Primary biliary cirrhosis. Subclinical inflammatory alveolitis in patients with normal chest roentgenograms.

To determine whether a subclinical inflammatory alveolitis is associated with primary biliary cirrhosis (PBC), we compared the numbers and types of cells recovered by bronchoalveolar lavage from 12 patients with PBC, ten healthy control subjects, and nine patients with alcoholic cirrhosis (AC). All were free of clinical pulmonary symptoms and had normal findings on chest roentgenograms. Total BAL cell count did not differ among patients with PBC (mean 9.6 X 10(4) cells/ml), patients with AC (mean 14.8 X 10(4) cells/ml), and control subjects (mean 9.9 X 10(4) cells/ml). Patients with PBC but not patients with AC had an increased proportion of lymphocytes in bronchoalveolar lavage fluid (respectively 22.4 percent +/- 5.2 and 11.6 percent +/- 2.52 compared with the normal value of 9.9 percent +/- 1.5 p less than 0.05). In the same way, alveolar lymphocytosis of the lower respiratory tract from PBC patients predominantly comprised T4+ (helper/inducer) T-lymphocyte subset in patients showing an increased alveolar lymphocytosis. Alveolar macrophages from PBC patients showed a dramatic increased chemiluminescence response before and after stimulation by phorbol-myristate-acetate, regardless of the intensity of alveolar lymphocytosis. Thus, our data demonstrated that subclinical alveolar inflammation comprising T-lymphocytes and activated alveolar macrophages mimicking sarcoid alveolitis is present in a high proportion of patients with PBC.