Alexander E L, Lijewski J E, Jerdan M S, Alexander G E
Arthritis Rheum. 1986 Oct;29(10):1223-31. doi: 10.1002/art.1780291007.
The pathogenesis of central nervous system complications in primary Sjögren's syndrome (CNS-SS) is unknown. In order to determine whether patients with active CNS-SS have cerebrospinal fluid (CSF) abnormalities indicative of CNS inflammation, CSF analyses from 30 patients with active CNS-SS (SSA) were contrasted with those from 20 SS patients without CNS involvement (SSI) and 20 patients with systemic lupus erythematosus and active CNS disease (SLEA). Elevations of total protein concentration, IgG concentration, IgG to total protein ratio, and IgG index were observed in patients with SSA, but not in those with SSI. Agarose gel electrophoresis results were abnormal, with 1 or more bands, in 25 of 29 SSA patients (86%), but in only 3 of 18 SSI patients (17%). Similar, but less striking, CSF abnormalities were seen in a minority of SLEA patients. Fifteen SSA patients (50%) had transient, mild-to-moderate CSF pleocytosis, while only 1 SSI patient and 2 SLEA patients had similar findings. Cytologic findings were abnormal in 18 SSA patients (60%); these included atypical mononuclear cells, lymphoblastoid cells, and plasma cells. The presence of immunocompetent cells and evidence for the intrathecal synthesis of IgG within the CSF of SSA, but not SSI, patients provide diagnostic parameters which are indicative of active disease and which can be monitored serially during therapy.
原发性干燥综合征中枢神经系统并发症（CNS - SS）的发病机制尚不清楚。为了确定活动性CNS - SS患者是否存在提示中枢神经系统炎症的脑脊液（CSF）异常，对30例活动性CNS - SS（SSA）患者的脑脊液分析结果与20例无中枢神经系统受累的干燥综合征患者（SSI）以及20例患有系统性红斑狼疮且有活动性中枢神经系统疾病（SLEA）患者的结果进行了对比。SSA患者的总蛋白浓度、IgG浓度、IgG与总蛋白比值以及IgG指数均升高，而SSI患者则未出现这种情况。29例SSA患者中有25例（86%）的琼脂糖凝胶电泳结果异常，出现1条或更多条带，而18例SSI患者中只有3例（17%）异常。少数SLEA患者也出现了类似但不太明显的脑脊液异常。15例SSA患者（50%）出现短暂的轻至中度脑脊液细胞增多，而只有1例SSI患者和2例SLEA患者有类似表现。18例SSA患者（60%）的细胞学检查结果异常；这些异常包括非典型单核细胞、淋巴母细胞样细胞和浆细胞。SSA患者而非SSI患者的脑脊液中存在免疫活性细胞以及鞘内合成IgG的证据，这些提供了诊断参数，可指示疾病的活动性，并可在治疗期间进行连续监测。
