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Clin Nephrol. 2018 Jan;89 (2018)(1):61-66. doi: 10.5414/CN109156.

Atypical case of classical polyarteritis nodosa: alveolar hemorrhage and positive antineutrophil cytoplasmic antibody


Classical polyarteritis nodosa (PAN) is a necrotizing arteritis affecting medium-sized vessels. A 73-year-old gentleman who presented with progressive anemia and scant hemoptysis developed acute kidney injury over the course of hospital admission. Kidney biopsy was initially interpreted as consistent with cephalosporin-associated acute interstitial nephritis (AIN) and acute tubular injury (ATI). After oral prednisone (5 days), the patient was discharged with improving serum creatinine. Two days later, he presented with diffuse alveolar hemorrhage. C-ANCA, ANA, anti-GBM, C4, cryoglobulin, HIV, HBV, and HCV studies were negative. P-ANCA was positive. Biopsy tissue was revisited, in addition to AIN, ATI, and non-caseating interstitial granulomas, lymphocytic infiltration of medium-sized vessels with endothelitis consistent with a diagnosis of PAN was noted. This case is notable for its atypical clinical presentation - alveolar hemorrhage - and atypical laboratory presentation - P-ANCA positivity for classical PAN. We describe an atypical presentation of PAN as a means of reviewing the range of clinical presentations, criteria for diagnosis, and treatment approaches for PAN and drug-induced vasculitis, which may present similar to PAN.

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