Antisynthetase Syndrome: two case report and literature review

Rev Bras Reumatol. 2015 Mar-Apr;55(2):177-80. doi: 10.1016/j.rbr.2013.05.007. Epub 2014 Oct 5.

Abstract

Antissintetase Syndrome (ASS) is characterized by myositis, Raunaud's phenomenon, fever, intertitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against tRNA synthetase, especially anti-Jo-1. This article aims to review the literature on ASS and report two cases where the first is a patient with polymyositis who developed subluxation on the proximal interphalangeal joint of bilateral first right finger after a few years of the disease, associated with pulmonary manifestations and positive anti-JO-1. In the second case, we present a patient with dermatomyositis, who developed a subluxation of the two first fingers, anti-Jo1 positive and chest CT changes, but without clinical evidence of pulmonary involvement. These cases reveal the importance of performing early diagnosis. The authors describe two cases of this rare syndrome, emphasizing the severity of interstitial lung disease and arthritis.

Keywords: Anti Jo1; Antissintetase Syndrome; Anti‐Jo 1; Doença intersticial pulmonar; Interstitial lung disease; Polidermatomiosite; Polydermatomyositis; Síndrome antissintetase.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Female
  • Humans
  • Middle Aged
  • Myositis / diagnosis*

Supplementary concepts

  • Antisynthetase syndrome