在日本多肌炎/皮肌炎患者中，与较轻肌肉疾病相关的抗PL - 7自身抗体出现频率异常高。
Unusually high frequency of autoantibodies to PL-7 associated with milder muscle disease in Japanese patients with polymyositis/dermatomyositis.
作者信息
Yamasaki Yoshioki, Yamada Hidehiro, Nozaki Toshiko, Akaogi Jun, Nichols Cody, Lyons Robert, Loy Anthony Chin, Chan Edward K L, Reeves Westley H, Satoh Minoru
机构信息
University of Florida, Gainesville, USA.
出版信息
Arthritis Rheum. 2006 Jun;54(6):2004-9. doi: 10.1002/art.21883.
OBJECTIVE
Autoantibodies to aminoacyl transfer RNA synthetases, such as histidyl (Jo-1), threonyl (PL-7), alanyl (PL-12), glycyl (EJ), and isoleucyl (OJ), are closely associated with a subset of patients with polymyositis/dermatomyositis (PM/DM) complicated by interstitial lung disease (ILD). Anti-Jo-1 is by far the most common, found in 15-25% of patients with PM/DM, whereas the other types are found in only approximately 3% of these patients. In this study, the clinical associations of these autoantibodies in Japanese patients with PM/DM were investigated.
METHODS
The diagnoses of PM/DM and amyopathic DM (ADM) were based on the Bohan and Peter criteria and Sontheimer's definition, respectively. Sera from 36 Japanese patients with PM/DM (13 with PM, 20 with DM, 3 with ADM) were screened by immunoprecipitation and by enzyme-linked immunosorbent assay (for Jo-1). Clinical and laboratory data were collected.
RESULTS
The frequencies of autoantibodies to Jo-1 (22%) and to EJ, OJ, and PL-12 (3-6%) were similar to those found in previous studies, including studies of Japanese subjects. However, anti-PL-7 was found in 17% of patients, in contrast to a frequency of 1-4% in previous studies (P < 0.02-0.0002). The 6 anti-PL-7-positive patients were not related, and no skewing in year or month of disease development, place of residence or work, or occupation was found. All patients had ILD, consistent with the clinical features of antisynthetase-positive patients. The patients with anti-PL-7 had lower serum muscle enzyme levels and milder muscle weakness (P < 0.05) compared with anti-Jo-1-positive patients.
CONCLUSION
Anti-PL-7 was found at an unusually high frequency in this group of Japanese patients with myositis. Although anti-PL-7, similar to anti-Jo-1, is associated with PM/DM with ILD, muscle involvement in the patients with anti-PL-7 appeared to be milder than that in the anti-Jo-1 subset.
目的
氨酰基转移RNA合成酶自身抗体，如组氨酰（Jo-1）、苏氨酰（PL-7）、丙氨酰（PL-12）、甘氨酰（EJ）和异亮氨酰（OJ）自身抗体，与多肌炎/皮肌炎（PM/DM）合并间质性肺病（ILD）的部分患者密切相关。抗Jo-1抗体是迄今为止最常见的，在15%-25%的PM/DM患者中可检测到，而其他类型仅在约3%的此类患者中出现。在本研究中，调查了这些自身抗体在日本PM/DM患者中的临床相关性。
方法
PM/DM和无肌病性皮肌炎（ADM）的诊断分别基于Bohan和Peter标准以及Sontheimer的定义。通过免疫沉淀和酶联免疫吸附测定（检测Jo-1）对36例日本PM/DM患者（13例PM、20例DM、3例ADM）的血清进行筛查。收集临床和实验室数据。
结果
Jo-1自身抗体（22%）以及EJ、OJ和PL-12自身抗体（3%-6%）的频率与先前研究（包括对日本受试者的研究）中发现的频率相似。然而，17%的患者检测到抗PL-7抗体，而先前研究中的频率为1%-4%（P<0.02-0.0002）。6例抗PL-7抗体阳性患者无亲缘关系，在疾病发生年份或月份、居住或工作地点或职业方面未发现偏差。所有患者均患有ILD，这与抗合成酶抗体阳性患者的临床特征一致。与抗Jo-1抗体阳性患者相比，抗PL-7抗体阳性患者的血清肌酶水平较低，肌肉无力症状较轻（P<0.05）。
结论
在这组日本肌炎患者中，抗PL-7抗体的出现频率异常高。尽管抗PL-7抗体与抗Jo-1抗体一样，与合并ILD的PM/DM相关，但抗PL-7抗体阳性患者的肌肉受累程度似乎比抗Jo-1抗体亚组患者更轻。
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