Clinical Course and Outcomes in Patients with Sjögren's Associated Interstitial Lung Disease

Lung involvement in Sjögren's disease (SjD) is common and significantly impacts patients' quality of life, with the most frequent manifestation being interstitial lung disease (ILD). This study explored the clinical course and prognostic factors in patients with SjD-associated ILD. We conducted a retrospective analysis of patients diagnosed with SjD-ILD across two tertiary care academic referral centers. We assessed key clinical, radiological, and histopathological features of patients with SjD-ILD to investigate the long-term outcomes and determine the factors that can help better prognosticate patients in the clinic. A total of 81 patients with SjD-ILD were included in our analysis. ILD was the presenting manifestation in 21% (n = 17) of the SjD patients. The median survival following diagnosis of SjD-ILD was 11 years. Among ILD subtypes, the UIP pattern was associated with more rapid lung function decline and higher mortality. In contrast, higher baseline forced vital capacity (FVC) and anti-SSA antibody positivity were linked to reduced mortality risk. ILD is a common manifestation that can lead to the diagnosis of SjD. The presence of ILD has an adverse effect on the overall survival of patients with SjD. Baseline lung function and serologies can further assist in prognostication. A critical review of imaging patterns to determine the underlying ILD pattern can aid individualized counseling and therapeutic decision-making in patients with SjD-ILD.