Clinical outcomes and risk factors of progressive pulmonary fibrosis in primary Sjögren’s syndrome-associated interstitial lung disease

Abstract Background To investigate the clinical outcomes and risk factors associated with progressive fibrosing interstitial lung disease (PF-ILD) in patients with primary Sjögren’s syndrome-associated interstitial lung disease (pSjS-ILD). Methods During 2015–2021, pSjS patients with ILD were retrospectively identified. Patients were grouped into non-PF-ILD and PF-ILD. Demographics, laboratory data, pulmonary function tests (PFTs), images, survival outcomes were compared between groups. Results 153 patients with SjS-ILD were reviewed, of whom 68 having primary SjS-ILD (pSjS-ILD) were classified into non-PF-ILD (n = 34) and PF-ILD groups (n = 34). PF-ILD group had persistently lower albumin levels and a smaller decline in immunoglobulin G (IgG) levels at the 3rd month of follow-up. The multivariate logistic regression analysis revealed that persistently low albumin levels were associated with PF-ILD. At the 12th month, the PF-ILD group experienced a smaller increase in FVC and a greater decline in the diffusion capacity of carbon monoxide (DLCO) than at baseline. The 3-year overall survival rate was 91.2%, and PF-ILD group had significantly poorer 3-year overall survival rate than non-PF-ILD group (82.4% vs. 100%, p = 0.011). Poor survival was also observed among female patients with PF-ILD. Conclusions Among patients with pSjS-ILD, the PF-ILD group had poorer 3-year survival outcomes. Persistent lower albumin level might be the risk factor of PF-ILD. Early lung function tests could be helpful for the early detection of PF-ILD.