One hundred fourteen patients with
sarcoidosis, who were diagnosed as having
sarcoidosis histologically, have been typed for HLA class 1 (A, B, and C) and class 2 (DR and DQ)
antigens. Controls consisted of 478 healthy Japanese subjects. The frequencies of
HLA-A1,
HLA-Bw46, HLA-Cx46,
HLA-DRw8,
HLA-DRw9, and
HLA-DRw52 were significantly increased in
sarcoidosis compared to control subjects, but only four patients were positive for
HLA-A1. Increased frequencies of
HLA-Bw46 and HLA-Cx46 were thought to be attributable to linkage disequilibrium with
HLA-DRw8. Patients with
HLA-DRw52 were the most frequent (84 cases of 113). No significant differences were observed between HLA-DRw52-positive and HLA-DRw52-negative patients in their clinical features, but all of the patients with muscular involvement (six cases) were positive for
HLA-DRw52. Among patients positive for
HLA-DRw52, those with
HLA-DR5 showed a significantly better
clinical course and earlier onset of the disease than those with
HLA-DRw8. These results suggest that
HLA antigens may play an important role in the pathogenesis of
sarcoidosis.