffuse proliferative glomerulonephritis with full-house staining pattern in immunofluorescent microscopic examination suggesting Class IV Lupus Nephritis. He was administered a total of six courses of monthly intravenous pulse methyl prednisolone, dipyridamole, oral cyclophosphamide followed by azothiopirine and oral prednisolone therapy. The renal functions and serum albumin levels turned normal but peritonitis persisted and disappeared after the third pulse steroid therapy. In conclusion, we presented this patient to remind the possibility of SLE in such seronegative patients with unusual findings in order to avoid the delay in the management of this disease with high mortality and morbidity if not treated. Full-house nephropathy is an important clue especially for the diagnosis of ANA negative SLE. PMID:20532511

Caltik, Aysun; Demircin, Gülay; Bülbül, Mehmet; Erdogan, Ozlem; Akyüz, Sare G; Arda, Nilüfer

2013-01-01

224

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

PubMed Central

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

2013-01-01

225

The effects of duration of glucocorticoid therapy on relapse rate in anti-neutrophil cytoplasm antibody associated vasculitis: A meta-analysis  

PubMed Central

Objective Disease relapses are common for patients with anti-neutrophil cytoplasm antibody associated vasculitis (AAV). The role of low-dose glucocorticoids (GC) in relapse prevention is controversial. We undertook a systematic review and meta-analysis to determine if GC target doses influence relapses of AAV. Methods Medline, EMBASE and Cochrane databases were searched for observational studies and randomized controlled trials of treatment of AAV that included a predefined GC treatment plan. The association of GC target dose with the proportion of relapses in studies was assessed using meta-regression and multi-level generalized linear modeling. Results Thirteen studies (983 patients) were identified for inclusion. There were no studies directly comparing GC regimens. We classified 288 patients as having a non-zero GC target dose by study end and 695 patients as having a zero GC target dose by study end. The pooled proportion of patients with a relapse was 36% (95% confidence interval [CI] 25 to 47%). GC regimen was the most significant variable explaining the variability between the proportions of patients with relapses. The proportion of patients with a relapse was 14% (95% CI 10 to 19%) in non-zero GC target dose and 43% (95% CI 33 to 52%) in zero GC target dose studies. Differences other than GC regimens exist between studies that complicate the comparability of trials and isolation of the variability in relapses due to GC target alone. Conclusions Studies with longer courses of GC in AAV are associated with fewer relapses. These results have implications for study design and outcome assessment in clinical trials of AAV. PMID:20235186

Walsh, Michael; Merkel, Peter A.; Mahr, Alfred; Jayne, David

2010-01-01

226

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

227

New horizons in renal vasculitis  

Microsoft Academic Search

Summary After a long period during which few new data on the vasculitides emerged, the past decade has seen a real explosion of data on the subject. This began with basic clinical descriptions and the deliniation of long term outcome as survival improved to a major extent under the influence of improved treatment regimes. In parallel, better understanding of the

J. S. Cameron

1991-01-01

228

Comparison of a novel chemiluminescence enzyme immunoassay (CLEIA) with enzyme-linked immunosorbent assay (ELISA) for the determination of MPO-ANCA in patients with ANCA-associated vasculitis.  

PubMed

Background. Myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA) represents the serological hallmark of ANCA-associated vasculitis (AAV). We evaluated the analytical and diagnostic accuracy of chemiluminescence enzyme immunoassay (CLEIA) versus enzyme-linked immunosorbent assay (ELISA) for the detection of MPO-ANCA. Methods. A total of 242 sera obtained from 51 patients with AAV and 103 patients without AAV were tested for MPO-ANCA by ELISA (NephroScholor MPOANC II) and CLEIA (the STACIA MEBLux test). Disease activity in the patients with AAV was determined based on the Birmingham Vasculitis Activity Score. We analyzed the correlations between the MPO-ANCA titers determined by the CLEIA and those determined by the ELISA, and also between the MPO-ANCA titers and the disease activity. Results. The MPO-ANCA titers determined by the CLEIA (x) were strongly correlated with those determined by the ELISA (y). The correlation could be expressed by the following equation in this study: y = 1.8x + 7.7 (r = 0.96; p < 0.0001). At the cutoff value of 3.5 U/ml, the CLEIA yielded positive test results for MPO-ANCA in 73 of the 242 sera (30.2%), while at the cutoff value of 20 U/ml, ELISA yielded positive test results in 57 of the 242 sera (23.6%). The CLEIA yielded false-positive test results in 4 of the 120 sera obtained from the non-AAV patients (3.3%), whereas the ELISA yielded a false-positive result in only 1 of the 120 sera obtained from the non-AAV patients (0.8%). The sensitivity and specificity of the CLEIA for the diagnosis of AAV were 100% and 96.7%, respectively, while those of the ELISA were 94.3% and 99.2%, respectively. The sensitivity and specificity of the CLEIA for the prediction of active disease were 100% and 64.4%, respectively, while those of the ELISA were 94.3% and 73.6%, respectively. Conclusion. The false positivity rate of the CLEIA for MPO-ANCA tended to be high as compared with that of the ELISA. Also, according to the correlation coefficient between the results of the CLEIA and the ELISA calculated in this study, it is necessary to pay attention to the differences in the sensitivity and specificity between CLEIA and ELISA. PMID:25388618

Hirose, Orie; Itabashi, Mitsuyo; Takei, Takashi; Nitta, Kosaku

2014-11-12

229

The immunopathology of ANCA-associated vasculitis.  

PubMed

The small-vessel vasculitides are a group of disorders characterised by variable patterns of small blood vessel inflammation producing a markedly heterogeneous clinical phenotype. While any vessel in any organ may be involved, distinct but often overlapping sets of clinical features have allowed the description of three subtypes associated with the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA), namely granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (eGPA, formerly known as Churg-Strauss syndrome). Together, these conditions are called the ANCA-associated vasculitidies (AAV). Both formal nomenclature and classification criteria for the syndromes have changed repeatedly since their description over 100 years ago and may conceivably do so again following recent reports showing distinct genetic associations of patients with detectable ANCA of distinct specificities. ANCA are not only useful in classifying the syndromes but substantial evidence implicates them in driving disease pathogenesis although the mechanism by which they develop and tolerance is broken remains controversial. Advances in our understanding of the pathogenesis of the syndromes have been accompanied by some progress in treatment, although much remains to be done to improve the chronic morbidity associated with the immunosuppression required for disease control. PMID:25056155

McKinney, Eoin F; Willcocks, Lisa C; Broecker, Verena; Smith, Kenneth G C

2014-07-01

230

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

231

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...

232

Granuloma formation, implications for the pathogenesis of vasculitis.  

PubMed

The pathogenesis of granulomatous inflammation is complex and involves a variety of mechanisms acting in concert to bring about an inflammatory lesion that is able to contain and destroy intracellular pathogens. While this process is crucial to host defense, it is also a two-edged sword in that excessive or inappropriate granulomatous inflammation results in considerable damage to normal tissue. In recent years, there has been significant progress in dissecting the immunologic events involved in granuloma formation and maintenance. A better understanding of these events will allow us to more precisely modulate the granulomatous inflammatory response to the benefit of patients with both infectious and autoimmune diseases. PMID:12086263

Sneller, Michael C

2002-01-01

233

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

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

2014-12-01

234

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

235

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

236

Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, diagnosis, and treatment.  

PubMed

This article provides an update on the diagnosis and management of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (formerly Wegener), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Focus is on new schemes of classification and the importance of ANCAs in the diagnosis and prognosis of these systemic vasculitides. Current therapeutic strategies consisting of glucocorticoids in conjunction with conventional or biologic agents for both induction of remission and remission maintenance are outlined. Future research directions include investigation of the optimal duration and frequency of maintenance therapy and development of targeted therapeutic agents. PMID:25399936

Lally, Lindsay; Spiera, Robert

2015-01-01

237

Central nervous system vasculitis and polyneuropathy as first manifestations of hepatitis C  

PubMed Central

Sensory or motor peripheral neuropathy may be observed in a significant proportion of hepatitis C virus (HCV)-infected patients. However, central nervous system (CNS) involvement is uncommon, especially in cryoglobulin-negative subjects. We describe a case of peripheral neuropathy combined with an ischemic CNS event as primary manifestations of chronic HCV infection without cryoglobulinemia. Significant improvement was observed after antiviral therapy. We discuss the spectrum of neurological manifestations of HCV infection and review the literature. PMID:22253526

Carvalho-Filho, Roberto J; Narciso-Schiavon, Janaína Luz; Tolentino, Luciano HL; Schiavon, Leonardo L; Ferraz, Maria Lucia G; Silva, Antonio Eduardo B

2012-01-01

238

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

E-print Network

be excluded from these latter diseases, based on laboratory findings. Ischemic manifestations in the aortic. Moadel K, Yannuzzi LA, Ho AC, Ursekar A. Retinal vascu- lar occlusive disease in a child, Albert DM, Solt LC, Hu DN, Wang WJ. Neurofi- bromatosis of the eyelid and orbit. Int Ophthalmol Clin 1982

Palczewski, Krzysztof

239

Digital vasculitis in a patient with rheumatoid arthritis responded well to adalimumab.  

PubMed

42-year-old old female patient, followed up with diagnosis of rheumatoid arthritis for 15 years, was admitted with necrotising ulcer of left hand 1st and 2nd fingertips and pain, swelling, limitation of movement, and morning stiffness at bilateral wrist, and metacarpophalangeal and proximal interphalangeal joints. Laboratory tests revealed elevated acute phase reactants. Radial and ulnar arteries were clear in upper extremity Doppler ultrasound. The patient was diagnosed as RA activation and digital ulcer and administered iloprost infusion for five days and 1?mg/kg corticosteroid and 20?mg/week methotrexate (MTX). After one month, a partial regression of clinical and laboratory findings was observed. However, 6 months later, due to relapsed and increased complaints and findings, adalimumab 40?mg was administered. Two months later, clinical and laboratory findings apparently decreased. PMID:25133007

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

2014-01-01

240

Digital Vasculitis in a Patient with Rheumatoid Arthritis Responded Well to Adalimumab  

PubMed Central

42-year-old old female patient, followed up with diagnosis of rheumatoid arthritis for 15 years, was admitted with necrotising ulcer of left hand 1st and 2nd fingertips and pain, swelling, limitation of movement, and morning stiffness at bilateral wrist, and metacarpophalangeal and proximal interphalangeal joints. Laboratory tests revealed elevated acute phase reactants. Radial and ulnar arteries were clear in upper extremity Doppler ultrasound. The patient was diagnosed as RA activation and digital ulcer and administered iloprost infusion for five days and 1?mg/kg corticosteroid and 20?mg/week methotrexate (MTX). After one month, a partial regression of clinical and laboratory findings was observed. However, 6 months later, due to relapsed and increased complaints and findings, adalimumab 40?mg was administered. Two months later, clinical and laboratory findings apparently decreased. PMID:25133007

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

2014-01-01

241

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.

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

242

Clinical review: Vasculitis on the intensive care unit – part 1: diagnosis  

PubMed Central

The first part of this review addresses the diagnosis and differential diagnosis of the primary vasculitides Wegener's granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and polyarteritis nodosa. Prompt diagnosis and treatment of these conditions ensures an optimal prognosis. The development of assays for antineutrophil cytoplasmic antibodies has aided the diagnosis of Wegener's granulomatosis and microscopic polyangiitis. However, even in cases where there is high clinical likelihood that these conditions are present, up to 20% may be antibody negative, whereas alternative diagnoses may be antibody positive. The final diagnosis rests on a balance of clinical, laboratory, radiological and histological features. The exclusion of alternative diagnoses is important in assuring appropriate therapy. Particular attention is paid to the more fulminant presentations of these conditions and the role of the critical care physician in their diagnosis and management. PMID:15693990

Semple, David; Keogh, James; Forni, Luigi; Venn, Richard

2005-01-01

243

The Association between Silica Exposure and Development of ANCA-Associated Vasculitis: Systematic Review and Meta-analysis  

PubMed Central

Background Crystalline silica is among the environmental exposures associated with increased risk of autoimmune diseases, including rheumatoid arthritis, systemic sclerosis and systemic lupus erythematosus. Silica exposure has also been related to the development of ANCA-associated vasculitides (AAV), but past studies appear to conflict as to the presence and magnitude of the associated risks of disease. We aimed to conduct a systematic review of the existing studies and meta-analysis of their results. Methods We searched EMBASE, MEDLINE and international scientific conference abstract databases for studies examining the association of silica exposure with AAV. Studies in English, French, or Spanish were included and those examining the association of silica with ANCA-positivity alone were excluded. We assessed study quality using the Newcastle-Ottawa scale. We meta-analyzed the results using random effects models and tested for heterogeneity. We performed sensitivity and subgroup analyses, examining studies that adjusted for smoking and occupational risk factors as well as studies that analyzed by subtypes of AAV. Results We identified 158 potential manuscripts and 3 abstracts related to silica exposure and risk of AAV. 147 were excluded after abstract review and 14 underwent detailed evaluation of full manuscript/abstract. After further application of exclusion criteria, 6 studies (all case-controls) remained. The studies had moderate heterogeneity in selection of cases and controls, exposure assessment, disease definition and controlling for potential confounders. We found an overall significant summary effect estimate of silica “ever exposure” with development of AAV (summary OR 2.56, 95% CI 1.51- 4.36), with moderate heterogeneity (I2=48.40%). ORs were similar for studies examining only MPA (OR 3.95, CI 95% 1.89-8.24), compared to those only studying GPA (OR 3.56, CI 95% 1.85-6.82). Conclusion Despite moderate heterogeneity among studies, the totality of the evidence after meta-analysis points to an association between silica exposure and risk for developing AAV. PMID:23820041

Gómez-Puerta, Jose A.; Gedmintas, Lydia; Costenbader, Karen H.

2014-01-01

244

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

245

Translational Mini-Review Series on Immunology of Vascular Disease: Mechanisms of vascular inflammation and remodelling in systemic vasculitis  

PubMed Central

Vessel walls are the primary inflammatory sites in systemic vasculitides. In most cases the initiating event is unknown, and a self-sustaining circuit attracts and activates inflammatory leucocytes in the wall of vessels of various size and anatomical characteristics. Recent studies have revealed homeostatic roles of vascular inflammation and have identified the action of humoral innate immunity, in particular injury-associated signals and acute phase proteins, on the activation of circulating leucocytes, platelets and endothelial cells. These advances have provided clues to the molecular mechanisms underlying the vicious circle that maintains and amplifies vessel and tissue injury. PMID:19309348

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

2009-01-01

246

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

PubMed Central

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

2013-01-01

247

High Basal Activity of the PTPN22 Gain-of-Function Variant Blunts Leukocyte Responsiveness Negatively Affecting IL10 Production in ANCA Vasculitis  

Microsoft Academic Search

Consequences of expression of the protein tyrosine phosphatase nonreceptor 22 (PTPN22) gain-of-function variant were evaluated in leukocytes from patients with anti-neutrophil cytoplasmic autoantibody (ANCA) disease. The frequency of the gain-of-function allele within the Caucasian patient cohort was 22% (OR 1.45), compared to general American Caucasian population (16.5%, p = 0.03). Examination of the basal phosphatase activity of PTPN22 gain-of-function protein

Yali Cao; Jiajin Yang; Kerry Colby; Susan L. Hogan; Yichun Hu; Caroline E. Jennette; Elisabeth A. Berg; Youkang Zhang; J. Charles Jennette; Ronald J. Falk; Gloria A. Preston

2012-01-01

248

Henoch-Schonlein purpura  

MedlinePLUS

Anaphylactoid purpura; Vascular purpura; Leukocytoclastic vasculitis ... Ardoin SP, Fels E. Vasculitis syndromes. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics . 19th ed. Philadelphia, Pa: Saunders ...

249

Genetics Home Reference: Adenosine deaminase 2 deficiency  

MedlinePLUS

... inflammation of various tissues, particularly the blood vessels (vasculitis). Signs and symptoms can begin anytime from early ... inflammation of blood vessels throughout the body (systemic vasculitis). However, not all researchers classify ADA2 deficiency as ...

