Vasculitis

Introduction

Vasculitis is the medical name for a group of conditions in which the body’s immune system causes inflammation of the blood vessels. When a blood vessel becomes inflamed, its wall can thicken, narrow, weaken, or even burst. Because blood vessels run through every part of the body, vasculitis can affect almost any organ — the skin, joints, kidneys, lungs, nerves, eyes, intestines, or brain.

If you are reading this, you have most likely been told that you or a family member has vasculitis, or that doctors are investigating it. The word covers a wide range of diseases, from mild skin rashes that settle on their own to serious illnesses that need urgent and long-term treatment. The type of vasculitis, the organs involved, and how active the disease is all shape the treatment plan.

This article explains what vasculitis is, the main types, how it is diagnosed, the current approach to treatment, and what life with vasculitis often looks like over time. It is written for patients and families who are planning the next phase of care.

What Is Vasculitis?

Vasculitis means “inflammation of blood vessels.” In a healthy body, the immune system attacks germs and other threats. In vasculitis, the immune system mistakenly attacks the walls of the body’s own blood vessels. This inflammation can:

• Narrow the vessel, reducing blood flow to the tissues it supplies
• Block the vessel completely, causing tissue damage from lack of oxygen
• Weaken the vessel wall, allowing a bulge (aneurysm) to form
• Cause the vessel to leak or tear, leading to bleeding

Because the problem is with the blood vessels themselves, the symptoms depend entirely on where in the body the affected vessels are. Inflammation in small skin vessels may cause a rash. Inflammation in the kidneys may cause blood in the urine and high blood pressure. Inflammation in the temporal arteries of the scalp may cause a severe headache and vision changes. This is why vasculitis can look very different from one person to another.

Vasculitis is generally classified by the size of the blood vessels it affects: large vessels (the aorta and its main branches), medium vessels (the main arteries supplying organs), or small vessels (capillaries, venules, and arterioles). The most widely used classification system, the Chapel Hill Consensus Conference nomenclature, groups the named diseases by this vessel size and by the underlying mechanism.

Types of Vasculitis

There are many named forms of vasculitis. The list below covers the most common and clinically important types you may hear discussed.

Large-vessel vasculitis

Giant cell arteritis (GCA), also called temporal arteritis, mainly affects people over 50, particularly those over 70. It inflames the arteries of the scalp, head, and sometimes the aorta. Typical features include a new headache, scalp tenderness, jaw pain when chewing, and sudden vision loss in one eye. GCA is treated urgently because untreated vision loss can become permanent. It is often linked with another condition called polymyalgia rheumatica, which causes shoulder and hip stiffness.

Takayasu arteritis mainly affects younger people, usually women under 40. It inflames the aorta and its large branches, sometimes causing weak or absent pulses in the arms (hence the older name “pulseless disease”), differences in blood pressure between the two arms, dizziness, and limb pain with use.

Medium-vessel vasculitis

Polyarteritis nodosa (PAN) affects medium-sized arteries and can damage the skin, nerves, gut, kidneys, and other organs. It can be linked to hepatitis B infection in some cases.

Kawasaki disease is a vasculitis of childhood, most often affecting children under five. It can damage the coronary arteries of the heart if not treated promptly and is discussed further in the section on vasculitis in children.

Small-vessel vasculitis (ANCA-associated)

These conditions are linked by the presence of antibodies in the blood called anti-neutrophil cytoplasmic antibodies (ANCA). The three main types are:

• Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis. It often affects the upper airways (sinuses, nose), lungs, and kidneys.
• Microscopic polyangiitis (MPA). It often affects the kidneys and lungs, but typically does not involve the upper airways in the same way as GPA.
• Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome. It is usually preceded by adult-onset asthma and allergy symptoms and can affect the lungs, nerves, heart, and skin.

Small-vessel vasculitis (immune complex)

IgA vasculitis, previously called Henoch-Schönlein purpura, mainly affects children but can occur in adults. It causes a raised purple rash typically on the legs and buttocks, joint pain, abdominal pain, and sometimes kidney involvement.

