Bizien N, Renault A, Boles J-M, Delluc A
Département de médecine interne et pneumologie, CHU de Brest, France.
Rev Pneumol Clin. 2011 Dec;67(6):367-70. doi: 10.1016/j.pneumo.2011.01.003. Epub 2011 Apr 29.
The antisynthetase syndrome is characterized by the presence of myositis, interstitial lung disease, arthritis, Raynaud's phenomenon, mechanics hands and anti-Jo1 antibody (histidyl tRNA synthetase). The prognosis of this syndrome is closely related to the severity of lung disease. Myositis can occur several years after lung disease and some patients with interstitial lung disease associated with anti-Jo1 antibodies will not suffer from muscle disease.
CASE-REPORT: We report the case of a 69-year-old man admitted to the medical intensive care unit for acute respiratory insufficiency related to rapidly progressive interstitial lung disease. Antisynthetase syndrome was diagnosed the presence of wrists' arthritis, 'mechanic's hands and anti-Jo1 antibodies. Despite the dramatic efficacy of corticosteroid therapy on ventilation parameters, the patient died from a Pseudomonas Aeruginosa nosocomial ventilator-acquired pneumonia.
Our case emphasizes the importance to search for anti-Jo1 antibodies in the presence of interstitial lung disease. During the course of antisynthetase syndrome, the occurrence of interstitial lung disease is almost always constant and is correlated with poor prognosis.
抗合成酶综合征的特征为存在肌炎、间质性肺病、关节炎、雷诺现象、技工手和抗Jo1抗体（组氨酰tRNA合成酶）。该综合征的预后与肺部疾病的严重程度密切相关。肌炎可在肺部疾病发生数年之后出现，一些伴有抗Jo1抗体的间质性肺病患者不会罹患肌肉疾病。
我们报告一例69岁男性患者，因与快速进展性间质性肺病相关的急性呼吸衰竭入住医疗重症监护病房。根据存在腕关节关节炎、“技工手”和抗Jo1抗体，诊断为抗合成酶综合征。尽管皮质类固醇疗法对通气参数有显著疗效，但患者死于铜绿假单胞菌医院获得性呼吸机相关性肺炎。
我们的病例强调了在存在间质性肺病时检测抗Jo1抗体的重要性。在抗合成酶综合征病程中，间质性肺病几乎总是存在，且与预后不良相关。
