Sjögren’s syndrome is a common auto-immune disease. The respiratory system is frequently involved with 10% of the patients developing a clinically significant lung disease. Respiratory disease may be the first manifestation of the syndrome. Beside interstitial lung diseases and bronchiolar disorders, cough is a very common symptom affecting about 50% of the patients in some studies, with limited therapeutic opportunities. Lung cysts, amyloid deposits, sometimes associated with lymphoma, have been recently described. Primary pulmonary lymphoma, usually from the MALT, is a very severe complication of the Sjögren’s syndrome.