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Primary CNS Vasculitis of Childhood Treatment & Management

  • Author: Jefferson R Roberts, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jul 06, 2016
 

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.[20] 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.
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Consultations

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  • 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.
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Diet

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  • 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.
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Activity

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  • 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.
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Contributor Information and Disclosures
Author

Jefferson R Roberts, MD Chief of Rheumatology Service, Tripler Army Medical Center; Assistant Clinical Professor of Medicine, Uniformed Services University of the Health Sciences

Jefferson R Roberts, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Society for Simulation in Healthcare

Disclosure: Nothing to disclose.

Coauthor(s)

Phalgoon A Shah, MD Resident Physician, Department of Medicine, Tripler Army Medical Center

Phalgoon A Shah, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

 

Disclosure: Nothing to disclose.

James J Kim, MD Resident Physician, Department of Internal Medicine, Tripler Army Medical Center

James J Kim, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

Clare M Hutchinson, MD Lecturer, Part-Time Faculty, Department of Pediatrics, University of Toronto Faculty of Medicine; Pediatrician, Pediatric Education Co-Lead, Department of Child and Teen Health, North York General Hospital, Canada

Disclosure: Nothing to disclose.

Susanne Maria Benseler, MD Pediatric Rheumatologist, Section Chief, Alberta Children's Hospital; Associate Professor, Department of Pediatrics, University of Calgary Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Acknowledgements

The authors thank Jorina Elbers, William Halliday, Suzanne Laughlin, Helen Branson, Harvey Lim, Robyn Westmacott, and Derek Armstrong for their significant contributions.

References
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