Interstitial lung disease in Sjögren’s syndrome

Abstract

<bold>Background:</bold> Sjögren’s syndrome (SS) is one of the most common autoimmune pathologies which can be responsible of a pulmonary involvement Our study aimed to describe the clinico-radiological presentation, the management and the course of interstitial lung disease(ILD)in SS <bold>Methods:</bold> Retrospective study including patients followed up for SS with ILD(confirmed by chest CT scan)at the Pulmonology Department of MongiSlim Hospital, inTunis-Tunisia, from January 2017to January2024 The diagnosis of SS was made according to the American-European Consensus Group criteria <bold>Results:</bold> A total of 26 cases were enrolled (Mean age=61 years, gender-ratio=0,36).SS was known before the onset of respiratory symptoms in 11 cases(associated toSystemic lupus erythematosus in1case)and diagnosed after ILD in 15cases(associated to rheumatoid arthritis in 2 cases). Dyspnea (n=23) and chronic cough (n=18) were the most frequently reported complaints. Average values of PFT were: SpO2=93%, FVC=1.69l (69%), TLC=3.14l (68%), DLCO=94%. On chest CTscan, radiological patterns were:NSIP (n=20),UIP (n=2),HSP(n=1),ILA (n=2), constrictive bronchiolitis (n=1). Bronchoalveolar lavage, performed in 10cases, showed lymphocytic alveolitis in 4cases and a predominance of neutrophiles in 4cases Systemic corticosteroid therapy was indicated in 21 cases and was associated to immunosuppressive treatment in 12 cases. The evolution was favorable for 21 patients, with improvement observed in 13 cases and stability in 8 cases There was worsening in 5 cases requiring long-term oxygen therapy <bold>Conclusion:</bold> More than 50%of cases of SS are diagnosed while etiological investigations for ILD.NSIP is the most frequent radiological pattern associated to SS. Worsening occurs in 20% of cases despite optimal treatment