Membranoproliferative Glomerulonephritis Secondary to Monoclonal Gammopathy

Membranoproliferative Glomerulonephritis Secondary to Monoclonal Gammopathy
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DOI:
10.2215/cjn.06760909
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发表时间:
2010-05-01
影响因子:
9.8
通讯作者:
Fervenza, Fernando C.
Fervenza, Fernando C.
中科院分区:
医学1区
文献类型:
--
作者:
Sethi, Sanjeev;Zand, Ladan;Fervenza, Fernando C.

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背景和目标:膜增生性肾小球肾炎(MPGN)是一种免疫复合物介导的肾小球肾炎,其特征是免疫复合物沉积于内皮下和系膜下。自身免疫性疾病和慢性感染,如丙型肝炎,是公认的MPGN的原因,但是,单克隆丙种球蛋白病是一个不太广泛认识的原因MPGN.Design,设置,参与者和测量:我们回顾了所有肾活检的MPGN患者在马约诊所在6年期间,以确定单克隆丙种球蛋白病与MPGN的关联。结果:126例MPGN患者中,20例未接受过B或C型肝炎检查。其余106例患者中,25例(23.5%)为B或C型肝炎阳性。在81例肝炎阴性患者中,13例未评价丙种球蛋白病。在其余68例患者中,28例(41.1%)的血清和/或尿液电泳研究单克隆丙种球蛋白病阳性。血清免疫固定电泳是诊断单克隆丙种球蛋白病最敏感的方法。肾活检显示膜增生性损伤模式,免疫荧光显微镜往往有助于诊断潜在的丙种球蛋白病。根据骨髓活检,意义不明的单克隆丙种球蛋白病是MPGN最常见的相关疾病。其他,不太常见的原因包括多发性骨髓瘤,低级别B细胞淋巴瘤,慢性淋巴细胞白血病。结论:单克隆丙种球蛋白病是一个重要的和常见的原因MPGN,因此,所有患者的诊断MPGN应评估为基础的单克隆丙种球蛋白病。Clin J Am Soc Nephrol 5:770-782,2010. doi:10.2215/CJN.06760909
Background and objectives: Membranoproliferative glomerulonephritis (MPGN) is an immune complex mediated glomerulonephritis characterized by subendothelial and mesangial deposition of immune complexes. Autoimmune diseases and chronic infections, such as hepatitis C, are commonly recognized causes of MPGN; however, monoclonal gammopathy is a less widely recognized cause of MPGN.Design, setting, participants, & measurements: We reviewed all renal biopsies of MPGN in Mayo Clinic patients during a 6-year period to determine the association of monoclonal gammopathy with MPGN. Results were correlated with electrophoresis studies and bone marrow biopsies to clarify the relationship between MPGN and gammopathies.Results: Of 126 patients with MPGN, 20 did not have workup for hepatitis B or C. Of the remaining 106 patients, 25 (23.5%) were positive for hepatitis B or C. Of the 81 hepatitis-negative patients, 13 were not evaluated for gammopathies. Of the remaining 68 patients, 28 (41.1%) had serum and/or urine electrophoresis studies positive for monoclonal gammopathy. Serum immunofixation electrophoresis was the most sensitive method for diagnosing monoclonal gammopathy. Renal biopsy showed a membranoproliferative pattern of injury; immunofluorescence microscopy was often instrumental in diagnosing the underlying gammopathy. On the basis of the bone marrow biopsy, monoclonal gammopathy of undetermined significance was the most common entity associated with MPGN. Other, less common causes included multiple myeloma, low-grade B cell lymphoma, and chronic lymphocytic leukemia.Conclusions: Monoclonal gammopathy is an important and common cause of MPGN; therefore, all patients with a diagnosis of MPGN should be evaluated for an underlying monoclonal gammopathy. Clin J Am Soc Nephrol 5: 770-782, 2010. doi: 10.2215/CJN.06760909