title: Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies-negative eosinophilic granulomatosis with polyangiitis.
abstract: 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.
authors.affiliation: Department of Internal Medicine and Clinical Immunology, University Hospital of Dijon, Dijon, France.
authors.forename: K
authors.initials: K
authors.lastname: Bouiller
authors.forename: M
authors.initials: M
authors.lastname: Samson
authors.forename: J-C
authors.initials: JC
authors.lastname: Eicher
authors.forename: S
authors.initials: S
authors.lastname: Audia
authors.forename: S
authors.initials: S
authors.lastname: Berthier
authors.forename: V
authors.initials: V
authors.lastname: Leguy
authors.forename: O
authors.initials: O
authors.lastname: Humbert
authors.forename: L
authors.initials: L
authors.lastname: Martin
authors.forename: L
authors.initials: L
authors.lastname: Lorgis
authors.forename: Y
authors.initials: Y
authors.lastname: Cottin
authors.forename: B
authors.initials: B
authors.lastname: Bonnotte
authors.forename: B
authors.initials: B
authors.lastname: Lorcerie
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D003520:Cyclophosphamide / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008875:Middle Aged; D009205:Myocarditis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D011379:Prognosis; D012720:Severity of Illness Index; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide
keywords: Churg-Strauss syndrome; eosinophilic granulomatosis with polyangiitis; hypereosinophilia; myocarditis