抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)是一组罕见的,小学,全身坏死性小血管血管炎。肉芽肿性多血管炎和显微镜下多血管炎占所有AAV的80%至90%。暴露于二氧化硅粉尘,农业,和慢性鼻金黄色葡萄球菌携带与发展AAV的风险增加有关。当怀疑有AAV的诊断时,如多系统器官功能障碍患者或具有慢性复发性鼻窦炎等特征的患者,空化肺结节,明显的紫癜,或急性肾损伤,那么需要适当的进一步调查,包括ANCA测试。在这种情况下,应进行结构化临床评估,评估所有可能涉及的器官,和组织活检可能是必要的,以确认诊断。治疗算法根据AAV的严重程度而有所不同,临床诊断/ANCA特异性,患者年龄,体重,合并症,和预后。最近的数据表明利妥昔单抗是诱导和维持缓解的首选方案。此外,与常规方案相比,使用较少糖皮质激素的方案在诱导缓解方面同样有效且更安全,和avacopan是一种有效的糖皮质激素节约选择。相比之下,没有令人信服的证据支持在AAV中除标准缓解诱导治疗外还常规使用血浆置换.ANCA和其他生物标志物可有助于与临床评估相关联,以指导诊断和治疗决策。在随访期间,应经常评估患者是否有可能的疾病复发或与治疗相关的发病率。为了监测损害的累积,尤其是代谢和心血管损害。
ANCA-associated vasculitides (AAV) are a group of rare, primary, systemic necrotizing small-vessel vasculitides. Granulomatosis with polyangiitis and microscopic polyangiitis account for ∼80-90% of all AAV. Exposure to silica dust, farming and chronic nasal Staphylococcus aureus carriage are associated with increased risk of developing AAV. When a diagnosis of AAV is suspected, as in patients with multisystem organ dysfunction or those with features such as chronic recurrent rhinosinusitis, cavitated lung nodules, palpable purpura or acute kidney injury, then appropriate further investigations are needed, including ANCA testing. In this scenario, a structured clinical assessment should be conducted, evaluating all the organs possibly involved, and tissue biopsy may be necessary for confirmation of the diagnosis. Therapeutic algorithms vary based on the severity of AAV, the clinical diagnosis/ANCA specificity, and the patient\'s age, weight, comorbidities and prognosis. Recent data favour rituximab as a preferable option for both induction and maintenance of remission. In addition, regimens with less glucocorticoids are equally effective and safer in inducing remission compared with conventional regimens, and avacopan is an effective glucocorticoid-sparing option. In contrast, there is not compelling evidence to support the routine use of plasma exchange in addition to standard remission-induction therapy in AAV. ANCA and other biomarkers can be helpful in association with clinical assessment to guide diagnosis and treatment decisions. Patients should be frequently evaluated during follow-up for possible disease relapses or treatment-related morbidity, and for monitoring damage accrual, especially metabolic and cardiovascular damage.