シェーグレン症候群の病理と病因

• Published in: 日本口腔外科学会雑誌 Vol. 57; no. 5; pp. 234 - 237
• Main Author: 林, 良夫
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.05.2011
• Subjects: RbAp48; α-フォドリン自己抗原; アポトーシス; シェーグレン症候群; 自己反応性T細胞
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.57.234

Primary Sjören’s syndrome （SS） is an autoimmune disorder characterized by lymphocytic infiltrates and destruction of the salivary and lacrimal glands, leading to clinical symptoms of dryness of the mouth and eyes. Autoreactive T cells bearing CD4 molecule may recognize unknown self antigen, triggering autoimmunity in the salivary and lacrimal glands. Recent evidences suggest that the apoptotic pathway plays a central role in tolerazing T cells to tissue-specific self antigen including 120KD α-fodrin, and may drive the autoimmune phenomenon. Cleavage of certain autoantigens during apoptosis may reveal immunocryptic epitopes that could potentially induce autoimmune response. It is now evident that the interaction of Fas with FasL regulates a large number of pathophysiological process of apoptosis including autoimmune diseases. Our data imply that tissue-specific apoptosis and caspase-mediated α-fodrin proteolysis are involved in the progression of autoimmune lesions in SS. We found that retinoblastoma-associated protein RbAp48 overexpression induces apoptosis in the salivary glands caused by estrogen deficiency. The studies reviewed the pathology and molecular mechanisms on the development of SS.