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DM、PM钙质沉着的临床和实验室预测指标及治疗选择

2019年01月17日 8860人阅读 返回文章列表

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20131月和20145月之间就诊笔者诊所的肌炎患者。

结  果:

本文报告74PM30例)、DM30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/SclNXP-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.

Calcinosis inpoly-dermatomyositis: clinical and laboratory predictors and treatment options

OBJECTIVES:

We aimed to identify the possible clinical andlaboratory predictors of calcinosis in a cohort of patients with adiagnosis of polymyositis (PM) and dermatomyositis (DM).

METHODS:

We carried out a retrospective analysis of acohort of myositis patients attending our clinic between January 2013 and May2014.

RESULTS:

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.

CONCLUSIONS:

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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