PULMONARY MANIFESTATIONS IN PRIMARY SJOGR ENS-SYNDROME - REVIEW OF 8 CASES IN A STUDY OF 35 PATIENTS

Review of eight cases in a study of 35 patients. Clinical, roentgenolo gic, functional and broncho-alveolar lavage features of lung involveme nt in primary Sjogren's syndrome were assessed in a retrospective stud y of 35 cases. Diffuse interstitial patterns on chest radiography were present in six patients and alveolar patterns were suggestive of lymp hoid interstitial pneumonitis or pseudolymphoma in two. Acute and febr ile onset mimicked infectious pneumonitis in three patients when dyspn ea was the most common clinical feature in others. Patients with prima ry Sjogren's syndrome and pulmonary disease were older (65 vs 56 years ) (P = 0.025), have more frequently extra-glandular manifestations (P = 0.03), keratoconjunctivis sicca (P = 0.018) and biological perturbat ions (hypergammaglobulinemia (P = 0.03), antinuclear antibodies (P = 0 .01)) than those without lung involvement. Low diffusion capacity was present in seven patients associated twice to small airways obstructio n. Bronchoalveolar lavage revealed in all cases an increased total cel ls count (mean: 6.96 10(5)/mm(3)) and a lymphocytic alveolitis (range: 11 to 66%; mean: 38%) associated with an elevated percentage of alveo lar neutrophils in four patients. A low CD4/CD8 ratio was, elated to a pejorative issue. Treatment consisted in corticotherapy combined with oral cyclophosphamide in case of pseudolymphoma.