抗合成酶综合征患者的人口学、临床、实验室数据、预后和治疗特征:一项国际双中心队列研究

IF 1.1 4区 医学 Q4 Medicine Archives of rheumatology Pub Date : 2022-09-01 DOI:10.46497/ArchRheumatol.2022.9108
Lila Morena Bueno Da Silva, Upendra Rathore, Vikas Agarwal, Latika Gupta, Samuel Katsuyuki Shinjo
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引用次数: 2

摘要

目的:比较印度和巴西两个不同中心抗合成酶综合征(ASSD)患者的临床、人口学、实验室数据、预后和治疗特点。患者和方法:这项国际、双中心、回顾性前瞻性队列研究于2000年1月至2020年1月在两个三级风湿病中心(一个在巴西,一个在印度)进行,共纳入115名ASSD患者(21名男性,94名女性;平均年龄;疾病诊断为40.3;范围:18至80年)。记录患者的人口学、临床和实验室资料。评估临床累及情况。结果:81例巴西人,34例印度人。与巴西患者相比,印度组在症状出现后的诊断延迟时间更长(分别为12个月和6个月;p = 0.026)。巴西患者表现出明显更高的关节和肺部受累、技工手和雷诺现象的患病率。抗jo -1抗体是两组中最常见的自身抗体。全身性动脉高血压,其次是糖尿病是最常见的合并症。关于先前使用的药物,印度患者有更多的患者接受抗疟药物治疗,而巴西组更多地使用硫唑嘌呤和静脉注射免疫球蛋白。印度患者使用一种免疫抑制药物治疗的比例较高(70.6%),而巴西组经常使用两种免疫抑制药物治疗(33%)。严重程度和预后的比较表明,巴西组复发次数较多,随访期间,两组的总体死亡率相似(巴西组为6.2%,印度组为8.8%)。结论:巴西和印度ASSD患者在发病年龄、性别分布、自身抗体等流行病学特征上具有可比性。诊断延迟见于印度患者,巴西患者表现出更高的关节和肺部受累、机械人的手、雷诺现象的患病率,复发率更高,尽管两组的死亡率似乎相似。
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Demographic, clinical, laboratory data, prognostic, and treatment features of patients with antisynthetase syndrome: An international, two-center cohort study.

Objectives: To compare clinical, demographic, laboratory data, prognostic and treatment characteristics of patients with antisynthetase syndrome (ASSD) treated in two different centers of India and Brazil.

Patients and methods: This international, two-center, retro-prospective cohort study which was conducted at two tertiary rheumatology centers (one in Brazil and one in India) between January 2000 to January 2020 included a total of 115 patients with ASSD (21 males, 94 females; mean age; at disease diagnosis at 40.3; range, 18 to 80 years). Demographic, clinical and laboratory data of the patients were recorded. Clinical involvement was evaluated.

Results: Of the patients, 81 were Brazilians and 34 were of Indian origin. The Indian group exhibited a greater delay in diagnosis after the onset of symptoms compared to Brazilian patients (12 vs. 6 months, respectively; p=0.026). Brazilian patients exhibited a significantly higher prevalence of joint and lung involvement, mechanic's hands, and Raynaud's phenomenon. Anti-Jo-1 was the most common autoantibodies in both groups. Systemic arterial hypertension, followed by diabetes mellitus were the most prevalent comorbidities. Concerning previously used drugs, the Indian patients had a larger group of patients treated with antimalarials, whereas the Brazilian group used more azathioprine and intravenous immunoglobulin. A higher proportion of Indian patients was treated with one immunosuppressive drug (70.6%), while the Brazilian group were often treated using two immunosuppressive drugs (33%). Comparison between the severity and prognosis showed that Brazilian group had a higher number of relapses, and during follow-up, the global mortality rates were similar in both groups (6.2% for Brazilian vs. 8.8% for Indian).

Conclusion: Brazilian and Indian patients with ASSD have comparable epidemiological characteristics such as age at the time of disease diagnosis, and sex distribution, and autoantibodies. Diagnostic delay is seen in Indian patients, and Brazilians exhibit a higher prevalence of joint and lung involvement, mechanic's hands, Raynaud's phenomenon with a higher number of relapses, although the mortality rate seems to be similar in both groups.

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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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