title: Retrospective analysis of long-term outcome of chronic progressive neurological manifestations in Behcet's disease.
abstract: OBJECTIVE
  Chronic progressive neuro-Behcet's disease (CPNBD) is characterized by progressive deterioration leading to disability and death. Although methotrexate has been found effective for CPNBD, its influences on the long-term outcome remain unclear. We therefore explored the effects of various treatments on the prognosis.
  
  
  METHODS
  Thirty-seven patients, who met the international classification criteria for BD and developed chronic progressive neuropsychiatric manifestations after 1988, were followed up until October 2013. The effects of various treatments on prevention of death or severe disability of bedridden state were examined by Kaplan-Meier analysis and Cox's proportional hazard model.
  
  
  RESULTS
  Twenty-eight of 37 patients with CPNBD (75.7%) received methotrexate. Among the 28 patients, none died and only 5 patients progressed to disability with bedridden state. By contrast, among the 9 patients without methotrexate, 5 patients died and 3 patients progressed to bedridden state. Thus, methotrexate significantly improved the survival of patients with CPNBD (HR 0.0507, p=0.020) as well as reduced the rate of progression into bedridden state or death (HR 0.2082, p=0.0126), but none of high doses of steroids, azathioprine or cyclophosphamide did.
  
  
  CONCLUSIONS
  The results indicate that methotrexate, but not high doses of steroids, azathioprine or cyclophosphamide, is effective to prevent the progression of CPNBD.
authors.affiliation: Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Kanagawa, Japan. Electronic address: shunsei_tenpoint@yahoo.co.jp.
authors.forename: Shunsei
authors.initials: S
authors.lastname: Hirohata
authors.affiliation: Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Hirotoshi
authors.initials: H
authors.lastname: Kikuchi
authors.affiliation: Department of Internal Medicine 3, Tokyo Medical University, Tokyo, Japan.
authors.forename: Tetsuji
authors.initials: T
authors.lastname: Sawada
authors.affiliation: Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Nagafuchi
authors.affiliation: Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Masataka
authors.initials: M
authors.lastname: Kuwana
authors.affiliation: Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Kanagawa, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Takeno
authors.affiliation: Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Kanagawa, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ishigatsubo
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001528:Behcet Syndrome / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology / Q000503:physiopathology; D002908:Chronic Disease; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D008875:Middle Aged; D009422:Nervous System Diseases / Q000188:drug therapy* / Q000401:mortality*; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D008727:Methotrexate
keywords: Behcet's disease; Central nervous system; Disability; Methotrexate; Mortality; Progression