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混合结缔组织疾病(MCTD:mixed con-nective tissue disease)是伴有系统性红斑狼疮(SLE),全身性硬皮症(PSS),多发性肌炎(PM)的症状,是具有对核糖核蛋白(RNP ribonucleoprotein)的高效价抗体的疾病。典型病例有高频度的雷诺氏现象,多发性关节痛或关节炎,香肠样手指肿胀,食道运动低下,肌炎,抗核抗体阳性,高γ-球蛋白血症,类风湿因子阳性,肺扩散能力低下等。还发现30%的病人有中等程度的贫血,白细胞减少,淋巴结肿大,发烧;10%的病人有肾小球肾炎,三叉神经障碍。在幼儿的MCTD 中,有64%合并心包炎和心肌炎,42%合并重症的血小板减少症,还有误诊为幼少年类风湿性关节炎(JRA)的。本病到目前已有300例报告,约80%为女
Mixed connective tissue disease (MCTD) is a condition associated with systemic lupus erythematosus (SLE), systemic sclerosis (PSS) and polymyositis (PM) (RNP ribonucleoprotein) high titer antibody disease. Typical cases are high-frequency Raynaud’s phenomenon, multiple joint pain or arthritis, sausage-like finger swelling, low esophageal motility, myositis, antinuclear antibody positive, high gamma-globulin, rheumatoid factor positive, lung Poor diffusion ability. Also found that 30% of patients with moderate anemia, leukopenia, lymphadenopathy, fever; 10% of patients with glomerulonephritis, trigeminal disorders. In young children with MCTD, 64% had combined pericarditis and myocarditis, 42% had severe thrombocytopenia and misdiagnosed as juvenile rheumatoid arthritis (JRA). So far, 300 cases of this disease have been reported, about 80% of them are women