POS1246 CHARACTERIZATION OF 785 PATIENTS WITH PULMONARY MANIFESTATIONS OF SJÖGREN DISEASE: RESULTS FROM THE BIG DATA SJÖGREN REGISTRY
Authors: A. Alunno, F. Carubbi, A. Flores-Chávez, A. Szántó, A. Sebastian, M. Rischmueller, W. F. Ng, S. Pasoto, B. Kaya, R. Solans-Laqué, A. Rasmussen, G. Hernandez-Molina, F. Reis de Oliveira, Y. Suzuki, M. Bandeira, L. Quartuccio, S. Melchor Díaz, R. Priori, V. Devauchelle-Pensec, E. Bartoloni, N. Inanc, A. Vissink, J. M. Lopez-Dupla, V. Valim, J. Álvarez Troncoso, G. Policarpo-Torres, C. Baldini, K. Perdan-Pirkmajer, M. Vázquez, A. González, B. Hofauer, F. Atzeni, E. Fonseca-Aizpuru, M. Akasbi Montalvo, H. Nakamura, M. Ramos-Casals, P. Brito-Zerón
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Pulmonary manifestations of primary Sjögren disease (SD) have been described in 9-20% of patients, are highly heterogeneous and have also been linked to increased mortality. <h3>Objectives:</h3> To characterize pulmonary manifestations in a large cohort of patients with SD. <h3>Methods:</h3> We collected data on pulmonary manifestations in patients with SD from the international Big Data Sjögren Registry. Inclusion criteria were fulfilling 2002/2016 classification criteria and having had at least once pulmonary manifestations (defined as an ESSDAI score ≥5 in the pulmonary domain).. <h3>Results:</h3> A total of 785 patients (89% females) from 17 countries worldwide were enrolled. Patients were mainly of White ethnicity (82%) and the majority were non-smokers (N=545, 69%) or former smokers (N=117, 15%). In 353 (45%) patients pulmonary manifestations occurred after SD diagnosis, whereas in 209 (27%) these appeared before SD diagnosis. The age of onset of pulmonary manifestations was comparable in these 2 groups. In 223 patients (28%) pulmonary manifestations occurred at the time of SD diagnosis. In 68% of patients radiologically evident interstitial lung disease (ILD) was detected (Table 1). For the other patients, other diagnoses were (but not limited to) airway disease, hypersensitivity pneumonitis and pulmonary amyloidosis. However, ILD was more frequent in patients developing pulmonary manifestations after SD diagnosis compared to those developing them before. In patients developing ILD before SD, non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP) were the most prevalent radiological patterns. Conversely, in patients developing ILD after SD lymphocytic interstitial pneumonia (LIP) was more likely to be observed (all odds ratios (OR) and 95% Cis &gt;1). A subgroup analysis according to smoking habit highlighted that among smokers the LIP pattern was the least frequent whereas NSIP was the most frequent pattern regardless of the time of ILD onset (before or after SD). Histological confirmation of ILD with surgical (57%) or transbronchial (43%) biopsy was performed only in 12% of the patients with radiological evidence. At the last follow up 170 (21%) patients were deceased (median age of death=75 years, range 27-96 years). When comparing these data with the overall mortality of the Big Data cohort [1] we calculated an unadjusted OR of 3.3 (95% CI=2.7-3.9) suggesting that patients SD patients with pulmonary manifestations may have a higher risk of death comparing to the overall SD population. <h3>Conclusion:</h3> Pulmonary manifestations of SD are heterogeneous, occur at different stages in the disease course and may increase the mortality risk. Further analysis to identify relationships with demographic and disease specific variables will be performed as patient enrolment is not yet completed. <h3>REFERENCES:</h3> [1] Brito-Zeron et al EClinicalMedicine. 2023 Jul 4:61:102062. doi: 10.1016/j.eclinm.2023.102062 <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>