Ralli Massimo, Campo Flaminia, Angeletti Diletta, Minni Antonio, Artico Marco, Greco Antonio, Polimeni Antonella, de Vincentiis Marco
Department of Sense Organs, Sapienza University of Rome, Italy.
Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Italy.
EXCLI J. 2020 Jun 18;19:817-854. doi: 10.17179/excli2020-1512. eCollection 2020.
Systemic vasculitides represent uncommon conditions characterized by the inflammation of blood vessels that can lead to different complex disorders limited to one organ or potentially involving multiple organs and systems. Systemic vasculitides are classified according to the diameter of the vessel that they mainly affect (small, medium, large, or variable). The pathogenetic mechanisms of systemic vasculitides are still partly unknown, as well as their genetic basis. For most of the primary systemic vasculitides, a single gold standard test is not available, and diagnosis is often made after having ruled out other mimicking conditions. Current research has focused on new management protocol and therapeutic strategies aimed at improving long-term patient outcomes and avoiding progression to multiorgan failure with irreversible damage. In this narrative review, authors describe different forms of systemic vasculitides through a review of the literature, with the aim of highlighting the current knowledge and recent findings on etiopathogenesis, diagnosis and therapy.
系统性血管炎是一类罕见疾病，其特征为血管炎症，可导致局限于一个器官或可能累及多个器官和系统的不同复杂病症。系统性血管炎根据其主要累及的血管直径（小、中、大或不定）进行分类。系统性血管炎的发病机制及其遗传基础仍部分未知。对于大多数原发性系统性血管炎而言，尚无单一的金标准检测方法，诊断通常在排除其他类似病症后做出。当前研究聚焦于新的管理方案和治疗策略，旨在改善患者长期预后并避免进展为具有不可逆损伤的多器官功能衰竭。在这篇叙述性综述中，作者通过文献回顾描述了系统性血管炎的不同形式，目的是突出关于病因发病机制、诊断和治疗的当前知识及最新发现。
