FRI0358 Factors associated with pulmonary manifestations in sjogren syndrome

• Published in: Annals of the rheumatic diseases Vol. 77; no. Suppl 2; p. 714
• Main Authors: Judez, E., Sanchez-Piedra, C., Fernández Castro, M., Andreu, J.L., Rosas, J., Martínez Taboada, V., Olivé, A.
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group LTD 01.06.2018
• Subjects: Age; Airway management; Amyloidosis; Cough; Diagnosis; Exocrine glands; Lung diseases; Morbidity; Multivariate analysis; Pneumonia; Pulmonary hypertension; Quality of life; Regression analysis; Respiratory tract diseases; Rheumatology; Sjogren's syndrome; Smoking; Vasculitis
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2018-eular.3151

BackgroundPrimary Sjögren’s Syndrome (pSS) is a systemic autoimmune disorder characterised by lymphocytic infiltration of the exocrine glands resulting in dry syndrome. Approximately one-third of patients have extraglandular systemic findings, such as respiratory symptoms (43%–75%), that are also considered to be a cause of morbidity and conditioning quality of life. The aim of the study is to estimate the prevalence of pulmonary manifestations in pSS, and to identify factors associated with its development.MethodsSJOGREN-SER (Spanish Rheumatology Society Registry of pSS) is a multicenter cross-sectional study of pSS patients under active follow-up at 33 rheumatology departments through Spain. Patients fullfilled the European-American consensus criteria of 2002. Airway disease (dry cough, xerotrachea, bronchial, hyperresponsiveness and airway obstruction) and pulmonary involvement (ILD, pulmonary amyloidosis, pulmonary arterial hypertension, vasculitis and pleural involvement) were considered according to the definition contained in EULAR Sjögren’s Disease Activity Index (ESSDAI), as well as Sjögren’s Syndrome Disease Damage Index. Bivariate logistic regression models and multivariate analysis were used to establish the independent effect of patient characteristics associated with pulmonary manifestations. The results were considered significant when the P value was less than 0.05.ResultsThe SJOGREN-SER registry included 437 patients (95% women, median age at inclusion 59 years [50–68 years] and mean of ESSDAI 2 (IQR 0–4)). One hundred and seventeen patients (26.8%) had pulmonary manifestations (19.2% airway disease and 9.8% pulmonary involvement). Ten patients presented both. Sociodemographic characteristics were: mean age 59.5 years (SD: 11.46), 94.9% women and 19.6% smokers or former smokers. Patients with pulmonary manifestations had higher ESSDAI score (6 (SD 6) vs 4 (SD 5)), prolonged disease duration (10.05 years (SD: 7.15) vs 7.7 (SD 6.3)) and were ANA positive more frequently (94.9% vs 62.2%). Airway involvement preceded or occurred at the time of diagnosis in 46.4% of patients. Pulmonary involvement occurred 5 years after the diagnosis of pSS in 37.2% of them. [RS1] Twenty-nine patients (6.6%) were diagnosed with ILD. The most frequent radiological patterns were: Non-Specific Interstitial Pneumonia n=14, Usual Interstitial Pneumonia n=5, Lymphocytic Interstitial Pneumonia n=5 and Cryptogenic Organised Pneumonia n=2. Stepwise multivariate analysis was performed including the following variables: sex, age, laboratory findings, disease duration, smoking and ESSDAI. Disease duration (OR of 1.05 (95% CI, 1.006–1.083)), ESSDAI score (OR of 1.044 (95% CI, 1.006–1.083)) and positivity for ANA (OR of 3.725 (95% CI, 1.141–12.159)) were found to be associated factors with pulmonary involvement in pSS.ConclusionsPrevalence of pulmonary manifestations in this cohort of pSS patients is substantial due to both airway disease and pulmonary involvement. Disease duration, activity of pSS according to ESSDAI score and ANA positivity were factors associated with the development of pulmonary manifestation.Disclosure of InterestNone declared