Severe renal impairment in the case of classic polyarteritis nodosa

A 14-year-old boy with classic polyarteritis nodosa (cPAN) and a clinical p icture resembling rapidly progressive glomerulonephritis (RPGN) is describe d. He had severe hypertension, malaise, weight loss, fever, myalgia, and ra pid deterioration of renal function. Renal biopsy revealed acute necrotizin g vasculitis. Angiography showed small saccular aneurysmatic dilatations in the intrarenal branches of the right renal artery and the intrahepatic bra nches of the hepatic artery, cPAN was diagnosed and pulse methylprednisolon e (MP), pulse cyclophosphamide (CYC) and subsequently oral prednisolone wer e given. Clinical and laboratory findings improved dramatically and remissi on was attained rapidly. The patient has remained in remission for the last 11 months. cPAN should be considered in patients who present with severe s ystemic symptoms and hypertension. Progressive renal insufficiency can occu r during the acute course of cPAN due to renal vascular involvement without glomerulonephritis. Prompt and aggressive corticosteroid and cytotoxic the rapy is essential to suppress disease activity and to maintain remission.