250

Granulomatosis with Polyangiitis (Wegener's)  

MedlinePLUS

... treatment are essential. Recurrences of this form of vasculitis are common. What is Granulomatosis with Polyangiitis (Wegener's)? ... group of diseases characterized by blood vessel inflammation (vasculitis). In GPA, organ damage occurs as a result ...

251

Cryoglobulinemia  

MedlinePLUS

... inflammation of the blood vessels throughout the body (vasculitis). There are three main types of the disorder. ... 193. Stone JH. Immune Complex-Mediated Small Vessel Vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et ...

252

The health resource utilization and economic burden of systemic autoimmune rheumatic diseases.  

E-print Network

??Background: SARDs (Systemic Autoimmune Rheumatic Diseases) are a group of rare, chronic conditions (systemic vasculitis, systemic lupus erythematosus, scleroderma, Sjogren's disease, and poly/dermatomyositis) associated with… (more)

McCormick, Natalie

2012-01-01

253

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

254

Isolated Aortitis  

MedlinePLUS

... diseases associated with aortitis include rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus, sarcoidosis, Behçet disease, relapsing polychondritis, ANCA associated vasculitis and Cogan syndrome. Infectious causes of aortitis include ...

255

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

256

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)

2014-07-29

257

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

258

A rare presentation of seizures in a not-so-rare disease: Henoch-Schönlein purpura presenting with repeated seizures.  

PubMed

Henoch-Schönlein purpura represents the most common form of systemic vasculitis in children. Although a very common cause of vasculitis, seizures are a very rare complication of this disorder. We report a 5-year-old boy who presents with no other clinical symptoms of the disorder other than a seizure. By presenting this case, we hope to expand the differential diagnosis of repeated seizures to include diseases in which the pathogenesis of diseases with small vessel vasculitis such as Henoch-Schönlein purpura is considered. PMID:24892684

Camacho, Christina; Leva, Ernest G

2014-06-01

259

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

260

Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)  

MedlinePLUS

You are here: Home / Types of Vasculitis / Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) First Description Who gets ...

261

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

262

Undifferentiated Connective Tissue Disease  

MedlinePLUS

... vessels. Examples of connective tissue diseases include lupus , scleroderma , rheumatoid arthritis , Sjögren's syndrome , myositis , and vasculitis . There ... connective tissue diseases, such as lupus, Sjögren's or scleroderma. More UCTD Information Causes Diagnosis Symptoms Treatment Print ...

263

THE VASCULAR LESIONS OF A COW AND BISON WITH SHEEP-ASSOCIATED MALIGNANT CATARRHAL FEVER CONTAIN OVINE HERPESVIRUS 2-INFECTED CD8+ T LYMPHOCYTES  

Technology Transfer Automated Retrieval System (TEKTRAN)

Malignant catarrhal fever (MCF) is a serious herpesviral disease syndrome of certain ruminant species. The microscopic pathology of MCF is characterized by lymphoid proliferation and infiltration, necrotizing vasculitis, and necrosis of lymphoid and epithelial tissues. Previous attempts to detect vi...

264

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

265

ESR  

MedlinePLUS

... fibrinogen levels in the blood) Macroglobulinemia - primary Necrotizing vasculitis Polymyalgia rheumatica An increased ESR rate may be due to some infections, including: Body-wide (systemic) infection Bone infections Infection of the heart or ...

266

Coughing up blood  

MedlinePLUS

... Inflammation of the blood vessels in the lung (vasculitis) Injury to the arteries of the lungs Irritation of the throat from violent coughing (small amounts of blood) Pneumonia or other lung infections Pulmonary edema Systemic lupus erythematosus Tuberculosis

267

Fingers that change color  

MedlinePLUS

... inflammation of small blood vessels Cryoglobulinemia Frostbite Necrotizing vasculitis Peripheral artery disease Raynaud's phenomenon - sudden change in the finger color ranges from pale to red to blue Scleroderma Systemic lupus erythematosus

268

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 ...

269

Cyclophosphamide (Cytoxan)  

MedlinePLUS

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

270

Sequential strokes in a hyperacute stroke unit  

PubMed Central

Vasculitis is a rare, but treatable condition that can present to hyperacute stroke units. Thrombolysis does not treat the underlying pathology, and a rapidly evolving clinical picture drives clinical decision often before all the investigation results are available. PMID:25289143

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

2014-01-01

271

Sequential strokes in a hyperacute stroke unit.  

PubMed

Vasculitis is a rare, but treatable condition that can present to hyperacute stroke units. Thrombolysis does not treat the underlying pathology, and a rapidly evolving clinical picture drives clinical decision often before all the investigation results are available. PMID:25289143

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

2014-08-01

272

Granulomatosis with Polyangiitis (Wegener's)  

MedlinePLUS

... vasculitis are common. What is Granulomatosis with Polyangiitis (Wegener's)? Most commonly, GPA affects the sinuses, lungs and ... in many instances. What causes Granulomatosis with Polyangiitis (Wegener's)? The cause of GPA is unknown. Who gets ...

273

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

274

Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease.  

PubMed

Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium-sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium-sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14-year-old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis-related PAN facilitates early diagnosis, and instigation of appropriate treatment. PMID:24730628

Ba?aran, Özge; Çakar, Nilgün; Gür, Gökçe; Kocaba?, Abdullah; Gülhan, Belgin; Çayc?, Fatma ?emsa; Çelikel, Banu Acar

2014-04-01

275

[Acute scrotum: unusual presentation of Schönlein-Henoch purpura].  

PubMed

Being a systemic vasculitis, Schöenlein-Henoch purpura may affect the scrotum and its content. According to the series studied, this occurs in about 10% patients (2-38%) and sometimes requires a differential diagnosis with the spermatic cord torsion. An isotopic study with 99Tc may avoid a surgical procedure quite often unnecessary. In the case reported here, vasculitis presented as an acute scrotum, which is highly infrequent in the literature and makes a correct presumption diagnosis extraordinary difficult. PMID:9182455

Suarez González, J A; Pello Fonseca, J M; Rivas del Fresno, M; Cuervo Calvo, F J; Castańo González-Coto, D; Alonso Ordońez, M A; Herrero Alvarez, M C; Muruamendiaraz Fernández, V

1997-01-01

276

A 34 Year Old Man with Purple Discoloration and Paresthesia  

PubMed Central

Secondary systemic vasculitis and nonbacterial endocarditis are rare events. We report a case presented with different manifestations of underlying malignancy such as systemic vasculitis, non bacterial endocarditis and DIC (disseminated intravascular coagulopathy). Efforts to find the source of malignancy was unsuccessful and due to patient's unwillingness for further evaluation, finally under the diagnosis of metastatic disease of unknown primary, patient is receiving cyclic chemotherapy. PMID:24505550

Mahmoodian, Reihaneh; Haghighi, Anoosheh; Vakili, Masoud; Shahriari-Ahmadi, Ali; Hajsadeghi, Shokoufeh; Arabi, Mohsen

2014-01-01

277

A 34 year old man with purple discoloration and paresthesia.  

PubMed

Secondary systemic vasculitis and nonbacterial endocarditis are rare events. We report a case presented with different manifestations of underlying malignancy such as systemic vasculitis, non bacterial endocarditis and DIC (disseminated intravascular coagulopathy). Efforts to find the source of malignancy was unsuccessful and due to patient's unwillingness for further evaluation, finally under the diagnosis of metastatic disease of unknown primary, patient is receiving cyclic chemotherapy. PMID:24505550

Iranpour, Aida; Mahmoodian, Reihaneh; Haghighi, Anoosheh; Vakili, Masoud; Shahriari-Ahmadi, Ali; Hajsadeghi, Shokoufeh; Arabi, Mohsen

2014-01-01

278

Livedoid Vasculopathy and Mononeuritis Multiplex, with a Fulminant Hepatic Failure which was caused by Herpes Simplex Hepatitis: A Case Report  

PubMed Central

Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis. PMID:23814745

Pai B, Sathish; Pai, Kanthilatha

2013-01-01

279

Livedoid Vasculopathy and Mononeuritis Multiplex, with a Fulminant Hepatic Failure which was caused by Herpes Simplex Hepatitis: A Case Report.  

PubMed

Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis. PMID:23814745

Pai B, Sathish; Pai, Kanthilatha

2013-05-01

280

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

281

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

PubMed Central

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

2014-01-01

282

Current concept and epidemiology of systemic vasculitides.  

PubMed

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

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

2014-12-01

283

Controversies in ANCA testing.  

PubMed

Antineutrophil cytoplasmic antibody (ANCA) detection is a well-known tool for diagnosing small-vessel vasculitis. Its diagnostic utility, however, depends on the methodologic accuracy of the test and the appropriate ordering of testing in the right clinical setting. While ANCA testing is of proven value, the utility of serial ANCA testing is not entirely clear. Correlation of ANCA levels with disease activity and predicted relapse remains unconfirmed. The best gauge of the predictive value of serial testing is to perform long-term serial testing for some individual patients in order to establish a relationship between ANCA level and clinical disease manifestation over time. ANCA-antigen specificity can be used to assess prognosis in patients with ANCA-associated vasculitis. Proteinase 3-ANCA is associated with higher mortality, higher relapse rate, and faster renal deterioration compared with myeloperoxidase-ANCA. Overall, ANCA is an important diagnostic and prognostic marker for small-vessel vasculitis and warrants further investigation. PMID:23203649

Specks, Ulrich

2012-11-01

284

Eritema elevatum diutinum as a differential diagnosis of rheumatic diseases: case report.  

PubMed

Erythema elevatum diutinum is a chronic and rare cutaneous leukocytoclastic vasculitis, characterized by red, purple and yellow papules, plaques and nodules, distributed symmetrically on the extensor surfaces of the limbs. It is associated with several autoimmune, neoplastic and infectious processes, mainly hematological malignancies in about 30% of the cases. Joint pain and arthritis are frequent symptoms, affecting approximately 40% of the patients, indicating the need for its inclusion in the differential diagnosis of rheumatic diseases, chiefly the other presentations of leukocytoclastic vasculitis, which are characterized by the combination of rheumatic manifestations and peculiar cutaneous lesions. We report the case of an 18-year-old female patient who developed erythema elevatum diutinum and whose diagnosis was based on the morphologic characteristics, the distribution pattern of the cutaneous lesions and the histopathological findings of leukocytoclastic vasculitis. The major systemic symptom was severe arthritis. PMID:22460417

Rosa, Dário Júnior de Freitas; Machado, Ronaldo Figueiredo; Fraga, Rafael de Oliveira; Scotton, Antônio Scafuto; de Souza, Viviane Angelina; Fernandes, Bruno

2012-01-01

285

Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge  

PubMed Central

Takayasu Arteritis (TA) is a rare, debilitating large vessel vasculitis occurring in patients of all ages, including infants, but the disease most commonly presents in the third decade. Diagnosis is often delayed and consequently TA is associated with significant morbidity and mortality. Accurate methods of monitoring disease activity or damage are lacking and currently rely on a combination of clinical features, blood inflammatory markers, and imaging modalities. In this report we describe a case of a 14-year-old boy with childhood-onset TA who, despite extensive negative investigations, did indeed have on-going active large vessel vasculitis with fatal outcome. Postmortem analysis demonstrated more extensive and active disease than originally identified. This report illustrates and discusses the limitations of current modalities for the detection and monitoring of disease activity and damage in large vessel vasculitis. Clinicians must be aware of these limitations and challenges if we are to strive for better outcomes in TA. PMID:24511407

Watson, L.; Brogan, P.; Peart, I.; Landes, C.; Barnes, N.; Cleary, G.

2014-01-01

286

Cavitary pulmonary nodules with systemic lupus erythematosus: differential diagnosis.  

PubMed

Cavitary pulmonary nodules rarely occur in patients with systemic lupus erythematosus and the lupuslike syndrome associated with mixed connective tissue disease. Although it has been suggested that such cavitary nodules represent areas of vasculitis and ischemic necrosis, five of seven occurrences of cavitary nodules in a series of six patients with systemic lupus erythematosus or mixed connective tissue disease proved to be the result of infection or pulmonary embolism. The causes in the other two cases are unknown, but no attempt was made to obtain a biopsy in either instance. It was concluded that vasculitis with ischemic necrosis is not a common cause of cavitary pulmonary nodules in patients with lupus. PMID:6779577

Webb, W R; Gamsu, G

1981-01-01

287

[Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].  

PubMed

Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), is a rare necrotizing vasculitis of small-sized vessels, associated to antimyeloperoxydase ANCA in 40% of patients. EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present, prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present. PMID:22921086

Guillevin, Loďc

2012-10-01

288

Livedoid vasculiltis associated with a double heterozygous Factor V Leiden and prothrombin G20210A gene mutations.  

PubMed

Livedoid vasculitis (LV) may be an isolated condition or associated with an underlying systemic disease, including coagulation disorders, periarteritis nodosa and systemic autoimmune diseases. The classic histological features of LV (fibrin deposition on dermal vessels and the absence of important perivascular infiltrate or leucocytoclasia) argue against a primary vasculitis and is more in keeping with a thrombotic process. Factor V Leiden mutations have rarely been reported in association with LV. We report a patient with LV, who had doubly inherited thrombophilia, with heterozygous mutations in the Factor V Leiden (G1691A) and prothrombin (G20210A) genes. PMID:19793098

Khenifer, S; Thomas, L; Balme, B; Dalle, S

2009-12-01

289

[Skin necrosis: report of eleven cases].  

PubMed

Skin necrosis must be considered as a syndrome, because it is a clinical manifestation of different diseases. An early diagnosis is very important to choose the appropriate treatment. Therefore, its causes should be suspected and confirmed quickly. We report eleven patients with skin necrosis seen at our Department, caused by different etiologies: Warfarin-induced skin necrosis, loxoscelism, diabetic microangiopathy, ecthyma gangrenosum, disseminated intravascular coagulation, necrotizing vasculitis, paraneoplastic extensive necrotizing vasculitis, livedoid vasculopathy, necrotizing fasciitis, necrosis secondary to the use of vasoactive drugs and necrosis secondary to the use of cocaine. We also report the results of our literature review on the subject. PMID:24861125

Molgó, Montserrat N; Arriagada, Camila E; Salomone, Claudia B; Vera, Cristián K; Giesen, Laura F; Solar, Antonieta G; González, Sergio B

2014-01-01

290

The first case of Henoch-Schonlein purpura associated with rosuvastatin: colonic involvement coexisting with small intestine.  

PubMed

Henoch-Schönlein purpura (HSP) is a systemic vasculitis affecting small vessels. It is the most common systemic vasculitis in children, and is rare in adults. Serious gastrointestinal complications are more common in childhood. Infections and drugs are the most prominent factors in the aetiology. Wall thickening in segments of the small intestine is commonly seen in imaging studies in gastrointestinal system (GIS) involvement. Simultaneous involvement of small intestine and colon is rare. An HSP case involving small intestine and colon in an adult patient due to the use of rosuvastatin, an antihyperlipidaemic agent, is presented, and is first of its kind reported in the literature. PMID:24648473

Gonen, Korcan Aysun; Erfan, Gamze; Oznur, Meltem; Erdogan, Cuneyt

2014-01-01

291

The diagnosis and classification of Henoch-Schönlein purpura: an updated review.  

PubMed

Henoch-Schönlein purpura (HSP) is a common childhood systemic vasculitis with clinical characteristics of cutaneous palpable purpura, arthralgia/arthritis, bowel angina, and hematuria/proteinuria. HSP is identified mainly based on the above presentations. Combined with pathohistological findings of leukocytoclastic vasculitis (LCV) and IgA-immune deposits in vessel walls and/or glomeruli increase the diagnostic sensitivity and specificity. However, considering the accessibility of biopsy and some patients with atypical presentations, there are still medical unmet needs in HSP diagnosis. This article reviews the diagnosis of HSP including the aspects of classification criteria, differential diagnosis, and some laboratory findings as the biomarkers with diagnostic potential. PMID:24424188

Yang, Yao-Hsu; Yu, Hsin-Hui; Chiang, Bor-Luen

2014-01-01

292

Superior mesenteric artery syndrome and intra-abdominal compartment syndrome in systemic lupus erythematosus.  

