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Semin Respir Crit Care Med 2014; 35(02): 255-264
DOI: 10.1055/s-0034-1371529
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Involvement in Sjögren Syndrome

Maryl Kreider
1   Division of Pulmonary, Allergy and Critical Care Department of Medicine Perelman School of Medicine of the University of Pennsylvania Philadelphia, PA
,
Kristin Highland
2   Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic Foundation, Cleveland, OH
› Author Affiliations
Further Information

Publication History

Publication Date:
25 March 2014 (online)

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Abstract

Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and a variety of extraglandular sites. Lung involvement as defined by symptoms and either pulmonary function testing or radiographic abnormalities occurs in approximately 10 to 20% of patients. Subclinical lung disease is even more frequent and often includes evidence of small airways disease and airway inflammation. In general, patients will have evidence of both airway and interstitial lung disease by radiographs and pathology. Bronchiolitis and bronchiectasis are the most common airway manifestations while the interstitial pathologies associated with SS include nonspecific interstitial pneumonitis, usual interstitial pneumonitis, and lymphocytic interstitial pneumonitis. Patients with SS are also at an increased risk of lymphoma. A protean of other lung abnormalities including amyloidosis, granulomatous lung disease, pseudolymphoma, pulmonary hypertension, and pleural disease have been described.

Keywords

Sjögren syndrome - airways disease - interstitial lung disease - lymphoma - prevalence - treatment
 

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