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2015 ; 2
(ä): 13
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A case report of adrenocorticotropic hormone to treat recurrent focal segmental
glomerular sclerosis post-transplantation and biomarker monitoring
#MMPMID25853133
Anwar S
; Larson DS
; Naimi N
; Ashraf M
; Culiberk N
; Liapis H
; Wei C
; Reiser J
; Brennan DC
Front Med (Lausanne)
2015[]; 2
(ä): 13
PMID25853133
show ga
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-?3 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, ?3 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-?3 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.
CONCLUSION: ACTH therapy was associated with improvement in renal function within
4?weeks. The use of rFSGS biomarkers may aid in predicting development of rFSGS.