Online first

Acta Reumatológica Portuguesa - Online First: 2018-11-12

Silent acute myocarditis in eosinophilic granulomatosis with polyangiitis

Ferreira R, Madureira P, Pinho T, Martins E, Pimenta S, Costa L


Eosinophilic granulomatosis with polyangiitis is a rare multisystemic disorder, characterized by necrotizing vasculitis affecting small to medium-sized vessels, associated with asthma and eosinophilia. Cardiac involvement is the most important predictor of mortality and it seems to be more frequent in anti–neutrophil cytoplasmic antibodies-negative patients. Cardiomyopathy and congestive heart failure can occur but a significant proportion of patients are asymptomatic. We present a case of this condition in a 65-year-old woman with a past medical history of rhinosinusitis and recent episodes of asthma, that developed palpable purpura, sensory deficiency and excruciating pain mainly in the lower limbs. A significant hypereosinophilia and elevated troponin level were found, although she had not cardiac symptomatology. Cardiovascular magnetic resonance revealed late gadolinium enhancement and a severe reduction of the left ventricular ejection fraction. Mononeuritis multiplex was documented and diagnosis was confirmed by biopsy. Complementary cardiac investigation is mandatory in any patient with suspicion of Eosinophilic granulomatosis with polyangiitis. Early detection and the appropriate treatment are crucial due to the possible life-threatening manifestations.