This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in


Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team


It is a comparatively rare disease with an incidence between 11.5 and 75.0 per 100 000 population in the northern Europe

  • in Scotland - 11.5 per 100,000
  • in Sweden - ranging from 19.6 to 39.2 per 100,000

The condition usually presents in the fourth and fifth decades (but may present at any age).

Men are affected more frequently than women (two thirds of patients are male) (3)

Caucasians are affected predominantly (1).


Create an account to add page annotations

Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.