EuCalNet Calciphylaxis Registry

Calcific uraemic arteriolopathy (CUA) or calciphylaxis as it is frequently called is a syndrome of calcification of the walls of small blood vessels which supply the skin and/or muscle. This leads to lack of blood flow causing rashes, ulcers and pain in the affected tissues.

It commonly affects the legs and arms but can affect the torso, back or breasts also. It occurs almost exclusively in patients with chronic kidney disease but remains very rare.

Although chronic kidney disease is the most important clinical risk factor, followed by malignancies, CUA does also occur in association with normal kidney function and liver cirrhosis. Other risk factors are female sex, obesity, some rare thrombophilia syndromes, treatment with vitamin K antagonists and/or corticosteroids and low albumin levels.

The trigger for the disease is not known but may include local injury.

There is no specific treatment as yet that has been shown to work in many or most cases. Doctors often concentrate on getting the balance of minerals in the body right ie calcium and phosphate control. This may require changing dialysis regimes, changing medications, or considering surgery for overactive parathyroid glands which release a hormone (PTH) which causes mineral to leech out of the bones. In addition, health-care teams focus on pain control, good nutrition and excellent ulcer care if the skin has broken down.

This collaborative research project with the UK & Germany has been set up to try and collect information on the patients who get the disease – to ask if there is anything in their treatment or previous blood tests etc, to help us identify who may be at risk of calciphylaxis. In addition, we are collecting blood samples and samples of tissue eg from skin biopsy to diagnose the condition. We will perform thorough tests on these to help increase understanding of what the disease process is, so that we can improve our treatments in the future.