What is vasculitis?
Vasculitis is inflammation of the blood vessels. While in essence it sounds relatively harmless vasculitis can lead to serious organ damage, especially in the kidneys (the condition effectively turns the body's immune system on itself).
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is a type of autoimmune disease which affects small blood vessels. Normally, antibodies are produced by white blood cells to help fight infection, but in ANCA associated vasculitis, abnormal autoantibodies are formed which attack one's own cells or tissues; specifically a white blood cell called a neutrophil.
Vasculitis may not be as well-known as its autoimmune disease cousins lupus and multiple sclerosis (MS), however improving outcomes for patients with this debilitating, often fatal and little-known inflammatory disease is a key research focus for clinician-scientists in the School of Clinical Sciences at Monash Health.
What are the symptoms?
ANCA vasculitis varies in severity and may result in inflammation in a number of organ systems. Fever, joint aches, skin rash, and fatigue are common symptoms. There may also be involvement of the eyes, sinuses, gastrointestinal tract and nervous system. Bleeding into the lungs, called pulmonary haemorrhage, and kidney involvement, called ANCA associated glomerulonephritis, are the most severe manifestations and may be life threatening.
Who does it affect?
Although lacking the profile of other autoimmune diseases, vasculitis in Australia has a similar incidence to MS; 1/50,000 people, and most common in people aged 65-74 years. Genetic and environmental factors can both play a role in the development of this disease. Without treatment, 85 per cent of patients with vasculitis currently die within five years. Even with treatment, up to 30 per cent of patients die and half of those die from the treatment itself.
Treatment of vasculitis
Treatment of vasculitis depends on the severity and the organ system involved. Left untreated, severe vasculitis results in very poor outcomes. Current treatment options for severe vasculitis are medications like steroids, cyclophosphamide and rituximab, which supress the immune system and therefore aim to improve the autoantibody related inflammation. However, these medications are non-specific and associated with serious side effects, most prominently an increased risk of infection and an increased risk of cancer with cyclophosphamide.
Vasculitis research at Monash
Autoimmune Kidney Disease and Vasculitis Research Group
We are working to better understand the mechanism of disease in ANCA vasculitis in order to define specific, less toxic therapies for this disease. Finding targeted treatments that produce fewer side-effects and greater quaility of life is a key aspect of our work.
Our researchers are clinician-scientists - a typical day at work might see them in the lab conducting research, teaching medical students face-to-face or online, and seeing patients in our clinics.
Find out more about our research.
The Monash Health Vasculitis Clinic
The new Monash Health Vasculitis Clinic, led by nephrologist and physician-scientist Professor Richard Kitching, from the Monash Health Department of Nephrology, and Head of the Centre for Inflammatory Diseases (CID) in the School of Clinical Sciences at Monash Health, is a collaboration between renal (kidney) medicine and rheumatology.
Find out more and make an appointment with the Monash Health Vasculitis Clinic.
Recommended vasculitis resources
For more information we recommend these resources:
- Vasculitis Foundation
- The Centre for Personalised Immunology
- Australasian Society of Clinical Immunology and allergy (ASCIA)
Image: Damaging white blood cells (green) and myeloperoxidase (red) in the kidney of a person with vasculitis.
From the work of the Autoimmune Kidney Disease and Vasculitis Group, Ms Kim O'Sullivan.