#Blueprint registry skin#
#Blueprint registry registration#

Strengthen anti-calcification properties of serum Therapy against uncontrolled hyperparathyroidismĮfforts to increase adherence to medical therapies Giving vitamin K (unless contraindications are present for one of these measures) Replacing vitamin K antagonist treatment by heparin or any other oral anticoagulation if really needed Using citrate as the buffer in the dialysis bath Reduction in calcium concentration in the dialysis bath Switch from peritoneal dialysis to haemodialysis/haemodiafiltration Obviously, superiority in terms of outcome improvement among these therapeutical steps cannot be established based on such purely observational, uncontrolled and sometimes anecdotal data. In addition, Table 1 summarizes the diversity of the therapeutic actions available. This impressive number of patients already provides a rough overview on what is generally considered ‘good clinical practice’ in the therapeutic management of these patients (Table 1). More than 230 patients have been registered in the registry up to the beginning of 2015. The German calciphylaxis registry has been actively recruiting patients since 2006. What do we know about calciphylaxis?Īs with any rare disease, registry studies such as the German CUA registry ( can effectively support central data collecting and analysis upon the entire spectrum of the disease from potential risk factors to good clinical practice.

Despite remarkable progress in recent years, evidence-based therapeutic options are unfortunately absent, since controlled randomized treatment trials have not yet been undertaken. In most cases, bone and mineral metabolism is altered and may play a significant role in the pathophysiology.

The aetiology of CUA is still not completely understood. CUA confers a high risk of morbidity and mortality, largely resulting from underlying cardiovascular disease, wound infection and septicaemia. Panniculitis and circumferential calcification of cutaneous arterioles dominate the histological picture together with endothelial detachment and luminal occlusion.

#Blueprint registry skin#

Skin necrosis and ulceration represent the full-blown, ‘late’ clinical picture. Clinically, CUA is characterized by the stepwise development of superficial painful sensations and cutaneous lesions similar to livedo reticularis. However, anecdotal reports also exist about cases in patients without relevant kidney disease (so-called non-uraemic calciphylaxis). CUA typically occurs in chronic dialysis patients and hence nephrologists are most likely involved in primary diagnosis and patient care. 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.Ĭalcific uraemic arteriolopathy, calcification, chronic kidney disease, dialysis, vascular disease IntroductionĬalciphylaxis (calcific uraemic arteriolopathy, CUA) is a rare disease (Orphanet number ORPHA280062) at the interface of nephrology, dermatology and cardiovascular medicine.

#Blueprint registry registration#

The next phase will be to allow international patient registration via as part of the multinational EuCalNet (European Calciphylaxis Network) initiative, which is supported by the ERA-EDTA scientific working group ‘CKD-MBD’. 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. In 2006, we established an internet-based registry in Germany ( to allow online notification of patients with established or suspected CUA. 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. Moreover, CUA treatment strategies vary significantly among centres and expert recommendations are heterogeneous. The epidemiology and risk factors associated with this disease are still not fully understood. Medial calcification of cutaneous arterioles and extensive extracellular matrix remodelling are the hallmarks of CUA. The typical course of CUA is characterized by painful skin discolouration and induration evolving to necrotic ulcerations. Calcific uraemic arteriolopathy (CUA) is a rare disease and continues to be a clinical challenge.