Objective: The aim of present study is to analyze clinical and laboratory features of mixed connective tissue disease (MCTD)-associated trigeminal neuropathy (TN). Methods: Clinical records of 12 cases of MCTD complicated with TN diagnosed in Peking University People's Hospital from January 2008 to October 2019 were analyzed retrospectively. Results: The present study included 12 cases, 1 males and 11 females, average age was(40±13)years. TN was developed before the diagosis of MCTD in 1 case. TN and MCTD were occurred simutaneously in 1 case. Raynaud phenomenon (12 cases), arthritis (10cases), edema of fingers (9cases), myositis (6 cases), and pulmonary involvement (11cases) were main cinical feature of MCTD-associate TN. Antinuclear antibody (ANA) and high titer anti-U1-RNP antibody could be detected in serum of all patients. Elevated erythrocyte sedimentation rate (ESR) and creatine kinase were found in serum of 7cases and 5cases, respectively. Blink reflex tests were positive in 6 cases. Neurological symptoms improve slowly without any progress by using glucocorticoid combined immunosuppressants or intravenous gamma globulin. Conclusions: TN is often associted with actived MCTD. Positive ANA and anti-U1-RNP antibody were common in MCTD-associated TN. Blink reflex test is essential to diagnose MCTD-associated TN. Intensive treatment of MCTD contributes to control the progress of TN.
目的： 了解合并三叉神经病变（TN）的混合性结缔组织病（MCTD）的临床特征、免疫学改变及预后，提高临床医师对MCTD并发TN的认识。 方法： 回顾性分析2008年1月至2019年10月在北京大学人民医院确诊为MCTD合并TN的12例患者临床资料。 结果： 12例患者中男1例，女11例，年龄（40±13）岁。1例以TN首发，10例以MCTD首发，1例两者同时发病。以雷诺现象（12例）、肺部受累（11例）、关节炎（10例）、手指肿胀（9例）及肌炎（6例）为主要表现。所有患者抗核抗体（ANA）及抗U1-RNP抗体均阳性，且抗U1-RNP抗体高滴度，7例血红细胞沉降率均不同程度升高，5例肌酶升高。9例行瞬目反射试验，其中6例阳性。糖皮质激素联合免疫抑制剂或联合静脉注射人免疫球蛋白治疗原发病为主，神经系统症状改善缓慢，但无进展。 结论： MCTD病情活动时出现TN，伴有TN的MCTD中ANA和抗U1-RNP抗体阳性率高，瞬目反射试验对明确诊断MCTD合并TN十分重要，治疗原发病有助于控制疾病的进展。.
Keywords: Blink reflex; Mixed connective tissue disease; Raynaud phenomenon; Trigeminal neuropathy.