Objectives Systemic sclerosis (SSc) is heterogeneous in its clinical presentation. Common manifestations cluster together, defining unique subgroups. This investigation aims to characterize gastrointestinal (GI) phenotypes and determine whether they can be distinguished by temporal progression. Methods We examined a well-established SSc patient cohort with a modified Medsger GI severity score measured over time to determine heterogeneity in disease progression. Growth mixture models estimated each patient's phenotype and disease severity trajectory over time. We compared the characteristics of estimated phenotypes using non-parametric statistics and linear and logistic regression to compare patient characteristics between phenotypes while adjusting for disease duration. Results We examined 2696 SSc patients with at least two Medsger GI scores, identifying four unique phenotypes. The most common phenotype (‘Stable’, n = 2325) had an average score of 1 that was consistent over time. Two phenotypes were progressive (‘Early Progressive’, n = 142, and ‘Late Progressive’, n = 115) with an initial average score of 1. The Early Progressive group increased initially and stabilized, and the Late Progressive group worsened slowly over time. A fourth phenotype (‘Early Severe GI’, n = 114) had an initial average Medsger GI score just below 3 with high mortality and improving GI severity over time. Conclusions Clinically distinct GI phenotypes exist among patients with SSc. These phenotypes are not only distinguished by GI and extra-intestinal SSc clinical complications, but they are also temporally distinct. Distinct autoantibody profiles are associated strongly with more severe GI disease.

中文翻译：

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系统性硬化症患者胃肠道进展的不同临床轨迹


目的 系统性硬化症 （SSc） 的临床表现具有异质性。常见的表现形式聚集在一起，定义了唯一的子组。本研究旨在表征胃肠道 （GI） 表型并确定它们是否可以通过时间进展来区分。方法 我们检查了一个成熟的 SSc 患者队列，随着时间的推移测量了改良的 Medsger GI 严重程度评分，以确定疾病进展的异质性。生长混合模型估计了每个患者的表型和疾病严重程度随时间的变化轨迹。我们使用非参数统计以及线性和 logistic 回归比较估计表型的特征，以比较不同表型的患者特征，同时调整疾病持续时间。结果 我们检查了 2696 例至少有 2 个 Medsger GI 评分的 SSc 患者，确定了 4 个独特的表型。最常见的表型 （'稳定'，n = 2325） 的平均评分为 1，随着时间的推移是一致的。两种表型是进行性的 （'Early Progressive'， n = 142， and 'Late Progressive'， n = 115），初始平均评分为 1。Early Progressive 组最初增加并稳定，而 Late Progressive 组随着时间的推移缓慢恶化。第四种表型 （'早期重度 GI'，n = 114） 的初始平均 Medsger GI 评分略低于 3，死亡率高，并且 GI 严重程度随着时间的推移而改善。结论 SSc 患者临床上存在不同的 GI 表型。这些表型不仅以胃肠道和肠外 SSc 临床并发症为特征，而且它们在时间上也是不同的。不同的自身抗体谱与更严重的胃肠道疾病密切相关。