Sjögren's syndrome (SS), an autoimmune disease characterized by lymphocytic infiltration of exocrine glands, diagnosed by the presence of xerophthalmia, xerostomia, and a connective tissue disease, may present extraglandular manifestations. In this review, we address the pulmonary manifestations, particularly of the primary syndrome (pSS) (without any other connective tissue disease). Pulmonary manifestations can be divided into desiccation of the tracheobronchial tree, interstitial lung disease (ILD), pulmonary vasculitis, and pleuritis. The frequency of such manifestations vary according to different diagnostic criteria. Pulmonary involvement is more frequent in secondary SS, but pSS is commonly associated with ILD. Desiccation of the tracheobronchial tree presents usually as dry cough, but production of sputum may occur. An obstructive functional pattern is reported on pulmonary function testing in up to 40% of pSS patients. Diffuse ILD, with a large spectrum of histologic patterns, pulmonary function tests showing a restrictive defect, and chest roentgenographic features of interstitial involvement, is found in the majority of symptomatic pulmonary patients studied so far. Subclinical alveolitis is common in pSS. The role of corticosteroids and immunosuppressive therapy in the management of such patients remains unclear; however, we recently treated a series of patients with azathioprine with significant lung function improvement. Pulmonary vasculitis and pleuritis are less common in pSS but are more frequent in secondary SS.
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