Sjogren's Syndrome and Pulmonary Disease

Adv Exp Med Biol. 2021:1303:193-207. doi: 10.1007/978-3-030-63046-1_12.


Sjogren's syndrome is an autoimmune connective tissue disease targeting the exocrine glands and frequently affecting the respiratory system. The pulmonary disease is the most important extra-glandular manifestation as it carries most of the morbidity and mortality. Typically, it affects the small airways ranging from mild to severe respiratory symptoms. The upper airways are also commonly involved, predisposing sinusitis to occur more frequently than in the normal population. Lymphocytic interstitial pneumonia was initially thought to be the prevailing parenchymal disease; however, multiple cohorts report non-interstitial pneumonia to be the most frequent subtype of interstitial lung disease. In the review of high-resolution computed tomography scans, cystic lesions are commonly found and associate with both the small airways and parenchymal disease. Under their presence, amyloidosis or lymphomas should be considered in the differential. Overall, Sjogren's syndrome has a higher risk for lymphoma, and in lungs this condition should be thought of, especially when the images reveal pulmonary nodularity, lymphocytic interstitial pneumonia and lymphadenopathy. Although, pulmonary artery hypertension was traditionally and exceptionally linked with Sjogren's syndrome, together with systemic lupus erythematosus, they are now acknowledged to be the most common pulmonary vascular disease in east Asian populations, even over patients with systemic sclerosis. Although there are no controlled prospective trials to treat pulmonary disease in Sjogren's syndrome, the mainstay treatment modality still falls on glucocorticoid therapy (systemic and inhaled), combined with immune modulators or alone. Most of the evidence sustains successful outcomes based on reported cases or case series.

Keywords: Airway disease; Interstitial lung disease; Sjogren’s syndrome.

MeSH terms

  • Humans
  • Lung
  • Lung Diseases, Interstitial* / diagnostic imaging
  • Lung Diseases, Interstitial* / epidemiology
  • Lung Diseases, Interstitial* / etiology
  • Lupus Erythematosus, Systemic*
  • Prospective Studies
  • Sjogren's Syndrome* / diagnosis
  • Sjogren's Syndrome* / drug therapy
  • Sjogren's Syndrome* / epidemiology