Wu Daoxu, Lu Shijiu, Hu Jiaying, Zeng Ming, Wu Jingjing, Li Cui, Tang Xingfang, Lu Tian, Zhu Yi, Liu Jiayin, Qin Lianju, Wang Ningning
Division of Nephrology, Department of Geriatrics, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
Department of Nephrology, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing 210029, China.
J Mol Cell Biol. 2025 Jul 28;17(2). doi: 10.1093/jmcb/mjaf009.
Calciphylaxis is a rare, progressive disorder characterized by subcutaneous adipose and dermal microvascular calcifications, microthrombi, and endothelial damage. It mainly affects patients with chronic kidney disease (CKD), which is also known as calcific uremic arteriolopathy. Skin biopsy is the gold standard for diagnosis, but it is an invasive procedure. Calciphylaxis frequently results in ischemic and nonhealing ulcerations with a high mortality rate. A multidisciplinary targeted approach is the primary treatment method. Vascular calcification, which is a common complication in patients with CKD, cannot completely explain the rapid progression of calciphylaxis. This article reviews the advances in the epidemiological characteristics, risk factors, and diagnosis, including non-uremic calciphylaxis and visceral calciphylaxis, pathogenesis, associated animal models, and treatment of calciphylaxis. The scarcity of animal models that mimic the clinical presentation of calciphylaxis hampers the understanding of its pathogenesis. The acute effects on progressive vascular injury, including the induction of severe ischemia and inflammatory responses, have been emphasized. Actively listening to the voices of patients and their families and building a multidimensional research system with artificial intelligence technologies based on the specific molecular makeup of calciphylaxis patients will help tailor regenerative treatment strategies. Mesenchymal stem cells (MSCs) may represent a novel therapy for calciphylaxis because of their regenerative effects, inhibition of vascular calcification, anti-infection and immunomodulation properties, and improvement of hypercoagulability. Safe, effective, accessible, and economical MSC strategies guided by biomarkers deserve consideration for the treatment of this devastating disease.
钙过敏是一种罕见的进行性疾病,其特征为皮下脂肪和真皮微血管钙化、微血栓形成以及内皮损伤。它主要影响慢性肾脏病(CKD)患者,也被称为钙化性尿毒症性小动脉病。皮肤活检是诊断的金标准,但它是一种侵入性操作。钙过敏常导致缺血性且不愈合的溃疡,死亡率很高。多学科靶向治疗方法是主要的治疗手段。血管钙化是CKD患者的常见并发症,但无法完全解释钙过敏的快速进展。本文综述了钙过敏在流行病学特征、危险因素、诊断(包括非尿毒症性钙过敏和内脏钙过敏)、发病机制、相关动物模型以及治疗方面的进展。模拟钙过敏临床表现的动物模型稀缺,这阻碍了对其发病机制的理解。文中强调了其对进行性血管损伤的急性影响,包括诱导严重缺血和炎症反应。积极倾听患者及其家属的声音,并基于钙过敏患者的特定分子构成,利用人工智能技术构建多维研究系统,将有助于制定再生治疗策略。间充质干细胞(MSCs)可能是一种治疗钙过敏的新疗法,因为它们具有再生作用、抑制血管钙化、抗感染和免疫调节特性,以及改善高凝状态。由生物标志物指导的安全、有效、可及且经济的间充质干细胞治疗策略值得考虑用于治疗这种毁灭性疾病。