Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey.
Department of Nephrology, Hacettepe University School of Medicine, Hacettepe University, Ankara, Turkey.
Int Urol Nephrol. 2023 Mar;55(3):661-669. doi: 10.1007/s11255-022-03357-4. Epub 2022 Aug 29.
We aimed to investigate the immuno-histochemical expression of C4d, ADAM10 and WT1 in kidney biopsies of immunoglobulin A nephropathy (IgAN) patients and correlate the findings with clinical, laboratory and histopathologic features in the hope of defining new parameters to better understand the pathogenesis of the disease, and predict prognosis.
Paraffin-embedded kidney biopsy samples of 128 IgAN patients were immuno-histochemically treated with C4d and ADAM10/WT1 dual stain. Results were evaluated according to Oxford classification parameters, epidemiologic features, laboratory findings at presentation and clinical follow-up.
We observed C4d positivity in 40.6% of our patients, 25% of which was mesangial/peri-mesangial (m/pm) staining. Only m/pmC4d positivity statistically correlated with progression to end-stage renal disease (ESRD). M/pmC4d positive patients had statistically significantly higher baseline proteinuria levels, presence of crescents and > 25% segmental sclerosis of glomeruli. There was cytoplasmic staining of WT1 in 11.2% of cases. Presence of cWT1 correlated with m/pmC4d positivity and progression to ESRD. There was no glomerular ADAM10 detected and tubular expression of this protein did not relate to amount of tubular damage or other parameters.
This study is the first to show that cWT1is involved in IgAN and appears as an independent variable for worse prognosis.
我们旨在研究免疫组织化学 C4d、ADAM10 和 WT1 在免疫球蛋白 A 肾病（IgAN）患者肾活检中的表达，并将这些发现与临床、实验室和组织病理学特征相关联，以期确定新的参数来更好地了解疾病的发病机制，并预测预后。
对 128 例 IgAN 患者的石蜡包埋肾活检样本进行 C4d 和 ADAM10/WT1 双重染色免疫组织化学处理。根据牛津分类参数、流行病学特征、初诊时的实验室发现和临床随访结果评估结果。
我们观察到 40.6%的患者 C4d 阳性，其中 25%为系膜/系膜旁（m/pm）染色。只有 m/pmC4d 阳性与进展为终末期肾病（ESRD）具有统计学相关性。m/pmC4d 阳性患者的基线蛋白尿水平、新月体形成和肾小球>25%节段硬化的发生率均显著更高。在 11.2%的病例中观察到 WT1 的细胞质染色。cWT1 的存在与 m/pmC4d 阳性和进展为 ESRD 相关。未检测到肾小球 ADAM10，而该蛋白的管状表达与肾小管损伤的程度或其他参数无关。
这项研究首次表明 cWT1 参与了 IgAN ，并且是预后较差的独立变量。
