Alcala-Gonzalez Luis G, Del-Castillo Alfredo Guillen-, Aguilar Ariadna, Barber Claudia, Malagelada Carolina, Polo Figueras Laura, Triginer Laura, Codina-Clavaguera Claudia, Hughes Michael, Serra Jordi, Simeón-Aznar Carmen P, McMahan Zsuzsanna H
Digestive System Research Unit, Department of Digestive Diseases, Vall d'Hebron University Hospital, Barcelona, Spain.
Systemic Autoimmune Diseases Unit, Internal Medicine Department, Vall d'Hebron University Hospital, Barcelona, Spain.
Rheumatology (Oxford). 2025 Aug 9. doi: 10.1093/rheumatology/keaf433.
Esophageal dysmotility is a common manifestation of SSc, contributing to substantial morbidity. We sought to determine whether esophageal dysmotility patterns, by high-resolution esophageal manometry were associated with distinct SSc clinical phenotypes and different outcomes.
We analyzed a cohort of SSc patients with detailed clinical and immunological data. Esophageal motility was classified using Chicago 4.0 criteria, and baseline characteristics were compared across motility patterns [Absent Contractility(AC), Ineffective Esophageal Motility(IEM), and Normal Motility]. Associations with adverse outcomes(death or lung transplantation) were evaluated using Kaplan-Meier and Cox regression analyses.
Our cohort included 201 patients with SSc(84% female, mean age 45±17 years, follow-up 442 person-years). Esophageal dysmotility patterns were classified as AC in 86(43%), IEM in 57(28%), and normal motility in 58(29%). AC was associated with dcSSc, more severe digital ulcers, gastric vascular ectasia, anti-Ro60 antibodies, and a late pattern on nailfold capillaroscopy (p < 0.05), while IEM was linked to limited SSc, anti-centromere antibodies, and an early/active nailfold pattern. Multivariate time-to-event analysis identified AC as an independent risk factor for lung transplantation(HR = 7.004, 95%CI: 1.481-33.135, p = 0.014) after adjusting for both interstitial lung disease and male sex, and for SSc-related death(HR = 3.472, 95%CI: 1.071-10.969, p = 0.038) after adjusting for diffuse cutaneous SSc and interstitial lung disease.
We found that patients with AC and IEM have distinct clinical phenotypes, suggesting they are distinct entities. In patients with SSc, AC is independently associated with worse outcomes. These data suggest that HREM may be useful in risk stratification and outcome predictions in patients with SSc.
食管动力障碍是系统性硬化症（SSc）的常见表现，会导致严重的发病情况。我们试图确定高分辨率食管测压所显示的食管动力障碍模式是否与不同的SSc临床表型及不同的预后相关。
我们分析了一组具有详细临床和免疫学数据的SSc患者。食管动力根据芝加哥4.0标准进行分类，并比较不同动力模式（无收缩功能（AC）、食管动力无效（IEM）和正常动力）的基线特征。使用Kaplan-Meier和Cox回归分析评估与不良结局（死亡或肺移植）的相关性。
我们的队列包括201例SSc患者（84%为女性，平均年龄45±17岁，随访442人年）。食管动力障碍模式分类为AC的有86例（43%），IEM的有57例（28%），正常动力的有58例（29%）。AC与弥漫性皮肤型SSc（dcSSc）、更严重的指端溃疡、胃血管扩张、抗Ro60抗体以及甲襞毛细血管镜检查的晚期模式相关（p<0.05），而IEM与局限性SSc、抗着丝点抗体以及甲襞早期/活动期模式相关。多因素事件发生时间分析确定，在调整间质性肺病和男性因素后，AC是肺移植的独立危险因素（风险比（HR）=7.004，95%置信区间（CI）：1.481-33.135，p=0.014），在调整弥漫性皮肤型SSc和间质性肺病后，AC是SSc相关死亡的独立危险因素（HR=3.472，95%CI：1.071-10.969，p=0.038）。
我们发现AC和IEM患者具有不同的临床表型，表明它们是不同的实体。在SSc患者中，AC与更差的预后独立相关。这些数据表明，高分辨率食管测压可能有助于SSc患者的风险分层和预后预测。
