Abstract
Severe gastrointestinal involvement of Henoch-Schönlein purpura (HSP) is rare but potentially life-threatening. Management of severe gastrointestinal involvement in HSP is not codified. Symptomatic care and steroids are a first-line therapy. Nonsteroidal immunomodulatory therapies have been anecdotally used to treat steroid-refractory forms. The aim of this study was to describe the outcome of patients with severe gastrointestinal involvement of HSP who required nonsteroidal immunomodulatory therapy. A French retrospective case series study was conducted. Pediatric consultants at 31French academic pediatric centers were contacted. Patients were identified from memory or via an informatics diagnosis-related code system. Clinical, paraclinical, and therapeutic data were collected. Twenty-nine responding centers provided nine cases, one of which was excluded. Five boys and three girls, aged 3-15years (median: 5.5years) from seven centers were included. Severe gastrointestinal involvement of HSP mainly included intense pain, digestive bleeding, and protein-losing enteropathy. All children had been treated with steroids at first line and intravenous immunoglobulins (IVIg) at second line. Six out of eight showed a complete response to IVIg within 7days and two out of eight had a partial response. Two out of eight relapsed with less severe gastrointestinal involvement requiring a second dose of IVIg and they did not relapse thereafter. Tolerance was good, but two out of eight developed high proteinuria on the day following IVIg infusion. Although a possible link with a flare-up of proteinuria needs to be addressed, IVIg appears to be a good candidate for treatment of severe gastrointestinal involvement of HSP.
Published Version
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