Lung involvement in primary Sjogren's syndrome, was first described by Henr
ik Sjogren more than 50 years ago, who reported similar histopathological c
hanges in the exocrine glands of the bronchi with those observed in the sal
ivary glands. Since then, pulmonary involvement in primary Sjogren's syndro
me, has been the subject of various studies and several pulmonary complicat
ions have been described, including bronchial and bronchiolar disease, inte
rstitial pneumonitis, and lymphoma. Although frequent and pleomorphic, pulm
onary involvement is rarely clinically severe. However, in the symptomatic
patient with significant functional and radiological abnormalities tissue d
iagnosis, either by transbronchial or by open lung biopsy, is mandatory bec
ause of the malignant potential of some lesions.