Lee Keum Hwa, Hong Sung Hwi, Jun Jinhae, Jo Youngheun, Jo Woogyeong, Choi Dayeon, Joo Jeongho, Jung Guhyun, Ahn Sunghee, Kronbichler Andreas, Eisenhut Michael, Shin Jae Il
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Korea.
Clin Exp Pediatr. 2020 May;63(5):158-163. doi: 10.3345/kjp.2019.00514. Epub 2019 Sep 24.
IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.
IgA血管炎，以前称为过敏性紫癜，是一种常见于儿童的由IgA介导的全身性小血管血管炎。IgA血管炎的自然病程通常是自限性的；然而，三分之一的患者会出现症状复发和难治性病程。本系统评价研究了氨苯砜在难治性IgA血管炎病例中的应用。对PubMed数据库进行文献检索，检索到截至2018年6月14日发表的13篇文章。最常见的临床特征是可触及的皮疹（100%的患者），其次是关节疼痛（69.2%）。26例患者中有6例（23.1%）在1 - 2天内出现治疗反应，17例患者（65.4%）在3 - 7天内出现治疗反应。17例患者（65.4%）报告在停药后复发，但3例患者（11.5%）未复发。26例患者中有4例（15.4%）报告了氨苯砜的不良反应，包括关节痛（7.7%）、皮疹（7.7%）和氨苯砜超敏综合征（3.8%）。我们的研究结果表明，氨苯砜可能对难治性IgA血管炎有影响。有必要进行多中心随机安慰剂对照试验，以确定IgA血管炎患者初始治疗或减量治疗时氨苯砜的标准剂量，并评估氨苯砜与类固醇或其他药物相比是否有显著益处。
