ConditionBlood & immune system
Vasculitis
Vasculitis is inflammation of the blood vessels. The inflamed vessel walls can narrow, weaken or close, reducing blood flow to the organs they supply. There are many types, classified by the size of vessels involved, from giant cell arteritis in large arteries to IgA vasculitis in small vessels of the skin and kidneys. Symptoms vary widely: fever, tiredness, weight loss, rashes, joint pain, numbness, headaches or kidney problems. Most forms are treated with steroids and other immune-suppressing medicines, and early treatment prevents organ damage.
Also called: Angiitis, arteritis, blood vessel inflammation, vasculitides
At a glance
- What it is
- Inflammation of blood vessel walls, causing narrowing, blockage or weakening.
- Classified by vessel size
- Large (giant cell, Takayasu), medium (polyarteritis nodosa, Kawasaki), small (ANCA-associated, IgA vasculitis), variable (Behçet's).
- Symptoms
- Fever, fatigue, weight loss, rash or purple spots, joint and muscle pain, numbness, headache, kidney or lung problems.
- Diagnosis
- Blood and urine tests, ANCA, imaging, and biopsy of an affected organ.
- Treatment
- Corticosteroids plus immunosuppressants or biologics; aspirin and IVIG for Kawasaki disease.
When to get help
Call 911 or go to the ER
- Sudden vision loss, double vision or jaw pain on chewing with a new headache in someone over 50 (possible giant cell arteritis)
- Coughing up blood or severe breathlessness with a rash or kidney problems
- Signs of stroke: face drooping, arm weakness, speech difficulty
- A child with 5 or more days of fever with red eyes, rash, red cracked lips, swollen hands and feet (possible Kawasaki disease)
See a doctor within a day or two
- A new rash of raised purple spots on the legs, especially with joint pain, belly pain or blood in the urine
- Numbness or weakness in a hand or foot (foot drop) with fever or weight loss
- Blood or protein in the urine with swelling or rising blood pressure
- New headache and scalp tenderness in someone over 50
Book a routine appointment
- Unexplained fever, night sweats, weight loss and fatigue for weeks
- Painful fingers that turn white or blue in the cold, with skin ulcers
- Recurrent mouth and genital ulcers
How vasculitis causes symptoms
In vasculitis, the immune system attacks blood vessel walls. Depending on the vessels affected, this can:
- narrow or block vessels, reducing blood supply (ischemia) to organs
- weaken the wall, causing an aneurysm or bleeding
- produce body-wide inflammation, causing fever and fatigue
Because any organ can be involved, symptoms vary greatly from person to person.
Main types of vasculitis
| Vessel size | Type | Typical features |
|---|---|---|
| Large | Giant cell arteritis | Over 50; new headache, scalp tenderness, jaw pain when chewing, vision loss; often with polymyalgia rheumatica |
| Large | Takayasu arteritis | Young women; arm pain with use, weak pulses, different blood pressures in each arm |
| Medium | Polyarteritis nodosa | Nerve damage, skin nodules and ulcers, belly pain, high blood pressure; sometimes linked to hepatitis B |
| Medium | Kawasaki disease | Young children; prolonged fever, rash, red eyes and lips; can damage heart arteries |
| Small (ANCA-associated) | Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis | Sinus and nose problems, lung nodules or bleeding, kidney inflammation; EGPA with asthma and high eosinophils |
| Small | IgA vasculitis (Henoch-Schönlein purpura) | Mainly children; purpura on legs and buttocks, joint pain, belly pain, kidney involvement |
| Small | Cutaneous small-vessel vasculitis | Purple spots on the legs, often triggered by drugs or infections |
| Small | Cryoglobulinemic vasculitis | Often linked to hepatitis C |
| Variable | Behçet's disease | Recurrent mouth and genital ulcers, eye inflammation, skin lesions, blood clots |
Vasculitis can also occur secondary to lupus, rheumatoid arthritis, infections, cancers and medicines.
Symptoms
General
- fever, night sweats
- fatigue
- weight loss
- muscle and joint aches
Organ-specific
- skin: palpable purpura (raised purple spots that do not blanch), ulcers, nodules, livedo (a lacy purple pattern)
- nerves: numbness, tingling, weakness, foot or wrist drop (mononeuritis multiplex)
- kidneys: blood or protein in urine, high blood pressure, kidney failure
- lungs: cough, breathlessness, coughing blood
- ears, nose and sinuses: chronic sinusitis, nosebleeds, crusting, hearing loss
- eyes: redness, pain, vision loss
- gut: belly pain, bleeding
- heart and brain: chest pain, stroke
Diagnosis
- history and examination, including blood pressure in both arms and pulses
- blood tests: inflammatory markers (ESR, CRP), blood count, kidney and liver function, ANCA, complement levels, hepatitis B and C, cryoglobulins
- urine tests for blood and protein, a key early sign of kidney involvement
- imaging: ultrasound of temporal arteries, CT or MR angiography, PET scans for large-vessel vasculitis; chest CT for lung involvement
- biopsy of an affected organ (skin, kidney, temporal artery, nerve) to confirm the diagnosis
Treatment
Treatment depends on the type and severity:
- corticosteroids such as prednisone, often at high doses initially, then tapered
- immunosuppressants: methotrexate, azathioprine, mycophenolate
- biologics: rituximab for ANCA-associated vasculitis; tocilizumab for giant cell arteritis; mepolizumab for EGPA
- cyclophosphamide for severe organ-threatening disease
- avacopan to reduce steroid exposure in ANCA-associated vasculitis
- plasma exchange in selected severe cases
- IVIG and aspirin for Kawasaki disease
- treating the trigger: stopping a causative drug, treating hepatitis B or C
- supportive care: blood pressure control, bone protection while on steroids, vaccines and infection prevention
Mild skin-limited vasculitis may settle with rest, leg elevation and stopping the trigger.
Outlook
Outcomes have improved dramatically. Many people achieve remission with treatment, though relapses are common in some types and long-term monitoring is needed. Prompt treatment prevents lasting organ damage such as blindness or kidney failure.
Frequently asked questions
What is vasculitis?
Vasculitis is inflammation of the blood vessels caused by the immune system attacking the vessel walls. It can narrow or block vessels and damage the organs they supply, such as the skin, kidneys, nerves, lungs and eyes.
What are the symptoms of vasculitis?
Common symptoms include fever, tiredness, weight loss and aches, plus organ-specific signs such as a purple spotty rash, numbness, headaches, vision changes, sinus problems, coughing blood or blood in the urine.
What causes vasculitis?
Most vasculitis is caused by an autoimmune process with no clear trigger. Some cases are linked to infections such as hepatitis B or C, medicines, cancers or other autoimmune diseases like lupus and rheumatoid arthritis.
Can vasculitis be cured?
Some forms, such as drug-induced or IgA vasculitis in children, often resolve completely. Many others can be brought into long-term remission with treatment, though relapses can occur and ongoing monitoring is needed.
Sources
This guide was written from current patient and clinical guidance, including:
- American College of Rheumatology/EULAR: Classification criteria for vasculitis
- American College of Rheumatology/Vasculitis Foundation: Guideline for the management of ANCA-associated vasculitis and giant cell arteritis
- National Heart, Lung, and Blood Institute: Vasculitis
- Vasculitis Foundation: Types of vasculitis
- MedlinePlus: Vasculitis