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논문분류	춘계학술대회 초록집
제목	Rituximab in Treating Collapsing Focal Segmental Glomerulosclerosis (cFSGS) in a 13-year-old presenting with Acute Kidney Injury (AKI) and Venous Sinus Thrombosis: A Case Report
저자	Christopher Sim Sze Bing
출판정보	2024; 2024(1):
키워드
초록	Collapsing FSGS (cFSGS) is a glomerular disease that is characterised by an aggressive nephrotic syndrome that is usually treatment resistant. Optimal treatment of resistant disease is still poorly understood and carries a high risk of progression to end stage kidney disease. We herein report a case of cFSGS in a 13-year-old Malay boy and subsequent treatment of resistant disease with Rituximab. He initially presented with lower limb swelling in October 2022 with a 24-hour urine protein of 11g/day, hypoalbuminemia of 11g/L and normal creatinine. He was diagnosed with nephrotic syndrome and subsequently started on Prednisolone. He achieved full remission after 2 months and steroids were tapered. However, he presented in January 2023 with generalised anasarca and severe headaches. His results revealed a severe relapse of nephrotic syndrome with AKI (creatinine peak of 271umol/L, albumin 5g/L and 24-hour urine protein of more than 11g/day). On physical examination, he had bilateral pleural effusion with ascites. Computed tomography (CT) scan revealed left transverse sinus thrombosis. A kidney biopsy was performed which showed cFSGS. High dose prednisolone, diuretics and warfarin was given but he remained in nephrotic syndrome with generalised anasarca requiring sequential ultrafiltration (SU), intermittent pleural and peritoneal tapping. He was started on Tacrolimus for resistant disease but had no remission. Genetic testing was not done due to high cost. He was then given 4 doses intravenous rituximab 375mg/m2 (total 2g) in June 2023 in the background of tacrolimus and low dose prednisolone therapy. He showed marked improvement in oedema after 2 months and went into full remission after 6 months. A repeat CT scan showed resolution of transverse sinus thrombosis and anticoagulation was withheld. In conclusion, our case demonstrates the efficacy of Rituximab as a therapeutic option in treating treatment resistant cFSGS, which presented with life threatening complications in a young boy.
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