A case of fatal anti-neutrophil cytoplasmic antibody-associated vasculitis with hypocomplementemia

Résumé fourni par la source

The case is a 92-year-old man. Skin purpura appeared frequently on his legs for four months and was followed up in our house department of dermatology. He was admitted to our hospital because of the expansion of the purpura, the swellings of the legs, and renal dysfunction. A skin biopsy showed leukocyto-clastic vasculitis and his serum tested positive for myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA). He was diagnosed with microscopic polyangiitis. The initial laboratory evaluation also revealed low complement levels (C4 : 7.5 mg/dL, CH50 : 9 U/mL) in his serum. After the administration of corticosteroids, an improvement in the purpura and renal function was observed, however, sudden dyspnea with the stridor developed on the fifth day of his illness and required respirator management. Steroid pulse therapy was conducted but his blood pressure decreased on the ninth day, and he died on the eleventh day of his illness. The autopsy revealed necrotizing crescentic glomerulonephritis in his kidney. In ANCA-related vasculitis, the complication of hypocomplementemia is rare. Through our patient.we discuss the role of the complement in the pathophysiology of ANCA-related vasculitis.

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