Mediastinal fibrosis is associated with human leukocyte antigen-A2

Objective: To determine the association between mediastinal fibrosis and hu man leukocyte antigen (HLA) genes. Design: Case-control study. Setting: Vanderbilt University Medical Center. Subjects: Nineteen consecutive patients with mediastinal fibrosis who prese nted to the pulmonary clinic at Vanderbilt University Medical Center from 1 987 to 1996. The control subjects were 21,086 whites who were cadaveric kid ney donors from October 1987 through December 1993. Measurements: HLA testing was performed on blood samples from all 19 cases. Information on HLA typing for the control subjects was obtained from the U nited Network for Organ Sharing. Frequency of HLA class I and II antigens f ound in the cases was compared with the frequency in the control subjects. Results: The relative risk of mediastinal fibrosis among subjects with the HLA-A2 antigen was 3.32 times that of those who lacked this antigen (95% co nfidence interval, 1.19 to 9.2), Conclusion: HLA-A2 was strongly associated with mediastinal fibrosis, sugge sting that an abnormal immune response is important in the pathogenesis of this disease.