A case report of adrenocorticotropic hormone to treat recurrent focal segmental glomerular sclerosis post-transplantation and biomarker monitoring.

摘要: Background: Recurrent focal segmental glomerular sclerosis (rFSGS) in renal transplant recipients (RTR) is difficult to predict and treat. Early rFSGS likely from circulating factors preformed antibodies. Methods: We present the case of a 23-year-old white man who presented with acute failure requiring dialysis 9-months after 1-haplotype matched living-related transplant. retrospectively analyzed serum samples various clinical stages for biomarkers: albumin permeability (Palb), soluble urokinase-type plasminogen activator receptor (suPAR) level suPAR-β3 integrin signaling on human podocytes, angiotensin II type I receptor-antibody (AT1R-Ab) titer. Results: All biomarkers were abnormal at 1-year pre-transplant prior initiation time After hemodialysis, β3 activity response patient serum, as well AT1R-Ab further elevated. At biopsy-proven recurrence, all abnormally high. One week therapy aborted plasmapheresis (secondary intolerance), high dose steroids, Palb suPAR- remained significantly positive. 12-weeks treatment high-dose rituximab, galactose, hemodialysis-dependent. Three-months his initial presentation we commenced adrenocorticotropic hormone (ACTH, Acthar® Gel), 80 units subcutaneously twice weekly. Four-weeks later he was able discontinue dialysis. 8-months maintenance ACTH therapy, creatinine stabilized 1.79 mg/dL less than 1 gram proteinuria. Conclusion: associated improvement function within 4 weeks. The use may aid predicting development rFSGS.