PubMed

Gastrointestinal manifestations of systemic lupus erythematosus (SLE) are common, occurring in about 50% of cases. They are usually mild, in the form of mouth ulcers, nausea, heartburn and mild abdominal pain, but they can be severe in cases of gastrointestinal vasculitis. In this report we describe an unusual combination of SLE complications, namely superior mesenteric artery syndrome (SMAS) and reversible acute obstructive renal failure. This was attributed to raised intra-abdominal pressure and hence intra-abdominal compartment syndrome (IACS) following weight loss secondary to an acute presentation of SLE with gastrointestinal vasculitis. PMID:24335010

Bedaiwi, M; Alkubeyyer, M A; Al Arfaj, A S

2014-02-01

293

Pancreatic mass as an initial presentation of severe Wegener's granulomatosis  

PubMed Central

Acute pancreatitis or a pancreatic mass is a very rare initial presentation of Wegener's granu-lomatosis. A 62-year-old woman presented with tumor-like pancreatitis and otitis media Abdominal ultrasound and magnetic resonance suggested the presence of pancreatic tumor. Ultrasound-guided fine needle aspiration was negative. Distal pancreatic resection and splenectomy were performed and histopathology proved Wegener's vasculitis of the pancreas and spleen. Azathioprine and steroids were subsequently started and six months later the patient was asymptomatic. Involvement of the pancreas could be considered as a presenting symptom of Wegener's vasculitis. PMID:24714250

Valerieva, Yana; Golemanov, Branimir; Tzolova, Nadezhda; Mitova, Rumiana

2013-01-01

294

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

2014-06-01

295

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

296

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

297

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

Microsoft Academic Search

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

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

1995-01-01

298

Robert Timmons McCluskey (19232006)  

E-print Network

McCluskey, and his mother, Lora M. Timmons, met in France during World War I. Charles was a civil for World War II. Summer classes, reduced vacations, and enrollment in the Navy's ROTC were the orders. Perhaps this was the seed of Mac's later focus on vasculitis. At NYU he rose quickly through the academic

Mootha, Vamsi K.

299

Lupus 101Lupus 101 Division of Allergy, Immunology & Rheumatology  

E-print Network

Lupus 101Lupus 101 Division of Allergy, Immunology & Rheumatology University of Rochester Medical myositis Multiple sclerosis Vasculitis Myasthenia gravis Lupus is a SystemicLupus is a Systemic Autoimmune DiseaseAutoimmune Disease #12;Systemic Lupus Erythematosus · Inflammatory multisystem disease primarily

Goldman, Steven A.

300

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

301

Non-Lethal Midline Granuloma of the Nose  

Microsoft Academic Search

Five cases of non-lethal midline granuloma of the nose are reported. Although the histopathological picture in all cases was identical, showing pleomorphic cellular infiltration, scattered areas of necrosis and vasculitis, the clinical picture and course of the disease were completely different from lethal midline granuloma. All the patients were in good general condition and had lived for quite long periods

A. M. Talaat; A. M. Bassiouny; M. K. Kutty

1982-01-01

302

[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

303

Dramatic decrease of circulating levels of monocyte chemoattractant protein-1 in Kawasaki disease after gamma globulin treatment  

Microsoft Academic Search

Kawasaki disease (KD) is a systemic vasculitis preferentially affecting coronary arter- ies. Extensive monocytes\\/macrophages infiltrate in the vascular lesions, implying the involvement of a chemotactic cytokine in their recruitment. We investigated the role of monocyte chemoattractant protein-1 (MCP-1, also termed monocyte chemotac- tic and activating factor) in KD. In the immunohis- tochemical studies using the cardiac tissues of patients with

Masaru Terai; Toshiaki Jibiki; Akihisa Harada; Yuya Terashima; Kumi Yasukawa; Shigeru Tateno; Hiromichi Hamad; Shinji Oana; Hiroo Niimi; Kouji Matsushima

304

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

PubMed

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

2015-01-21

305

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 ...

306

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...

307

Behcet's disease: evaluation of a new instrument to measure clinical activity  

Microsoft Academic Search

Objective. Behcet's disease (BD) is a rare multisystem disorder characterized by vasculitis. At present, there are no laboratory markers that correlate well with the clinical activity in BD. This has led to the development of an instrument (BD Current Activity Form) to measure activity. Scoring is based on the history of new clinical features present over the preceding 4 weeks

B. B. Bhakta; P. Brennan; T. E. James; M. A. Chamberlain; B. A. Noble; A. J. Silman

1999-01-01

308

Current Concepts in the Etiology and Treatment of Behçet Disease  

Microsoft Academic Search

Behçet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce

Cem Evereklioglu

2005-01-01

309

Essential type II mixed cryoglobulinemia causing pyoderma gangrenosum-like ulcers.  

PubMed

A 47-year-old, otherwise healthy woman presented with multiple deep ulcers located primarily on her lower extremities that presented 6 days ago as blisters. A biopsy revealed livedoid vasculopathy, without any evidence of vasculitis. Extensive laboratory workup was positive only for type II cryoglobulins. PMID:18335149

Namazi, M R; Kerchner, K R; Pichardo, R O

2008-01-01

310

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

311

Asymptomatic Vasculitic Neuropathy.  

PubMed

Objectives: We did a retrospective analysis of the clinical, pathological, and electrophysiological features of 21 cases of asymptomatic vasculitic neuropathy (AsVN). Methods: Among 270 patients with biopsy proven vasculitic neuropathy, we identified 21 (7.8%) who had asymptomatic neuropathy. Results: Of the 21 patients with AsVN, 11 were women and 10 were men. The mean age was 62.5 years. Referring physicians suspected systemic vasculitis on the basis of clinical and laboratory features, but none of the patients had neuropathy by examination. Screening nerve conduction studies identified neuropathy in all, leading us to perform a sural nerve, biopsy which confirmed the diagnosis of vasculitis. Twelve patients had active (Type I), 6 had inactive (Type II), and 3 had probable (Type III) vasculitis. Vasculitis was primary in 10 patients and secondary in 11. Conclusions: Nerve conduction study is an important tool for identifying AsVN, a sub-type of vasculitic neuropathy. © 2014 Wiley Periodicals, Inc. PMID:25354330

Kurt, Semiha; Alsharabati, Mohammad; Lu, Liang; Claussen, Gwendolyn C; Oh, Shin J

2014-10-29

312

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

313

Linear Branching Echogenicities in the Basal Ganglia and Thalami  

Microsoft Academic Search

Echogenic vasculature in the basal ganglia and thalami of neonatal brain have been associated with congeni- tal infections such as cytomegalovirus (CMV), rubella, and syphilis, trisomy 13 syndrome, Down syndrome, maternal drug use, neonatal asphyxia, nonimmune hydrops, and fetal alcohol syndrome. This abnormality is believed to result from necrotizing vasculitis with subsequent mineralization. In our study, we encountered 8 small

Han-Hsi Wang; Chih-Hao Chien; Min-Hou Liao; Yu-Nian Wu; Yu-Hsien Su

1998-01-01

314

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.

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

2015-01-01

315

Rituximab Induced Remission in a Patient with Wegener’s Granulomatosis  

Microsoft Academic Search

Background: Wegener’s granulomatosis is a form of systemic vasculitis typically involving the kidneys and upper and lower respiratory tract. Treatment employing cyclophosphamide and prednisone has improved prognosis, but relapses and treatment-induced side effects still cause severe morbidity and frequent mortality. There is therefore an urgent need to find new treatment modalities that are efficient and cause few side effects. Chimeric

Maria Kallenbach; Hongmei Duan; Troels Ring

2005-01-01

316

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

317

Mesenteric Phlebosclerosis  

Microsoft Academic Search

PURPOSE: Nonthrombotic stenosis or occlusion of the mesenteric veins is a rare cause of intestinal ischemia. The aim of this study was to describe a new disease entity causing chronic ischemic colitis. METHODS: Seven patients were diagnosed as having mesenteric phlebosclerosis. All seven patients had calcifications in the small mesenteric veins and their intramural branches. No evidence of vasculitis or

Akinori Iwashita; Tsuneyoshi Yao; Ronald J. Schlemper; Yasuyuki Kuwano; Takashi Yao; Mitsuo Iida; Masahiro Kikuchi

2003-01-01

318

Hepatitis B virus-related polyarteritis nodosa presenting with multiple lung nodules and cavitary lesions.  

PubMed

The patient presented here is a 59-year-old Japanese man with active chronic hepatitis B with precore and core promoter mutated virus, presenting with high fever, bloody sputum, and multiple lung nodules with excavation. Surgical biopsy of the lung nodule showed necrotizing vasculitis affecting pulmonary arteries without granulomatous changes. The pulmonary manifestations of this patient resembled Wegener granulomatosis. However, the pathologic findings showing nongranulomatous necrotizing vasculitis involving the small pulmonary arteries, presence of circulating immune complex, absence of antineutrophil cytoplasmic antibodies, and excellent response to the combination therapy of corticosteroid and an anti-hepatitis B virus agent, entecavir, led us to the diagnosis of hepatitis B virus-related polyarteritis nodosa (PAN). Radiographic evidence of lung nodules or cavitations seen in systemic vasculitis patients has been considered a sign suggestive of granulomatous disease and a diagnostic surrogate marker for necrotizing granulomatous vasculitis, but a clinical relevance to hepatitis B virus-related PAN has not been reported before this case. PMID:20605819

Naniwa, Taio; Maeda, Tomoyo; Shimizu, Shigeki; Ito, Rei

2010-07-01

319

Pulmonary Lymphatics Are Primary Sites of Mycobacterium tuberculosis Infection in Guinea Pigs Infected by Aerosol  

Microsoft Academic Search

Mycobacterium tuberculosis causes a lymphatic vasculitis in the lungs of guinea pigs infected by a low-dose aerosol. This observation suggests that in addition to being a direct conduit from the lungs to the regional lymph nodes, pulmonary lymphatics are themselves sites of infection and could be the site of latent infection. The pathogenesis of Mycobacterium tuberculosis infection in the guinea

Randall J. Basaraba; Erin E. Smith; Crystal A. Shanley; Ian M. Orme

2006-01-01

320

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

321

Behçet's disease in a patient with immunodeficiency virus infection  

Microsoft Academic Search

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

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

1991-01-01

322

The potential role of bacterial superantigens in the pathogenesis of Kawasaki syndrome  

Microsoft Academic Search

Kawasaki syndrome is an acute multisystem vasculitis of infancy and early childhood associated with high fever, mucocutaneous inflammation, and the development of coronary artery abnormalities. Despite the widely held belief that Kawasaki syndrome is an infectious disease, investigations have failed to identify a causal organism. Previous studies have demonstrated that this illness is associated with marked activation of monocyte\\/macrophages and

Donald Y. M. Leung; Cody Meissner; David Fulton; Patrick M. Schlievert

1995-01-01

323

Ischemic Colitis Revealing Polyarteritis Nodosa  

PubMed Central

Ischemic colitis is one of the most common intestinal ischemic injuries. It results from impaired perfusion of blood to the bowel and is rarely caused by vasculitis. We report a case of ischemic colitis revealing polyarteritis nodosa (PAN) in a 55-year-old man. Histological examination of the resected colon led to the diagnosis of PAN. PMID:24382967

Hamzaoui, Amira; Litaiem, Noureddine; Smiti Khanfir, M.; Ayadi, Sofiene; Nfoussi, Haifa; Houman, M. H.

2013-01-01

324

Neuritis and Gastrointestinal Hemorrhage in Scrub Typhus Patients.  

PubMed

A 78-year-old woman with scrub typhus exhibited gastric hemorrhage with multiple gastric ulcers. This is the first report to confirm pathologically the presence of not only vasculitis but also, neuritis. The results suggest the necessity of studies confirming neuritis as the cause of gastric ulcer and bleeding in scrub typhus. PMID:25385859

Kim, Dong-Min; Yun, Na Ra; Lim, Sung-Chul

2014-11-10

325

Peripheral nerve involvement in Churg-Strauss syndrome  

Microsoft Academic Search

Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common

Raffaella Marazzi; Davide Pareyson; Amerigo Boiardi; Massimo Corbo; Vidmer Scaioli; Angelo Sghirlanzoni

1992-01-01

326

Management of alveolar hemorrhage in lung vasculitides.  

PubMed

Alveolar hemorrhage (AH) is an important pulmonary manifestation of small vessel vasculitis because severe presentations are the most common vasculitic cause of early death. Renal vasculitis is usually present with AH; the combination is known as pulmonary-renal syndrome. Early diagnosis and intensive therapy are of particular importance to reduce early mortality and improve longer-term outcomes. The commonest immune-mediated cause of AH is anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (80%), with other vasculitides, including systemic lupus erythematosus and anti-glomerular basement membrane disease accounting for 20%. One quarter of AAV patients develop AH, which when mild is associated with a good outcome, but mortality rises to 50% for cases with respiratory failure requiring ventilator support. The prognosis of AH in the other vasculitides is generally favorable, but cases are rare and experience is limited. Treatment follows similar regimens to those for other AAV presentations, although when severe there is widespread use of parenteral glucocorticoids together with plasma exchange. These interventions have developed empirically supported by a theoretical rationale but have not been validated by randomized clinical trials. Sepsis and cardiovascular and thromboembolic events are important early complications. and long-term follow-up is required to monitor for and prevent relapse and manage disease-related damage. A minority of cases develop on a background of pulmonary fibrosis, or progressive pulmonary fibrosis develops after vasculitis has gone into remission. PMID:21674419

Casian, Alina; Jayne, David

2011-06-01

327

[A woman with red discolouration of the lower leg].  

PubMed

A 22-year-old women visited the Emergency Department with purpura of the right lower extremity subsequent to four days of hiking 40 kilometres daily. The diagnosis 'exercise-induced vasculitis' was made. This is a harmless and self-limiting disease that can occur due to exercising in warm weather. PMID:24382041

van Deudekom, Floor J A; Stradmeijer, Marloes D; Kuijer, Philomeen M M

2013-01-01

328

Erythema nodosum in children: A prospective study  

Microsoft Academic Search

Background: The studies of series of children with erythema nodosum (EN) are limited and mostly retrospective. Objective: We evaluated the epidemiology, etiology, clinical manifestations, course, and prognosis of EN in children. Methods: Thirty-five children with EN (17 boys, 18 girls; mean age, 8.79 years) have been studied. Four excluded children proved, on biopsy, to have leukocytoclastic vasculitis (n = 3)

Talia Kakourou; Panayiota Drosatou; Fotini Psychou; Kyriaki Aroni; Polyxeni Nicolaidou

2001-01-01

329

Severe drug-induced dermatoses.  

PubMed

A variety of common dermatoses are known to have drug-induced variants. This article discusses the clinical presentation, time frames, reported culprit medications, pathophysiology and management of drug-induced lupus, cutaneous vasculitis, pemphigus, pemphigoid, linear IgA bullous dermatosis, Sweet's syndrome, erythema nodosum, pyoderma gangrenosum, pseudolymphoma, lichen planus, and psoriasis. PMID:25037258

Ahronowitz, Iris; Fox, Lindy

2014-03-01

330

Naturally occurring sheep-associated malignant catarrhal fever in North American pigs  

Technology Transfer Automated Retrieval System (TEKTRAN)

Two cases of sheep-associated malignant catarrhal fever (MCF) in pigs were diagnosed on a small farm in New York State, and in Kentucky, U.S.A. In both cases initial diagnosis was based on histopathological changes representing typical lymphoproliferative vasculitis in multiple tissues of the affect...

