Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature.
BACKGROUND
Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated.


OBJECTIVE
The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement.


METHODS
Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS.


RESULTS
The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample.


CONCLUSIONS
CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.
Agard
C
C
Internal Medicine, Hôtel-Dieu Hospital, CHU Nantes, Place Alexis Ricordeau, 44093 Nantes Cedex 01, France. christian.agard@chu-nantes.fr
Rendu
E
E
Leguern
V
V
Ponge
T
T
Masseau
A
A
Barrier
J H
JH
Trochu
J N
JN
Hamidou
M A
MA
Guillevin
L
L
D000208:Acute Disease; D000284:Administration, Oral; D000368:Aged; D002305:Cardiac Tamponade / Q000175:diagnosis* / Q000188:drug therapy; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006099:Granuloma / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007275:Injections, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010493:Pericarditis / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone; D008775:Methylprednisolone