2009 Southern Regional Meeting Abstracts

Case Report: The most common systemic vasculitis in children, Henoch-Schönlein purpura (HSP) is a small vessel vasculitis mediated by IgA-dominant immune complex deposits. It commonly affects the skin, gastrointestinal tract, joints, and kidneys. Gastrointestinal bleeding occurs in about half of children with HSP and is usually self-limited. We report a case of severe HSP in which the patient had persistent small bowel hemorrhage, requiring multiple blood transfusions, despite treatment with high dose steroids and cyclophosphamide. His intestinal bleeding resolved only after treatment with rituximab.
A previously healthy 17 year old male presented with abdominal pain and bloody stools. He developed a purpuric rash and joint pain 7 days later. Protein and blood were found in his urine. The diagnosis of HSP was made, and he initially improved with symptomatic care. He was admitted to the hospital 4 more times over the next 2 months for severe intestinal bleeds requiring multiple transfusions. Renal biopsy showed proliferative glomerulonephritis with IgA deposits in the mesangium. Endoscopy revealed vasculitic lesions in the stomach. Multiple areas of small bowel mucosal bleeding were noted on video capsule endoscopy. These were not amenable to either endoscopic or surgical intervention. He received high dose intravenous methylprednisolone multiple times and then cyclophosphamide along with the high dose steroids; however, severe intestinal bleeding recurred upon each attempted steroid taper. One month after initiation of rituximab (1g IV twice, 2 weeks apart), our patient's previously refractory intestinal bleeding resolved. Mucosal healing was documented on repeat video capsule endoscopy. He is maintained in remission 4 months later on mycophenolate mofetil.
A monoclonal anti-CD20 antibody, rituximab is approved to treat refractory rheumatoid arthritis and non-Hodgkin's lymphoma and is used off-label in the treatment of other rheumatologic diseases. Rituximab has not previously been documented as a successful treatment for GI complications of HSP. This case demonstrates that rituximab can be effective for severe complications of HSP such as gastrointestinal hemorrhage refractory to conventional treatment.