ANALYSIS OF THE PATHOGENESIS OF THE SERONEGATIVE AUTOIMUNE DISEASE

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As most patients with myasthenia gravis(MG)of childhood onset have low or negative ant-acetylcholine receptor(AChR) autoantibody titier, the role of anti-AChR autoantibodies as the cause of muscle weakness remains as open question.As for the prevalence of child-hood MG in Japan, the largest onset-age group was found to be under three years.To elucidate whether the Japanese patients with childhood-onset MG is a variant type, the correlation between clinical features and HLA DR/DQ allele frequencies was examined in 87 Japanese patients with childhood-onset disease.HLA genotypes DRB1^*1302/DQA1^*0102/DQB1^*0604 and DRB1^*1302/DQA1^*0102/DQB1^*0604 were signiticant higher in patients than in healthy controls(Pc<0.0001, RR=8.5 ; Pc<0.0001, RR=5.5, for two genotypes, respectively).Patients who had a significant higher likelihood of the HLA types DRB1^*1302/DQA1^*0102/DQB1^*0604 or DRB1^*1302/DQA1^*0102/DQB1^*0604 belonged to the latent general type(LG) of MG ; this is clinically ocular type, but shows myasthenic electromyographic findings in extremity muscles.The LG type of MG was observed in 78% of patients exhibiting the clinically ocular type ; this group comprised approximately 75% of patients with childhood-onset MG.These data suggested that the LG type with childhood-onset associated with HLA types DRB1^*1302/DQA1^*0102/DQB1^*0604 is a specific clinical type of childhood-onset MG which may carry a specific genes predisposing toward the disease.