Calatroni Marta, Moroni Gabriella, Reggiani Francesco, Ponticelli Claudio
Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20072, Pieve Emanuele, MI, Italy.
Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Via Manzoni 56, 20089, Rozzano, MI, Italy.
J Nephrol. 2023 Jan;36(1):5-15. doi: 10.1007/s40620-022-01369-y. Epub 2022 Jun 27.
Sarcoidosis is a systemic inflammatory disease of unknown etiology. The pathogenesis rests on an aberrant T cell response to unidentified antigens in individuals predisposed by genetic and environmental factors. Increased expression of polarized macrophages and disequilibrium between effector and regulator T cells contribute to the formation of noncaseating granulomas, that are frequently found in affected organs. The main kidney abnormalities in sarcoidosis are granulomatous interstitial nephritis (GIN) and hypercalcemia-related disorders. The clinical diagnosis is difficult. The outcome is variable, ranging from spontaneous remission to end-stage kidney disease (ESKD). Early diagnosis and prompt treatment with corticosteroids can improve the prognosis. Hypercalcemia may be responsible for acute kidney injury (AKI) caused by vasoconstriction of afferent arterioles. Complications of persistent hypercalcemia include nephrocalcinosis and renal stones. In patients with ESKD, dialysis and transplantation can offer results comparable to those observed in patients with other causes of kidney failure. Based on a review of the literature, we present an overview of the etiopathogenesis, the renal manifestations of sarcoidosis and their complications, management and prognosis.
结节病是一种病因不明的全身性炎症性疾病。其发病机制基于遗传和环境因素易患个体中T细胞对未知抗原的异常反应。极化巨噬细胞表达增加以及效应T细胞和调节性T细胞之间的失衡促成了非干酪样肉芽肿的形成，这些肉芽肿常见于受累器官。结节病主要的肾脏异常是肉芽肿性间质性肾炎（GIN）和高钙血症相关疾病。临床诊断困难。其预后各异，从自发缓解到终末期肾病（ESKD）。早期诊断并及时使用糖皮质激素治疗可改善预后。高钙血症可能是由入球小动脉血管收缩导致急性肾损伤（AKI）的原因。持续性高钙血症的并发症包括肾钙质沉着症和肾结石。对于ESKD患者，透析和移植的结果与其他肾衰竭病因患者的结果相当。基于文献综述，我们概述了结节病的病因发病机制、肾脏表现及其并发症、管理和预后。
