Lionaki Sophia, Marinaki Smaragdi, Panagiotellis Konstantinos, Tsoumbou Ioanna, Liapis George, Vlahadami Ioanna, Tzioufas Athanasios, Sfikakis Petros, Boletis Ioannis
Nephrology Department & Transplantation Unit, Faculty of Medicine, Laiko Hospital, National & Kapodistrian University of Athens, 11527 Athens, Greece.
Pathology Department, Faculty of Medicine, Laiko Hospital, National & Kapodistrian University of Athens, 11527 Athens, Greece.
Antibodies (Basel). 2020 May 25;9(2):18. doi: 10.3390/antib9020018.
Glomerular diseases (GD) associated with malignancies (AM, GDAM) have unique features, which are important to recognize, in the light of the progress made in cancer therapy. We aimed to describe the clinical and histopathological characteristics of patients with GDAM in relation to the presence of circulating autoantibodies, pointing to potential immune pathogenic pathways connecting cancer to GD.
The included patients were studied retrospectively on the basis of a kidney biopsy proving GD and a related biopsy to establish the diagnosis of AM. We recorded patients' demographics, serological and laboratory parameters, histopathological findings, and the type of malignancy, GD, and therapy.
In total, 41 patients with GDAM, with a mean age of 63.1 (±10.7) years, were studied. In 28 (68.3%) cases, GD was associated with a solid tumor, and in 13 (31.7%) patients with a lymphoid malignancy. The most frequent histopathological pattern was membranous nephropathy (43.9%). Overall, at the time of GD diagnosis, 17% of the patients were positive for antinuclear antibodies (ANA), and 12.2% for antineutrophil cytoplasmic autoantibodies (ANCA), all against myeloperoxidase (MPO). In addition, 93.3% of the patients who had membranous nephropathy were negative for transmembrane glycoprotein M-type phospholipase A receptor (PLAR) antibody. Sixteen patients (39.0%) presented with acute nephritic syndrome, of whom five (31.25%) developed rapidly progressive glomerulonephritis. In a mean follow-up time of 36.1 (±28.3) months, nine (21.95%) patients ended up with end-stage kidney disease, and eight (19.5%) died.
We found that 3.2% of patients who underwent a native kidney biopsy in our institution during the past decade, for any reason, were identified as having some type of GD associated with a malignancy. Serology indicated a significant presence of ANA or MPO-ANCA antibodies in patients with nephritic syndrome and the absence of PLAR antibodies in patients with membranous nephropathy.
鉴于癌症治疗取得的进展，与恶性肿瘤相关的肾小球疾病（AM，肾小球疾病伴发恶性肿瘤，GDAM）具有独特特征，认识这些特征很重要。我们旨在描述GDAM患者的临床和组织病理学特征，并探讨循环自身抗体的存在情况，以指出将癌症与GD联系起来的潜在免疫致病途径。
纳入的患者基于肾活检确诊为GD以及相关活检确诊为AM进行回顾性研究。我们记录了患者的人口统计学信息、血清学和实验室参数、组织病理学结果以及恶性肿瘤的类型、GD和治疗情况。
共研究了41例GDAM患者，平均年龄63.1（±10.7）岁。28例（68.3%）患者的GD与实体瘤相关，13例（31.7%）患者与淋巴系统恶性肿瘤相关。最常见的组织病理学类型是膜性肾病（43.9%）。总体而言，在GD诊断时，17%的患者抗核抗体（ANA）呈阳性，12.2%的患者抗中性粒细胞胞浆自身抗体（ANCA）呈阳性，均为抗髓过氧化物酶（MPO）抗体。此外，93.3%的膜性肾病患者跨膜糖蛋白M型磷脂酶A受体（PLAR）抗体呈阴性。16例患者（39.0%）表现为急性肾炎综合征，其中5例（31.25%）发展为快速进展性肾小球肾炎。平均随访时间为36.1（±28.3）个月，9例（21.95%）患者最终发展为终末期肾病，8例（19.5%）患者死亡。
我们发现，在过去十年中，因任何原因在我们机构接受自体肾活检的患者中，3.2%被确定患有某种与恶性肿瘤相关的GD。血清学检查表明，肾炎综合征患者中ANA或MPO-ANCA抗体显著存在，而膜性肾病患者中PLAR抗体缺失。
