@article{oai:kanazawa-u.repo.nii.ac.jp:00013572, author = {濱口, 儒人}, issue = {3}, journal = {日本臨床免疫学会会誌 = Japanese Journal of Clinical Immunology}, month = {Jan}, note = {全身性強皮症(Systemic sclerosis : SSc)は皮膚をはじめ内臓諸臓器に線維化を生じる.その病因は不明であるが,抗核抗体が高率に検出されることから自己免疫疾患に位置づけられる.SScの病態における抗核抗体の役割は未だ明らかではないが,個別のSSc特異的あるいはSSc関連自己抗体は特徴的な臨床像と密接に結びついているため,SScの診療において自己抗体を同定することは,病状の把握と臨床経過,予後の推測に重要である.SScでみられる自己抗体として代表的なものは抗セントロメア抗体,抗トポイソメラーゼI抗体,抗RNAポリメラーゼ抗体であるが,抗Th/To抗体,抗U3RNP抗体,抗human upstream-binding factor (hUBF)抗体,抗セントリオール抗体,抗U1RNP抗体,抗Ku抗体,抗PM-Scl抗体なども頻度は低いながら特徴的な臨床像を有しているため重要な抗体である.SScでみられる自己抗体の頻度には人種差があり,これは自己抗体が特定のHLAと相関していることが一因と考えられている.SScにおける自己抗体の同定は手技の煩雑な免疫沈降法を要するものが多く,ELISA法が利用できる自己抗体は限られている.簡便に測定できる測定法の開発が望まれる. Systemic sclerosis (SSc) is a connective tissue disorder characterized by microvascular damage and excessive fibrosis of the skin and internal organs with autoimmune background. One representative feature of the immunological abnormalities in SSc patients is the presence of antinuclear antibodies (ANA). More than 90% of patients with SSc are positive for ANA. Although a role of ANA in the pathogenesis in SSc remains unclear, the particular ANA are often indicative of clinical feature, disease course, and overall severity. Therefore, subgrouping patients based on the type of autoantibodies present can be useful in diagnosis and management. Anticentromere antibody (ACA), anti-DNA topoisomerase I antibody (Ab), and anti-RNA polymerase Ab are the representative autoAbs found in patients with SSc. Other serum ANA found in SSc include anti-Th/To Ab, anti-U3RNP Ab, anit-human upstream-binding protein (hUBF) Ab, anti-centriole Ab, anti-U1RNP Ab, anti-Ku Ab, and anti-PM-Scl Ab. It is documented that the prevalence of SSc-related Abs and clinical characteristics of patients with SSc are influenced by ethnicity. Identifying several SSc-related Abs requires a complicated technique that include immunoprecipitation assay. Establishment of a system routinely available to screen ANA specificities such as enzyme-linked immunosorbent assay is needed.}, pages = {139--147}, title = {全身性強皮症における自己抗体とその臨床的特徴}, volume = {36}, year = {2013} }