title: Recurrent Focal Segmental Glomerulosclerosis and Abatacept: Case Report.
abstract: Focal segmental glomerulosclerosis is a common cause of end-stage renal disease in children. Focal segmental glomerulosclerosis recurrence in renal transplants is a challenging disease, and can cause graft dysfunction and loss. Different therapies exist with varying responses, from complete remission to resistance to all modes of treatment. Abatacept was recently introduced as a treatment for primary focal segmental glomerulosclerosis in native kidneys and in recurrent disease after transplant. We present a pediatric case with immunosuppression-resistant primary NPHS2-negative focal segmental glomerulosclerosis recur-rence after renal transplant. The standard therapy for recurrent focal segmental glomerulosclerosis (rituximab, plasmapheresis, high-dose cyclosporine, and corticosteroids) was tried but failed to induce remission. Abatacept (10 mg/kg) was given at 0, 2, and 4 weeks (total, 3 doses) with no good response. We conclude that abatacept may work in patients with B7-1-positive focal segmental glomerulosclerosis recurrence and its efficacy is uncertain in disease with B7-1-negative or unknown staining status.
authors.affiliation: From the Division of Pediatric Nephrology, Mubarak Al-Kabeer Hospital; and the Division of Nephrology, Hamid Al-Essa Organ Transplant Center, Sabah Health Region, Shuwaikh, Kuwait.
authors.forename: Omar
authors.initials: O
authors.lastname: Alkandari
authors.forename: Narayanan
authors.initials: N
authors.lastname: Nampoory
authors.forename: Prasad
authors.initials: P
authors.lastname: Nair
authors.forename: Ahmed
authors.initials: A
authors.lastname: Atta
authors.forename: Zakaria
authors.initials: Z
authors.lastname: Zakaria
authors.forename: Ahmed
authors.initials: A
authors.lastname: Mossad
authors.forename: Jude
authors.initials: J
authors.lastname: Yagan
authors.forename: Torki
authors.initials: T
authors.lastname: Al-Otaibi
mesh_terms: D000069594:Abatacept / Q000627:therapeutic use*; D002648:Child; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects*; D012008:Recurrence; D017211:Treatment Failure
chemical_list: D007166:Immunosuppressive Agents; D000069594:Abatacept