331

[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

332

www.nature.com/reviews Leadingthe field  

E-print Network

* Nature Reviews Nephrology* Nature Reviews Neurology* Nature Reviews Rheumatology* Nature Reviews Urology to classification, ANCA detection and trial design focus Vasculitis RHEUMATOLOGY nrrheum_OFC_AUG14.indd 1 15. www.nature.com/reviews/neurol Nature Reviews Rheumatology Chief Editor: Jenny Buckland, PhD Impact

Cai, Long

333

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

334

Association of killer cell immunoglobulin-like receptor genotypes with vascular arterial events and anticardiolipin antibodies in patients with lupus.  

PubMed

To determine whether killer cell immunologlobulin-like receptor (KIR) genotypes are associated with vasculitis, vascular arterial events or anticardiolipin (aCL) antibodies in patients with lupus. A total of 304 patients followed prospectively at the University of Toronto Lupus Clinic were assessed for the occurrence of vasculitis and vascular arterial events. Molecular HLA-C and KIR (presence or absence of KIR2DL1, 2DL2, 2DL3, 2DS1 and 2DS2) genotyping were performed. Chi-square and logistic regression were used to analyse association between KIR genes and vascular arterial events and aCL antibodies. In patients with vascular arterial events, there was a significant increase in KIR2DS2 (60% vs 45%, P = 0.02) and in KIR2DL2 (62% vs 47%, P = 0.01) compared with patients without events. There was no increase in activating KIR genotypes in patients with vasculitis. In patients with aCL antibodies, significant increases were seen in KIR2DS2 (54% vs 41%, P = 0.03) and KIR2DL2 (58% vs 41%, P = 0.003), but KIR2DL3 was decreased (87% vs 95%, P = 0.03). Logistic regression confirmed independent association of KIR2DS2 with vascular arterial events. We found an increase in KIR2DS2 in lupus patients with vascular arterial events, but not in patients with vasculitis. PMID:18755860

Toloza, Sma; Pellett, Fj; Chandran, V; Ibanez, D; Urowitz, Mb; Gladman, Dd

2008-09-01

335

Spontaneous Superficial Femoral Artery Pseudoaneurysm in Behcet's Disease.  

PubMed

Behcet's disease is an autoimmune multisystemic disorder on vasculitis base. Cardiovascular involvement is the most important predictor of morbidity and mortality. The treatment should be planned carefully for pathologies requiring interventions. In our report, we present a 45-year-old patient with spontaneous superficial femoral artery pseudoaneurysm, our treatment strategy, and circumstances we faced. PMID:24822070

Ugurlucan, Murat; Sendil, Selin; Sayin, Omer Ali; Barburoglu, Mehmet; Gok, Emre; Turkyilmaz, Gulsum; Basaran, Murat; Alpagut, Ufuk; Dayioglu, Enver

2014-01-01

336

Spontaneous Superficial Femoral Artery Pseudoaneurysm in Behcet's Disease  

PubMed Central

Behcet's disease is an autoimmune multisystemic disorder on vasculitis base. Cardiovascular involvement is the most important predictor of morbidity and mortality. The treatment should be planned carefully for pathologies requiring interventions. In our report, we present a 45-year-old patient with spontaneous superficial femoral artery pseudoaneurysm, our treatment strategy, and circumstances we faced. PMID:24822070

Sendil, Selin; Sayin, Omer Ali; Barburoglu, Mehmet; Gok, Emre; Turkyilmaz, Gulsum; Basaran, Murat; Alpagut, Ufuk; Dayioglu, Enver

2014-01-01

337

State of the art in the treatment of systemic vasculitides.  

PubMed

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

338

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

339

Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study  

PubMed Central

Background Kawasaki disease is recognized as the most common cause of acquired heart disease in children in the developed world. Clinical, epidemiologic, and pathologic evidence supports an infectious agent, likely entering through the lung. Pathologic studies proposing an acute coronary arteritis followed by healing fail to account for the complex vasculopathy and clinical course. Methodology/Principal Findings Specimens from 32 autopsies, 8 cardiac transplants, and an excised coronary aneurysm were studied by light (n=41) and transmission electron microscopy (n=7). Three characteristic vasculopathic processes were identified in coronary (CA) and non-coronary arteries: acute self-limited necrotizing arteritis (NA), subacute/chronic (SA/C) vasculitis, and luminal myofibroblastic proliferation (LMP). NA is a synchronous neutrophilic process of the endothelium, beginning and ending within the first two weeks of fever onset, and progressively destroying the wall into the adventitia causing saccular aneurysms, which can thrombose or rupture. SA/C vasculitis is an asynchronous process that can commence within the first two weeks onward, starting in the adventitia/perivascular tissue and variably inflaming/damaging the wall during progression to the lumen. Besides fusiform and saccular aneurysms that can thrombose, SA/C vasculitis likely causes the transition of medial and adventitial smooth muscle cells (SMC) into classic myofibroblasts, which combined with their matrix products and inflammation create progressive stenosing luminal lesions (SA/C-LMP). Remote LMP apparently results from circulating factors. Veins, pulmonary arteries, and aorta can develop subclinical SA/C vasculitis and SA/C-LMP, but not NA. The earliest death (day 10) had both CA SA/C vasculitis and SA/C-LMP, and an “eosinophilic-type” myocarditis. Conclusions/Significance NA is the only self-limiting process of the three, is responsible for the earliest morbidity/mortality, and is consistent with acute viral infection. SA/C vasculitis can begin as early as NA, but can occur/persist for months to years; LMP causes progressive arterial stenosis and thrombosis and is composed of unique SMC-derived pathologic myofibroblasts. PMID:22723916

Orenstein, Jan Marc; Shulman, Stanford T.; Fox, Linda M.; Baker, Susan C.; Takahashi, Masato; Bhatti, Tricia R.; Russo, Pierre A.; Mierau, Gary W.; de Chadarévian, Jean Pierre; Perlman, Elizabeth J.; Trevenen, Cynthia; Rotta, Alexandre T.; Kalelkar, Mitra B.; Rowley, Anne H.

2012-01-01

340

[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

341

Detection of circulating immune complexes by Raji cell assay: comparison of flow cytometric and radiometric methods  

SciTech Connect

Several flow cytometric methods for the measurement of circulating immune complexes (CIC) have recently become available. We report a Raji cell flow cytometric assay (FCMA) that uses aggregated human globulin (AHG) as primary calibrator. Technical advantages of the Raji cell flow cytometric assay are discussed, and its clinical usefulness is evaluated in a method comparison study with the widely used Raji cell immunoradiometric assay. FCMA is more precise and has greater analytic sensitivity for AHG. Diagnostic sensitivity by the flow cytometric method is superior in systemic lupus erythematosus (SLE), rheumatoid arthritis, and vasculitis patients: however, diagnostic specificity is similar for both assays, but the reference interval of FCMA is narrower. Significant correlations were found between CIC levels obtained with both methods in SLE, rheumatoid arthritis, and vasculitis patients and in longitudinal studies of two patients with cerebral SLE. The Raji cell FCMA is recommended for measurement of CIC levels to clinical laboratories with access to a flow cytometer.

Kingsmore, S.F.; Crockard, A.D.; Fay, A.C.; McNeill, T.A.; Roberts, S.D.; Thompson, J.M.

1988-01-01

342

Pulsed-laser therapy (GA-As) in combined treatment of post-traumatic swellings and some dermatological disorders  

NASA Astrophysics Data System (ADS)

The effect of a pulsed gallium arsenide infrared laser radiation was studied on 64 patients with post traumatic swellings, allergic vasculitis and varicose crural ulcers, therapy resistant. The soft laser therapy was conducted in combination with classical therapy and was compared with a non irradiated control group of 52 patients treated only by classical therapy. Laser irradiation was directed to the skin damage by laser scanning. Segmental and dermatomic areas of the skin lesions were irradiated by laserpuncture. Therapeutic protocol included an average of nine sessions. The statistical analysis shows a significant difference of the efficiency between Ga-As pulsed laser treated group and the control group, especially in the case of post-traumatic swellings and less in the case of allergic vasculitis. The effects reported by this study are relevant for clinical application of infrared pulsed low lasers in dermatology.

Antipa, Ciprian; Dona, Dumitru; Podoleanu, Adrian Gh.

1994-02-01

343

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

344

[Dermatologic manifestations of the antiphospholipid syndrome].  

PubMed

A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies. PMID:22078802

Francčs, C; Barete, S; Soria, A

2012-04-01

345

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

346

An often unrecognized cause of thunderclap headache: reversible cerebral vasoconstriction syndrome.  

PubMed

Thunderclap headache (TCH) can have several causes of which subarachnoid hemorrhage (SAH) is most common and well known. A rare cause of TCH is the reversible cerebral vasoconstriction syndrome (RCVS) which is characterized by a reversible segmental vasoconstriction of the intracranial vessels. We describe two patients with TCH due to RCVS and the probable precipitating factor, namely, cannabis and an anti-migraine drug. In RCVS, cerebrospinal fluid examination is (near) normal, in contrast to SAH and (primary) cerebral vasculitis. Brain MRI may be normal or shows infarction. MRA can demonstrate vasoconstriction of the great arteries, but a normal MRA does not rule out the diagnosis. Caliber changes on cerebral angiography cannot adequately differentiate between RCVS and vasculitis. Calcium-channel antagonists may be a good therapy and repeated transcranial Doppler ultrasonography can be a reliable non-invasive investigation to monitor the effect of treatment and demonstrate reversibility of the vasoconstriction. PMID:18810315

Koopman, K; Teune, L K; ter Laan, M; Uyttenboogaart, M; Vroomen, P C; De Keyser, J; Luijckx, G J

2008-12-01

347

Think clearly, be sincere, act calmly: Adolf Kussmaul (February 22, 1822-May 28, 1902) and his relevance to medicine in the 21st century.  

PubMed

This past year, 2002, marked the 100th anniversary of the death of Adolf Kussmaul. To rheumatologists, he is important as the author of the first case of idiopathic vasculitis, polyarteritis nodosa, which even today is the basis of our understanding of vasculitis. To general internists, his name is linked to Kussmaul's sign and Kussmaul breathing, and to gastroenterologists, he is in many ways-through the development of the gastric pump and the first gastroscope-the father of modern gastroenterology. His reputation as an excellent teacher, his compassion for his patients, and his ability as a clinician scientist led him to be regarded as a giant of 19th century medicine. Kussmaul's life and career, dedicated foremost to reducing human suffering and advancing medicine as a scientific discipline with a strong humanistic foundation, make him a role model for physicians of today. PMID:12496507

Matteson, Eric L; Kluge, Friedrich J

2003-01-01

348

A purple rash: a case study of Henoch-Schönlein purpura.  

PubMed

Rashes are one of the most common presenting symptoms in the emergency department setting; they may be a primary diagnosis or a symptom of systemic disease. Accurate diagnosis of a rash may be challenging and requires clinical acumen, systematic history and physical examination, and judicious use of diagnostic testing. This case study reviews Henoch-Schönlein purpura (HSP) or IgA vasculitis, a common vasculitis of childhood. Although typically a self-limited disease, HSP may cause serious complications in a small subset of patients and requires monitoring. In addition, there are several differential diagnoses that may mimic HSP that are more serious. This article reviews the case of a 6-year-old boy who presented to a community emergency department and had a nontypical clinical course. PMID:25076400

Contino, Christopher; Bradbury-Golas, Kathleen

2014-01-01

349

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

350

A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase  

PubMed Central

This report concerns a 53-year-old male patient with idiopathic hypertrophic cranial pachymeningitis who presented with multiple cranial nerve palsies (I, II, III, IV, V, VI). Brain magnetic resonance imaging showed diffuse thickening and gadolinium enhancement of the cerebral dura mater. A biopsy of the cerebral dura mater showed granulomatous vasculitis with histiocyte infiltration. Although both the serum rheumatoid factor (RF) and matrix metalloproteinase-3 (MMP-3) were high, the patient showed no signs of arthritis. He was anti-cyclic citrullinated peptide antibody negative, which makes the presence of comorbid chronic rheumatoid arthritis (RA) unlikely. The aetiology of the pachymeningitis was unknown, which led to the diagnosis of idiopathic hypertrophic cranial pachymeningitis. Steroid pulse therapy successfully diminished the patient’s pachymeningitis and lowered both RF and MMP-3. High values of RF suggest the possible involvement of an autoimmune mechanism, and the MMP value may be an important indicator of the aetiology of pachymeningitis with granulomatous vasculitis. PMID:22368690

Nakamagoe, Kiyotaka; Hosaka, Ai; Kondo, Yuzuru; Ishikawa, Eiichi; Tamaoka, Akira

2010-01-01

351

An unusual manifestation of Wegener's granulomatosis in a 4-year-old girl.  

PubMed

We report a female who was diagnosed with Wegener's granulomatosis at 4 years of age with life-threatening intracranial bleeding. The patient's serum was positive for c-antineutrophilic cytoplasmic antibodies, and histologic analysis of the lung biopsy revealed evidence of granulomatous vasculitis. Initial treatment with steroids and cyclophosphamide was successfully converted to a long-term medication regimen consisting of azathioprine, trimethoprim, and sulfamethoxazole. Thereafter the patient showed no signs of disease activity for more than 3 years and manifested only a low-grade neurologic handicap. In February 2001, 5 years after the initial diagnosis, she presented with altered consciousness and myoclonic multifocal seizures. Subsequent diagnostic studies confirmed the diagnosis of disseminated cerebral vasculitis unresponsive to steroid treatment. Acute neurologic symptoms relented immediately after cyclophosphamide pulse therapy. Magnetic resonance imaging of the brain demonstrated complete remission within 8 weeks. Her current treatment includes steroids and monthly pulses of cyclophosphamide. PMID:12160980

Haas, Johannes-Peter; Metzler, Markus; Ruder, Hans; Waldherr, Rüdiger; Böswald, Michael; Rupprecht, Thomas

2002-07-01

352

Progressive pigmentary purpura.  

PubMed

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

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

2011-01-01

353

ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies  

PubMed Central

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy. PMID:24265538

Kim, Sung-Hee; Park, Jin; Bae, Jung Ho; Cho, Min-Sun; Park, Kee Duk

2013-01-01

354

Placental intravascular organisms: a case report.  

PubMed

Ascending amniotic fluid bacterial infection is a cause of perinatal morbidity and mortality. A diagnosis of amniotic cavity infection can be inferred by documenting maternal (acute chorioamnionitis) and/or fetal (chorionic plate vasculitis; umbilical vasculitis/funisitis) inflammatory response. A definitive diagnosis of intrauterine/neonatal sepsis as a cause of stillbirth requires positive blood cultures obtained at postmortem examination. However, if postmortem examination is not performed, acute chorioamnionitis with/without fetal inflammatory response cannot be classified as a cause of demise. We present a case of intrauterine demise associated with acute chorioamnionitis, villitis, and intervillositis of the placenta. Although postmortem examination was denied, a conclusive diagnosis of intrauterine sepsis could be rendered by demonstration of gram-positive cocci within fetal vessels of umbilical cord, chorionic plate, and stem villi. This report highlights the importance of identification of placental intravascular organisms as unequivocal evidence of fetal sepsis, especially in cases where cultures cannot be obtained. PMID:20877362

Matoso, A; Shapiro, S; De Paepe, M E; Gundogan, F

2010-10-01

355

Biologic therapies in the vasculitides.  

PubMed

Monoclonal antibody and recombinant DNA technologies have led to the development of biologic therapies capable of directly targeting selected components of the immune response. With the steady expansion of knowledge regarding the mechanisms of vascular inflammation, the safety and efficacy of biologic agents in the vasculitic diseases are being increasingly investigated. By targeting specific effector mechanisms involved in the pathogenesis of vasculitis, these agents may provide a less toxic means of inducing remission and lessening relapse. However, the study of biologic therapies in the vasculitides must be approached with caution, as unanticipated effects on disease activity and disease-specific toxicities can occur. Studies to examine these agents must recognize the potential for active vasculitis to be organ- or life-threatening as well as the current existence of effective therapies. In the research setting, investigation of biologic agents in the treatment of vasculitic diseases may also provide important insights into pathogenesis of these syndromes. PMID:12496503

Langford, Carol A; Sneller, Michael C

2003-01-01

356

[Eosinophilic angiocentric fibrosis and granuloma facial with extra facial presentation, the same pathology?].  

