Clinical Case of Nodular Polyarteritis

Abstract

Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis affecting small- and medium-sized arteries, leading to vascular inflammation, thrombosis, aneurysm formation, and tissue ischemia. Juvenile-onset PAN is exceptionally rare and often presents with nonspecific manifestations, resulting in delayed diagnosis and irreversible organ damage. We report a case of juvenile-onset PAN with prolonged disease duration complicated by extensive digital necrosis and multiple amputations.

Case presentation: A 42-year-old woman was admitted with necrotic lesions of the cheeks, nose, fingers, and toes, accompanied by cold extremities, dyspnea, chest pain, headache, and generalized weakness. The disease began at the age of 15 years with Raynaud-like symptoms and was managed as Raynaud’s syndrome for many years without immunosuppressive therapy. Despite repeated vascular treatment and sympathectomy, progressive ischemia resulted in multiple finger and toe amputations. Laboratory investigations revealed leukocytosis, thrombocytopenia, elevated inflammatory markers, mild renal impairment, and proteinuria. Hepatitis B serology was negative. Cardiac evaluation demonstrated secondary cardiomyopathy, left ventricular hypertrophy, and grade II aortic regurgitation. Based on the characteristic clinical manifestations and multisystem involvement, a diagnosis of juvenile-onset polyarteritis nodosa with moderate disease activity affecting the skin, peripheral vessels, cardiovascular system, kidneys, liver, and joints was established.

Conclusion: This case highlights the diagnostic challenge of juvenile-onset PAN and its potential to mimic Raynaud’s syndrome. Persistent digital ischemia, progressive tissue necrosis, and multisystem involvement should raise suspicion for systemic vasculitis. Early diagnosis and timely immunosuppressive therapy are crucial to prevent irreversible vascular damage, amputations, and long-term disability.