Vasculitis

Vasculitis

Author
Gene G Hunder, MD
Section Editor
John H Stone, MD, MPH
Deputy Editor
Paul L Romain, MD

Disclosures

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.

The inflammation caused by vasculitis can affect the lining of the blood vessels (endothelium) or the wall of an artery or vein. This can cause the vessel to become thickened, weakened, narrowed, or scarred.

The damaged vessel may not function normally, which can affect blood flow to the tissues that the vessel normally serves. Damaged blood vessels can lead to decreased blood flow, 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, long-term side effects of these treatments are possible, and it is important to see your doctor or nurse on a regular basis.

NORMAL BLOOD VESSELS — There are many different types of blood vessels in the body.

Arteries are part of the large system of blood vessels that delivers blood throughout the body. This includes large- and medium-sized arteries and smaller arterial branches (arterioles).
The arterioles deliver oxygen and nutrients to a network of tiny vessels called capillaries.
The capillaries drain into the venous system (veins) and help to remove waste products.
The smallest veins are venules; these connect to form veins.
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 information: Rheumatoid arthritis symptoms and diagnosis” and “Patient information: Systemic lupus erythematosus (SLE)”.)
Sometimes, vasculitis develops as a reaction to a drug or other substance (called hypersensitivity vasculitis).
In still other cases, vasculitis occurs with a viral illness, such as hepatitis B or C, HIV (the virus that causes AIDS), cytomegalovirus, Epstein-Barr virus, or parvovirus B19.
TYPES OF VASCULITIS — There are many different types of vasculitis. These are often classified according to the type and location of the blood vessels that are involved. Some types of vasculitis are more serious than others. The names and characteristics of the most common types of vasculitis are listed below. These are organized according to the size of the blood vessel affected.

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 not common in the United States. The disease usually affects women who are between 10 and 40 years old. 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; this can cause abdominal pain after eating. If the heart is affected, there can be chest pain with activity (walking, running). (See “Patient information: Peripheral artery disease and claudication”.)

Takayasu arteritis is diagnosed by looking at the arteries with an imaging test. This usually involves an MRI (magnetic resonance imaging). 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 is available separately. (See “Patient information: Polymyalgia rheumatica and giant cell (temporal) arteritis” and “Diagnosis of giant cell (temporal) arteritis”.)

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

Medium-sized vessel vasculitis can develop in people without other medical problems; this is called polyarteritis.
Medium-sized vessel vasculitis can also develop in people with certain medical problems, like rheumatoid arthritis, lupus, scleroderma, hairy cell leukemia, and hepatitis B or C infection.
Polyarteritis nodosa — Polyarteritis nodosa causes inflammation of medium to small arteries. In the skin, the inflammation causes the blood vessels to become thickened so that they can be felt. Some people develop skin ulcers (open sores).

Inflammation in the blood vessels caused by polyarteritis nodosa decreases or blocks blood flow to certain parts of the body. This can damage the kidneys, intestines, heart, or the nerves in the arms or legs.

Polyarteritis nodosa is diagnosed with an imaging test like arteriography or by taking a sample of tissue (a biopsy) from an involved blood vessel. (See “Clinical manifestations and diagnosis of polyarteritis nodosa”.)

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”.)

Primary central nervous system vasculitis — Primary central nervous system vasculitis (also called isolated central nervous system vasculitis) affects medium and small arteries in the central nervous system (the brain and spinal cord). It tends to occur in middle-aged people. Common symptoms include headache, confusion, and stroke.

Small vessel vasculitis — Several types of vasculitis affect small vessels (eg, very small arteries, arterioles, capillaries, and small veins [venules]).

Churg-Strauss vasculitis — Churg-Strauss vasculitis occurs almost exclusively in people who have asthma. The condition often causes lung damage. 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 Churg-Strauss syndrome (allergic granulomatosis and angiitis)”.)

Granulomatosis with polyangiitis (Wegener’s) — Granulomatosis with polyangiitis (Wegener’s) 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 (Wegener’s) and microscopic polyangiitis”.)

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

Henoch-Schönlein purpura is diagnosed based on the symptoms and characteristic skin rash. A skin or kidney biopsy can confirm the diagnosis. (See “Clinical manifestations and diagnosis of Henoch-Schönlein purpura” and “Management of Henoch-Schönlein purpura”.)

Cryoglobulinemia — Cryoglobulins are a combination of the body’s infection-fighting proteins (antibodies, immunoglobulins) and their target proteins (antigens). When the serum (liquid part) of the blood is cooled, the complexes become so large that they form visible clumps (called precipitates or cryoglobulins).

There are several types of cryoglobulinemia, and the symptoms depend upon the type. Common symptoms include:

Muscle and joint pain
Fatigue
Raised red bumps on the legs
These symptoms can get worse after being in cold temperatures, because the clumped immunoglobulins plug small blood vessels. (See “Treatment of essential mixed cryoglobulinemia”.)

