University of Connecticut School of Medicine, Farmington, CT, USA.
Louisiana State University Health Sciences Center School of Medicine, New Orleans, LA, USA.
Adv Ther. 2020 Dec;37(12):4797-4807. doi: 10.1007/s12325-020-01504-w. Epub 2020 Sep 30.
Calciphylaxis is a deadly, painful disease with a 1-year mortality of up to 50%. The disease is commonly associated with patients with end-stage kidney disease (ESKD), but it can manifest in non-uremic patients as well. In patients who are undergoing dialysis, the incidence of calciphylaxis can range from 0.04% to 4%. The progressive arterial calcification seen in calciphylaxis can affect multiple body organs, including the skin, brain, lungs, and muscle. In cutaneous calciphylaxis, painful and non-healing nodules, plaques, and ulcers may appear, increasing morbidity for patients. Diagnosis can be difficult, and the condition can clinically appear similar to other dermatological diseases, especially in non-uremic patients. Currently, skin biopsy with histological analysis is the most reliable method to help diagnose the condition. In certain cases, the use of medical imaging may be helpful. Treatment of pain in this condition can be difficult and should be multimodal and include wound care as well as modification of risk factors. Analgesic options include opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), as well as analgesic options that are targeted for specific patients. There are currently multiple clinical trials underway that are studying targeted therapies for this condition.
钙化防御是一种致命性、疼痛性疾病，其 1 年死亡率高达 50%。该疾病通常与终末期肾病（ESKD）患者相关，但也可在非尿毒症患者中表现出来。在接受透析的患者中，钙化防御的发病率范围为 0.04%至 4%。钙化防御中可见的进行性动脉钙化可影响多个身体器官，包括皮肤、脑、肺和肌肉。在皮肤钙化防御中，可能会出现疼痛且不易愈合的结节、斑块和溃疡，增加了患者的发病率。诊断可能具有挑战性，且该病症在临床上可能与其他皮肤病相似，尤其是在非尿毒症患者中。目前，皮肤活检联合组织学分析是帮助诊断该病症的最可靠方法。在某些情况下，使用医学影像学可能会有所帮助。这种情况下的疼痛治疗可能很困难，应该采用多模式治疗，包括伤口护理以及对危险因素进行修正。镇痛选择包括阿片类药物、非甾体抗炎药（NSAIDs），以及针对特定患者的镇痛选择。目前，有多项临床试验正在研究针对该病症的靶向治疗方法。
