Outcomes in Progressive Pulmonary Fibrosis in Systemic Autoimmune Rheumatic Diseases: Real‐World Data From the Interstitial Lung Disease (ILD) ‐ PRO Registry
Authors' conclusion
In a large prospective multicenter US cohort, SARD-PPF was characterized by advanced physiologic impairment, high treatment burden, and high risk for further progression and death or lung transplant. After accounting for demographic factors and baseline severity, outcomes were broadly similar across SARD diagnoses
Study context
- Population
- Patients with SARD-associated progressive pulmonary fibrosis (ILD other than idiopathic pulmonary fibrosis, reticulation and traction bronchiectasis on high-resolution CT and/or lung biopsy, PPF criteria met within prior 24 months), ILD-PRO Registry, US
- Participants
- 585 patients
- Exposure
- SARD subtypes of progressive pulmonary fibrosis
- Outcome
- Clinical outcomes by SARD subtype
- Follow-up
- 24 months
Methodological detailsmulticentre, US registry, real-world data
- Multicentre
- US registry
- Real-world data
Main finding
As reported in the abstract
ILD progression across SARD subtypes
31.3% to 62.1%
Death or lung transplant across SARD subtypes
9.3% to 37.6%
Observational data: associations, not effects.
Further findings
Baseline characteristics at enrollment
- Median FVC, % predicted
- 64.5%
- Median DLCO, % predicted
- 38.0%
- Supplemental oxygen use
- 39.2%
- Receiving immunomodulatory therapy
- 73.9%
- Taking nintedanib
- 22.6%
Original publication
Outcomes in Progressive Pulmonary Fibrosis in Systemic Autoimmune Rheumatic Diseases: Real‐World Data From the ILD ‐ PRO Registry. Arthritis Rheumatol. Published 4 August 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →