Rituximab for severe refractory pediatric Wegener granulomatosis.
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.
Patel
Aarat M
AM
Department of Medicine/Pediatrics, Stony Brook University Medical Center, Stony Brook, NY, USA. aarat.patel@chp.edu
Lehman
Thomas J A
TJ
D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D005919:Glomerular Filtration Rate; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage; D000069283:Rituximab; D014057:Tomography, X-Ray Computed
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide; D011239:Prednisolone