제한형 전신피부경화증에 병발한 현미경적 다발혈관염

Abstract

We report a case of microscopic polyangiitis in a 52-year-old woman combined with systemic sclerosis. She was diagnosed as having limited systemic sclerosis based on symmetric skin thickening of fingers and distal arms, loss of substance from the finger pad, and bibasilar pulmonary fibrosis. Laboratory investigations showed positive antitopoisomerase I antibody. Subsequent evaluation revealed proteinuria and hematuria. Her serum creatinine concentration was elevated with detoriorated glomerular filtration. Serum antimyeloperoxidase antibody was strongly positive. A sural nerve biopsy revealed axonopathy due to early manifestations of vasculitis. Percutaneous renal biopsy showed focal necrotizing glomerulonephritis. Her renal function recovered with immunosuppressive therapy with high dose prednisolone and oral cyclophosphamide.