Cryoglobulinemic vasculitis is linked to abnormal proteins called cryoglobulins in the blood, often associated with hepatitis C infection. It can cause skin rash, joint pain, nerve damage, and kidney problems.

Anti-GBM disease (formerly Goodpasture’s) is a rare but serious condition affecting the kidneys and lungs.

Variable-vessel vasculitis

Behçet’s disease can affect blood vessels of any size and is known for causing recurrent mouth ulcers, genital ulcers, eye inflammation, and skin lesions.

Single-organ and secondary vasculitis

Vasculitis can also be limited to a single organ, such as cutaneous (skin-limited) vasculitis or isolated central nervous system vasculitis. It can also occur as a complication of other diseases, such as rheumatoid arthritis or lupus, or as a reaction to certain infections, medications, or cancers. This is sometimes called secondary vasculitis.

Causes and Risk Factors

In most cases, the exact reason a person develops vasculitis is not known. It is not caused by anything you did or did not do. Doctors believe it usually results from a combination of factors:

• Genetic background. Certain genes can make a person more susceptible, although vasculitis is not directly inherited in most families.
• Immune system triggers. An infection, medication, or environmental exposure may trigger the immune system to attack the blood vessels in someone who is already susceptible.
• Other autoimmune diseases. Conditions such as rheumatoid arthritis, lupus, or Sjögren’s syndrome are associated with a higher risk of secondary vasculitis.
• Chronic infections. Hepatitis B is associated with some cases of polyarteritis nodosa. Hepatitis C is associated with cryoglobulinemic vasculitis.
• Medications. A small number of drugs can trigger a drug-induced vasculitis that often improves once the drug is stopped.
• Age and sex. Some types have strong age and sex patterns — giant cell arteritis in older adults, Takayasu arteritis in younger women, Kawasaki disease in young children.

Because vasculitis can affect almost any part of the body, symptoms vary widely. If you are already under investigation or have a diagnosis, the section that follows may help you understand the pattern of your illness and recognise signs of a flare — a return or worsening of disease activity.

General symptoms

Many people with active vasculitis feel generally unwell. Common features include:

• Fatigue that is out of proportion to activity
• Fevers, often low-grade and persistent
• Unintended weight loss
• Night sweats
• Muscle aches and joint pains
• Loss of appetite

Organ-specific symptoms

Symptoms depend on which vessels are inflamed:

• Skin: raised purple spots (purpura), often on the legs; ulcers; nodules under the skin; livedo (a lace-like purple pattern)
• Joints: pain and swelling, often in several joints
• Kidneys: usually silent in early stages, but tests may show blood and protein in the urine; later, swelling of the legs and high blood pressure
• Lungs: cough, shortness of breath, coughing up blood
• Upper airways: persistent blocked or runny nose, sinus pain, nosebleeds, crusting inside the nose, hearing loss
• Nerves: numbness, tingling, weakness, or pain — sometimes a specific pattern called mononeuritis multiplex, where individual nerves are affected one by one
• Eyes: redness, pain, blurred vision, sudden vision loss (an emergency)
• Brain: headache, stroke-like symptoms, seizures, confusion
• Gut: abdominal pain, particularly after eating; bleeding from the digestive tract
• Heart: chest pain, breathlessness, irregular heartbeat

Recognising a flare

If you have already been diagnosed, knowing what your own disease looked like at the start is useful, because flares often resemble the original presentation. New skin rash, new sinus crusting, new blood in the urine, new numbness, or a return of fevers and weight loss should prompt contact with your rheumatology team. Sudden severe symptoms — vision loss, severe breathlessness, coughing blood, stroke-like changes, or severe abdominal pain — need emergency care.

Diagnosis

Medical history and examination

Your doctor will ask in detail about your symptoms, their timeline, previous infections, medications, family history, and any earlier autoimmune diagnoses. The physical examination looks for skin changes, differences in pulses or blood pressure between limbs, joint swelling, neurological changes, and signs of organ involvement.

