Prognostic and Functional Trajectories in Idiopathic Pulmonary Fibrosis and Systemic Autoimmune Rheumatic Disease–Associated Interstitial Lung Disease: Insights From an Italian Multicenter Cohort
Authors' conclusion
SARD-ILD is associated with higher survival than IPF. Functional trajectories improved in patients with ASyS and DM, in contrast to the decline observed in those with IPF.
Study context
- Population
- Patients with interstitial lung disease (ILD) (idiopathic pulmonary fibrosis (IPF) or SARD-ILD) from 6 Italian centres
- Participants
- 154 / 256 / 58 / 55 / 106 / 37
- Exposure
- IPF vs SARD-ILD
- IPFn = 154reference
- SARD-ILDn = 256
- Antisynthetase syndrome (ASyS)n = 58
- Dermatomyositis (DM)n = 55
- Systemic sclerosis (SSc)n = 106
- Sjögren disease (SjD)n = 37
- Outcome
- Survival (5-year) and pulmonary function test (PFT) changes
- Follow-up
- 5 years
Methodological detailsmulticentre
- Multicentre
Main finding
As reported in the abstract
5-year survival, mean
P < 0.001
Observational data: associations, not effects.
Further findings
| Outcome | Values | aHR |
|---|---|---|
| 5-year survival by SARD subtype, mean (comparable) | ASyS 58.2 months · DM 52.9 months · SSc 55.2 months · SjD 58.6 months | |
| FVC change | ASyS 71% to 81% · relative change +14.1% · DM 69% to 78% · relative change +13% · IPF 78% to 72% · relative change −7.7% | |
| Usual interstitial pneumonia pattern | IPF universal · SARD-ILD < 20% | |
| Prognostic factors (multivariable analysis)SARD-ILD vs IPF · favorable prognostic factor | 0.21 |
- Prognostic factors (multivariable analysis) — Age, aHR
- 1.06
- Prognostic factors (multivariable analysis) — Male sex, aHR
- 1.98
Forced vital capacity (FVC) change
- Significant functional improvement in ASyS and DM
Determinant of functional trajectory in SARD-ILD
- ILD pattern did not significantly influence functional trajectory; diagnosis was the primary determinant (multivariable ANOVA)
- P < 0.001
Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.
Original publication
Prognostic and Functional Trajectories in Idiopathic Pulmonary Fibrosis and Systemic Autoimmune Rheumatic Disease–Associated Interstitial Lung Disease: Insights From an Italian Multicenter Cohort. J Rheumatol. Published 1 February 2026. doi:10.
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