Current and Emerging Therapies for C3 Glomerulopathy and Primary (Idiopathic) Immune Complex Membranoproliferative Glomerulonephritis
Bibliographic record
Abstract
C3 glomerulopathy (C3G) and primary (idiopathic) immune complex membranoproliferative glomerulonephritis (IC-MPGN) are rare kidney diseases characterized by dysregulation of the complement system and progressive deposition of C3 and its breakdown products in the glomeruli, ultimately leading to kidney failure in up to 50% of patients within 10 years. Until recently, standard approaches to treatment included supportive measures common to many kidney diseases and immunosuppression to mitigate inflammation, rather than specific therapies addressing the underlying C3 dysregulation. However, recent advances in targeted complement inhibitor therapy have been made in these diseases with positive results from phase 3 clinical trials of both the factor B inhibitor, iptacopan (in adults with native kidney C3G) and the C3/C3b inhibitor, pegcetacoplan (in adults and adolescents with native or posttransplant C3G or primary IC-MPGN). In this review, we summarize what is known and what questions still remain regarding the effect of complement inhibitors on widely accepted surrogate end points for efficacy in C3G/primary IC-MPGN (proteinuria, estimated glomerular filtration rate [eGFR], and kidney biopsy histology). Additional controversies, including candidate patient populations, optimal treatment duration, and how best to monitor patients on complement inhibitor therapy are also discussed, in an effort to prepare the nephrology community for innovative therapeutic options for patients whose long-term prognosis has generally been dismal.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| 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 | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".