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结节性红斑麻风:被忽视疾病治疗的最新进展与挑战

Erythema Nodosum Leprosum: Update and challenges on the treatment of a neglected condition.

作者信息

Costa Perpétua do Socorro Silva, Fraga Lucas Rosa, Kowalski Thayne Woycinck, Daxbacher Egon Luiz Rodrigues, Schuler-Faccini Lavínia, Vianna Fernanda Sales Luiz

机构信息

Postgraduate Program in Genetics and Molecular Biology. Universidade Federal do Rio Grande do Sul, Av. Bento Gonçalves, 9500 - Prédio 43312 M, Porto Alegre, RS, Brazil; INAGEMP, Instituto Nacional de Genética Médica Populacional, R. Ramiro Barcelos, 2350 - 21506 - Santa Cecilia, Porto Alegre, RS, Brazil; Center of Social Sciences, Health and Technology. Universidade Federal do Maranhão, R. Urbano Santos, S/N, Imperatriz, MA, Brazil.

Postgraduate Program in Genetics and Molecular Biology. Universidade Federal do Rio Grande do Sul, Av. Bento Gonçalves, 9500 - Prédio 43312 M, Porto Alegre, RS, Brazil; INAGEMP, Instituto Nacional de Genética Médica Populacional, R. Ramiro Barcelos, 2350 - 21506 - Santa Cecilia, Porto Alegre, RS, Brazil; Teratogen Information Service, Medical Genetics Service, Hospital de Clínicas de Porto Alegre, R. Ramiro Barcelos, 2350, Santa Cecília, Porto Alegre, RS, Brazil.

出版信息

Acta Trop. 2018 Jul;183:134-141. doi: 10.1016/j.actatropica.2018.02.026. Epub 2018 Feb 21.

Abstract

Erythema Nodosum Leprosum (ENL) occurs due to the immunological complication of multibacillary leprosy and is characterized by painful nodules and systemic compromising. It is usually recurrent and/or chronic and has both physical and economic impact on the patient, being a very important cause of disability. In addition, ENL is a major health problem in countries where leprosy is endemic. Therefore, adequate control of this condition is important. The management of ENL aims to control acute inflammation and neuritis and prevent the onset of new episodes. However, all currently available treatment modalities have one or two drawbacks and are not effective for all patients. Corticosteroid is the anti-inflammatory of choice in ENL but may cause dependence, especially for chronic patients. Thalidomide has a rapid action but its use is limited due the teratogenicity and neurotoxicity. Clofazimine and pentoxifylline have slow action and have important adverse effects. Finally, there is no pattern or guidelines for treating these patients, becoming more difficult to evaluate and to control this condition. This review aims to show the main drugs used in the treatment of ENL and the challenges in the management of the reaction.

摘要

结节性红斑麻风(ENL)是多菌型麻风的免疫并发症,其特征为疼痛性结节和全身损害。它通常呈复发性和/或慢性,对患者的身体和经济都有影响,是导致残疾的一个非常重要的原因。此外,在麻风流行的国家,ENL是一个主要的健康问题。因此,充分控制这种病症很重要。ENL的治疗旨在控制急性炎症和神经炎,并预防新发作的出现。然而,目前所有可用的治疗方式都有一两个缺点,并非对所有患者都有效。皮质类固醇是ENL治疗中首选的抗炎药物,但可能会导致依赖,尤其是对慢性病患者。沙利度胺起效迅速,但其使用因致畸性和神经毒性而受到限制。氯法齐明和己酮可可碱起效缓慢且有重要的不良反应。最后,治疗这些患者没有固定模式或指南,使得评估和控制这种病症变得更加困难。本综述旨在展示治疗ENL的主要药物以及反应管理中的挑战。

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