文献简介

出版社:Best Practice & Research Clinical Rheumatology

作  者:Oscar-Danilo Ortega-Hernandez , Yehuda Shoenfeld

编  号:10.1016/j.berh.2012.01.009

关键字:Mixed connective tissue disease | Raynaud’s phenomenon | Erosive arthritis | Anti-RNP antibodies | Myositis | Organ involvement and treatment

年  份:2012   点击量:1002

文献摘要

The most common clinical manifestations of mixed connective disease are Raynaud’s phenomenon, arthralgias, swollen joints, esophageal dysfunction, muscle weakness and fingers sausage-like appearance together with the presence of anti-ribonucleoprotein (RNP) antibodies. However, organ involvement is more extensive than first descriptions reported. The disease can be serious with development of pulmonary, kidney, cardiovascular, gastrointestinal and central nervous system manifestations. The worst prognosis and high mortality are associated with the presence of pulmonary disease. Although a different set of clinical criteria have been proposed, there is no consensus about the most accurate. There is no full agreement about treatment and the initial impression of a satisfactory response to low doses of steroids is not always the rule. Herein, we review available evidence to a better approach to all previous topics.

混合性结缔组织病最常见的临床表现有雷诺现象、关节痛、关节肿胀、食道功能障碍、肌肉无力和手指外观香肠状,这些连同抗核糖核蛋白(RNP)抗体存在。然而,器官受累比已经报道的第一描述更为广泛。本病可出现严重的肺、肾、心血管、胃肠道和中枢神经系统症状。预后差、死亡率高与肺部疾病的存在有关。虽然我们已提出一组不同的临床标准,但无最准确的共识。对于有关治疗我们没有达成一致,以及低剂量的类固醇满意的最初印象是不规则的。在此,我们回顾了现有的证据以一种更好的方法去处理之前所有的观点。