Granulomatosis With Polyangiitis (Wegener Granulomatosis): Then and Now
Bibliographic record
Abstract
Context.—: In 1954 Godman and Churg published an article in the Archives of Pathology & Laboratory Medicine entitled "Wegener's Granulomatosis" (now replaced by "granulomatosis with polyangiitis" [GPA]) and linked various forms of what is currently recognized as pauci-immune small-vessel vasculitis. Objective.—: To put the 1954 article by Godman and Churg into context and review current ideas of GPA. Data Sources.—: Published literature. Conclusions.—: Godman and Churg proposed the name Wegener's granulomatosis and defined the essential features as granulomatous inflammation and necrotizing vasculitis involving, typically, the upper respiratory tract, lung, and kidney. They suggested that GPA, allergic angiitis, and granulomatosis (later known as Churg-Strauss syndrome, now "eosinophilic granulomatosis with polyangiitis" [EGPA]), and what were referred to as "microscopic forms of periarteritis nodosa," now "microscopic polyangiitis," were related entities producing small-vessel vasculitis. We currently recognize these diseases as the spectrum of pauci-immune ANCA (anti-neutrophil cytoplasmic antibody)-associated small-vessel vasculitis, in contrast to small-vessel vasculitis characterized by immune complex deposits. ANCAs are antibodies directed against the neutrophil proteins proteinase 3 (PR3, usually associated with GPA) and myeloperoxidase (MPO, usually associated with microscopic polyangiitis and EGPA). A modern definition of GPA discloses that it can affect any organ in the body, characteristically with upper respiratory tract involvement. In the lung it is associated with necrotizing nodules and/or capillaritis with hemorrhage. It has been proposed that these forms of vasculitis should in the future be primarily classified by using ANCA specificity-that is, PR3 or MPO-as a disease definition, based on observations that the type of ANCA is what determines the location/pattern of organ involvement.
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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) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.003 |
| Science and technology studies | 0.001 | 0.004 |
| Scholarly communication | 0.003 | 0.005 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.003 | 0.005 |
| Insufficient payload (model declined to judge) | 0.003 | 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".