Vasculitis: Symptoms, Types & Treatment Guide

What Is Vasculitis and How Does It Affect the Body?

Vasculitis is a group of autoimmune diseases that cause inflammation in blood vessel walls, potentially affecting vessels of any size throughout the body. This inflammation can restrict blood flow or cause blood vessel walls to weaken, leading to organ damage if untreated. The condition affects approximately 40-54 people per million annually.

The term vasculitis comes from the Latin words "vas" (vessel) and "-itis" (inflammation), literally meaning inflammation of blood vessels. When blood vessels become inflamed, the walls can thicken, narrow, or weaken. This disrupts normal blood flow and can deprive tissues and organs of the oxygen and nutrients they need to function properly.

Vasculitis can affect blood vessels of any size in any part of the body. Large vessel vasculitis affects the aorta and its major branches. Medium vessel vasculitis primarily affects arteries that supply blood to organs like the kidneys, intestines, and heart. Small vessel vasculitis affects the smallest blood vessels including capillaries, venules, and arterioles that reach throughout every organ and tissue.

The underlying cause of most vasculitis types remains unknown, though researchers believe it involves an abnormal immune response where the body's defense system mistakenly attacks blood vessel walls. In some cases, vasculitis can be triggered by infections (particularly hepatitis B and C), medications, or other autoimmune conditions. The immune system produces antibodies and inflammatory cells that attack the vessel walls, causing damage and inflammation.

Different types of vasculitis tend to affect different organs and have distinct patterns of involvement. For example, giant cell arteritis typically affects arteries in the head and can cause vision problems, while ANCA-associated vasculitis often affects the kidneys and lungs. Understanding which type of vasculitis a person has is crucial for determining the most appropriate treatment approach.

How Vasculitis Is Classified

Medical professionals classify vasculitis primarily by the size of the blood vessels affected. This classification system, developed by the Chapel Hill Consensus Conference and updated in 2012, helps guide diagnosis and treatment decisions. The three main categories are large vessel vasculitis, medium vessel vasculitis, and small vessel vasculitis.

Within each category, specific conditions are further distinguished by their characteristic features, such as the type of antibodies involved, the organs primarily affected, and the appearance of affected tissues under microscopy. Some forms of vasculitis overlap between categories, and a person may have features of more than one type.

What Are the Common Symptoms of Vasculitis?

Common vasculitis symptoms include fatigue, fever, unexplained weight loss, and general malaise. Specific symptoms depend on which organs are affected: headache and vision changes (giant cell arteritis), skin rashes and purpura (small vessel vasculitis), joint and muscle pain, numbness or weakness in limbs, shortness of breath, and kidney problems.

Vasculitis symptoms typically develop gradually over weeks to months, though some forms can appear suddenly. The symptoms experienced depend heavily on which blood vessels are affected and which organs are involved. Most people with vasculitis experience a combination of general (systemic) symptoms along with organ-specific symptoms.

General symptoms that occur across most types of vasculitis include persistent fatigue that doesn't improve with rest, low-grade fever that comes and goes, unintentional weight loss, night sweats, and a general feeling of being unwell (malaise). These non-specific symptoms often appear before more distinctive signs develop, which can make early diagnosis challenging.

The pattern and combination of symptoms often provide important clues about which type of vasculitis is present. For instance, a person over 50 with new severe headaches and jaw pain when chewing likely has giant cell arteritis, while someone with recurrent nosebleeds, bloody urine, and lung nodules may have granulomatosis with polyangiitis (formerly Wegener's granulomatosis).

Skin Symptoms

Skin involvement is one of the most visible and common manifestations of vasculitis, particularly in small vessel vasculitis. Purpura, which appears as small red or purple spots caused by bleeding under the skin, is characteristic of vasculitis and differs from other rashes because it doesn't fade when pressed (non-blanching). The spots may be flat or slightly raised (palpable purpura).

Other skin manifestations include ulcers that may be painful and slow to heal, livedo reticularis (a lacy, net-like pattern of discoloration), nodules (firm bumps under the skin), and digital ischemia (fingers or toes that become pale, blue, or painful due to reduced blood flow). In some cases, skin lesions may develop areas of tissue death (necrosis) if blood supply is severely compromised.

Eye Symptoms

Eye involvement can be a serious complication of several vasculitis types. Giant cell arteritis can cause sudden vision loss if the blood vessels supplying the eye become affected, making it a medical emergency. Other eye symptoms include redness, pain, double vision, and inflammation of various eye structures (uveitis, scleritis, episcleritis).

Respiratory Symptoms

When vasculitis affects the lungs or upper respiratory tract, symptoms may include shortness of breath, persistent cough, coughing up blood (hemoptysis), recurrent sinus infections, nosebleeds, and ear problems including hearing loss. These symptoms are particularly common in ANCA-associated vasculitis, which frequently involves the respiratory system.

Kidney Symptoms

Kidney involvement is a common and potentially serious complication of vasculitis. Early kidney damage may cause no noticeable symptoms, which is why regular urine tests are important for people with vasculitis. When symptoms do occur, they may include blood in the urine (which may appear pink, red, or brown), foamy urine (indicating protein), decreased urine output, high blood pressure, and swelling in the legs, ankles, or around the eyes.

Musculoskeletal Symptoms

Joint pain and swelling (arthralgia and arthritis) are common across many vasculitis types, often affecting multiple joints simultaneously. The joints most commonly affected include the knees, ankles, wrists, and elbows. Muscle pain (myalgia) and muscle weakness can also occur, particularly when vasculitis affects the blood vessels supplying muscles.

Neurological Symptoms

Nerve involvement in vasculitis can cause peripheral neuropathy, manifesting as numbness, tingling, burning sensations, or weakness in the hands and feet. A characteristic pattern called mononeuritis multiplex involves sudden damage to two or more separate nerves in different parts of the body. Central nervous system involvement, though less common, can cause headaches, strokes, seizures, or cognitive changes.

Gastrointestinal Symptoms

When vasculitis affects blood vessels in the digestive system, it can cause abdominal pain (particularly after eating), nausea, vomiting, diarrhea, and blood in the stool. Severe gastrointestinal involvement can lead to bowel perforation or significant bleeding, which are medical emergencies.

Cardiac Symptoms

Heart involvement may cause chest pain, shortness of breath with exertion, palpitations, or symptoms of heart failure. Some types of vasculitis can affect the coronary arteries (blood vessels supplying the heart muscle) or cause inflammation of the heart muscle itself (myocarditis) or the sac surrounding the heart (pericarditis).

What Are the Different Types of Vasculitis?

Vasculitis is classified by vessel size: Large vessel types include giant cell arteritis (most common over age 50) and Takayasu arteritis. Medium vessel types include polyarteritis nodosa and Kawasaki disease. Small vessel types include ANCA-associated vasculitis (GPA, MPA, EGPA) and IgA vasculitis (Henoch-Schönlein purpura, most common in children).

Understanding the different types of vasculitis is essential because each type has distinct characteristics, affects specific organs, and requires tailored treatment approaches. The classification system organizes vasculitis primarily by the size of blood vessels affected, which correlates with the clinical features and prognosis of each condition.

Large Vessel Vasculitis

Giant Cell Arteritis (GCA), also called temporal arteritis, is the most common form of vasculitis in adults over 50 years old. It primarily affects the large arteries of the head, particularly the temporal arteries (located near the temples), but can also involve the aorta and its branches. Approximately 15-20 people per 100,000 over age 50 develop GCA each year, with women affected about twice as often as men.

The hallmark symptoms of GCA include severe, new-onset headache (often described as unlike any previous headache), scalp tenderness, jaw pain when chewing (jaw claudication), and visual disturbances. Vision loss is the most feared complication, occurring in 15-20% of patients, and can become permanent if not treated promptly. Other symptoms include fatigue, fever, and muscle stiffness, particularly in the shoulders and hips (polymyalgia rheumatica occurs in about 40-60% of GCA patients).

Takayasu Arteritis is a rare form of large vessel vasculitis that primarily affects the aorta and its major branches. Unlike GCA, it typically occurs in younger people, most commonly women under 40 years old. It's sometimes called "pulseless disease" because inflammation can reduce or block blood flow in major arteries, leading to weak or absent pulses in the arms or legs.

Symptoms of Takayasu arteritis can be subtle initially and may include fatigue, weight loss, muscle aches, and low-grade fever. As the disease progresses, more specific symptoms develop depending on which arteries are affected, such as arm pain or weakness with use, dizziness, fainting, high blood pressure, chest pain, and vision changes. The disease progresses through an inflammatory phase followed by a fibrotic (scarring) phase that can permanently narrow affected arteries.

Medium Vessel Vasculitis

Polyarteritis Nodosa (PAN) is a rare vasculitis affecting medium-sized arteries, particularly those supplying the skin, nerves, muscles, kidneys, and gastrointestinal tract. It does not typically affect the lungs, which helps distinguish it from ANCA-associated vasculitis. PAN can be associated with hepatitis B infection, though most cases have no identifiable cause.

Symptoms of PAN are highly variable and depend on which organs are affected. Common manifestations include skin changes (nodules, ulcers, livedo reticularis), peripheral neuropathy causing pain, numbness, and weakness, muscle pain, abdominal pain (especially after eating), hypertension due to kidney involvement, and testicular pain in men. The disease can range from mild to life-threatening depending on the extent of organ involvement.

Kawasaki Disease is an acute vasculitis that almost exclusively affects children, typically under 5 years of age. It's the leading cause of acquired heart disease in children in developed countries because it can cause coronary artery aneurysms if not treated promptly. The cause is unknown, though it may be triggered by infections in genetically susceptible individuals.

Kawasaki disease is characterized by prolonged fever (at least 5 days) along with several distinctive features: red eyes without discharge (conjunctivitis), changes in the lips and mouth (red, cracked lips; strawberry tongue), skin rash, changes in the hands and feet (swelling, redness, later peeling), and enlarged lymph nodes in the neck. Early treatment with intravenous immunoglobulin (IVIG) dramatically reduces the risk of coronary artery complications.

Small Vessel Vasculitis

ANCA-Associated Vasculitis refers to a group of three conditions characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) in the blood: Granulomatosis with Polyangiitis (GPA, formerly Wegener's), Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss).

Granulomatosis with Polyangiitis (GPA) commonly affects the upper and lower respiratory tract and kidneys. Classic symptoms include chronic sinusitis, nosebleeds, ear problems, cough (sometimes with blood), and kidney inflammation (glomerulonephritis). The condition forms granulomas (clusters of inflammatory cells) in affected tissues. Without treatment, GPA can be life-threatening, but with modern immunosuppressive therapy, many patients achieve remission.

Microscopic Polyangiitis (MPA) primarily affects the kidneys and lungs but doesn't form granulomas. It often presents with rapidly progressive glomerulonephritis (quickly worsening kidney function) and pulmonary hemorrhage (bleeding into the lungs). MPA tends to affect older adults and can be difficult to distinguish from GPA based on symptoms alone.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is distinguished by its association with asthma and elevated eosinophils (a type of white blood cell). Patients typically have a history of adult-onset asthma and allergic rhinitis before vasculitis develops. EGPA can affect the lungs, heart, nerves, and gastrointestinal tract. Heart involvement is the main cause of mortality in EGPA.

IgA Vasculitis (Henoch-Schönlein Purpura) is the most common vasculitis in children, typically affecting those between 4 and 10 years old, though it can occur in adults. It's characterized by a distinctive rash (palpable purpura) typically on the legs and buttocks, joint pain and swelling, abdominal pain, and kidney involvement.

The condition often follows an upper respiratory infection and is usually self-limiting in children, resolving within a few weeks to months without long-term complications. Adults with IgA vasculitis tend to have more severe disease and are more likely to develop chronic kidney problems. Treatment is mainly supportive, though severe cases may require corticosteroids or other immunosuppressive medications.

When Should You Seek Medical Care for Vasculitis?

Seek medical care promptly if you experience unexplained fatigue, fever, weight loss lasting more than a few weeks, or any organ-specific symptoms. Seek emergency care immediately for sudden vision loss, severe new headache (especially if over 50), difficulty breathing, coughing blood, chest pain, or stroke symptoms like sudden weakness or speech problems.

Because vasculitis symptoms can be vague and develop gradually, knowing when to seek medical evaluation is important. Many vasculitis symptoms overlap with common, less serious conditions, but certain combinations of symptoms or their persistence should prompt medical attention. Early diagnosis and treatment can prevent serious organ damage and improve long-term outcomes.

Contact a healthcare provider if you experience persistent, unexplained symptoms lasting more than a few weeks, particularly if you have multiple symptoms that don't have an obvious explanation. Key symptoms warranting evaluation include unexplained fatigue that significantly affects daily activities, recurrent low-grade fevers, unintentional weight loss of more than 5-10 pounds, persistent muscle or joint aches, unusual rashes (especially purpura), recurring sinus infections or nosebleeds, blood in urine, or numbness and tingling in hands or feet.

If you have a known diagnosis of vasculitis, contact your healthcare team if you experience any new or worsening symptoms, as this may indicate a disease flare or treatment side effects. Regular follow-up appointments are essential even when feeling well, as some complications (particularly kidney problems) may develop without causing noticeable symptoms initially.

How Is Vasculitis Diagnosed?

Vasculitis diagnosis requires a combination of clinical evaluation, blood tests (inflammatory markers, ANCA antibodies, organ function tests), urine analysis, imaging studies (CT angiography, MRI, PET scan), and often tissue biopsy to confirm inflammation in blood vessels. No single test diagnoses vasculitis; doctors piece together findings from multiple sources.

Diagnosing vasculitis can be challenging because symptoms vary widely, overlap with many other conditions, and no single test can definitively confirm the diagnosis. The diagnostic process typically involves a thorough medical history, physical examination, laboratory tests, imaging studies, and often tissue biopsy. Specialists such as rheumatologists, nephrologists, or pulmonologists often coordinate the evaluation depending on which organs are affected.

The medical history focuses on understanding the pattern of symptoms, their timeline, and any potential triggers. Doctors will ask about the specific symptoms experienced, when they started, how they've progressed, any medications being taken (some can cause drug-induced vasculitis), recent infections, and personal or family history of autoimmune conditions. A detailed physical examination looks for signs of vasculitis including skin changes, joint swelling, neurological abnormalities, and signs of organ involvement.

Blood Tests

Several blood tests help evaluate for vasculitis and assess organ function. Inflammatory markers including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are often elevated during active vasculitis but are nonspecific. A complete blood count may show anemia (common in chronic inflammation), elevated white blood cells, or abnormal platelet counts.

ANCA testing (anti-neutrophil cytoplasmic antibodies) is crucial for diagnosing ANCA-associated vasculitis. Two main patterns are recognized: c-ANCA (cytoplasmic pattern, usually indicating antibodies against proteinase 3, associated with GPA) and p-ANCA (perinuclear pattern, usually indicating antibodies against myeloperoxidase, associated with MPA and some cases of EGPA). However, ANCA can be negative in some vasculitis cases and positive in other conditions, so results must be interpreted in clinical context.

Other antibody tests may include antinuclear antibodies (ANA), complement levels, cryoglobulins, and hepatitis B and C testing (as these infections can trigger certain vasculitis types). Kidney function tests (creatinine, estimated glomerular filtration rate) and liver function tests help assess organ involvement.

Urine Tests

Urinalysis is important for detecting kidney involvement, which occurs in many vasculitis types. Findings suggestive of kidney inflammation include blood in the urine (hematuria), protein in the urine (proteinuria), and red blood cell casts (clumps of red blood cells that form in damaged kidney tubules). A 24-hour urine collection may be needed to quantify protein loss.

Imaging Studies

Various imaging techniques help visualize blood vessel inflammation and organ involvement. CT angiography (CTA) and magnetic resonance angiography (MRA) can show narrowing, blockages, or aneurysms in medium and large blood vessels. These are particularly useful for diagnosing Takayasu arteritis and large vessel involvement in GCA.

PET-CT scans (positron emission tomography combined with CT) can detect areas of active inflammation in blood vessel walls and are increasingly used to diagnose and monitor large vessel vasculitis. Ultrasound of the temporal arteries can show characteristic changes in giant cell arteritis and is often the first imaging test performed when GCA is suspected.

Chest X-rays and CT scans of the chest assess for lung involvement, which may show nodules, infiltrates, or signs of bleeding in the lungs. CT scans of the sinuses help evaluate upper respiratory tract involvement in GPA.

Tissue Biopsy

Biopsy of affected tissue often provides definitive diagnosis by showing inflammation in blood vessel walls under microscopic examination. The location of biopsy depends on which organs are affected and accessible. Common biopsy sites include skin (for skin vasculitis), kidney (when kidney involvement is suspected), temporal artery (for suspected GCA), nerve (for vasculitic neuropathy), and lung (when lung involvement is present).

Biopsy findings vary by vasculitis type but generally show inflammation in and around blood vessel walls, with possible damage to the vessel wall structure, thickening or narrowing of the vessel, and sometimes granuloma formation (in GPA and EGPA). A negative biopsy doesn't always rule out vasculitis, as the inflammation can be patchy and may be missed if the biopsy sample doesn't include an affected area.

Additional Tests

Depending on symptoms and suspected vasculitis type, additional tests may be needed. Pulmonary function tests assess lung capacity if respiratory symptoms are present. Nerve conduction studies and electromyography (EMG) evaluate for peripheral neuropathy. Echocardiography (heart ultrasound) assesses heart function and structure. Lumbar puncture (spinal tap) may be needed if central nervous system vasculitis is suspected.

How Is Vasculitis Treated?

Vasculitis treatment typically involves two phases: induction (achieving remission using corticosteroids with immunosuppressants like cyclophosphamide or rituximab) and maintenance (preventing relapse using lower-intensity medications like methotrexate or azathioprine). Treatment duration varies from months to years depending on vasculitis type and disease course.

The primary goals of vasculitis treatment are to control inflammation, prevent organ damage, achieve remission, and maintain remission while minimizing treatment side effects. Treatment approaches vary based on the type of vasculitis, severity of disease, organs involved, and individual patient factors. Most treatment regimens involve medications that suppress the immune system to reduce the abnormal inflammatory response.

Treatment is typically divided into two phases: induction therapy (intensive treatment to bring active disease under control and achieve remission) and maintenance therapy (less intensive treatment to prevent relapses once remission is achieved). The specific medications and duration of each phase depend on the vasculitis type and individual response to treatment.

Induction Therapy

Corticosteroids (such as prednisone or methylprednisolone) are the foundation of treatment for most vasculitis types. They rapidly reduce inflammation and are usually started at high doses, then gradually tapered as the disease comes under control. High-dose intravenous corticosteroids (pulse therapy) may be used initially for severe disease or life-threatening organ involvement.

While highly effective, long-term high-dose corticosteroid use causes significant side effects including weight gain, increased blood sugar, high blood pressure, bone loss (osteoporosis), increased infection risk, mood changes, and skin thinning. Therefore, doctors aim to reduce corticosteroid doses as quickly as safely possible by adding other immunosuppressive medications.

Cyclophosphamide is a powerful immunosuppressant traditionally used for severe vasculitis, particularly ANCA-associated vasculitis with kidney or lung involvement. It can be given orally or intravenously (IV pulse therapy). While effective, cyclophosphamide has significant potential side effects including increased infection risk, bladder toxicity, reduced fertility, and increased long-term cancer risk. Its use is typically limited to the induction phase due to these concerns.

Rituximab is a biologic medication that depletes B cells (a type of immune cell) and has become a first-line option for ANCA-associated vasculitis. Clinical trials have shown it's as effective as cyclophosphamide for inducing remission with a different side effect profile. Rituximab is given as IV infusions and is now widely used both for induction and maintenance therapy.

Maintenance Therapy

Once remission is achieved, maintenance therapy aims to prevent disease relapses while minimizing long-term treatment side effects. Common maintenance medications include:

Methotrexate is taken weekly (usually orally) and is commonly used for maintenance in GPA and other vasculitis types. It requires regular blood test monitoring and is typically taken with folic acid to reduce side effects.

Azathioprine is an immunosuppressant used for maintenance in various vasculitis types. It requires regular blood monitoring and dosing may need adjustment based on genetic testing (TPMT enzyme activity).

Mycophenolate mofetil is another immunosuppressant option, particularly useful for patients who can't tolerate other maintenance medications.

Rituximab is increasingly used for maintenance therapy in ANCA-associated vasculitis, given as periodic infusions every 6 months or based on monitoring of B cell levels and ANCA titers.

Treatment for Specific Vasculitis Types

Giant Cell Arteritis: Treatment begins with high-dose corticosteroids, which should be started immediately when GCA is suspected (even before biopsy confirmation) to prevent vision loss. Tocilizumab (a biologic medication blocking interleukin-6) is now approved as a steroid-sparing treatment for GCA and allows faster corticosteroid tapering. Low-dose aspirin is often recommended to reduce the risk of stroke and heart attack.

Takayasu Arteritis: Corticosteroids are first-line treatment. Additional immunosuppressants (methotrexate, azathioprine, or biologics like tocilizumab or TNF inhibitors) are often needed. Some patients may require surgical procedures or angioplasty/stenting to treat damaged blood vessels.

IgA Vasculitis (HSP): Mild cases, especially in children, often resolve without specific treatment beyond supportive care (rest, pain relief, adequate hydration). More severe cases, particularly with kidney involvement, may require corticosteroids or other immunosuppressive therapy.

Kawasaki Disease: Standard treatment is high-dose intravenous immunoglobulin (IVIG) plus aspirin, which dramatically reduces the risk of coronary artery complications when given within the first 10 days of illness. Some children who don't respond to initial IVIG may need additional treatments.

Treatment for Organ Damage

In addition to treating the vasculitis itself, patients may need treatment for organ damage caused by the disease. This might include dialysis for kidney failure, antihypertensive medications for high blood pressure, cardiac medications for heart involvement, or surgical intervention for severely damaged blood vessels (angioplasty, stenting, or bypass surgery).

What Is It Like Living with Vasculitis?

Many people with vasculitis can live full, active lives with proper treatment and monitoring. Living well requires understanding your condition, attending regular follow-up appointments, recognizing signs of flares, managing medication side effects, and making healthy lifestyle choices. Support from healthcare teams, family, and patient organizations helps cope with the challenges of chronic illness.

Living with vasculitis presents both physical and emotional challenges, but with proper management, most people can maintain a good quality of life. The experience varies considerably depending on the type of vasculitis, organs affected, disease severity, and response to treatment. Understanding what to expect and how to actively participate in your care can help you navigate life with this condition.

The initial period after diagnosis often involves frequent medical appointments, tests, and adjustments to treatment. As the disease comes under control and treatment is optimized, appointments typically become less frequent. However, ongoing monitoring remains important even during remission because vasculitis can relapse and some complications develop gradually without symptoms.

Regular Monitoring and Follow-Up

Regular follow-up appointments allow your healthcare team to assess disease activity, monitor for organ damage, adjust medications, and detect treatment side effects. The frequency of visits depends on disease activity and treatment phase, ranging from every few weeks during active disease to every few months during stable remission.

Routine monitoring typically includes blood tests (to check inflammatory markers, organ function, and medication effects), urine tests (to monitor kidney health), and periodic imaging studies as needed. ANCA levels may be monitored in ANCA-associated vasculitis, though rising levels don't always predict relapse and treatment decisions aren't based on ANCA levels alone.

Recognizing Disease Flares

Vasculitis relapses (flares) are common, occurring in approximately 30-50% of people with ANCA-associated vasculitis within 5 years, even with maintenance therapy. Learning to recognize early signs of a flare allows for prompt treatment and can prevent serious complications. Warning signs may include return of original symptoms, new symptoms, increased fatigue, low-grade fever, or general feeling of being unwell.

It's important to distinguish between vasculitis flares, medication side effects, and other conditions (like infections). Contact your healthcare team if you notice concerning changes rather than assuming it's a flare and adjusting medications on your own.

Lifestyle Considerations

Healthy lifestyle choices support overall well-being and may help manage vasculitis and treatment side effects. A balanced, nutritious diet supports immune function and helps manage weight gain from corticosteroids. Regular physical activity (as tolerated and approved by your doctor) helps maintain muscle strength, bone health, cardiovascular fitness, and mood. Adequate rest is important, especially during disease flares when fatigue is common.

Avoiding smoking is crucial as smoking worsens blood vessel health and may interfere with treatment effectiveness. Limiting alcohol consumption is generally recommended, especially when taking certain medications like methotrexate. Sun protection is important because some immunosuppressive medications increase skin sensitivity to sun damage.

Infection Prevention

Immunosuppressive medications increase susceptibility to infections, making prevention strategies important. Washing hands frequently, avoiding close contact with sick individuals, food safety practices, and staying current with recommended vaccinations all help reduce infection risk. Report symptoms of infection (fever, chills, cough, painful urination, skin changes) to your healthcare team promptly, as infections may require early treatment and temporary adjustment of immunosuppressive medications.

Pregnancy Considerations

If you have vasculitis and are considering pregnancy, planning ahead is essential. Some vasculitis medications (like cyclophosphamide and methotrexate) can harm a developing fetus and must be stopped well before conception. Other medications may be continued during pregnancy under careful monitoring. Disease activity should ideally be well-controlled before becoming pregnant, as active vasculitis during pregnancy carries risks for both mother and baby.

Work closely with your rheumatologist and an obstetrician experienced in high-risk pregnancies to plan and manage pregnancy safely. Medication adjustments may be needed, and more frequent monitoring is typically required throughout pregnancy and after delivery.

Emotional Well-Being

Living with a chronic illness like vasculitis can affect mental health and emotional well-being. Common experiences include anxiety about disease progression or relapses, frustration with symptoms and treatment side effects, grief over lifestyle changes, and depression. These feelings are normal, and support is available.

Talking to healthcare providers about emotional concerns, connecting with patient support organizations, joining support groups (in-person or online), and seeking counseling or therapy when needed can all help. Building a support network of family, friends, and others who understand chronic illness provides valuable emotional support.

What Is Vasculitis Like in Children?

IgA vasculitis (Henoch-Schönlein purpura) is the most common vasculitis in children, typically affecting ages 4-10 years. It usually resolves within weeks without long-term complications. Kawasaki disease is another important childhood vasculitis that can cause coronary artery problems if not treated promptly. Pediatric vasculitis generally has better outcomes than adult forms.

While vasculitis can occur at any age, certain types are more common in children, and childhood vasculitis often has different characteristics and outcomes than adult forms. IgA vasculitis (formerly Henoch-Schönlein purpura or HSP) is by far the most common vasculitis in children, while Kawasaki disease is an important cause of acquired heart disease in young children.

IgA Vasculitis in Children

IgA vasculitis most commonly affects children between 4 and 10 years old, occurring in approximately 10-20 per 100,000 children annually. It often develops a few weeks after an upper respiratory infection (like a cold). Boys are affected slightly more often than girls.

The characteristic features of IgA vasculitis in children include a distinctive rash (palpable purpura) that typically appears on the legs and buttocks, though it can spread to the arms and trunk. The spots don't blanch (fade) when pressed. Joint pain and swelling, most commonly affecting the knees and ankles, occurs in about 75% of children. Abdominal pain, which can be severe and may include bloody stools, occurs in about 50-75%. Kidney involvement (blood or protein in urine) affects about 20-50% but is usually mild.

The good news is that most children with IgA vasculitis recover fully within a few weeks to months without any long-term problems. Treatment is mainly supportive, including rest, adequate hydration, and pain relief with nonsteroidal anti-inflammatory drugs (NSAIDs). Corticosteroids may be used for severe abdominal pain or significant kidney involvement.

While recurrences can happen (in about one-third of children), particularly in the first few months, repeated episodes are usually milder than the initial illness. Long-term kidney problems are uncommon in children (occurring in less than 2%) but are more common when initial kidney involvement is severe. Follow-up monitoring of blood pressure and urine tests is recommended for several months after the illness to detect any kidney problems.

Kawasaki Disease

Kawasaki disease primarily affects children under 5 years old and is characterized by prolonged fever (at least 5 days) along with specific clinical features: red eyes without discharge, changes in the mouth and lips (red, cracked lips, strawberry tongue), skin rash, changes in hands and feet (swelling, redness, later peeling), and swollen lymph nodes in the neck.

The main concern with Kawasaki disease is coronary artery involvement, which can lead to coronary artery aneurysms (abnormal widening) if not treated promptly. Without treatment, about 25% of children develop coronary artery abnormalities. With prompt treatment (high-dose intravenous immunoglobulin plus aspirin within 10 days of fever onset), this risk drops to about 4%.

Most children recover fully from Kawasaki disease with appropriate treatment. Those who develop coronary artery changes require long-term cardiac follow-up, and some may need blood-thinning medications or other cardiac interventions depending on the severity of involvement.