Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights

Autoimmun Rev. 2007 Aug;6(7):432-7. doi: 10.1016/j.autrev.2007.01.013. Epub 2007 Feb 20.


A characteristic feature of patients suffering from connective tissue diseases such as polymyositis (PM), dermatomyositis, systemic sclerosis (scleroderma, SSc, Scl), systemic lupus erythematosus or overlap syndromes thereof are anti-nuclear antibodies and anti-nucleolar antibodies. Antibodies to the PM/Scl complex, also known as the human exosome complex, belong to the anti-nucleolar antibodies and are mainly found in patients with PM/SSc overlap syndrome and related diseases. Until recently, the detection of anti-PM/Scl antibodies was laborious and relied largely on indirect immunofluorescence and immunodiffusion techniques. With the identification and characterization of the autoantigens, especially PM/Scl-75, PM/Scl-100 and a synthetic peptide (PM1-Alpha) thereof, newly developed assays based on recombinant proteins and peptides have allowed the development of a new generation of anti-PM/Scl tests with high sensitivity and specificity. These novel assays (i.e. ELISA, line immunoassays and protein arrays) enable testing for anti-PM/Scl in modern, automated, multi-parametric assay settings. The present review focuses on recent insights on anti-PM/Scl autoantibodies with special emphasis on clinical, genetic and diagnostic aspects.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antibodies, Antinuclear
  • Autoantibodies*
  • Cell Nucleolus / immunology
  • Dermatomyositis / diagnosis
  • Dermatomyositis / genetics
  • Dermatomyositis / immunology
  • Humans
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology
  • Polymyositis / diagnosis
  • Polymyositis / genetics
  • Polymyositis / immunology*
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / genetics
  • Scleroderma, Systemic / immunology*


  • Antibodies, Antinuclear
  • Autoantibodies