Blood and urine tests

Common tests include:

• Inflammatory markers: ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein) are typically raised in active vasculitis.
• Full blood count: can show anaemia or a high white cell count.
• Kidney and liver function tests.
• Urinalysis: looking for blood and protein, which can be an early sign of kidney involvement.
• ANCA testing: antibodies associated with GPA, MPA, and EGPA.
• Other autoantibodies: ANA, rheumatoid factor, complement levels, cryoglobulins.
• Hepatitis B and C testing, because these infections are linked to certain types.

Imaging

Imaging helps show where vessels are involved. Tests may include CT, MRI, ultrasound (often used for temporal arteries in suspected giant cell arteritis), angiography (CT, MR, or conventional), and PET-CT, which can show inflammation in large vessels.

Biopsy

For many forms of vasculitis, a small tissue sample — from skin, kidney, lung, nerve, or the temporal artery — gives the most definitive answer. The pathologist looks for the characteristic pattern of vessel inflammation under the microscope.

Bringing it together

Diagnosis often involves a rheumatologist working alongside specialists in nephrology (kidneys), pulmonology (lungs), ophthalmology (eyes), neurology, dermatology, or ear-nose-throat surgery, depending on the organs involved. The process can feel slow, but accurate classification matters because it shapes treatment.

Treatment and Management

The goal of vasculitis treatment is to calm the abnormal immune response, prevent damage to organs, and then keep the disease in remission — a state of low or absent activity — for as long as possible. Treatment usually has two phases: induction (bringing the disease under control) and maintenance (keeping it controlled).

Major international guidelines, including those of the American College of Rheumatology, the Vasculitis Foundation, and EULAR (European Alliance of Associations for Rheumatology), describe the broad principles below. The exact regimen depends on the type of vasculitis, the organs involved, the severity, your age, other health problems, and your response to treatment. Your rheumatologist will tailor the plan with you.

Glucocorticoids (steroids)

Glucocorticoids such as prednisolone or methylprednisolone work quickly to reduce inflammation and are the backbone of treatment for most serious vasculitis. In severe disease, very high intravenous doses (pulse steroids) may be used initially, followed by oral tablets that are gradually tapered over months. Current guidance from major societies favours faster steroid tapers than in the past, to limit long-term side effects.

Conventional immunosuppressants

To control the disease without relying on long-term high-dose steroids, doctors add other immunosuppressants. The choice depends on the type and severity:

• Cyclophosphamide: a strong immunosuppressant used in severe ANCA-associated vasculitis and some other forms, typically for a limited induction period.
• Methotrexate: used in milder disease and for maintenance.
• Azathioprine: commonly used for maintenance after remission.
• Mycophenolate mofetil: an alternative for maintenance, particularly when other options are not tolerated.
• Leflunomide: sometimes used as a maintenance option.

Biologic therapies

• Rituximab targets B cells and is now widely used both for induction and maintenance in ANCA-associated vasculitis. ACR and EULAR guidelines describe it as a first-line option alongside cyclophosphamide for severe disease, and as a preferred option in many situations such as relapsing disease or in younger patients where fertility preservation matters.
• Tocilizumab targets a pathway called IL-6 and is used in giant cell arteritis, allowing lower steroid exposure.
• Mepolizumab targets eosinophils and is used in eosinophilic granulomatosis with polyangiitis (EGPA).
• Avacopan, a newer oral medication that blocks part of the complement immune pathway, is approved in some countries for ANCA-associated vasculitis and can reduce the need for steroids.

Plasma exchange

Plasma exchange (plasmapheresis) removes antibodies from the blood and may be used in selected severe situations, such as rapidly progressive kidney failure or pulmonary haemorrhage in ANCA-associated vasculitis or anti-GBM disease. Recent evidence has refined when it is most useful, and current guidelines describe it as one option in carefully selected severe cases rather than a routine therapy.

Treatment of underlying causes

Where a specific trigger is identified, treating that trigger is part of the plan. Examples include antiviral treatment for hepatitis B–associated polyarteritis nodosa and for hepatitis C–associated cryoglobulinemic vasculitis, or stopping a medication that has caused drug-induced vasculitis.

