SCDU of Pediatrics, Department of Health Sciences, Università degli Studi del Piemonte Orientale, Novara, Italy.
Pediatrics and Pediatric Emergency Unit, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
Rheumatology (Oxford). 2021 Aug 2;60(8):3868-3871. doi: 10.1093/rheumatology/keaa743.
Henoch-Schönlein purpura (HScP) may present in children with severe, occasionally refractory, gastrointestinal (GI) involvement. The use of corticosteroids (CSs) is commonplace in the management of the disease, but to date no standardized protocol is available and, although rare, resistance to CS therapy may be challenging to clinicians. IVIG has been proposed as an effective alternative to CSs, but to date no controlled trial has been conducted to ascertain their real efficacy. We share our personal experience of successful IVIG treatment in two cases of GI HScP, comparing it with similar experiences reported in literature.
Retrospective clinical data collection, comparison with available literature.
We describe two children with severe HScP GI vasculitis refractory to high-dose intravenous CSs that responded rapidly to IVIG administration, with complete recovery within a few days. Patient characteristics and response to IVIG administration were comparable to those of other previously reported cases.
Our observation confirms that IVIG may be useful in the treatment of CS-resistant HScP-related GI vasculitis in children, and highlights the need for more structured research, including a randomized trial against CSs, in order to ascertain their real effectiveness.
过敏性紫癜（HScP）可表现为严重的胃肠道（GI）受累，偶尔难以治疗。皮质类固醇（CSs）的使用在疾病管理中很常见，但迄今为止尚无标准化方案，尽管很少见，但 CS 治疗的耐药性可能对临床医生构成挑战。静脉注射免疫球蛋白（IVIG）已被提议作为 CSs 的有效替代方法，但迄今为止，尚无对照试验来确定其真正的疗效。我们分享了我们在两例 GI HScP 中成功使用 IVIG 治疗的个人经验，并与文献中报道的类似经验进行了比较。
回顾性临床数据收集，与现有文献比较。
我们描述了两例对大剂量静脉 CS 治疗难治的严重 HScP GI 血管炎儿童，他们对 IVIG 治疗迅速反应，在几天内完全恢复。患者特征和 IVIG 治疗反应与其他先前报道的病例相似。
我们的观察结果证实，IVIG 可能对治疗 CS 耐药性 HScP 相关 GI 血管炎有效，并强调需要进行更多的结构化研究，包括与 CSs 进行随机试验，以确定其真正的疗效。
