Detection, screening, and classification of interstitial lung disease in patients with systemic sclerosis

Interstitial lung disease (ILD) associates with disease burden and reduced life expectancy in systemic sclerosis (SSc). As ILD afflicts around 50% of SSc patients and is often present from early on, there is rationale for early and universal ILD screening. With the approval of the first SSc-ILD therapy last year, there is an increasing need for ILD classifications to assign the right treatment to the right patient. Here, we discuss recent advances on screening, detection, and classification of SSc-ILD.

Although prospective datasets from a nationwide population-based SSc cohort confirm insufficient sensitivity of pulmonary function tests (PFTs) for ILD screening, they provide strong support for lung high resolution computed tomography (HRCT) as the primary tool to detect ILD. Lung ultrasound shows promise as an additional screening tool. Interpreting statements from a new European consensus on SSc-ILD management, we propose an urgent need for integrated SSc classification, grading ILD severity at time of diagnosis and evaluate risk for ILD progression. We discuss advances on potential parameters for such classification, including PFTs, quantitative HRCT analyses, patient-reported outcome measures, functional exercise capacity tests, and soluble biomarkers.

Early screening to diagnose ILD is feasible. With new therapies at hand, there is a need for integrated ILD classification including severity grading and risk for progression.