以中枢神经系统损害为首发症状的干燥综合征3例报道并文献复习

Report of 3 Cases of SjÖgren’s Syndrome with Central Nervous System Damage as the First Symptom and Review of the Literature

摘要: 目的：探讨以中枢神经系统表现为首发症状的干燥综合征的临床特点，以指导诊断、及时治疗。方法：回顾性分析3例以中枢神经系统表现为首发症状的干燥综合征患者的临床特征、诊疗情况，并结合国内外相关文献进行文献分析。结果：2例患者为中年女性，1例为青年男性。中枢神经系统受累表现分别为脊髓炎、Weber综合征、脑干病变合并脑梗死并继发性癫痫。影像学表现多为颅脑核磁FLAIR高信号及T2WI高信号。治疗上3例患者均用大剂量糖皮质激素，并分别联合吗替麦考酚酯、环磷酰胺、丙种球蛋白治疗，2例治疗后病情好转，1例病情恶化死亡。结论：对不能用其他原因解释的中枢神经系统损害，无论年龄性别均应警惕原发性干燥综合征的可能。重视病史采集及实验室指标检测，以早期诊断、及时治疗，减少患者的致残率和致死率。

Abstract: Objective: To explore the clinical characteristics of Sjögren’s syndrome with central nervous system manifestations as the first symptom to guide diagnosis and timely treatment. Methods: The clinical characteristics, diagnosis and treatment of 3 patients with Sjögren’s syndrome with central nervous system damage as the first symptom were retrospectively analyzed, and literature analysis was conducted based on relevant domestic and foreign literature. Results: Two patients were middle-aged women and one was a young man. The manifestations of central nervous system involvement include myelitis, Weber syndrome, brainstem lesions combined with cerebral infarc-tion and secondary epilepsy. Imaging manifestations are mostly high signal on cranial magnetic resonance imaging FLAIR and high signal on T2WI. All 3 patients were treated with high-dose glu-cocorticoids, combined with mycophenolate mofetil, cyclophosphamide, and immunoglobulin re-spectively. After treatment, the condition improved in two patients and worsened in one patient who ended up with death. Conclusion: For central nervous system damage that cannot be explained by other reasons, one should be alert to the possibility of primary Sjögren’s syndrome, regardless of age and gender, paying attention to medical history collection and laboratory index testing to make early diagnosis and timely treatment, to reduce the morbidity and mortality of the patients.