伴MPO-ANCA阳性的狼疮性肾炎的临床和病理特点（附18例报告）

目的：分析伴髓过氧化物酶-抗中性粒细胞胞质抗体（MPO-ANCA）阳性的狼疮性肾炎（LN）患的临床和病理特点。方法：回顾分析18例伴MPO-ANCA阳性LN患的临床和病理资料，并将不同临床转归患各项资料进行对比。结果：这18例伴MPO-ANCA阳性LN患的临床和病理表现有以下特点；（1）均有肾外病变，突出表现为血液系统病变，高血压和浆膜腔炎；（2）肾脏病变主要为蛋白尿伴血尿，肉眼血尿和肾功能不全发生率较高；（3）多种自身抗体阳性，冷球蛋白血症发生率也较高；（4）肾脏病理，多数患为Ⅳ型LN，表现为节段坏死性新月体性肾炎伴肾小球免疫复合物沉积和间质血管炎，（5）预后情况，终末期肾衰（ESRF）发生率和死亡率与一般LN相近，预后差的患肾小管萎缩的发生率显高于存活患。结论：MPO-ANCA阳必的LN患有别于一般LN，但MPO-ANCA阳性可能与患的预后无直接相关。

Clinical and pathologic features of lupus nephritis patients with positive MPO-ANCA: a report of 18 cases

Objective:To study the clinical and pathologic features of patients with lupus nephritis (LN) whose myeloperoxidase anti neutrophil cytoplasmic antibody (MPO ANCA) were positive. Methods:The clinical and pathological features were analyzed in 18 patients with LN whose MPO ANCA were positive. And the data of patients with different clinical outcomes were compared. Results:(1)The hematological abnormalities, hypertension and serositis in these patients were more common than general ones with LN. (2)Proteinuria and hematuria were common, the morbidities of gross hematuria and renal failure in these patients were higher than general ones with LN.(3)Various autoantibodies were positive in these patients.(4)Segmental necrosis crescentic nephritis accompanied by density of immunocomplex in glomeruli and vasculitis in intestitium were common.(5)The morbidity of ESRF and mortality of these patients were similar to general ones with LN. The morbidity of tubular atrophy in those with poor prognosis was significantly higher than those survived. Conclusion:The patients with LN whose MPO ANCA are positive have some difference from those with negative MPO ANCA, but positive MPO ANCA is not directly related to the prognosis. [