Acute heart failure: on the track of a rare disease

Abstract

Eosinophilic granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis with cardiac involvement in more than 60% of cases. Authors describe the case of a 48-year-old woman who presented with progressively worsening asthenia, dyspnoea and macular, non-painful, non-itchy cutaneous lesions. She had signs of congestion on clinical examination and a history of asthma and nasal polyps. Blood tests showed eosinophilia (11.2%), positive troponin I (9698 μg/L), elevated B-type natriuretic peptide (2047 pg/mL) and positive C reactive protein (6.68 mg/dL). Echocardiogram displayed moderate left ventricular enlargement, left ventricular ejection fraction of 28% and mild pericardial effusion. Levosimendan relieved the congestion. Additional testing confirmed positive antinuclear antibodies with ANCA-negative autoimmune pattern. Cardiac magnetic resonance showed severely depressed systolic function due to diffuse hypokinesia. Cardiac biopsy had intercellular oedema and eosinophilic infiltrate. Treatment with prednisolone and cyclophosphamide was started. This is a case of a rare disease presenting with life-threatening cardiac involvement.