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具有“狼疮样”特征的HIV相关性免疫复合物性肾小球肾炎:14例临床病理研究

HIV-associated immune complex glomerulonephritis with "lupus-like" features: a clinicopathologic study of 14 cases.

作者信息

Haas Mark, Kaul Sadhana, Eustace Joseph A

机构信息

Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.

出版信息

Kidney Int. 2005 Apr;67(4):1381-90. doi: 10.1111/j.1523-1755.2005.00215.x.

Abstract

BACKGROUND

While the most common glomerular lesion associated with human immunodeficiency virus (HIV) infection is collapsing focal segmental glomerulosclerosis (FSGS) [HIV-associated nephropathy (HIVAN)], immune complex-mediated forms of glomerulonephritis have been increasingly reported. One form of glomerulonephritis that has been described in the HIV-infected population is immune complex glomerulonephritis with "lupus-like" features, characterized by histologic, immunohistologic, and ultrastructural features resembling lupus nephritis, but occurring in patients without evidence of systemic lupus erythematosus (SLE). Data regarding clinical outcomes in patients with this form of glomerulonephritis are very limited.

METHODS

We reviewed pathology reports for all native renal biopsy specimens from HIV-positive patients processed at our center from January 1999 through December 2003. Of 77 total specimens, 14 met the following criteria for lupus-like glomerulonephritis: (1) immunofluorescence microscopy showed granular glomerular staining for IgG, IgA, IgM, C3 and C1q, with > or=1+ (0 to 4+ scale) staining for C1q; and (2) the patient's serum was negative for antinuclear antibodies (ANA), or weakly positive (titer < or =1:80) for ANA and negative for antidouble-stranded DNA.

RESULTS

Clinically, ten of the 14 patients with lupus-like glomerulonephritis presented with nephrotic syndrome, all had microscopic hematuria, and nine had serum creatinine >3.0 mg/dL. All but one were African American. Histologically, seven biopsies showed diffuse proliferative glomerulonephritis, six focal proliferative glomerulonephritis, and one membranous nephropathy. All but two biopsies showed moderate or severe chronic change, and three showed concurrent HIVAN. Ten of the 14 patients developed end-stage renal disease (ESRD) within 1 year of the biopsy. Nine of these ten patients presented with proteinuria >5.0 g/24 hours and nephrotic syndrome, while three of four patients who did not develop ESRD had proteinuria < or =3.0 g/24 hours.

CONCLUSION

Lupus-like glomerulonephritis, defined by immunohistologic features and absence of serologic evidence of SLE, is not an uncommon form of glomerular disease in HIV-infected patients undergoing a renal biopsy. Renal outcomes in these patients were poor, although this may be due largely to most patients presenting with advanced disease.

摘要

背景

虽然与人类免疫缺陷病毒(HIV)感染相关的最常见肾小球病变是塌陷型局灶节段性肾小球硬化(FSGS)[HIV相关性肾病(HIVAN)],但免疫复合物介导的肾小球肾炎形式的报道日益增多。在HIV感染人群中描述的一种肾小球肾炎形式是具有“狼疮样”特征的免疫复合物性肾小球肾炎,其组织学、免疫组织学和超微结构特征类似于狼疮性肾炎,但发生在无系统性红斑狼疮(SLE)证据的患者中。关于这种形式的肾小球肾炎患者临床结局的数据非常有限。

方法

我们回顾了1999年1月至2003年12月在我们中心处理的所有HIV阳性患者的肾活检标本的病理报告。在77份标本中,14份符合以下狼疮样肾小球肾炎标准:(1)免疫荧光显微镜显示肾小球IgG、IgA、IgM、C3和C1q呈颗粒状染色,C1q染色≥1+(0至4+分级);(2)患者血清抗核抗体(ANA)阴性,或ANA弱阳性(滴度≤1:80)且抗双链DNA阴性。

结果

临床上,14例狼疮样肾小球肾炎患者中有10例表现为肾病综合征,均有镜下血尿,9例血清肌酐>3.0mg/dL。除1例外均为非裔美国人。组织学上,7例活检显示弥漫性增生性肾小球肾炎,6例局灶性增生性肾小球肾炎,1例膜性肾病。除2例活检外,均显示中度或重度慢性改变,3例同时存在HIVAN。14例患者中有10例在活检后1年内发展为终末期肾病(ESRD)。这10例患者中有9例蛋白尿>5.0g/24小时且患有肾病综合征,而4例未发展为ESRD的患者中有3例蛋白尿≤3.0g/24小时。

结论

由免疫组织学特征定义且无SLE血清学证据的狼疮样肾小球肾炎,在接受肾活检的HIV感染患者中并非罕见的肾小球疾病形式。这些患者的肾脏结局较差,尽管这可能主要归因于大多数患者就诊时已处于疾病晚期。

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