Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies-negative eosinophilic granulomatosis with polyangiitis

Intern Med J. 2014 Sep;44(9):928-31. doi: 10.1111/imj.12525.

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.

Keywords: Churg-Strauss syndrome; eosinophilic granulomatosis with polyangiitis; hypereosinophilia; myocarditis.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Churg-Strauss Syndrome / complications*
  • Churg-Strauss Syndrome / diagnosis*
  • Churg-Strauss Syndrome / drug therapy
  • Cyclophosphamide / therapeutic use*
  • Diagnosis, Differential
  • Female
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / diagnosis*
  • Granulomatosis with Polyangiitis / drug therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Middle Aged
  • Myocarditis / diagnosis*
  • Myocarditis / drug therapy
  • Myocarditis / etiology*
  • Myocarditis / immunology
  • Prognosis
  • Severity of Illness Index
  • Steroids / therapeutic use*
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Steroids
  • Cyclophosphamide