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Abstract: PO1653

A Multicenter, Prospective, Open-Labeled Study of Acthar Gel Alone or with Tacrolimus to Reduce Urinary Proteinuria in Patients with Idiopathic DNA-JB9-Positive Fibrillary Glomerulopathy

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Tumlin, James A., Emory University, Atlanta, Georgia, United States
  • Lafayette, Richard A., Stanford University, Stanford, California, United States
  • Bomback, Andrew S., Columbia University, New York, New York, United States
  • Podoll, Amber S., University of Colorado - Anschutz Medical Campus, Aurora, Colorado, United States

Group or Team Name

  • FACT Trial Group
Background

Fibrillary Glomerulopathy (FGN) is a rare primary glomerular disease characterized by glomerular accumulation of nonbranching, randomly arranged 10-30 nm in diameter fibrils. The resulting podocyte dysfunction and progressive proteinuria leads to ESRD rates of 50% within 4 years. Herein we present data on 15 patients treated with ACTH or ACTH + Tacrolimus completing 12 months of therapy.

Methods

Study Design: Randomized prospective open labeled study of 12 months of SQ ACTH alone or with Tacrolimus in 15 patients with biopsy proven, DNA-B9 + Fibrillary glomerulopathy.
Study Drug Dosing: ACTH 80 units SQ 2X/week, Tacrolimus-1.0 mg PO BID.
Primary Endpoint: Change in UP/Cr ratio (mg/gm) in patients after 12 months of ACTHar gel alone or in combination with Tracrolimus
Definitions: Responders-Complete-Partial-Clinical response defined at < 300 mg/gm or > 50% reduction or > 30% reduction in UP/Cr from baseline at 12 months.

Results

Of the 15 patients completing 12 months of treatment, 14.3% acheived complete remission (UP/Cr ratio < 300 mg/gm); 26.0% acheived a > 75% reduction from baseline, while 60.7% acheived a > 50% reduction in UP/Cr at 12 months. A total of 86.6% acheived a minimum 30% reduction in UP/Cr. The addition of Tacrolimus to ACTH tended to further lower UP/Cr at 12 months (1654.7+317 vs.4449.5+1665 ) respectively, but did not reach statistical significance

Conclusion

In summary, depo-repository ACTH induced a complete or partial remission in 75% of patients with DNA-JB9 + Fibrillary GN. The addition of Tacrolimus in this population tended to improve complete-partial response rates.

Responder Group

Non-Responder Group

Funding

  • Commercial Support