Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study.

IF 1.1 4区 医学 Q4 Medicine
Archives of rheumatology Pub Date : 2022-09-20 eCollection Date: 2023-03-01 DOI:10.46497/ArchRheumatol.2023.9597
Jucier Gonçalves Júnior, Naoki Mugii, Pleiades Tiharu Inaoka, Renata Miossi, Fernando Henrique Carlos De Souza, João Calvino Soares De Oliveira, Fujiko Someya, Yasuhito Hamaguchi, Takashi Matsushita, Percival Degrava Sampaio-Barros, Samuel Katsuyuki Shinjo
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引用次数: 0

Abstract

Objectives: This study aims to describe and compare the demographic, clinical, and laboratory characteristics and follow-up of representative samples of patients with myopathies and systemic sclerosis overlap syndromes (Myo-SSc) from two tertiary centers.

Patients and methods: This is a cross-sectional and retrospective study conducted between January 2000 and December 2020. Fourty-five patients were analyzed with Myo-SSc (6 males, 39 females; mean age: 50.2±15.4 years; range, 45 to 65 years) from two tertiary centers (n=30 from Brazil and n=15 from Japan).

Results: The median follow-up was 98 (range, 37 to 168) months. Muscle impairment started simultaneously with the diagnosis of systemic sclerosis in 57.8% (26/45) of cases. Muscle involvement occurred before the onset of systemic sclerosis in 35.5% (16/45) of cases, and after in 6.7% (3/45). Polymyositis was observed in 55.6% (25/45) of cases, followed by dermatomyositis in 24.4% (11/45) and antisynthetase syndrome in 20.0% (9/45). Concerning systemic sclerosis, the diffuse and limited forms occurred in 64.4% (29/45) and 35.6% (16/45) of the cases, respectively. Comparing the subgroups, Myo or SSc onset was earlier in Brazilian patients, and they had a higher frequency of dysphagia (20/45, [66.7%]) and digital ulcers (27/45, [90%]), whereas Japanese patients had higher modified Rodnan skin scores (15 [9 to 23]) and prevalence of positive anti-centromere antibodies (4/15 [23.7%]). The current disease status and mortality were similar in both groups.

Conclusion: In the present study, Myo-SSc affected middle-aged women, and its manifestation spectrum varied according to geographic distribution.

炎症性肌病与系统性硬化重叠的患者:一项巴西-日本双中心研究。
目的:本研究旨在描述和比较来自两个三级中心的肌病和系统性硬化重叠综合征(Myo-SSc)患者的人口统计学、临床和实验室特征以及随访。患者和方法:这是一项在2000年1月至2020年12月期间进行的横断面和回顾性研究。对来自两个三级中心(巴西n=30,日本n=15)的45名Myo-SSc患者(6名男性,39名女性;平均年龄:50.2±15.4岁;范围:45-65岁)进行分析。结果:中位随访时间为98个月(范围:37-168)。57.8%(26/45)的病例在诊断为系统性硬化的同时出现肌肉损伤。35.5%(16/45)的病例在系统性硬化症发作前发生肌肉受累,6.7%(3/45)的患者在发作后发生肌肉受累。55.6%(25/45)的病例观察到多发性肌炎,其次是皮肌炎24.4%(11/45)和抗合成酶综合征20.0%(9/45)。关于系统性硬化症,弥漫型和局限型分别发生在64.4%(29/45)和35.6%(16/45)的病例中。比较这些亚组,巴西患者的Myo或SSc发病较早,吞咽困难(20/45,[66.7%])和手指溃疡(27/45,[90%])的发生率较高,而日本患者的改良Rodnan皮肤评分(15[9-23])和抗着丝粒抗体阳性率(4/15[23.7%])较高。两组目前的疾病状况和死亡率相似。结论:在本研究中,Myo-SSc影响中年妇女,其表现谱因地理分布而异。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Archives of rheumatology
Archives of rheumatology Medicine-Rheumatology
CiteScore
2.00
自引率
9.10%
发文量
15
期刊介绍: The Archives of Rheumatology is an official journal of the Turkish League Against Rheumatism (TLAR) and is published quarterly in March, June, September, and December. It publishes original work on all aspects of rheumatology and disorders of the musculoskeletal system. The priority of the Archives of Rheumatology is to publish high-quality original research articles, especially in inflammatory rheumatic disorders. In addition to research articles, brief reports, reviews, editorials, letters to the editor can also be published. It is an independent peer-reviewed international journal printed in English. Manuscripts are refereed by a "double-blind peer-reviewed" process for both referees and authors. Editorial Board of the Archives of Rheumatology works under the principles of The World Association of Medical Editors (WAME), the International Council of Medical Journal Editors (ICMJE), and Committee on Publication Ethics (COPE).
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