Systemic Sclerosis-Associated ILD: Insights and Limitations of ScleroID

Background/Objective: Pulmonary involvement in systemic sclerosis (SSc) is typically assessed using pulmonary function tests (PFTs), high-resolution CT (HRCT), and composite indices. Patient-reported outcomes (PRO), including ScleroID, provide insight into quality of life, but their relationship with clinical measures and role in overall disease assessment remain unclear. To assess the correlation between ScleroID scores and both lung involvement and disease activity/damage in a cohort of SSc-ILD patients from a large tertiary care center. Methods: Disease activity [European Scleroderma Study Group Activity Index (EScSG-AI), Scleroderma Clinical Trials Consortium Activity Index (SCTC-AI)], disease severity [Medsger severity scale (MSS)], and PRO measure ScleroID were assessed for associations with the extent and severity of SSc-ILD. Results: In 82 patients with SSc-ILD (mean age 56.0 ± 10.8 years; median disease duration 4.2 ± 4.7 years), higher fibrosis extent (>20%) was associated with worse lung function, greater exercise limitation, and higher ScleroID scores, particularly in fatigue, social life, and body mobility domains (all p ≤ 0.03). Patients with >20% fibrosis also had worse NYHA class and Borg scores during 6-MWD (p < 0.001). Cross-sectional correlations showed that ScleroID total and individual domains were negatively associated with FVC% and 6-MWD, and positively with ILD extent on HRCT. Fatigue, social impact, and mobility domains correlated most strongly with disease activity and severity scores, especially in patients with > 20% fibrosis (r = 0.384-0.635, all p ≤ 0.016), whereas breathlessness showed minimal associations (r < 0.2). Conclusions: In SSc-ILD, greater lung fibrosis and functional impairment are associated with worse patient-reported quality of life, particularly in fatigue, mobility, and social domains. ScleroID scores reflect both physiological severity and disease burden highlighting its value as a multidimensional outcome measure in patients with more advanced disease.