Cherqaoui B, Chausset A, Stephan J-L, Merlin E
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; Pédiatrie générale multidisciplinaire, CHU Saint-Étienne, avenue Albert-Raimond, 42270 Saint-Priest-en-Jarez, France.
Arch Pediatr. 2016 Jun;23(6):584-90. doi: 10.1016/j.arcped.2016.03.018. Epub 2016 Apr 26.
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.
过敏性紫癜（HSP）严重的胃肠道受累情况罕见，但可能危及生命。HSP严重胃肠道受累的管理尚无规范。对症治疗和使用类固醇是一线治疗方法。非甾体免疫调节疗法已被用于治疗类固醇难治性病例。本研究的目的是描述需要非甾体免疫调节疗法的HSP严重胃肠道受累患者的治疗结果。
开展一项法国回顾性病例系列研究。联系了法国31个学术儿科中心的儿科顾问。通过记忆或信息诊断相关编码系统识别患者。收集临床、辅助检查和治疗数据。
29个回复中心提供了9例病例，其中1例被排除。纳入了来自7个中心的5名男孩和3名女孩，年龄3 - 15岁（中位数：5.5岁）。HSP严重胃肠道受累主要包括剧烈疼痛、消化道出血和蛋白丢失性肠病。所有儿童一线均接受了类固醇治疗，二线接受了静脉注射免疫球蛋白（IVIg）治疗。8例中有6例在7天内对IVIg完全反应，8例中有2例部分反应。8例中有2例复发，胃肠道受累程度较轻，需要第二剂IVIg，此后未再复发。耐受性良好，但8例中有2例在IVIg输注后第二天出现高蛋白尿。
尽管需要探讨与蛋白尿发作的可能联系，但IVIg似乎是治疗HSP严重胃肠道受累的良好选择。
