Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies-negative eosinophilic granulomatosis with polyangiitis.
Fiche publication
Date publication
septembre 2014
Auteurs
Membres identifiés du Cancéropôle Est :
Pr BONNOTTE Bernard, Pr MARTIN Laurent
Tous les auteurs :
Bouiller K, Samson M, Eicher JC, Audia S, Berthier S, Leguy V, Humbert O, Martin L, Lorgis L, Cottin Y, Bonnotte B, Lorcerie B
Lien Pubmed
Résumé
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is frequent and severe, and accounts for half of EGPA-related deaths. ANCA-positive EGPA differs from ANCA-negative EGPA in that the former is significantly associated with renal involvement, peripheral neuropathy and biopsy proven vasculitis, whereas the latter is associated with cardiac involvement. Herein, we report a case of EGPA with myocarditis in a woman, who was successfully treated with steroids and cyclophosphamide. This report highlights the importance of diagnosing cardiac involvement in EGPA early, especially in ANCA-negative patients.
Référence
Intern Med J. 2014 Sep;44(9):928-31