RESUMEN
Classic polyarteritis nodosa (c-PAN), first described by Kussmaul and Maier in 1866, is a systemic illness characterized by necrotizing inflammation of medium- sized arteries. Rapid progressive renal failure is very rare in c-PAN in contrast to microscopic polyangiitis (MPA). We report a case of c-PAN presented with rapidly progressive renal failure. A 58 year-old male was referred to our hospital for evaluation of asthenia and right visual loss. An increase in serum creatinine level was developed in the 6th day of hospitalization and acute renal failure was aggravated rapidly. Renal biopsy showed the transmural fibrinoid necrosis of small and medium sized arteries with few glomerular crescents. P-ANCA was detected in serum, and all clinical and laboratory findings supported the diagnosis of c-PAN. He was treated with pulse methylprednisolone, followed by oral corticosteroid and cyclophosphamide, and received temporary hemodialysis. After the treatment, he recovered and was discharged but his visual defect was irreversible despite the treatment.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Lesión Renal Aguda , Anticuerpos Anticitoplasma de Neutrófilos , Arterias , Astenia , Biopsia , Creatinina , Ciclofosfamida , Diagnóstico , Hospitalización , Inflamación , Metilprednisolona , Poliangitis Microscópica , Necrosis , Poliarteritis Nudosa , Diálisis Renal , Insuficiencia RenalRESUMEN
Classic polyarteritis nodosa (PAN) is a systemic segmental necrotizing vasculitis which shows transmural fibrinoid necrosis with surrounding inflammation in small to medium-sized muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation. Classic PAN commonly affects the kidney, liver, muscle, nerve, gastrointestinal tract and heart. Arteriography demonstrates microaneurysm or occlusion of the visceral arteries in 90% of the patients with classic PAN. However, rupture of the hepatic or renal artery aneurysm is uncommon cascade. We report two exceptional cases who presented with aneurysmal ruptures of the hepatic or renal artery and visceral infarction.