Dataset Information

Clinical features and prognostic factors of Churg-Strauss syndrome.

ABSTRACT:

Background/aims

Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans.

Methods

Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011.

Results

Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not.

Conclusions

ANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.

SUBMITTER: Kim MY

PROVIDER: S-EPMC3932399 | biostudies-literature | 2014 Jan

REPOSITORIES: biostudies-literature

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Publications

Clinical features and prognostic factors of Churg-Strauss syndrome.

Kim Mi-Yeong MY Sohn Kyoung-Hee KH Song Woo-Jung WJ Park Heung-Woo HW Cho Sang-Heon SH Min Kyung-Up KU Kang Hye-Ryun HR

The Korean journal of internal medicine 20140102 1

<h4>Background/aims</h4>Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans.<h4>Methods</h4>Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital betwe ...[more]

PMID: 24574837

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Project description:Churg-Strauss syndrome (CSS) is a small vessel systemic vasculitis associated with asthma and eosinophilia that causes glomerulonephritis (GN) in ?25% of patients. Rituximab (RTX) is a chimeric anti-CD20 monoclonal antibody that depletes B cells and is effective in numerous autoimmune diseases including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We aim to evaluate the safety and efficacy of RTX in inducing remission of renal disease activity in patients with CSS.We conducted a single-center, open-label pilot study using RTX (375 mg/m(2)/week × 4) for induction of remission in CSS patients with renal involvement [defined as having >25% dysmorphic red cells, red blood cell casts or pauci-immune GN on biopsy]. Written informed consent was obtained from all individuals. Patients were eligible if they were untreated, had failed glucocorticoid therapy or had failed glucocorticoid dose reductions because of disease relapses. The primary outcome was remission of renal disease activity defined as stability or improvement of creatinine clearance, absence of active urinary sediment and reduction of the glucocorticoid dose to <50% of the average dose received over 3 months before enrollment or <10 mg/day (whichever is smaller) at 6 months. Patients were followed up for 1 year.Only three patients (two females; ages 54, 55 and 65) were enrolled. All patients had positive myeloperoxidase-ANCA and renal involvement. Two patients had biopsy-proven pauci-immune crescentic GN. All achieved the primary end point of renal remission within the first 3 months and remained in renal remission during the year following RTX treatment. One patient experienced a nonrenal relapse (eye and joint involvement) at 6 months coinciding with the reconstitution of CD19+ cells and eosinophilia. He was retreated with RTX and achieved remission within 6 weeks. No major adverse effects were recorded.In this pilot study, RTX was safe and successful in controlling renal disease activity in three patients with CSS. This agent deserves further study in CSS.

Project description:BackgroundMyocardial infarction is most commonly caused by thrombosis occurring on a background of coronary atherosclerosis, resulting in reduced coronary flow. Less often, myocardial infarction can occur in the absence of coronary disease. The pathomechanism of myocardial infarction in such patients is heterogeneous and more challenging to diagnose and treat. European Society of Cardiology published a position paper on myocardial infarction in patients with non-obstructive coronary disease, with definitions and recommendations for investigations, in what has hitherto been an under-recognized and under-investigated Cinderella-like condition. However, the importance of obtaining a diagnosis is all the more important, since one treatment approach with revascularization and antithrombotic treatment does not 'fit all'.Case summaryA 70-year-old male patient presented with chest pain at rest, associated with rise in troponin and without ECG changes. A diagnosis of non-ST elevation myocardial infarction was made. Coronary angiography showed a smooth stenosis which resolved with administration of intracoronary nitrate. A diagnosis of coronary artery spasm was made, and treatment initiated. After 18?months, the patient had recurrent chest pains at rest, unresponsive to glyceryl trinitrate (GTN). Cardiac magnetic resonance revealed extension of subendocardial infarction, without inducible ischaemia. CT coronary angiogram (CTCA) showed non-obstructive coronaries. Blood tests showed significant eosinophilia, raised troponin, and C-reactive protein (CRP) that fluctuated without correlation with symptoms or any ECG changes. A diagnosis of Churg-Strauss syndrome was made, and immunosuppression commenced.DiscussionChurg-Strauss syndrome is an autoimmune vasculitis in patients with history of atopy or late-onset asthma which when involving coronary arteries can lead to myocardial injury mimicking acute coronary syndrome (ACS). Identification is important to allow initiation of immunosuppression which can prevent development or progression.

Dataset Information

Clinical features and prognostic factors of Churg-Strauss syndrome.

Background/aims

Methods

Results

Conclusions

Publications

Clinical features and prognostic factors of Churg-Strauss syndrome.

Similar Datasets

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