Abstract
We describe a case of pediatric Wegener granulomatosis initially treated with cyclophosphamide and oral corticosteroids resulting in remission for 5 years. Of note in this case is relapse with severe pulmonary disease treated with multiple regimens, all unsuccessful. Patient achieved remission with rituximab infusion therapy. This demonstrates how rituximab may be beneficial for childhood-onset Wegener granulomatosis unresponsive to conventional therapy. The case is followed by a review of the current treatment options.
MeSH terms
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Adolescent
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Antibodies, Monoclonal / administration & dosage
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Antibodies, Monoclonal / therapeutic use*
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Antibodies, Monoclonal, Murine-Derived
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Cyclophosphamide / administration & dosage
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Disease Progression
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Glomerular Filtration Rate
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Granulomatosis with Polyangiitis / diagnosis
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Granulomatosis with Polyangiitis / diagnostic imaging
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Granulomatosis with Polyangiitis / drug therapy*
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Granulomatosis with Polyangiitis / physiopathology
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Humans
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Immunologic Factors / administration & dosage
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Immunologic Factors / therapeutic use*
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Immunosuppressive Agents / administration & dosage
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Male
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Prednisolone / administration & dosage
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Rituximab
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Tomography, X-Ray Computed
Substances
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Murine-Derived
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Immunologic Factors
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Immunosuppressive Agents
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Rituximab
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Cyclophosphamide
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Prednisolone