Clinical Reviews in Allergy & Immunology

, Volume 52, Issue 1, pp 1–19 | Cite as

A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy

  • Minoru SatohEmail author
  • Shin Tanaka
  • Angela Ceribelli
  • S. John Calise
  • Edward K. L. Chan


Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP). Anti-Jo-1 is the first autoantibodies to ARS detected in 15–25 % of patients. In addition to anti-Jo-1, antibodies to seven other aminoacyl tRNA synthetases (ARS) have been reported with prevalence, usually 1–5 % or lower. Patients with any anti-ARS antibodies are associated with anti-synthetase syndrome characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud’s phenomenon, and others. Several recent studies suggested heterogeneity in clinical features among different anti-ARS antibody-positive patients and anti-ARS may also be found in idiopathic ILD without myositis. Anti-Mi-2 is a classic marker for DM and associated with good response to steroid treatment and good prognosis. Anti-SRP is specific for PM and associated with treatment-resistant myopathy histologically characterized as necrotizing myopathy. In addition to classic MSAs, several new autoantibodies with strong clinical significance have been described in DM. Antibodies to transcription intermediary factor 1γ/α (TIF1γ/α, p155/140) are frequently found in DM associated with malignancy while anti-melanoma differentiation-associated gene 5 (MDA5; CADM140) are associated with clinically amyopathic DM (CADM) complicated by rapidly progressive ILD. Also, anti-MJ/nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modifier-1 (SUMO-1) activating enzyme (SAE) are recognized as new DM-specific autoantibodies. Addition of these new antibodies to clinical practice in the future will help in making earlier and more accurate diagnoses and better management for patients.


Autoantibodies Anti-nuclear antibodies Polymyositis Dermatomyositis Inflammatory myopathy 



This work was supported by JSPS KAKENHI (Grants-in-Aid for Scientific Research) grant number 15K08790.


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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • Minoru Satoh
    • 1
    Email author
  • Shin Tanaka
    • 2
  • Angela Ceribelli
    • 3
    • 4
  • S. John Calise
    • 5
  • Edward K. L. Chan
    • 5
  1. 1.Department of Clinical Nursing, School of Health SciencesUniversity of Occupational and Environmental Health, JapanKitakyushuJapan
  2. 2.Department of Human Information and Sciences, School of Health SciencesUniversity of Occupational and Environmental HealthKitakyushuJapan
  3. 3.Rheumatology and Clinical ImmunologyHumanitas Clinical and Research CenterRozzano (Milan)Italy
  4. 4.BIOMETRA DepartmentUniversity of MilanMilanItaly
  5. 5.Department of Oral BiologyUniversity of FloridaGainesvilleUSA

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