소아 IgA 신병증의 장기 추적(평균 10.8년)에 따른 임상 경과 및 병리학적 변화
Clinicopathologic Changes of IgA Nephropathy in Children During Long-term (average 10.8yrs) Follow-up
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목 적:소아 IgA 신병증의 임상 양상 및 병리학적 분류(Haas 분류)와 임상 경과와의 상관 관계를 조사하여 이들이 예후를 반영할 수 있는지 알아보고자 하였다. 방 법:병리학적으로 IgA 신병증으로 진단받고 추적 중이던 환자들 중 추적 신생검을 시행한 20명의 환자를 대상으로 후향적으로 자료를 분석하였다. 결 과:최초 신생검시 평균 9.5세였고 남자 16명, 여자 4명이었으며, 최종 조직 검사까지 평균 10.8년이 경과하였다. 안지오텐신 전환효소 억제제 등으로 치료를 지속한 후 정상뇨 소견, 정상 혈압, 정상 크레아티닌 청소율(CCr)을 보이는 임상적 관해 상태의 환자는 10명(50%)이었으며, 비관해 상태의 환자 10명(50%)도 초기와 비교하여 호전된 경과를 보였고, CCr이 중등도 이상 저하되거나 말기 신질환 양상을 보이는 환자는 없었다. 최초 Haas 분류는 임상 경과와 상관 관계가 없었다. 초기 고혈압은 5명(25 %)에서 보였고 이는 임상 경과(P=0.010) 및 최종 Haas 분류(P=0.007)와 유의성이 있었다. 초기 CCr의 중등도 저하는 Haas 분류와 유의성이 있었으나(P=0.048), 임상 경과와는 상관관계가 없었다. 결 론:소아 IgA 신병증의 추적 기간 중 임상 경과는 양호하나 최초 Haas 분류로 임상 경과를 예측할 수는 없었으나 적극적인 조기 진단 및 치료로 말기 신질환으로의 진행을 지연시킬 수 있을 것으로 보인다. 초기 고혈압은 임상 경과 및 최종 Haas 분류와 유의한 상관관계를 보여 적합한 예후인자로 보인다. 최초 병리학적 소견은 임상 경과를 반영하지 못하나, 추적 Haas 분류가 예후를 반영할 수 있을지에 대해서는 보다 많은 추적 관찰 및 추적 신생검을 통한 연구가 있어야 할 것으로 보인다.
Abstract
Purpose:We know little about the natural course of IgA nephropathy (IgAN) in association with histologic changes especially in children. We investigated clinicopathologic features with long-term follow-up biopsy to clarify the outcomes and prognostic indicators for childhood IgAN. Methods:From our patients’ medical records, we retrieved 20 patients with IgAN, to whom renal biopsies had been performed for the initial diagnosis and follow-up to find out any histologic changes. Initial and follow-up biopsies were classified by Haas classification. The changes of these parameters were compared with the evolution of clinical features. Results:Patients were treated with angiotensin-converting enzyme inhibitors in combination with angiotensin receptor blockers (in subclass II or above) and short-term cyclosporine A(in patients showing nephrotic syndrome). Histologic improvement in 7 cases and deterioration in 3 cases were observed. At the time of last biopsy, 10 cases (50%) showed clinical remission and the others showed improved clinical features. These clinical outcomes did not correlate with initial Haas classifications. Hypertension at onset observed in 5 cases (25%) revealed significant correlation with clinical outcome (P=0.01) and last Haas classification (P=0.007). None of the cases showed progression to CRF or ESRD. Conclusion:During a mean follow-up of 10.8±3.4 years, childhood IgAN showed good clinicopathologic outcome. Hypertension at onset was only a strong predictor of clinicopathologic outcomes, but initial Haas classification cannot predict outcomes in children. Histologic change of IgAN in long term follow-up period cannot be completely predicted by clinical data and vice versa. Therefore, a renal biopsy should be considered as a part of follow-up plan.
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