What is ANCA associated vasculitis?
Vasculitis is an inflammation of the blood vessels often leading to a closing of the vessels. As a result, there is a shortage of oxygen supplied to body tissues reliant on the blood supply through these vessels. Sometimes, vasculitis is limited to just one organ but it can also affect multiple organs. There are different vasculitis conditions but in the case of ANCA associated vasculitis (AAV), smaller blood vessels are affected by the presence of ANCA, a sort of antibody in the blood. The following conditions are forms of ANCA associated vasculitis:

  • Granulomatous polyangiitis (GPA, previously known as Wegener’s granulomatosis)
  • Microscopic polyangiitis (MPA)
  • Eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome)

It is unclear how many people have been diagnosed with ANCA associated vasculitis in the Netherlands although it is estimated that some 350 diagnoses are made each year. The average age is somewhere between 40 and 70 years and the disease is just as common in men as in women. As ANCA associated vasculitis causes inflammation in the small blood vessels, the most vascularised organs, the kidneys, lungs and brain, are the most affected.

More information can be found on the Vasculitis Foundation website (Dutch only).

ARCH ANCA associated vasculitis working group
The ARCH ANCA associated vasculitis working group aims to create a care path for ANCA associated vasculitis that can be implemented nationally, and determines and implements improvement where necessary. In addition, the working group gathers information about the current care being offered and who the relevant experts are

Working group members
The working group’s project leader is Dr Y.K. Onno Teng, internist-nephrologist at Leiden University Medical Center (LUMC).  Onno is supported bij Ebru Dirikgil, project manager. The other members of the working group are:

  • Dr Sander Tas – rheumatologist, AMC, Amsterdam
  • Dr Bram Rutgers – clinical immunologist, UMCG, Groningen
  •  Mr Peter Verhoeven – chair of the Vasculitis Foundation, Netherlands
  • Prof. Coen Stegeman – internist-nephrologist, UMCG, Groningen
  • Dr Chris Hagen – internist-nephrologist, Amersfoort
  • Dr Janneke Tekstra – rheumatologist, UMCU, Utrecht
  • Dr Willem-Jan Bos – internist-nephrologist, Antoniuisziekenhuis, Nieuwegein
  • Dr Liesbeth Hak – clinical immunologist, AMC, Amsterdam
  • Dr Pieter van Paassen – internist-nephrologist, MUMC+, Maastricht
  • Dr Marc Kok – rheumatologist, Maasstad Hospital, Rotterdam

The first consensus document on the diagnosis and treatment of AAV has been compiled and will be issued in early 2019 along with the input from the survey and the study into practice variation. If you have any questions, please contact Ebru Dirikgil at e.dirikgil@lumc.nl