To conclude the session, Dr. Carteron drew the audience’s attention to cystic lung disease in Sjögren’s syndrome, a complication of which she was not particularly aware prior to working on the new consensus guidelines herself.
“Cystic lung disease appears to be more common in [Sjögren’s syndrome] than other connective tissue diseases,” she explained. “It’s associated with lymphocytic interstitial pneumonia [LIP] and lymphoid follicular bronchiolitis, and may suggest the presence of amyloid or mucosa-associated lymphoid tissue [MALT] lymphoma, especially if associated with nodules. Furthermore, cysts may become infected or rupture, leading to pneumothorax. There is more we have to learn about this entity, but we should all keep this in mind when caring for patients [with Sjögren’s syndrome].”7–9
Summary
Pulmonary disease is common, variable and frequently underdiagnosed in Sjögren’s syndrome. New consensus guidelines provide much needed guidance to our community regarding screening, monitoring and management of these patients. Close collaboration with our pulmonary colleagues, as well as other specialists, optimizes care.
Samantha C. Shapiro, MD, is an academic rheumatologist and an affiliate faculty member of the Dell Medical School at the University of Texas at Austin. She received her training in internal medicine and rheumatology at Johns Hopkins University, Baltimore. She is also a member of the ACR Insurance Subcommittee.
References
- Lee AS, Scofield RH, Hammitt KM, et al. Consensus guidelines for evaluation and management of pulmonary disease in Sjögren’s. Chest. 2021 Feb;159(2):683–698.
- Franquet T, Díaz C, Domingo P, et al. Air trapping in primary Sjögren syndrome: Correlation of expiratory CT with pulmonary function tests. J Comput Assist Tomogr. Mar–Apr 1999;23(2):169–173.
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- Casal Moura M, Navin PJ, Johnson GB, et al. Pulmonary nodules in patients with primary Sjögren’s syndrome: Causes, clinico-radiologic features and outcomes. Respir Med. Nov–Dec 2020;174:106200.
- MacMahon H, Naidich DP, Goo JM, et al. Guidelines for management of incidental pulmonary nodules detected on CT images: From the Fleischner Society 2017. Radiology. 2017 Jul;284(1):228–243.
- Huang YT, Lu TH, Chou PL, et al. Diagnostic delay in patients with primary Sjögren’s syndrome: A population-based cohort study in Taiwan. Healthcare (Basel). 2021 Mar 23;9(3):363.
- Lechtman S, Debray MP, Crestani B, et al. Cystic lung disease in Sjögren’s syndrome: An observational study. Joint Bone Spine. 2017 May;84(3):317–321.
- Martínez-Balzano CD, Touray S, Kopec S. Cystic lung disease among patients with Sjögren syndrome: Frequency, natural history and associated risk factors. Chest. 2016 Sep;150(3):631–639.
- Baqir M, Kluka EM, Aubry MC, et al. Amyloid-associated cystic lung disease in primary Sjögren’s syndrome. Respir Med. 2013 Apr;107(4):616–621.
