摘 要

        背景：系统性硬化症(SSc)是未知病因的影响身体结缔组织的疾病。它可以侵犯皮肤和各脏器。在近90%的患者中观察到胃肠道受累，其中食道为最常受累部位。食管运动障碍通常表现为食管下端括约肌压力降低(LESP)和远端食管蠕动下降。通常SSc患者食管受累比较复杂，可能存在侵蚀性食管炎、Barrett食管和食管腺癌。局限性硬皮病，其特征主要表现为皮肤受累，偶尔侵袭肌肉，通常不会累及内脏。局限性硬皮病中的食管受累已经很少研究了。

        本研究主要评估和比较系统性硬化症和局限性硬皮病的食管受累情况，以及是否需要进一步评估局限性硬皮病患者的上消化道情况。

        方法：56例和31例新诊断的SSc和局限性硬皮病病例进行研究。所有患者都询问消化不良症状(胃烧心和/或反酸和/或吞咽困难)。在SSc患者中分别有52、47、41例进行上消化道内窥镜检查、食管测压和24小时pH监测;在局限性硬皮病患者中分别有28、25和20例行上消化道内窥镜检查、食管测压和24小时pH监测。

        结果：SSc患者中有食管症状的39例(69.6%)，其中轻度22例(39.3%)，中度14例(25%)，严重3例(5.3%);而局限性硬皮病患者中只有4例(7.1%)有食管症状，且为轻度。反流性食管炎在SSc中有17例(32.7%)，而局限性硬皮病中仅有2例(7.14%)。 食道压力异常在SSc中有32例(68.1%)而局限性硬皮病中没有。动态24小时食管pH监测在SSc中记录了33例(80.5%)异常反流，而局限性硬皮病患者中也没有。

        结论：食管受累在SSc中较常见但在局限性硬皮病中并没有观察到。细致的上消化道评估在SSc患者中是合理的，而不适用于局灶性硬皮病。

        原 文

        Abstract

        BACKGROUND: Systemicsclerosis(SSc) is a generalized disorder of unknown etiology affecting the connectivetissue of the body. It affects the skin and various internal organs.Gastrointestinal tract involvement is seen in almost 90% of thepatients.Esophagusis the most frequently affected part of thegastrointestinal tract. Esophageal motility disturbance classically manifestsas a reduced lower esophageal sphincter pressure (LESP) and loss of distalesophageal body peristalsis. Consequently, SSc patients may be complicated byerosive esophagitis and eventually by Barrett'sesophagusandesophageal adenocarcinoma. Morphea, also known aslocalized scleroderma, ischaracterized by predominant skin involvement, with occasional involvement ofsubjacent muscles and usually sparing the internal organs. The involvementofesophagusin morphea has been studied very scarcely. The proposedstudy will investigate the esophageal involvement in the two formsofscleroderma(systemicandlocalized), compare the sameand address any need of upper gastrointestinal evaluation in morphea (localizedscleroderma) patients.

        METHODS: 56 and 31 newly and already diagnosed cases of SSc andmorphea respectively were taken up for the study. All the patients wereinquired about the dyspeptic symptoms (heartburn and/or acid regurgitationand/or dysphagia). Upper gastrointestinal endoscopy, esophageal manometry and24-hour pH monitoring were done in 52, 47 and 41 patients of SSc; and 28, 25and 20 patients of morphea respectively.

        RESULTS: Esophageal symptoms were present in39 cases (69.6%) of SSc which were mild in 22 (39.3%), moderate in 14 (25%),severe in three (5.3%); while only four cases (7.1%) of morphea had esophagealsymptoms all of which were mild in severity. Reflux esophagitis was seen in 17cases (32.7%) of SSc and only two cases (7.14%) of morphea. Manometricabnormalities were seen in 32 cases (68.1%) of SSc and none in morphea.Ambulatory 24-hour esophageal pH monitoring documented abnormal reflux in 33cases (80.5%) of SSc and no such abnormality in morphea.

        CONCLUSION: While the esophagealinvolvement is frequent in SSc, no such motility disorder is seen in morphea.Meticulous upper gastrointestinal tract evaluation is justified only in SSc andnot in morphea.

        引自：Arif T,Masood Q,Singh J,Hassan I.Assessment of esophageal involvement insystemicsclerosisand morphea (localized scleroderma) by clinical, endoscopic, manometric and pHmetric features: a prospective comparative hospital based study. BMC Gastroenterol.2015 Feb15;15:24.