Instituto de Ciências Biológicas e Naturais, Universidade Federal do Triângulo Mineiro, Uberaba, MG, Brasil.
Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil.
J Bras Nefrol. 2020 Mar;42(1):113-117. doi: 10.1590/2175-8239-JBN-2018-0239. Epub 2019 Feb 25.
Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary.
A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II.
In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.
一些膜性肾病（MGN）病例表现为局灶节段性肾小球硬化症（FSGS），通常与疾病进展相关。然而，我们报告了一例患者，其似乎同时患有原发性 MGN 和 FSGS。
一名 17 岁的白人女性，出现下肢水肿，伴有泡沫尿和高血压，体格检查和实验室检查均提示肾病综合征。进行了肾活检，在一些肾小球中观察到局灶和节段性肾小球硬化症，呈尖端病变，而在其他肾小球中则伴有足细胞肥大和足细胞脱落到尿腔中，符合足细胞病变 FSGS。此外，还有伴有“棘突”的增厚毛细血管袢，伴有基底膜不规则，符合 MGN 二期。免疫荧光显示在毛细血管袢中存在细颗粒 IgG、IgG4 和 PLA2R 沉积，在电子显微镜下可见上皮下沉积和足细胞足突消失。这些形态学发现与 FSGS 和 MGN 二期相符。
在本病例中，临床和形态学特征显示原发性 FSGS 和 MGN 可能存在重叠，FSGS 似乎与 MGN 进展无关，而是与足细胞病变 FSGS 有关。
