Uppsala researcher seeking genetic explanation for Viking disease

Stephan Wilbrand, chief physician and researcher
in the Department of Surgical Sciences.

What is Viking disease?
“It is a heritable disease in which connective tissue fibres form in the palm of the hand, causing one or more fingers to become permanently curved. Those most likely to be afflicted are middle-aged men, and since the disease occurs most commonly in Scandinavia, it is often called Viking disease. In Sweden, for example, one out of five men over age 60 – and also many women – suffer from Dupuytren’s contracture, which is the clinical name. A few treatments currently are available, but still no permanent cure, and symptoms often recur after a few years.”

How could this widespread disease remain so unnoticed?
“Maybe because it historically has been considered the result of an immoral lifestyle. In Swedish it was even called tjyvaklo, a word associated with theft. Maybe because treatment of Dupuytren’s contracture had a somewhat lower status within the medical profession for a long time and was something only somewhat younger surgeons treated. However, interest in the disease has increased since introduction of a new enzyme treatment in 2011, and today the field has a relatively prominent place in many orthopaedic conferences.”

What does your own research about the disease focus on?
“Soon a younger colleague and I will begin a study to identify genes that increase the risk of developing Dupuytren’s contracture. Our starting point is an Icelandic gene bank of very high quality, and we hope to later combine our results with ongoing genetic studies in the Netherlands and England. Our goal is to block growth factors at an early stage or make genetic pre-treatments possible instead of treating the malady only after it is already an established fact, as we do today.”

Are new treatments being explored for those already affected?
“Not to any great extent. If anything, there is a relatively animated debate about which of the three main options should be used: surgery, enzyme treatment or needle fasciotomy. I personally maintain that we should consider all the options and choose the most suitable in each case. But to provide a scientific answer, we are now compiling a study of patients’ experience before and after each treatment, and we hope to publish our results next year.”

What developments can we expect in the field?
“The disease continues to spread. For example, we are now encountering cases of Dupuytren’s contracture among the Sami people, a group that previously had been free of the disease. On the other hand, research funding is increasing. We are also seeing patients in the United States and Germany form their own organisations, and I would be happy to assist a Swedish counterpart to the best of my ability if only we can find the right enthusiast among those receiving care. In fact, here in Uppsala we have already provided enzyme treated for the most people in Europe, and it never fails to inspire me when a former patient returns with a grateful handshake and straight fingers!”