Efficacy of Acthar gel and Tacrolimus in DNA-JB9 Positive Fibrillary Glomerulopathy
Bibliographic record
Abstract
Introduction Fibrillary glomerulopathy (FGN) is a rare glomerular disease characterized by the deposition of randomly arranged fibrils that results in proteinuria and ESRD in up to 50% within two 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 depository ACTH (ACTHar gel) alone or in combination with Tacrolimus on proteinuria and change in eGFR. Methods Patients (N=34) were randomized to ACTH 80 units SQ 2X/week alone or in combination with Tacrolimus (1.0 mg BID) for 12 months. Changes in the mean UPCR and eGFR were reported at 6 and 12 months and last follow up. Results A total of 34 patients completing one year of therapy were analyzed. In the ACTH-alone group (19 pts), UPCR fell from a mean of 6.21+0.8 gm/gm at baseline to 2.92+0.90 gm/gm (p<0.009) at six months and 1.76+1.3 gm/gm (p<0.02) by month 12. In the combination group (15 pts) mean UPCR at baseline fell significantly from 6.00+1.4 gm/gm to 4.27+1.1 gm/gm (P<0.01) and to 1.83+0.90 gm/gm (P<0.0006) at 6 and 12 months respectively. At 12 months, combination therapy induced complete or partial responses in 13.3% and 53.3% respectively compared to 15.8% and 26.3% in the ACTH alone group. Conclusions Depo-repository ACTH (ACTHar gel) significantly reduced UPCR at 6 and 12 months. The addition of tacrolimus was not additive with ACTH in reduction of proteinuria or stabilization of eGFR. ACTHar gel is an effective anti-proteinuric therapy for DNA-JB9 positive fibrillary glomerulopathy.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".