title: Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases.
abstract: Several case reports suggest that rituximab (RTX) could be effective in steroid-dependent nephrotic syndrome, but RTX efficacy has not yet been studied in a series of patients. Safety and efficacy of RTX were assessed in a multicenter series of 22 patients aged 6.3-22 years with severe steroid-dependent nephrotic syndrome or steroid-resistant but cyclosporin-sensitive idiopathic nephrotic syndrome. Patients were treated with two to four infusions of RTX. Seven patients were nephrotic at the time of RTX treatment. Peripheral B cells were depleted in all subjects. Remission was induced in three of the seven proteinuric patients. One or more immunosuppressive (IS) treatments could be withdrawn in 19 patients (85%), with no relapse of proteinuria and without increasing other IS drugs. RTX was effective in all patients when administered during a proteinuria-free period in association with other IS agents. When relapses occurred, they were always associated with an increase in CD19 cell count. Adverse effects were observed in 45% of cases, but most of them were mild and transient. This study suggests that RTX could be an effective treatment for severe steroid-dependent nephrotic syndrome.
authors.affiliation: Department of Pediatrics, Centre de référence des maladies rénales rares du Sud-Ouest, Hôpital de la mère et de l'enfant, 8 Ave Dominique Larrey, 87000 Limoges, France. vincent.guigonis@chu-limoges.fr
authors.forename: Vincent
authors.initials: V
authors.lastname: Guigonis
authors.forename: Aymeric
authors.initials: A
authors.lastname: Dallocchio
authors.forename: Véronique
authors.initials: V
authors.lastname: Baudouin
authors.forename: Maud
authors.initials: M
authors.lastname: Dehennault
authors.forename: Caroline
authors.initials: C
authors.lastname: Hachon-Le Camus
authors.forename: Mickael
authors.initials: M
authors.lastname: Afanetti
authors.forename: Jaap
authors.initials: J
authors.lastname: Groothoff
authors.forename: Brigitte
authors.initials: B
authors.lastname: Llanas
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Hubert
authors.initials: H
authors.lastname: Nivet
authors.forename: Natacha
authors.initials: N
authors.lastname: Raynaud
authors.forename: Sophie
authors.initials: S
authors.lastname: Taque
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
authors.forename: François
authors.initials: F
authors.lastname: Bouissou
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage* / Q000009:adverse effects; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19 / Q000378:metabolism; D001402:B-Lymphocytes / Q000166:cytology / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage* / Q000009:adverse effects; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000276:immunology; D011446:Prospective Studies; D011507:Proteinuria / Q000188:drug therapy / Q000276:immunology; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab; D012720:Severity of Illness Index; D013256:Steroids / Q000008:administration & dosage* / Q000009:adverse effects; D013375:Substance Withdrawal Syndrome / Q000517:prevention & control
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018941:Antigens, CD19; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab; D016572:Cyclosporine