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Detail
ArtikelInclusion Body Myositis  
Oleh: Greenberg, Steven A.
Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: Continuum vol. 22 no. 06 (Dec. 2016), page 1871-1888.
Topik: Inclusion Body Myositis; IBM
Ketersediaan
  • Perpustakaan FK
    • Nomor Panggil: C17.K
    • Non-tandon: 1 (dapat dipinjam: 0)
    • Tandon: tidak ada
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Isi artikelPurpose of Review: Inclusion body myositis (IBM) is an enigmatic progressive disease of skeletal muscle. This review provides a summary of the clinical and pathophysiologic aspects of IBM. Recent Findings: The development of diagnostic blood testing for IBM followed from the discovery of a B-cell pathway in IBM muscle and circulating autoantibodies against NT5C1A, further establishing IBM’s status as an autoimmune disease. The key role of cytotoxic T cells in IBM is further supported by the identification of a link between IBM and T-cell large granular lymphocytic leukemia. The testing of research diagnostic criteria in patients is improving its accuracy. Increases in estimated prevalences may be due to a combination of true increases and improved recognition of disease. Summary: IBM has high unmet medical need. Advances in the mechanistic understanding of IBM as an autoimmune disease will drive effective therapeutic approaches. The identification of a B-cell pathway has resulted in the first identification of an IBM autoantigen and emphasized its status as an autoimmune disease. The recognition that large granular lymphocyte CD8+ T-cell expansions are present in both blood and muscle provides additional biomarkers for IBM and suggests a mechanistic relationship to the neoplastic disease T-cell large granular lymphocytic leukemia.
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