结节性多动脉炎诊疗的现代观

作者：Armando De Virgilio,et al.

翻译：李玉翠 审阅：刘湘源

摘要：结节性多动脉炎(PAN)是一种主要针对中等大小动脉的系统性坏死性血管炎，是一种罕见的血管炎，其发病率尚不清楚。与结节性多动脉炎发病相关的主要环境因素是乙肝病毒感染。结节性多动脉炎对免疫抑制剂有效，提示免疫机制参与发病，但“特发性结节性多动脉炎”的发病机制仍不清楚。结节性多动脉炎可累及单器官，也可多脏器，任何器官都可能受累，但不清楚为何不影响肺。除 “全身特发性结节性多动脉炎”外,还有两种变异型——“皮肤型结节性多动脉炎”和“乙型肝炎病毒相关性结节性多动脉炎”。结节性多动脉炎的诊断需结合临床、血管造影和活检。近几十年来，因早期诊断和积极治疗，该病的总体预后已得到改善。全身特发性结节性多动脉炎应使用糖皮质激素联合环磷酰胺治疗，乙肝病毒相关性结节性多动脉炎应使用抗病毒药控制感染，皮肤型结节性多动脉炎需短期用非甾体抗炎药等较温和的治疗方法。

附原文：ABSTRACT Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly targeting medium-sized arteries.PAN is a rare form of vasculitis, and the precise frequency of this disease is difficult to determine. The major environmental factor associated with PAN is HBV infection.The pathogenesis of “idiopathic PAN” remains enigmatic, although the clinical responses to immunosuppressive therapy support the concept that immunological mechanisms play an active pathogenic role.The spectrum of disease ranges from involving a single organ to polyvisceral failure. Any organ might be affected;however, for reasons that are not understood, PAN does not affect the lungs. In addition to the systemic idiopathic form, called “idiopathic generalized PAN,” there are 2 clinical variants of this disease: “cutaneous PAN” and“hepatitis B virus (HBV)-associated PAN”.Diagnosis requires the integration of clinical, angiographic, and biopsy findings. The overall prognosis of this disease has been improved in recent decades, primarily reflecting early diagnosis and more effective treatments.Idiopathic generalized PAN should be treated with a combination of glucocorticoids and cyclophosphamide. The treatment of HBV-associated PAN involves a different approach, centered on the use of an antiviral agent to control the infection. The therapy for cutaneous PAN requires a less aggressive approach based on the administration of non-steroidal anti-inflammatory drugs over short periods of time.

引自：Armando De Virgilio, Antonio Greco , Giuseppe Magliuloa, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev, 2016 ,15(6):564-70.