PubMed

Eosinophilic angiocentric fibrosis is a rare fibro inflammatory lesion of unknown etiology which occurs usually in the upper respiratory tract mucosa of middle-aged adults. The histologic features show an eosinophilic vasculitis and an angiocentric fibrosis with onion-skin pattern. Firstly described as a mucosal variant of the granuloma facial, which is a rare cutaneous vasculitis with eosinophils, it is considerated by some authors as separated entities. Four cases have been described in the orbit and three of them were in fact an extension of a sinusal lesion. We report the first case affecting a 69-years-old male patient who showed an isolated orbital involvement in association with granuloma facial, extra facial. This observation illustrates the relationship between these two pathologies and consolidates the first hypothesis of a single disease with cutaneous or mucosal involvement. PMID:21736992

Depaepe, Lauriane; Chouvet, Brigitte; Claudy, Alain; Thomas, Luc; Berger, Françoise; Balme, Brigitte

2011-06-01

357

Positivity to p-ANCA in patients with status epilepticus  

PubMed Central

Background Status epilepticus (SE) may occur in the setting of several internal or neurologic diseases. Anti-neutrophilic cytoplasmic antibodies (ANCA) are a group of Ig that may be observed in patients with different autoimmune disorders but are particularly associated with systemic vasculitis named ANCA-associated-vasculities (AAV). We herein report 3 patients with SE and positivity to p-ANCA. Case presentation One patient had a catastrophic evolution and died 5 months after disease onset. The other two patients had a good outcome and remained seizure-free at 30 months and 5 years of follow-up respectively. Conclusion This report highlights the importance of considering ANCA dosage in patients with SE of unclear origin. PMID:25033936

2014-01-01

358

Detection of circulating immune complexes by Raji cell assay: comparison of flow cytometric and radiometric methods.  

PubMed

Several flow cytometric methods for the measurement of circulating immune complexes (CIC) have recently become available. We report a Raji cell flow cytometric assay (FCMA) that uses aggregated human globulin (AHG) as primary calibrator. Technical advantages of the Raji cell flow cytometric assay are discussed, and its clinical usefulness is evaluated in a method comparison study with the widely used Raji cell immunoradiometric assay. FCMA is more precise and has greater analytic sensitivity for AHG. Diagnostic sensitivity by the flow cytometric method is superior in systemic lupus erythematosus (SLE), rheumatoid arthritis, and vasculitis patients: however, diagnostic specificity is similar for both assays, but the reference interval of FCMA is narrower. Significant correlations were found between CIC levels obtained with both methods in SLE, rheumatoid arthritis, and vasculitis patients and in longitudinal studies of two patients with cerebral SLE. The Raji cell FCMA is recommended for measurement of CIC levels to clinical laboratories with access to a flow cytometer. PMID:3219778

Kingsmore, S F; Crockard, A D; Fay, A C; McNeill, T A; Roberts, S D; Thompson, J M

1988-01-01

359

[Multiple extrarenal complications in Wegener granulomatosis].  

PubMed

This is a case report of a patient with Wegener's granulomatosis (WG), who initially presented with a corneal perforation. In addition to the eye involvement, the pauciimmune necrotizing glomerulonephritis with crescent formation, E.N.T. and pulmonary involvement were diagnosed. The patient also suffered from the acute myocardial infarction, most likely due to coronal arteritis. In addition to the coronal ischaemia she also had vasculitis of the aortic valve due to the WG. Another rare complication was the massive intestinal bleeding. The patient had also a skin vasculitis and non-specific symptoms such as artralgias and fever. The correct diagnosis was supported by positive cANCA. A clinical and laboratory remission of the disease was achieved by combined immunosuppressive therapy. Subsequently, she developed a subglotic stenosis possibly due to reparative changes. PMID:11678030

Ríhová, Z; Merta, M; Rysavá, R; Bezdícek, P; Danzig, V; Gorican, K; Lukás, J; Skalická, P; Vernerová, Z; Tesar, V

2001-08-01

360

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

361

Infective endocarditis mimics ANCA associated glomerulonephritis  

PubMed Central

Background: Sub-acute bacterial endocarditis (SBE) rarely presents with features of a small vessel vasculitis. Patients with SBE can also develop multiple serological abnormalities including ANCA. In this report, we present a case of infective endocarditis mimicked ANCA associated glomerulonephritis. Case presentation: A 57-year old male with a clinical picture of rapidly progressive renal failure (RPGN) and positive seology for PR3-ANCA (C-ANCA) was referred to our hospital. The renal histology findings were compatible with focal and segmental glomerular necrosis. After receiving corticosteroid therapy, the patient became febrile and his general condition worsened. Cardiac ultrasound echocardiographic study disclosed multiple large vegetations on the aortic valve. After appropriate antibiotic therapy and valvular surgery, the patient’s condition improved and his serum creatinine reached 1.7 mg/d. Conclusion: Misdiagnosis of SBE as ANCA-associated vasculitis and an inappropriate immunosuppressive therapy can have catastrophic consequences. PMID:24009921

Reza Ardalan, Mohammad; Trillini, Matias

2012-01-01

362

Idiopathic atrophie blanche.  

PubMed

Idiopathic atrophie blanche (segmental hyalinizing vasculitis; livedo reticularis with summer ulceration) is a chronic cutaneous disorder of young to middle-aged women that is characterized by persistent painful leg ulcerations. Primary lesions consist of purpuric macules and papules which undergo superficial ulceration, followed eventually by the development of irregular, atrophic, porcelain white scars with fine borders of ectatic vessels. We have studied twelve patients with idiopathic atrophie blanche by immunofluorescence, thin section light microscopy, and electron microscopy. All patients were examined extensively in order to rule out primary immunologic and vaso-occlusive disorders that may result in a similar clinical appearance. Subsequently, ten patients were treated for 1 to 12 years with combinations of phenformin and ethylestrenol. Each treated patient noted rapid improvement in existing lesions, significantly less pain, and a decrease in the development of new lesions. Side effects in all but two patients were minimal and well tolerated. Light and electron microscopic examination of early and well-developed lesions revealed fibrin plugs which first occlude superficial dermal vessels, followed by necrosis and obliteration of the affected vessel walls. Eventually, new vessel formation occurs in some areas of fibrin deposition. Polymorphonuclear leukocytes and mononuclear cells only rarely participate in this process. Although this disorder has previously been considered a localized form of cutaneous vasculitis, the absence of both leukocytes and nuclear fragmentation from the neighborhood of vascular structures in early lesions differentiates this disorder from immune complex-mediated necrotizing vasculitis. Thus the term vasculopathy describes this disorder more appropriately than the term vasculitis. PMID:6345609

Shornick, J K; Nicholes, B K; Bergstresser, P R; Gilliam, J N

1983-06-01

363

Multiple idiopathic arterial aneurysms masquerading as aortic dissection  

PubMed Central

We report the case of a 58-year-old lady who presented with abdominal pain and backache. Although initial evaluation was strongly suggestive of abdominal aortic dissection, she was ultimately found to have multiple arterial aneurysms. Work-up for underlying vasculitis was negative. Surgical repair was planned and the patient was referred to a cardiovascular surgeon. This case highlights the importance of careful radiological assessment in patients with suspected aortic dissection. PMID:23420724

Naha, Kushal; Vivek, G; Shetty, Ranjan K; Dias, Lorraine Simone

2013-01-01

364

[Acute myocardial infarction as Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome) initial presentation].  

PubMed

Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine. PMID:25627304

Sulaiman, Wahinuddin; Seung, Ong Ping; Noor, Sabariah Mohd

2014-01-01

365

Fifteen-year experience of children with Henoch-Schönlein purpura in southern Taiwan, 1991-2005  

Microsoft Academic Search

Background and Purpose: Although Henoch-Schönlein purpura (HSP) is the most common cause of systemic vasculitis in children, long-term and large-scale Taiwanese studies on HSP are rare. We reviewed the records of 107 Taiwanese pediatric patients diagnosed with HSP at our institution between 1991 and 2005. Methods: The first clinical manifestations, laboratory findings, and outcome evaluations of the patients were analyzed.

Bao-Ren Nong; Yung-Feng Huang; Chih-Ming Chuang; Chia-Chia Liu; Kai-Sheng Hsieh

2007-01-01

366

Paroxysmal drastic abdominal pain with tardive cutaneous lesions presenting in Henoch-Schönlein purpura  

PubMed Central

Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. The diagnosis of HSP is difficult, especially when abdominal symptoms precede cutaneous lesions. We report a rare case of paroxysmal drastic abdominal pain with gastrointestinal bleeding presented in HSP. The diagnosis was verified by renal damage and the occurrence of purpura. PMID:22563183

Chen, Xiao-Liang; Tian, Hong; Li, Jian-Zhong; Tao, Jin; Tang, Hua; Li, Yang; Wu, Bin

2012-01-01

367

Sudden death of a child from myocardial infarction due to arteritis of the left coronary trunk.  

PubMed

An eight-year-old Japanese boy developed abdominal pain, followed by convulsion and loss of consciousness. He was taken to an emergency room but could not be resuscitated. At autopsy, the left main coronary trunk (LMT) demonstrated an increase in caliber with severe luminal narrowing, and the left anterior descending branch (LAD) subsequent to the LMT showed severe stenosis. Microscopically, the intima of the LMT demonstrated severe fibrosis and infiltration of lymphocytes and histiocytes suggesting vasculitis, and the small lumen was occupied by a fresh thrombus. The LAD showed significant intimal thickening with strong lymphocytic inflammation at the edge of the thickening. The left ventricle showed widespread myocardial infarction in the recovery stage. There were no findings of atherosclerosis, vasculitis or fibrocellular changes in the ascending aorta or intravisceral arteries other than the LMT and the LAD under investigation. The increase in the caliber of the LMT and the limitation of arteritis to the LMT and the subsequent branch suggested Kawasaki disease (KD), but it was atypical that the patient had no clinical history consistent with KD. The present case showed no findings suggesting classical polyarteritis nodosa (cPAN) at the acute or scar stage in the other vessels being investigated, and cPAN in childhood is rare compared to KD. A nonspecific inflammatory reaction (single organ vasculitis, SOV) was also considered as a possible cause, but it is difficult to determine whether the cause of the coronary stenosis in the present case was SOV because the sampling of arteries was insufficient. If forensic pathologists make unusual findings suggesting vasculitis at autopsy, the collection of a sufficient number of vessels of various sizes is warranted. PMID:25239164

Takahashi, Shirushi; Takada, Aya; Saito, Kazuyuki; Hara, Masaaki; Yoneyama, Katsumi; Nakanishi, Hiroaki; Takahashi, Kei; Moriya, Takuya; Funayama, Masato

2015-01-01

368

Actinic granulomas and relapsing polychondritis.  

PubMed

A patient developed concomitantly chondritis of the two auricles, diffuse cutaneous vasculitis and actinic granulomas. Alterations in skin and cartilage were prominent in the elastic tissue. Anticollagen type II antibodies were absent from the serum and there was no deposit of immunoreactants in cartilages. In this form of relapsing polychondritis, the pathomechanism resembles that of diffuse actinic arteritis as proposed by O'Brien. It is concluded that relapsing polychondritis may represent a heterogeneous syndrome with regard to its pathogenesis. PMID:6187164

Pierard, G E; Henrijean, A; Foidart, J M; Lapiere, C M

1982-01-01

369

A case of extreme upper limb surgical revascularization in Buerger's disease.  

PubMed

Thromboangiitis obliterans or Buerger's disease is a rare nonatherosclerotic 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 51-year-old man was found to have critical ischemia of his left hand, with necrosis of 3 fingers. He underwent extremely distal surgical revascularization using a cephalic vein bypass graft. PMID:24887859

Massara, Mafalda; De Caridi, Giovanni; Spinelli, Francesco

2015-01-01

370

Gastrointestinal Manifestations of Behçet’s Disease  

Microsoft Academic Search

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

Ellen C. Ebert

2009-01-01

371

Mimics and rare presentations of pediatric demyelination.  

PubMed

This article reviews the features that should prompt consideration of diseases that mimic acquired demyelinating syndromes and multiple sclerosis using vignettes to highlight unusual clinical and radiologic features. Cases of transverse myelitis, spinal infarction, acute disseminated encephalomyelitis, fever-induced refractory epileptic encephalopathy in school-aged children, small-vessel vasculitis, Griscelli syndrome type 2, cysticercosis, vitamin B12 deficiency, and chronic relapsing inflammatory optic neuropathy are presented. PMID:23608693

O'Mahony, Julia; Shroff, Manohar; Banwell, Brenda

2013-05-01

372

Oral manifestations of giant cell arteritis  

Microsoft Academic Search

Summary  Giant cell arteritis is a systemic disease of unknown origin characterized by vasculitis of mediumsized and larger vessels,\\u000a found mainly in elderly women. It presents with a variety of symptoms, including temporal pain and tenderness, headache, and\\u000a neuro-ophthalmic features. This report describes a case of giant cell arteritis in a 78-year-old woman with a large ulceration\\u000a on the right side

J. E. van der Wal; I. van der Waal

1997-01-01

373

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

374

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

375

HLA-B?5101 in Greek Patients with Behçet’s Disease  

Microsoft Academic Search

Behçet’s disease (BD) is a recurrent systemic vasculitis of unknown etiology. Genetic factors and infectious agents seem to be related to the etiology and pathogenesis of the disease. BD is strongly associated with HLA-B51 antigen in many ethnic groups. As there are differences in HLA profile in different ethnic groups, we designed this case-control study to examine the association of

Yvonni Koumantaki; Catherine Stavropoulos; Marylin Spyropoulou; Helen Messini; Miltos Papademetropoulos; Ernestini Giziaki; Nicolaos Marcomichelakis; Gerasimos Palimeris; Phaedon Kaklamanis; Evangelia Kaklamani

1998-01-01

376

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

Microsoft Academic Search

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

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

1988-01-01

377

Ketamine: An Introduction for the Pain and Palliative Medicine Physician  

Microsoft Academic Search

A history of an escalating chronic intractable pain in a patient with cryoglob- ulinemia, vasculitis, and severe cutaneous ulcerations is presented. A strategy of progressive, multi-agent, N-methyl-D-aspartate-receptor (NMDA-R) blockade that resulted in adequate pain control and a three-fold reduction in opioid con- sumption is described. Diagnostic process of neuropathic pain and the role of NMDA-R in the development of hyperalgesia

Tomasz Okon

2007-01-01

378

Value of F-18 FDG hybrid camera PET and MRI in early takayasu aortitis  

Microsoft Academic Search

.   Takayasu aortitis (TA) is a chronic inflammatory and fibrotic vasculitis of large- and medium-sized arteries. Early stages\\u000a of the disease show a panarteritis and inflammatory wall thickening of the aorta and its branches, whereas advanced (fibrotic)\\u000a stages comprise stenosis, aneurismatic transformation and occlusion. Magnetic resonance imaging visualises early-stage disease\\u000a with high accuracy and is considered to be the method

J. Meller; E. Grabbe; W. Becker; R. Vosshenrich

2003-01-01

379

[Acute haemorrhagic oedema of infancy.  

PubMed

This is a case report of acute haemorrhagic oedema of infancy. An 11-month-old boy with a prolonged relapsing course within three weeks with fever and an increase in CRP necessitating antibiotic treatment. This disease is a leukocytoclastic vasculitis sharing features with Schönlein-Henoch purpura, but with a more benign self-limiting course with minimal risk of organ involvement. The characteristic distribution and appearance of the cutaneous symptoms often allows a clinical diagnosis of the condition. PMID:25294333

Knakkergaard, Anna; Thelle, Thomas

2014-09-22

380

Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report.  

