Boki K A, Dafni U, Karpouzas G A, Papasteriades C, Drosos A A, Moutsopoulos H M
Department of Pathophysiology, School of Medicine, National University of Athens, Greece.
Br J Rheumatol. 1997 Oct;36(10):1059-66. doi: 10.1093/rheumatology/36.10.1059.
The clinical spectrum and outcome of necrotizing vasculitis were evaluated in a retrospective study in order to assess: (1) the clinical expression and evolution of the disease; (2) the response to several therapeutic approaches based on major events (organ involvement causing disability or death); (3) the immunogenetic background of patients. Sixty-six Greek patients fulfilling the ACR criteria for the diagnosis of vasculitis entered the study. Thirty-seven were diagnosed with Wegener's granulomatosis (WG), 22 with polyarteritis nodosa (PAN) and seven with Churg-Strauss syndrome (CSS). The demographic characteristics of patients with WG and PAN were similar. Cutaneous manifestations, gastrointestinal and peripheral nervous system involvement occurred more often in patients with PAN, whereas pulmonary and upper respiratory tract involvement, renal, ear abnormalities and fever were more frequent in patients with WG. Muscle weakness and asthma were found exclusively in patients with PAN and CSS, respectively, while the presence of classic-antineutrophil cytoplasmic antibodies (c-ANCA) characterized WG patients. Hepatitis B surface antigen (HBsAg) was found in 22% of PAN patients. No significant differences were detected when comparing the PAN and WG groups with respect to the first major event (log-rank P = 0.50) with and without potential confounders (age, gender, therapy or c-ANCA). For WG patients, a statistically significant difference was found on different routes of administration of cyclophosphamide (oral vs pulse) (P = 0.006). Regarding the HLA antigens, an increased frequency of DR1 (26.9% vs 10.3%, P = 0.057) in WG and the absence of DR3 in patients with PAN and CSS were noted. It appears that although the immunogenetic background and the clinical expression of the diseases differ, the response to treatment as well as the evolution and the survival rate of these patients are similar in the two groups.
为了评估以下方面，在一项回顾性研究中对坏死性血管炎的临床谱和结局进行了评估：(1) 疾病的临床表现和演变；(2) 基于重大事件（导致残疾或死亡的器官受累）对几种治疗方法的反应；(3) 患者的免疫遗传背景。66 名符合美国风湿病学会（ACR）血管炎诊断标准的希腊患者进入了该研究。37 名被诊断为韦格纳肉芽肿（WG），22 名被诊断为结节性多动脉炎（PAN），7 名被诊断为变应性肉芽肿性血管炎（CSS）。WG 和 PAN 患者的人口统计学特征相似。皮肤表现、胃肠道和周围神经系统受累在 PAN 患者中更常见，而肺部和上呼吸道受累、肾脏、耳部异常和发热在 WG 患者中更频繁。肌肉无力和哮喘分别仅在 PAN 和 CSS 患者中发现，而典型抗中性粒细胞胞浆抗体（c-ANCA）的存在是 WG 患者的特征。22% 的 PAN 患者发现乙肝表面抗原（HBsAg）。在比较 PAN 和 WG 组的第一个重大事件时（对数秩检验 P = 0.50），无论有无潜在混杂因素（年龄、性别、治疗或 c-ANCA），均未检测到显著差异。对于 WG 患者，在环磷酰胺的不同给药途径（口服与脉冲）上发现了统计学上的显著差异（P = 0.006）。关于 HLA 抗原，注意到 WG 中 DR1 的频率增加（26.9% 对 10.3%，P = 0.057），而 PAN 和 CSS 患者中没有 DR3。似乎尽管这些疾病的免疫遗传背景和临床表现不同，但两组患者对治疗的反应以及疾病的演变和生存率相似。
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