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

Abstract Objectives To analyze the prevalence, characteristics and outcome of cystic lung disease associated with Sjogren's syndrome (SS). Methods From 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. Results Twenty-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. Conclusions Cystic lung disease is frequent, benign, associated with anti-SSB/La antibodies and has no impact on outcome in SS.