Polyarteritis nodosa with isolated organ involvement requiring resection in the genitourinary system

Abstract

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium-sized arteries that is classically described as a multisystem disease. However, PAN may rarely present with isolated organ involvement, occasionally leading to irreversible organ loss before a definitive diagnosis is established. Data on such presentations remain limited and are largely confined to isolated case reports. We retrospectively evaluated eight patients diagnosed with PAN from multiple centers, focusing on clinical presentation, imaging findings, serological results, histopathological features, treatment approaches, and outcomes. The cohort included six women and two men, with ages ranging from 25 to 76 years. Clinical presentation was highly heterogeneous and frequently dominated by life-threatening genitourinary events, including massive renal hemorrhage, retroperitoneal hematoma, renovascular disease with aneurysm formation and infarction, and acute testicular pain and swelling. Five of the eight patients were classified as having isolated, single-organ PAN, predominantly involving the kidney and, less frequently, the testis. In these patients, organ loss was often the event that led to definitive diagnosis. Imaging findings supported vascular patterns consistent with PAN, including hematoma, arterial stenosis, aneurysmal changes, and ischemic sequelae. Serological evaluation was largely nondiagnostic, with predominant ANCA negativity. Histopathological examination consistently demonstrated necrotizing arteritis of medium-sized arteries with fibrinoid necrosis, thrombosis, and transmural inflammation, without glomerular or granulomatous involvement. Multisystem disease was identified in three patients, including one fatal presentation diagnosed at autopsy. This multicenter case series highlights isolated-organ PAN as a rare but clinically significant presentation, frequently recognized only after catastrophic vascular complications result in resection. Our findings emphasize the pivotal role of surgical pathology and clinicopathological correlation in establishing the diagnosis and underscore the need to consider PAN in unexplained renal or testicular vascular catastrophes, even in the absence of classic systemic features.