Intravenous immunoglobulins for severe gastrointestinal involvement in pediatric Henoch-Schönlein purpura: A French retrospective study

• Published in: Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2016-06, Vol.23 (6), p.584-590
• Main Authors: Cherqaoui, B., Chausset, A., Stephan, J.-L., Merlin, E.
• Format: Article
• Language: English
• Subjects: Abdominal Pain - drug therapy; Abdominal Pain - etiology; Adolescent; Child; Child, Preschool; Female; France; Gastrointestinal Hemorrhage - drug therapy; Gastrointestinal Hemorrhage - etiology; Humans; Immunoglobulins, Intravenous - therapeutic use; Male; Protein-Losing Enteropathies - drug therapy; Protein-Losing Enteropathies - etiology; Proteinuria - etiology; Purpura, Schoenlein-Henoch - complications; Purpura, Schoenlein-Henoch - drug therapy; Retrospective Studies
• ISSN: 0929-693X; 1769-664X
• DOI: 10.1016/j.arcped.2016.03.018

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 31 French 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. Les atteintes gastro-intestinales sévères du purpura rhumatoïde (PR-GI) sont rares mais peuvent mettre en jeu le pronostic vital. Leur prise en charge n’est pas codifiée. Les mesures symptomatiques et les corticostéroïdes constituent la première ligne thérapeutique. Les traitements immunomodulateurs non stéroïdiens (INST) ont été ponctuellement utilisés pour traiter les formes réfractaires aux stéroïdes. Le but de cette étude était de décrire le devenir des patients avec PR-GI sévères ayant requis un INST. Une série nationale française rétrospective a été constituée, en contactant les pédiatres référents de 31 hôpitaux pédiatriques universitaires. Les patients ont été identifiés de mémoire ou via le Programme de médicalisation des systèmes d’information (P