Clinicopathological Profile and Outcomes of Kidney Amyloidosis: A Single-Centre Retrospective Biopsy Registry Cohort Study
Bibliographic record
Abstract
Background: Kidney is the most common organ involved in systemic amyloidosis. The two most common types of amyloidosis affecting the kidneys are immunoglobulin (Ig)-derived and AA amyloidosis. Although classified under the same bracket of amyloidosis, each of these subtypes vary in their presentation, histology and outcomes which is not well defined. Methods: This was an observational retrospective biopsy registry cohort study. done at a single centre in South East Asia. In total, 260 consecutive patients of amyloidosis who underwent kidney biopsy between January 2005 and December 2022 were included in this study. Results: The mean age of the cohort was 52.5 ± 11.4 years and 67.3% were males. The most common histological type of amyloidosis was Ig amyloidosis (73.1%) followed by AA (14.6%) and undifferentiated amyloidosis (11.5%). Ig amyloidosis included 186 patients of AL amyloidosis and 4 patients of AHL amyloidosis. Extra-kidney involvement was seen in 22.7% patients. Patients with AA amyloidosis were younger, had a lower baseline estimated glomerular filtration rate (eGFR) and none of the patients had cardiovascular involvement. Patients with Ig amyloidosis were older, and almost half of the patients presented with nephrotic syndrome. Histologically, half of the patients had both glomerular and vascular deposits. Vascular and interstitial involvement was less commonly seen in undifferentiated amyloidosis. Majority (82%) of patients with Ig amyloidosis had lambda monoclonal light chain deposits. CyBorD was the most used chemotherapeutic regimen in Ig amyloidosis (38%). Of the 121 patients with follow-up >3months, 21 (17%) patients progressed to kidney failure over a median period of 11 (IQR, 8.5-29.5 months). The predictors of kidney failure were baseline eGFR < 30 ml/min, extensive glomerular amyloid deposits, moderate to severe IFTA and persistent proteinuria at follow-up. On Cox proportional hazard model, the only significant predictor was baseline eGFR < 30 ml/min [ HR 4.37(95% CI 1.01-18.88)]. Patients of Ig amyloidosis who did not attain VGPR were more likely to have persistent proteinuria at follow up (73% vs. 42%). Conclusion: Clinicopathological presentation and outcomes differ based on the type of amyloidosis. Attainment of VGPR is associated with better kidney outcomes in Ig amyloidosis.
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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 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.000 | 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".