Endothelial microparticles as a diagnostic aid in Churg-Strauss vasculitis-induced cardiomyopathy

Churg-Strauss Syndrome (CSS) is characterized by allergic rhinitis, asthma and prominent blood and tissue eosinophilia. Although CSS can affect any organ system, isolated cardiac manifestation is a rare feature that is often characterized by rapidly progressive congestive heart failure. We present t...

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Bibliographic Details
Published in:Clinical and experimental rheumatology 2008-05, Vol.26 (3), p.S86
Main Authors: KÜMPERS, P, ERDBRÜGGER, U, GROSSHEIM, M, MEYER, G. P, HISS, M, GWINNER, W, HAILER, H, HAUBITZ, M
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - therapeutic use
Biological and medical sciences
Cardiomyopathies - etiology
Cell-Derived Microparticles
Churg-Strauss Syndrome - complications
Churg-Strauss Syndrome - diagnosis
Churg-Strauss Syndrome - drug therapy
Computerized, statistical medical data processing and models in biomedicine
Coronary Angiography
Cyclophosphamide - therapeutic use
Diseases of the osteoarticular system
Eosinophilia
Female
Flow Cytometry
Humans
Immunosuppressive Agents - therapeutic use
Medical management aid. Diagnosis aid
Medical sciences
Middle Aged
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
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Summary:Churg-Strauss Syndrome (CSS) is characterized by allergic rhinitis, asthma and prominent blood and tissue eosinophilia. Although CSS can affect any organ system, isolated cardiac manifestation is a rare feature that is often characterized by rapidly progressive congestive heart failure. We present the case of a 48-year-old woman with acute dyspnoea and chest pain. Her past medical history was significant for asthma and frequently relapsing minimal-change glomerulonephritis. Echocardiogram and coronary angiography revealed cardiomyopathy and coronary small-vessel vasculitis in the presence of blood eosinophilia and elevated IgE. In the absence of infective agents, neoplastic diseases and further vasculitic manifestations, a flow cytometry-based analysis of markedly elevated endothelial microparticles supported the diagnosis of CSS. Cardiomyopathy resolved completely after initiation of immunosuppressive treatment with corticosteroids and cyclophosphamide pulses. Elevated endothelial, leukocytic and platelet-derived microparticles decreased during follow-up and closely paralleled vasculitic activity. Endothelial microparticles might be an additional tool to diagnose and monitor cases of suspected vasculitic cardiac involvement in CSS.
ISSN:0392-856X
1593-098X