Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression

Harsha Gunawardena, Zoe E Betteridge, Neil J McHugh

Research output: Contribution to journalArticle

203 Citations (Scopus)

Abstract

The idiopathic inflammatory myopathies (IIMs)DM and PMhave been historically defined by broad clinical and pathological criteria. These conditions affect both adults and children with clinical features including muscle weakness, skin disease, internal organ involvement and an association with cancer in adults. Using a clinico-serological approach, DM and PM can be defined into more homogeneous subsets. Over the last few years, myositis-specific autoantibodies (MSAs) have been better characterized including autoantibodies directed against the aminoacyl tRNA-synthetase enzymes, the signal-recognition particle and the Mi-2 protein. In addition, clinically significant novel autoantibodiesanti-CADM-140, anti-SAE (small ubiquitin-like modifier activating enzyme), anti-p155/140 and anti-p140have been described in the adult and juvenile disease spectrum. MSAs are directed against cytoplasmic or nuclear components involved in key regulatory intracellular processes including protein synthesis, translocation and gene transcription. The striking association between unique serological profiles and distinct clinical phenotypes suggests that target autoantigens may play a role in disease induction and propagation. In this review, we discuss the clinical utility and pathogenic significance of MSAs in disease expression.
Original languageEnglish
Pages (from-to)607-612
Number of pages6
JournalRheumatology
Volume48
Issue number6
DOIs
Publication statusPublished - 2009

Keywords

  • Dermatomyositis
  • Myositis-specific autoantibodies
  • Polymyositis
  • Inflammatory myopathy

Fingerprint Dive into the research topics of 'Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression'. Together they form a unique fingerprint.

  • Cite this