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...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.展开更多
基金supported by the National Natural Science Foundation of China(81570666)the Outstanding Young and Middle-Aged Talents Support Program of the First Affiliated Hospital with Nanjing Medical University(Jiangsu Province Hospital)+4 种基金the Clinical Capacity Enhancement Project(JSPH-MA-2023-7)the Specialized Diseases Clinical Research Fund of Jiangsu Province Hospital(XB202403)supported by Jiangsu Provincial Medical Key Discipline(Laboratory)Cultivation Unit(JSDW202206)the ISN Clinical Research Program(18-01-0247)Key Projects of Medical Scientific Research Funded by the Health Commission of Jiangsu Province(K2024005).
文摘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.
