Vasculitis refers to a group of conditions that are characterised by inflammation of the blood vessels.^1,2 This can affect any blood vessel in the body, with the type of vasculitis and its symptoms depending on the tissues and organs that are involved.¹ The inflammation can result in narrowing and weakening of the blood vessels, obstruction of blood flow (ischaemia), tissue damage (necrosis) and blood clots (thrombosis).² Early detection and treatment are crucial for reducing the risk of life-threatening complications.¹

A list of the different types of vasculitis can be found at RARE Portal: List of Vasculitis types. Individual pages for the different types of vasculitis will be added to the RARE Portal over time.

There are no known synonyms.

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:52759 Vasculitis

ICD-11: 4A44 Vasculitis

Please refer to the individual types of vasculitis for their specific classifications.

General symptoms of vasculitis include fever, weakness and fatigue, muscle and joint pain, poor appetite and weight loss.^2,3

Other symptoms depend on the type of vasculitis and the organs that are affected.¹ Symptoms can vary between individuals and can range from mild to severe with life-threatening complications such as blood clots, enlarged arteries (aneurysm), as well as organ damage and failure.¹

Please speak to your medical team to learn more about the specific symptoms of a particular type of vasculitis.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

The underlying causes of vasculitis are not always well understood.¹

Vasculitis may be caused by:^2,3

• autoimmunity (when the body produces antibodies attacking itself, which are also known as autoantibodies)
• allergies and hypersensitivity to medication, toxins and other environmental trigger
• viral or parasite infections

Allergies, hypersensitivity, and infections should be ruled out as the cause before considering if it is an autoimmune condition.^2,3

Diagnosis of vasculitis may be based on a detailed medical history and physical examination.^1,2 Imaging studies such as X-rays, computed tomography (CT), or magnetic resonance imaging (MRI) scans, may be performed to visualise the blood vessels (angiogram).¹ A biopsy may be done to look for signs of inflammation and damage in specific tissues. Blood and urine tests may also be conducted.^1-3

Diagnosis of the exact type of vasculitis is important to enable appropriate treatment.¹

Please speak to your medical team to learn more about the diagnostic pathways for a specific type of vasculitis.

Treatment of vasculitis often involves targeting the underlying cause of the condition, as well as treating and managing the symptoms and complications.¹

If the vasculitis is caused by allergies or hypersensitivity, avoidance of the trigger/s may be sufficient to resolve or control the symptoms.³

Treatment of autoimmune vasculitis may include:^1-3

• corticosteroids to reduce inflammation
• medication that suppresses the immune system (immunosuppressive drugs)
• plasmapheresis, which involves the filtering of autoantibodies out of the blood plasma

Further management of vasculitis may depend on the symptoms. Regular monitoring is recommended to identify any complications, which can be life-threatening.¹

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the treatment of vasculitis may include general practitioners (GP), clinical immunologists, nephrologists, rheumatologists, dermatologists, respiratory physicians, gastroenterologists, cardiologists, neurologists, and ENT (ear, nose and throat) specialists.¹ The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

A list of relevant specialists can be found at Australia and New Zealand Vasculitis Society (ANZVASC): Find a Doctor.

There may be specific clinical care guidelines for the individual vasculitis conditions.

If you know of any relevant care guidelines, please let us know via the Contribute page.

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

It may be important to consider the following when managing individuals living with vasculitis at emergency departments/services:¹

• Some individuals with vasculitis may be at risk of life-threatening complications, including blood clots, enlarged arteries (aneurysm), as well as organ damage and failure

AnzVasculitis: ANZ Vasculitis Registry is a vasculitis registry established by ANZVASC in partnership with Monash University.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

Australian Organisation:

Australia and New Zealand Vasculitis Society (ANZVASC)
Website: https://www.anzvasculitis.org/

The Australian and New Zealand Vasculitis Society (ANZVASC) is a collaborative organisation of physicians, scientists, and communities that focuses on research, education, and clinical practices in vasculitis.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Vasculitis varies between individuals, and each person’s experience is unique.   

Please visit Australia and New Zealand Vasculitis Society (ANZVASC): Patient Stories to read the personal stories of people living with vasculitis. 

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

Australia and New Zealand Vasculitis Society (ANZVASC): Patient Support lists a range of support resources for individuals with vasculitis.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on vasculitis be found at:

• healthdirect: Vasculitis
• National Organization for Rare Disorders (NORD): Vasculitis

Australasian Society of Clinical Immunology and Allergy (ASCIA): Vasculitis Disorders

Australia and New Zealand Vasculitis Society (ANZVASC): Medical Professionals 

Orphanet: Vasculitis 

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.