Predictive factors and clinical outcomes of progressive pulmonary fibrosis in anti-threonyl (PL7) positive anti-synthetase syndrome
Authors' conclusion
PL7-ASS patients with ILD are at significant risk of developing PPF, which is associated with poor survival outcomes. Elevated LDH and CA125 levels may serve as reliable predictors of PPF
Study context
- Population
- PL7-positive ASyS patients with interstitial lung disease (ILD) at a single tertiary centre, January 2018–December 2022 (mean age 54.5 years; 73.9% female)
- Participants
- 69 patients
- Outcome
- Development of progressive pulmonary fibrosis (PPF) and its predictive factors
Methodological detailssingle tertiary centre
- Single tertiary centre
Main finding
As reported in the abstract
Progressive pulmonary fibrosis (PPF) developed
39.1%
Elevated LDH, independent predictor of PPF: HR
1.021
95% CI 1.002–1.04P = 0.031
Elevated CA125, independent predictor of PPF: HR
1.164
95% CI 1.023–1.326P = 0.022
Observational data: associations, not effects.
Further findings
| Outcome | HR for death (95% CI) | P value | |
|---|---|---|---|
| Death, PPFvs no PPF (significantly lower survival with PPF) | 5.120 (1.566–16.740) | P = 0.018 | |
| 0.050.21520HR for death (log scale) |
Baseline CT patterns
- Cellular non-specific interstitial pneumonia (cNSIP)
- 29%
- Fibrotic NSIP (fNSIP)
- 18.8%
- Organizing pneumonia (OP)
- 14.5%
- OP-cNSIP overlap
- 15.9%
- OP-fNSIP overlap
- 14.5%
- Usual interstitial pneumonia (UIP)
- 7.2%
Survival and PPF prevalence by CT pattern (UIP or OP-NSIP overlap vs other patterns)
- Worse survival with UIP or OP-NSIP overlap patterns
- P = 0.0479
- Higher PPF prevalence with UIP or OP-NSIP overlap patterns
- P = 0.029
Original publication
Predictive factors and clinical outcomes of progressive pulmonary fibrosis in anti-threonyl (PL7) positive anti-synthetase syndrome. Rheumatology (Oxford). Published 3 June 2025. doi:10.
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