The following is a summary of “Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry,” published in the March 2024 issue of Pulmonology by Buschulte et al.

Interstitial lung diseases (ILD) encompass a diverse spectrum of predominantly chronic lung conditions with varying disease trajectories. Progression, particularly in progressive fibrosing ILD (PF-ILD), affects up to 50% of patients and is linked to heightened mortality risk.

This study analyzed data from the EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry to delineate disease trajectories across different ILDs. Disease courses were categorized as significant or moderate progression, stable disease, or improvement based on changes in forced vital capacity (FVC) over time. PF-ILD was defined as an absolute decline in FVC % predicted of ≥10% within 24 months or ≥1 respiratory-related hospitalization. Risk factors for progression were determined using Cox proportional-hazard models and logistic regression. Survival time and time to progression were estimated using Kaplan-Meier curves.

Of the 601 patients included in the EXCITING-ILD registry, 28.5% died, primarily due to ILD-related causes. The median survival time from diagnosis was 15.5 years. Progression was observed in 50.6% of patients, with the shortest median time to progression seen in idiopathic nonspecific interstitial pneumonia (iNSIP) and idiopathic pulmonary fibrosis (IPF). Factors such as reduced baseline FVC and older age were significant predictors of progression. Higher GAP indices were associated with shorter survival times. Notably, patients with ILD exacerbations leading to hospitalization had markedly shorter median survival times compared to those without exacerbations.

Disease progression is prevalent across all ILDs and is strongly correlated with increased mortality. Baseline FVC impairment, advanced age, and acute exacerbations are key risk factors for disease progression and mortality. Early progression detection remains challenging, underscoring the need for additional clinical criteria beyond pulmonary function tests to facilitate timely intervention and improve outcomes for ILD patients.

Source: respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02731-3