IPF tied to lower survival than SARD-ILD

Patients with systemic autoimmune rheumatic disease (SARD)-associated interstitial lung disease (ILD) tend to live longer than those with idiopathic pulmonary fibrosis (IPF), reveals a study.

A retrospective analysis was performed in 410 patients with ILD (154 IPF, 256 SARD-ILD) from six centres in Italy. SARD-ILD subtypes were as follows: antisynthetase syndrome (ASyS; n=58), dermatomyositis (DM; n=55), systemic sclerosis (SSc; n=106), and Sjogren disease (SjD; n=37). Five-year survival and pulmonary function test (PFT) changes were the primary outcomes.

At 5 years, patients with IPF showed a lower survival than those with SARD-ILD (mean 33.6 vs 56.0 months; p<0.001). Survival across SARD subtypes was comparable: 58.3 percent in patients with ASyS, 52.9 months in DM, 55.2 months in SSc, and 58.6 months in SjD.

Significant functional improvements were seen among patients with ASyS and DM, with forced vital capacity (FVC) rising from 71 percent to 81 percent in ASyS and from 69 percent to 78 percent in DM. However, those with IPF showed a decline in FVC, from 78 percent to 72 percent.

Usual interstitial pneumonia pattern was universal in IPF but seen in less than one in five (<20 percent) patients with SARD-ILD. ILD pattern did not significantly modify the functional trajectory among those with SARD-ILD, but rather the diagnosis was the primary determinant (multivariable ANOVE p<0.001).

In multivariable analysis, SARD-ILD was ascertained to be a favourable prognostic factor (adjusted hazard ratio [aHR], 0.21), with age (aHR, 1.06) and male sex (aHR, 1.98) predicting poorer outcomes.

“Functional trajectories improved in patients with ASyS and DM, in contrast to the decline observed in those with IPF,” the authors said. “Prognosis is more strongly influenced by the underlying diagnosis, supporting a diagnosis-centred approach to disease management.”