Second Trial of Cyclosporin A-Induced Remission in Other Immunosuppressant Therapy-Resistant FSGS Patient

Cho, Hee-Yeon; Lee, Bum-Hee; Kang, Ju-Hyung; Ha, Il-Soo; Cheong, Hae-Il; Choi, Yong

J Korean Soc Pediatr Nephrol > Volume 9(1); 2005 > Article

J Korean Soc Pediatr Nephrol 2005;9(1): 83-90.

다른 면역 억제제에 듣지 않는 국소성 분절성 사구체 경화증 환자에서 Cyclosporin A 2차 치료에 의한 완해 경험

조희연, 이범희, 강주형, 하일수, 정해일, 최용

¹서울대학교 의과대학 소아과학교실
²서울대학교 의과대학 소아과학교실
³서울대학교 의과대학 소아과학교실
⁴서울대학교 의과대학 소아과학교실
⁵서울대학교 의과대학 소아과학교실
⁶서울대학교 의과대학 소아과학교실

Second Trial of Cyclosporin A-Induced Remission in Other Immunosuppressant Therapy-Resistant FSGS Patient

Hee-Yeon Cho, Bum-Hee Lee, Ju-Hyung Kang, Il-Soo Ha, Hae-Il Cheong, Yong Choi

¹Department of Pediatrics, Seoul National University College of Medicine
²Department of Pediatrics, Seoul National University College of Medicine
³Department of Pediatrics, Seoul National University College of Medicine
⁴Department of Pediatrics, Seoul National University College of Medicine
⁵Department of Pediatrics, Seoul National University College of Medicine
⁶Department of Pediatrics, Seoul National University College of Medicine

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.

Key words: Steroid-resistant FSGS | Cyclosporin A | Remission | Renal insufficiency