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Endomyocardial fibrosis in a patient with eosinophilic granulomatosis with polyangiitis

Publication |
2017

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome is classified as systemic necrotizing vasculitis. Clinically and laboratory is characterized by allergic rhinitis, bronchial asthma, mononeuropathy, migrating pulmonary infiltrates and significant eosinophilia.

Etiopathogenesis is not clear, but autoimmune etiology is presumed. Cardiac involvement is described in 15-60% of cases, particularly in ANCA-negative patients.

We present a case of 64 years old patient who was admitted from the local internal department in august of 2016 for slight elevation of troponin without dynamics, high elevation of NT-proBNP and nonspecific repolarization ECG changes. A history of extreme tiredness, weight loss and laboratory finding of marked eosinophilia started diagnostic process at the end of which diagnosis of ANCA negative EGPA was set.

Cardiac involvement was verified by echocardiography, cardiac MRI and endomyocardial biopsy. The patient was referred for further treatment to the rheumatology department, where he was treated until 14 December of 2016 with 4 pulses of cyclophosphamide and chronic prednisone therapy was initiated.

Current treatment led to significant improvement.