Division of Nephrology, Department of Internal Medicine, Massachusetts General Hospital, Boston, MA, USA.
Department of Cardiology and Nephrology, Rhein-Maas Klinikum, Würselen, Germany.
QJM. 2019 Jan 1;112(1):29-34. doi: 10.1093/qjmed/hcy234.
Calciphylaxis is a disease of significant morbidity and mortality, predominantly affecting dialysis patients. The term 'calciphylaxis' was coined by Seyle et al. in 1961 to describe calcium deposition in the skin and subcutaneous soft tissue of uremic rats in response to 'triggers' (e.g. trauma, metallic salts) after exposure to 'sensitizing agents' (e.g. vitamin D and parathyroid hormone). In humans, calciphylaxis, however, is not a disorder of induced hypersensitivity. Instead, it is a disorder of cutaneous microvascular occlusion caused by thrombosis and calcification. Progressive, excruciatingly painful, non-healing wounds develop in these patients, pre-disposing them to high risk of sepsis and death. Calciphylaxis has no approved therapies. Increased awareness and research in this field have facilitated identification of risk factors and causation pathways. Development of therapeutic options and wound care management, however, are still at a nascent stage. Certain therapies have shown a promise that needs evaluation in prospective clinical trials. It is hoped that ongoing research will help us better understand the pathogenesis of this complex disease and develop efficacious treatment options. In this review, we outline the components involved in calciphylaxis diagnosis and treatment.
钙化防御是一种具有显著发病率和死亡率的疾病,主要影响透析患者。“钙化防御”一词由 Seyle 等人于 1961 年创造,用于描述尿毒症大鼠在接触“敏化剂”(如维生素 D 和甲状旁腺激素)后,对“触发因素”(如创伤、金属盐)的皮肤和皮下软组织中的钙沉积。然而,在人类中,钙化防御不是诱导性过敏的疾病。相反,它是一种由血栓形成和钙化引起的皮肤微血管闭塞的疾病。这些患者会出现进行性、剧烈疼痛、无法愈合的伤口,使他们极易发生败血症和死亡。钙化防御目前尚无批准的治疗方法。该领域的认识提高和研究促进了对危险因素和发病机制的识别。然而,治疗方案的开发和伤口护理管理仍处于起步阶段。某些疗法已显示出希望,需要在前瞻性临床试验中进行评估。希望正在进行的研究将帮助我们更好地了解这种复杂疾病的发病机制,并开发出有效的治疗方法。在这篇综述中,我们概述了钙化防御诊断和治疗中涉及的组成部分。