Cardiac involvement in Wegener granulomatosis diagnosed at autopsy

Wegener granulomatosis (WG) is a systemic vasculitis classically involving the lungs, kidneys, and upper respiratory tract. Involvement of other sites does occur but is less frequent. Clinically evident cardiac involvement is uncommon. There are only a few cases in the literature with documentation of the histologic appearance of cardiac involvement in WG. We report a case of a previously healthy 37-year-old man who presented with a one-week history of cough and weakness and a one-day history of shortness of breath. At presentation, he was hypoxic and required intubation. Upon hospitalization, he deteriorated rapidly, became bradycardic and expired the same night. Infection was suspected clinically as the cause of illness and death. At autopsy, the lungs showed diffuse alveolar hemorrhage with capillaritis, diffuse alveolar damage, and parenchymal necrosis with necrotizing granulomas. The heart was enlarged and showed a mixed inflammatory infiltrate composed of neutrophils, eosinophils, and histiocytes, with focal myocyte necrosis. Granulomas, giant cells, and vasculitis were absent. The esophagus showed submucosal vasculitis. A diagnosis of WG was made. Postmortem serology for c-ANCA was positive, and all cultures were negative, confirming the diagnosis. Cardiac involvement is an underrecognized and potentially fatal complication of WG. The histologic findings in the heart may consist of a non-specific inflammatory infiltrate without granulomas or vasculitis, raising a wide differential diagnosis.