Pagnoux Christian, Seror Raphaèle, Henegar Corneliu, Mahr Alfred, Cohen Pascal, Le Guern Véronique, Bienvenu Boris, Mouthon Luc, Guillevin Loïc
Université Paris Descartes, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France.
Arthritis Rheum. 2010 Feb;62(2):616-26. doi: 10.1002/art.27240.
Previous studies of polyarteritis nodosa (PAN) included patients with microscopic polyangiitis, because these entities were not distinguished prior to the Chapel Hill Consensus Conference (CHCC). This study was undertaken to describe the main characteristics of and long-term outcomes in patients with well-characterized PAN diagnoses.
We conducted a systematic retrospective study of 348 patients who were diagnosed as having PAN between March 1963 and October 2005, were registered in the French Vasculitis Study Group database, and satisfied the American College of Rheumatology and CHCC criteria. Patient characteristics and outcomes were analyzed and compared according to hepatitis B virus (HBV) status.
At diagnosis, the mean +/- SD age was 51.2 +/- 17.3 years. The most frequent findings were general symptoms (93.1%), neurologic manifestations (79%), skin involvement (49.7%), abdominal pain (35.6%), and hypertension (34.8%); 66.2% had renal artery microaneurysms; 70.1% had histologically proven PAN. Patients with HBV-related PAN (n = 123) had more frequent peripheral neuropathy, abdominal pain, cardiomyopathy, orchitis, and hypertension compared with patients with non-HBV-related PAN (n = 225). During a mean +/- SD followup of 68.3 +/- 63.5 months, 76 patients (21.8%) relapsed (63 with non-HBV-related PAN [28%] versus 13 with HBV-related PAN [10.6%]; P < 0.001); 86 patients (24.7%) died (44 with non-HBV-related PAN [19.6%] versus 42 with HBV-related PAN [34.1%]; P = 0.003). Five-year relapse-free survival rates were 59.4% (95% confidence interval [95% CI] 52.6-67.0) versus 67.0% (95% CI 58.5-76.8) for non-HBV-related PAN and HBV-related PAN, respectively. Multivariate analysis retained age >65 years, hypertension, and gastrointestinal manifestations requiring surgery or at least consultation with a surgeon as independent predictors of death, whereas patients with cutaneous manifestations or non-HBV-related PAN had a higher risk of relapse.
Our findings indicate that the rate of mortality from PAN remains high, especially for the elderly, and relapses do occur, particularly in patients with non-HBV-related PAN with cutaneous manifestations.
结节性多动脉炎（PAN）既往研究纳入了显微镜下多血管炎患者，因为在查珀尔希尔共识会议（CHCC）之前这些疾病实体未被区分。本研究旨在描述确诊为PAN患者的主要特征和长期预后。
我们对1963年3月至2005年10月期间被诊断为PAN、登记在法国血管炎研究组数据库且符合美国风湿病学会和CHCC标准的348例患者进行了系统回顾性研究。根据乙型肝炎病毒（HBV）状态分析并比较患者特征和预后。
诊断时，平均±标准差年龄为51.2±17.3岁。最常见的表现为全身症状（93.1%）、神经表现（79%）、皮肤受累（49.7%）、腹痛（35.6%）和高血压（34.8%）；66.2%有肾动脉微动脉瘤；70.1%经组织学证实为PAN。与非HBV相关PAN患者（n = 225）相比，HBV相关PAN患者（n = 123）周围神经病变、腹痛、心肌病、睾丸炎和高血压更为常见。在平均±标准差68.3±63.5个月的随访期间，76例患者（21.8%）复发（非HBV相关PAN患者63例[28%]，HBV相关PAN患者13例[10.6%]；P < 0.001）；86例患者（24.7%）死亡（非HBV相关PAN患者44例[19.6%]，HBV相关PAN患者42例[34.1%]；P = 0.003）。非HBV相关PAN和HBV相关PAN的5年无复发生存率分别为59.4%（95%置信区间[95%CI]52.6 - 67.0）和67.0%（95%CI 58.5 - 76.8）。多因素分析显示年龄>65岁、高血压以及需要手术或至少咨询外科医生的胃肠道表现是死亡的独立预测因素，而有皮肤表现的患者或非HBV相关PAN患者复发风险更高。
我们的研究结果表明，PAN的死亡率仍然很高，尤其是老年人，并且确实会复发，特别是在有皮肤表现的非HBV相关PAN患者中。
