Bronchiectasis in primary Sjögren's syndrome: prevalence and clinical significance.

OBJECTIVES To analyse the prevalence and clinical significance of bronchiectasis in a large series of patients with primary Sjögren's syndrome (SS) and evaluate its impact on disease expression and outcomes. METHODS The study cohort included 507 patients with primary SS. Bronchiectasis were diagnosed according to pulmonary computed tomography (CT). As a control group, we included 37 consecutive SS patients evaluated by pulmonary CT during the same study period without pulmonary involvement. RESULTS Fifty primary SS patients had bronchiectasis according to the pulmonary CT. Nine patients were excluded due to non-autoimmune processes and 41 were classified as bronchiectasis associated with primary SS (40 women, mean age of 64 years). All cases of bronchiectasis were of the cylindrical type and were located in the inferior lobes in 29 cases (71%). Patients with bronchiectasis were older at diagnosis of SS (60.39 vs. 52.54 years, p=0.022) and had a higher frequency of hiatus hernia (41% vs. 16%, p=0.024) in comparison with controls. Immunologically, patients with bronchiectasis had a lower frequency of anti-Ro/SS-A antibodies (27% vs. 54%, p=0.022) but a higher frequency of anti-smooth muscle--SMAantibodies (82% vs. 60%, p=0.043). During follow-up, patients with bronchiectasis had a higher frequency of respiratory infections (56% vs. 3%, p<0.001) and pneumonia (29% vs. 3%, p=0.002) in comparison with those without. CONCLUSIONS Patients with primary SS and bronchiectasis are characterised by an older age, a high frequency of hiatus hernia, a specific immunologic pattern (low frequency of anti-Ro/SS-A and high frequency of anti-SMA) and during follow-up a much higher frequency of respiratory infections and pneumonia.