Pulmonary Involvement in Sjögren Syndrome

Jake G Natalini; Chadwick Johr; Maryl Kreider

doi:10.1016/j.ccm.2019.05.002

Pulmonary Involvement in Sjögren Syndrome

Clin Chest Med. 2019 Sep;40(3):531-544. doi: 10.1016/j.ccm.2019.05.002. Epub 2019 Jul 6.

Authors

Jake G Natalini¹, Chadwick Johr², Maryl Kreider³

Affiliations

¹ Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, 836 W. Gates Building, Philadelphia, PA 19104, USA.
² Division of Rheumatology, Perelman School of Medicine, University of Pennsylvania, 3737 Market Street, 8th floor, Philadelphia, PA 19104, USA.
³ Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, 836 W. Gates Building, Philadelphia, PA 19104, USA. Electronic address: maryl.kreider@uphs.upenn.edu.

PMID: 31376889
DOI: 10.1016/j.ccm.2019.05.002

Abstract

Sjögren syndrome (SS) is a progressive autoimmune disease characterized by dryness, predominantly of the eyes and mouth, caused by chronic lymphocytic infiltration of the lacrimal and salivary glands. Extraglandular inflammation can lead to systemic manifestations, many of which involve the lungs. Studies in which lung involvement is defined as requiring the presence of respiratory symptoms and either radiograph or pulmonary function test abnormalities quote prevalence estimates of 9% to 22%. The most common lung diseases that occur in relation to SS are airways disease and interstitial lung disease. Evidence-based guidelines to inform treatment recommendations for lung involvement are largely lacking.

Keywords: Airways; Autoimmune; Lung; Sjögren syndrome.

Publication types

Research Support, N.I.H., Extramural
Review

MeSH terms

Humans
Lung / pathology*
Lung Diseases / etiology*
Lung Diseases / pathology
Sjogren's Syndrome / complications*
Sjogren's Syndrome / pathology

Grants and funding

T32 HL007891/HL/NHLBI NIH HHS/United States