结节性多动脉炎诊疗规范

Recommendations of diagnosis and treatment of polyarteritis nodosa

结节性多动脉炎(PAN)是主要累及中小动脉的一种少见的血管炎,好发于血管的分叉处,导致微动脉瘤、血栓形成、动脉瘤破裂出血及器官梗死。约1/3的患者与乙型肝炎病毒(HBV)感染相关。全身各组织器官均可受累,以皮肤、关节、外周神经最常见。病理改变急性期为血管壁纤维蛋白样坏死,炎性细胞浸润和管腔内血栓形成,慢性期为血管壁纤维性增生。近几十年来,PAN的总体预后得到了改善,主要反映了早期诊断和更有效的治疗方法。PAN治疗主要是糖皮质激素和环磷酰胺,而HBV相关PAN患者应接受抗病毒治疗和血浆置换。中华医学会风湿病学分会组织有关专家,在借鉴国内外诊治经验和指南的基础上,制定了本规范,旨在规范PAN的诊断和治疗,以降低致死率及严重并发症的发生率,更好地控制病情,改善患者预后。

Polyarteritis nodosa(PAN)is a rare vasculitis that mainly involves small and medium arteries.It often occurs at the points where the vessels bifurcate,leading to microaneurysm formation,thrombosis,aneurysm rupture and bleeding,and infarction of organs.About a third of cases are associated with hepatitis B virus(HBV)infection.All tissues and organs of the body can be affected,with skin,joints and peripheral nerves being the most common.The pathological changes were fibrinoid necrosis,inflammatory cell infiltration and luminal thrombosis in the acute stage,and fibrous hyperplasia in the chronic stage.Overall outcomes for the disease have improved in recent decades,mainly reflecting early diagnosis and more effective treatments.The main treatments for PAN are glucocorticoid and cyclophosphamide.Patients with HBV-associated PAN should receive antiviral therapy and plasma exchange.