PDF(Pubmed)
Abstract：
Some myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM).
To assess the significance of DH/BS in patients with IM.
Practitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1.
49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05).
In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).
To assess the significance of DH/BS in patients with IM.
Practitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1.
49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05).
In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).
摘要：
背景：某些肌病可导致头部下垂或脊柱弯曲综合征（DH/BS）。尚未在炎性肌病（IM）中研究该症状的重要性。
目的：评估IM患者中DH/BS的意义。
方法：邀请来自五个IM网络的从业者报告患有DH/BS的IM患者（除IM外没有其他已知原因）。IM患者无DH/BS，在每个参与中心随机选择，作为对照，比例为2：1。
结果：49例DH/BS-IM患者（DH：57.1％，BS：42.9%）与98例对照IM患者进行了比较。DH/BS-IM患者年龄较大（65岁vs53岁，p<0.0001），IM的诊断延迟（6个月vs3个月，p=0.009)。上肢普遍虚弱（42.9%vs15.3%），吞咽困难（57.1%vs25.5%），肌肉萎缩（65.3%vs34.7%），体重减轻（61.2％vs23.5％）和行走能力丧失（24.5％vs5.1％）是DH/BS-IM的标志（p≤0.0005），患者更频繁地接受静脉注射免疫球蛋白(65.3%vs34.7%，p=0.0004)。此外，DH/BS-IM患者经常表现为系统性硬化症（SSc）的体征和/或并发症，在40.8%的病例中，符合美国风湿病学会/欧洲风湿病学协会联盟的标准(vs5.1%，p<0.0001)。肌病的分布，其严重程度及其与SSc的相关性与DH/BS独立相关(p<0.05)。DH/BS-IM患者的死亡率更高，步行能力丧失与生存率独立相关（p<0.05）。
结论：在IM患者中，DH/BS是严重程度的标志物并且与SSc(巩膜肌炎)相关。
目的：评估IM患者中DH/BS的意义。
方法：邀请来自五个IM网络的从业者报告患有DH/BS的IM患者（除IM外没有其他已知原因）。IM患者无DH/BS，在每个参与中心随机选择，作为对照，比例为2：1。
结果：49例DH/BS-IM患者（DH：57.1％，BS：42.9%）与98例对照IM患者进行了比较。DH/BS-IM患者年龄较大（65岁vs53岁，p<0.0001），IM的诊断延迟（6个月vs3个月，p=0.009)。上肢普遍虚弱（42.9%vs15.3%），吞咽困难（57.1%vs25.5%），肌肉萎缩（65.3%vs34.7%），体重减轻（61.2％vs23.5％）和行走能力丧失（24.5％vs5.1％）是DH/BS-IM的标志（p≤0.0005），患者更频繁地接受静脉注射免疫球蛋白(65.3%vs34.7%，p=0.0004)。此外，DH/BS-IM患者经常表现为系统性硬化症（SSc）的体征和/或并发症，在40.8%的病例中，符合美国风湿病学会/欧洲风湿病学协会联盟的标准(vs5.1%，p<0.0001)。肌病的分布，其严重程度及其与SSc的相关性与DH/BS独立相关(p<0.05)。DH/BS-IM患者的死亡率更高，步行能力丧失与生存率独立相关（p<0.05）。
结论：在IM患者中，DH/BS是严重程度的标志物并且与SSc(巩膜肌炎)相关。
