Department of Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France.
Vasculitides and Scleroderma, Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Université Paris Descartes, 27, rue Fg Saint-Jacques, Paris 75679 Cedex 14, France.
Neurol Clin. 2019 May;37(2):345-357. doi: 10.1016/j.ncl.2019.01.007. Epub 2019 Mar 16.
Polyarteritis nodosa (PAN) is a necrotizing vasculitis affecting medium-sized vessels whose main manifestations are weight loss, fever, peripheral neuropathy, renal, musculoskeletal, gastrointestinal tract and/or cutaneous involvement(s), hypertension and/or cardiac failure. Peripheral neuropathy is one of the most frequent and earliest symptoms, affecting 50% to 75% of PAN patients. Central nervous system involvement affects only 2% to 10% of PAN patients, often late during the disease course. Treatment relies on combining corticosteroids and an immunosuppressant (mainly cyclophosphamide) in patients with poor prognoses. In patients with hepatitis B virus-related PAN, plasma exchanges and antiviral drugs should be combined with corticosteroids.
结节性多动脉炎(PAN)是一种影响中等大小血管的坏死性血管炎,其主要表现为体重减轻、发热、周围神经病、肾脏、肌肉骨骼、胃肠道和/或皮肤受累、高血压和/或心力衰竭。周围神经病是最常见和最早的症状之一,影响 50% 至 75%的 PAN 患者。中枢神经系统受累仅影响 2% 至 10%的 PAN 患者,通常在疾病后期。对于预后不良的患者,治疗依赖于将皮质类固醇和免疫抑制剂(主要是环磷酰胺)联合使用。对于乙型肝炎病毒相关的 PAN 患者,应将血浆置换和抗病毒药物与皮质类固醇联合使用。