目的：分析报道1例干燥综合征（Sjögren syndrome,SS）继发隐球菌性脑膜脑炎（cryptococcal meningoencephalitis, CM）患者,结合文献探讨自身免疫病继发CM的临床、病理特征及诊治方法。 方法：分析2019年上海交通大学附属第六人民医院收治的1例SS继发CM患者的临床资料,并在国内外数据库中检索相关文献,汇总分析自身免疫病继发CM病例的临床、病理特征和诊治方法。 结果：本例患者有SS病史,本次以头痛、发热及颅内压升高起病,经脑脊液墨汁染色涂片明确诊断为CM。患者经抗真菌治疗后,因疾病复发死于两性霉素B导致的药物不良反应。回顾分析文献和本例患者后发现,9%~18%的CM患者罹患有自身免疫病,且不同研究中患者的发热症状表现及发热率差异较大（33%~82%）;患者的临床表现以脑脊液压力增高引起的头痛（38%~100%）为多见。目前,CM的诊断仍依赖于脑脊液检查。联合两性霉素B和氟胞嘧啶的治疗方案疗效并不令人满意,安全性差。患者预后不佳,死亡率高（5%~42%）。 结论：包括SS在内的自身免疫病患者继发CM时,其临床表现不典型,而脑脊液压力异常增高是其特点,目前其治疗方法缺乏,患者的死亡率高、预后不佳。

Objective: To investigate a case of cryptococcal meningoencephalitis (CM) secondary to Sjögren′s syndrome (SS), and to analyze the clinical, pathological features, diagnosis and treatment of the diseases combined with literature review. Methods: Clinical data of 1 case of CM secondary to SS patient were collected, and literatures online were reviewed. Results: The main clinical manifestation consisted of headache, fever and intracranial hypertension. CM was confirmed by cerebrospinal fluid ink stain smear, but the patient still died of complication of amphotericin B treatment. Literature review indicated 9%-18% CM may had a history of autoimmune diseases. Headache because of increased cerebrospinal fluid pressure ocuured in 38%-100%. The efficacy of amphotericin B combined with fluorocytosine in recommended treatment was not satisfactory, and mortality was high (5%-42%). Conclusions: The clinical manifestation of CM secondary to autoimmune diseases is not typical, the abnormal increase of cerebrospinal fluid pressure is its characteristic. The efficacy of treatment is not satisfactory, and the mortality rate is high.