Kidney Health Service, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia.
Conjoint Internal Medicine Laboratory, Chemical Pathology, Pathology Queensland, Health Support Queensland, Herston, Queensland, Australia.
Nephrology (Carlton). 2020 Jun;25(6):502-506. doi: 10.1111/nep.13696. Epub 2020 Feb 7.
Bile cast nephropathy (BCN) is an underdiagnosed cause of acute kidney injury (AKI). The precise pathogenesis of bilirubin tubular toxicity remains unknown. The aim of this study is to explore the cellular and molecular pathophysiology of human BCN. Paraffin-embedded sections of renal biopsy tissue from a BCN patient were stained by immunohistochemistry (IHC) for oxidative stress (4-hydroxynonenal), immune cell subpopulations, including dendritic cells (CD1c), macrophages (CD68) and T cells (CD3), and inflammasome activation by staining for active-caspase-1 and the inflammasome adaptor protein, ASC (apoptosis-associated speck-like protein containing a caspase activation and recruitment domain). Quantitative analyses of IHC staining were compared to healthy renal cortical tissue. We identified yellow to brown granular casts within the BCN case, consistent with the presence of bile pigment. The presence of bile pigment was associated with strong tubular 4-hydroxynonenal staining intensity, a marker of oxidative stress. Diffuse tubulointerstitial inflammatory cell infiltrate was detected, with elevated CD1c, CD68 and CD3 staining. Foci of inflammasome activity were co-localized with this intense immune cell infiltration, with increased active-caspase-1 and ASC staining. Our findings are the first to suggest that bile casts may lead to oxidative stress and trigger the inflammasome signalling cascade, leading to interstitial inflammation and driving AKI pathobiology. SUMMARY AT A GLANCE The report suggests that bile casts may lead to oxidative stress and trigger the inflammasome signalling cascade, leading to interstitial inflammation and driving bile cast nephropathy pathobiology.
胆色素管型肾病 (BCN) 是一种被低估的急性肾损伤 (AKI) 病因。胆红素肾小管毒性的确切发病机制尚不清楚。本研究旨在探讨人 BCN 的细胞和分子病理生理学。通过免疫组织化学 (IHC) 对 BCN 患者的肾活检组织石蜡切片进行染色,检测氧化应激(4-羟壬烯醛)、免疫细胞亚群(包括树突状细胞 (CD1c)、巨噬细胞 (CD68) 和 T 细胞 (CD3))和炎性体激活,通过染色检测活性半胱天冬酶-1 和炎性体衔接蛋白 ASC(含有半胱氨酸天冬氨酸蛋白酶激活和募集结构域的凋亡相关斑点样蛋白)。将 IHC 染色的定量分析与健康的肾皮质组织进行比较。我们在 BCN 病例中发现了黄色到棕色的颗粒状管型,与胆色素的存在一致。胆色素的存在与强烈的肾小管 4-羟壬烯醛染色强度相关,这是氧化应激的标志物。检测到弥漫性肾小管间质炎症细胞浸润,CD1c、CD68 和 CD3 染色增加。炎性体活性灶与这种强烈的免疫细胞浸润共定位,活性半胱天冬酶-1 和 ASC 染色增加。我们的研究结果首次表明,胆色素管型可能导致氧化应激,并触发炎性体信号级联反应,导致间质炎症,并驱动 AKI 病理生物学。
本报告表明,胆色素管型可能导致氧化应激,并触发炎性体信号级联反应,导致间质炎症,并驱动胆色素管型肾病的病理生物学。