INTRODUCTION
Aminoacyl tRNA synthetases (ARSs) are a group of cytoplasmic enzymes
that bind to transcribed RNA during protein synthesis, esterify amino
acids to transcribed RNA, and function as catalysts for
aminoacyl-transcribed RNA.1 Patients who test positive
for anti-ARS antibodies present with various extramuscular symptoms,
such as interstitial pneumonia, mechanic’s hands, Raynaud phenomenon,
polyarthritis, fever, and myositis. These symptoms share common clinical
features, which led Targoff in 1992 to term them ”antisynthetase
antibody syndrome.”2 Since Jo-1 (histidyl-tRNA
synthetase: HisRS) antibody was discovered in 19803,
eight ARS antibodies have been identified: histidyl-tRNA synthetase-1
(Jo-1), anti-threonyl (PL-7), anti-alanyl (PL-12), anti-glycyl (EJ),
anti-isoleucyl (OJ) anti-asparaginyl (KS), anti-phenylalanyl (Zo) and
anti-tyrosyl (Ha) tRNA synthetase4.5, 6 Among these antibodies,
anti-OJ is the least prevalent (3.1%).7 Anti-OJ-
positive anti-ARS syndrome is extremely rare, and is usually accompanied
by interstitial pneumonia.8 Anti-OJ and
anti-Sjögren’s-syndrome-related antigen A (Ro-52) antibodies are
occasionally detected in patients with dermatomyositis and are a poor
prognostic factor when associated with interstitial
pneumonia.9 However, we encountered a patient with
OJ-and Ro-52 positive anti-ARS syndrome without interstitial pneumonia
and with severe phalanx necrosis due to Raynaud phenomenon. The activity
of disease could be suppressed with steroid pulse therapy. We believe
that this is an atypical clinical presentation that deserves
acknowledgement by means of this case report. We also report it here
with a review of the literature.