Bibliographic record
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis. The disease can present with predominantly proliferative features, such as swollen lacrimal and salivary glands, orbital pseudotumor, autoimmune pancreatitis, polyclonal hypergammaglobulinemia (PHGG), eosinophilia, and tubulointerstitial nephritis of the kidneys, or predominantly fibrotic disease, including mediastinal and retroperitoneal fibrosis, sclerosing mesenteritis, and hypertrophic pachymeningitis. This review focuses on 4 key hematological manifestations: PHGG, IgG4-positive plasma cell enriched lymphadenopathy (LAD), eosinophilia, and retroperitoneal fibrosis (RPF). These features are found in 70%, 60%, 40%, and 25% of IgG4-RD patients, respectively, but can also represent key hematological "mimickers" of IgG4-RD, including Castleman disease (PHGG, LAD), eosinophilic vasculitis (eosinophilia, PHGG, LAD), hypereosinophilic syndromes (eosinophilia, LAD, PHGG), and histiocyte disorders (PHGG, LAD, RPF). An organized approach to these 4 manifestations, and how to distinguish IgG4-RD from its mimickers, is explained. Proliferative manifestations typically respond very well to treatment corticosteroids, rituximab, and other immunosuppressives, whereas chronic fibrotic disease may not be reversible with current treatment modalities.
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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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.003 | 0.002 |
| 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 | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 0.003 |
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".