title: Efficacy of rituximab therapy against intractable steroid-resistant nephrotic syndrome.
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.
authors: Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan.
authors: Taku
authors: T
authors: Nakagawa
authors: Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan.
authors: Atsutoshi
authors: A
authors: Shiratori
authors: Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University, Yonago, Tottori, Japan.
authors: Yasuo
authors: Y
authors: Kawaba
authors: Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan.
authors: Kyoko
authors: K
authors: Kanda
authors: Department of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan. tanaka_kch@hp.pref.hyogo.jp.
authors: Ryojiro
authors: R
authors: Tanaka
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D013256:Steroids / Q000494:pharmacology*; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D013256:Steroids; D000069283:Rituximab
keywords: methylprednisolone pulse; rituximab; steroid-resistant nephrotic syndrome