Calcinosis and malignancy are rare in Chinese adult patients with myositis and nuclear matrix protein 2 antibodies identified by an unlabeled immunoprecipitation assay
Calcinosis and malignancy are rare in Chinese adult patients with myositis and nuclear matrix protein 2 antibodies identified by an unlabeled immunoprecipitation assay
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钙质沉着症和恶性肿瘤在中国成年肌炎患者中很少见，通过未标记的免疫沉淀试验鉴定出核基质蛋白 2 抗体
DOI：
10.1007/s10067-018-4216-x
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发表时间：
2018
影响因子：
3.4
通讯作者：
张华莉
中科院分区：
文献类型：
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作者：
张华莉
肌炎患者的自身抗体可能与特定的临床表现有关。本研究旨在使用未标记免疫沉淀（IP）试验鉴定携带抗核基质蛋白2（抗NXP-2）抗体的肌炎患者亚组，并阐明中国队列中这些患者的特征。我们开发了新的方法，用于未标记的蛋白质IP和Myc标记的截短NXP-2片段的免疫印迹，用于抗NXP-2检测。采用IP和免疫印迹法对120例中国成年肌炎患者血清进行抗NXP-2筛查。使用已建立的未标记蛋白IP和免疫印迹法，在120名患者中的10名（8.3%）中检测到抗NXP-2抗体，其中70%（7/10）表现出日光疹或Gottron丘疹。所有10例抗恩智浦-2阳性患者均表现出肌病，60%的患者主诉吞咽困难。重度弥漫性钙质沉着症（10%）和鼻咽癌（10%）分别仅存在于单个抗NXP-2阳性肌炎患者中。在四名存活但三名死亡的抗NXP-2阳性患者中发现了抗Ro-52抗体。对13项抗恩智浦-2研究的综合审查表明，日本、中国和匈牙利成年肌炎患者中抗恩智浦-2的患病率明显低于美国和意大利，抗恩智浦-2与钙质沉着症的相关性也低于美国和意大利。在不同的研究中，抗NXP-2抗体与成人患者体内恶性肿瘤的相关性从0到50%不等。开发了一种新的IP测定法来检测表达抗NXP-2的肌炎患者。在抗恩智浦-2抗体阳性的中国成年肌炎患者中，钙质沉着和恶性肿瘤是罕见的。文献综述表明，在美国和意大利肌炎队列中，抗恩智浦-2抗体的患病率最高，抗恩智浦-2抗体与钙质沉着症相关。
Autoantibodies in patients with myositis may associate with specific clinical manifestations. This study aimed to identify a subset of patients with myositis carrying antinuclear matrix protein 2 (anti-NXP-2) antibodies using an unlabeled immunoprecipitation (IP) assay, and clarify the features of these patients in a Chinese cohort. We developed novel methods for unlabeled protein IP and immunoblotting of Myc-tagged truncated NXP-2 fragments for anti-NXP-2 detection. The sera of 120 Chinese adult patients with myositis were screened for anti-NXP-2 by IP and immunoblot. Anti-NXP-2 antibodies were detected in 10 of the 120 patients (8.3%) using the established unlabeled protein IP and immunoblotting, with 70% (7/10) exhibiting either heliotrope rash or Gottron’s papules. All 10 anti-NXP-2-positive patients exhibited myopathy and 60% complained of dysphagia. Severe diffuse calcinosis (10%) and nasopharyngeal carcinoma (10%) were each only present in single anti-NXP-2-positive patients with myositis. Antibodies against Ro-52 were found in four living but not in three deceased anti-NXP-2-positive patients. A comprehensive review of 13 anti-NXP-2 studies demonstrated markedly lower anti-NXP-2 prevalence among adult patients with myositis and lower association of anti-NXP-2 with calcinosis in Japan, China, and Hungary than in the USA and Italy. Anti-NXP-2 antibody association with internal malignancy in adult patients varied from 0 to 50% across different studies. A novel IP assay was developed to detect patients with myositis expressing anti-NXP-2. Calcinosis and malignancy are rare in Chinese adult patients with myositis positive for anti-NXP-2. Literature review indicated highest anti-NXP-2 prevalence and association of anti-NXP-2 with calcinosis in US and Italian myositis cohorts.