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氨苯砜在 IgA 血管炎(过敏性紫癜)中的适应证和疗效:病例系列及文献复习。

Indications and efficiency of dapsone in IgA vasculitis (Henoch-Schonlein purpura): case series and a review of the literature.

机构信息

Department of Pediatrics, Hôpital Civil Marie Curie, CHU of Charleroi, 140 Chaussée de Bruxelles, 6042, Charleroi (Lodelinsart), Belgium.

Pediatric Department, Cliniques de l'Europe Sainte-Elisabeth, 206 Avenue de Frélaan, 1180, Brussels, Belgium.

出版信息

Eur J Pediatr. 2019 Aug;178(8):1275-1281. doi: 10.1007/s00431-019-03409-5. Epub 2019 Jun 22.

Abstract

Immunoglobulin A (IgA) vasculitis (Henoch-Schonlein purpura (HSP)) is the most common vasculitis in children. It is characterized by purpuric rash, arthritis, gastrointestinal, and/or renal involvement. Spontaneous resolution is the typical outcome. In chronic cutaneous manifestations of IgA vasculitis, dapsone seems to show a good effectiveness. Multiple case reports and case series about dapsone in chronic IgA vasculitis are available. However, no clear evaluation of its indications, its effectiveness, or its usage guidelines (optimal dosage or duration of treatment) is available. We reviewed the published cases of IgA vasculitis treated by dapsone and compared them with 2 similar cases that we encountered. Seventeen patients (ranging from 22 months old to 16 years old) with severe or persistent clinical signs of IgA vasculitis were included. Dapsone showed good results on the resolution of cutaneous lesions but not on renal manifestations. Complications (methemoglobinemia) were observed on 1 patient. Half of the patients relapsed after treatment discontinuation. The difference between the time lapse before initiation and the duration of the treatment was not significant.Conclusion: We suggest that dapsone can have a positive effect in chronic IgA vasculitis when cutaneous manifestations last more than 6 weeks at the dosage of 1-2 mg/kg once per day during 1 week. What is Known: • IgA vasculitis or Henoch-Schonlein purpura is the most common vasculitis in children and affects mostly small vessels of the skin, kidney, and gastrointestinal tract. It resolves spontaneously in most of the cases. Exceptionally, cutaneous lesions can last several weeks. • Dapsone is a bacteriostatic antibacterial sulfonamide drug found to be effective in the treatment of some inflammatory dermatological diseases like IgA vasculitis. What is New: • Dapsone is effective against chronic purpuric lesion (> 6 weeks) at the minimal dose of 1 mg/kg/day. • Relapse occurs frequently after discontinuation but responds after a second course of treatment. A longer duration of treatment or a delay in treatment by dapsone does not seem to influence the relapse rate.

摘要

免疫球蛋白 A(IgA)血管炎(过敏性紫癜(HSP))是儿童中最常见的血管炎。其特征为紫癜性皮疹、关节炎、胃肠道和/或肾脏受累。自发缓解是典型的结局。在 IgA 血管炎的慢性皮肤表现中,氨苯砜似乎显示出良好的疗效。有许多关于氨苯砜治疗慢性 IgA 血管炎的病例报告和病例系列。然而,对于其适应症、疗效或使用指南(最佳剂量或治疗持续时间),尚无明确的评估。我们回顾了已发表的用氨苯砜治疗的 IgA 血管炎病例,并将其与我们遇到的 2 例相似病例进行了比较。纳入了 17 例(年龄 22 个月至 16 岁)有严重或持续性 IgA 血管炎临床体征的患者。氨苯砜在皮肤病变的消退方面显示出良好的效果,但对肾脏表现无效。1 例患者出现并发症(高铁血红蛋白血症)。一半的患者在停药后复发。开始治疗与治疗持续时间之间的差异无统计学意义。结论:我们建议,当皮肤表现持续超过 6 周时,氨苯砜可在每天 1-2mg/kg 剂量下每天 1 次治疗 1 周,对慢性 IgA 血管炎有积极作用。已知情况:IgA 血管炎或过敏性紫癜是儿童中最常见的血管炎,主要影响皮肤、肾脏和胃肠道的小血管。在大多数情况下,它会自发缓解。例外情况下,皮肤损伤可持续数周。氨苯砜是一种抑菌性磺胺类抗菌药物,已被证明对一些炎症性皮肤病如 IgA 血管炎有效。新情况:氨苯砜对慢性紫癜性病变(>6 周)有效,最低剂量为 1mg/kg/天。停药后常复发,但在第二次疗程后可缓解。延长治疗时间或延迟氨苯砜治疗似乎不会影响复发率。

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