局限型和严重型韦格纳肉芽肿病的临床特征分析

Limited and severe Wegener's granulomatosis:clinical features

目的比较局限型韦格纳肉芽肿病(WG)与严重型WG患者的临床特征、实验室检查和对治疗反应的差异。方法回顾性比较14例局限型WG与15例严重型WG患者临床特征、实验室检查和治疗反应之间的差异。结果与严重型WG比较,局限型WG患者发病年龄小[(41±14)岁与(62±12)岁,P<0.01],女性所占比例高(64%与22%,P=0.033)。严重型WG贫血发生率高(87%与43%,P=0.013),红细胞沉降率高的比例大(93%与64%,P=0.046)、抗中性粒细胞胞质抗体(ANCA)阳性率高(57%与93%,P=0.023)、胞质型抗中性粒细胞胞质抗体(cANCA)阳性率高(43%与80%,P=0.039)、蛋白酶3(PR3)阳性率高(36%与80%,P=0.016)。局限型WG对标准方案的诱导缓解率高(87%与46%,P=0.027),并且平均诱导缓解时间短[(5.6±0.7)个月与(11.5±3.4)个月P<0.01]。结论局限型WG和重型WG在年龄、性别和临床特征方面有差异。标准治疗对局限型WG的诱导缓解率高,诱导缓解期短。严重型WG应选择更积极的免疫抑制治疗。

Objective To compare the clinical and laboratory features and treatment response between limited and severe Wegener＇s granulomatosis（WG）.Methods All available data from patients with a confirmed diagnosis of limited WG（n=14） and severe WG（n=15） were retrospectively reviewed.Results There were significant differences between patients with limited WG and those with severe WG with regard to age（41±14 vs 62±12 yr,P〈0.001） and female gender（64% vs 22%,P=0.033）.The prevalence of anemia（43% vs 87%,P=0.013） was significantly lower in limited than in severe WG.More severe WG cases were found to be associated with increased erythrocyte sedimentation rate（93% vs 64%,P=0.046）,positive ANCA rate（93% vs 57%,P=0.023）,positive cANCA rate（80% vs 43%,P= 0.039） and positive PR3 rate（80% vs 36%,P=0.016）.The remission induction rate was higher in limited WG than that in severe WG（87%vs 46%,P=0.027）.The mean time to remission induction in severe WG was longer than that in limited WG（5.6±0.7 vs 11.5±3.4 months,P〈0.01）.Conclusion There were significant differences between patients with limited WG and those with severe WG with regard to age,sex and clinical features.Limited WG showed a higher rate of remission induction and a shorter duration of remission induction than as for severe WG with standard treatment.More aggressive immunodepressant should be employed in severe WG.