[Antisynthetase syndromes]. / Antisynthetasesyndrome.

ABSTRACT

Antisynthetase syndromes (ASS) are rare autoimmune diseases. Characteristic is the presence of at least one of the three main symptoms myositis, interstitial lung disease (ILD) and arthritis with possible accompanying symptoms, such as mechanic's hands and feet, Raynaud's disease and/or fever in combination with detection of an aminoacyl-tRNA synthetase antibody in peripheral blood. In addition to myositis, ILD is a frequent and often predominant organ involvement and is responsible for morbidity and mortality. Autoantibodies to 11 aminoacyl-tRNA synthetases are known of which 8 have so far been associated with the clinical manifestation of ASS. The Jo-1 antibody is by far the most frequent one. The antibodies differ in the rate and severity of the main and accompanying symptoms. Treatment with selected immunosuppressive medication depends on the extent and severity of organ involvement. With a 5-year survival rate of approximately 90%, the Jo-1 syndrome has the best prognosis.