链球菌抗原对于泛发性脓疱型银屑病不同IL36RN基因突变类型的影响研究

Generalized pustular psoriasis (GPP) is a special type of psoriasis. Our research group has confirmed that IL36RN is closely associated with GPP in Chinese children. However, it is found that homozygous mutation c.115 + 6C> T with no incidence in Chinese Han normal population, coupled with the mutation rate up to 3.6% carried in the normal population, suggesting the inevitably existence of other factors except for IL36RN which directly leads to GPP. We has studied the role of type A β-hemolytic streptococcus infection on GPP and found enhanced proliferation and activation of PBMCs and lymphocytes in GPP patients and IL36RN-negative GPP patients stimulated by streptococcal antigen, which demostrated the initial role of streptococcus. Further research should be continued on the difference between types of IL36RN mutation (wild genotype, heterozygous, homozygous patients and normal people carrying homozygous mutation) after streptococcal antigen stimulation and the impact on patients stimulated by streptococcal antigen carrying IL36RN mutation in acute exacerbation and remission. So we can confirm the pathogenesis of GPP and impact of streptococcal infection on GPP in the Chinese population.

泛发性脓疱型银屑病（GPP）是一种特殊类型的银屑病，课题组已研究证实IL36RN基因与中国儿童GPP发病密切相关。然而，在中国汉族正常人群中还发现未发病的纯合c.115+6C>T突变，加上该突变在正常人群中高达3.6%的携带比率，提示除外IL36RN基因突变作为导致GPP发病的直接原因以外，还存在其他诱发因素。课题组研究A型β溶血型链球菌感染在GPP中的作用，发现链球菌抗原刺激GPP患者PBMCs，其增值能力显著高于正常组、IL36RN基因非突变组较突变组高，链球菌抗原显著促进淋巴细胞增殖活化，证实了链球菌的始动作用。进一步的研究针对不同IL36RN基因突变类型（野生基因型、杂合子、纯合子患者以及携带纯合突变的正常人）对于链球菌抗原刺激后的差异、 IL36RN基因突变患者对于急性发作期与缓解期受链球菌抗原刺激的影响，以阐明链球菌感染在中国人群中GPP发病机制中的作用。