Crescentic glomerulonephritis requiring hemodialysis and elevated MPO-ANCA level and vasculitis allergica cutis in a 21-year-old girl.
Microscopic polyangiitis (mPA) is a systemic necrotizing nongranulomatous vasculitis that affects small blood vessels. Clinical features include constitutional symptoms such as fever, anorexia, fatigue, and weight loss. Skin lesions include purpura and splinter hemorrhages, which occur in 50% of patients. Another characteristic feature is rapidly progressive glomerulonephritis, which often affects the kidneys in the early stages of the condition. Diagnosis is based on typical clinical features, biopsy, and presence of antineutrophil cytoplasmic antibodies (ANCA). This disorder tends to involve middle-aged and older persons, with a predilection for males; it is very rare in children. A 21-year-old female patient with mPA who did not respond well to treatment, required hemodialysis, developed vasculitis allergica cutis (VAC) later, and had a relatively short-term survival is reported herein.
Kara
Ismail H
IH
Department of Family Practice, Dicle University Medical School, Diyarbakir, Turkey. ihkara13@hotmail.com
Yilmaz
M Emin
ME
Sari
Yasin
Y
Gür
Ali
A
Büyükbayram
Hüseyin
H
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D017809:Fatal Outcome; D005260:Female; D005921:Glomerulonephritis / Q000097:blood / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D009195:Peroxidase / Q000276:immunology*; D011665:Pulmonary Valve Insufficiency / Q000097:blood / Q000473:pathology; D006435:Renal Dialysis / Q000009:adverse effects / Q000379:methods*; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000276:immunology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase