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Patient education: Vasculitis (Beyond the Basics)

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Peter A Merkel, MD, MPH
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Eric L Matteson, MD, MPH
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Monica Ramirez Curtis, MD, MPH
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INTRODUCTION

The word “vasculitis” means inflammation of blood vessels. Blood vessels include the huge network of arteries and veins that deliver blood from the heart to all of the organs and tissues throughout the body and then return the blood back to the heart. Blood vessels range in size from very large (eg, the aorta, the body's largest artery which carries blood from the heart) branching out to very small (eg, capillaries that bring oxygen to the body's tissues).

The inflammation caused by vasculitis can affect the lining of the blood vessels (called the endothelium) or the wall of an artery or vein. This can cause the vessel to become thickened, weakened, narrowed (stenosis), blocked (occlusion), enlarged (aneurysm), or scarred. A damaged vessel may not function normally, which can affect blood flow to the tissues that the vessel normally serves. This can lead to partial or complete organ failure due to lack of blood flow, or bleeding into the skin or other part of the body due to rupture of the blood vessel wall. This damage is sometimes permanent.

Some types of vasculitis resolve without treatment, while others require lifelong treatment with medicine. Fortunately, treatments can usually control or minimize vessel damage in the short term. However, both short- and long-term side effects of these treatments are common. Furthermore, relapses of vasculitis (also known as “flares”) are also common. It is extremely important that patients with vasculitis see their doctor or nurse on a regular basis.

VASCULITIS CAUSES

In most cases, the cause of vasculitis is unknown. A combination of factors likely sets the inflammatory process in motion.

Vasculitis can occur in conjunction with another illness, such as lupus or rheumatoid arthritis. (See "Patient education: Rheumatoid arthritis symptoms and diagnosis (Beyond the Basics)" and "Patient education: Systemic lupus erythematosus (SLE) (Beyond the Basics)".)

Vasculitis can develop as a reaction to certain drugs or other substances (called drug-induced vasculitis).

Vasculitis can also occur as a result of infection such as the following viruses: hepatitis B or C, HIV (the virus that causes AIDS), cytomegalovirus, Epstein-Barr virus, or parvovirus B19. Vasculitis can also occur with some infections caused by bacteria.

The group of diseases labeled as “vasculitis” often refers to several types of vasculitis with no obvious cause, also known as “idiopathic” vasculitis; this set of diseases is the focus of this discussion.

TYPES OF VASCULITIS

There are many different types of vasculitis. The types are grouped based on the size of the blood vessels affected: large, medium, and small. Some types of vasculitis are more serious than others. The names and characteristics of the most common types of vasculitis are listed below.

Large-vessel vasculitis — Vasculitis that affects large arteries includes Takayasu arteritis and giant cell (temporal) arteritis.

Takayasu arteritis — Takayasu arteritis usually affects the main artery that receives blood from the heart (the aorta) and its branches (figure 1).

Takayasu arteritis is quite rare. While seen throughout the world, it is more common in parts of Asia and the Middle East than in North America or Northern Europe. The disease usually affects women who are between 10 and 40 years old at the time of diagnosis. Common symptoms include pain and weakness when trying to use the arms or legs (called claudication). Other organs, such as the intestines, can also be affected, causing abdominal pain after eating. If the heart is affected, there can be chest pain with activity (eg, walking, running). (See "Patient education: Peripheral artery disease and claudication (Beyond the Basics)".)

Takayasu arteritis is diagnosed by looking at the arteries with an imaging test. This usually involves magnetic resonance imaging (MRI) or computed tomography (CT). Other possible tests include angiography or arteriography, which uses an injection of dye into a vein to see the blood vessels of the heart with X-ray. (See "Clinical features and diagnosis of Takayasu arteritis".)

Giant cell arteritis — Giant cell arteritis can affect the aorta and its branches. Another name for giant cell arteritis is temporal arteritis. Temporal refers to the arteries of the face and scalp, particularly those near the temples (figure 2).

Common symptoms of giant cell arteritis include headache, tiring of jaw muscles during chewing, and visual changes or loss of vision.

More detailed information about giant cell arteritis is available separately. (See "Patient education: Polymyalgia rheumatica and giant cell (temporal) arteritis (Beyond the Basics)" and "Diagnosis of giant cell (temporal) arteritis".)

Medium-sized vessel vasculitis — Some types of vasculitis affect medium-sized arteries.

Polyarteritis nodosa — Polyarteritis nodosa causes inflammation of medium to small arteries, decreasing or blocking blood flow to certain parts of the body. This can damage the skin, kidneys, intestines, heart, or nerves, or cause other problems. The majority of cases of polyarteritis nodosa is caused by infection with the hepatitis C virus. Polyarteritis nodosa is diagnosed with an imaging test like arteriography or by a sample of tissue (a biopsy) from an involved blood vessel. (See "Clinical manifestations and diagnosis of polyarteritis nodosa in adults".)

