芝加哥分类4.0在硬皮病食管动力障碍中的应用：中国的一项病例对照研究

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中文摘要

背景： 系统性硬化症（SSc）是一种罕见的自身免疫性结缔组织病，以多器官纤维化和血管病变为特征，常累及食管。本研究采用高分辨率测压（HRM）评估SSc患者的食管动力障碍，并依据芝加哥分类4.0版对这些障碍进行分类，旨在探讨中国SSc患者食管功能障碍与临床表型之间的关联，同时探索可能有助于提高芝加哥v4.0方案在SSc患者中适用性的修改。
方法： 我们回顾性识别了符合2013年ACR标准、在本中心（2021-2023年）接受HRM并按芝加哥v4.0分类的SSc患者。人口学资料、器官受累、肺功能检查、超声心动图和反流症状评分（RDQ/GERDQ）从医院信息系统中提取；年龄和性别匹配的非SSc HRM受试者作为对照。通过多项logistic回归检验芝加哥分类与临床变量之间的关联。
结果： 在本研究中，76.7%的系统性硬化症患者存在食管动力障碍，主要特征为无效食管动力（IEM）（35%）和无收缩（AC）（36.67%）。AC的发生率随年龄增长而升高。胃食管反流症状在AC组中更为常见[比值比：3.23（1.02-10.22）对1.43（0.52-3.95），P<0.05，E值=2.99]。一氧化碳弥散量（DLCO）在IEM和AC中均显著降低（-2.03对-2.70，P值<0.05），其中AC降低更为明显。肺总量（TLC）和第一秒用力呼气容积（FEV1）与AC的关联更强（分别为-1.04和-0.8，p值<0.05）。直立位HRM显示，SSc患者中EGJ压力和远端收缩积分（DCI）较仰卧位下降更明显，提示食管异常在直立位检查时可能更易显现。
结论： SSc中的食管动力障碍常见，并与年龄较大、胃食管反流症状和肺功能降低显著相关。未来需要研究以阐明早期食管动力障碍是否与患者疾病进展和预后相关。

英文摘要

BACKGROUND: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by multi-organ fibrosis and vasculopathy that frequently involves the esophagus. This study assessed esophageal motility disorders in patients with SSc using High-resolution manometry (HRM) and classified these disorders according to the Chicago Classification version 4.0, aims to investigate the associations between esophageal dysfunction and clinical phenotypes in Chinese SSc patients, while exploring potential modifications to enhance the applicability of the Chicago v4.0 protocol in SSc patients.
METHODS: We retrospectively identified SSc patients fulfilling 2013 ACR criteria who underwent HRM classified by Chicago v4.0 at our center (2021-2023). Demographics, organ involvement, pulmonary-function tests, echocardiography and reflux symptom scores (RDQ/GERDQ) were extracted from the hospital information system; age and sex matched non-SSc HRM subjects served as controls. Associations between Chicago categories and clinical variables were examined by multinomial logistic regression.
RESULTS: In our study, esophageal motility disorders were present in 76.7% of patients with systemic sclerosis, predominantly characterized by ineffective esophageal motility (IEM) (35%) and absent contractility (AC) (36.67%). The occurrence rate of AC rose with age. Symptoms of gastroesophageal reflux were more common in the AC group [Odds Ratio: 3.23 (1.02-10.22) vs 1.43(0.52-3.95), P<0.05, E value=2.99]. Diffusion capacity for carbon monoxide (DLCO) was significantly reduced in both IEM and AC (-2.03 vs -2.70, P value<0.05), with a more pronounced decrease in AC. Total lung capacity (TLC) and forced expiratory volume in one second (FEV1) exhibited stronger associations with AC (-1.04 and -0.8 respectively, p value<0.05). Upright HRM revealed more marked decreases in EGJ pressure and distal contractile integral (DCI) relative to the supine position in SSc patients, indicating that esophageal abnormalities may be more readily apparent during upright examination.
CONCLUSIONS: Esophageal motility disorders in SSc were common and significantly associated with older age, gastroesophageal reflux symptoms, and reduced lung function. Future studies are needed to clarify whether early esophageal motility disorders are associated with disease progression and prognosis in patients.