Prevalence and Prognosis of Interstitial Lung Disease in a Large Cohort of Chinese Primary Sjögren's Syndrome Patients

Hui Gao^1,2, Xuewu Zhang¹, Jing He³, Jing Zhang⁴, Ye Sun⁵ and Zhan-Guo Li¹, ¹Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China, ²Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China, ³Rheumatology, Peking University People's Hospital, Beijing, China, ⁴Peking University International Hospital, Beijing, China, ⁵Department of Radiology, Peking University People's Hospital, Beijing, China

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: interstitial lung disease and prognostic factors, Sjogren's syndrome

Session Information

Date: Monday, October 22, 2018

Title: Sjögren's Syndrome – Basic and Clinical Science Poster

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: To determine the prevalence and identify the prognosis associated with pulmonary involvement in pSS patients.

Methods: A total of 1341 hospitalized patients (853 with pSS and 488 with secondary Sjögren’s syndrome [sSS]) were recruited.

Results:

Pulmonary involvement rates were 19.34% (165/853) and 25.82% (126/488) for pSS and sSS patients, respectively. Of the 165 pSS-ILD patients, 69 patients underwent HRCT in this hospital. Non-specific interstitial pneumonia (NSIP) was the predominant HRCT pattern (n = 27, 39.1%). Chest HRCT findings revealed a lymphocytic interstitial pneumonia (LIP) pattern in 12 patients (17.4%), a NSIP+LIP pattern in 4 (5.8%), and a usual interstitial pneumonia (UIP) pattern in 11 (15.9%). The rest of the findings were as follows: 1 (1.4%) cryptogenic organizing pneumonia (COP), 1 (1.4%) respiratory bronchiolitis-interstitial lung disease (RBILD), and 13 indeterminates. The total HRCT score was 9.71 ± 4.77. Impairment in diffusion capacity was the most common manifestation of pulmonary involvement (74.3%) and the most severe complication. The 5-year survival rate for pSS-ILD patients was 88.5%. Thirty-five (21.2%) of 165 patients died during the follow-up period. Causes of death were as follows: respiratory failure (n = 27), progression of malignant disease (n = 5), gastrointestinal bleeding (n = 1), viral meningoencephalitis (n = 1), and cerebral hemorrhage (n = 1). Multivariate analysis showed that age, disease duration, smoke, HRCT scores, and TLC, TLco/VA, FEV1 were independent risk factors for mortality for pSS-ILD patients.

Conclusion: Lung involvement is a common complication of pSS and the outcome is not favorable.

Disclosure: H. Gao, None; X. Zhang, None; J. He, None; J. Zhang, None; Y. Sun, None; Z. G. Li, None.

To cite this abstract in AMA style:

Gao H, Zhang X, He J, Zhang J, Sun Y, Li ZG. Prevalence and Prognosis of Interstitial Lung Disease in a Large Cohort of Chinese Primary Sjögren’s Syndrome Patients [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/prevalence-and-prognosis-of-interstitial-lung-disease-in-a-large-cohort-of-chinese-primary-sjogrens-syndrome-patients/. Accessed .

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