Cryoglobulinemia is diagnosed with a blood test for cryoglobulins or a kidney biopsy. (See “Patient information: Renal (kidney) biopsy”.)

Hypersensitivity vasculitis — Hypersensitivity vasculitis can develop after taking a medicine. To be diagnosed with hypersensitivity-type vasculitis, a person must have the following characteristics:

Older than 16 years
Recently used a medicine that can cause the reaction
A skin reaction called purpura
A rash
A positive result on a biopsy (see “Hypersensitivity vasculitis in adults”)
Behcet’s disease — Behcet’s disease is an inflammatory disease that causes:

Ulcers (sores) inside the mouth (picture 2) or on the genitals
Eye problems
A rash or sore on the skin
Joint pain and swelling
Behcet’s disease 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 Behcet’s disease is based on symptoms. Unfortunately, there is no test that can tell for certain if a person’s symptoms are caused by Behcet’s disease. (See “Clinical manifestations and diagnosis of Behçet’s disease”.)

The treatment of Behcet’s disease depends on which symptoms are present.

VASCULITIS SYMPTOMS — Symptoms of vasculitis vary from one person to another and depend upon the type of vasculitis. Some common symptoms include:

Fatigue or weakness
Fever
Muscle and joint pain
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 and perform a physical exam, and may order tests.

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 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 measures.

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

Some people require long-term glucocorticoids to control symptoms and prevent worsening of their condition. Close monitoring for possible side effects of glucocorticoids (diabetes, weight gain, bone thinning) is needed. There are medicines that can reduce the risk of bone fractures for people who take long-term glucocorticoids. (See “Patient information: Osteoporosis prevention and treatment”.)
A treatment that suppresses the immune system might be needed for more serious types of vasculitis. This type of treatment is known as cytotoxic treatment. One cytotoxic medication, cyclophosphamide, has dramatically improved the outlook for people with some types of vasculitis.

Cytotoxic treatments can be used along with steroids and can be taken by mouth every day or given into a vein every three to four weeks. Treatment is usually continued until the disease is under control (called remission). Cytotoxic treatment can have serious side effects (eg, low blood cell counts, cancer). As a result, close monitoring is required.
Azathioprine and methotrexate are medicines that suppress the immune system but are not as strong as cytotoxic treatments. These treatments can be used for less severe forms of vasculitis and as maintenance therapy after treatment with cyclophosphamide.
Further information about azathioprine and methotrexate is available in a separate article. (See “Patient information: Disease modifying antirheumatic drugs (DMARDs)”.)

Hypersensitivity vasculitis treatment — Hypersensitivity vasculitis is treated differently than other types of vasculitis. In most people, stopping the medicine that caused the vasculitis is the only treatment needed. If your symptoms do not resolve after stopping the medicine or your symptoms are severe, your doctor or nurse might refer you to a vasculitis specialist, called a rheumatologist. (See “Hypersensitivity vasculitis in adults”.)

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 every four months on our Web site (www.uptodate.com/patients). Additional topics, as well as selected discussions written for healthcare professionals, are also available for those who would like more detailed information.

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:

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

Professional Level Information:

Alternative agents in the treatment of granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis
Classification of and approach to the vasculitides in adults
Clinical features and diagnosis of Churg-Strauss syndrome (allergic granulomatosis and angiitis)
Clinical features and diagnosis of Takayasu arteritis
Clinical manifestations and diagnosis of polyarteritis nodosa
Clinical manifestations and diagnosis of rheumatoid vasculitis
Clinical manifestations and diagnosis of granulomatosis with polyangiitis (Wegener’s) 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 Churg-Strauss syndrome (allergic granulomatosis and angiitis)
Gastrointestinal manifestations of vasculitis
Hypersensitivity vasculitis in adults
Initial immunosuppressive therapy in granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis
Maintenance immunosuppressive therapy in granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis
Overview of the management of the vasculitides in adults
Pathogenesis of granulomatosis with polyangiitis (Wegener’s) and related vasculitides
Primary angiitis of the central nervous system
Relapsing disease in granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis
Serum sickness and serum sickness-like reactions
Treatment and prognosis of Churg-Strauss syndrome (allergic granulomatosis and angiitis)
Treatment of cyclophosphamide-resistant granulomatosis with polyangiitis (Wegener’s) 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
Clinical manifestations and diagnosis of Henoch-Schönlein purpura
Management of Henoch-Schönlein purpura
Treatment of essential mixed cryoglobulinemia
Clinical manifestations and diagnosis of Behçet’s disease

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)

REFERENCES
Langford CA, Klippel JH, Balow JE, et al. Use of cytotoxic agents and cyclosporine in the treatment of autoimmune disease. Part 2: Inflammatory bowel disease, systemic vasculitis, and therapeutic toxicity. Ann Intern Med 1998; 129:49.
Langford CA. Chronic immunosuppressive therapy for systemic vasculitis. Curr Opin Rheumatol 1997; 9:41.
Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med 2003; 349:160.