Update on Morbidity and Mortality in Systemic Sclerosis-Related Interstitial Lung Disease

Abstract

Contemporary studies of systemic sclerosis (SSc) consistently demonstrate that interstitial lung (ILD) is a leading cause of disease-related death. This review summarizes morbidity and mortality outcomes in SSc-ILD patients from high-quality observational and interventional studies over the last 50 years. The data presented suggest a trend for improved morbidity and mortality outcomes among present day SSc-ILD patients. Specifically, SSc-ILD patients appear to be living longer from the time of the initial diagnosis. Despite improved survival, the number one cause of death for most SSc-ILD patients remains respiratory failure from ILD. This review describes the most important demographic, clinical, and biological factors, which affect mortality in SSc-ILD, and could be used to help stratify patients for closer monitoring and more aggressive initial treatment. The review concludes with an overview of future research needed to (1) understand how to personalize the care of SSc-ILD patients to improve morbidity and mortality outcomes; and (2) investigate whether novel therapeutic interventions (e.g., anti-fibrotics, hematopoetic stem-cell transplantation) offer any meaningful long-term survival advantage over the current standard of care.

Keywords: immunosuppression; interstitial lung disease; mortality; scleroderma; systemic sclerosis.

Figure 1.

High resolution computed tomography (HRCT) scan of a patient with SSc-ILD. This image demonstrates the radiographic features characteristic of a non-specific interstitial pneumonia pattern commonly observed in patients with SSc-ILD. Source: With permission from AF.

Figure 2.

Predictors of mortality in SSc-ILD. This figure describes the key factors associated with mortality in SSc-ILD. Source: This figure was adapted and modified from the systematic review by Winstone et al. to include only those predictor variables, which have been found to predict mortality in two independent SSc-ILD cohorts, which used HRCT to define the presence of ILD.