LSU Health Science Center, New Orleans, LA, USA.
Curr Rheumatol Rep. 2018 Nov 17;20(12):87. doi: 10.1007/s11926-018-0788-3.
Autoinflammatory diseases (AIDs) constitute several disorders that share similar characteristics, clinical features, disease course, and prognosis. They are characterized by the presence of recurrent episodes of unprovoked inflammation due to dysregulated innate immune system in the absence of autoantibodies or infections. AIDs include periodic fever syndromes and other less commonly growing list of syndromes. In this review, vasculitis associated with different AIDs will be highlighted.
Vasculitis is inflammation and necrosis of the blood vessels causing impaired blood flow, ischemia, and infarction of the dependent tissues. It is a very rare manifestation of AIDs and when it occurs, the skin is the most affected tissue than any other organs such as kidneys, lungs, or CNS. Although vasculitis and AIDs share similar manifestations such as fever, skin rashes, and neuropathy, vasculitis is not a characteristic feature of AIDs and still not clear if it represents a main clinical feature or a manifestation of other disease process.
自身炎症性疾病（AIDs）由多种具有相似特征、临床表现、病程和预后的疾病组成。它们的特征是由于先天免疫系统失调，在没有自身抗体或感染的情况下，反复发作无诱因的炎症。AIDs 包括周期性发热综合征和其他不太常见的综合征。在这篇综述中，将重点介绍与不同 AIDs 相关的血管炎。
血管炎是血管的炎症和坏死，导致血流受损、缺血和依赖组织梗死。它是 AIDs 非常罕见的表现，当发生时，皮肤是受影响最严重的组织，而不是其他器官，如肾脏、肺部或中枢神经系统。尽管血管炎和 AIDs 具有相似的表现，如发热、皮疹和神经病变，但血管炎不是 AIDs 的特征性表现，目前尚不清楚它是否代表主要的临床特征或其他疾病过程的表现。
