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抗-Jo1 阳性和抗-PL7/PL12 阳性患者的抗合成酶综合征的长期预后比较。

Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome.

机构信息

Department of Internal Medicine, CHU Rouen, and INSERM U 905, University of Rouen IFRMP, Institute for Biochemical Research, Rouen, France.

出版信息

Autoimmun Rev. 2012 Aug;11(10):739-45. doi: 10.1016/j.autrev.2012.01.006. Epub 2012 Feb 3.

Abstract

The aims of the present study were to: compare the characteristics between antisynthetase syndrome (ASS) patients with anti-Jo1 antibody and those with anti-PL7/PL12 antibody. The medical records of 95 consecutive patients with ASS were reviewed. Seventy-five of these patients had anti-Jo1 antibody; the other patients had anti-PL7 (n=15) or anti-PL12 (n=5) antibody. At ASS diagnosis, the prevalence of myalgia (p=0.007) and muscle weakness (p=0.02) was significantly lower in the group of anti-PL7/PL12-positive patients than in those with anti-Jo1 antibody; median value of CK (p=0.00003) was also lower in anti-PL7/PL12 patients. Anti-Jo1 positive patients developed more rarely myositis resolution (21.3% vs. 46.2%); in addition, the overall recurrence rate of myositis was higher in anti-Jo1 positive patients than in patients with anti-PL7/PL12 antibody (65.9% vs. 19.4%). Anti-Jo1-positive patients, compared with those with anti-PL7/PL12 antibody, more often experienced: joint involvement (63.3%vs. 40%) and cancer (13.3% vs. 5%). By contrast, anti-PL7/PL12 positive patients, compared with those with anti-Jo1 antibody, more commonly exhibited: ILD (90% vs. 68%); in anti-PL7/PL12 positive patients, ILD was more often symptomatic at diagnosis, and led more rarely to resolution of lung manifestations (5.6% vs. 29.4%). Finally, the group of anti-PL7/PL12 positive patients more commonly experienced gastrointestinal manifestations related to ASS (p=0.02). Taken together, although anti-Jo1 positive patients with ASS share some features with those with anti-PL7/PL12 antibody, they exhibit many differences regarding clinical phenotype and long-term outcome. Our study underscores that the presence of anti-Jo1 antibody results in more severe myositis, joint impairment and increased risk of cancer. On the other hand, the presence of anti-PL7/PL12 antibody is markedly associated with: early and severe ILD, and gastrointestinal complications. Thus, our study interestingly indicates that the finding for anti-Jo1 and anti-PL7/PL12 antibodies impacts both the long-term outcome and prognosis of patients with ASS.

摘要

本研究的目的是

比较抗合成酶综合征(ASS)患者抗 Jo1 抗体阳性和抗 PL7/PL12 抗体阳性的特征。回顾了 95 例连续 ASS 患者的病历。其中 75 例患者抗 Jo1 抗体阳性;其余患者抗 PL7(n=15)或抗 PL12(n=5)抗体阳性。在 ASS 诊断时,抗 PL7/PL12 阳性患者的肌痛(p=0.007)和肌肉无力(p=0.02)的发生率明显低于抗 Jo1 抗体阳性患者;CK 的中位数(p=0.00003)也较低。抗 Jo1 阳性患者肌炎缓解的发生率较低(21.3% vs. 46.2%);此外,抗 Jo1 阳性患者的肌炎总体复发率高于抗 PL7/PL12 抗体患者(65.9% vs. 19.4%)。与抗 PL7/PL12 抗体阳性患者相比,抗 Jo1 阳性患者更常出现:关节受累(63.3% vs. 40%)和癌症(13.3% vs. 5%)。相比之下,与抗 Jo1 抗体阳性患者相比,抗 PL7/PL12 阳性患者更常见:ILD(90% vs. 68%);在抗 PL7/PL12 阳性患者中,ILD 在诊断时更常出现症状,且肺部表现缓解的比例较低(5.6% vs. 29.4%)。最后,抗 PL7/PL12 阳性患者更常出现与 ASS 相关的胃肠道表现(p=0.02)。综上所述,尽管 ASS 抗 Jo1 阳性患者与抗 PL7/PL12 抗体阳性患者有一些共同特征,但在临床表现和长期预后方面存在许多差异。我们的研究强调,抗 Jo1 抗体的存在导致更严重的肌炎、关节损伤和癌症风险增加。另一方面,抗 PL7/PL12 抗体的存在与:早期和严重的 ILD 以及胃肠道并发症显著相关。因此,我们的研究有趣地表明,抗 Jo1 和抗 PL7/PL12 抗体的发现不仅影响 ASS 患者的长期预后,也影响患者的预后。

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