摘 要

        背景：係統性硬化症(SSc)是未知病因的影響身體結締組織的疾病。它可以侵犯皮膚和各髒器。在近90%的患者中觀察到胃腸道受累，其中食道為最常受累部位。食管運動障礙通常表現為食管下端括約肌壓力降低(LESP)和遠端食管蠕動下降。通常SSc患者食管受累比較複雜，可能存在侵蝕性食管炎、Barrett食管和食管腺癌。局限性硬皮病，其特征主要表現為皮膚受累，偶爾侵襲肌肉，通常不會累及內髒。局限性硬皮病中的食管受累已經很少研究了。

        本研究主要評估和比較係統性硬化症和局限性硬皮病的食管受累情況，以及是否需要進一步評估局限性硬皮病患者的上消化道情況。

        方法：56例和31例新診斷的SSc和局限性硬皮病病例進行研究。所有患者都詢問消化不良症狀(胃燒心和/或反酸和/或吞咽困難)。在SSc患者中分別有52、47、41例進行上消化道內窺鏡檢查、食管測壓和24小時pH監測;在局限性硬皮病患者中分別有28、25和20例行上消化道內窺鏡檢查、食管測壓和24小時pH監測。

        結果：SSc患者中有食管症狀的39例(69.6%)，其中輕度22例(39.3%)，中度14例(25%)，嚴重3例(5.3%);而局限性硬皮病患者中隻有4例(7.1%)有食管症狀，且為輕度。反流性食管炎在SSc中有17例(32.7%)，而局限性硬皮病中僅有2例(7.14%)。 食道壓力異常在SSc中有32例(68.1%)而局限性硬皮病中沒有。動態24小時食管pH監測在SSc中記錄了33例(80.5%)異常反流，而局限性硬皮病患者中也沒有。

        結論：食管受累在SSc中較常見但在局限性硬皮病中並沒有觀察到。細致的上消化道評估在SSc患者中是合理的，而不適用於局灶性硬皮病。

        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.