Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease

Arthritis Rheum. 2005 Jul;52(7):2159-67. doi: 10.1002/art.21144.


Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aspirin / therapeutic use
  • Azathioprine / therapeutic use
  • Brain / pathology*
  • Cerebral Angiography*
  • Child
  • Child, Preschool
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Prednisone / therapeutic use
  • Treatment Outcome
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / drug therapy


  • Immunosuppressive Agents
  • Cyclophosphamide
  • Azathioprine
  • Aspirin
  • Prednisone