An Unusual Case of Dilated Cardiomyopathy in Wegner's Granulomatosis

Abstract

A 33-year-old male presented to the emergency with cough, hemoptysis, and shortness of breath. He was on steroids for suspected Still's disease due to arthralgias and fever prior to presentation to the emergency. He developed sudden hypoxic respiratory failure and required mechanical ventilation. The initial imaging studies of the chest including computed tomography (CT) of the chest showed marked diffuse central and basilar predominant opacities with associated smooth septal thickening. Furthermore, the patient's creatinine, troponin, B-type natriuretic peptide (BNP), rheumatoid factor, and D-dimer were elevated. Vasculitis workup, bronchoscopy, and echocardiogram were performed. The echocardiogram revealed severely decreased left ventricular systolic function with an ejection fraction of 24% with dilated left ventricle. The electrocardiogram did not show any findings of acute ischemia. He was started on pulse dose steroid and dobutamine drip along with intermittent diuresis. The patient was successfully extubated after two days of mechanical ventilation. He was started on cyclophosphamide in the hospital. Dobutamine was discontinued. He was moved to the general medical floor as his oxygenation improved, but later at night, he developed respiratory failure and required a bumetanide drip. The cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) (anti-PR-3 antibody) came back positive with titer >1:40, so Wegner's granulomatosis was diagnosed. He received three sessions of plasmapheresis. The patient's kidney function improved significantly, and the bumetanide drip was transitioned to intravenous pushes. His oxygenation improved significantly with saturations of 92% on room air. The patient was discharged on steroid, Bactrim, and systolic heart failure medications to follow up with rheumatology, nephrology, pulmonology, and cardiology in the office. Due to insurance issues, his outpatient care was delayed significantly. The patient followed up with rheumatology after two months and has been planned for rituximab induction and to continue steroid along with Bactrim. This case is worth reporting because it describes dilated cardiomyopathy (DCM) as a cardiac manifestation of Wegner's granulomatosis. Early cardiac evaluation should be incorporated into the management of the patient suspected of Wegner's granulomatosis.

Keywords: anca-associated vasculitis; dilated cardiomyopathy (dcm); granulomatosis with polyangiitis (gpa); heart failure; wegner’s granulomatosis.

Figure 1. Chest radiography showing diffuse multifocal nodular opacities

Figure 2. Computed tomography with angiography of the chest showing diffuse multifocal opacities

Figure 3. Transthoracic echocardiogram showing elevated systolic left ventricle internal dimension with left ventricular dilation