Taku Nakagawa1, Atsutoshi Shiratori1, Yasuo Kawaba2, Kyoko Kanda1, Ryojiro Tanaka3. 1. Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan. 2. Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University, Yonago, Tottori, Japan. 3. Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan. tanaka_kch@hp.pref.hyogo.jp.
Abstract
BACKGROUND: Some cases of childhood steroid-resistant nephrotic syndrome (SRNS) are intractable. We examined the cases of three patients with SRNS resistant to various treatment, but who achieved complete remission after being treated with rituximab (RTX) followed by methylprednisolone pulse (MP) therapy. METHODS: A retrospective chart review of all new-onset SRNS in the period from January 1997 to December 2013 was performed. Three of the 13 patients who received conventional treatment continued to have NS for >6 months, despite also being treated with immunosuppressants and receiving frequent albumin treatment. In addition, two of the patients received plasma exchange therapy, but it was ineffective. Therefore, RTX was used once a week for 4 weeks, followed by several courses of MP therapy. RESULTS: Two of the three intractable SRNS patients achieved complete remission after treatment with RTX followed by MP therapy, and the remaining patient achieved incomplete remission after the first round of this treatment. That patient subsequently achieved complete remission after the second round of the treatment. RTX did not cause any serious side-effects, and all three patients had normal renal function at the final observation. CONCLUSIONS: Complete remission was achieved in all 13 SRNS patients. RTX followed by MP therapy might be effective against SRNS refractory to conventional treatments and requiring frequent albumin treatment. Prospective clinical study examining the effectiveness and safety of this approach is required.
BACKGROUND: Some cases of childhood steroid-resistant nephrotic syndrome (SRNS) are intractable. We examined the cases of three patients with SRNS resistant to various treatment, but who achieved complete remission after being treated with rituximab (RTX) followed by methylprednisolone pulse (MP) therapy. METHODS: A retrospective chart review of all new-onset SRNS in the period from January 1997 to December 2013 was performed. Three of the 13 patients who received conventional treatment continued to have NS for >6 months, despite also being treated with immunosuppressants and receiving frequent albumin treatment. In addition, two of the patients received plasma exchange therapy, but it was ineffective. Therefore, RTX was used once a week for 4 weeks, followed by several courses of MP therapy. RESULTS: Two of the three intractable SRNS patients achieved complete remission after treatment with RTX followed by MP therapy, and the remaining patient achieved incomplete remission after the first round of this treatment. That patient subsequently achieved complete remission after the second round of the treatment. RTX did not cause any serious side-effects, and all three patients had normal renal function at the final observation. CONCLUSIONS: Complete remission was achieved in all 13 SRNS patients. RTX followed by MP therapy might be effective against SRNS refractory to conventional treatments and requiring frequent albumin treatment. Prospective clinical study examining the effectiveness and safety of this approach is required.
Authors: Yo Han Ahn; Seong Heon Kim; Kyoung Hee Han; Hyun Jin Choi; Heeyeon Cho; Jung Won Lee; Jae Il Shin; Min Hyun Cho; Joo Hoon Lee; Young Seo Park; Il-Soo Ha; Hae Il Cheong; Su Young Kim; Seung Joo Lee; Hee Gyung Kang Journal: Medicine (Baltimore) Date: 2018-11 Impact factor: 1.817