A prospective multicenter US registry study (ILD-PRO) evaluated baseline characteristics, treatment patterns, and clinical outcomes in 585 patients with systemic autoimmune rheumatic disease-associated progressive pulmonary fibrosis (SARD-PPF), comparing outcomes across SARD subtypes over 24 months.
At enrollment, median FVC was 64.5% predicted and median DLco was 38.0% predicted; by 24 months, 31.3%–62.1% experienced ILD progression and 9.3%–37.6% died or underwent lung transplant. After adjusting for age, sex, and baseline FVC, no significant differences in outcomes were found across SARD subtypes.
Observational registry design limits causal inference; enrollment required meeting PPF criteria within the prior 24 months, which may introduce selection bias toward more severe or rapidly progressive disease; unadjusted subtype comparisons (e.g., RA-PPF showing highest progression) may reflect differences in age, sex, and baseline severity rather than true subtype effects.
Clinicians should apply phenotype-focused (rather than SARD-subtype-focused) risk stratification for SARD-PPF, as outcomes are broadly similar across diagnoses once baseline severity is accounted for. Systematic monitoring and timely optimization of therapy—including immunomodulatory agents and nintedanib—are essential given the high rates of progression and death in this population.
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