Abstract

Fibrosing alveolitis in systemic sclerosis is histologically identical to lone cryptogenic fibrosing alveolitis (CFA) (idiopathic pulmonary fibrosis). The inflammatory cell content of bronchoalveolar lavage (BAL) samples has been used as a guide to prognosis and treatment in CFA. In this study, the relationship was explored between BAL findings and the extent and pattern of disease within the lavaged lobe, as judged by thin-section computed tomography (CT), in systemic sclerosis. Thirty-eight nonsmoking patients were studied; none had been treated with corticosteroids or immunosuppressive agents, and 11 had no evidence of fibrosing alveolitis on CT. BAL neutrophils were markedly increased in association with extensive disease on CT compared with less extensive disease (p < 0.001) and normal appearances (p < 0.001); the extent of a reticular pattern on CT (denoting fibrosis) correlated with the neutrophil percentage count (p < 0.005) and total neutrophil count/ml (p < 0.01). BAL eosinophils were increased in less extensive as well as in extensive disease when compared with lobes with a normal CT appearance (p < 0.01); eosinophil percentage counts but not total eosinophil counts/ml correlated with the extent of a ground-glass pattern on CT (through to denote inflammation) (p < 0.05). These findings indicate that in systemic sclerosis a BAL neutrophilia is generally associated with extensive fibrotic disease, whereas a BAL eosinophilia is often seen in less advanced disease, particularly when CT appearances suggest lung inflammation.