Qiao Lin, Wang Qian, Fei Yunyun, Zhang Wen, Xu Yan, Zhang Yao, Zhao Yan, Zeng Xiaofeng, Zhang Fengchun
From the Department of Rheumatology and Clinical Immunology(LQ,WQ,YF,WZ,Yan Zhao,XZ,FZ); and Department of Neurology(YX,Yao Zhang), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Medicine (Baltimore). 2015 Jul;94(28):e1145. doi: 10.1097/MD.0000000000001145.
The aim of the present study was to analyze the clinical characteristics of primary Sjogren's syndrome (pSS) with neuromyelitis optica spectrum disorder (NMOSD). We retrospectively reviewed the medical records of 616 patients who were admitted to the Peking Union Medical College Hospital from 1985 to 2013. Of these patients, 43 developed NMOSD. The median duration of symptoms was 60 months and 72% of the patients experienced neurological complications onset in the pSS with NMOSD group. Twenty-one out of 43 patients had neuromyelitis optica (NMO), and 22 exhibited a limited form of NMO. Serum anti-aquaporin-4 (AQP4) antibody positivity was detected in 89.3% of the patients. A total of 60.5% of the patients (26 patients) complained of dry mouth, 72.1% were positive for objective xerostomia, 53.5% complained of dry eyes, and 74.4% had a positive ocular test. Biopsy of the minor salivary glands was performed in 33 patients, 28 of whom (84.8%) had a lymphocytic focus score of ≥1. Anti-Ro/SSA or anti-La/SSB antibodies were detected in 41 patients (95.3%). Compared with the pSS patients without NMOSD, the incidences of xerophthalmia, xerostomia, arthritis, interstitial lung disease, and renal tubular acidosis were significantly lower in the patients with NMOSD. NMOSD is a neurologic complication of pSS. The presence of anti-AQP4 antibody may be a predictor for pSS patients with NMOSD. Neurological manifestations are prominent in these patients. In clinical scenarios involving pSS or NMOSD, rheumatologists and neurologists should be aware of this association and perform the appropriate tests.
本研究的目的是分析原发性干燥综合征（pSS）合并视神经脊髓炎谱系障碍（NMOSD）的临床特征。我们回顾性分析了1985年至2013年在北京协和医院住院的616例患者的病历。其中，43例发生了NMOSD。症状的中位持续时间为60个月，72%的患者在pSS合并NMOSD组中出现神经并发症。43例患者中有21例患有视神经脊髓炎（NMO），22例表现为局限性NMO。89.3%的患者血清抗水通道蛋白4（AQP4）抗体呈阳性。共有60.5%的患者（26例）主诉口干，72.1%的患者客观存在口干，53.5%的患者主诉眼干，74.4%的患者眼部检查呈阳性。对33例患者进行了小唾液腺活检，其中28例（84.8%）淋巴细胞灶性评分≥1。41例患者（95.3%）检测到抗Ro/SSA或抗La/SSB抗体。与无NMOSD的pSS患者相比，NMOSD患者干眼症、口干症、关节炎、间质性肺病和肾小管酸中毒的发生率显著降低。NMOSD是pSS的一种神经并发症。抗AQP4抗体的存在可能是pSS合并NMOSD患者的一个预测指标。这些患者的神经表现较为突出。在涉及pSS或NMOSD的临床情况中，风湿病学家和神经科医生应意识到这种关联并进行适当的检查。
