霉酚酸酯治疗ANCA阳性重型狼疮性肾炎的初步临床观察

TREATMENT OF ANCA POSITIVE DIFFUSE PROLIFERATIVE LUPUS NEPHRITIS WITH MYCOPHENOLATE MOTEFIL(A REPORT OF THREE CASES)

目的 :观察霉酚酸酯 (MMF)治疗血清抗中性粒细胞胞浆抗体 (ANCA)阳性Ⅳ型狼疮性肾炎 (Ⅳ LN)的疗效。 方法 :采用激素联合MMF( 1 0～ 1 5 g/d)治疗临床确诊为系统性红斑狼疮 ,病理类型为Ⅳ LN ,血清ANCA阳性的 3例患者 ,随访 6～ 9个月 ,观察肾脏和肾外脏器损害的变化。 结果 :3例患者的血清ANCA ,荧光法检测均阴性 ,ELISA法测定均为抗髓过氧化酶抗体 (MPO ANCA)阳性。均有大量蛋白尿和血尿 ,并伴严重肾外脏器损伤 ,1例患者血清肌酐升高。肾活检显示不同程度肾小球毛细血管袢坏死及新月体形成、间质血管炎。经激素联合MMF治疗后 ,尿蛋白均显著减少 ( 1例尿蛋白转阴 ) ,血尿均显著下降。 2例原有大量心包积液者治疗后消失。血清MPO ANCA和抗 dsDNA均转为阴性。 2例患者治疗 6个月时重复肾活检 ,肾小球新月体、袢坏死和间质血管炎病变均消失。肾脏活动指数 (AI)分别由 13分、2 0分降至 5分和 2分。 结论 :初步临床观察显示MMF对ANCA阳性的Ⅳ LN有较好疗效 。

OBJECTIVE Lupus nephritis(LN) with positive serum anti neutrophil cytoplasmic antibody(ANCA) usually has severe renal involvement unresponsive to traditional immunosuppressive treatment. Here we report our experience in the treatment of serum ANCA positive diffuse proliferative LN (LN Ⅳ) with mycophenolate mofetil(MMF). METHODOLOGY Three cases(2 female,1 male) of biopsy proven LN Ⅳ with positive serum MPO ANCA were included.The regime of treatment consists of pulse methylprednisonlone(2 3 time/d) followed by oral prednison(45mg/d×4 weeks,then gradually tampered to 15 20 mg/day) and MMF(1 0 1 5g/d).All the three patients were followed for 6 9 months. RESULTS All the three patients had severe proteinuria, hematuria and extre renal involvement. Renal biopsy showed segmental glomerular necrosis with crescent formation(25%～40%) and small vasculitis. After treatment with steroids and MMF,proteinuria were significantly reduced in all the 3 patients,one of whom got complete remission after 3months’ treatment.Both serum MPO ANCA and anti dsDNA became negative after treatment.Repeated renal biopsy in two patients after 3 months’ treatment showed that glomerular necrosis, crescents and active vasculitis were significantly diminished.Active index decreased from 13 and 20 to 5 and 2 respectively. CONCLUSION This preliminary study showed that MMF was effective for ANCA positive severe lupus nephritis,but further clinical studies are needed.