PF-ILD predicts poor outcomes in connective tissue disease associated-ILD

Progressive fibrosing ILD (PF-ILD) is associated with increased mortality in patients with connective tissue disease associated-ILD, according to a study

In this retrospective single-center cohort study, clinical and biochemical data, pulmonary function tests (PFT) and high-resolution computed tomography (HRCT) results from 150 patients with CTD-ILD were included.

During the median follow-up of 40 months, 30 (20%) patients died, with 6 (4%) deaths attributed to pulmonary infection, 10 (7%) attributed to respiratory failure due to PF-ILD, and 14 attributed to “other causes”

Half of all patients had PF-ILD (50.7%; n = 76) and it was associated with poor overall survival.

Additional factors independently associated with increased mortality include age, smoking, C-reactive protein, and steroid-use.

There was a higher risk of PF-ILD in patients with diabetes mellitus, steroid-use, and a fibrotic HRCT pattern at baseline.

Reference
Chiu YH, Spierings J, de Jong PA, et al. Predictors for progressive fibrosis in patients with connective tissue disease associated interstitial lung diseases. Respir Med. 2021;187:106579. doi: 10.1016/j.rmed.2021.106579. Epub ahead of print. PMID: 34438351.