[Severe pulmonary fibrosis disclosing primary Gougerot-Sjögren syndrome. Apropos of 5 cases, 2 of which were fatal].

Five cases (four women and one man) with primary Gougerot-Sjögren syndromes presenting as interstitial pulmonary fibrosis are reported. The main point of interest was the severity of the pulmonary changes: effort dyspnoea from the outset, radiological interstitial pneumonitis, restrictive syndrome with a CO diffusing capacity less than 50 p. 100 of theoretical values in 4 cases. Broncho-alveolar lavage was performed in all patients: it showed a high polynuclear neutrophil and particularly high eosinophil counts. Lung biopsy showed severe fibrotic changes. The pulmonary changes dominated the clinical presentation in all patients and the Gougerot-Sjögren syndrome was only diagnosed after systematic examination completed by biopsy of the accessory salivary glands. In the absence of an associated connective tissue disease, these cases of the Gougerot-Sjögren syndrome were considered to be primary. Four patients were given steroid therapy but this did not prevent a fatal outcome in two cases after 6 and 14 months respectively. The clinical states of the other patients were unaffected by treatment. Twenty-one other, but less severe cases, have been previously reported. They confirm that interstitial pulmonary fibrosis observed during the Gougerot-Sjögren syndrome does not differ from idiopathic pulmonary fibrosis and that observed in other connective tissue diseases. Nevertheless, analysis of our cases showed that biopsy of the accessory salivary glands should be part of the routine investigation of apparently idiopathic pulmonary fibrosis. These pulmonary changes may be life-threatening when associated with a primary Gougerot-Sjögren syndrome.