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.