Progressive fibrosis linked to high mortality in connective tissue disease-ILDs

Progressive fibrosing interstitial lung disease was associated with poorer survival in patients with connective tissue disease-associated ILDs, according to new results published in Respiratory Medicine.

“Despite the advances in management and treatment, some patients with [connective tissue disease-associated] ILD still develop [progressive fibrosing] ILD,” Yu-Hsiang Chiu, from the department of rheumatology and clinical immunology at the University Medical Centre Utrecht, Utrecht, the Netherlands and the division of rheumatology/immunology/allergy at Tri-Service General Hospital at the National Defense Medical Center, Taipei, Taiwan, and colleagues wrote. “To optimize management of [connective tissue disease-associated ILD], predictors for progressive fibrosis early in the disease course are needed.”

Researchers performed a retrospective, single-center cohort study composed of 150 patients with connective tissue disease-associated ILD (CTD-ILD) from 2004 to 2018. The investigators analyzed clinical and biochemical data, pulmonary function tests and high-resolution CT results to identify clinical predictors of CTD-ILD with poor outcomes.

Thirty (20%) deaths occurred during a median follow-up of 40 months. Six deaths were attributed to pulmonary infection, 10 to respiratory failure due to progressive fibrosing ILD and 14 to other causes.

Seventy-six patients (50.7%) had progressive fibrosing ILD. Progressive fibrosing ILD was associated with poor overall survival (adjusted HR = 5.73; 95% CI, 1.17-28.11).

Factors independently associated with increased mortality in included age, smoking, C-reactive protein and steroid use.

In addition, patients with diabetes (adjusted OR = 4.52; 95% CI, 1.1-18.51), those who used steroids (adjusted OR = 2.26; 95% CI, 1.04-4.93) and those with a fibrotic high-resolution CT pattern at baseline (adjusted OR = 3.11; 95% CI, 1.15-8.38) had increased risk for progressive fibrosing ILD.

“Since antifibrotic treatment can be used in addition to intensive immunosuppressive treatment nowadays, it is important to closely monitor patients with CTD-ILD for [progressive fibrosing] ILD,” the researchers wrote. “Furthermore, education on smoking cessation and minimizing steroid use are also crucial in managing [connective tissue disease associated]-ILD.”