PubMed

Henoch-Schonlein purpura (HSP) is a multisystem disease and immunoglobulin A-mediated vasculitis with a self-limited course affecting the skin, joints, gastrointestinal tract, and kidneys. Severe renal and central nervous system disease may lead to life-threatening conditions, and immunosuppressive agents and plasmapheresis may be needed. We report successful management of a 6-year-old patient with HSP for drainage of cervical lymphadenitis. PMID:24843348

Hasija, Neha; Taxak, Susheela; Bhardwaj, Mamta; Vashist, Kirti

2014-04-01

381

PFAPA Syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis)  

Microsoft Academic Search

:   This paper aims to remind paediatric clinicians to suspect and confirm ‘PFAPA’ syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years\\u000a 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall’s\\u000a criteria. The

Wen-I Lee; Mei-Hui Yang; Kam-Fai Lee; Li-Chen Chen; Syh-Jae Lin; Kuo-Wei Yeh; Jing-Long Huang

1999-01-01

382

Hearing organ disorders in patients with systemic sclerosis  

Microsoft Academic Search

In systemic sclerosis (SSc), there may develop hearing and balance disorders as a result of the immune-mediated vasculitis\\u000a and fibrosis in the inner ear. The objective of the study was evaluation of the hearing organ function in patients with SSc\\u000a with relationship to duration of the disease and Raynaud phenomenon and also to type and severity of the disease. Twenty

Katarzyna Maciaszczyk; El?bieta Waszczykowska; Anna Pajor; Bo?ena Bartkowiak-Dziankowska; Tomasz Durko

383

Bilateral--otitis media, dacroadenitis, parotitis and left sided infranuclear facial palsy: unusual manifestations of Wegener's granulomatosis.  

PubMed

Wegener's granulomatosis is a systemic vasculitis of unknown aetiology. Although it classically involves the upper respiratory tract, lungs and kidneys, virtually any organ may be affected. We report a rare case of a 45 year old female who presented with bilateral-dacroadenitis, otitis media, parotid enlargement and left sided lower motor neuron type of facial palsy as the initial manifestations of Wegener's granulomatosis. PMID:24968554

Rajiv, Keni Ravish; Balaji, G; Nagabushan, M V; Reddy, Y J Visweswara

2013-12-01

384

Manifestations cutanées des syndromes myélodysplasiques  

Microsoft Academic Search

Purpose. – Our purpose was to describe cutaneous manifestations associated with myelodysplastic syndromes.Methods. – Data from seven patients with cutaneous vasculitis (four cases), neutrophilic dermatosis (one case), relapsing polychondritis (one case), and possible erythema elevatum diutinum (one case) in association with myelodysplastic syndrome (refractory anaemia RA, RA with excess of blasts – RAEB-, RAEB in transformation RAEBt, chronic myelomonocytic leukaemia – CMML-), and analysis of the

J. J Morand; E Lightburn; M. A Richard; S Hesse-Bonerandi; F Carsuzaa; J. J Grob

2001-01-01

385

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

Microsoft Academic Search

Behcet’s disease is a complex multisystemic chronic inflammatory disease that is characterized by oral and genital aphtous\\u000a ulcers and vasculitis. Aneurysms of major arteries are the most important cause of mortality in Behcet’s disease. Four patients\\u000a with superior mesenteric artery (SMA) aneurysms related to Behcet’s disease have been reported in the literature. We report\\u000a here the first successful endovascular treatment

Koray Güven; Izzet Rozanes; Murat Kayabal?; Özenç Minareci

2009-01-01

386

Cerebral Aspergillus Infections and Meningitis  

Microsoft Academic Search

\\u000a Cerebral aspergillosis is notoriously difficult to treat and increasingly recognised in immunocompromised patients. These\\u000a infections frequently result in brain abscess formation, but can also cause cerebral embolism or vasculitis, fungal aneurysms,\\u000a cerebral granuloma or meningitis. Cerebral aspergillosis carries a particular poor prognosis with an almost 100% mortality\\u000a in patients treated with either amphotericin B or itraconazole. Penetration of most available

Stefan Schwartz

387

Inflammatory Diseases of the Meninges  

Microsoft Academic Search

Neuroimaging is of major importance in all cases of suspected infectious meningitis in order to get quick information about\\u000a the extent of the disease, typical lesion patterns, and potential complications, such as hydrocephalus, involvement of the\\u000a underlying brain parenchyma, or vasculitis. In bacterial meningitis, abnormal and asymmetrical enhancement of the leptomeninges\\u000a and the subarachnoid space is typical. Initial neuroimaging has

Stefan Rohde

388

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

PubMed Central

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

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

2011-01-01

389

Granuloma annularis revealing Wegener's granulomatosis.  

PubMed

Skin manifestations are often associated with systemic autoimmune diseases (SAD). Some SAD, such as systemic lupus erythematosus, psoriatic arthritis and scleroderma display pathognomonic dermatological features, whereas other systemic diseases such as sarcoidosis, vasculitis and rheumatoid arthritis can present with non-specific skin manifestations that range from erythema nodosum to necrotic lesions. Here we report the case of a 25-year-old man with uveitis, polyarthrirtis, pulmonary involvement, nephrotic syndrome, cutaneous granuloma and pneumonia by E. coli. PMID:25033485

Del Porto, F; Proietta, M; Muscianese, M; Tamburi, F; Cifani, N; Ferri, L; Nisticň, S; Bottoni, U; Bruno, G; Pranteda, G

2014-01-01

390

Granulomatosis with polyangiitis and constrictive pericarditis - a case report.  

PubMed

Polyangiitis with granulomatosis, previously known as Wegener's granulomatosis, is a systemic necrotising granulomatous vasculitis. It predominantly affects the upper and lower respiratory tracts and the kidneys, but can potentially affect any organ system. It is diagnosed by clinical features, immunology (anti-neutrophil cytoplasmic antibodies) and histology. Cardiac involvement occurs in 6 to 44% of cases. We present a case of polyangiitis with granulomatosis and constrictive pericarditis, which occurred despite vigorous immunosuppression and which required surgical pericardectomy. PMID:25516896

Horne, A E; Henriksen, P A; Amft, E N

2014-12-01

391

An adolescent with both Wegener's Granulomatosis and chronic blastomycosis  

PubMed Central

We report a case of Wegener's Granulomatosis (WG) associated with blastomycosis. This appears to be the first case report of WG co-existing with a tissue proven blastomycosis infection. The temporal correlation of the two conditions suggests that blastomycosis infection (and therefore possibly other fungal infections), may trigger the systemic granulomatous vasculitis in a predisposed individual; a provocative supposition warranting further study. PMID:18673582

Abdwani, Reem; Houghton, Kristin; Dobson, Simon; O' Sullivan, Maureen; Malleson, Peter

2008-01-01

392

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

393

Henoch-Schönlein purpura presenting as terminal ileitis and complicated by thrombotic microangiopathy.  

PubMed

We report here on a 40-year-old woman with abdominal pain, low-grade fever, and diarrhea in whom the cutaneous features of Henoch-Schönlein purpura (HSP) appeared only a few days after acute abdominal symptoms. Endoscopy showed terminal ileitis, and histopathological examination of a biopsy of the ileum showed a picture of IgA vasculitis. The clinical course was further complicated by the development of microangiopathic hemolytic anemia, thrombocytopenia, and severe renal failure. PMID:16275547

Al-Toma, Abdulbaqi A; Brink, Menno A; Hagen, E C

2005-11-01

394

A Rare Cause of Pericardial Effusion: Giant Cell Arteritis  

PubMed Central

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

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

2014-01-01

395

The evolution of a case of overlap syndrome with systemic sclerosis, rheumatoid arthritis and systemic lupus erythematosus.  

PubMed

An unusual case of overlap syndrome which evolved over a 12-year period is described. The patient initially presented with limited cutaneous systemic sclerosis. She then developed seropositive erosive rheumatoid arthritis and subsequently vasculitis with positive lupus serology. There was no evidence that she had mixed or undifferentiated connective tissue disease, and antibody to ribonuclearprotein was negative. This unusual combination of connective tissue disorders in one patient is reported and the literature is reviewed. PMID:1450805

Gendi, N; Gordon, T; Tanner, S B; Black, C M

1992-11-01

396

Behçet’s Syndrome  

Microsoft Academic Search

Behçet’s syndrome is a systemic vasculitis with an unknown etiology affecting the small and large vessels of the venous and\\u000a arterial systems. At least two clusters of disease expression have been described. The first includes superficial vein thrombosis,\\u000a deep vein thrombosis, and dural sinus thrombi. The second includes acne, arthritis, and enthesitis. The presence of these\\u000a clusters suggests there may

Yusuf Yazici; Sebahattin Yurdakul; Hasan Yazici

2010-01-01

397

[Hemoptysis revealing pulmonary artery aneurysm associated with intracardiac thrombosis: a delicate anticoagulation situation (a case report of Behçet's disease)].  

PubMed

Behçet's disease is a vasculitis affecting both arteries and veins. Cardiac involvement is less well known. The association of an aneurysm of the pulmonary artery and intracardiac thrombosis is rare, and a therapeutic challenge. We report the case of a 26-year-old patient hospitalized for moderately abundant hemoptysis and New York Heart Association (NYHA) class III dyspnea, which illustrates the difficulty encountered when using anticoagulants in this complex situation. PMID:23410873

Bouzelmat, H; Chaib, A; Kheyi, J; Kotni, M; Ghafir, D; Moustaghfir, A

2013-05-01

398

Atherosclerosis associated with vasculopathic lesions in a golden retriever with hypercholesterolemia  

PubMed Central

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

Boynosky, Nicole A.; Stokking, Laura

2014-01-01

399

Transfer of human leukocytes into double-knockout Pfp-/-Rag2-/- mice grafted with human skin: increased accumulation of neutrophils in human dermal microvessels.  

PubMed

Severe combined immunodeficient mice reconstituted with human leukocytes have been useful to model parts of the human immune system, including some of its diseases (e.g., AIDS). Because no human polymorphonuclear leukocytes (huPMN) develop in these xenograft models, diseases such as several forms of vasculitis cannot be modeled using this approach. To provide such a model for vasculitis, human skin patches were grafted onto double-knockout Pfp(-/-)Rag2(-/-) mice, which not only lack functional T and B cells but which are also devoid of natural killer cells. After intravenous injection, a high proportion of huPMNs survived within the circulation and accumulated in the human blood vessels. The accumulation increased considerably after the endothelium of the skin patches had been stimulated by tumor necrosis factor-alpha. Alpha mild perivascular neutrophilic infiltration and vascular necrosis was observed in the microvessels of the skin patches. Thus, a xenograft model of vasculitis with predominant huPMNs infiltration has been established for the first time. PMID:15599322

Ullrich, Sebastian; Schumacher, Udo; Ai, Maixing; Tiemann, Bastian; Gay, Steffen; Schechner, Jeffery S; Pober, Jordan S; Gross, Wolfgang L; Csernok, Elena

2004-11-27

400

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

401

Contribution to the pathogenesis of radiation-induced injury to large arteries.  

PubMed

We report a case of a 35-year-old man who died of a brain infarct 20 months after radiotherapy for carcinoma of the tonsil with metastases to the cervical lymph nodes. Histology revealed mild atherosclerosis, necrotizing vasculitis, and occlusive thrombosis of the internal carotid artery. Significant changes were observed in the vasa vasorum: swelling and detachment of the endothelium, subendothelial oedema, hyaline change, fibrinoid necrosis of the vessel walls with mononuclear cellular infiltration, accompanied by focal haemorrhages and chronic inflammation in the periadventitial soft tissue. We believe that these changes of the vasa vasorum and necrotizing vasculitis are causally related and that vasculitis represents focal ischaemic necroses with inflammatory reaction. Our findings support the hypothesis, based on experimental studies, that injury to the vasa vasorum is an important mechanism in the development of radiation-induced vasculopathy of large arteries. They also suggest an evolution of the injury to the vasa vasorum and periadventitial tissue from the early lesions described in our patient, to late stages resulting in dense periadventitial fibrosis as reported previously. We suggest that injury to the vasa vasorum and the consequent ischaemic lesions of the arterial wall are morphological features distinguishing radiation-induced arterial injury from spontaneous atherosclerosis. PMID:9425496

Zidar, N; Ferluga, D; Hvala, A; Popovi?, M; Soba, E

1997-10-01

402

Diagnosis and classification of autoimmune orchitis.  

PubMed

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

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

2014-01-01

403

Vascular damage in giant cell arteritis  

PubMed Central

Immune-mediated damage to medium-sized arteries results in wall remodeling with intimal hyperplasia, luminal stenosis and tissue ischemia. In the case of the aorta, vasculitis may result in dissection, aneurysm or rupture. The response-to-injury program of the blood vessel is a concerted action between the immune system and wall-resident cells, involving the release of growth and angiogenic factors from macrophages and giant cells and the migration and hyperproliferation of vascular smooth muscle cells. Innate immune cells, specifically, dendritic cells (DC) positioned in the vessel wall, have been implicated in the earliest steps of vasculitis. Pathogen-derived molecular patterns are capable of activating vascular DC and initiating adaptive immune responses. The pattern of the emerging vessel wall inflammation is ultimately determined by the initial insult. Ligands to toll-like receptor (TLR) 4, such as lipopolysaccharides, facilitate the recruitment of CD4 T cells that invade deep into the wall and distribute in a panarteritic pattern. Conversely, ligands for TLR5 condition vascular DC to support perivasculitic infiltrates. In essence, both innate and adaptive immune reactions collaborate to render the arterial wall susceptible to inflammatory damage. Unique features of the tissue microenvironment, including specialized DC, shape the course of the inflammatory response. Differences in vascular damage pattern encountered in different patients may relate to distinct instigators of vasculitis. PMID:19657775

PIGGOTT, KISHA; BIOUSSE, VALERIE; NEWMAN, NANCY J.; GORONZY, J?RG J.; WEYAND, CORNELIA M.

2014-01-01

404

Wegener's granulomatosis: an update on diagnosis and therapy.  

PubMed

Wegener's granulomatosis (WG) is a unique clinicopathological disease characterized by necrotizing granulomatous vasculitis of the respiratory tract, pauci-immune necrotizing glomerulonephritis and small-vessel vasculitis. Owing to its wide range of clinical manifestations, WG has a broad spectrum of severity that includes the potential for alveolar hemorrhage or rapidly progressive glomerulonephritis, which are immediately life threatening. WG is associated with the presence of circulating antineutrophil cytoplasm antibodies (c-ANCAs). The most widely accepted pathogenetic model suggests that c-ANCA-activated cytokine-primed neutrophils induce microvascular damage and a rapid escalation of inflammation with recruitment of mononuclear cells. The diagnosis of WG is made on the basis of typical clinical and radiologic findings, by biopsy of involved organ, the presence of c-ANCA and exclusion of all other small-vessel vasculitis. Currently, a regimen consisting of daily cyclophosphamide and corticosteroids is considered standard therapy. A number of trials have evaluated the efficacy of less-toxic immunosuppressants and antibacterials for treating patients with WG, resulting in the identification of effective alternative regimens to induce or maintain remission in certain subpopulations of patients. Recent investigation has focused on other immunomodulatory agents (e.g., TNF-alpha inhibitors and anti-CD20 antibodies), intravenous immunoglobulins and antithymocyte globulins for treating patients with resistant WG. PMID:20477576

Manna, R; Cadoni, G; Ferri, E; Verrecchia, E; Giovinale, M; Fonnesu, C; Calň, L; Armato, E; Paludetti, G

2008-07-01

405

Assessment of anti-endothelial cell antibodies in systemic sclerosis and Sjögren's syndrome  

PubMed Central

OBJECTIVES—Anti-endothelial cell antibodies (AECA) have been detected in 19 to 30% of patients with systemic sclerosis (SSc). The objective of this study was first to assess the role of a secondary Sjögren's syndrome (SS) in the occurrence of AECA in SSc. Secondly, we researched AECA in patients with primary SS, and investigated whether AECA were associated with vascular manifestations (Raynaud's phenomenon and vasculitis).?METHODS—IgG-AECA were tested by an ELISA method in serum samples from 50 patients with SSc (16 of them had also a secondary SS), 50 patients with primary SS, and 50 healthy controls.?RESULTS—AECA levels were significantly higher in patients with SSc or primary SS than in healthy controls (p < 0.01 and p < 0.01, respectively). In patients with SSc, AECA values were significantly higher in patients with secondary SS (p < 10?5). In patients with primary SS, AECA levels were significantly higher in patients with Raynaud's phenomenon (p < 0.01), but not in patients with vasculitis.?CONCLUSION—In patients with SSc, AECA are associated with a secondary SS. In patients with primary SS, AECA are associated with Raynaud's phenomenon, but not with vasculitis.?? PMID:9165994

Hebbar, M.; Lassalle, P.; Delneste, Y.; Hatron, P.; Devulder, B.; Tonnel, A.; Janin, A.

