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 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.
Kümpers
P
P
Department of Nephrology, Hannover Medical School, Hannover, Germany. keumpers.philipp@mh-hannover.de
Erdbrügger
U
U
Grossheim
M
M
Meyer
G P
GP
Hiss
M
M
Gwinner
W
W
Haller
H
H
Haubitz
M
M
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D009202:Cardiomyopathies / Q000209:etiology; D055252:Cell-Derived Microparticles; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D017023:Coronary Angiography; D003520:Cyclophosphamide / Q000627:therapeutic use; D004802:Eosinophilia; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide