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The Clinical Study of IgG4 Associate Pancreatitis (Autoimmune Pancreatitis) and Development of its Diagnostic System

IgG4相关性胰腺炎（自身免疫性胰腺炎）的临床研究及诊断系统的开发

基本信息

批准号：
13557047
负责人：
KAWA Shigeyuki
金额：
$ 9.09万
依托单位：
Shinshu University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (B)
财政年份：
2001
资助国家：
日本
起止时间：
2001 至 2003
项目状态：
已结题

项目摘要

The present study revealed that autoimmune pancreatitis is characterized by elderly male preponderance and obstructive jaundics as an initial symptome. Sometimed it is difficult to differentiate this disease from pancreatic cancer clinically, but serum IgG4 measurement is useful tool for differentiation. Significant changes of pancreatigram in follow-up study showed the diversity of clinical features of this disease. We found low frequency of authentic autoantibodies, such as SS-A, SS-B and anti-mitochondrial antibody in this disease. We found anti-alfa-fodrin antibody in sera of autoimmune pancreatitis patients with sclerosing cholangitis, suggesting that this antibody is associated the formation of such an extra-pancreatic lesion.Auotimmune pancreatitis is characterized by co-occurrence of various exta-pancreatic lesions._The present study showed hilar lesions by gallium-67 scintigrphy and retroperitoneal fibrosis, suggesting that this disease is regarded as systemic disease, such as multifocal fibrosclerosis. The autoimmune pancreatitis is significantly associated with HLA DRB1*0405-DQB1*0401 haplotype, which may present specific antigen peptide to T cells, resulting in the initiation of autoi, mmune response.The present study revealed that sera of the patient with this disease react specifically to 25 kDa band, which was later confirmed as superoxide dismutase 2 (SOD2). In many autoimmune diseases, autoantibodies react to SOD2, suggesting the candidate of target antigen. However, recombinant SOD2 protein could not react with patient sera. We should perform further study to confirm whether glucoconjugate portion of this protein is antigen determinant.

本研究表明，自身免疫性胰腺炎以老年男性为主，以梗阻性黄疸为首发症状。临床上有时很难将本病与胰腺癌鉴别，但血清IgG4测定可作为鉴别诊断的有用工具。随访胰腺图有明显变化，显示本病临床特征的多样性。发现SS-A、SS-B和抗线粒体抗体在本病中出现频率较低。我们在自身免疫性胰腺炎合并硬化性胆管炎患者的血清中发现了抗α-fodrin抗体，提示这种抗体与这种胰腺外病变的形成有关。自身免疫性胰腺炎的特点是胰腺外多种病变并存。本研究通过~(67)镓扫描显示肝门部病变和腹膜后纤维化，提示该病为全身性疾病，如多灶性纤维硬化。自身免疫性胰腺炎与人类白细胞抗原DRB1*0405-DQB1*0401单倍型显著相关，单倍型DRB1*0405-DQB1*0401可能向T细胞递呈特异性抗原肽，引起自身免疫应答。在许多自身免疫性疾病中，自身抗体对SOD2起反应，提示是靶抗原的候选者。但重组SOD2蛋白不能与患者血清发生反应。我们应该进行进一步的研究，以确认该蛋白的糖结合部分是否是抗原决定簇。