Registry Documents High Progression Risk in Autoimmune Rheumatic Disease–Associated Pulmonary Fibrosis

Researchers used the prospective, multicenter US ILD-PRO Registry to examine baseline characteristics, treatment patterns, and outcomes in 585 patients with systemic autoimmune rheumatic disease–associated progressive pulmonary fibrosis (SARD-PPF). Participants had interstitial lung disease other than idiopathic pulmonary fibrosis, qualifying fibrotic findings on imaging and/or biopsy, and had met PPF criteria within the preceding 24 months. Outcomes included ILD progression and death or lung transplantation.

At enrollment, median forced vital capacity (FVC) was 64.5% predicted and median diffusing capacity for carbon monoxide was 38.0% predicted. Supplemental oxygen was used by 39.2%, immunomodulatory therapy by 73.9%, and nintedanib by 22.6%. Across SARD subtypes, 24-month estimates ranged from 31.3% to 62.1% for ILD progression and from 9.3% to 37.6% for death or lung transplantation. Rheumatoid arthritis–associated PPF had the highest unadjusted probability of progression, but subtype differences were not significant after adjustment for age, sex, and/or baseline FVC.

The findings support attention to physiologic severity and progression phenotype, rather than diagnosis alone, when assessing risk in SARD-PPF. This observational study does not establish treatment effects or prove equivalent outcomes across subtypes. Only the abstract was available; detailed outcome definitions, subtype sample sizes, and precision estimates could not be assessed.