Primary CNS Vasculitis of Childhood Treatment & Management

Updated: Dec 14, 2018
  • Author: Jefferson R Roberts, MD; Chief Editor: Lawrence K Jung, MD  more...
  • Print

Medical Care

No controlled trials have described treatment protocols for primary CNS vasculitis of childhood. The treatment of progressive large-medium vessel disease and small vessel disease consists of immunosuppression in induction and maintenance phases, with anticoagulation as appropriate. [13]

  • Induction immunosuppression occurs over a 6-month period and includes 7 pulses of intravenous cyclophosphamide administered every 4 weeks, as well as high-dose corticosteroids. [23] Prophylaxis against Pneumocystis jirovecipneumonia (formerly Pneumocystis carinii pneumonia) with a medication such as cotrimoxazole is important while receiving cyclophosphamide.

  • Following the completion of induction with cyclophosphamide, maintenance therapy that consists of oral azathioprine or mycophenolate mofetil continues for 18 months, with weaning doses of oral corticosteroids to continue administration of immunosuppressive therapy.

  • In adults, rituximab, a monoclonal antibody that depletes B cells, is challenging the role of cyclophosphamide. However in pediatric systemic vasculitis, its role is limited to those patients who have disease refractory to current conventional therapy.

  • Low molecular weight heparin administered for a brief period at presentation, followed by an antiplatelet agent, is frequently used in progressive large-medium vessel disease.

  • The treatment of nonprogressive large-medium vessel disease is controversial but often consists of a 3-month course of high-dose corticosteroids and an antiplatelet agent.

  • Control of symptoms such as seizures and psychosis is of paramount importance and may require anticonvulsants, psychiatric medications, or other medications.



See the list below:

  • A multidisciplinary approach that involves a rheumatologist, neurologist, radiologist, physiotherapist, occupational therapist, and social worker is required.

  • Involving a psychiatrist, speech and language pathologist, or neuropsychologist for both assessment and treatment of this condition may be appropriate.



See the list below:

  • A healthy diet with low fat and sodium intake is appropriate when a patient begins corticosteroid treatment.

  • Adequate intake of calcium and vitamin D, with supplementation when necessary, is essential when children are treated with corticosteroids.



See the list below:

  • Activity restrictions should be based on symptoms. For example, this may include support with ambulation if instability is present during the initial stages or restrictions on driving if seizures are part of the disease.