Cystic lung disease in Sjögren's syndrome: An observational study

ObjectivesTo analyze the prevalence, characteristics and outcome of cystic lung disease associated with Sjögren's syndrome (SS).MethodsFrom June 2010 to February 2015, 90 consecutive SS patients [60.1 ± 14.8 years; 88 (97.8%) female, 75 (83.3%) primary SS] had a systematic chest CT-scan. The presence of thin-walled cysts was analyzed by one experienced radiologist. Demographic data, clinical history, laboratory findings, and pulmonary function tests were extracted retrospectively from medical records.ResultsTwenty-one (23.3%) patients had cysts on CT scan performed 40.5 ± 54.5 months after SS diagnosis. Cysts number ranged from 1 to 25 were often bilateral (52.4%) and mostly located in the middle lung zone (76.2%). Cysts were isolated (n = 6, 28.6%) or associated with other lesions, including bronchiectasis (n = 5, 23.8%), micronodules (n = 5, 23.8%), ground-glass opacity (n = 4, 19%) and/or air trapping (n = 3, 14.3%). Most patients with cysts (57.1%) had no respiratory symptoms. When comparing SS patients with and without cysts, patients with cysts tended to be older (65.3 ± 15.3 versus 58.5 ± 14.4 years, P = 0.06). Smoking habits were similar in both groups. Anti-SSB antibodies were more frequently detected in patients with cysts (57.1% vs. 26.1%, P = 0.02). Pulmonary function tests were normal or displayed only mild small airways obstruction and reduced diffusion capacity to carbon monoxide. Four (19%) patients with cysts had a past history of associated pulmonary disease, including interstitial lung disease. During follow-up (25.1 ± 17.7 months), no patient developed specific lung disease or lymphoproliferative disorders.ConclusionsCystic lung disease is frequent, benign, associated with anti-SSB/La antibodies and has no impact on outcome in SS.