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韩国炎性肌病患者的肌炎自身抗体:抗 140kDa 多肤抗体主要与快速进展性间质性肺病相关,而与无肌病性皮肌炎无关。

Myositis autoantibodies in Korean patients with inflammatory myositis: anti-140-kDa polypeptide antibody is primarily associated with rapidly progressive interstitial lung disease independent of clinically amyopathic dermatomyositis.

机构信息

Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.

出版信息

BMC Musculoskelet Disord. 2010 Sep 28;11:223. doi: 10.1186/1471-2474-11-223.

Abstract

BACKGROUND

To investigate the association between myositis autoantibodies and clinical subsets of inflammatory myositis in Korean patients.

METHODS

Immunoprecipitation was performed using the sera of classic polymyositis (PM) (n = 11) and dermatomyositis (DM) (n = 38) patients who met the Bohan and Peter criteria for definite inflammatory myositis. A panel of defined myositis autoantibodies was surveyed to investigate the association between each autoantibody and clinical subsets of inflammatory myositis.

RESULTS

Either MSAs, anti-p140, or anti-p155/140 antibodies were found in 63.3% (31/49) of the study subjects. Anti-140-kDa-polypeptide (anti-p140) (18.4%, 9/49) and anti-155/140-kDa polypeptide (anti-p155/140) (16.3%, 8/49) antibodies were the most common, followed by anti-Mi2 (14.3%, 7/49), anti-ARS (12.2%, 6/49) and anti-SRP (2.0%, 1/49) antibodies. All MSAs and anti-p140 and anti-p155/140 antibodies were mutually exclusive. Anti-p140 (23.7%, 9/38), anti-p155/140 (21.1%, 8/38), and anti-Mi2 (18.4%, 3/38) antibodies were found exclusively in DM patients. Anti-p140 antibody was associated with rapidly progressive interstitial lung disease (ILD) (p = 0.001), with a sensitivity of 100.0% (4/4) and a specificity of 85.3% (29/34) in DM patients. Anti-p155/140 antibody was associated with cancer-associated DM (p = 0.009), with a sensitivity of 55.6% (5/9) and a specificity of 89.7% (26/29). Cancer-associated survival was significantly worse when anti-p155/140 antibody was present (19.2 ± 7.6 vs. 65.0 ± 3.5 months, p = 0.032). Finally, anti-ARS antibodies were associated with stable or slowly progressive ILD in PM and DM patients (p = 0.005).

CONCLUSIONS

Anti-p140 and anti-p155/140 antibodies were commonly found autoantibodies in Korean patients with inflammatory myositis. Despite the lack of clinically amyopathic DM patients in the study subjects, a strong association was observed between anti-p140 antibody and rapidly progressive ILD. Anti-p155/140 antibody was associated with cancer-associated myositis and poor survival.

摘要

背景

为了研究肌炎自身抗体与韩国炎性肌病患者临床亚型之间的关系。

方法

使用经典多发性肌炎(PM)(n=11)和皮肌炎(DM)(n=38)患者的血清进行免疫沉淀,这些患者符合明确炎性肌病的 Bohan 和 Peter 标准。调查了一组明确的肌炎自身抗体,以研究每种自身抗体与炎性肌病的临床亚型之间的关系。

结果

在 49 名研究对象中,有 63.3%(31/49)检测到 MSAs、抗-p140 或抗-p155/140 抗体。抗-140 kDa 多肤(抗-p140)(18.4%,9/49)和抗-155/140 kDa 多肽(抗-p155/140)(16.3%,8/49)抗体最为常见,其次是抗 Mi2(14.3%,7/49)、抗 ARS(12.2%,6/49)和抗 SRP(2.0%,1/49)抗体。所有的 MSAs 和抗-p140 和抗-p155/140 抗体都是相互排斥的。抗-p140(23.7%,9/38)、抗-p155/140(21.1%,8/38)和抗-Mi2(18.4%,3/38)抗体仅在 DM 患者中发现。抗-p140 抗体与快速进展性间质性肺病(ILD)相关(p=0.001),在 DM 患者中具有 100.0%(4/4)的敏感性和 85.3%(29/34)的特异性。抗-p155/140 抗体与癌症相关的 DM 相关(p=0.009),在 DM 患者中具有 55.6%(5/9)的敏感性和 89.7%(26/29)的特异性。当存在抗-p155/140 抗体时,癌症相关的生存率显著降低(19.2±7.6 vs. 65.0±3.5 个月,p=0.032)。最后,抗 ARS 抗体与 PM 和 DM 患者的稳定或缓慢进展性 ILD 相关(p=0.005)。

结论

抗-p140 和抗-p155/140 抗体是韩国炎性肌病患者常见的自身抗体。尽管研究对象中缺乏临床上无肌病的皮肌炎患者,但抗-p140 抗体与快速进展性 ILD 之间存在很强的关联。抗-p155/140 抗体与癌症相关的肌炎和不良预后相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e92/2954990/c965c42f7dae/1471-2474-11-223-1.jpg

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