Karamanolis Georgios P, Denaxas Konstantinos, Panopoulos Stylianos, Bournia Kalliopi-Vasiliki, Zorbala Alexandra, Kamberoglou Dimitrios, Schizas Dimitrios, Ladas Spiros D, Sfikakis Petros P
Academic Department of Gastroenterology, National and Kapodistrian University of Athens, Medical School, "Laiko" Hospital, Athens, Greece.
Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):82-85. Epub 2017 Aug 30.
Severe oesophageal disease in patients with systemic sclerosis (SSc), referred as scleroderma oesophagus, is characterised by ineffective or absent peristalsis along with hypotensive oesophagogastric junction (hEGJ). The associations between scleroderma oesophagus and different clinical and laboratory manifestations of SSc is still controversial. In this study we aimed to assess associations between scleroderma oesophagus, diagnosed by high resolution manometry (HRM), and other manifestations of disease.
Fifty-four consecutive SSc patients (49 women, mean age 50.6±11.6) with oesophageal symptoms underwent clinical interview, medical records review and HRM. HRMs were analysed according to the Chicago Classification in order to provide oesophageal motility diagnosis; EGJ <9 mmHg was considered hypotensive. Demographic characteristics, patient-reported symptoms, SSc subtypes, pulmonary fibrosis, cutaneous ulcers, and anti-Scl-70 positivity were compared between SSc patients with or without scleroderma oesophagus. Comparison was also performed in computed tomography (CT) findings of oesophageal lumen in 26 patients with available data. Oesophageal dilatation was deemed present when the diameter was >9 mm.
Absent contractility was present in 37 (68.5%) patients; among these patients hEGJP was found in 32, thus 32/54 (59.2%) patients had classic scleroderma oesophagus. There were no associations with gender, age, oesophageal symptoms, skin involvement extent, anti-Scl-70, pulmonary fibrosis and cutaneous ulcers. Notably, oesophageal dilation on chest CT was more frequent in patients with scleroderma oesophagus compared to those without (77% vs. 7%, p=0.04, respectively).
Scleroderma oesophagus diagnosed by HRM was present in less than 2/3 of symptomatic patients with SSc and associated only with oesophageal dilation in CT. Although further studies are needed, oesophageal dilation on chest CT may be a non-invasive alternative for evaluation of SSc patients with oesophageal symptoms.
系统性硬化症（SSc）患者的严重食管疾病，即硬皮病食管，其特征是蠕动无效或消失，同时食管胃交界处压力降低（hEGJ）。硬皮病食管与SSc的不同临床和实验室表现之间的关联仍存在争议。在本研究中，我们旨在评估通过高分辨率测压法（HRM）诊断的硬皮病食管与疾病的其他表现之间的关联。
对54例有食管症状的连续性SSc患者（49例女性，平均年龄50.6±11.6岁）进行临床访谈、病历审查和HRM检查。根据芝加哥分类法对HRM进行分析，以提供食管动力诊断；EGJ<9 mmHg被认为是压力降低。比较有或无硬皮病食管的SSc患者的人口统计学特征、患者报告的症状、SSc亚型、肺纤维化、皮肤溃疡和抗Scl-70阳性情况。对26例有可用数据患者的食管腔计算机断层扫描（CT）结果也进行了比较。当直径>9 mm时，认为存在食管扩张。
37例（68.5%）患者存在收缩性缺失；在这些患者中，32例发现hEGJP，因此32/54（59.2%）例患者患有典型的硬皮病食管。与性别、年龄、食管症状、皮肤受累程度、抗Scl-70、肺纤维化和皮肤溃疡均无关联。值得注意的是，与无硬皮病食管的患者相比，硬皮病食管患者胸部CT上食管扩张更为常见（分别为77%对7%，p = 0.04）。
通过HRM诊断的硬皮病食管在有症状的SSc患者中不到2/3，且仅与CT上的食管扩张相关。尽管需要进一步研究，但胸部CT上的食管扩张可能是评估有食管症状的SSc患者的一种非侵入性替代方法。
