Primary antiphospholipid syndrome and the kidney: biopsy-proven insights from two referral Italian centres
Bibliographic record
Abstract
OBJECTIVES: To investigate the prevalence, clinical features, histological spectrum and outcomes of intra-renal involvement in patients with primary APS (PAPS), with particular attention to both aPL nephropathy (aPL-N) and non-aPL-N renal lesions. METHODS: This multicentric retrospective case-control study included 258 PAPS patients followed between 1984 and 2023 at two Italian referral centres. Patients with a biopsy-proven intra-renal involvement (n = 17) were compared with those without renal manifestations. Histologic classification followed the updated aPL-N criteria. Clinical, laboratory, therapeutic features, and 12-month renal responses were assessed. RESULTS: Seventeen of 258 (7%) patients underwent renal biopsy. Two out of 17 (12%) presented with acute kidney injury, 5/17 (29%) with nephrotic syndrome and 5/17 (29%) had isolated urinary abnormalities; the remaining 5 patients presented with different combination of signs/symptoms. Six (2.3%) were diagnosed with aPL-N, while 11 (4.2%) exhibited alternative glomerular lesions (e.g. membranous nephropathy, focal segmental glomerulosclerosis). Compared with controls, patients with intra-renal involvement had higher frequencies of CAPS (18% vs 0.4%, P = 0.001), thrombocytopenia (35% vs 10%, P = 0.006), epilepsy (24% vs 5%, P = 0.018) and LA positivity (100% vs 62%, P = 0.002). At 12 months, partial renal response was observed in most cases, with a worse prognosis in those with aPL-N despite anti-thrombotic treatment. All non-aPL-N patients had aPL-related events, including thrombotic, obstetric and non-thrombotic manifestations, with non-aPL-N renal disease preceding these events in 45%. CONCLUSION: Intra-renal involvement in PAPS is rare but heterogeneous, encompassing both vascular and glomerular pathologies. These findings support the need for heightened renal surveillance in PAPS, even in the absence of classic thrombotic manifestations, and highlight the importance of histologic assessment to guide tailored therapy. From a nephrologist's perspective, routine screening for aPL in glomerular disease assessment may be relevant, particularly by providing prognostic value for subsequent aPL-related events.
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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 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 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".