Cojocaru Manole, Cojocaru Inimioara Mihaela, Chicos Bogdan
Faculty of Medicine, "Titu Maiorescu" University, Bucharest, Romania.
"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
Maedica (Bucur). 2016 Jun;11(2):130-135.
Antisynthetase syndrome (ASS) is a rare chronic autoimmune disorder (2-3 times more common in women than in men), associated with interstitial lung disease (the most important feature), dermatomyositis (DM), and polymyositis (PM). The cause of ASS is unknown. Recent developments in immunology have improved our knowledge and it is now possible to classify ASS according to the presence of myositis specific autoantibodies. The hallmark of ASS is the presence of serum autoantibodies directed against aminoacyl-tRNA synthetases (anti-ARS involved in protein synthesis). ASS is due to IgG antibodies directed against the enzyme synthase. Antisynthetase antibodies (ASAb) include: anti-histidyl- (anti-Jo-1, being the best known), anti-threonyl- (anti-PL-7), anti-alanyl (anti-PL-12), anti-isoleucyl- (anti-OJ), anti-glycyl- (anti-EJ), anti-asparaginyl- (anti-KS), anti-Wa, anti-tyrosil- (anti-YRS), anti-phenylalanyl-transfer RNA synthetase (anti-Zo), and anti-signal recognition particle (anti-SRP). Anti-Jo-1 is the most common ASAb (in ~20-30% of PM/DM patients).
抗合成酶综合征（ASS）是一种罕见的慢性自身免疫性疾病（女性发病率比男性高2至3倍），与间质性肺病（最重要的特征）、皮肌炎（DM）和多发性肌炎（PM）相关。ASS的病因尚不清楚。免疫学的最新进展增进了我们的了解，现在可以根据肌炎特异性自身抗体的存在对ASS进行分类。ASS的标志是存在针对氨酰-tRNA合成酶的血清自身抗体（参与蛋白质合成的抗ARS）。ASS是由针对酶合成酶的IgG抗体引起的。抗合成酶抗体（ASAb）包括：抗组氨酰-（抗Jo-1，最为人熟知）、抗苏氨酰-（抗PL-7）、抗丙氨酰-（抗PL-12）、抗异亮氨酰-（抗OJ）、抗甘氨酰-（抗EJ）、抗天冬酰胺酰-（抗KS）、抗Wa、抗酪氨酰-（抗YRS）、抗苯丙氨酰转移RNA合成酶（抗Zo）和抗信号识别颗粒（抗SRP）。抗Jo-1是最常见的ASAb（在约20%至30%的PM/DM患者中）。
