Korean J Med.
1999 Jan;56(1):139-145.
One Case of Microscopic Polyangiitis
- Affiliations
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- 1Department of Internal Medicine and Pathology, Inha University, College of Medicine.
- 2Department of Internal medicine, College of Medicine, Pochon CHA University.
Abstract
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Microscopic polyangiitis is a primary systemic vasculitis which involve small arterioles and
capillaries. Clinical manifestations show generalized weakness, fever, myalgia, weight loss.
oliguria, proteinuria, skin rash, neuromuscular symtoms, and pulmonary, gastrointestinal symtoms.
Prednisolone and cyclophosphamide therapy is effectve, more than 80% of patients with microscopic
polyangiitis survive for longer than 5years. However, about 50% of patients will have at least
one relapse in the 4-5years after initial treatment. There is a very strong correlation between
the presence of ANCA(especially p-type) and microscopic polyangiitis. We herein report a case
of microscopic polyandiitis with the review of literature. A 65 years-old woman admitted to our
hospital due to general weakness, fever, generalized edema, and dyspnea. Laboratory result showed
high serum creatinine and CRP, moderate proteinuria, and microscopic hematuria. P-ANCA was 1:1280
positive in indirect immunofluorescence method, and kidney biopsy showed focal segmental
necrotizing glomerulonephritis, crescent formation, fibrin clots, PMN and lymphocytes
infiltrations in small arterioles and capillries. Immunofluorescence study was negative.
Her clinical feature, laboratory findings, and the kidney biopsy result were compatible with
microscopic polyangiitis. The patient had been treated with prednisolone and cyclophosphamide.
P-ANCA titration was decreased 1:320 at the 19th day of treatment and was negative about 2months later.