Mycophenolate Mofetil Effective in Children With Refractory Primary CNS Angiitis
Last Updated: 2010-02-18 15:55:12 -0400 (Reuters Health)
By Will Boggs, MD
NEW YORK (Reuters Health) - Mycophenolate mofetil may be effective in children with refractory primary angiitis of the central nervous system (CNS), UK researchers report.
In a January 25th online report in Rheumatology, senior author Dr. Athimalaipet V. Ramanan from Bristol Royal Hospital for Children and colleagues describe 3 children who responded to first-line immunosuppression but then relapsed.
Each child -- a 5-year-old boy, a 9-year-old girl, and a 6-year-old girl - had initially presented with visual disturbances in addition to other neurologic findings, including severe headache with nausea and vomiting or fever, generalized tonic clonic seizures, and weakness in the legs.
In addition, they all had significant inflammatory CNS changes on magnetic resonance imaging scans, and the 5-year-old had abnormalities on cerebral angiography in his distal posterior cerebral arteries.
All 3 children relapsed during maintenance therapy with methotrexate or azathioprine. With mycophenolate mofetil, starting at a dose of 300 mg/m2 twice daily and increasing to 500 or 600 mg/m2 twice/day, each child's symptoms resolved. In 2 children, remission persisted (with mycophenolate mofetil and methotrexate) after steroids were withdrawn. The third child was being weaned from steroids as the authors wrote their paper.
"We believe that mycophenolate mofetil might add to the therapeutic armamentarium" for children with primary CNS angiitis, the authors conclude.
In comments to Reuters Health, Dr. Ramanan said that standard therapy for the disease includes a 6-month course of cyclophosphamide. "Like in lupus nephritis it is possible that mycophenolate mofetil might prove as effective as cyclophosphamide," Dr. Ramanan said by email. "This would need more evidence, but if it is shown that mycophenolate mofetil is as effective, it would be more desirable as it is definitely a safer agent than cyclophosphamide."
Rheumatology 2010.
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