Management of Anti-Neutrophil Cytoplasmic Antibody Vasculitis-Associated Orbital Inflammatory Disease: A Systematic Literature Review
Bibliographic record
Abstract
Objectives Ocular manifestations are common in ANCA-associated vasculitides (AAV). Orbital inflammatory disease (OID) represents a significant subset and remains a therapeutic challenge, as few management recommendations exist. This systematic review aimed to evaluate the effectiveness and safety of AAV-associated OID interventions. Methods We searched for clinical trials, case-control studies, observational studies and case series (≥ 5 patients) of adults undergoing therapy for AAV-associated OID within Embase Classic + Embase, MEDLINE(R) via Ovid, Web of Science Core Collection, Clinicaltrials.gov , and Cochrane Central Register of Controlled Trials without language or date restrictions. Studies were required to clearly report types and dosing of therapies, and clinical response after at least 1 month of follow-up. The primary outcome was clinical response, defined as any improvement in signs and/or symptoms, up to and including clinical remission. Secondary outcomes included clinical remission, relapse, sustained (>6 months) remission and serious adverse events. Two reviewers independently screened records and critically appraised articles using the Newcastle Ottawa Scale (NOS). We synthesized results qualitatively and performed a meta-analysis of proportions for outcomes with sufficient homogeneity using a random-effects model and restricted maximum likelihood approach. Analyses were conducted using Stata SE (v18.0). Results Of 1001 unique studies identified, 119 were included in the full-text review, and 18 (14 retrospective cohort studies and 4 case series, 277 patients) met inclusion criteria (Table 1). Most (17/18) studies had NOS scores of ≥5 (Good/Satisfactory). Treatment regimens (generally combined with glucocorticoids) varied across and within studies: 13 studies included patients who received rituximab (RTX), 10 studies included patients who received cyclophosphamide (CYC), 7 studies included patients who received RTX plus another immunosuppressant (IS), 11 studies included patients who received conventional IS, and 7 studies included patients treated with surgery. Most studies (12/18) reported aggregate outcomes of multiple therapies. The most common time point for reporting outcomes was 6 months (5 studies, 4 of RTX and 1 of RTX+IS). Of these, 5/5 observed a clinical response in nearly all individuals. In the 4 studies of RTX monotherapy, 84% (95% CI 72%-95%, z=14.35, p< 0.001) achieved a complete response at 6 months. Other secondary outcomes are summarized in Table 1. Conclusion Studies reported on several therapies for AAV-associated OID. Pooled estimates showed high rates of remission with RTX at 6 months, and qualitatively, high rates of remission were also reported for RTX + conventional IS. Our results emphasize the necessity of larger, prospective studies examining treatments for AAV-associated OID.
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 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 | 0.009 | 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) | 0.004 | 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".