原发系统性抗中性粒细胞胞质抗体阴性小血管炎的临床病理表现

Clinical and renal pathological features of anti-neutrophil cytoplasmic antibodies-negative primary systemic small blood vessel vasculitis

目的总结分析原发系统性小血管炎(PSV)抗中性粒细胞胞质抗体(ANCA)阴性小血管炎患者的临床病理特点,并与ANCA阳性患者进行比较,以提高对该类疾病的认识。方法回顾性总结并分析我科近7年来确诊的13例原发系统性ANCA阴性小血管炎的临床病理资料,并与同期30例ANCA阳性原发性小血管炎患者进行比较。结果ANCA阴性小血管炎组患者13例,占同期诊断的原发性小血管炎患者的7.14%。与ANCA阳性组相比,阴性组皮肤和消化道受损比率显著增高,而发热及中重度贫血发生率低于阳性组,阴性组蛋白尿水平高于阳性组,两组血管炎活动积分无差异。阴性组无一例血免疫球蛋白G升高,与阳性组相比差异有统计学意义(0vs50%,P<0.01)。阴性组肾脏新月体形成发生率低于阳性组,坏死和间质小管病变两组差异无统计学意义。两组免疫抑制治疗方案和临床缓解率差异无统计学意义。结论原发性ANCA阴性小血管炎并不罕见,毛细血管襻纤维素样坏死和无免疫球蛋白G升高有助于诊断。

Objective Primary systemic small blood vessel vasculitis （PSV） including Wegener＇s granulomatosis and microscopic polyangiitis are usually associated with anti-neutrophil cytoplasmic antibodies （ANCAs）. However, ANCA＇s are absent in some of cases, which constitutes a rarely studied variant of vasculitis. This study aims to analyze the clinical and renal pathological features of ANCA-negative PSV. Methods Patients with PSV who were admitted to the hospital in the past 7 years were retrospectively studied. The clinical and renal pathological features were compared between ANCA-negative and positive PSV patients. Results The data of this study showed that ANCA-negative patients accounted for 7.14% of the whole PSV patients. Compared with 30 ANCA-positive PSV patients, 13 patients with ANCA-negative PSV had lower prevalence of fever and anaemia but higher skin and gastrointestinal involement. The Birmingham vasculitis activity scores were compatible in the two groups. There was no case with increased level of serum IgG in ANCA-negative groups （0 vs 50%, P〈0.01）. Renal biopsy revealed less crescent formation in ANCA-negative groups than in ANCA-positive groups. The treatment regimens and prognosis had no difference in the two groups. Conclusion Patients with ANCA-negative PSV are not rare. The glomerular necrosis and normal or lo4 level of serum IgG are helpful for the diagnosis of ANCA-negative PSV.