Necrotizing myocardial vasculitis in Churg-Strauss syndrome: Clinicohistologic evaluation of steroids and immunosuppressive therapy

Andrea Frustaci, Nicola Gentiloni, Cristina Chimenti, Luigi Natale, Giovanni Gasbarrini, Attilio Maseri

Research output: Contribution to journalArticlepeer-review

Abstract

Treatment of cardiac dysfunction associated with Churg-Strauss syndrome (CSS) is empiric since the histologic findings provided by endomyocardial biopsy are rare and often nondiagnostic. Myocardial necrotizing vasculitis presenting as restrictive cardiomyopathy has not been reported before. A case of CSS, presenting with fever and progressive heart failure due to pericarditis, eosinophilic endomyocarditis, and myocardial necrotizing vasculitis, is reported. Cardiac involvement assessed by noninvasive (cardiac two-dimensional echocardiogram and nuclear magnetic resonance [NMR] imaging) and invasive (cardiac catheterization, angiography, and biopsy) studies showed a moderate degree of pericardial effusion and left ventricular (LV) dysfunction (ejection fraction 0.40), severe diastolic dysfunction (increased right and LV filling pressure with a dip and plateau pattern) and a severe reduction of cardiac index (1.6 L/min/m2). Histologic characteristics showed marked eosinophilic infiltration of the endocardium and myocardium with myocitolysis and fibrinoid necrosis of arterioles, venules, and capillaries. Combination therapy of steroids and cyclophosphamide resulted in both a clinical (regression of pericardial effusion, normalization of systolic and diastolic dysfunction, and increase of cardiac index to 2.8 L/min/m2) and histologic (sequential endomyocardial biopsies at 1, 3, and 6 months of follow-up) resolution of cardiac involvement. No recurrences were registered at 12-month follow-up with the patient receiving a maintenance drug regimen.

Original languageEnglish
Pages (from-to)1484-1489
Number of pages6
JournalChest
Volume114
Issue number5
Publication statusPublished - 1998

Keywords

  • Churg-Strauss syndrome
  • Heart failure
  • Myocardial necrotizing vasculitis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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