title: Intravenous immunoglobulins for severe gastrointestinal involvement in pediatric Henoch-Schönlein purpura: A French retrospective study.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Pédiatrie générale multidisciplinaire, CHU Estaing, 1, place Lucie-et-Raymond-Aubrac, 63003 Clermont-Ferrand, France.
authors.forename: B
authors.initials: B
authors.lastname: Cherqaoui
authors.affiliation: Inserm CIC 1405, 1, place Lucie-et-Raymond-Aubrac, 63003 Clermont-Ferrand, France; Pédiatrie générale multidisciplinaire, CHU Saint-Étienne, avenue Albert-Raimond, 42270 Saint-Priest-en-Jarez, France.
authors.forename: A
authors.initials: A
authors.lastname: Chausset
authors.affiliation: Pédiatrie générale multidisciplinaire, CHU Saint-Étienne, avenue Albert-Raimond, 42270 Saint-Priest-en-Jarez, France.
authors.forename: J-L
authors.initials: JL
authors.lastname: Stephan
authors.affiliation: Pédiatrie générale multidisciplinaire, CHU Estaing, 1, place Lucie-et-Raymond-Aubrac, 63003 Clermont-Ferrand, France; Inserm CIC 1405, 1, place Lucie-et-Raymond-Aubrac, 63003 Clermont-Ferrand, France. Electronic address: e_merlin@chu-clermontferrand.fr.
authors.forename: E
authors.initials: E
authors.lastname: Merlin
mesh_terms: D015746:Abdominal Pain / Q000188:drug therapy* / Q000209:etiology; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005602:France; D006471:Gastrointestinal Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000188:drug therapy*; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology; D011507:Proteinuria / Q000209:etiology; D012189:Retrospective Studies
chemical_list: D016756:Immunoglobulins, Intravenous