Features and Outcomes of Patients with C1q Nephropathy in the NEPTUNE and CureGN Cohorts: Comparisons to Minimal Change Disease and Focal Segmental Glomerulosclerosis
Bibliographic record
Abstract
Background: Predominant immunostaining for C1q distinguishes a subset of patients with primary glomerular disease. C1q nephropathy (C1qN) has been proposed but not universally accepted as a distinct glomerular disease. This study describes clinical characteristics and short-term outcomes of patients meeting the provisional CureGN definition of C1qN, including comparisons to patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) without C1qN. Methods: MCD and FSGS patients with C1qN were identified from the Cure Glomerulonephropathy Network (CureGN) and NEPTUNE cohorts. Comparisons were made to MCD and FSGS patients without C1qN, based on age, disease, and time since kidney biopsy, using 5-to-1 matching. We performed cross-sectional analyses of clinical and treatment data at enrollment and a longitudinal analysis of disease course. Results: A total of 42 patients met the provisional CureGN definition for C1qN (16 adults >18yo, 11 teens 12-18yo, and 15 children <12yo), including 15 with MCD and 27 with FSGS. Those with C1qN were more commonly female (60 vs 49%, p=0.2) and black (34 vs 27%, p=0.4). At enrollment, those with and without C1qN had comparable kidney function (eGFR 90 vs 88 mL/min/1.73m2, p=0.8). Individuals with C1qN were equally likely to have been treated with steroids or other immunosuppressive therapy (76 vs 81%, p=0.4) and to have ever achieved complete remission of proteinuria (defined as uPCR <0.3) (54 vs 65%, p=0.2). Median time to last follow-up was 3.1 yrs (IQR 1.9, 4.4) from enrollment and 4.8 yrs (IQR 3.3, 6.5) from biopsy date. While proportions with kidney failure were higher for FSGS compared to MCD (14 vs 1%, p=0.01), they were similar between patients with and without C1qN (7 vs 10%, p=0.6). There was a trend towards steeper GFR slope in C1qN patients (-4.8 vs -0.2 mL/min/yr, p=0.06). Conclusions: FSGS and MCD patients with and without C1qN have comparable demographics and short-term outcomes in CureGN and NEPTUNE. Outcomes do not appear to be biased by differences in immunosuppressive therapies. Further interrogation of genetic and molecular profiles between patients with and without C1q immunostaining on biopsy may be more informative. Funding: Private Foundation Support
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.000 | 0.000 |
| 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".