1997-01-01

406

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

407

Giant coronary artery aneurysms in juvenile polyarteritis nodosa: a case report  

PubMed Central

Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN. PMID:22222048

2012-01-01

408

Pulmonary-renal syndromes.  

PubMed

Pulmonary-renal syndromes or lung-kidney syndromes are clinical syndromes defined by a combination of diffuse alveolar haemorrhage (DAH) and glomerulonephritis. Pulmonary-renal syndromes are not a single entity, but are caused by a wide variety of diseases, including various forms of primary systemic vasculitis (especially Wegener's granulomatosis and microscopic polyangiitis), Goodpasture's syndrome (associated with autoantibodies to the alveolar and glomerular basement membrane) and systemic lupus erythematosus. The diagnosis rests on the identification of particular patterns of clinical, radiologic, pathologic and laboratory features. Serologic testing is important in the diagnostic work-up of patients presenting with a pulmonary-renal syndrome. The majority of cases of pulmonary-renal syndrome are associated with ANCAs, either c-ANCA or p-ANCA, due to autoantibodies against the target antigens proteinase-3 and myeloperoxidase respectively. The antigen target in Goodpasture's syndrome is type IV collagen, the major component of basement membranes. Diffuse alveolar haemorrhage is characterized by the presence of a haemorrhagic bronchoalveolar lavage (BAL) in serial BAL samples. In the clinical setting of an acute nephritis syndrome, percutaneous renal biopsy is commonly performed for histopathology and immunofluorescence studies. Treatment of generalized ANCA-associated vasculitis consists of corticosteroids and immunosuppressive agents such as cyclophosphamide (as induction therapy) or azathioprine (as maintenance therapy once remission has been achieved). The combination of plasmapheresis with these cytotoxic agents and steroids is effective in patients with Goodpasture's syndrome, especially if instituted early in the course of the disease. Recent evidence suggests that patients with severe ANCA-associated vasculitis, defined by the presence of diffuse alveolar haemorrhage and/or severe renal involvement (creatinine concentration > 5.7 mg/dl), might benefit from plasma exchange in combination with cyclophosphamide and corticosteroids. PMID:17547289

Brusselle, G G

2007-01-01

409

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

410

An audit of ANCA in routine clinical practice.  

PubMed Central

We have reviewed the medical records of 301/327 consecutive patients in whom anti-neutrophil cytoplasmic antibodies (ANCA) were detected by the Regional Immunology Laboratory in Northern Ireland between January 1988 and October 1991 (45 months). We have collected data for each patient regarding age, sex, smoking habit, area of residence, and details of any other autoantibody activity. Clinical diagnosis was established, with the number of organ systems involved and the evidence for that involvement (symptomatic, biochemical, radiological, and histological). Diagnoses were divided into four groups according to their recognised vasculitic features and these were related to the pattern of immunofluorescence and maximum ANCA titre detected. The most frequent diagnosis was rheumatoid arthritis (18.2% of patients) and the connective tissue disorders as a whole accounted for 27.9% of patients. ANCA were also detected in a wide range of clinical conditions which are not associated with vasculitis and these patients were an important source of 'false-positives'. The positive predictive value (PPV) of ANCA of all patterns and titres for vasculitic conditions was 27%, however, the detection of a classical ANCA pattern at high titre (> or = 1:640) was associated with an increased PPV of 75%. The coexistence of an antinuclear antibody (ANA) reduces the PPV of both classical and perinuclear ANCA, although perinuclear ANCA with antimyeloperoxidase specificity had an improved PPV. We conclude that ANCA testing should not be used as the only screening investigation for vasculitis but should be included in a rational investigative scheme. The interpretation of a positive ANCA result must take into account the presence of other autoantibodies and the full range of non-vasculitic conditions when the clinical situation is not typical of vasculitis. PMID:8545289

Edgar, J. D.; McMillan, S. A.; Bruce, I. N.; Conlan, S. K.

1995-01-01

411

[Infective endocarditis due to high level aminoglycoside resistant Enterococcus faecalis and methicillin resistant coagulase-negative staphylococci presenting with rheumatic manifestations].  

PubMed

Infective endocarditis has variable clinical presentations and may present with rheumatologic manifestations. Infective endocarditis due to high level aminoglycoside resistant enterococci represents a severe therapeutic challenge as none of the currently recommended treatment regimens are bactericidal against these isolates. In this report, a case of infective endocarditis with double aetiology, high level aminoglycoside resistant Enterococcus faecalis together with methicillin-resistant coagulase-negative staphylococci (MR-CNS), presenting with leukocytoclastic vasculitis and rapidly progressive glomerulonephritis, has been presented. A 48-years-old woman was admitted to our hospital with malaise and non-pruritic purpural rush on her lower extremities. On admission she had no fever or leukocytosis. Skin biopsy showed leukocytoclastic vasculitis and steroid therapy was started. On 12th day of admission rapidly progressive glomerulonephritis was diagnosed and she received plasmapheresis and haemodialysis support. Transthoracic echocardiography (TTE) demonstrated 1 x 1.5 cm vegetation on the mitral valve. An initial diagnosis of infective endocarditis was made and empirical treatment with vancomycin and gentamicin was started. All blood cultures yielded high level aminoglycoside resistant E. faecalis and additionally two of them yielded MR-CNS. Vancomycin was administered in combination with high dose ampicillin and repeated blood cultures taken after administration of ampicillin, revealed no growth. The patient remained afebrile, renal functions improved and a repeat TTE done on 20th day of ampicillin therapy showed waning of the vegetation. On 42nd day of treatment repeat TTE showed new vegetation on the mitral valve and severe valve insufficiency, so the patient was scheduled for mitral valve replacement. She was treated for 12 weeks with vancomycin and ampicillin and recovered successfully. In conclusion; infective endocarditis should be considered in the differential diagnosis of leukocytoclastic vasculitis and rapidly progressive glomerulonephritis. Physicians should document their treatment outcomes and experience with high level aminoglycoside resistant enterococcal infective endocarditis, which is a therapeutic challenge, so that the best therapeutic options can be identified. PMID:18822897

Pi?kin, Nihal; Akduman, Deniz; Aydemir, Hande; Celebi, Güven; Oztoprak, Nefise; Akta?, Elif

2008-07-01

412

Behçet's disease  

PubMed Central

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

Kontogiannis, V; Powell, R

2000-01-01

413

Pulmonary Mycobacterium massiliense disease with septicemia during immunosuppressive treatment.  

PubMed

A 75-year-old man with interstitial pneumonia due to ANCA-related vasculitis requiring immunosuppressive treatment was admitted to our hospital because of fever and rapidly progressive dyspnea. Chest CT showed diffuse ground-glass opacity with infiltration shadow in the bilateral lungs. We established a definitive diagnosis by isolating Mycobacterium massiliense on culture examination of acid-fast bacilli from peripheral blood and sputum. We began to administer CAM, LVFX, AMK, IPM/CS to this patient two weeks after admission. However, he died of respiratory failure and septic shock. There are few case reports of pulmonary lesion with septicemia due to Mycobacterium massiliense. PMID:21532236

Kobashi, Yoshihiro; Mouri, Keiji; Obase, Yasushi; Miyashita, Naoyuki; Nakanaga, Kazue; Oka, Mikio

2011-01-01

414

Riluzole-induced lung injury in two patients with amyotrophic lateral sclerosis.  

PubMed

Riluzole has recently been proven as the first effective drug for the treatment of amyotrophic lateral sclerosis (ALS). We report two rare cases of lung injury caused by riluzole therapy in patients with ALS. Chest radiographs showed bilateral lower lobe, dorsal-dominant ground glass opacity, and/or consolidation. A drug lymphocyte stimulation test (DLST) of peripheral blood or bronchoalveolar lavage cells was positive for riluzole. Histopathological examination of lung biopsy specimens revealed lung injury without fungoid granuloma, vasculitis, or diffuse alveolar damage. To the best of our knowledge, this is the first report of riluzole-induced lung injury with positive DLST results. PMID:22821110

Kakuta, Takuya; Hirata, Hirokuni; Soda, Sayo; Shiobara, Taichi; Watanabe, Mineaki; Tatewaki, Masamitsu; Fukushima, Fumiya; Chibana, Kazuyuki; Sugiyama, Kumiya; Arima, Masafumi; Koichi, Honma; Fukuda, Takeshi; Fukushima, Yasutsugu

2012-01-01

415

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

PubMed Central

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

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

2011-01-01

416

Linezolid-induced purpuric medication reaction.  

PubMed

A 64-year-old Caucasian male was seen in consultation for a petechial eruption that began 9 days after he started linezolid therapy for a retroperitoneal abscess. Skin findings included confluent non-blanching petechiae and purpura covering his entire body, without any active bleeding. A punch biopsy from the left lateral arm was performed and showed a perivascular inflammatory infiltrate composed of lymphocytes, histiocytes and rare eosinophils, and extravasated erythrocytes. Changes of leukocytoclastic vasculitis were not noted. Linezolid was promptly discontinued. To our knowledge, this is the first report of a biopsy-proven purpuric medication reaction secondary to linezolid therapy. PMID:19519611

Kim, Flora S; Kelley, Walter; Resh, Brooke; Goldenberg, Gary

2009-07-01

417

[Critical limb ischemia in systemic sclerosis].  

PubMed

Vascular complications are common in systemic sclerosis (SSc). Critical limb ischemia leading to gangrene or amputation occurs in more than 10% of these patients and hence is a common emergency. This report highlights the different pathogenetic mechanisms leading to critical ischemic events and provides guidance for the diagnosis and therapy. Apart from SSc-associated vasculopathy and peripheral arterial disease, thromboembolic events and rarely also vasculitis may cause critical limb ischemia. An interdisciplinary approach to the diagnosis and therapy of these lesions is mandatory. Therapy goals are the prevention of further ischemia and, if possible, revascularization as well as optimal pain management. PMID:22538844

Becker, M O; Riemekasten, G

2012-06-01

418

Warfarin-induced skin necrosis diagnosed on clinical grounds and treated with maggot debridement therapy  

PubMed Central

A patient with a history of deep vein thrombosis presented with painful bruising and blistering on his left leg 7–10?days after warfarin treatment. A complicated 2-month treatment followed, where vasculitis was originally diagnosed from histological findings before the final diagnosis of warfarin-induced skin necrosis (WISN) was made on clinical grounds. Warfarin was stopped, reversed and low molecular weight heparin started but, the lesions had progressed to full thickness necrosis. This was originally treated with conventional surgical debridement before introducing maggot debridement therapy (MDT) in an effort to try to salvage the limb. PMID:23362073

Biscoe, Anna Louise; Bedlow, Alison

2013-01-01

419

Clinical immunology - Autoimmunity in the Netherlands.  

PubMed

Clinical immunology is in the Netherlands a separate clinical specialty within internal medicine and pediatrics. Clinical immunologists work closely together with nephrologists, rheumatologists and many other medical specialists. Apart from research and teaching, clinical immunologists are taking care of patients with immune-deficiencies, vasculitides and systemic auto-immune diseases. Clinical immunology in the Netherlands has always been an important contributor to basic and clinical science in the Netherlands. Major scientific contributions were made in the field of Systemic Lupus Erythematosus and ANCA associated vasculitis. These Dutch contributions will be reviewed in this article. PMID:25455600

Tervaert, Jan Willem Cohen; Kallenberg, Cees G M

2014-12-01

420

[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

421

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

PubMed Central

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

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

2012-01-01

422

Wegener's granulomatosis complicated by central diabetes insipidus and peripheral neutrophy with normal pituitary in a patient.  

PubMed

Wegener's granulomatosis (WG) is a necrotizing systemic vasculitis that any organ system can be involved in. We report a patient who was hospitalized with recurrent nodules in pulmonary computed tomograph and symptoms such as intermitted fever, polydipsia, insensibility and pain on extremities. Laboratory investigation showed positive antineutrophil cytoplasmic antibody in a cytoplastic pattern. The histopathologic result of the lung nodule revealed multiloci necrosis of lung tissue accompanied with large amount of neutrophils. She was diagnosed as WG with multi-systemic involvements and almost recovered on 6 months' prednisone and cyclophosphamide treatment. PMID:19172275

Xue, Jing; Wang, Huiying; Wu, Huaxiang; Jin, Qiaofei

2009-08-01

423

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

424

Splenic Infarct Due to Scrub Typhus  

PubMed Central

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

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

2014-01-01

425

[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

426

Motor Branch Biopsy of the Pronator Teres Muscle in a Patient with Painful Forearm Neuropathy  

PubMed Central

Histological evaluation of a peripheral nerve is often the final diagnostic work-up for a neuropathy of unknown origin, and a distal sensory nerve is usually biopsied. Here, we report the case of a female patient with painful unilateral neuropathy in the upper arm. According to the histological evaluation of the pronator teres motor branch, vasculitis seemed to be the most probable cause of the condition, and steroid therapy improved the patients’ symptoms. A biopsy of the motor branch of the pronator teres muscle nerve may be considered a valuable diagnostic option in selected cases with neuropathy affecting the upper limb, when performed in cooperation with neurologists and orthopedic surgeons. PMID:25232332

Kinoshita, Tomomi; Fukushima, Kazuhiro; Abe, Ryu-ta; Ogawa, Yuka; Nakagawa, Michitaka; Katoh, Nagaaki; Yoshida, Takuhiro; Kato, Hiroyuki; Ikeda, Shu-ichi

2014-01-01

427

The ocular manifestations of Rocky Mountain spotted fever.  

PubMed

To our knowledge, this is the first report of an iris nodule in a case of Rocky Mountain spotted fever (RMSF). The iris nodule may be similar to the "typhus nodule" of the central nervous system reported at autopsy in patients with typhus rickettsial disease. Previous case reports of RMSF have emphasized either anterior uveitis or retinal vasculitis. In the case reported here, both occurred in our patient as a result of RMSF. Also, we review the ophthalmic literature on rickettsial disease. PMID:3662314

Duffey, R J; Hammer, M E

1987-08-01

428

[How to treat systemic necrotizing vasculitides?].  

PubMed

Corticosteroids and, according to severity or vasculitis type, cytotoxic agents are prescribed to treat systemic necrotizing vasculitides. Usually, in most vasculitides, treatment is prescribed for at least 18 months. Treatment is able to obtain a remission in approximatively 90% of patients. Relapse rate is high. Cyclophosphamide is usually prescribed for a few months, and administered by pulses. A maintenance treatment is compulsory and comprises usually azathioprine and methotrexate. Biologics can be proposed as ad on therapy like intravenous immunoglobulins and anti-TNF. Rituximab, an anti-CD20 drug is as effective as cyclophosphamide. It can be also prescribed to maintain remission. PMID:22906773

Guillevin, Loďc

2012-10-01

429

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

430

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

431

Patient stratification and genomics: flares, fizzlers and foxes  

E-print Network

INVITED SPEAKER PRESENTATION Open Access Patient stratification and genomics: flares, fizzlers and foxes Kenneth GC Smith From 7th European Workshop on Immune-Mediated Inflammatory Diseases Noordwijk aan Zee, the Netherlands. 28-30 November 2012... , Rees AJ, Clayton DG, Smith KGC: Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med 2012, 367:214-223. 2. McKinney EF, Lyons PA, Carr EJ, Hollis JL, Jayne DRW, Willcocks LC, Koukoulaki M, Hatton A, MacAry PA, Brazma A, Chaudhry...

