title: A case report of adrenocorticotropic hormone to treat recurrent focal segmental glomerular sclerosis post-transplantation and biomarker monitoring.
abstract: BACKGROUND
  Recurrent focal segmental glomerular sclerosis (rFSGS) in renal transplant recipients (RTR) is difficult to predict and treat. Early rFSGS is likely from circulating factors and preformed antibodies.
  
  
  METHODS
  We present the case of a 23-year-old white man who presented with rFSGS and acute renal failure, requiring dialysis 9-months after a 1-haplotype matched living-related transplant. We retrospectively analyzed serum samples from various clinical stages for rFSGS biomarkers: serum glomerular albumin permeability (Palb), soluble urokinase-type plasminogen activator receptor (suPAR) serum level with suPAR-β3 integrin signaling on human podocytes, and angiotensin II type I receptor-antibody (AT1R-Ab) titer.
  
  
  RESULTS
  All biomarkers were abnormal at 1-year pre-transplant prior to initiation of dialysis and at the time of transplant. After initiation of hemodialysis, β3 integrin activity on human podocytes, in response to patient serum, as well as AT1R-Ab were further elevated. At the time of biopsy-proven recurrence, all biomarkers were abnormally high. One week after therapy with aborted plasmapheresis (secondary to intolerance), and high dose steroids, the Palb and suPAR-β3 integrin activity remained significantly positive. After 12-weeks of treatment with high-dose steroids, rituximab, and galactose, the patient remained hemodialysis-dependent. Three-months after his initial presentation, we commenced adrenocorticotropic hormone (ACTH, Acthar(®) Gel), 80 units subcutaneously twice weekly. Four-weeks later, he was able to discontinue dialysis. After 8-months of maintenance ACTH therapy, his serum creatinine stabilized at 1.79 mg/dL with <1 g of proteinuria.
  
  
  CONCLUSIONS
  ACTH therapy was associated with improvement in renal function within 4 weeks. The use of rFSGS biomarkers may aid in predicting development of rFSGS.
authors: Department of Medicine, Washington University School of Medicine , St. Louis, MO , USA.
authors: Siddiq
authors: S
authors: Anwar
authors: Department of Medicine, Washington University School of Medicine , St. Louis, MO , USA.
authors: Derek S
authors: DS
authors: Larson
authors: Department of Medicine, Washington University School of Medicine , St. Louis, MO , USA.
authors: Nima
authors: N
authors: Naimi
authors: Department of Medicine, Washington University School of Medicine , St. Louis, MO , USA.
authors: Muhammad
authors: M
authors: Ashraf
authors: Department of Medicine, Washington University School of Medicine , St. Louis, MO , USA.
authors: Nancy
authors: N
authors: Culiberk
authors: Department of Pathology, Washington University School of Medicine , St. Louis, MO , USA.
authors: Helen
authors: H
authors: Liapis
authors: Department of Medicine, Rush University Medical Center , Chicago, IL , USA.
authors: Changli
authors: C
authors: Wei
authors: Department of Medicine, Rush University Medical Center , Chicago, IL , USA.
authors: Jochen
authors: J
authors: Reiser
authors: Department of Medicine, Washington University School of Medicine , St. Louis, MO , USA.
authors: Daniel C
authors: DC
authors: Brennan
keywords: acute kidney injury; albumin permeability factor; angiotensin 1 receptor antibody; podocyte; recurrent focal segmental glomerular sclerosis; soluble urokinase plasminogen activator receptor