Antisynthetase-Syndrome

ZUSAMMENFASSUNG

Die Antisynthetase-Syndrome bilden eine Untergruppe der idiopathischen inflammatorischen Myopathien. Sie zeichnen sich aus durch mindestens eines der 3 Hauptsymptome Myositis, interstitielle Lungenerkrankung (ILD) und Arthritis in Kombination mit dem serologischen Nachweis eines Antikörpers gegen Aminoacyl-tRNA-Synthetasen. Zusätzlich können die Nebensymptome Mechanikerhände/-füße, Raynaud-Syndrom und/oder Fieber auftreten. Eine ILD ist häufig und bestimmt maßgeblich die Morbidität und Mortalität. 8 Antisynthetase-Antikörper sind bekannt. Häufigkeit und Schwere der Haupt- und Nebensymptome unterscheiden sich zwischen den einzelnen Antikörpern, an denen der anti-Jo-1-Antikörper den größten Anteil ausmacht. Die Wahl der immunsuppressiven Therapie richtet sich nach der Organbeteiligung. Die beste Prognose hat das Jo-1-Syndrom mit einer 5-Jahres-Überlebensrate von ca. 90 %.

ABSTRACT

Antisynthetase syndromes belong to the idiopathic inflammatory myopathies. Occurrence of at least one of the 3 main symptoms in combination with serological evidence of an aminoacyl-tRNA-synthetase antibody is characteristic. Main symptoms are myositis, interstitial lung disease (ILD) and arthritis. Possible accompanying symptoms as mechanic’s hands/feet, Raynaud’s disease and/or fever can additionally occur. ILD is frequent and predominantly responsible for morbidity and mortality. 8 antisynthetase antibodies are known. Rate and seriousness of the main and accompanying symptoms differ between the different antibodies, of which Jo-1 antibody is by far the most common one. Choice of immunosuppressive medication considers to what extent and how seriously organs are affected. With a 5-year survival rate of about 90 %, Jo-1 syndrome has the best prognosis.

Schlüsselwörter

Antisynthetase-Antikörper - Polymyositis - interstitielle Lungenerkrankung, Jo-1 - Mechanikerhände

Keywords

Antisynthetase antibodies - polymyositis - interstitial lung disease - Jo-1, mechanic hands

Publication History

Article published online:
07 September 2022

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