Division of Nephrology, Kidney Research institute, West China Hospital of Sichuan University, Chengdu, China; Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee.
Department of Pathology & Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California.
Am J Kidney Dis. 2022 Sep;80(3):416-421. doi: 10.1053/j.ajkd.2021.11.009. Epub 2021 Dec 30.
We report 7 native kidney biopsies with diffuse endocapillary hypercellularity without immune deposits, affecting 5 women and 2 men aged 52-85 years. All patients had acute kidney injury, and 4 had nephrotic-range proteinuria. Comorbidities included breast cancer in 2, pancreatitis in 1, and para-aortic lymphadenopathy and bilateral carpal tunnel syndrome in 1. Kidney biopsies were characterized by predominant T-cell and CD68-positive macrophage infiltration in glomerular capillaries without deposits. Coexisting lesions included small cellular crescents in 5, mild peritubular capillaritis in 1, mononuclear cell intimal arteritis in 1, acute tubulointerstitial nephritis in 4, and mild arteriolosclerosis in 1. During the mean follow-up duration of 24.8 months, 4 patients showed partial or complete initial remission in response to immunosuppression. However, 2 deteriorated when prednisone was rapidly tapered (1 of them achieved subsequent remission with increased prednisone). Three patients developed kidney failure. We propose that this unusual pattern of injury is mediated by abnormal cell-mediated immune response. The underlying causes and pathogenesis of this cell-mediated glomerulonephritis will require further study.
我们报告了 7 例无免疫沉积物的弥漫性毛细血管内细胞增多症的原发性肾脏活检,影响 5 名女性和 2 名年龄在 52-85 岁之间的男性。所有患者均有急性肾损伤,4 例有肾病范围的蛋白尿。合并症包括 2 例乳腺癌、1 例胰腺炎和 1 例腹主动脉旁淋巴结病和双侧腕管综合征。肾脏活检的特征是肾小球毛细血管中主要有 T 细胞和 CD68 阳性巨噬细胞浸润,无沉积物。并存病变包括 5 例小细胞性新月体、1 例轻度肾小管周围毛细血管炎、1 例单核细胞内膜性动脉炎、4 例急性肾小管间质性肾炎和 1 例轻度小动脉硬化。在平均 24.8 个月的随访期间,4 例患者对免疫抑制有部分或完全的初始缓解。然而,当泼尼松迅速减量时,2 例患者病情恶化(其中 1 例增加泼尼松后随后缓解)。3 例患者发生肾衰竭。我们提出,这种异常的损伤模式是由异常的细胞介导的免疫反应引起的。这种细胞介导的肾小球肾炎的潜在原因和发病机制需要进一步研究。