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胃肠道型亨诺克-舒恩莱因紫癜经霉酚酸酯成功治疗:2 例病例报告描述。

Gastrointestinal Henoch-Schönlein purpura successfully treated with Mycophenolate Mofetil: Description of 2 case reports.

机构信息

Department of Woman, Child and General and Specialized Surgery.

Department of Precision Medicine, University of the Study of Campania "Luigi Vanvitelli," Naples, Italy.

出版信息

Medicine (Baltimore). 2021 Jan 8;100(1):e24093. doi: 10.1097/MD.0000000000024093.

Abstract

RATIONALE

Henoch-Schönlein Purpura (HSP) is an acute small vessel vasculitis. It is the most common vasculitis in children. In majority of the cases, the disease is self-limited. Relapses can occur, in particular during the first year of the disease. There is no consensus on a specific treatment. The efficacy and safety of steroidal treatment in treating HSP is still controversial. Immunosuppressive treatment of HSP nephritis is used in patients with severe renal involvement (nephrotic range proteinuria and/or progressive renal impairment). The literature on immunosuppressive treatment of severe HSP without kidney involvement is scanty.

PATIENTS CONCERNS

We report 2 case reports of 2 adolescents affected from Henoch-Schönlein Purpura and severe gastrointestinal involvement. Both patients presented a poor response to steroids treatment.

DIAGNOSES

The diagnosis of HSP was made according to the diagnostic criteria published by European League against Rheumatism and Pediatric Rheumatology European Society in 2006.

INTERVENTIONS

In consideration of the recurrence of the Henoch Schönlein Purpura and the gastrointestinal involvement, we decided to start Mycophenolate Mofetil treatment.

OUTCOMES

In both patients all clinical manifestations resolved in few days.

LESSONS

In our cases of HSP with gastrointestinal involvement Mycophenolate Mofetil treatment has been very effective. This experience teaches us that immunosuppressive agents may be very useful to induce and maintain remission not only in renal involvement, but in all cases of persistent, recurrent, or complicated Henoch Schönlein Purpura in children.

摘要

背景

过敏性紫癜(HSP)是一种急性小血管血管炎。它是儿童中最常见的血管炎。在大多数情况下,这种疾病是自限性的。疾病会复发,尤其是在发病的第一年。目前对于特定的治疗方案还没有达成共识。皮质类固醇治疗 HSP 的疗效和安全性仍存在争议。免疫抑制剂治疗 HSP 肾炎用于严重肾受累(肾病范围蛋白尿和/或进行性肾功能损害)的患者。对于无肾脏受累的严重 HSP 免疫抑制剂治疗的文献很少。

病例报告

我们报告了 2 例青少年 HSP 伴严重胃肠道受累的病例。这 2 名患者对皮质类固醇治疗反应不佳。

诊断

根据 2006 年欧洲抗风湿病联盟和儿科风湿病学会发表的诊断标准诊断 HSP。

干预

鉴于 Henoch Schönlein 紫癜的复发和胃肠道受累,我们决定开始使用霉酚酸酯治疗。

结果

在这 2 名患者中,所有临床表现均在数天内得到缓解。

结论

在我们 HSP 伴胃肠道受累的病例中,霉酚酸酯治疗非常有效。这一经验告诉我们,免疫抑制剂不仅在肾受累的情况下,而且在儿童持续性、复发性或复杂 HSP 的所有情况下,都可能非常有用,有助于诱导和维持缓解。

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Henoch-Schönlein Purpura in Children: An Updated Review.儿童过敏性紫癜:最新综述。
Curr Pediatr Rev. 2020;16(4):265-276. doi: 10.2174/1573396316666200508104708.

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