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复发性多软骨炎,一种被低估的皮肤科急症:病例报告及文献复习。

Relapsing polychondritis, an underestimated dermatological urgency: case report and literature review.

机构信息

Dermatology Service, Samaritana University Hospital - ESE, Bogotá, Colombia.

Dermatology Program, El Bosque University, Bogotá, Colombia.

出版信息

Int J Dermatol. 2017 Dec;56(12):1379-1386. doi: 10.1111/ijd.13755. Epub 2017 Oct 9.

Abstract

BACKGROUND

Relapsing polychondritis is an autoimmune multisystemic disease with primary chondral involvement. Its high mortality and morbidity make it a real clinical challenge.

CASE DESCRIPTION

A 32-year-old woman with a history of relapsing polychondritis, refractory to multiple treatments, with multisystem compromise, imminent risk of death due to severe tracheobronchial damage and difficult ventilatory support, and successful treatment with infliximab.

DISCUSSION AND EVALUATION

Several treatments have been described in the literature, such as nonsteroidal anti-inflammatory drugs, corticosteroids, dapsone, azathioprine, cyclosporine, cyclophosphamide, and methotrexate. However, the cases refractory to conventional therapy may lead to chronicity, irreversibility, and death. As a result, a third-line therapy could improve the prognosis of these patients.

CONCLUSIONS

Biological therapy is a good option for disease control and quality of life improvement. In addition, the physician should consider these treatments to avoid the chronicity and risk of death of these patients.

摘要

背景

复发性多软骨炎是一种自身免疫性多系统疾病,主要累及软骨。其高死亡率和发病率使其成为真正的临床挑战。

病例描述

一名 32 岁女性,有复发性多软骨炎病史,对多种治疗方法均无效,多系统受损,因严重气管支气管损伤和通气支持困难,有死亡风险,后用英夫利昔单抗治疗成功。

讨论和评估

文献中描述了多种治疗方法,如非甾体抗炎药、皮质类固醇、氨苯砜、硫唑嘌呤、环孢素、环磷酰胺和甲氨蝶呤。然而,对常规治疗无效的病例可能导致慢性化、不可逆转和死亡。因此,三线治疗可能改善这些患者的预后。

结论

生物治疗是控制疾病和改善生活质量的一种好方法。此外,医生应考虑这些治疗方法,以避免这些患者的慢性化和死亡风险。

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