Brandenburg Vincent, Adragao Teresa, van Dam Bastiaan, Evenepoel Pieter, Frazão João M, Ketteler Markus, Mazzaferro Sandro, Urena Torres Pablo, Ramos Rosa, Torregrosa Jose-Vicente, Cozzolino Mario
Department of Cardiology , University Hospital RWTH Aachen , Aachen , Germany; on behalf of the ERA-EDTA scientific working group 'CKD-MBD'
Diaverum Unidade do Estoril , Lisboa , Portugal.
Clin Kidney J. 2015 Oct;8(5):567-71. doi: 10.1093/ckj/sfv056. Epub 2015 Jul 6.
Calcific uraemic arteriolopathy (CUA) is a rare disease and continues to be a clinical challenge. The typical course of CUA is characterized by painful skin discolouration and induration evolving to necrotic ulcerations. Medial calcification of cutaneous arterioles and extensive extracellular matrix remodelling are the hallmarks of CUA. The epidemiology and risk factors associated with this disease are still not fully understood. Moreover, CUA treatment strategies vary significantly among centres and expert recommendations are heterogeneous. Registries may provide important insights and information to increase our knowledge about epidemiology and clinical aspects of CUA and may help to optimize its therapeutic management. In 2006, we established an internet-based registry in Germany (www.calciphylaxie.de) to allow online notification of patients with established or suspected CUA. The registry includes a comprehensive database with questions covering >70 parameters and items regarding patient-related and laboratory data, clinical background and presentation as well as therapeutic strategies. The next phase will be to allow international patient registration via www.calciphylaxis.net as part of the multinational EuCalNet (European Calciphylaxis Network) initiative, which is supported by the ERA-EDTA scientific working group 'CKD-MBD'. Based on the valuable experience with the previous German CUA registry, EuCalNet will be a useful tool to collect data on the rare disease CUA and may become a basis for prospective controlled trials in the near future.
钙化性尿毒症小动脉病（CUA）是一种罕见疾病，仍然是一项临床挑战。CUA的典型病程特征为皮肤疼痛性变色和硬结，进而发展为坏死性溃疡。皮肤小动脉的中层钙化和广泛的细胞外基质重塑是CUA的标志。与该疾病相关的流行病学和危险因素仍未完全明确。此外，各中心的CUA治疗策略差异很大，专家建议也不一致。登记处可能会提供重要的见解和信息，以增加我们对CUA流行病学和临床方面的了解，并可能有助于优化其治疗管理。2006年，我们在德国建立了一个基于互联网的登记处（www.calciphylaxie.de），以允许在线报告确诊或疑似CUA的患者。该登记处包括一个综合数据库，其中的问题涵盖了70多个与患者相关及实验室数据、临床背景和表现以及治疗策略有关的参数和项目。下一阶段将允许通过www.calciphylaxis.net进行国际患者登记，这是跨国EuCalNet（欧洲钙化防御网络）倡议的一部分，该倡议得到了ERA-EDTA科学工作组“CKD-MBD”的支持。基于之前德国CUA登记处的宝贵经验，EuCalNet将成为收集罕见病CUA数据的有用工具，并可能在不久的将来成为前瞻性对照试验的基础。
