Management of antineutrophil cytoplasmic antibody vasculitis-associated orbital inflammatory disease: A systematic literature review
Bibliographic record
Abstract
OBJECTIVE: Ocular manifestations frequently occur in ANCA-associated vasculitides (AAV). Orbital inflammatory disease (OID) is a subset with limited management guidelines. This systematic review evaluated interventions for AAV-OID. METHODS: We searched Embase, MEDLINE, Web of Science, ClinicalTrials.gov, and Cochrane Central without language or date restrictions for clinical trials, case-control studies, observational studies, and case series (≥5 patients) of adults treated for AAV-OID. Included studies reported therapy type and clinical response after ≥1 month. The primary outcome was clinical response, defined as any improvement in signs or symptoms, up to and including remission. Secondary outcomes included remission, relapse, sustained remission (>6 months), and serious adverse events. Two reviewers independently screened records and appraised studies using the Newcastle-Ottawa Scale (NOS). A qualitative synthesis and meta-analysis of proportions were performed using a random effects model. RESULTS: Of 1001 studies screened, 18 met inclusion criteria (277 patients; 14 retrospective cohorts, 4 case series). Most (17/18) had NOS scores ≥5. Thirteen studies included rituximab (RTX), 10 cyclophosphamide, 7 RTX + another immunosuppressant (IS), 11 conventional IS, and 7 surgical therapies. Most studies involved refractory/relapsing cases. In 4 RTX monotherapy series, 95% (95 %CI 89-100%, p < 0.001) had a clinical response and 84% (95%CI 72-95%, p < 0.001) achieved remission at 6 months. CONCLUSION: Our results suggest high remission rates of OID with RTX but highlight the need for high-quality prospective studies examining treatments for AAV-OID.
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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 | 0.006 | 0.022 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.008 | 0.007 |
| Bibliometrics | 0.010 | 0.009 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.002 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.005 | 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".