HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH)/MACROPHAGE ACTIVATION SYNDROME (MAS) IN SYSTEMIC LUPUS ERYTHEMATOSUS - PREVALENCE, DRIVERS, TREATMENT AND PROGNOSIS OF A SINGLE-CENTER MULTIDISCIPLINARY HLH/MAS TEAM
Bibliographic record
Abstract
PV142 / #812 Poster Topic: AS17 - Miscellaneous Background/Purpose Haemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) can, though rarely, be the first presentation of systemic lupus erythematosus (SLE). It may also develop during the course of SLE, often triggered by disease flares, infections, medications, or concomitant neoplasms, especially lymphoma. While the mortality rate of HLH/MAS episodes in SLE is lower than in other conditions driving to HLH/MAS, it remains higher than that of non-HLH SLE-related hospital admissions. Aim: To describe the experience of a single tertiary center experience with both SLE and HLH/MAS cohorts, focusing on the demographics, drivers, treatment, and prognosis of HLH/MAS complicating SLE. Methods HLH/MAS episodes were identified from our retrospective SLE cohort (1985-2023). Subsequent review of demographics, drivers, treatment and outcomes of SLE patients in our HLH/MAS cohort, which includes retrospective cohort (1-1-2004 to 31-12-2018) and a prospective cohort following the establishment of a multidisciplinary team (MDT) (1-1-2019 to 31-12-2023). Results HLH/MAS complicating SLE occurred in 9 patients out of the 974 SLE patients (1985-2023, all cases since 2004), representing a 0.92% prevalence. These 9 patients experienced had 11 HLH/MAS episodes, accounting for a subset of our HLH/MAS cohort of 54 patients with 60 episodes. All patients were female, with ages ranging from 19 to 44 years old at the time of their first HLH/MAS episode. In 5 patients (55.5%), HLH/MAS was the initial presentation of SLE. Concomitant triggers included infections in 7 episodes (bacterial, 1 post-COVID, 1 post-CMV) and lupus flares in 4 episodes (2 neuropsychiatric, 1 lupus nephritis, 1 fever and lymphadenopathy). Treatment options for HLH/MAS included glucocorticoids (various dosages of methylprednisolone boluses, followed by prednisolone) and IVIG, which were administered to all patients. Additional therapies included short cycles of high-dose intravenous anakinra, cyclophosphamide, rituximab, ruxolitinib, and baricitinib. One patient died (11.1%) due to multiorgan dysfunction and hemorrhage within the first 48h of treatment. Compared to other HLH/MAS drivers, SLE had better prognosis (lymphoproliferative HLH-related mortality: 58,8% (10/17 patients); solid tumor HLH-related mortality: 40% (2/5 patients); HIV infection HLH-related mortality: 42,9% (3/7 patients); Chronic Active Epstein-Barr virus infection HLH-related mortality: 100% (2/2 patients)). Only 2 patients with SLE experienced HLH/MAS episodes following the establishment of the HLH/MAS MDT. Conclusions Our findings align with the limited published data on HLH/MAS in SLE. It highlights that although HLH/MAS-related mortality in SLE is lower than in other HLH/MAS drivers, it remains significantly higher than that of non-HLH/MAS severe lupus flares.
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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 | 0.001 | 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) | 0.003 | 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".