J Korean Rheum Assoc.  2003 Sep;10(3):310-314.

A Case of Microscopic Polyangiitis Combined with Limited Systemic Sclerosis

Affiliations
  • 1Department of Internal Medicine, Myongji Hospital, Kwandong University College of Medicine, Gyunggi-do, Korea.
  • 2Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea. sookonlee@yumc.yonsei.ac.kr
  • 3Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.

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.

Keyword

Systemic sclerosis; Microscopic polyangiitis

MeSH Terms

Arm
Biopsy
Creatinine
Cyclophosphamide
Female
Filtration
Fingers
Glomerulonephritis
Hematuria
Humans
Microscopic Polyangiitis*
Middle Aged
Prednisolone
Proteinuria
Pulmonary Fibrosis
Scleroderma, Systemic*
Skin
Sural Nerve
Vasculitis
Creatinine
Cyclophosphamide
Prednisolone
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