IntroductionAntisynthetase syndrome (ASS) is a rare idiopathic inflammatory myopathy with nearly 89% showing interstitial lung disease (ILD). The hallmark of ASS is the presence of serum autoantibodies directed against aminoacyl-tRNA synthetases that include Jo-1 (most commonly detected), PL-7, PL-12, OJ, EJ, KS, Wa, YRS and Zo. However, in a small subpopulation without evidence of myositis, the diagnosis may be critically delayed, hindering management of this rapidly progressive disease.We report an interesting case of anti-PL-12/anti-SSA 52kD ASS presenting as acute digital ischemia, an association rarely described previously. In cases with ILD, the severity of lung condition generally determines the prognosis.
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