Kawasaki disease — Kawasaki disease is an arteritis of large, medium, and small arteries, particularly the arteries in the heart. The disease mainly occurs in young children. (See "Kawasaki disease: Clinical features and diagnosis" and "Kawasaki disease: Initial treatment and prognosis".)

Small-vessel vasculitis — Several types of vasculitis affecting small vessels (very small arteries, arterioles, capillaries, and small veins [venules]) are described below. In some cases, the exact type of small-vessel vasculitis is unknown.

ANCA-associated vasculitis — Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of three diseases that share similar symptoms and organ involvement:

Eosinophilic granulomatosis with polyangiitis (EGPA, also called Churg-Strauss syndrome)

Granulomatosis with polyangiitis (GPA, also called Wegener's granulomatosis)

Microscopic polyangiitis

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) — EGPA occurs almost exclusively in people who have asthma. The condition often causes damage to the lungs as well as damage to other tissues. To confirm the diagnosis, a doctor will take a sample of tissue (biopsy) of the lung or other involved tissue. (See "Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)".)

Granulomatosis with polyangiitis (Wegener’s) — GPA usually affects the nose, sinuses, lungs, and kidneys. A biopsy of the lining of the nose, sinus, part of a lung, or kidney can confirm the diagnosis. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis".)

Microscopic polyangiitis — Microscopic polyangiitis usually affects the lungs, kidneys, or nerves in the same way that GPA affects these organs. A biopsy of a lung or kidney can confirm the diagnosis. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis".)

IgA vasculitis (Henoch-Schönlein) — IgA vasculitis (also called Henoch-Schönlein purpura) usually affects children, although it can occasionally affect adults. Symptoms include abdominal and joint pain; a rash consisting of small, red to purple, slightly raised areas (picture 1); and kidney involvement that may cause the urine to appear bloody or darkly colored, like tea or coffee.

IgA vasculitis is diagnosed based on the symptoms and characteristic rash. A skin or kidney biopsy can confirm the diagnosis. (See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis" and "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management".)

Cryoglobulinemic vasculitis — Cryoglobulins are a specific type of protein in the blood; when the patient’s serum (the liquid part of the blood) is cooled, the cryoglobulins form visible clumps (called precipitates or cryoglobulins). There are several types of cryoglobulins that can lead to vasculitis; this disease is most commonly caused by infection with hepatitis C virus, although other causes are also possible. Common symptoms and problems of cryoglobulinemic vasculitis include:

Muscle and joint pain

Fatigue

Raised red bumps on the legs (purpura)

Kidney disease

Other major organ problems

Cryoglobulinemic vasculitis is diagnosed with a positive blood test for cryoglobulins combined with relevant symptoms and/or a biopsy (for example of the kidney). (See "Patient education: Renal (kidney) biopsy (Beyond the Basics)".)

Treatment of cryoglobulinemic vasculitis is centered around treating the patient for hepatitis C if the virus is present, and sometimes treatment with drugs that suppress the immune system. (See 'Vasculitis treatment' below.)

Behçet's syndrome — Behçet's syndrome is an inflammatory disease that can affect both small and large vessels which can lead to blockage of blood vessels, aneurysm (weakening of the vessel wall), and blood clots.

The diagnosis of Behçet's syndrome is based on a combination of signs and symptoms of disease. Some of the symptoms include:

Ulcers (sores) inside the mouth (picture 2) or on the genitals

Eye problems

A rash or sore on the skin

Joint pain and swelling

The treatment of Behçet's syndrome depends on which symptoms are present. (See "Clinical manifestations and diagnosis of Behçet’s syndrome" and "Treatment of Behçet’s syndrome".)

Single-organ vasculitis — Single-organ vasculitis refers to vasculitis that affects arteries or veins of any size in a single organ, but does not affect other parts of the body. However, some patients initially diagnosed with single-organ vasculitis may eventually develop another type of vasculitis.

Primary central nervous system vasculitis — Primary central nervous system vasculitis is one example of single-organ vasculitis. Also called isolated central nervous system vasculitis or primary angiitis of the central nervous system, it affects medium-sized and small arteries in the brain. It is a rare form of vasculitis and tends to occur in middle-aged people. Common symptoms include headache, confusion, and stroke.

VASCULITIS SYMPTOMS

Symptoms of vasculitis vary from one person to another and depend upon the type of vasculitis and location in the body of inflamed vessels. Some common symptoms include:

Fatigue or weakness

Fever

Muscle and joint pain

Rash

Lack of appetite and weight loss

Abdominal pain

Kidney problems (bloody or dark-colored urine)

Nerve problems (numbness, weakness, pain)

VASCULITIS DIAGNOSIS

It can be difficult to diagnose vasculitis because the symptoms are similar to those caused by a number of other illnesses. Your doctor or nurse will talk to you, will perform a physical exam, and may order tests to help establish a diagnosis.

