title: Adult primary central nervous system vasculitis treatment and course: analysis of one hundred sixty-three patients.
abstract: OBJECTIVE
  To describe the treatment and outcomes of patients with primary central nervous system (CNS) vasculitis.
  
  
  METHODS
  We retrospectively studied a cohort of 163 consecutive patients with primary CNS vasculitis who were seen at the Mayo Clinic over a 29-year period. We analyzed treatments, treatment responses, and factors predictive of outcomes.
  
  
  RESULTS
  A favorable response was observed in 85% of patients treated with prednisone alone and in 80% of patients treated with prednisone and cyclophosphamide. Relapses were observed in 27% of patients, and 25% of patients had discontinued therapy by the time of the last followup visit. Treatment with prednisone alone was associated with more frequent relapses (odds ratio [OR] 2.90), while large vessel involvement (OR 6.14) and cerebral infarcts at the time of diagnosis (OR 3.32) were associated with a poor response to treatment. Prominent gadolinium-enhanced cerebral lesions or meninges were linked with continued treatment at the last followup encounter (OR 2.28). Higher disability scores at the last followup visit were associated with increasing age at the time of diagnosis (OR 1.44) and cerebral infarctions (OR 3.74), while lower disability scores were associated with gadolinium-enhanced cerebral lesions or meninges (OR 0.35) and cerebral amyloid angiopathy (OR 0.24). Increased mortality was associated with increasing age at diagnosis (hazard ratio [HR] 1.39), diagnosis by angiography (HR 3.28), cerebral infarction (HR 4.44), and large vessel involvement (HR 4.98), while reduced mortality was associated with gadolinium-enhanced cerebral lesions or meninges (HR 0.20).
  
  
  CONCLUSIONS
  The majority of patients with primary CNS vasculitis responded to treatment. Recognition of findings at diagnosis that predict the course or outcome may aid in decision-making regarding therapy.
authors.affiliation: Mayo Clinic, Rochester, Minnesota, and Azienda Ospedaliera IRCCS di Reggio Emilia, Reggio Emilia, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Salvarani
authors.affiliation: Mayo Clinic, Rochester, Minnesota.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Brown
authors.affiliation: Mayo Clinic, Rochester, Minnesota.
authors.forename: Teresa J H
authors.initials: TJ
authors.lastname: Christianson
authors.affiliation: Mayo Clinic, Rochester, Minnesota.
authors.forename: John
authors.initials: J
authors.lastname: Huston
authors.affiliation: Mayo Clinic, Rochester, Minnesota.
authors.forename: Caterina
authors.initials: C
authors.lastname: Giannini
authors.affiliation: Mayo Clinic, Rochester, Minnesota.
authors.forename: Dylan V
authors.initials: DV
authors.lastname: Miller
authors.affiliation: Mayo Clinic, Rochester, Minnesota.
authors.forename: Gene G
authors.initials: GG
authors.lastname: Hunder
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016657:Cerebral Amyloid Angiopathy / Q000150:complications; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011241:Prednisone / Q000627:therapeutic use*; D016016:Proportional Hazards Models; D012008:Recurrence; D012189:Retrospective Studies; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone