SUBCLINICAL PULMONARY INVOLVEMENT IN COLLAGEN-VASCULAR DISEASES ASSESSED BY BRONCHOALVEOLAR LAVAGE - RELATIONSHIP BETWEEN ALVEOLITIS AND SUBSEQUENT CHANGES IN LUNG-FUNCTION

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Abstract
Collagen-vascular disorders (CVD) are commonly associated with chronic interstitial lung disease. Clinicopathologic observations suggest that inflammatory process of the lower respiratory tract may appear prior to fibrosis. Subclinical pulmonary involvement, as assessed by bronchoalveolar lavage (BAL) was evaluated in 61 patients with various CVD but free of clinical pulmonary symptoms and with normal chest roentgenograms. Eight of 61 had abnormal pulmonary function tests(PFT) at entry to the study. Total BAL cell yield from nonsmokers was greater in patients with abnormal than in those with normal PFT (p < 0.05). Abnormal differential count of BAL cells was noted in 29 of 61 patients (48%). Lymphocyte alveolitis (lymphocytes .gtoreq. 18%) was a characteristic finding in patients with primary Sjogren''s syndrome (11 of 25) or Sjogren''s syndrome associated with another CVD (4 to 8). Neutrophil alveolitis (neutrophils > 4%) with or without increased percentage of lymphocytes occurred in patients with CVD classically associated with pulmonary fibrosis: progressive systemic sclerosis (6 to 10), rheumatoid arthritis (1 to 4), dermatopolymyositis (2 of 3), and mixed connective tissue disease (3 of 8). An increased percentage of eosinophils was detected in 1 patient with progressive systemic sclerosis. Bronchoalveolar lavage abnormalities were more frequently detected in patients with active and severe extrapulmonary disease. On follow-up PFT 12 months later, 11 patients with normal BAL and 10 patients with lymphocyte alveolitis had not deteriorated. In marked contrast, the presence of neutrophils in BAL was associated with a progressive deterioration of PFT in 6 of 7 untreated patients, whereas 4 corticosteroid-treated patients with neutrophil alveolitis had not deteriorated. Sequential evaluation of BAL in 12 patients demonstrated that alveolitis may be cyclic; corticosteroids had no consistent effect on the proportions of neutrophils in BAL. In conclusion, BAL permits early detection of inflammatory alveolitis in CVD. The presence of neutrophils in BAL fluid is associated with a high risk of deterioration in lung function.