title: Immune activation in amyloid-β-related angiitis correlates with decreased parenchymal amyloid-β plaque load.
abstract: BACKGROUND
  Primary angiitis of the central nervous system (PACNS) is a rare but serious condition. A fraction of patients suffering from PACNS concurrently exhibit pronounced cerebral amyloid angiopathy (CAA) which is characterized by deposits of amyloid-β (Aβ) in and around the walls of small and medium-sized arteries of the brain. PACNS with CAA has been identified as a distinct disease entity, termed Aβ-related angiitis (ABRA). Evidence points to an immune reaction to vessel wall Aβ as the trigger of vasculitis.
  
  
  OBJECTIVE
  To investigate whether the inflammatory response to Aβ has (1) any effect on the status of immune activation in the brain parenchyma and (2) leads to clearance of Aβ from brain parenchyma.
  
  
  METHODS
  We studied immune activation and Aβ load by quantitative immunohistochemical analysis in brain parenchyma adjacent to affected vessels in 11 ABRA patients and 10 matched CAA controls.
  
  
  RESULTS
  ABRA patients showed significantly increased immune activation and decreased Aβ loads in the brain parenchyma adjacent to affected vessels.
  
  
  CONCLUSIONS
  Our results are in line with the hypothesis of ABRA being the result of an excessive immune response to Aβ and show that this can lead to enhanced clearance of Aβ from the brain parenchyma by immune-mediated mechanisms.
authors.affiliation: Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Bogner
authors.forename: C
authors.initials: C
authors.lastname: Bernreuther
authors.forename: J
authors.initials: J
authors.lastname: Matschke
authors.forename: A
authors.initials: A
authors.lastname: Barrera-Ocampo
authors.forename: D
authors.initials: D
authors.lastname: Sepulveda-Falla
authors.forename: F
authors.initials: F
authors.lastname: Leypoldt
authors.forename: T
authors.initials: T
authors.lastname: Magnus
authors.forename: F
authors.initials: F
authors.lastname: Haag
authors.forename: M
authors.initials: M
authors.lastname: Bergmann
authors.forename: W
authors.initials: W
authors.lastname: Brück
authors.forename: S
authors.initials: S
authors.lastname: Vogelgesang
authors.forename: M
authors.initials: M
authors.lastname: Glatzel
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000032:analysis / Q000276:immunology*; D001921:Brain / Q000276:immunology* / Q000473:pathology*; D016022:Case-Control Studies; D016657:Cerebral Amyloid Angiopathy / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008262:Macrophage Activation; D008264:Macrophages / Q000276:immunology; D008297:Male; D008875:Middle Aged; D058225:Plaque, Amyloid / Q000276:immunology / Q000473:pathology; D020293:Vasculitis, Central Nervous System / Q000276:immunology* / Q000473:pathology*
chemical_list: D016229:Amyloid beta-Peptides