Bibliographic record
Abstract
Hemophagocytic lymphohistiocytosis (HLH) represents a potentially life-threatening episode of overwhelming inflammation characterized by activation of T lymphocytes, hemophagocytic macrophages, and marked hyperferritinemia1,2. Historically, HLH has been classified as having a primary, genetic cause (familial HLH) or occurring secondary to an identified trigger including various infections, malignancies, or rheumatic diseases. When occurring in the setting of inflammatory or autoimmune disease, rheumatologists call secondary HLH the macrophage activation syndrome (MAS), and it is reflected as MAS-HLH in the updated Histiocyte Society nomenclature3. Episodes of MAS-HLH have been reported in nearly all rheumatic diseases, but are most commonly recognized in patients with systemic juvenile idiopathic arthritis (sJIA) or adult-onset Still disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease, and certain monogenic autoinflammatory disorders4,5. In contrast, the inflammatory myopathies including dermatomyositis (DM) and polymyositis (PM) have typically been considered low-risk for development of MAS-HLH. In the current issue of The Journal , Liang and colleagues6 present a large series of patients with adult idiopathic inflammatory myopathies that challenges this conventional wisdom, and suggest that MAS-HLH may represent an underappreciated cause of mortality in these disorders. In this report, the authors identified 424 adult patients admitted to their institution over an 8-year period with DM, PM, or clinically amyopathic dermatomyositis (CADM)6. Interestingly, they found that 18 (4.2%) satisfied the 2004 diagnostic criteria for HLH7. … Address correspondence to Dr. G.S. Schulert, Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45208 USA. Email: Grant.schulert{at}cchmc.org.
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.003 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.002 | 0.000 |
| Research integrity | 0.001 | 0.008 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".