視神經脊髓炎譜系疾病合并干燥綜合征臨床病例研究

[摘要] 目的 探討視神經脊髓炎譜系疾病合并干燥綜合征的臨床表現、影像特點、血中相關抗體和腦脊液、致殘程度的特點。方法 回顧性分析12例2008年5月—2015年9月住院的符合診斷標準的視神經脊髓炎譜系疾病合并干燥綜合征患者的臨床資料。結果12例患者均為女性，年齡21～59歲，平均38.17歲，其中NMOSD發生于SS之前有4例，發生于SS之后有5例，同時被診斷有3例；12例中初發者為5例，其EDSS 3.0～6.5，平均5.2；8例患者行血AQP4檢測6例陽性，SSA陽性者12例，SSB陽性者9例，12例患者脊髓MRI檢查提示脊髓異常信號長度均大于3個椎體節段，大多數主要累及頸胸段脊髓，12例中MR檢查強化者4例，12例患者均行唇腺組織活檢符合SS診斷。結論 NMOSD合并SS是兩種各自獨立的疾病，NMOSD患者中應注意篩查干燥綜合征可能，在SS患者中，如果癥狀或影像有提示神經系統受累，亦應予以注意篩查NMOSD的可能。

[關鍵詞] 視神經脊髓炎譜系疾病；干燥綜合征；抗AQP4抗體

[中圖分類號] R5 [文獻標識碼] A [文章編號] 1674-0742（2016）12（b）-0060-03

Clinical Analysis of Neuromyelitis Optica Spectrum Disorders with Primary Sjogrens’s Syndrome

LI Jun-yi1，LIANG Hui1，CUI Xiao-ping2，WANG Tian-hong3

1.the Affiliated People's Hospital of Fujian University of Traditional Chinese Medicine， Fuzhou， Fujian Province， 350000 China；2.Department of Neurology， Fuzhou General Hospital， Fuzhou， Fujian Province， 350000 China；3.Department of Neurology， First Hospital Affiliated to Lanzhou University， Lanzhou，Gansu Province ， 730000 China

[Abstract] Objective To investigate the clinical features of NMOSD with Sjogren’s syndrome. Methods 12 inpatients（from May 2008 to September 2015） met with the criteria of NMOSD with Sjogren’s syndrome were retrospectively analyzed. Results 12 patients were females， age 21～59 years old（mean 38.17 ）， 4 of 12 patients had NMOSD attack before Sjogren’s syndrome，5 patients had Sjogren’s syndrome before NMOSD ， NMOSD and SS were diagnosed simultaneously in 3 patients； 5 of 12 patients had first NMOSD attack； EDSS 3.0～6.5（ mean 5.2）； Serum anti-AQP4-Ab were positive in 6 of 8 patients tested， SSA-Ab were positive in all 12 inpatients and SSB-Ab were positive in 9 patients； 12 patients had spinal cord involved extending morn than 3 s vertebral segment ， cervical and/or thoracic cord were involved in most of patients， 4 patients had enhanced foci on MR， biopsy of labial gland in 12 patients fulfill SS. Conclusion NMOSD and SS were distinct disorders； Screen to comitant SS were suggested in NMOSD patients ，and Screen to comitant NMOSD were suggested in SS patients who had clinical presentation or imaging of CNS involved

[Key words] Neuromyelitis optica spectrum disorders；Primary Sjogren’s；Syndrome anti-AQP4 antibody

原發性干燥綜合征是一種主要累及外分泌腺的慢性炎癥性自身免疫病，外分泌腺局灶淋巴細胞浸潤，既往累及神經系統疾病往往認為是繼發于SS的血管炎等所致。視神經脊髓炎譜系疾病是一組主要累及視神經和脊髓的中樞神經系統脫髓鞘疾病，該組疾病抗AQP4抗體陽性率高，易復發，多次復發后致殘率高。近年來，隨著相關抗體檢測技術提高，病理活檢，影像學技術進步，臨床上發現兩者合并出現的病例并不少見，該研究收集2008年5月—2015年9月在福建中醫藥……