THU0263 LUNG INVOLVEMENT IN PRIMARY SJÖGREN SYNDROME – AN UNDER-DIAGNOSED ENTITY. (2nd June 2020)

• Record Type: Journal Article
• Authors: Sogkas, G.; Hirsch, S.; Olsson, K.; Schmidt, R.; Witte, T.; Jabonka, A.; Ernst, D.
• Is Part Of: Annals of the rheumatic diseases. Volume 79(2020)Supplement 1
• Page Start: 358
• Page End: 359
• Publication Date: 2020-06-02
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2020-eular.1756
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Interstitial lung disease (ILD) represents a frequent extra-glandular manifestation of primary Sjögren's Syndrome (pSS). Limited published data regarding phenotyping and treatment exists. Advances in managing specific ILD phenotypes have not been comprehensively explored in patients with coexisting pSS. Objectives: This retrospective study aimed to phenotype lung diseases occurring in a well-described pSS cohort and describe treatment course and outcomes. Methods: Between April 2018 and September 2019, all pSS patients attending our Outpatient clinic were screened for possible lung involvement. Clinical, laboratory and computer tomography (CT) findings were analysed. Patients were classified according to CT findings into 5 groups: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), combined pulmonary fibrosis and emphysema (CPFE) and unspecific-ILD. Results: Lung involvement was confirmed in 24/240 patients (10%). Clinically manifest pSS occurred later in patients with ILD vs. non-ILD (53.2 [42.0-61.7] vs. 62.3 [55.6-68.8] years; p=0.0016). The commonest phenotype was UIP n=10 (41%), followed by NSIP n=7 (29%), DIP n=2 (8%), CPFE n=2 (8%) and unspecific-ILD n=3 (13%). Forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO ) appeared lower in UIP and DIP, without reaching a significant difference. Treatment focused universally on intensified immunosuppression, with 12/24 patients (50%) receiving cyclophosphamide. No anti-fibrotic treatments were used. Follow-up was median 13.2 [7.9-72.3] months, during which 6/24 (25%) patients exhibited a further decline in FVC. No deaths occurred. Conclusion: Lung involvement in pSS is heterogeneous. Better phenotyping and tailored treatment may improve outcomes and requires further evaluation in larger prospective studies. Disclosure of Interests: None declared