Smith, Kenneth GC

2012-11-28

432

Diagnostic algorithm for Raynaud's phenomenon and vascular skin lesions in systemic lupus erythematosus.  

PubMed

Skin discolorations and skin lesions due to vascular pathologies are common clinical features in systemic lupus erythematosus. A variety of clinical manifestations such as Raynaud's phenomenon, acrocyanosis, livedo patterns, erythematous or violaceous macules and papules or necrosis are triggered by heterogeneous pathophysiological mechanisms such as vasospasm, vasculitis or thromboembolism. A standardized macro- and microvascular assessment is necessary to establish the correct diagnosis. We describe and illustrate common clinical features of vascular skin manifestations in systemic lupus erythematosus and present a diagnostic algorithm. PMID:20693202

Richter, J G; Sander, O; Schneider, M; Klein-Weigel, P

2010-08-01

433

Case of livedoid vasculopathy with peripheral neuropathy successfully treated with low-dose warfarin.  

PubMed

We report herein a case of a 28-year-old woman with persistent livedo racemosa and recurrent ulcerations on the lower extremities. The clinical presentation, together with histopathological findings of vascular occlusion without overt vasculitis in the dermis, led to the diagnosis of livedoid vasculopathy. The patient experienced recurrence of ulcerations and developed peripheral neuropathy affecting the distal extremities during the course of treatment with sarpogrelate hydrochloride. The skin lesions and neurological symptoms improved dramatically after adding low-dose warfarin potassium to the treatment regimen. This case suggests that administration of low-dose warfarin is an effective therapy of choice for patients with livedoid vasculopathy. PMID:20175830

Osada, Shin-Ichi; Kimura, Yoichi; Kawana, Seiji

2010-01-01

434

[Livedoid vasculopathy. Pathogenesis, diagnosis and treatment of cutaneous infarction].  

PubMed

Livedo vasculopathy is a chronic recurrent disease of the cutaneous circulation and is characterized by episodic occurrence of painful ulcerations of the lower leg. These heal slowly leaving small porcelain-white scars called atrophie blanche. Recent research has shown that livedoid vasculopathy is a coagulation disorder classified as a vasculopathy different from inflammatory vasculitis. Distinguishing between the disorders enhances the pathophysiologic understanding and supports the therapeutic rationale. The prevention of irreversible residual scarring is the main goal in treating cutaneous infarction; prompt treatment is required. PMID:21786003

Goerge, T

2011-08-01

435

Livedoid vasculopathy: thrombotic or inflammatory disease?  

PubMed

The pathogenesis of livedoid vasculopathy (LV) is still unclear. However, with increasing knowledge of disorders of coagulation over the past few years, the cause of some cases of LV has been elucidated. LV has now been described in association with hyperhomocysteinaemia, activated protein C resistance, and prothombin gene mutations in the absence of significant underlying inflammatory disease. When LV is seen in association with systemic lupus erythematosus or polyarteritis nodosa, it is probably due to the pro-coagulable state induced by these diseases rather than being true vasculitis. We review recent insights into LV provided by published clinical cases and discuss its pathogenesis. PMID:19874344

Khenifer, S; Thomas, L; Balme, B; Dalle, S

2010-10-01

436

Polyarteritis nodosa.  

PubMed

Polyarteritis nodosa (PAN) is a systemic disease, but variants are cutaneous PAN and single-organ disease. Histologic confirmation of vasculitis in medium-sized arteries is desirable, and biopsies should be obtained from the symptomatic and least invasive sites. Angiography can show multiple microaneurysms in the viscera. Treatment includes high-dose corticosteroids, which are combined with immunosuppressive agents when internal organs are involved and with life-threatening disease. Once remission is achieved, maintenance agents are initiated. PAN is becoming a rare disease. International collaborative efforts are under way to establish better diagnostic and classification for all vasculitides, including PAN. PMID:25399938

Forbess, Lindsy; Bannykh, Serguei

2015-01-01

437

[Systemic urticaria in 2014].  

PubMed

Systemic urticaria are defined as urticaria, most often chronic, associated with systemic diseases. At present time, urticarial vasculitis and neutrophilic urticarial dermatosis associated to autoinflammatory syndromes are not considered to be subtypes of chronic spontaneous urticaria due to their distinctly clinical and histological characteristics as well different pathomechanisms. Sometimes, chronic urticaria is associated to thyroid autoimmunity. However, the majority of cases of chronic spontaneous urticaria have no discernible cause and further investigations are not necessary, as already suggested by some authors and French consensus conference more than 10 years ago. PMID:25539679

Doutre, M-S

2014-11-01

438

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

439

[Spinal infarction in the anterior spinal territory with possible relation with bilharziasis].  

PubMed

We report a case of spinal cord infarction in the anterior spinal artery territory with selective involvement of the anterior horns. A 35-year-old Mauritanian woman was hospitalized because of an acute and severe flaccid paraplegia without any sphincter dysfunction or sensory disturbance. MRI abnormalities (hypersignal on T2-weighted sequences) were restricted to the anterior horns of the lower thoracic spinal cord and conus medullaris. Adamkiewicz's artery appeared abnormally thin at arteriography. The infarction was probably related to vasculitis of schistosomal origin. PMID:1962071

Liblau, R; Chiras, J; Orssaud, C; Dormont, D; Duclos, H; Gentilini, M

1991-01-01

440

Imaging Characteristics of Takayasu Arteritis  

SciTech Connect

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

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

2007-07-15

441

Safety and Efficacy of Vaccination Against Influenza in Patients With Rheumatoid Arthritis  

PubMed Central

Vaccination against influenza is currently recommended for patients with rheumatoid arthritis (RA). The safety and efficacy of vaccination in patients suffering from rheumatic diseases is still a matter of debate. This review summarizes the studies performed on the safety and immunogenicity of influenza vaccination in patients with RA as well as the rheumatic complications of the vaccine in otherwise healthy persons. Several trials have shown that the vaccine induces an adequate humoral response and does not induce clinical exacerbation of RA. Rheumatic complications (mainly vasculitis) following influenza vaccination in the general population are scarce. PMID:17162376

Elkayam, Ori

2006-01-01

442

[Unusual presentation of Wegener's granulomatosis].  

PubMed

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved. PMID:20605284

Jamoussi, S Ketari; Ben Dhaou, B; Boussema, F; Turki, E; Kochbati, S; Cherif, O; Maamar, M; Rokbani, L

2010-08-01

443

Gas6 promotes inflammation by enhancing interactions between endothelial cells, platelets, and leukocytes.  

PubMed

The role of Gas6 in endothelial cell (EC) function remains incompletely characterized. Here we report that Gas6 amplifies EC activation in response to inflammatory stimuli in vitro. In vivo, Gas6 promotes and accelerates the sequestration of circulating platelets and leukocytes on activated endothelium as well as the formation and endothelial sequestration of circulating platelet-leukocyte conjugates. In addition, Gas6 promotes leukocyte extravasation, inflammation, and thrombosis in mouse models of inflammation (endotoxinemia, vasculitis, heart transplantation). Thus, Gas6 amplifies EC activation, thereby playing a key role in enhancing the interactions between ECs, platelets, and leukocytes during inflammation. PMID:18156494

Tjwa, Marc; Bellido-Martin, Lola; Lin, Yuan; Lutgens, Esther; Plaisance, Stéphane; Bono, Françoise; Delesque-Touchard, Nathalie; Hervé, Caroline; Moura, Rute; Billiau, An D; Aparicio, Cristina; Levi, Marcel; Daemen, Mat; Dewerchin, Mieke; Lupu, Florea; Arnout, Jef; Herbert, Jean-Marc; Waer, Mark; García de Frutos, Pablo; Dahlbäck, Björn; Carmeliet, Peter; Hoylaerts, Marc F; Moons, Lieve

2008-04-15

444

Technetium scanning in Kaposi's sarcoma and its simulators  

SciTech Connect

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.

Gunnoe, R.; Kalivas, J.

1982-04-01

445

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

PubMed

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

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

2014-08-01

446

Rituximab seems a promising therapeutic option in granulomatosis with polyangiitis with intestinal perforation: a case report and literature review  

PubMed Central

Granulomatosis with polyangiitis (Wegener's) (GPA) is a chronic disease of unknown aetiology that leads to necrotising vasculitis in small and medium-sized vessels characterised by respiratory system and kidney involvement. Intestinal involvement is rare and perforation is even rarer in GPA. In this study, we are presenting a literature review of related cases, and a 29-year-old man referred from the emergency department with a multiple distal ileal perforation that was diagnosed with GPA, and successfully treated with rituximab. PMID:23345478

Dag, Muhammet Sait; Pehlivan, Yavuz; Tutar, Ediz; Kisacik, Bunyamin

2013-01-01

447

A case of polyarteritis nodosa with periurethralaseptic abscesses and testicular lesions.  

PubMed

We describe a 54-year-old man presenting with cutaneous ulcerations, livedo reticularis, numbness of the legs, and skin histological findings compatible with the diagnosis of polyarteritis nodosa (PAN). Initial treatment with 50 mg/day of prednisolone (PSL) was effective. However, the symptoms and signs recurred, and the patient developed multiple periurethral aseptic abscesses, urethra-cutaneous fistula, and testicular lesions after tapering of PSL therapy. The condition improved with PSL and cyclophosphamide administration. Since penile and testicular vasculitis could be associated with PAN, although rarely, we should carefully distinguish such an involvement from infection and malignancy. PMID:19210882

Watanabe, K; Nanki, T; Sugihara, T; Miyasaka, N

2008-01-01

448

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

PubMed Central

Giant cell arteritis (GCA) is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities. PMID:25400969

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

2014-01-01

449

The great imitator-still today! A case of meningovascular syphilis affecting the posterior circulation.  

PubMed

Cerebral ischemia due to meningovascular syphilis is rare and more frequently affects the anterior circulation than the posterior circulation. We describe clinical features and imaging studies of a 50-year-old patient with Parinaud syndrome and a syphilitic dorsal midbrain infarction. Brain magnetic resonance imaging indicated vasculitis of the posterior circulation. The diagnosis of meningovascular syphilis was established by serum and cerebrospinal fluid examinations. Although rare, because of the high impact on treatment, clinicians should always be aware of meningovascular syphilis in the differential diagnosis of stroke, particularly in young and male patients with cryptogenic stroke. PMID:25282187

Bäuerle, Jochen; Zitzmann, Anna; Egger, Karl; Meckel, Stephan; Weiller, Cornelius; Harloff, Andreas

2015-01-01

450

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

451

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

452

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

453

Ocular manifestations of the potentially lethal rheumatologic and vasculitic disorders.  

PubMed

Vision threatening ocular inflammation may occur in patients with any of the acquired connective tissue disorders and vasculitic diseases. Additionally, the ocular inflammation may be the presenting manifestation of the disease, which leads the patient to seek medical care. Other manifestations of the potentially lethal disease may be subtle or absent, presenting the thoughtful ophthalmologist with the opportunity to make life saving discoveries. Necrotizing scleritis, peripheral ulcerative keratitis, and retinal vasculitis are the ocular findings which should prompt the ophthalmologist to initiate very aggressive measures aimed at discovering any evidence of extra-ocular abnormalities, laboratory or otherwise. Appropriate therapy will be sight saving and may be life saving. PMID:23688612

Foster, C Stephen

2013-06-01

454

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

455

Hepatitis C-associated rheumatic disorders.  

PubMed

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

Buskila, Dan

2009-02-01

456

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

457

[Behçet's disease - ophthalmological and general aspects part I : etiology, pathogenesis and diagnostics].  

PubMed

Behçet's disease (also known as morbus Behcet or Admantiades-Behcet syndrome) is a chronic vasculitis mainly characterized by recurrent mucocutaneous lesions and sight threatening uveitis. It may also involve joints, vessels of all sizes and the central nervous system. Because of its severe morbidity and considerable mortality early diagnosis and treatment is important. Treatment and prognosis of this disorder have profited considerably in recent years following the introduction of biologic agents. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the diagnosis and therapy of Behçet's disease. PMID:23179819

Pleyer, U; Hazirolan, D; Winterhalter, S; Stübiger, N

2012-11-01

458

Giant aortic arch aneurysm complicating Kawasaki disease: an original case report  

PubMed Central

Summary 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, related reports on peripheral arterial and aortic aneurysms are scarce. We report an original case of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28 months- old patient diagnosed with Kawasaki Disease. This complication was managed by steroids therapy in the beginning than surgery was indicated because of mechanical complication and risk of rupture.

Kaouthar, Hakim; Rafik, Boussaada; Jihen, Ayari; Imen, Hamdi; Lilia, Chaker; Fatma, Ouarda; Hela, Msaad

2014-01-01

459

Splenic infarct due to scrub typhus.  

PubMed

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

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

2014-04-01

460

Takayasu's Arteritis and Crohn's Disease in a Young Hispanic Female  

PubMed Central

Takayasu's arteritis (TA) and Crohn's disease (CD) are chronic inflammatory granulomatous disorders of undetermined etiology. TA is a large vessel vasculitis with a predilection for the aorta and its branches in young women of Asian descent; whereas CD has characteristic gastrointestinal manifestations more prevalent in young Caucasians. We describe a case of both diseases in a young Hispanic female, review the literature, and impart new insight on possible genetic linkage and the role of interleukin 12 B (IL-12B) as the common autoimmune mechanism and potential therapeutic target in this rare disease combination. PMID:25152825

Saurabh, Shireesh; Tan, Irene J.

2014-01-01

461

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

PubMed

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/mm(3). 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

2014-11-01

462

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

PubMed

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

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

2000-01-01

463

A case report of cavernous sinus syndrome in a patient with Takayasu's arteritis.  

PubMed

Neurologists must entertain a broad differential diagnosis when considering a patient with cavernous sinus syndrome, including neoplasm, trauma, vascular causes, inflammatory processes, and infections. We report the case of a 37-year-old woman initially diagnosed with cavernous sinus syndrome, where subsequent investigations revealed findings of Takayasu's arteritis, a large vessel vasculitis. The patient also tested positive for perinuclear antineutrophil cytoplasmic antibodies, suggesting the possibility of a vasculitic spectrum disorder although no clinical features of Wegener's granulomatosis were present. Criteria for Takayasu's arteritis and its protean neurologic manifestations are reviewed. This case highlights the spectrum of vasculitic conditions that may be associated with cavernous sinus inflammation. PMID:24827146

Rotstein, Dalia L; Tyndel, Felix J; Tang-Wai, David F

2014-09-01

464

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

465

A case of fever of unknown origin: Necrotizing sarcoid granulomatosis.  

PubMed

Necrotizing sarcoid granulomatosis is a rare type of vasculitis; its etiology and pathogenesis are still unknown. The disease primarily affects the lungs, although extra-pulmonary involvement has been reported. The typical symptoms are cough, chest pain, dyspnea, and weight loss; high temperatures have been reported in rare cases. We present the case of a 65-year-old woman who was diagnosed with lymph node tuberculosis, for which she received treatment for six months. The patient experienced no improvement in her symptoms, which included fever, weakness and dyspnea. A re-evaluation of previously collected thoracoscopic biopsy material revealed compatibility with necrotizing sarcoid granulomatosis. PMID:25366946

Unlü, G; Ony?lmaz, T A; Bar??, S A; Turhan, N; Vural, C; Ba?yi?it, I; Boyac?, H

2014-01-01

466

Diabetic myonecrosis: uncommon complications in common diseases.  

PubMed

We report a case of sudden thigh pain from spontaneous quadriceps necrosis, also known as diabetic myonecrosis, in a 28-year-old patient with poorly controlled diabetes mellitus. Diabetic muscle infarction is a rare end-organ complication seen in patients with poor glycemic control and advanced chronic microvascular complications. Proposed mechanisms involve atherosclerotic microvascular occlusion, ischemia-reperfusion related injury,