Background:Interstitial lung disease(ILD) is one of the major contributor to morbidity and mortality in primary Sjögren’s syndrome(pSS)1. However, the clinical features and prognosis were various in literatures1-3; and the optimal management were still under investigationObjectives:To characterize the clinical feature, treatment and prognosis predictor of ILD of pSS in a tertiary medical center in Taiwan.Methods:Patients who had pSS and ILD confirmed with HRCT of lung between 1999-2019 were retrospectively identified. Demographic data, autoimmune feature, complementary examinations and treatments were recorded. Change in pulmonary function tests compared with pre-rituximab or pre-cyclophosphamide levels was assessed at 6–12 months post-treatment. Secondary analyses included acute exacerbation(AE), infection, and survival between different treatment groups.Results:112 patients (mean age 68(56-80); 67% female) were analyzed with the median pSS duration 3.1 (0.8-8.0) years. Most patients presented dyspnea and cough. NSIP was the most common radiological pattern (42.9%), followed by UIP (31.3%). Rituximab was used in 11(9.8%) patients, cyclophosphamide(CYC) was used in 28(25%) patients, and combination was used in 20(17.9%) patients. The FVC level and DLCO% were stable after 6-12month post-treatment in these groups. During the median 3.4(1.3-7.7) years ILD follow up, 88 patients (78.6%) had been hospitalized because of AE or infection; with 34(30.4%) of them experiencing respiratory failure. 1-year and 5-year survival was 88.6% and 67.1%. No significant overall survival was distinguished between different treatment groups(p=0.36). Frequent AE (HR=2.17, 95% CI: 1.36-3.5) and respiratory failure (HR=29, 95% CI: 4.61-191) contributed to mortality, while UIP pattern did not (HR=1.6, 95% CI: 0.77-3.3).Figure 1.Kaplan-Meier survival plot of different treatment group: R, E+R, EConclusion:Rituximab or CYC may stabilize pulmonary function but may not benefit on overall survival. Frequent ILD AE and respiratory failure are risk factors of mortality in pSS-ILD.References:[1] McCoy SS, Sandbo N. Sjögren’s Syndrome-Associated Lung Disease.Clinical Pulmonary Medicine.2017;24(6):232-238.[2] Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH. Interstitial Lung Disease in Primary Sjögren Syndrome.Chest.2006;130(5):1489-1495.[3] Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjögren’s syndrome.European Respiratory Review.2016;25:110-123.Acknowledgments:Thanks to Dr. Ting-Yuan Lan, Dr. Ko-Jen Li, Dr. Hsieh Song-Chou, Dr. I-Hsien Lee, Dr. Chen-Yen Lin, Dr. Wang Hao ChienDisclosure of Interests:None declared
Risk factors for progression of interstitial lung disease in Sjögren’s syndrome
