Ciang Natalia C O, Pereira Nídia, Isenberg David A
Division of Rheumatology, Department of Medicine, Queen Elizabeth Hospital, Hong Kong.
Internal Medicine Department, Hospital Pedro Hispano, Matosinhos, Portugal.
Rheumatology (Oxford). 2017 Mar 1;56(3):326-333. doi: 10.1093/rheumatology/kew265.
In 1972, Sharp et al. described a new autoimmune rheumatic disease that they called MCTD, characterized by overlapping features of SSc, SLE, PM/DM, high levels of anti-U1snRNP and low steroid requirements with good prognosis. MCTD was proposed as a distinct disease. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial. We undertook a literature review, focusing on the articles reporting new data about MCTD published in the last decade, to determine whether any new observations help to answer the conundrum of MCTD. After reviewing recent data, we question whether the term MCTD is appropriately retained, preferring to use the term undifferentiated autoimmune rheumatic disease.
1972年,夏普等人描述了一种新的自身免疫性风湿性疾病,他们称之为混合性结缔组织病(MCTD),其特征为硬皮病(SSc)、系统性红斑狼疮(SLE)、多发性肌炎/皮肌炎(PM/DM)的重叠特征、抗U1核糖核蛋白(U1snRNP)水平高以及类固醇需求低且预后良好。MCTD被提出作为一种独特的疾病。然而,在最初描述后不久,关于这种综合征是否存在的问题以及对最初描述的特征的争议开始出现。MCTD是否是一种独特的疾病实体这一难题仍然存在争议。我们进行了文献综述,重点关注过去十年发表的报道有关MCTD新数据的文章,以确定是否有任何新的观察结果有助于解答MCTD之谜。在回顾了近期数据后,我们质疑MCTD这个术语是否应适当保留,更倾向于使用未分化自身免疫性风湿性疾病这一术语。