"Pauci-immune" proliferative and necrotizing glomerulonephritis with thrombotic microangiopathy in patients with systemic lupus erythematosus and lupus-like syndrome
"Pauci-immune" proliferative and necrotizing glomerulonephritis with thrombotic microangiopathy in patients with systemic lupus erythematosus and lupus-like syndrome
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DOI：
10.1016/s0272-6386(00)70058-8
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发表时间：
2000-06-01
影响因子：
13.2
通讯作者：
Nadasdy, T
中科院分区：
文献类型：
--
作者：
Charney, DA;Nassar, G;Nadasdy, T
在系统性红斑狼疮（SLE）的肾小球肾炎中，如果存在内皮下沉积物，其数量通常与光镜下肾小球细胞过多的程度相对应;只有极少数情况下，没有或很少这样的存款存在的情况下，（WHO III类）或弥漫性（WHO IV类）增生性狼疮性肾炎，我们最近遇到五例活动性弥漫增生性肾小球肾炎，没有内皮下和很少或没有系膜沉积和血栓性微血管病（TMA）4例SLE患者和1例狼疮样综合征患者。五名患者中有三名进行了循环狼疮抗凝剂或抗心磷脂抗体检测，其中两名呈阳性。所有5名患者的抗神经细胞胞浆抗体（ANCA）检测均为阴性。3例患者对类固醇和环磷酰胺治疗有反应，尽管其中1例死于急性细菌性支气管肺炎，1例患者失访。我们的结论是，“少免疫”增生性狼疮肾炎是罕见的，应作为增生性狼疮肾炎与一定比例的内皮下存款。ANCA阴性提示这些病例并不代表SLE患者中偶发的ANCA相关的少免疫性坏死性和新月体性肾小球肾炎。特别值得注意的是，在SLE患者中，如果与TMA相关，即使没有显著的肾小球免疫复合物沉积，也可能存在活动性增殖性坏死性肾小球肾炎。(C)2000年由国家肾脏基金会，公司。
In the glomerulonephritides of systemic lupus erythematosus (SLE), the number of subendothelial deposits, when present, generally corresponds to the degree of light microscopic glomerular hypercellularity; only very rarely are no or few such deposits present in cases of focal (WHO class III) or diffuse (WHO class IV) proliferative lupus nephritis, We have recently encountered five cases of active diffuse proliferative glomerlonephritis with no subendothelial and few or no mesangial deposits and thrombotic microangiopathy (TMA) in four patients with SLE and one patient with lupus-like syndrome. Three of the five patients were tested for circulating lupus anticoagulants or anticardiolipin antibodies, and two were positive. All five patients tested negatively for antineutrophil cytoplasmic antibodies (ANCA). Three patients responded to steroid and cyclophosphamide treatment, although one of them died of acute bacterial bronchopneumonia, One patient was lost to follow-up. We conclude that "pauci-immune" proliferative lupus nephritis is rare and should be treated as proliferative lupus nephritis with a proportionate number of subendothelial deposits. The negative ANCA suggests that these cases do not represent incidental ANCA-associated pauci-immune necrotizing and crescentic glomerulonephritis in patients with SLE, Of particular interest is that, in patients with SLE, if associated with TMA, an active proliferative necrotizing glomerulonephritis may be present even in the absence of significant glomerular immune complex deposition. (C) 2000 by the National Kidney Foundation, Inc.