Faculty of Medicine, KU Leuven, Leuven, Belgium.
Division of Rheumatology, University Hospitals Leuven, Leuven, Belgium.
Clin Rheumatol. 2020 Jan;39(1):149-157. doi: 10.1007/s10067-019-04794-w. Epub 2019 Nov 11.
Systemic sclerosis (SSc) affects the upper gastrointestinal (GI) system in 90% of patients. High-resolution manometry (HRM) assesses esophageal dysmotility, but its role in diagnosis and follow-up remains unclear. The objectives of this systematic review were to investigate the role of HRM in the assessment of SSc-associated upper GI involvement and to evaluate the correlation between HRM abnormalities and clinical characteristics and the effects of therapeutic interventions on HRM findings. Fifteen articles were included. Most (11/15) studies were of very good or good quality. Most studies assessed correlations between esophageal symptoms and esophageal dysmotility. Two studies assessed the effectiveness of buspirone and reported HRM findings. Studies assessing upper GI symptoms using validated questionnaires, such as the University of California Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 or Gastrointestinal Symptoms Severity Index score, found an association between absent contractility on HRM and upper GI symptoms, but even asymptomatic patients often have esophageal body dysmotility on HRM. Esophageal dysmotility positively correlates with the presence of interstitial lung disease on high-resolution computed tomography and reduced diffusion capacity (< 0.8 of predicted value). Trials investigating the effect of buspirone demonstrate both increased lower esophageal sphincter resting pressure and reduced upper GI symptoms. Most studies report on limited patient numbers and retrospective data. Potential bias was minimized using quality appraisal. HRM findings correlate to upper GI symptoms when assessed by validated questionnaires and can detect response to therapy in buspirone trials. Esophageal body dysmotility on HRM positively correlates with the presence of interstitial lung disease. KEY POINTS: • Esophageal body dysmotility on HRM correlates with presence of ILD. • HRM findings seem to correspond to clinical symptom alleviation in interventional trials, but data are still limited. • At present HRM, a procedure with a high negative burden to the patient, offers little to no role in the therapeutic strategy.
系统性硬化症（SSc）影响 90%的患者的上胃肠道（GI）系统。高分辨率测压（HRM）评估食管动力障碍，但它在诊断和随访中的作用仍不清楚。本系统评价的目的是探讨 HRM 在评估 SSc 相关上 GI 受累中的作用，并评估 HRM 异常与临床特征的相关性以及治疗干预对 HRM 结果的影响。纳入了 15 篇文章。大多数（11/15）研究的质量为很好或良好。大多数研究评估了食管症状与食管动力障碍之间的相关性。两项研究评估了丁螺环酮的有效性，并报告了 HRM 结果。使用经过验证的问卷（如加利福尼亚大学硬皮病临床试验联盟胃肠道 2.0 或胃肠道症状严重程度评分）评估上 GI 症状的研究发现，HRM 上无收缩力与上 GI 症状之间存在关联，但即使无症状患者，HRM 上也常有食管体动力障碍。食管动力障碍与高分辨率计算机断层扫描上存在间质性肺病和弥散能力降低（<0.8 预测值）呈正相关。丁螺环酮治疗效果的试验表明，均可增加食管下括约肌静息压力并减少上 GI 症状。大多数研究报告的患者数量有限且数据为回顾性。使用质量评估最大限度地减少了潜在的偏倚。通过经过验证的问卷评估时，HRM 结果与上 GI 症状相关，并且可以在丁螺环酮试验中检测到对治疗的反应。HRM 上的食管体动力障碍与间质性肺病的存在呈正相关。要点：• HRM 上的食管体动力障碍与 ILD 的存在相关。• HRM 结果似乎与干预试验中的临床症状缓解相对应，但数据仍然有限。• 目前 HRM 对患者具有较高的阴性负担，在治疗策略中几乎没有作用。
