Antisynthetase Syndrome

  • Elena Katzap
  • Maria-Louise Barilla-LaBarca
  • Galina Marder
Article

DOI: 10.1007/s11926-011-0176-8

Cite this article as:
Katzap, E., Barilla-LaBarca, ML. & Marder, G. Curr Rheumatol Rep (2011) 13: 175. doi:10.1007/s11926-011-0176-8

Abstract

Autoantibodies to eight of the aminoacyl-transfer RNA synthetases—the most well-recognized of which is anti-histidyl (Jo-1)—have all been implicated in the pathogenesis of antisynthetase syndrome (AS). AS is characterized by varying degrees of interstitial lung disease, myositis, arthropathy, fever, Raynaud’s phenomenon, and mechanic’s hands, and the morbidity and mortality of the disease are usually linked to the pulmonary findings. The value of a lung biopsy in AS cannot be overemphasized, as it serves to describe the underlying etiology of the interstitial lung disease, guide therapy, and estimate prognosis. Muscle disease shares many clinical features of polymyositis, yet histologically, the inflammatory involvement resembles that of dermatomyositis. Because inflammatory arthritis mimics rheumatoid arthritis, AS should be considered in atypical cases. Corticosteroids are the mainstay of acute therapy, although treatment often requires immunosuppressant medications such as cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, or rituximab.

Keywords

Antisynthetase syndrome Jo-1 Interstitial lung disease Arthritis Autoantibodies Antisynthetase autoantibodies Myositis Cutaneous Corticosteroid Pulmonary disease 

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Elena Katzap
    • 1
  • Maria-Louise Barilla-LaBarca
    • 1
  • Galina Marder
    • 1
  1. 1.Division of Rheumatology and Allergy-Clinical ImmunologyNorth Shore Long Island Jewish Health SystemLake SuccessUSA

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