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翻译者:高洁,西京医院临床免疫科
目 标:
为已诊断是多发性肌炎(PM)和皮肌炎(DM)的患者确定提示可能出现钙质沉着的临床和实验室预测因子。
方 法:
回顾性分析2013年1月和2014年5月之间就诊笔者诊所的肌炎患者。
结 果:
本文报告74例PM(30例)、DM(30例)、重叠综合征(13例)、包涵体肌炎(1例)。钙质沉着症16例(21.6%),发生于诊断为PM/DM平均43.7个月后。在多元分析中,与不伴有钙质沉着症的患者相比,钙质沉着症患者经历更长的随访时间(p=0.006)、抗-PM/Scl(p=0.033)和抗-NXP2(p=0.024)阳性率也高于无钙质沉着的患者。此外,抗-NXP-2阳性C+患从一开始即表现为弥漫性钙质沉着,但呼吸道受累频率较低。在治疗钙质沉着症方面,目前尚没有单一药物或药物联合使用明确有效者。
结 论:
随访时间长、诊断为皮肌炎者和PM/Scl或NXP-2阳性均可被认为是预测钙质沉着症发展的危险因素。此外,认为NXP-2抗体的阳性为钙质沉着症的特异性抗体,有发病早、进展迅速的特点。
参考文献:
Clin Exp Rheumatol. 2017 Mar-Apr;35(2):303-308. Epub 2016Nov 14.
We aimed to identify the possible clinical andlaboratory predictors of calcinosis in a cohort of patients with adiagnosis of polymyositis (PM) and dermatomyositis (DM).
We carried out a retrospective analysis of acohort of myositis patients attending our clinic between January 2013 and May2014.
74 patients (58 females, 16 males) with PM (30cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis(1 case) were enrolled. Sixteen patients (21.6%) had calcinosis thatoccurred a mean of 43.7 months after diagnosis of PDM. At multivariateanalysis, patients with calcinosis experienced longer follow-upduration (p=0.006), anti-PM/Scl (p=0.033) and anti-NXP2 (p=0.024) positivitycompared to patients without calcinosis. Furthermore, anti-NXP-2 positiveC+ showed a diffuse form of calcinosis from the beginning and lowerfrequency of respiratory tract involvement. No single drug or associations ofdrugs was found effective in the treatment of calcinosis.
A longer follow-up period of time, DM diagnosisand positivity for PM/Scl and NXP-2 could all be considered risk factors whichforesee the development of calcinosis. Moreover, the positivity forantibodies to NXP-2 depicts a distinct phenotype of calcinosis with anearly onset and quick widespread dissemination.
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