Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options.
Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options.
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发表时间：
2017-03
影响因子：
3.7
通讯作者：
M. Fredi;F. Bartoli;I. Cavazzana;A. Ceribelli;N. Carabellese;A. Tincani;M. Satoh;F. Franceschini
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作者：
M. Fredi;F. Bartoli;I. Cavazzana;A. Ceribelli;N. Carabellese;A. Tincani;M. Satoh;F. Franceschini
我们的目的是在一组诊断为多发性肌炎（PM）和皮肌炎（DM）的患者中确定可能的临床和实验室预测因素。方法：我们对2013年1月至2014年5月期间在我们诊所就诊的一组肌炎患者进行了回顾性分析。结果74例患者中，女性58例，男性16例，其中PM 30例，DM 30例，重叠综合征13例，包涵体肌炎1例。16名患者（21.6%）在PDM诊断后平均43.7个月发生钙质沉着。在多变量分析中，与无钙质沉着的患者相比，钙质沉着患者的随访时间更长（p=0.006），抗PM/Scl（p=0.033）和抗NXP 2（p=0.024）阳性。此外，抗NXP-2阳性C+从一开始就表现出弥漫性钙质沉着，呼吸道受累频率较低。没有发现单一药物或药物联合治疗钙质沉着症有效。结论：较长的随访时间、DM诊断和PM/Scl和NXP-2阳性都可以被认为是预测钙质沉着发展的危险因素。此外，恩智浦-2抗体阳性描述了一种独特的钙质沉着症表型，具有早期发病和快速广泛传播。
OBJECTIVES We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM). METHODS We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014. RESULTS 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled. Sixteen patients (21.6%) had calcinosis that occurred a mean of 43.7 months after diagnosis of PDM. At multivariate analysis, patients with calcinosis experienced longer follow-up duration (p=0.006), anti-PM/Scl (p=0.033) and anti-NXP2 (p=0.024) positivity compared to patients without calcinosis. Furthermore, anti-NXP-2 positive C+ showed a diffuse form of calcinosis from the beginning and lower frequency of respiratory tract involvement. No single drug or associations of drugs was found effective in the treatment of calcinosis. CONCLUSIONS A longer follow-up period of time, DM diagnosis and positivity for PM/Scl and NXP-2 could all be considered risk factors which foresee the development of calcinosis. Moreover, the positivity for antibodies to NXP-2 depicts a distinct phenotype of calcinosis with an early onset and quick widespread dissemination.