Cardiac magnetic resonance imaging in Churg-Strauss-syndrome. Impact of immunosuppressants on outcome assessed in a prospective study on 8 patients.
OBJECTIVE
To evaluate the effects of immunosuppressive therapy on cardiac abnormalities observed by cardiac magnetic resonance imaging (CMRI) in patients with Churg-Strauss syndrome (CSS).


METHODS
We studied 8 patients with CSS and myocardial involvement on initial CMR images, who underwent follow-up CMRI after 6 months of immunosuppressive therapy.


RESULTS
Among the 8 patients (mean age: 43 years; 4 women), 7 had clinical cardiac signs at CSS onset (cardiac insufficiency, 3; angina pectoris, 2; atrial fibrillation, 1; and pericarditis, 1); 4 of them had myocardial-delayed enhancement, 2 had perfusion defects and 1 had both CMRI anomalies. The patient without clinical manifestations of heart disease had myocardial delayed enhancement on CMRI. After 6 months of therapy, CMR images normalised for the patient without clinical cardiac signs at diagnosis, and 3 symptomatic patients, and abnormalities had regressed for 2 other symptomatic patients. Theses 5 initially symptomatic patients became asymptomatic after immunosupressive treatment. The last 2 patients with cardiac insufficiency at CSS diagnosis are still symptomatic with unchanged CMRI abnormalities.


CONCLUSIONS
CMRI is a sensitive, non-invasive method to detect cardiac lesions in patients whose conventional investigations indicated no cardiac disease and to assess the extent of cardiac involvement in symptomatic patients. CMRI can help evaluate the therapeutic effect of immunosuppressants in CSS.
Marmursztejn
Julien
J
Department of Cardiology, Hopital Cochin, Université de Paris, France. julien.marmursztejn@cch.aphp.fr
Cohen
Pascal
P
Duboc
Denis
D
Pagnoux
Christian
C
Mouthon
Luc
L
Guilpain
Philippe
P
Legmann
Paul
P
Guillevin
Loic
L
Vignaux
Olivier
O
D000328:Adult; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology*; D016903:Drug Monitoring / Q000379:methods; D042241:Early Diagnosis; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006331:Heart Diseases / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D012680:Sensitivity and Specificity; D013318:Stroke Volume / Q000187:drug effects
D007166:Immunosuppressive Agents