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Clinical Heterogeneity and Outcomes of Antisynthetase Syndrome

  • Baptiste HervierEmail author
  • Olivier Benveniste
INFLAMMATORY MUSCLE DISEASE (I LUNDBERG, SECTION EDITOR)
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease

Abstract

The autoimmune connective tissue disease antisynthetase syndrome (ASS) is an inflammatory myopathy associated with myositis-specific autoantibodies, e.g. anti-tRNA-synthetase antibodies (ASA). Since 1976 eight different ASA have been rigorously identified, of which anti-hystidyl-tRNA synthetase (anti-Jo1) is the most prevalent. Other phenotype features of ASS include interstitial lung disease (ILD), Raynaud’s phenomenon, polyarthritis, fever, and mechanic’s hands. The clinical presentation of ASS varies greatly, as does the severity of involvement of different organs—both among patients and/or over the course of the disease. ILD has been associated with poor outcomes, but in general the heterogeneity of ASS prevents identification of robust prognosis indicators. Early identification of patients requiring aggressive immunosuppressive treatment is very challenging, and there are very few prospective trials available to help match treatment management to ASS clinical characteristics. This review will focus on the biological, clinical, functional, and morphological features of ASS associated with patient outcome. Our objective is to use compiled data on these subjects to discuss the usefulness of patient stratification in developing future prospective therapeutic trials.

Keywords

Inflammatory myopathy Myositis Myositis-specific antibodies Antisynthetase Anti-Jo1 Interstitial lung disease Clinical heterogeneity 

Abbreviations

ASS

Anti-synthetase syndrome

ASA

Anti-synthetase antibody

ILD

Interstitial lung disease

SSc

Systemic sclerosis

SS

Sjögren syndrome

Notes

Compliance with Ethics Guidelines

Conflict of Interest

Baptiste Hervier declares that he has no conflict of interest.

Olivier Benveniste declares that he has no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Internal Medicine DepartmentFrench Referral Center for Lupus and Antiphospholipid Syndrome, APHP, Hôpital Pitié SalpêtrièreParis Cedex 13France
  2. 2.Internal Medicine DepartmentFrench Referral Center for Muscular Diseases, APHP, Hôpital Pitié SalpêtrièreParisFrance

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