25 November 2021

Who’s most at risk of dying from ILD complications?

ACR Interstitial Lung Disease

Several key features predict mortality in patients who have connective tissue disease-associated interstitial lung disease (CTD-ILD).

Experts presenting new research at the American College of Rheumatology Convergence 2021 said that follow-up monitoring and patient education may be able to save lives and improve patients’ quality of life.

The study of 150 outpatients with the condition treated at a medical centre between 2004 and 2018 found that several features increased the odds of developing progressive fibrosing ILD (PF-ILD).

Half of the participants were found to have progressive fibrosis. One in five of the total groupdied over a 3-year follow-up period – 4% from pulmonary infection, 7% from respiratory failure due to the progressive fibrosis and 9%from other causes.

The odds of developing PF-ILD were more than four times as high if they had diabetes mellitus than if they didn’t, more than twice as high if they used steroids and three times as high if they had a fibrotic high-resolution computed tomography pattern, according to the research.

Reducing PF-ILD was important, because people with the condition were six times more likely to die within the 14-year study period. Patients were also seven times more likely to die early if they were smokers, and five times more likely if they used steroids, depending on the dose.

Clinical and biochemical data, pulmonary function tests and high-resolution computed tomography results were recorded. Researchers also looked at overall survival and PF-ILD, defined as 10% or greater decline in FVC (forced vital capacity), 15% or more decline in DLCO (diffusing capacity of lung for carbon monoxide) or fibrosis progression within two years on high-resolution computed tomography.

While patients with CTD-ILD can have a significantly worse quality of life and major adverse outcomes, it is understudied and difficult to diagnose and manage.

The findings had important implications for clinical practice, said the authors.

“[It] confirms the central place of routine pulmonary function tests and high-resolution computed tomography in follow-up in order to detect PF-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 PF-ILD. Furthermore, education on smoking cessation and minimising steroid use are also crucial in managing CTD-ILD,” they added.

Presenting author, Dr Yu-Hsiang Chiu, noted that channelling bias for steroid use was possible since steroids are used to control connective tissue diseases, although baseline FVC and DLCO did not differ depending on steroid use.

One surprise was that obesity appeared to be a protective factor against progressive fibrosing interstitial lung disease, reducing the risk by more than 80%, which researchers were not yet able to explain.

0387 Predictors for Progressive Fibrosis in Patients with Connective Tissue Disease Associated Interstitial Lung Diseases