Department of Dermatology, University of Fukui, Fukui, Japan.
Department of Dermatology, Graduate School of Medicine, Osaka University, Osaka, Japan.
J Dermatol. 2024 Oct;51(10):1290-1297. doi: 10.1111/1346-8138.17403. Epub 2024 Sep 5.
As the clinical course of systemic sclerosis (SSc) varies widely, prognostic indicators have been sought to predict the outcomes of individual patients. Racial differences in SSc render it necessary to validate prognostic indicators in different patient cohorts. In this study, we aimed to assess clinical and laboratory parameters in Japanese patients with early-stage SSc with diffuse cutaneous involvement and/or interstitial lung disease, and identify predictive factors for disease progression. We performed multivariate analyses of baseline clinical information to estimate symptoms 4 years later in Japanese patients with diffuse cutaneous SSc and/or SSc with interstitial lung disease. Patients were enrolled in the study within 5 years of disease onset at 10 Japanese SSc centers. Over 12 years, 115 patients followed up for 4 years were included in this study. The modified Rodnan skin score (mRSS) at 4 years correlated with the baseline mRSS and finger-to-palm distance, defined as the average length from the distal tip of the fourth finger to the distal palmar crease. The percentage predicted vital capacity (%VC) in year 4 positively and negatively correlated with initial %VC and the presence of anti-topoisomerase I antibodies, respectively. The Health Assessment Questionnaire Disability Index (HAQ-DI) at 4 years was positively and negatively associated with baseline HAQ-DI and %VC, respectively. The occurrence of digital ulcers within 4 years was associated with the initial presence of digital ulcers, finger-to-palm distance, and the presence of digital pitting scars and anti-topoisomerase I antibodies. This study identified several factors that may predict the progression of early-stage SSc in Japanese patients. Finger-to-palm distance may be a useful tool for predicting the progression of skin thickening and the development of digital ulcers in the early stages of severe SSc, but larger, long-term prospective studies are needed to confirm our findings.
系统性硬化症（SSc）的临床病程差异很大，因此一直在寻找预测个体患者结局的预后指标。由于 SSc 在不同种族中的差异，有必要在不同的患者队列中验证预后指标。在这项研究中，我们旨在评估患有弥漫性皮肤受累和/或间质性肺病的早期弥漫性皮肤 SSc 的日本患者的临床和实验室参数，并确定疾病进展的预测因素。我们对基线临床信息进行了多变量分析，以估计弥漫性皮肤 SSc 和/或 SSc 伴间质性肺病患者 4 年后的症状。患者在发病后 5 年内，在日本的 10 个 SSc 中心入组本研究。经过 12 年的时间，共有 115 名患者在发病后 4 年接受了随访，被纳入本研究。4 年后的改良 Rodnan 皮肤评分（mRSS）与基线 mRSS 和指掌距离相关，指掌距离定义为从第四指远端指尖到远端掌纹的平均长度。4 年后的预计肺活量百分比（%VC）与初始 %VC 和抗拓扑异构酶 I 抗体的存在呈正相关和负相关。4 年后的健康评估问卷残疾指数（HAQ-DI）与基线 HAQ-DI 和 %VC 呈正相关和负相关。4 年内发生的手指溃疡与初始手指溃疡、指掌距离以及手指凹陷性瘢痕和抗拓扑异构酶 I 抗体的存在相关。本研究确定了一些可能预测日本患者早期 SSc 进展的因素。指掌距离可能是预测早期严重 SSc 皮肤增厚进展和手指溃疡发生的有用工具，但需要更大规模的长期前瞻性研究来证实我们的发现。
