Interstitial lung disease in systemic sclerosis-a retrospective cross-sectional study in Taiwan

Area of Science:

• Rheumatology
• Pulmonology
• Internal Medicine

Background:

• Interstitial lung disease (ILD) is a significant complication of systemic sclerosis (SSc).
• Data on ILD prevalence and characteristics in Asian SSc populations are limited.
• Understanding SSc-ILD is crucial for improving patient outcomes.

Purpose of the Study:

• To determine the prevalence and characteristics of ILD in an Asian cohort of SSc patients.
• To identify risk factors for ILD development and mortality in SSc.
• To evaluate a predictive score for ILD in SSc.

Main Methods:

• Retrospective analysis of 110 SSc patients' electronic medical records (April 2000 - December 2020).
• Inclusion of clinical data, laboratory tests, pulmonary function tests (PFTs), and high-resolution computed tomography (HRCT).
• Calculation of a weighted score (TLC - 0.1*ESR - 1.5*CRP) to predict ILD risk.

Main Results:

• 47.3% of SSc patients presented with ILD, predominantly usual interstitial pneumonitis (UIP).
• SSc patients with ILD showed lower total lung capacity (TLC)/diffusion capacity of carbon monoxide (DLCO), poorer renal function, and higher C-reactive protein (CRP) levels.
• A weighted score demonstrated modest predictive power for ILD (ROC AUC 0.7242).
• Infections, pulmonary hypertension (PH), and renal failure were major causes of mortality in SSc-ILD.

Conclusions:

• SSc-ILD is characterized by reduced lung function, impaired renal function, and elevated CRP compared to SSc without ILD.
• Early dyspnea, PH, and renal failure are independent risk factors for mortality in SSc-ILD.
• The developed weighted score may aid in predicting ILD risk within this cohort.