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Professor David Jayne
Professor of Clinical Autoimmunity, University of Cambridge
Although rare, vasculitis affects around 40,000 people in the UK with 18 different forms of the disease, it is estimated a quarter of these individuals are affected by ANCA-associated vasculitis. But the widely varying clinical features, which can mimic other diseases, frustrate prompt diagnosis.
When first given a diagnosis of vasculitis a patient’s first thoughts often are, ‘What is that?’ followed by relief that a reason for their months of ill health has been discovered and there is a way forward.
Understanding the symptoms
Vasculitis means inflammation of blood vessels and the different vasculitis syndromes are defined by which blood vessels are involved. ANCA-associated vasculitis (AAV) is a group of diseases that affects the small blood vessels. Small and medium blood vessels can be found in various parts of the body which can be affected including kidney, lungs and ears. AAV is divided into three subgroups depending on clinical features which are GPA, MPA and EGPA.
When a vessel becomes inflamed the wall is damaged and the lumen blocks, denying the tissue it supplies enough blood. In the skin this results in a ‘purpuric’ or spotty rash which can recover completely, while in the kidney this can harm the filters ultimately causing kidney failure, requiring dialysis or a transplant.
Patients experience non-specific symptoms related to activation of the immune system, including fevers, sweats, weight loss and muscle pain, as if they had flu. Then there are symptoms related to the particular tissue the vasculitis attacks such as arthritis, rash, breathlessness or loss of vision.
The disease occurs spontaneously as a result of an autoimmune process. Several genes have been identified that predispose to different vasculitis syndromes, although familial cases are rare and one of the more common syndromes.
ANCA-associated vasculitis is characterised by an auto (anti-self) antibody that attacks white blood cells. Occasional cases are linked to an environmental trigger such as silica exposure, infection, drugs and cancer, but in most cases the cause is unknown.
ANCA-associated vasculitis (AAV) is a group of diseases that affects the small blood vessels.
Finding effective treatment options
Steroids, introduced over 70 years ago, remain a widely used treatment because they work fast and are reliable. However, their psychologic and physical side-effects remain a major problem and a challenge to develop alternatives.
More recently, immune suppressant drugs borrowed from cancer or transplantation are combined with steroids with monoclonal antibody therapies joining treatment regimens.
Challenges in diagnosis
Treatment controls the disease but delays in diagnosis are common and can result in irreversible damage. As treatment is reduced the disease can return indicating the need for prolonged treatment.
The multifaceted nature of ANCA-associated vasculitis is a challenge for health care, with many medical specialties involved and ‘ad-hoc’ vasculitis clinics have been developed to try and coordinate care around the patient.
While there have been efforts by the NHS through specialist commissioning, better organisation is needed to improve care and the longer-term health of ANCA-associated vasculitis patients.
Learn more about Vasculitis: