系统性硬化病并发三叉神经病变五例临床分析并文献复习

Trigeminal neuropathy as a complication of systemic sclerosis:case reports and literature review

目的分析SSc合并三叉神经病变(TN)的临床特点、免疫学异常及预后,提高临床医师对SSc并发TN的认识.方法对2009年1月至2019年7月在北京大学人民医院确诊为SSc合并TN的5例患者临床资料进行回顾性分析.结果本研究5例及文献报道8例患者中,男性2例,女性11例,平均年龄(50±9)岁.TN可早于、同时或在SSc确诊之后出现.以雷诺现象(100%,13/13)、肺部受累(75%,9/12)及食管功能异常(60%,6/10)为主要表现.12例患者行瞬目反射试验,10例阳性.92%(11/12)血清ANA阳性,40%(4/10)抗Scl-70抗体阳性,36%(4/11)抗RNP抗体阳性,43%(3/7)患者ESR升高,57%(4/7)患者血清免疫球蛋白升高.主要采用糖皮质激素联合免疫抑制剂治疗原发病,神经系统症状逐渐改善,无进展.结论SSc病情活动时出现TN,且易合并雷诺现象、肺部受累及食管功能异常,伴有TN的SSc患者血清ANA、抗Scl-70抗体和抗RNP抗体阳性率高,瞬目反射试验可以辅助SSc检测三叉神经感觉支病变.积极治疗原发病可以控制疾病的进展.

Objective Our study aimed to investigate clinical and immunological features,and prognosis of systemic sclerosis(SSc)complicated with trigeminal neuropathy(TN).Methods Data of SSc-associated TN were collected from clinical records in Peking University People's Hospital between January 2009 and July 2019.Results Five cases collected from our cohort and 8 cases from literature.Out of 13 cases,11 were females,with the average age of(50±9)years.TN occurred before,concurrent,or after SSc.Raynaud's phenomenon(100%,13/13),lung involvement(75%,9/12)and esophageal dysfunction(60%,6/10)were key clinical features of SSc-associated TN patients.Twelve cases received blink reflex tests and 10 of them had positive results.In SSc-associated TN patients,ANA was detected in 92%(11/12)of cases,anti-Scl-70 antibody was detected in 40%(4/10)of cases,and anti-RNP antibody was detected in 36%(4/11)of patients.Elevated ESR and immunoglobulin were found in 43%(3/7)of patients,and 57%(4/7)of patients,respectively.Symptoms of nervous system gradually improved after treated with glucocorticoid and immuno-suppressants.Conclusion TN is identified as a complication of SSc.SSc patients with Raynaud's pheno-menon,lung involvement,esophageal dysfunction,positive ANA,anti-Scl-70 antibody and anti-RNP antibody are prone to develop TN and should receive more effective intervention.Blink reflex test is useful for patients with SSc-associated trigeminal sensory neuropathy.