Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis.
Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis.
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DOI：
10.1186/ar3822
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发表时间：
2012-04-30
影响因子：
4.9
通讯作者：
Satoh M
中科院分区：
文献类型：
--
作者：
Ceribelli A;Fredi M;Taraborelli M;Cavazzana I;Franceschini F;Quinzanini M;Tincani A;Ross SJ;Chan JY;Pauley BA;Chan EK;Satoh M
多发性肌炎/皮肌炎（PM/DM）患者的自身抗体与独特的亚群、临床病程和结局相关。据报道，约25%的青少年DM中存在识别核蛋白NXP-2/MORC 3的抗MJ抗体。研究了意大利成年PM/DM患者抗MJ抗体的患病率和临床意义。采用35 S标记的K562细胞提取物免疫沉淀、ELISA（抗MJ，Jo-1）、Western blot和间接免疫荧光法分析了58例连续的意大利成年PM/DM患者血清。使用来自病历的信息分析临床关联。抗MJ抗体是最常见的特异性（17%），主要见于DM（30%，8例）和PM（8%，2例，P = 0.02）。10例抗MJ（+）与48例抗MJ（-）患者比较，DM更常见（P = 0.03），抗MJ（+）患者发病年龄更年轻（P = 0.0006）。在抗MJ（+）组中，日光性皮疹（P = 0.01）和钙质沉着（P = 0.09）更常见。他们都没有心脏或肺受累，或恶性肿瘤。抗MJ（+）患者的肌病对治疗反应良好，末次访视时无一例CPK升高（0% vs抗MJ（-）患者25%）。尽管NXP-2/MORC 3位于PML中，但只有60%的抗MJ（+）显示免疫荧光核点染色。抗MJ抗体是我们的意大利PM/DM成人队列中最常见的特异性。抗MJ（+）与年轻发病的DM、钙质沉着、无内脏器官受累和肌病对治疗反应良好有关。在青少年DM中发现的抗MJ抗体在成人PM/DM中也存在，可能是一种新的有用的生物标志物。
Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent nuclear dots staining, despite PML localization of NXP-2/MORC3. Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
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影响因子：
15.9
作者：
CHAN, EKL;HAMEL, JC;TAN, EM
通讯作者：
TAN, EM
DOI：
10.1073/pnas.0601066103
发表时间：
2006-04-04
影响因子：
11.1
作者：
Rosendorff, A;Sakakibara, S;Gill, G
通讯作者：
Gill, G
影响因子：
--
作者：
Yamasaki, Yoshioki;Narain, Sonali;Satoh, Minoru
通讯作者：
Satoh, Minoru
影响因子：
4.8
作者：
Kimura, Y;Sakai, F;Osumi, T
通讯作者：
Osumi, T
影响因子：
4
作者：
Mimura, Yasuhiro;Takahashi, Keiko;Inoue, Norimitsu
通讯作者：
Inoue, Norimitsu