Case Presentation: A 69 year-old man with no known prior medical history presented with one month of dyspnea on exertion associated with abdominal, scrotal, and bilateral leg swelling. He reported orthopnea, a dry cough, and 50lb weight gain. His exam was notable for a fever to 100.8, bilateral injected conjunctiva, bibasilar rales, jugular venous distension to the angle of the jaw, tachycardia with regular rate, and significant pitting edema extending above the knees. Initial labs showed mild leukocytosis of 13,000/uL, BNP of 2844pg/mL and chest X-ray with marked pulmonary congestion. An urgent echocardiogram demonstrated four chamber dilation, markedly reduced left ventricular ejection fraction of 15% and pulmonary hypertension. Acute systolic heart failure (HF) was presumed and aggressive diuresis was begun. As part of the HF work-up, a left heart catheterization revealed mild non-obstructive coronary disease raising the suspicion of non-ischemic cardiomyopathy.

While the patient’s respiratory status improved with diuresis, his course was complicated by a sudden decline in renal function. Simultaneously his WBC count rose accompanied by worsening anemia and the onset of intermittent blood-streaked sputum. Urinalysis showed elevated WBCs, RBCs, significant protein, and granular casts concerning for acute nephritis. Workup was notable for +C-ANCA antibody. The patient’s injected conjunctiva, scant hemoptysis, worsening anemia, positive C-ANCA, and progressive non-oliguric renal failure raised suspicion for granulomatosis with polyangiitis (GPA). Renal biopsy showed cellular crescents, segmental fibrinoid necrosis, global glomerulosclerosis, tubular atrophy and interstitial fibrosis/inflammation with eosinophils and moderate arteriosclerosis consistent with pauci-immune glomerulonephritis. Standard treatment for GPA with steroids and cyclophosphamide was started, but despite initial therapy, worsening uremia and new asterixis were noted. Due to his rapidly progressive renal disease, dialysis and plasma exchange were required. After further cycles of treatment his renal function recovered and he was weaned off dialysis.

Discussion: While studies of the prevalence of cardiac involvement in GPA has ranged from 6 to 44% of all cases, acute HF as a presenting manifestation of GPA has not yet been reported. GPA typically presents with nonspecific complaints including fever, malaise, myalgias, weight loss and fatigue. Cardiac manifestations previously described include conduction abnormalities and in rare cases, myocarditis. Our patient presented with severe volume overload in the setting of acute systolic HF. The pathophysiologic mechanism remains unknown and requires further study.

Conclusions: Patients presenting with severe HF that lack significant coronary disease require further workup for non-ischemic cardiomyopathy. Although rare, GPA has been associated with cardiac manifestations. In these patients, early identification could reduce both cardiac and renal morbidity.