当前位置: X-MOL 学术Kidney Int. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
DNAJB9-positive monotypic fibrillary glomerulonephritis is not associated with monoclonal gammopathy in the vast majority of patients.
Kidney International ( IF 19.6 ) Pub Date : 2020-03-28 , DOI: 10.1016/j.kint.2020.02.025
Samar M Said 1 , Nelson Leung 2 , Mariam Priya Alexander 1 , Lynn D Cornell 1 , Mary E Fidler 1 , Joseph P Grande 1 , Loren Hernandez Herrera 1 , Sanjeev Sethi 1 , Pingchuan Zhang 1 , Samih H Nasr 1
Affiliation  

The association of fibrillary glomerulonephritis (FGN) with monoclonal gammopathy has been controversial, although monotypic FGN is currently classified as a monoclonal gammopathy of renal significance (MGRS) lesion. To define this lesion, we correlated findings by immunofluorescence on frozen and paraffin tissue, IgG subtype staining and serum protein electrophoresis with immunofixation in patients with monotypic FGN. Immunofluorescence was performed on paraffin sections from 35 cases of DNAJB9-associated FGN that showed apparent light chain restriction of glomerular IgG deposits by standard immunofluorescence on frozen tissue. On paraffin immunofluorescence, 15 cases (14 lambda and one kappa restricted cases on frozen tissue immunofluorescence) showed no light chain restriction, 19 showed similar light chain restriction, and one was negative for both light chains. Seven of the 15 cases with masked polyclonal deposits also had IgG subclass restriction and these cases would have been diagnosed as a form of monoclonal protein-associated glomerulonephritis if paraffin immunofluorescence was not performed. Monotypic FGN (confirmed by paraffin immunofluorescence and IgG subclass restriction) accounted for only one of 151 (0.7%) patients with FGN encountered during the last two years. Only one of 11 of cases had a detectable circulating monoclonal protein on serum protein electrophoresis with immunofixation. We propose that paraffin immunofluorescence is required to make the diagnosis of lambda-restricted monotypic FGN as it unmasked polytypic deposits in over half of patients. When confirmed by paraffin immunofluorescence and IgG subclass staining, DNAJB9-positive monotypic FGN is very rare and is not associated with monoclonal gammopathy in the vast majority of patients. Thus, there is a question whether this lesion should be included in MGRS-related diseases.



中文翻译:

在绝大多数患者中,DNAJB9 阳性单型纤维性肾小球肾炎与单克隆丙种球蛋白病无关。

纤维性肾小球肾炎 (FGN) 与单克隆丙种球蛋白病的关联一直存在争议,尽管单型 FGN 目前被归类为具有肾脏意义的单克隆丙种球蛋白病 (MGRS) 病变。为了定义这种病变,我们将冷冻和石蜡组织的免疫荧光、IgG 亚型染色和血清蛋白电泳与单型 FGN 患者的免疫固定相关联。对来自 35 例 DNAJB9 相关 FGN 的石蜡切片进行免疫荧光检查,通过冷冻组织上的标准免疫荧光显示肾小球 IgG 沉积物的明显轻链限制。在石蜡免疫荧光中,15 例(冷冻组织免疫荧光中 14 例 λ 和 1 例 kappa 限制病例)显示无轻链限制,19 例显示类似的轻链限制,一个对两条轻链都是阴性的。15 例有隐蔽多克隆沉积的病例中有 7 例也有 IgG 亚类限制,如果不进行石蜡免疫荧光检查,这些病例将被诊断为一种单克隆蛋白相关性肾小球肾炎。单型 FGN(通过石蜡免疫荧光和 IgG 亚类限制证实)仅占过去两年中遇到的 151 名(0.7%)FGN 患者中的一名。11 例病例中只有 1 例在免疫固定的血清蛋白电泳中可检测到循环单克隆蛋白。我们建议需要石蜡免疫荧光来诊断 λ 限制性单型 FGN,因为它揭示了超过一半患者的多型沉积。当通过石蜡免疫荧光和 IgG 亚类染色证实时,DNAJB9 阳性单型 FGN 非常罕见,并且在绝大多数患者中与单克隆丙种球蛋白病无关。因此,存在一个问题,该病变是否应包括在 MGRS 相关疾病中。

更新日期:2020-03-28
down
wechat
bug