Predictors of clinical features in early-onset severe systemic sclerosis: An analysis from a multicenter prospective observational Japanese cohort

IF 2.9 3区 医学 Q2 DERMATOLOGY Journal of Dermatology Pub Date : 2024-09-05 DOI:10.1111/1346-8138.17403
Saori Uesugi-Uchida, Manabu Fujimoto, Yoshihide Asano, Hirahito Endo, Daisuke Goto, Masatoshi Jinnin, Yasushi Kawaguchi, Sumiaki Tanaka, Takahiro Tokunaga, Katsunari Makino, Takashi Matsushita, Sei-Ichiro Motegi, Ayumi Yoshizaki, Shinichi Sato, Minoru Hasegawa
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Abstract

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.

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早期重度系统性硬化症临床特征的预测因素:日本多中心前瞻性观察队列分析。
由于系统性硬化症(SSc)的临床过程千差万别,人们一直在寻找预后指标来预测个体患者的预后。由于系统性硬化症的种族差异,有必要在不同的患者群体中验证预后指标。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 SSc 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伴间质性肺病的日本患者4年后的症状。日本 10 家 SSc 中心在患者发病 5 年内将其纳入研究。在 12 年的时间里,本研究共纳入了 115 名随访 4 年的患者。4 年后的改良罗德南皮肤评分(mRSS)与基线 mRSS 和指掌距离相关,指掌距离是指从四指远端到远端掌皱襞的平均长度。第 4 年的预测生命容量百分比(%VC)分别与初始生命容量百分比和抗拓扑异构酶 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)分别与基线HAQ-DI和%VC呈正相关和负相关。4 年内出现的数字溃疡与最初出现的数字溃疡、手指到手掌的距离、数字点状疤痕和抗拓扑异构酶 I 抗体有关。这项研究发现了一些可预测日本患者早期 SSc 病情发展的因素。手指到手掌的距离可能是预测严重 SSc 早期皮肤增厚进展和出现数字溃疡的有用工具,但还需要更大规模的长期前瞻性研究来证实我们的发现。
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来源期刊
Journal of Dermatology
Journal of Dermatology 医学-皮肤病学
CiteScore
4.60
自引率
9.70%
发文量
368
审稿时长
4-8 weeks
期刊介绍: The Journal of Dermatology is the official peer-reviewed publication of the Japanese Dermatological Association and the Asian Dermatological Association. The journal aims to provide a forum for the exchange of information about new and significant research in dermatology and to promote the discipline of dermatology in Japan and throughout the world. Research articles are supplemented by reviews, theoretical articles, special features, commentaries, book reviews and proceedings of workshops and conferences. Preliminary or short reports and letters to the editor of two printed pages or less will be published as soon as possible. Papers in all fields of dermatology will be considered.
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Issue Information Issue Information Correction to “dietary habits in Japanese patients with palmoplantar pustulosis” Correction to “Ozenoxacin suppresses sebum production by inhibiting mTORC1 activation in differentiated hamster sebocytes” Golden palms: Xanthoma striatum palmare: An illustrative image of an uncommon finding in a quotidian setting
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