Presentation: Severe Cardiac Involvement in Churg-Strauss Syndrome (2007)

2024 Severe Cardiac Involvement in Churg-Strauss Syndrome

PURPOSE: In Churg-Strauss syndrome (CSS), heart involvement is associated with a poor outcome and represents the major cause of mortality, accounting for about 50% of deaths. This study aimed to define the characteristics and the outcome of severe heart involvement in the most recent cases of CSS.
METHODS: We retrospectively reviewed the charts of 26 CSS patients with cardiac involvement, followed between 1995 and 2006 and entered in the French Vasculitis Study Group database. Diagnosis of CSS was made according to the criteria established by the ACR and the Chapel Hill Conference. Inclusion criteria were as follows: (1) severe cardiac involvement as defined by at least one of the following manifestations: heart failure, myocardium involvement, endocardium involvement, cardiac tamponade, heart thrombus, arrhythmia, severe conduction abnormality; (2) well-documented clinical and ultrasonographic data at baseline and during follow-up.
RESULTS : Cardiac manifestations were present at CSS diagnosis in all but 2 patients, (92%). Anatomic involvement of the heart at diagnosis was as follows: myocardial involvement in 25 patients (96%) with left ventricle dilatation in 12 patients (46%), pericardium involvement in 14 patients (54%) including 3 with cardiac tamponade, endocardium involvement in 6 patients (23%). Two patients had anti-myeloperoxidase ANCA. Combination of prednisone and cyclophosphamide (CYC) was given to all patients except one (96%). Mean follow-up was 50.2 +/- 32.7 months. One patient died from congestive heart failure and another had dilated cardiomyopathy with severe heart failure. Mean left ventricle ejection fraction measured by echocardiogram was significantly higher at the end of the follow-up than at diagnosis of heart involvement (48,8 +/- 14,5 % vs 42,6 +/- 11,1 % ; p <0,05). CSS was in remission in all living patients at the end of follow-up. All these patients were still on low doses of prednisone, less than 15 mg/d.
CONCLUSION: With systematic use of CYC and improved cardiologic management, CSS heart involvement appears to have a better outcome than previously reported.

 P. Guilpain, None; P. Cohen, None; C. Pagnoux, None; D. Duboc, None; O. Vignaux, None; J. Viallard, None; O. Fain, None; L. Mouthon, None; J. Cordier, None; L. Guillevin, None.