Pulmonary involvement in primary Sjögren's syndrome.

Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common presentation of pulmonary involvement in primary Sjögren's syndrome. There is wide spectrum of less common manifestations, including pulmonary arterial hypertension, pseudolymphoma, pulmonary lymphoma, lymphocytic interstitial pneumonitis, amyloidosis, and pleurisy. Pulmonary function test abnormalities showing a restrictive pattern and cellular abnormalities in bronchoalveolar lavage fluid for prevalent in patients without respiratory complaints and normal chest radiographs. Long-term prospective controlled studies are needed to determine the clinical course and significance of these findings.