Sjogren 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.
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