Newly updated advice on the treatment of patients with AAV

A central focus of newly updated recommendations on treating ANCA-associated vasculitis (AAV) is shared decision-making between patients and doctors. The updated recommendations, produced by a collaboration between the European League Against Rheumatism (EULAR) and European Renal Association – European Dialysis and Transplant Association (ERA-EDTA), taking account of recent research on the benefits and safety treatments for AAV.

When someone has ANCA-associated vasculitis (AAV), their immune system -which normally fights infection – mistakenly attacks their blood vessels. This can compromise the tissues which the blood vessels supply, leading to a whole range of signs and symptoms including: bleeding and crusting from the nose, cough and shortness of breath, tiredness, aching in muscles, loss or blurring of vision and double vision which may also be associated with eye pain, swollen, stiff, and painful joints, pain or numbness in arms and legs and confusion. Immunosuppressive drugs can help stop this happening by reducing damage to the blood vessels and helping prevent irreversible damage and disability. Immunosuppressive drugs are the main treatment for AAV, and there are many different types including:

  • Older, standard types, which are manufacturered chemically: cyclophosphamide, steroids, azathioprine, methotrexate and mycophenolate.
  • Newer types, often called ‘biologics’. The main drug of this type used to treat AAV is rituximab.

In certain situations giving patients blood proteins via a drip or even removing the immune proteins via a machine that ‘washes’ the blood may be appropriate. With so many options to consider, deciding on a treatment approach can be a challenge, particularly since research does not provide clear answers on which works best and is safest. To help with this, the European groups of both EULAR and ERA-EDTA convened a task force of doctors specialising in AAV, nurses and patient representatives to review the current research and provide guidance. They have now released their recommendations which are an update of those published in 2009.

The updated recommendations emphasise the importance of doctors, nurses and patients working together to find the best care approach, stressing that treatment must be based on shared decisions between the patient and their doctor. Other key principles are:

  • Specialists who have a special interest and expertise in AAV should primarily care for people with AAV.
  • Biopsy of an affected organ (most commonly the kidney) can be helpful in confirming a new diagnosis of AAV and in those patients having a relapse.
  • The aim of the treatment should be remission.
  • Doctors should monitor patients using structured clinical assessment and make use of validated tools to monitor for complications of AAV and also the drugs used to treat it.
  •  A combination of steroid and either cyclophosphamide or rituximab should be the first-line treatment doctors and patients to consider for newly diagnosed organ or lifethreatening AAV.
  •  Patients and doctors can consider using steroids (glucocorticoids), as part of patients’ initial treatment (along with immunosuppressive drugs) but these drugs should be reduced and stopped as soon as possible.
  •  If the first treatment approach doesn’t work well enough, patients should be referred to an expert centre for ongoing management and enrolment in clinical trials.
  •  For patients who relapse with either an organ or life-threatening disease, a combination of steroid and either cyclophosphamide or rituximab should be the firstline treatment doctors and patients consider.
  •  When a patient has been successfully treated for their initial disease or relapse then a period of treatment with remission-maintenance should occur. The drug choices include: azathioprine, rituximab, methotrexate and mycophenolate. The precise drug will depend both on the type of AAV you have and which drug was used to bring about remission in the first place.
  • If a patient’s AAV is not active (in remission) then the immunosuppressive drugs including the steroids should be reduced. However treatment with remissionmaintenance therapy should be continued for at least 24 months in those patients in stable remission (i.e. have not suffered relapses during that period). This is a decision that should be carefully considered by the patient and their doctor.
  •  When treatment needs to be adjusted, other things need to be taken into account, along with a patient’s disease activity and ANCA titre result, include any other illnesses, possible side effects of current or previous treatment and the development of damage over time.

These recommendations are based on a thorough review of the current research and knowledge, as well as discussions among experts and patient representatives. They should provide reliable guidance on the best approach to treating AAV.

If you have AAV, these recommendations should provide useful insight into what treatments you are likely to be offered and when. They also emphasise that as a patient, you should have a voice in your treatment. If you have any questions or concerns, be sure to speak with your specialist.

Disclaimer: This is a summary of a scientific article written by a medical professional (“the Original Article”). The Summary is written to assist non medically trained readers to understand general points of the Original Article. It is supplied “as is” without any warranty. You should note that the Original Article (and Summary) may not be fully relevant nor accurate as medical science is constantly changing and errors can occur. It is therefore very important that readers not rely on the content in the Summary and consult their medical professionals for all aspects of their health care and only rely on the Summary if directed to do so by their medical professional. Please view our full Website Terms and Conditions.

Date prepared: September 2015

Summary based on research article published on: 23 June 2016

From: Yates M, Watts RA, Bajema IM, et al. EULAR and ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016;75:1583-1594 doi:10.1136/annrheumdis-2016-209133

Copyright © 2016 BMJ Publishing Group Ltd & European League Against Rheumatism. Medical professionals may print copies for their and their patients and students non commercial use. Other individuals may print a single copy for their personal, non commercial use. For other uses please contact our Rights and Licensing Team.