Vasculitis & polymyalgia rheumatica
ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome
Authors' conclusion
Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.
Study context
- Population
- Patients with scleritis diagnosed January 2007–April 2022, French Vasculitis Study Group network and three French tertiary ophthalmologic centres (median age 48 years; 75% female)median follow-up 28 months
- Participants
- 120 patients
- Comparison
- Isolated ANCA-associated scleritis vs idiopathic scleritis with negative ANCA tests
- ANCA-associated scleritisn = 38
- Controln = 82reference
- Outcome
- Need for systemic GC and RTX, ANCA-associated vs ANCA-negative idiopathic scleritis
Methodological detailsmulticentre
- Multicentre
Main finding
As reported in the abstract
ANCA-associated scleritis vs Control: P < 0.001 · ANCA-associated scleritis vs Control: P = 0.03
- Scleromalacia more frequent in ANCA-positive patients
- P = 0.027
Observational data: associations, not effects.
Further findings
Risk factor for progression to systemic ANCA-associated vasculitis (AAV): CRP >5 mg/l at diagnosis
| aHR (95% CI) | P value | ||
|---|---|---|---|
| CRP >5 mg/lOnly significant risk factor | 5.85 (1.10–31.01) | P = 0.038 | |
| 0.050.21520Adjusted hazard ratio (log scale) |
| Outcome | P value |
|---|---|
| Systemic GC required | P < 0.001 |
RTX required
- More frequent in ANCA-associated scleritis
- P = 0.03
Remission after first- and second-line treatment
- Lower remission rate in ANCA-associated scleritis
Progression to systemic AAV in PR3- or MPO-ANCA patients
- Systemic AAV occurred
- 30.7%
- Median interval to AAV, months (IQR 16.3-44)
- 30
Original publication
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