7.3 Successful use of anakinra, a soluble IL-1 receptor antagonist, in pediatric rheumatic diseases associated macrophage activation syndrome/reactive hemophagocytic lymphohistiocytosis
Bibliographic record
Abstract
Increased interleukin 1 (IL-1) production characterizes macrophage activation syndrome/reactive hemophagocytic lymphohistiocytosis (MAS/rHLH), a potentially lethal complication of pediatric rheumatic diseases. Standard treatment (corticosteroids, cyclosporine, +/- IVIg) is not always effective. To test effectiveness of anakinra, a soluble IL-1 receptor antagonist, in pediatric rheumatology patients who failed to respond to standard MAS/rHLH therapy. 6 pediatric rheumatology patients (3 F:3 M); mean (range) age 8.14 (0.5–13.3) years, with MAS/rHLH were enrolled (SoJIA n = 4; Churg Strauss vasculitis n = 1; and infant onset ANCA +ve pulmonary renal syndrome n = 1). The infant patient was moribund in ICU. Histiocytosis society's 2004 criteria (HLH2004), including a T-cell activation marker, soluble IL-2 receptor (sIL2r), were used to confirm MAS/rHLH. Subcutaneous anakinra (2 mg/kg/day) was added to existing therapy (high dose IV Methylprednisolone n = 5/6; IVIg n = 6/6, and cyclosporine n = 5/6). HLH2004 specified clinical and laboratory data were collected pre, 48 hours, and 2 weeks after initiation of anakinra; including sIL2r in 3/6 patients. All patients defrevesced within 24 hours of first anakinra dose, and ventilatory and dialysis support was discontinued within 96 hours in the ICU patient. All patients recovered from MAS/rHLH by 2 weeks; 5/6 pts discontinued corticosteroids by 5 weeks. Abnormally elevated baseline sIL2r resolved by 48 hours post first dose of anakinra in all 3 patients tested. 1) Anakinra, in combination with corticosteroids, IVIG, +/- cyclosporine, was effective in controlling MAS/rHLH in all patients, allowing rapid discontinuation of corticosteroids in 5/6 patients. 2) Elevated baseline sIL2r level normalized rapidly following anakinra, suggesting resolution of abnormal T-cell activation.
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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 | 0.000 | 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) | 0.002 | 0.001 |
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".