Morita Thâmara Cristiane Alves Batista, Trés Gabriela Franco S, Criado Roberta Fachini Jardim, Sotto Mirian Nacagami, Criado Paulo Ricardo
Department of Dermatology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
Department of Dermatology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
An Bras Dermatol. 2020 May-Jun;95(3):355-371. doi: 10.1016/j.abd.2020.01.003. Epub 2020 Mar 26.
The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.
血管炎一词指血管壁的炎症。其严重程度可从单一器官的自限性疾病到因多器官衰竭导致的危及生命的疾病。血管炎有多种病因,尽管它们仅导致少数几种血管炎症的组织学模式。任何类型和任何器官的血管都可能受到影响,这一事实导致了广泛的体征和症状。临床表现难以区分的不同血管炎预后和治疗方法却大不相同。这种疾病在分类、诊断、适当的实验室检查和治疗方面给医生带来了诸多挑战。此外,还需要仔细随访。本文回顾了2012年 Chapel-Hill 分类、病因、病理生理学的最新见解、一些重要的诊断皮肤线索,并总结了其中一些复杂血管炎综合征的治疗方法。