title: Refractory focal segmental glomerulosclerosis in the adult: complete and sustained remissions of two episodes of nephrotic syndrome after a single dose of rituximab.
abstract: Rituximab therapy may achieve remission of proteinuria in children or adolescents with refractory focal segmental glomerulosclerosis (FSGS), but its effectiveness in adults is uncertain. We describe the case of a 22-year-old Caucasian woman with refractory FSGS that achieved complete and sustained remission of nephrotic syndrome (NS) with one single 375 mg/m(2) rituximab infusion. At 4 months after complete circulating B-cell depletion, proteinuria declined from 4.5 to 0.27 g/24 h and serum albumin normalised. Rituximab was well tolerated and allowed complete withdrawal of previous immunosuppression with steroids and azathioprine. The patient was in sustained remission up to month 32, when she experienced a relapse. A second infusion of rituximab (375 mg/m(2)) achieved prompt proteinuria reduction with no additional immunosuppressants. At 48 months after the initial treatment, this patient is in complete remission without any immunosuppression. This case suggests that rituximab, even a single dose, may safely promote NS remission in adults with refractory FSGS.
authors.affiliation: IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", Bergamo, Italy.
authors.forename: Maddalena
authors.initials: M
authors.lastname: Marasà
authors.affiliation: Icahn School of Medicine at Mount Sinai, New York, New York, USA.
authors.forename: Paolo
authors.initials: P
authors.lastname: Cravedi
authors.affiliation: IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", Bergamo, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Ruggiero
authors.affiliation: IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", Bergamo, Italy Unit of Nephrology and Dialysis, Hospital Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Ruggenenti
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D018951:Antigens, CD20; D001706:Biopsy; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007262:Infusions, Intravenous; D007668:Kidney / Q000473:pathology; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D012074:Remission Induction; D000069283:Rituximab; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007155:Immunologic Factors; D000069283:Rituximab