The tests used to diagnose vasculitis depend on the type of vasculitis that is suspected and may include blood or urine tests, imaging tests (like X-ray or magnetic resonance imaging [MRI]), nerve testing, or lung function testing. A biopsy is often required to be sure of the diagnosis before starting treatment.

VASCULITIS TREATMENT

The treatment of vasculitis will depend on the specific type of vasculitis and the areas of the body that are involved.

General measures — Treatment may include one or more of the following approaches.

Glucocorticoids (also called “steroids”), such as prednisone, can be taken by mouth (as a pill) in most cases; high doses may be given into a vein. Because there are risks when glucocorticoids are taken for long periods of time, the goal is to take them only as long as needed.

Some people require treatment with long-term glucocorticoids to control symptoms and prevent worsening of their condition. Close monitoring for possible side effects of glucocorticoids, such as diabetes, weight gain, or osteoporosis (bone thinning), is needed. There are medicines that can reduce the risk of bone fractures for people who take long-term glucocorticoids. (See "Patient education: Osteoporosis prevention and treatment (Beyond the Basics)".)

Additional treatment (beyond glucocorticoids) that suppresses the immune system might be needed for more serious types of vasculitis. One type of immunosuppressive medication, cyclophosphamide, has dramatically improved the outlook for people with some types of vasculitis.

Azathioprine, methotrexate, and mycophenolate are medicines that also suppress the immune system but are not as strong as cyclophosphamide. These treatments can be used for less severe forms of vasculitis and as maintenance therapy after treatment with cyclophosphamide. (See "Patient education: Disease-modifying antirheumatic drugs (DMARDs) (Beyond the Basics)".)

A newer set of medications, called “biologics,” are increasingly being used to treat some forms of vasculitis. These newer drugs include rituximab, tocilizumab, and anti-tumor necrosis factor (TNF) medications.

All immunosuppressive medications have the potential to increase the risk of infections, and each type of medication has other important side effects that must be monitored for by a doctor.

WHERE TO GET MORE INFORMATION

Your healthcare provider is the best source of information for questions and concerns related to your medical problem.

This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.

Patient level information — UpToDate offers two types of patient education materials.

The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.

Patient education: Polymyalgia rheumatica and giant cell arteritis (The Basics)
Patient education: Henoch-Schönlein purpura (IgA vasculitis) (The Basics)
Patient education: Optic neuritis (The Basics)
Patient education: Polyarteritis nodosa (The Basics)
Patient education: Vasculitis (The Basics)
Patient education: Antinuclear antibodies (The Basics)
Patient education: Behçet's syndrome (The Basics)
Patient education: Kawasaki disease (The Basics)

Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.

Patient education: Rheumatoid arthritis symptoms and diagnosis (Beyond the Basics)
Patient education: Systemic lupus erythematosus (SLE) (Beyond the Basics)
Patient education: Peripheral artery disease and claudication (Beyond the Basics)
Patient education: Polymyalgia rheumatica and giant cell (temporal) arteritis (Beyond the Basics)
Patient education: Renal (kidney) biopsy (Beyond the Basics)
Patient education: Osteoporosis prevention and treatment (Beyond the Basics)
Patient education: Disease-modifying antirheumatic drugs (DMARDs) (Beyond the Basics)

Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.

Alternative agents in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis
Overview of and approach to the vasculitides in adults
Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Clinical features and diagnosis of Takayasu arteritis
Clinical manifestations and diagnosis of polyarteritis nodosa in adults
Clinical manifestations and diagnosis of rheumatoid vasculitis
Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis
Clinical manifestations of giant cell (temporal) arteritis
Clinical manifestations of relapsing polychondritis
Clinical manifestations of vasculitic neuropathy
Clinical spectrum of antineutrophil cytoplasmic antibodies
Diagnosis of giant cell (temporal) arteritis
Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Gastrointestinal manifestations of vasculitis
Overview of cutaneous small vessel vasculitis
Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis
Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis
Overview of the management of vasculitis in adults
Pathogenesis of granulomatosis with polyangiitis and related vasculitides
Primary angiitis of the central nervous system in adults
Identification and management of relapsing disease in granulomatosis with polyangiitis and microscopic polyangiitis
Serum sickness and serum sickness-like reactions
Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Treatment-resistant granulomatosis with polyangiitis and microscopic polyangiitis
Treatment of giant cell (temporal) arteritis
Treatment of Takayasu arteritis
Kawasaki disease: Clinical features and diagnosis
Kawasaki disease: Initial treatment and prognosis
Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis
Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management
Treatment of the mixed cryoglobulinemia syndrome
Clinical manifestations and diagnosis of Behçet’s syndrome

The following organizations also provide reliable health information.

National Library of Medicine

(www.nlm.nih.gov/medlineplus/vasculitis.html, available in Spanish)

National Heart Lung and Blood Institute

(www.nhlbi.nih.gov/health/dci/Diseases/vas/vas_whatis.html)

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Literature review current through: Aug 2016. | This topic last updated: Aug 24, 2016.
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