title: Comparison of clinical, immunological and neuroimaging features between anti-aquaporin-4 antibody-positive and antibody-negative Sjogren's syndrome patients with central nervous system manifestations.
abstract: OBJECTIVE
  The objective of this study is to clarify clinical, immunological, and neuroimaging features in anti-aquaporin-4 (AQP4) antibody-positive and antibody-negative Sjögren's syndrome (SS) patients with central nervous system (CNS) involvement.
  
  
  METHODS
  Medical records and MRI scans were retrospectively analyzed in 22 consecutive SS patients with CNS manifestations.
  
  
  RESULTS
  Seven (31.8%) patients were positive for anti-AQP4 antibodies. The frequency of visual impairment was higher in anti-AQP4 antibody-positive patients than in antibody-negative patients (71.4% vs. 0.0%, p = 0.0008). Brain MRI showed that discrete lesions were more commonly found in the cerebrum, brainstem, and optic nerve in anti-AQP4 antibody-positive patients than in antibody-negative patients (p = 0.002, p = 0.006, and p = 0.004, respectively), while spinal cord MRI showed that posterior column lesions in the cervical spinal cord were more frequent in anti-AQP4 antibody-positive patients than in antibody-negative patients (71.4% vs. 14.3%, p = 0.01). SS-A antibody titers were higher in anti-AQP4 antibody-positive patients than in antibody-negative patients (p = 0.012) and were also higher in patients with longitudinally extensive spinal cord lesions (LESCLs) than in those without LESCLs (p = 0.019).
  
  
  CONCLUSIONS
  In SS, the presence of anti-AQP4 antibodies is associated with involvement of the optic nerve, cerebrum and brainstem, and with cervical posterior column lesions in the spinal cord.
authors.affiliation: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Riwanti
authors.initials: R
authors.lastname: Estiasari
authors.forename: Takuya
authors.initials: T
authors.lastname: Matsushita
authors.forename: Katsuhisa
authors.initials: K
authors.lastname: Masaki
authors.forename: Takuya
authors.initials: T
authors.lastname: Akiyama
authors.forename: Tomomi
authors.initials: T
authors.lastname: Yonekawa
authors.forename: Noriko
authors.initials: N
authors.lastname: Isobe
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Kira
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D001933:Brain Stem / Q000473:pathology; D002490:Central Nervous System / Q000473:pathology*; D054022:Cerebrum / Q000473:pathology; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D007564:Japan; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging / Q000379:methods*; D009900:Optic Nerve / Q000473:pathology; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D013116:Spinal Cord / Q000473:pathology; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D001324:Autoantigens; D015415:Biomarkers