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Pulmonary manifestations of systemic lupus erythematosus and Sjögren's syndrome

Lopez Velazquez, Marcoa; Highland, Kristin B.b

doi: 10.1097/BOR.0000000000000531

Purpose of review Systemic lupus erythematosus (SLE) and Sjögren syndrome are chronic autoimmune inflammatory disorders that can present with multiorgan involvement including the lungs. This review will focus on recent literature pertaining to the epidemiology, pathogenesis, clinical presentation and diagnosis and management of SLE and Sjögren syndrome-associated pulmonary conditions.

Recent findings Pulmonary manifestations of both disease entities have been well characterized and lung involvement can be observed during the course of the disease in most cases. Pulmonary manifestations of SLE and Sjögren syndrome can be classified based on anatomical site of involvement; and the large and small airways, lung parenchyma, lung vasculature, pleura and respiratory muscles can be involved. The pleura is most commonly involved in SLE, whereas the airways are most commonly involved in primary Sjögren's syndrome (pSS). Sleep disturbances have also been described in both entities.

Summary Although further research into treatment strategies for the pulmonary complications seen in SLE and pSS is needed, the clinician should be aware of the risk factors and clinical presentation of the various pulmonary complications in SLE and pSS in order to identify patients who should be screened and/or have modifications in treatment strategies to mitigate the morbidity and mortality associated with these complications.

aDepartment of Internal Medicine

bDepartment of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, Ohio, USA

Correspondence to Kristin B. Highland, MD, MSCR, Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, 9500 Euclid Avenue, Desk A90, Cleveland, OH 44195, USA. Tel: +1 216 445 5429; fax: +1 216 445 6024; e-mail:

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