Supportive treatment

Alongside the immune-targeted drugs, supportive care often includes:

• Medications to protect the stomach from steroids
• Bone protection (calcium, vitamin D, and sometimes bone-strengthening drugs) for those on long-term steroids
• Prevention of infections, including pneumocystis pneumonia in patients on strong immunosuppression
• Vaccinations planned around the immunosuppression schedule
• Blood pressure control, particularly when the kidneys are involved
• Pain management, physiotherapy, or rehabilitation as needed

Surgery and procedures

Surgery is sometimes needed for complications — for example, repairing damaged sinuses or narrowed airways in GPA, bypassing or stenting narrowed arteries in Takayasu arteritis, or repairing aneurysms. These are usually carried out once the disease is under good control.

Lifestyle and Self-Management

Day-to-day choices play an important role in living well with vasculitis, particularly while on immunosuppression.

• Take medications as prescribed. Stopping immunosuppressants or steroids suddenly can trigger flares or, in the case of steroids, cause serious withdrawal effects.
• Attend monitoring appointments. Many medications need regular blood tests to check that they are working safely.
• Look after your bones. Steroids can weaken bone over time. Adequate calcium, vitamin D, weight-bearing exercise, and not smoking all help.
• Watch your blood pressure and cardiovascular risk. Vasculitis itself and some treatments raise long-term cardiovascular risk. Healthy diet, regular activity, blood pressure control, and managing cholesterol all matter.
• Stop smoking. Smoking worsens almost every aspect of vasculitis, including lung involvement and cardiovascular risk.
• Eat well. A balanced diet, with attention to sodium intake if blood pressure or kidneys are affected, supports overall health. There is no specific “vasculitis diet.”
• Stay active. Gentle, regular exercise helps maintain strength, bone density, mood, and cardiovascular health. The level should match how well you feel.
• Mind mental health. Living with a chronic illness, side effects of treatment, and uncertainty about flares can be emotionally taxing. Counselling, peer support, and patient organisations help many people.
• Plan vaccinations. Inactivated vaccines (such as influenza and pneumococcal) are generally encouraged and timed around treatment. Live vaccines may need to be avoided on certain immunosuppressants — your rheumatologist will advise.
• Sun protection. Some medications, particularly methotrexate, increase sun sensitivity and long-term skin cancer risk.

Monitoring and Long-term Targets

Vasculitis is a long-term condition. Even when in remission, regular monitoring is important to catch flares early and to watch for treatment side effects and organ damage.

Monitoring usually includes:

• Regular rheumatology appointments — more frequent in early treatment and during tapering, less frequent in stable remission
• Blood tests for inflammation, kidney and liver function, blood counts, and drug levels where relevant
• Urine tests, particularly important for ANCA-associated vasculitis and lupus-related vasculitis where new blood or protein in the urine may signal a kidney flare
• Repeat ANCA testing in some forms, though ANCA levels do not always match disease activity
• Imaging to monitor large-vessel disease, such as periodic MRI or ultrasound in Takayasu arteritis or giant cell arteritis
• Eye checks if you are on long-term steroids or have eye involvement
• Bone density scans for those on prolonged steroid courses
• Cardiovascular risk assessment

The broad target is sustained remission with the lowest effective dose of immunosuppression, balanced against the risk of side effects. Some people are able to come off treatment entirely after a period of stable remission; others need long-term low-dose maintenance therapy.

Complications

Complications can arise from the disease itself, from organ damage that occurred before treatment took effect, or from treatment side effects.

From the disease

• Chronic kidney disease, sometimes progressing to the need for dialysis or transplant
• Permanent vision loss, particularly in untreated giant cell arteritis
• Lung scarring or recurrent bleeding
• Permanent nerve damage causing weakness or sensory loss
• Hearing loss or nasal deformity in GPA
• Strokes, aneurysms, or limb ischaemia from large-vessel involvement
• Bowel perforation or bleeding
• Heart involvement, including coronary artery damage

From treatment

• Increased risk of infections, including reactivation of latent infections such as tuberculosis or hepatitis B
• Osteoporosis from long-term steroids
• Diabetes and weight gain from steroids
• Cataracts and glaucoma from steroids
• Bladder problems and reduced fertility from cyclophosphamide
• Higher long-term risk of certain cancers, particularly bladder cancer and skin cancer, especially with cyclophosphamide and prolonged immunosuppression
• Hair thinning, mood changes, sleep disruption

Many of these can be reduced by careful drug choice, minimising steroid dose, and proactive screening. This is one reason guidelines have moved towards steroid-sparing strategies using rituximab, tocilizumab, and avacopan where appropriate.

Living with Vasculitis

Most people newly diagnosed with vasculitis have a lot of questions about how it will shape their everyday life. The honest answer is that experience varies. Many people, once in remission, return to full activity, work, and family life. Others live with some level of ongoing symptoms or organ damage that needs adjustment.

Work and activity

During active disease and the early months of treatment, fatigue and side effects often limit what you can do. Many people need time off work or reduced hours. Once in stable remission, most return to their previous activities. Jobs with very high infection exposure may require some adjustment while on strong immunosuppression.

Relationships and emotional health

Fertility, pregnancy, and contraception

Several vasculitis medications can affect fertility or are unsafe in pregnancy. Cyclophosphamide can reduce fertility, particularly in women approaching their late 30s and in men, so options to protect fertility may be discussed before treatment. Methotrexate and mycophenolate are unsafe in pregnancy and must be stopped well in advance of conception. Azathioprine and certain biologics can be used in pregnancy when needed. Pregnancy in vasculitis should be planned, ideally during stable remission and with input from a rheumatologist experienced in pregnancy care. Reliable contraception is important during treatment with teratogenic drugs.

Travel

Many people on stable treatment travel without difficulty. Travel vaccinations need planning, particularly for live vaccines. Carrying a summary of your diagnosis, current medications, and emergency contact information is useful, especially when travelling for medical care.

Peer support

Vasculitis patient organisations and online peer groups can be a valuable source of practical information and emotional support, particularly for the rarer forms of the disease where local support is limited.

Vasculitis in Children

Although vasculitis is more common in adults, several types occur in childhood. The two most common are IgA vasculitis and Kawasaki disease.

IgA vasculitis (formerly Henoch-Schönlein purpura)

IgA vasculitis is the most common vasculitis in children. It often follows a viral infection and causes:

• A raised purple rash, typically on the legs and buttocks
• Joint pain and swelling, often in the ankles and knees
• Abdominal pain, sometimes with nausea or vomiting
• Kidney involvement in a minority of cases, shown by blood and protein in the urine

Most children recover fully within weeks. Treatment is often supportive, with pain relief and monitoring. Steroids may be used for severe abdominal pain or kidney involvement. Children need a period of urine monitoring after the rash settles, because kidney involvement can develop or persist quietly.

Kawasaki disease

• Fever lasting more than five days
• Red eyes without discharge
• Red, cracked lips and a “strawberry” tongue
• Rash
• Swelling and redness of the hands and feet, later with peeling of the skin
• Swollen neck lymph nodes

Kawasaki disease is important because it can damage the coronary arteries of the heart, sometimes causing aneurysms. Prompt treatment with intravenous immunoglobulin (IVIG) and aspirin, ideally within the first 10 days, greatly reduces this risk. Children diagnosed with Kawasaki disease are followed up with echocardiograms to check the coronary arteries.

Other vasculitides in children

Less commonly, children can develop ANCA-associated vasculitis, Takayasu arteritis, polyarteritis nodosa, or primary central nervous system vasculitis. These are managed by paediatric rheumatologists, often together with paediatric nephrologists, pulmonologists, or neurologists. The general principles of induction and maintenance treatment are similar to those in adults, with attention to growth, development, schooling, and the special considerations of long-term immunosuppression starting in childhood.

Preventing Flares and Complications

While not every flare can be prevented, several actions reduce the risk and limit damage when flares occur.

