Rheumatology Section, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", c/o II Policlinico, via Pansini 5, 80131, Naples, Italy.
Division of General, Mini-Invasive and Bariatric Surgery, Department of Surgery, University of Campania "Luigi Vanvitelli", II Policlinico, Via Pansini 5, 80131, Naples, Italy.
Clin Rheumatol. 2018 May;37(5):1239-1247. doi: 10.1007/s10067-018-4026-1. Epub 2018 Feb 13.
This study aims to investigate pre-clinical esophageal involvement in systemic sclerosis (SSc) by high-resolution impedance manometry (HRiM), its associations with disease features including lung involvement, and its predictivity of esophageal symptoms overtime. Charts of 45 asymptomatic (no heartburn/regurgitation/dysphagia) SSc patients (96% females; mean age 46 years) with at least one follow-up (FU) visit and complete clinical, serological, functional, and radiological assessment, including high-resolution computed tomography (HRCT) of the chest and lung function tests, that had undergone esophageal HRiM were retrospectively evaluated. Esophagogastric junction-contractile integral (EGJ-CI) and esophageal body motility, as evaluated by mean distal contractile integral (DCI), were assessed. SSc patients had a normal esophageal motility in 7/45 cases, a defective EGJ-CI in 28, an ineffective esophageal motility (IEM) in 17, and aperistalsis in 12. Defective EGJ-CI was associated with IEM/aperistalsis in 20 cases, while 9 patients had isolated IEM. Defective EGJ-CI and/or IEM/aperistalsis were associated with a diffusing lung capacity for CO < 80% of predicted value (all p < 0.05), while defective EGJ-CI was also associated with interstitial lung disease on HRCT (p = 0.03). Prevalence of any HRiM abnormality was higher in anti-centromere antibody negative patients (all p < 0.05). IEM/aperistalsis independently increased the risk of esophageal symptoms by 2.3-fold (95% CI 1.1-5.7) and was associated with their higher cumulative incidence with respect to patients with other HRiM patterns at FU (χ = 4.63; p = 0.03). SSc patients asymptomatic for esophageal involvement can have HRiM abnormalities in up to 84% of cases. A baseline-impaired motility is a risk factor for symptomatic esophageal disease.
本研究旨在通过高分辨率阻抗测压(HRiM)研究系统性硬化症(SSc)的食管前临床受累情况,及其与包括肺受累在内的疾病特征的关联,并预测其随时间发展的食管症状。对 45 例无症状(无心酸/反流/吞咽困难)SSc 患者(96%为女性;平均年龄 46 岁)的图表进行了回顾性评估,这些患者至少有一次随访(FU)就诊且完成了完整的临床、血清学、功能和影像学评估,包括胸部高分辨率计算机断层扫描(HRCT)和肺功能检查,均进行了食管 HRiM。评估了食管胃交界处收缩积分(EGJ-CI)和食管体运动,通过平均远端收缩积分(DCI)进行评估。45 例 SSc 患者中有 7 例食管运动正常,28 例 EGJ-CI 受损,17 例无效食管动力(IEM),12 例蠕动不良。20 例 EGJ-CI 受损与 IEM/蠕动不良有关,9 例患者为孤立性 IEM。EGJ-CI 受损和/或 IEM/蠕动不良与弥散性肺活量低于预测值的 80%(所有 p<0.05)有关,而 EGJ-CI 受损也与 HRCT 上的间质性肺病有关(p=0.03)。抗着丝粒抗体阴性患者的任何 HRiM 异常的患病率均较高(所有 p<0.05)。IEM/蠕动不良独立使食管症状的风险增加 2.3 倍(95%CI 1.1-5.7),并且在 FU 时与其他 HRiM 模式患者相比,其食管症状的累积发生率更高(χ2=4.63;p=0.03)。无症状食管受累的 SSc 患者在高达 84%的情况下可能存在 HRiM 异常。基线运动障碍是食管疾病症状的危险因素。