What is Vasculitis?
Vasculitis is a group of disorders characterised by inflammation of the blood vessels (arteries, veins, and capillaries). When blood vessel walls become inflamed, they can become weakened, stretch and bulge (aneurysm), or narrow and close off, restricting blood flow to organs and tissues. This can damage affected organs and may be life-threatening if not treated.
Types of Vasculitis
- Giant cell arteritis (GCA) — affects large arteries, especially the temporal artery; most common in older adults
- ANCA-associated vasculitis (AAV) — includes granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA)
- IgA vasculitis (Henoch-Schönlein purpura) — common in children
- Polyarteritis nodosa
- Behçet's disease
- Kawasaki disease — in children
Symptoms
Symptoms vary depending on the type of vasculitis and the organs affected, but general signs include:
- Fever and fatigue
- Weight loss
- Purpura — reddish-purple spots on the skin
- Joint and muscle pain
- Numbness or weakness
- Organ-specific symptoms (e.g. blood in urine for kidney involvement, shortness of breath for lung involvement)
Treatment
Treatment depends on the type and severity. Most types are treated with corticosteroids to reduce inflammation. Immunosuppressant medications (such as cyclophosphamide, methotrexate, or rituximab) are used for severe or refractory disease. Early diagnosis and treatment are essential to prevent permanent organ damage.
Urgent warning signs
Some types of vasculitis can damage the eyes, kidneys, lungs or nerves quickly, so a few symptoms need urgent attention.
- A new headache, scalp tenderness, jaw pain when chewing or any change in vision, especially if you are over 50: contact your GP the same day or call NHS 111. If you have lost vision, go to A&E. This can be giant cell arteritis.
- Coughing up blood or sudden breathlessness: call 999 or go to A&E.
- Blood in your urine, a new rash of purple spots, sudden numbness, weakness or foot drop, or feeling very unwell with fever: get medical help the same day.
The main types
Vasculitis is grouped by the size of the blood vessels involved.
- Large vessels: giant cell arteritis (GCA), which affects arteries in the head and is the most common type in older adults and often overlaps with polymyalgia rheumatica; and Takayasu arteritis, which is rare and usually affects younger people.
- Medium vessels: polyarteritis nodosa and Kawasaki disease, which mainly affects young children.
- Small vessels: granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA), often grouped as ANCA-associated vasculitis; and IgA vasculitis (Henoch-Schönlein purpura), most common in children.
- Vasculitis linked to other conditions, such as lupus, rheumatoid arthritis, infections including hepatitis, or certain medicines.
How it is diagnosed
Diagnosis combines your symptoms with tests. Blood tests look for inflammation (such as CRP and ESR), kidney function and particular antibodies. A urine dip test checks for blood or protein that may signal kidney involvement, and it is simple, quick and important. Scans such as ultrasound, CT or PET, and sometimes a biopsy of affected tissue, help confirm the type and extent. Because vasculitis can affect several organs, you may see more than one specialist.
Treatment
Treatment aims to switch off the inflammation quickly to protect organs, then keep it under control with the lowest effective treatment. Steroids are often used first because they work fast, with the dose reduced gradually over time. Other immune-calming medicines, such as methotrexate, azathioprine, mycophenolate, rituximab or cyclophosphamide, may be added to control the disease and reduce the steroid dose. Some biologics are also used for specific types. Your team will explain the monitoring your medicines need, including blood tests, bone protection and infection precautions.
Living with vasculitis
Many people reach remission, although vasculitis can come back, so ongoing follow-up matters. Learn the early signs of a relapse that apply to you and how to contact your specialist team. Keep your vaccinations up to date as advised, particularly if you take immunosuppressants. Stay as active as you can, as this helps with fatigue, mood and bone health after steroids. Tell every health professional you see about your diagnosis and medicines, and carry a steroid card if you have been given one.
About this page
Written by Louis Maxwell, First Contact Practitioner (HCPC PH128483), and pending clinical review. This is general information, not a diagnosis. Always follow the advice of your own GP, physiotherapist or rheumatology team. For emergencies call 999; for urgent advice use NHS 111.