混合性结缔组织病（MCTD）是系统性硬化症的一个亚型吗？

IF 4.6 2区医学 Q1 RHEUMATOLOGY

Seminars in arthritis and rheumatism Pub Date : 2025-03-01 DOI:10.1016/j.semarthrit.2025.152678

Yoshiya Tanaka

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引用次数: 0

摘要

1972年，Sharp等人提出混合性结缔组织病（mixed connective tissue disease， MCTD）是一种以系统性红斑狼疮、系统性硬化症（systemic sclerosis, SSc）和多发性肌炎的临床特征重叠，以及血清抗u1 - rnp抗体高滴度为特征的疾病实体。然而，MCTD的疾病概念有时远未得到充分承认。因此，我们通过共识方法和使用典型和边缘性MCTD临床数据的评估，报告了修订的MCTD 2019诊断标准。MCTD的疾病实体及其与SSc的区别可以通过以下方面进一步强调：1)甲襞视频毛细血管镜检测的微血管病变，2)流式细胞术免疫表型分析的统计聚类，3)免疫细胞特异性基因调控，4)抗smn复合物抗体的临床相关性。

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Is mixed connective tissue disease (MCTD) a subtype of systemic sclerosis?

In 1972, mixed connective tissue disease (MCTD) was proposed by Sharp et al. as a disease entity characterized by overlapping clinical features of systemic lupus erythematosus, systemic sclerosis (SSc), and polymyositis, as well as high titers of serum anti-U1-RNP antibody. However, the disease concept of MCTD is sometimes far from being sufficiently acknowledged. We, therefore, reported the revised diagnostic criteria for MCTD 2019 by consensus method and evaluation using clinical data of typical and borderline cases of MCTD. The disease entity of MCTD and its difference from SSc can be further emphasized by 1) microvasculopathy detected using nailfold videocapillaroscopy, 2) statistical clustering based on immunophenotypic analysis using flow cytometry, 3) immune cell-specific gene regulation, 4) clinical relevance of anti-SMN complex antibodies.

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来源期刊

Seminars in arthritis and rheumatism 医学-风湿病学

CiteScore

9.20

自引率

4.00%

发文量

176

审稿时长

46 days

期刊介绍： Seminars in Arthritis and Rheumatism provides access to the highest-quality clinical, therapeutic and translational research about arthritis, rheumatology and musculoskeletal disorders that affect the joints and connective tissue. Each bimonthly issue includes articles giving you the latest diagnostic criteria, consensus statements, systematic reviews and meta-analyses as well as clinical and translational research studies. Read this journal for the latest groundbreaking research and to gain insights from scientists and clinicians on the management and treatment of musculoskeletal and autoimmune rheumatologic diseases. The journal is of interest to rheumatologists, orthopedic surgeons, internal medicine physicians, immunologists and specialists in bone and mineral metabolism.