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血管炎性肾小球肾炎与膜性肾病的关联:10例报告

Association of vasculitic glomerulonephritis with membranous nephropathy: a report of 10 cases.

作者信息

Tse W Y, Howie A J, Adu D, Savage C O, Richards N T, Wheeler D C, Michael J

机构信息

Department of Nephrology, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK.

出版信息

Nephrol Dial Transplant. 1997 May;12(5):1017-27. doi: 10.1093/ndt/12.5.1017.

Abstract

BACKGROUND

The concomitant occurrence of a vasculitic glomerulonephritis and membranous nephropathy in the same patient is unusual. We report data on 10 patients with this unusual combination.

METHODS

Ten patients (nine males/one female; median age 63.5 years, range 30-70 years) presented between 1981 and 1995 with: acute renal failure (n = 3), nephrotic syndrome (n = 4), non-nephrotic range proteinuria and renal insufficiency (n = 3). The median serum creatinine at presentation was 296 mumol/l (range 65-1749 mumol/l). One patient had a vasculitic transformation from membranous nephropathy 5 years after the original presentation, coincident with an acute deterioration of renal function requiring dialysis; in all other patients the two glomerular disorders were seen together at presentation. Treatment was with oral prednisolone and cyclophosphamide (eight patients), of whom one also had plasma exchange; and oral prednisolone and azathioprine (one patient). Specific immunosuppressive treatment was withheld in one patient with histological evidence of chronic renal damage. Sera from four patients out of nine tested were positive for ANCA.

RESULTS

After a median follow-up of 3.5 years (range 2 months-10 years), renal function had improved in three patients and remained stable in two. Two patients required renal replacement therapy. Three patients had died: one was ANCA-negative and died of a systemic vasculitis, and the other two died of sepsis.

CONCLUSION

Membranous nephropathy complicated by a vasculitic glomerulonephritis: (1) has a more aggressive clinical course than membranous nephropathy alone, (2) appears to have an association with ANCA, (3) should be considered in those patients with an accelerated decline in renal function, and (4) may respond to treatment with immunosuppressive drugs.

摘要

背景

同一患者同时发生血管炎性肾小球肾炎和膜性肾病的情况并不常见。我们报告了10例这种不寻常组合的患者的数据。

方法

10例患者(9例男性/1例女性;中位年龄63.5岁,范围30 - 70岁)于1981年至1995年间就诊,表现为:急性肾衰竭(n = 3)、肾病综合征(n = 4)、非肾病范围蛋白尿和肾功能不全(n = 3)。就诊时血清肌酐中位数为296 μmol/l(范围65 - 1749 μmol/l)。1例患者在最初发病5年后发生了从膜性肾病到血管炎的转变,同时肾功能急性恶化需要透析;在所有其他患者中,两种肾小球疾病在就诊时同时出现。8例患者接受口服泼尼松龙和环磷酰胺治疗,其中1例还进行了血浆置换;1例患者接受口服泼尼松龙和硫唑嘌呤治疗。1例有慢性肾损伤组织学证据的患者未进行特异性免疫抑制治疗。9例检测患者中有4例血清ANCA阳性。

结果

中位随访3.5年(范围2个月 - 10年)后,3例患者肾功能改善,2例保持稳定。2例患者需要肾脏替代治疗。3例患者死亡:1例ANCA阴性,死于系统性血管炎,另外2例死于败血症。

结论

膜性肾病合并血管炎性肾小球肾炎:(1)临床病程比单纯膜性肾病更具侵袭性,(2)似乎与ANCA有关,(3)对于肾功能加速下降的患者应予以考虑,(4)可能对免疫抑制药物治疗有反应。

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