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HLA-B51 involved in the hyper function of peripheral blood neutrophils from patients with Beh~et's disease

H. Kaneoka, H. Furukawa, M. Takeno, Y. Mizushima, T. Sakane

It has been elucidated that the abnormal hyperfunction of neutrophils plays a pivotal role for the pathogenesis and clinical course of Behget's disease, and it has also been widely accepted that HLA-B51 is one of the most important genetic factors of the disease in several ethnic groups. To clarify the relationship between the hyperfunction of neutro- phils and HLA-B51 in Behget's disease, we determined the productivity of superoxide by the purified peripheral blood neutrophils and HLA phenotypes of the patients. We dem- onstrate here that neutrophils from the patients produced sig- nificantly higher amounts of superoxide than those from

healthy controls, and those neutrophils secreted higher level of TNF-c~ and IL-8, as compared to controls. Moreover, the higher productions of superoxide, TNF-o~ and IL-8 by the neutrophils are closely related to the possession of HLA-B51 in the patients. These results suggest that the abnormal hyperfunction of neutrophils in the patients with Behget's disease, which may be controlled through cytokine produc- tions in the autocrine manner, is determined in part by HLA- B51 molecule.

St. Marianna University School of Medicine, Kawasaki, Kanagawa 216, Japan

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Molecular genetic studies on Behget's disease S. Ohno, N. Mizuki, S. Nakamura, H. Ando, H. Inoko

We have been studying the immunogenetic background of Beh~et's disease, and found that HLA-B51 is closely asso- ciated with this disease. Our recent molecular genetic studies revealed that the frequency of 10.5 kb Nco! TNF-[3 gene fragment was significantly increased in the patients as com- pared with the normal controls. HLA class II genes were not involved in the development of this disease. Further studies

showed that HLA-B* 5101 was significantly associated with this disease, but not HLA-B* 5102 or HLA-B* 5103. These results indicate that the susceptibility gene to Beh~et's dis- ease is present in the HLA class I region.

Department of Ophthalmology, Yokohama City University School of Me- dicine, Yokohama, Japan

1993- Vol 14, Suppl 1