title: Antineutrophil cytoplasmic antibodies in patients with idiopathic inflammatory-demyelinating diseases.
abstract: OBJECTIVE
  We assessed the clinical significance of antineutrophil cytoplasm antibodies (ANCA) in patients with idiopathic inflammatory-demyelinating disease (IIDD).
  
  
  METHODS
  A consecutive cohort of 269 subjects with IIDD and 595 controls was analyzed retrospectively.
  
  
  RESULTS
  Among all subjects, ANCA positivity rates were low [9.5% in a perinuclear pattern (pANCA) and 2.3% in a cytoplasmic pattern (cANCA)]. One of the 117 patients with multiple sclerosis (MS) had cANCA and 2 had pANCA. Ten patients with neuromyelitis optica (NMO; 13.9%) had pANCA and 3 (4.2%) had cANCA. Four patients with recurrent longitudinal extensive transverse myelitis (RLETM; 19.0%) had pANCA and 1 (4.8%) had cANCA. In monophasic TM, 22.9% were pANCA seropositive. Among patients with brainstem syndromes, 14.3% were pANCA seropositive. Patients with NMO, RLETM or monophasic TM had higher pANCA levels than patients with MS. There was a positive association between spinal cord (SC) lesions and ANCA, and especially between longitudinal extensive transverse myelitis and ANCA. Among anti-aquaporin 4 antibody-positive patients, ANCA-positive patients (n = 16) were older and had higher Expanded Disability Status Scale scores, more antinuclear antibodies, longer SC lesions and fewer brain abnormalities than the ANCA-negative patients (n = 68). In the NMO subgroup, ANCA-positive patients were older and had more antinuclear antibodies and longer SC lesions than ANCA-negative patients.
  
  
  CONCLUSIONS
  Among the IIDDs, we found a higher occurrence of ANCA in patients with NMO spectrum disorders than in patients with MS. Therefore, ANCA is another interesting marker of autoimmunity in IIDD patients, especially those with anti-aquaporin 4 antibody.
authors.affiliation: Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China, Institute of Neuroscience and The Second Affiliated Hospital of GuangZhou Medical University, and Department of Neurology, The Second Affiliated Hospital of GuangZhou Medical University, GuangZhou, PR China.
authors.forename: Youming
authors.initials: Y
authors.lastname: Long
authors.forename: Yangbo
authors.initials: Y
authors.lastname: Zheng
authors.forename: Mengyu
authors.initials: M
authors.lastname: Chen
authors.forename: Bin
authors.initials: B
authors.lastname: Zhang
authors.forename: Cong
authors.initials: C
authors.lastname: Gao
authors.forename: Qingchun
authors.initials: Q
authors.lastname: Gao
authors.forename: Jian-Rui
authors.initials: JR
authors.lastname: Yin
authors.forename: Shuxiang
authors.initials: S
authors.lastname: Pu
authors.forename: Changlong
authors.initials: C
authors.lastname: Xie
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D051401:Aquaporin 4 / Q000276:immunology; D001933:Brain Stem / Q000473:pathology; D002648:Child; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000097:blood* / Q000276:immunology; D009188:Myelitis, Transverse / Q000097:blood* / Q000276:immunology; D009471:Neuromyelitis Optica / Q000097:blood* / Q000276:immunology; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D051401:Aquaporin 4