根据不同的地理区域,系统性硬化症患者的临床和血清学特征:来自两个独立队列的观察性横断面研究的见解

IF 3.4 4区 医学 Q2 RHEUMATOLOGY
Vasiliki Liakouli, Hesham Hamoud, Mahmoud Ibrahim Risha, Mohamed Elsayed Hanafy, Abdelhafeez Moshrif, Rasha A Abdel-Magied, Gihan M Omar, Adel M Elsayed, Abdelazeim Elhefny, Sameh Mobasher, Mervat Abo Gabal, Manal Hassanien, Fatma H El Nouby, Khaled Abdelgalil, Giulio Forte, Luca Navarini, Piero Ruscitti, Francesco Ciccia, Roberto Giacomelli
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引用次数: 0

摘要

目的:根据地理区域评估意大利和埃及系统性硬化症(SSc)患者的临床、血清学特征和治疗方法。方法:3个意大利中心和5个埃及中心参与了2017年的患者招募。根据先前制定的严重程度评分和活动指数收集人口统计学、临床和血清学数据并进行定义。该数据库包括261名连续的意大利患者(242名女性/19名男性)和197名埃及患者(177名女性/20名男性),所有患者均符合ACR/EULAR 2013的分类标准和LeRoy和Medsger提出的标准。结果:埃及人更年轻,首次出现非雷诺氏和雷诺氏现象的时间更早,皮肤改良评分也更严重。与意大利人相比,埃及人患有活动性肺疾病的比例更高,超声心动图估计肺动脉压为35mmHg,并且患有间质性肺疾病。埃及人的严重程度评分较高,意大利人抗拓扑异构酶I (ATA)的频率较高,埃及人抗着丝粒抗体和ATA抗体均阴性的可能性高于意大利人。埃及人使用合成抗风湿药物的比例高于意大利人;意大利人接受血管活性治疗,而埃及人没有。值得注意的是,受该疾病有限形式影响的埃及人与意大利同行相比,表现出更严重的临床病程,其特征是更高的改良罗南皮肤评分(mRSS)、更频繁的肺部受累、缺血性数字溃疡的频率增加、症状发作更早、严重程度评分更高。结论:意大利和埃及SSc患者可能存在临床差异。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical and serological features of systemic sclerosis patients, according to different geographic areas: insights from an observational, cross-sectional study of two independent cohorts.

Objectives: To evaluate the clinical, and serological features and treatments in two independent cohorts of Italian and Egyptian systemic sclerosis (SSc) patients, according to geographic areas.

Methods: 3 Italian and 5 Egyptian centres participated in patient recruitment in 2017. The demographic, clinical, and serological data were collected and defined according to the previously developed severity score and activity index. The database included 261 consecutive Italian patients (242 women/19 men) and 197 Egyptian patients (177 women/20 men), all of whom fulfilled the classification criteria of ACR/EULAR 2013 and criteria proposed by LeRoy and Medsger.

Results: Egyptians were younger, had an earlier onset of both the first non-Raynaud's and Raynaud's phenomenon and a more severe modified skin score. A greater percentage of Egyptians had the active form of the disease, a pulmonary arterial pressure estimated by echocardiography>35mmHg than Italians and interstitial lung disease. The severity score was higher in Egyptians, the frequency of anti-topoisomerase I (ATA) was higher in Italians, and Egyptians were more likely to be negative for both anticentromere and ATA antibodies than Italians. Egyptians had higher rates of synthetic disease-modifying antirheumatic drugs use than Italians; Italians but not Egyptians were under treatment with vasoactive therapy. Notably, Egyptians affected by the limited form of the disease exhibited a more severe clinical course when matched with their Italian counterparts, characterised by higher modified Rodnan skin score (mRSS), more frequent pulmonary involvement, increased frequency of ischaemic digital ulcers, earlier onset of symptoms, and higher severity scores.

Conclusions: Clinical differences may be shown between Italian and Egyptian SSc patients.

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来源期刊
CiteScore
6.10
自引率
18.90%
发文量
377
审稿时长
3-6 weeks
期刊介绍: Clinical and Experimental Rheumatology is a bi-monthly international peer-reviewed journal which has been covering all clinical, experimental and translational aspects of musculoskeletal, arthritic and connective tissue diseases since 1983.
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