Sjogren's syndrome is a common auto-immune disease.
Clinically significant pulmonary involvement affects approximately 10% of patients and may be the first manifestation of the disease, putting the respiratory physician in a position to suspect and confirm the diagnosis. Besides interstitial lung disease and bronchial disorders, cough is a common symptom of the disease and particularly difficult to treat. Lung cysts and amyloid deposits, sometimes associated with lymphoma, have recently been described. The development of a primary pulmonary lymphoma, usually from MALT, is a major complication of the disease.
Characterisation of the pathophysiology of pulmonary involvement in Sjogren's syndrome and the institution of specific treatment merits the interest of the respiratory physician.
The respiratory physician should consider the diagnosis of Sjogren's syndrome in many different clinico-pathological situations.