title: [Retrospective study of clinical features of neuromyelitis optica spectrum disease with connective tissue disease].
abstract: OBJECTIVE
  To explore the clinical features of neuromyelitis optica (NMO) spectrum disease (NMOSD) with connective tissue disease (CTD).
  
  
  METHODS
  The clinical features of 184 NMO/NMOSD patients (NMO/NMOSD: 119/184, 64.7%; NMO/NMOSD-CTD: 65/184, 35.3%) from May 2013 to May 2014 were analyzed retrospectively. And the effectiveness of long-term treatment of immunosuppressive drugs in NMOSD was evaluated.
  
  
  RESULTS
  NMO/NMOSD-CTD patients had significantly higher female percentage (93.8% vs 83.2%, P < 0.05) and significantly higher percentage of patients with cerebral spinal fluid (CSF)-restricted oligoclonal band (OB)(41.5% vs 21.9%, P < 0.05). As compared with NMO/NMOSD-CTD counterparts, NMO/NMOSD patients had significantly higher percentage of non-specific lesions on brain MRI (62.5% vs 35.9%, P < 0.01). After >6 months consecutive long-term treatment of immunosuppressive drugs, the relapse rate post-treatment (0.36 ± 0.85) was significantly lower than that pre-treatment (2.91 ± 4.10, P < 0.01). And no significant difference existed in expanded disability status scale (EDSS) score between pre-and post-treatment. When using azathioprine (AZA), the percentage of relapse was significantly higher in NMO/NMOSD-CTD patients (50.0%) versus NMO/NMOSD ones (18.5%, P = 0.064); When using cyclophosphamide (CTX), there was no such significant difference.
  
  
  CONCLUSIONS
  Female patients are more susceptible to have NMO/NMOSD with CTD. NMO/NMOSD-CTD patients tend to have higher percentage of CSF-restricted OB and fewer non-specific lesions on brain MRI. AZA and CTX may effectively reduce relapses in both NMO/NMOSD and NMO/NMOSD-CTD patients. However CTX is superior to AZA for reducing relapses in NMO/NMOSD-CTD patients.
authors.affiliation: Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
authors.forename: Yao
authors.initials: Y
authors.lastname: Zhang
authors.forename: Yunyun
authors.initials: Y
authors.lastname: Fei
authors.forename: Jingwen
authors.initials: J
authors.lastname: Niu
authors.forename: Haitao
authors.initials: H
authors.lastname: Ren
authors.forename: Jiuliang
authors.initials: J
authors.lastname: Zhao
authors.forename: Qian
authors.initials: Q
authors.lastname: Wang
authors.affiliation: Email: xuyanpumch@hotmail.com.
authors.forename: Yan
authors.initials: Y
authors.lastname: Xu
mesh_terms: D051401:Aquaporin 4; D003240:Connective Tissue Diseases; D003520:Cyclophosphamide; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009471:Neuromyelitis Optica; D012008:Recurrence; D012189:Retrospective Studies
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D003520:Cyclophosphamide