{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T11:40:25Z","timestamp":1756294825769,"version":"3.44.0"},"reference-count":11,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2016,2,3]]},"abstract":"<jats:p>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\u2005years 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\u2014though he did not experience any side effects, there was no change in proteinuria nor in creatinine.<\/jats:p>","DOI":"10.1136\/bcr-2016-214396","type":"journal-article","created":{"date-parts":[[2016,2,17]],"date-time":"2016-02-17T07:32:47Z","timestamp":1455694367000},"page":"bcr2016214396","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":7,"title":["Abatacept experience in steroid and rituximab-resistant focal segmental glomerulosclerosis"],"prefix":"10.1136","volume":"2016","author":[{"given":"Vinothkumar","family":"Kavarthapol Jayaraman","sequence":"first","affiliation":[{"name":"Royal Perth Hospital, Perth, Western Australia, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark","family":"Thomas","sequence":"additional","affiliation":[{"name":"Royal Perth Hospital, Perth, Western Australia, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2016,2,17]]},"reference":[{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.1","doi-asserted-by":"publisher","DOI":"10.5414\/CNP63001"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(97)90485-6"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1106556"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.4","first-page":"53","article-title":"The impact of prolonged immunosuppression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephrotic syndrome in adults. A collaborative retrospective study","volume":"36","author":"Banfi","year":"1991","journal-title":"Clin Nephrol"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.5","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfs563"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.6","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/13.6.1524"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1304572"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc063706"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.9","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.00570109"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.10","doi-asserted-by":"crossref","unstructured":"Novelli R , Gagliardini E , Ruggiero B , et al . Any value of podocyte B7-1 as a biomarker in human MCD and FSGS? Am J Physiol Renal Physiol 2015. doi:10.1152\/ajprenal.00510.2015","DOI":"10.1152\/ajprenal.00510.2015"},{"key":"2025082704060873000_2016.feb17_1.bcr2016214396.11","doi-asserted-by":"crossref","unstructured":"Delville M , Baye E , Durrbach A , et al . B7-1 blockade does not improve post-transplant nephrotic syndrome caused by recurrent FSGS. J Am Soc Nephrol 2015:Dec 23. pii: ASN.2015091002. [Epub ahead of print]","DOI":"10.1681\/ASN.2015091002"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2016-214396","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T11:06:16Z","timestamp":1756292776000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2016-214396"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,2,17]]},"references-count":11,"alternative-id":["10.1136\/bcr-2016-214396"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2016-214396","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2016,2,17]]}}}