Multiple extremity necrosis in fatal calciphylaxis: Case report

ABSTRACT Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease. Methods: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution. Discussion: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism. Conclusion: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.

摘要
引言：血管钙化对心血管疾病发病率与死亡率的临床影响已得到广泛证实，但其他临床综合征，如钙化防御（calciphylaxis），尽管发病率较低，却对慢性肾脏病患者存在显著危害。
方法：本研究为1例27岁女性患者的病例报告。患者因双侧足趾疼痛3天就诊，受累部位可见小片坏死区域。患者有25年1型糖尿病病史，并发慢性肾脏病，接受腹膜透析治疗，同时合并类风湿关节炎。患者此前因类风湿关节炎急性加重入院，期间因静脉导管并发症出现心腔内血栓，遂开始服用华法林。患者足部缺血症状持续进展，最终需接受双侧截肢手术，手指亦受累。研究对血栓性疾病、血管炎、心内膜炎及其他栓子来源进行了排查并均予以排除。病理报告显示皮肤坏死、浅表软组织存在近期动脉血栓形成，以及门克贝格（Monckeberg）血管中层钙化。我们给予患者双膦酸盐与硫代硫酸钠治疗，将腹膜透析转为血液透析，并将华法林替换为普通肝素。尽管采取了上述治疗措施，患者在病程进展4个月后死亡。
讨论：钙化防御是一种罕见的微血管钙化综合征，可导致严重缺血性损伤。其发病机制与慢性肾脏病患者的矿物质与骨代谢紊乱、血管钙化促进因子与抑制因子失衡相关，其中维生素K拮抗作用具有特殊临床意义。
结论：由于治疗方案复杂且疗效缺乏充分验证，预防策略至关重要。