Swyer-James syndrome: bronchoalveolar lavage findings in two patients

Swyer-James syndrome (SJS) is a rare constrictive bronchiolitis that appears to be the result of acute bronchiolitis in infancy or early childhood. In the present study the cytological and immunophenotypical profile of bronchoalveolar lavage (BAL) was studied in two patients with SJS who showed a different spectrum of clinical outcome. The total BAL yield was markedly increased in the patient with chronic cough and acute episodes of dyspnoea but not in the patient with decreased exercise tolerance and longer duration of disease. In the two patients, the differential cell counts in percentage were characterized by a significant increase of neutrophils and a slight increase of lymphocytes. The analysis of lymphocyte subsets showed a significant increase of CD8+ cells (T-suppressor-cytotoxic) in both cases, resulting in a decreased CD4/CD8 ratio. In addition, an increase of cells bearing a B-phenotype (CD19+ cells) was noted in the fluid recovered from the patient with chronic cough and acute episodes of dyspnoea. In conclusion, our data suggest that SJS is an active process with inflammatory characteristics. Further studies are needed to explain the mechanism leading to the expansion in situ of immunocompetent cells.