급속 진행성 사구체신염으로 시작된 현미경적 다발성동맥염(Microscopic Polyangiitis) 환자의 7년간의장기 추적관찰 1례
Seven-Year Follow Up of Microscopic Polyangiitis Presentingwith Rapidly Progressive Glomerulonephritis
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초록
현미경적 다발성 동맥염은 폐출혈과 급속 진행성 사구체심염을 특징으로 하는 전신성 혈관염의 일종으로 소아에서는 매우 드문 질환이다. 저자들은 폐출혈과 급성 신부전을 동반한 7세 여아에서 신장조직검사와 p-ANCA 검사로 현미경적 다발성 동맥염으로 진단 후 5년 뒤 2차 신장조직검사와 경구 ACE inhibitor, angiotensin II receptor blocker와 저용량의 cyclophosphamide를 투여 받은 환아를 7년간 추적관찰하였다. 발병 당시에는 BUN 117 mg/dL, Cr 2.3 mg/dL이었으나, 퇴원시 BUN 20.8 mg/dL, Cr 1.6 mg/dL이었고, 최근 검사에서는 BUN 51.7 mg/dL, Cr 3.2 mg/dL으로 만성 신병증 소견을 보이고 있으며, 외래 추적관찰 지속 중에 있다. 이에 문헌 고찰과 함께 증례 보고하는 바이다.
Abstract
Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.
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