Efficacy of Acthar gel and Tacrolimus in DNA-JB9 Positive Fibrillary Glomerulopathy.
Publication/Presentation Date
9-1-2025
Abstract
INTRODUCTION: Fibrillary glomerulopathy (FGN) is a rare glomerular disease characterized by the deposition of randomly arranged fibrils that results in proteinuria and end-stage renal disease (ESRD) in up to 50% of patients within 2 years. The FACT trial is a prospective, randomized, open-labeled study of patients with biopsy-proven, DNA-JB9 positive FGN comparing the safety and efficacy of repository corticotropin (ACTH) injection, Acthar gel alone or in combination with tacrolimus on proteinuria and change in estimated glomerular filtration rate (eGFR).
METHODS: Patients (
RESULTS: A total of 34 patients completing 1 year of therapy were analyzed. In the ACTH-alone group (19 patients), UPCR decreased from a mean of 6.21 ± 0.8 g/g at baseline to 2.92 ± 0.90 g/g (
CONCLUSION: Repository ACTH (Acthar gel) significantly reduced UPCR at 6 and 12 months. The addition of tacrolimus was not additive with ACTH in reducing proteinuria or stabilization of eGFR. Acthar gel is an effective antiproteinuric therapy for DNA-JB9 positive FGN.
Volume
10
Issue
9
First Page
3128
Last Page
3137
ISSN
2468-0249
Published In/Presented At
Tumlin, J. A., Podoll, A., Kopyt, N., Rovin, B., Lafayette, R., Bomback, A., Glassock, R., Whitson, J., Press, A., & Appel, G. B. (2025). Efficacy of Acthar gel and Tacrolimus in DNA-JB9 Positive Fibrillary Glomerulopathy. Kidney international reports, 10(9), 3128–3137. https://doi.org/10.1016/j.ekir.2025.06.042
Disciplines
Medicine and Health Sciences
PubMedID
40980649
Department(s)
Department of Medicine
Document Type
Article