• Stay on prescribed maintenance treatment. Stopping medication early is one of the most common reasons for relapse.
• Keep monitoring appointments, even when you feel well. Early changes in blood and urine tests can show a flare before symptoms appear.
• Know your warning signs. Note the symptoms you had at the original presentation and report similar new symptoms quickly.
• Manage infections carefully. Some infections can trigger flares. Vaccination, sensible hygiene, and prompt treatment of infections help.
• Avoid smoking and limit alcohol.
• Treat coexisting conditions — high blood pressure, diabetes, high cholesterol — that increase the risk of complications.
• Take care of your bones, heart, and mental health as long-term priorities, not just the immediate disease.

When to Seek Urgent Care

Some symptoms in vasculitis need urgent assessment because delay can lead to permanent damage. Seek emergency care if you experience:

• Sudden vision loss or significant new visual changes
• Severe new headache, particularly with scalp tenderness or jaw pain when chewing
• Sudden weakness or numbness on one side of the body, slurred speech, or other stroke-like symptoms
• Coughing up blood or significant new shortness of breath
• Severe abdominal pain, particularly with vomiting or blood in the stool
• Significant new blood in the urine, or a sudden drop in urine output
• High fever, especially if you are on immunosuppression
• New severe chest pain

If you are on immunosuppression and develop a fever, contact your healthcare team promptly even if the cause seems minor. Infections can progress more quickly in people whose immune systems are damped down.

Frequently Asked Questions

Is vasculitis curable?

Most forms of vasculitis are treatable rather than curable. Many people achieve long periods of remission, sometimes off all treatment. Some forms can recur years later. A few self-limiting forms, such as many cases of IgA vasculitis in children, do resolve completely.

Is vasculitis genetic? Will my children inherit it?

Vasculitis is generally not directly inherited. Genetic background can influence susceptibility, but most relatives of people with vasculitis do not develop it. The overall risk to children is low.

Is vasculitis contagious?

No. Vasculitis is not infectious and cannot be passed from person to person.

How long will I need treatment?

This depends on the type, severity, and how well the disease responds. Induction usually lasts three to six months. Maintenance treatment commonly continues for at least two years and sometimes much longer. Decisions about stopping treatment are individual.

Will I be on steroids forever?

Long courses of high-dose steroids are no longer the standard for most vasculitis types. Modern guidelines favour faster steroid tapers and earlier use of steroid-sparing medications such as rituximab. Some people remain on a low maintenance dose; others come off steroids entirely.

Can I exercise?

Yes, in most cases. During active disease, gentle activity is reasonable; during remission, regular exercise is encouraged for cardiovascular health, bone strength, and mood. If large arteries or the heart are involved, your specialist may suggest specific limits.

Can I get vaccinated?

Inactivated vaccines such as influenza and pneumococcal are generally recommended for people with vasculitis. Live vaccines may need to be avoided on some immunosuppressants. Vaccination is best planned around the treatment schedule with your rheumatology team.

Is pregnancy possible with vasculitis?

Many people with vasculitis have successful pregnancies. Planning is important: pregnancy is best timed during stable remission, and some medications need to be changed in advance. A team approach with rheumatology and obstetrics is usual.

What is the difference between vasculitis and lupus or rheumatoid arthritis?

All three are autoimmune diseases, but they affect different tissues. Vasculitis primarily inflames blood vessels. Lupus and rheumatoid arthritis can sometimes cause vasculitis as a complication, but their main targets are different (multiple organs in lupus; joints in rheumatoid arthritis).

How will my doctor know if the treatment is working?

Improvement is judged by symptoms, examination findings, blood and urine tests, and sometimes repeat imaging. Inflammatory markers like ESR and CRP often fall when treatment is working. ANCA levels may fall in ANCA-associated vasculitis but do not always match disease activity exactly.

Conclusion

Vasculitis is a broad family of conditions that share a common theme — inflammation of the blood vessels — but differ widely in how they appear and how they are treated. Modern care has shifted considerably in the last decade, with shorter steroid courses, wider use of targeted biologic medicines, and a stronger focus on protecting long-term health alongside controlling disease activity.

For most people with vasculitis, the path forward involves working closely with a rheumatologist over time, attending regular monitoring, taking treatment consistently, and learning the signs that suggest a flare. With current treatment, many people achieve long remissions and a good quality of life. Where organs have already been affected, ongoing care from a specialist team helps limit further damage and supports day-to-day living.