Concomitant presentation of myocarditis and non-bacterial thrombotic endocarditis in a patient with granulomatosis with polyangiitis

Abstract

Grsatnrualocmtatosis with polyangiitis (GPA) is a necrotizing vasculitis. The etiology of the disease is not completely known. In this report, we describe a 47-year-old male who presented with ongoing dyspnea. He is a known case of GPA for 3 years based on skin biopsy and positive antineutrophil cytoplasmic antibody. The electrocardiogram showed changes suggestive for myocarditis. A transthoracic and trans esophageal echocardiogram revealed left ventricular systolic dysfunction, thickened left atrial and ventricular wall and vegetation on mitral valve. Blood cultures were negative. Myocarditis and nonbacterial thrombotic endocarditis (NBTE) were diagnosed. As far as we know, NBTE is a rare manifestation of GPA and its presentation with myocarditis is not reported to date. This case indicates the uncommon manifestations of GPA and notifies the importance of performing echocardiography in order to prevent life threatening cardiac complications of the disease. InGtrraonduluomcattioosins with polyangiitis (GPA) is a necrotizing vasculitis of small vessels that affect respiratory tract and the kidneys.1 The etiology of the disease is not completely explained yet, but it seems that both environmental and genetic factors play roles in this disease.2 GPA involve many organs; among them kidneys, ear, nose, throat and lungs are more frequently involved; but eyes, cartilages, skin and other organs can also be affected.1 In a patient presenting with clinical findings suggestive for GPA, the confirm diagnosis is made by a tissue biopsy or sufficient positive titer of antineutrophil cytoplasmic antibody (c-ANCA) which is highly specific for GPA.3,4 Suggested treatment for GPA consists of IV glucocorticoids and oral cyclophosphamide.5,6 About 50- 70% of patients experience relapsing episodes.1 According to different studies, echocardiographic abnormalities due to cardiac involvement in GPA is relatively common, including systolic dysfunction, pericardial effusion, valvular involvement and so far.7,8 Nonbacterial thrombotic endocarditis (NBTE) is a rare manifestation of GPA and to our knowledge its concomitant presentation with myocarditis is not reported to date. Herein, we report a case of GPA, who diagnosed with both myocarditis and NBTE. © A. Yazdi et al., 2014.