Esophageal Motor Abnormalities in Patients With Scleroderma: Heterogeneity, Risk Factors, and Effects on Quality of Life
Esophageal Motor Abnormalities in Patients With Scleroderma: Heterogeneity, Risk Factors, and Effects on Quality of Life
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DOI：
10.1016/j.cgh.2016.08.034
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发表时间：
2017-02-01
影响因子：
12.6
通讯作者：
Vela, Marcelo F.
中科院分区：
文献类型：
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作者：
Crowell, Michael D.;Umar, Sarah B.;Vela, Marcelo F.
背景与目的：系统性硬皮病(SSC)与食道不张和低血压的食管胃交界处压力有关，尽管食道运动功能障碍可能存在分级。我们通过高分辨率食道测压(HRM)来表征食道运动功能，并评估SSC严重程度、健康相关生活质量(HRQOL)和HRM结果之间的关系。方法：我们对2006年5月至2015年1月在亚利桑那州梅奥诊所接受HRM的200名SSC患者和102名非SSC患者(对照组)进行了前瞻性研究。我们使用综合松弛压力、远端收缩积分和远端潜伏期的数据，根据芝加哥分类v3.0对食道动力障碍进行分类。一组受试者(n=122)完成了SSC特有的胃肠道症状和HRQOL问卷。比较弥漫性、局限性和对照组受试者的HRM结果、症状和HRQOL数据。分类变量比较采用chi(2)或Fisher精确检验，连续变量比较采用Mann-Whitney或Kruskal-Wallis检验。结果：在SSc患者中，弥漫性SSC患者83例(42%)，局限性SSC患者117例(58%)。SSC患者缺乏收缩功能的发生率高于对照组(56%比13%；P<；.001)。不同患者的HRM表现各不相同；最常见的诊断是收缩功能缺失(56%)，其次是运动正常(26%)和食道动力无效(10%)。典型硬皮病(食管胃交界处压力缺乏收缩)仅见于33%的患者(弥漫性SSC占34%，局限性SSC占32%)(P=.880)。严重的食道动力障碍与病程、间质性肺病和较高的胃肠道症状评分有关(P<；.001)。结论：尽管重度运动障碍在SSc患者中比对照组更常见，但我们只在三分之一的SSC患者中观察到所谓的食道硬皮病。SSC患者的食道运动功能表现出异质性。食道动力障碍降低SSc患者的HRQOL。
BACKGROUND & AIMS: Systemic scleroderma (SSc) is associated with esophageal aperistalsis and hypotensive esophagogastric junction pressure, although there could be a gradation in esophageal motor dysfunction. We characterized esophageal motor function by high-resolution esophageal manometry (HRM) and assessed associations between SSc severity, health-related quality of life (HRQOL), and HRM findings in patients.METHODS: We performed a prospective study of 200 patients with SSc and 102 patients without SSc (controls) who underwent HRM at Mayo Clinic Arizona from May 2006 through January 2015. We used data on integrated relaxation pressure, distal contractile integral, and distal latency to classify esophageal motility disorders according to the Chicago Classification v 3.0. A subset of subjects (n = 122) completed SSc-specific gastrointestinal symptom and HRQOL questionnaires. HRM findings, symptoms, and HRQOL data were compared among diffuse SSc, limited SSc, and control subjects. Categorical variables were compared by using the chi(2) or Fisher exact test; continuous variables were compared by using Mann-Whitney or Kruskal-Wallis test. Multivariable logistic regression was used to assess the association between severity of esophageal dysmotility and baseline clinical factors.RESULTS: Among patients with SSc, 83 had diffuse SSc (42%), and 117 had limited SSc (58%). Absent contractility was more frequent in patients with SSc than in controls (56% vs 13%; P < .001). HRM findings varied among the patients; absent contractility (56%) was the most frequent diagnosis, followed by normalmotility (26%) and ineffective esophagealmotility (10%). Classic scleroderma esophagus (esophagogastric junction pressure with absent contractility) was only observed in 33% of patients (34% with diffuse SSc vs 32% limited SSc) (P = .880). Severe esophageal dysmotility was associated with disease duration, interstitial lung disease, and higher gastrointestinal symptom scores (P < .001). HRQOL was decreased in patients with SSc and severe esophageal dysmotility.CONCLUSIONS: Although severe dysmotility is more common in patients with SSc than in controls, we observed the so-called scleroderma esophagus in only one- third of patients with SSc. Esophageal motor function appears to be heterogeneous in SSc. Esophageal dysmotility reduces HRQOL in patients with SSc.