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皮膚筋炎は多様な疾患であり,その診療にはより均質なサブセットに分けて考えることが必要である。自己抗体は臨床所見と強く相関するため,このようなサブセットに分類するうえで有用なツールとなる。皮膚筋炎では,これまでに5つの自己抗体が特異的自己抗体としてその臨床的意義が確立している。その他にも,さまざまな自己抗体が報告されており,病因や病態を考えるうえでも興味深いものがある。
Abstract
Dermatomyositis is a heterogeneous disorder that can be classified into more homogeneous subsets. Autoantibodies are a useful tool in identifying such subsets because they correlate strongly with clinical phenotypes. In dermatomyositis, five disease-specific autoantibodies have been established to date, including antiMi-2, anti-melanoma differentiation-associated gene 5, anti-transcriptional intermediary factor 1, anti-nuclear matrix protein 2, anti-transcriptional intermediary factor 1, and anti-small-ubiquitin-like activating enzyme antibodies. In addition, several novel autoantibodies have recently been demonstrated in patients with dermatomyositis, including anti-four-and-a-half-LIM-domain 1, anti-cell division cycle and apoptosis regulator protein 1, anti-specificity protein 4, anti-cortactin, and IgM anti-angiotensin converting enzyme 2 antibodies.
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