title: Abatacept experience in steroid and rituximab-resistant focal segmental glomerulosclerosis.
abstract: Primary focal segmental glomerular sclerosis (FSGS), one of the major causes of nephrotic syndrome, eventually results in end-stage renal disease. Currently, FSGS is treated with immunosuppressive therapies, which include calcinuerin inhibitors (cyclosporine), glucocorticoids, B-cell depleting agents (rituximab) and, recently, a T-cell co-stimulatory inhibitor (abatacept). Until recently, there had been no cases reporting resistance to all current therapies. We report a case of a 62-year-old Caucasian man with biopsy-proven FSGS, who responded well to oral prednisolone therapy. However, 2 years later, he had a relapse and failed to respond to prednisolone. Subsequent treatments then included cyclosporine, rituximab and cyclophosphamide, which were not successful. The patient was then administered abatacept, a novel T-cell co-stimulatory inhibitor-though he did not experience any side effects, there was no change in proteinuria nor in creatinine.
authors.affiliation: Royal Perth Hospital, Perth, Western Australia, Australia.
authors.forename: Vinothkumar Kavarthapol
authors.initials: VK
authors.lastname: Jayaraman
authors.affiliation: Royal Perth Hospital, Perth, Western Australia, Australia.
authors.forename: Mark
authors.initials: M
authors.lastname: Thomas
mesh_terms: D000069594:Abatacept / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy*; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use*; D017211:Treatment Failure
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D000069283:Rituximab; D000069594:Abatacept; D016572:Cyclosporine; D003520:Cyclophosphamide; D011239:Prednisolone