다른 면역 억제제에 듣지 않는 국소성 분절성 사구체 경화증 환자에서Cyclosporin A 2차 치료에 의한 완해 경험
Second Trial of Cyclosporin A-Induced Remissionin Other Immunosuppressant Therapy-ResistantFSGS Patient
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국소성 분절성 사구체 경화증은 소아 특발성 신증후군 환아의 약 10%를 차지하는데 초기 스테로이드 치료에 잘 반응하지 않고 만성 신부전으로 진행하는 경우가 많으며 신이식 후의 재발률도 높은 것으로 알려져 있다. 저자들은 생후 26개월에 스테로이드 저항성 국소성 분절성 사구체 경화증으로 진단 받고 9년 동안 경구 스테로이드, cyclophosphamide, methylprednisolone pulse therapy, cyclosporin A, ibuprofen을 포함한 치료를 받았으나 관해가 오지 않고 신기능은 정상으로 유지되던 10세 남아에게 두번째로 cyclosporin A를 저용량의 경구 스테로이드와 병용하여 투여한 결과 완전 관해에 이른 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. 또한 불응성 국소성 분절성 사구체 경화증에서 신기능이 유지된다면 단백뇨를 줄이기 위한 적극적인 치료가 지속적으로 필요할 수 있음을 알리는 바이다.
Abstract
Focal segmental glomerulosclerosis(FSGS) has been detected in approximately 10% of cases of idiopathic nephrotic syndrome in children, and exhibits a poor response to initial steroid therapy, as well as a higher rate of progression to chronic renal failure and relapse after kidney transplantation. We describe a case of an eleven year-old boy with steroid-resistant FSGS who exhibited a response to a second trial of cyclosporin A(CsA) therapy. At the age of 26 months, this patient was diagnosed with steroid-resistant FSGS. For 9 years, he had undergone a gauntlet of therapies to induce remission; oral steroids, cyclophosphamide, methylprednisolone(mehylPd) pulse therapy, CsA, and ibuprofen therapy. Although these therapies failed to induce remission, the patient's renal function remained in the normal range during the nine years of treatment. At the age of ten years, the patient's proteinuria decreased, and complete remission was attained with a second administration of CsA, coupled with a low dose of oral steroids. This patient continues to receive CsA without relapse. Therefore, our major concern involves the possibility of relapse after the discontinuation of CsA therapy. Our findings in this case suggest that, in cases of refractory FSGS, if renal insufficiency does not emerge, aggressive therapy for the amelioration of proteinuria should be continuously pursued.
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