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Wegener’s Granulomatosis (WG)

What is Wegener’s Granulomatosis?

Wegener’s Granulomatosis (WG) is a rare form of vasculitis mainly involving small and medium-sized blood vessels. The disease can affect most parts of the body. It commonly affects the sinuses, nose, throat, lungs, ears, eyes, kidneys, skin, joints, brain and other parts of the nervous system. Not every one with Wegener’s granulomatosus is affected in the same way. Some patients have mild disease, while others may have severe damage to these organs which can be life-threatening.

Who gets Wegener's Granulomatosis?

Wegener’s Granulomatosus occurs in both men and women and can affect children and adults. Although Caucasians are affected more often, people all over the world can get Wegener’s Granulomatosus.

What causes Wegener's Granulomatosis?

Wegener's granulomatosis is thought to be an auto-immune disease for which there is no known cause.

How is Wegener's Granulomatosis diagnosed?

The diagnosis of Wegener’s Granulomatosis is made by combining clinical features with laboratory tests (including tests for ANCA) and biopsy of affected tissues.

What is the treatment for Wegener's Granulomatosis?

Treatment of Wegener's granulomatosis usually includes a combination of glucocorticoids and an immunosuppressive drug such as cyclophosphamide, methotrexate, or azathioprine. If diagnosed promptly, treatment can bring about early remission and prevent organ failure. It is a chronic disease, and although remission of symptoms is usually achieved, the relapse rate remains high.

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Microscopic Polyangiitis (MPA)

What is Microscopic Polyangiitis?

Microscopic Polyangiitis (MPA) is a rare form of vasculitis which mainly affects small and medium-sized blood vessels. The disease commonly affects the lungs, kidneys, skin, ears, and nervous system but can affect most parts of the body including the eyes, joints, and brain. Severe damage to these organs can occur. Involvement and severity varies widely from patient to patient, from mild to life-threatening.

Who gets Microscopic Polyangiitis?

MPA can affect children and adults and occurs in both men and women. Although Caucasians are affected more often, people all over the world can get MPA.

What causes Microscopic Polyangiitis?

Microscopic polyangiitis is thought to be an auto-immune disease for which there is no known cause.

How is Microscopic Polyangiitis diagnosed?

The diagnosis of MPA made by combining clinical features with laboratory tests (including tests for ANCA) and tissue biopsies.

What is the treatment of Microscopic Polyangiitis?

Like Wegener’s granulomatosis, treatment of MPA usually includes a combination of glucocorticoids and an immunosuppressive drug such as cyclophosphamide, methotrexate, or azathioprine. If diagnosed early, treatment can bring about early remission and prevent organ failure. It can be a chronic disease. While remission of symptoms is usually achieved, the relapse rate remains high.

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Churg-Strauss Syndrome (CSS)

What is Churg Strauss Syndrome?

Churg Strauss Syndrome (CSS), also known as “allergic granulomatosis”, is a rare form of vasculitis which, like Wegener’s Granulomatosis and microscopic polyangiitis, affects small and medium-sized vessels. Most patients with Churg Strauss syndrome have asthma, nasal and sinus allergies. Patients which CSS often have unusually high numbers of eosinophils, a type of while blood cell in their blood. Asthma, as well as the presence of eosinophils are common and by themselves are not enough to base the diagnosis of CSS on. Other problems caused by CSS include lung infiltrates, rashes, peripheral nervous system disease, abdominal pain, kidney, and cardiac disease.

Who gets Churg Strauss syndrome?

CSS affects people of all ages and both sexes.

What causes Churg Strauss Syndrome?

The cause of CSS is not known.

How is Churg Strauss Syndrome diagnosed?

CSS is diagnosed on the basis of a combination of symptoms and abnormal laboratory tests including an ANCA test as well as biopsy of affected tissues.

How is Churg Strauss Syndrome treated?

Treatment of Churg Strauss syndrome usually includes a combination of glucocorticoids and an immunosuppressive drug such as cyclophosphamide, methotrexate, or azathioprine. If diagnosed early, treatment can bring about early remission and prevent organ failure. It is important to understand that the asthma patients with CSS are treated in the same way as other patients with asthma. Worsening of asthma in patients with CSS does not necessarily mean that the CSS is active in other organs. Unfortunately, while remission of symptoms is usually achieved, the relapse rate remains high.

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Polyarteritis Nodosa (PAN)

What is Polyarteritis Nodosa?

Polyarteritis Nodosa (PAN) is a very rare form of vasculitis involving predominantly medium-sized blood vessels. Inflammation of the blood vessels may cause segments of vessels to weaken and stretch, resulting in an aneurysm (weakening of the vessel wall). Inflammation of the vessel wall can also lead to thickening and subsequent partial blockage (stenosis) or complete blockage (occlusion) of the artery. These aneurysms and blockages can result in the surrounding tissues being deprived of an adequate blood supply. The disease commonly affects the intestines, kidneys, skin, and peripheral nervous system but can affect most parts of the body. As is true of other forms of vasculitis, how PAN affects the body differs widely from patient to patient.

What causes Polyarteritis Nodosa?

PAN is thought to be an auto-immune disease for which there is no known cause in most cases. There is an important known association between infection with hepatitis B or hepatitis C viruses and development of PAN. However, only a small fraction of patients infected with these viruses develop PAN.

How is Polyarteritis Noda diagnosed?

The diagnosis of PAN is made by combining clinical features with the results of angiograms and tissue biopsies.

How is Polyarteritis Nodosa treated?

Treatment of PAN almost always involves use of glucocorticoids in high doses that are slowly reduced over many months. Often an immunosuppressive drug such as cyclophosphamide, methotrexate, or azathioprine, is added to the glucocorticoid therapy. If diagnosed early, treatment can bring about early remission and prevent organ failure. Unfortunately, while remission is usually achieved, relapses do occur.

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Takayasu's Arteritis (TAK)

What is Takayasu’s Arteritis?

Takayasu’s Arteritis (TAK) is a rare form of vasculitis affecting medium and large sized blood vessels, primarily of the aorta (the main blood vessel that leaves the heart) and its large branches going to the arms, abdominal organs, legs and the head. As with other forms of vasculitis, inflammation of the large blood vessels may cause segments of vessels to weaken and stretch, resulting in an aneurysm (weakening of the vessel wall) or, more commonly, the inflammation of the vessel wall leads to thickening and subsequent partial blockage (stenosis) or complete blockage (occlusion) of the artery. These blockages can result in the surrounding tissues being deprived of an adequate blood supply which causes mild to very severe problems including claudication (cramping) in the arms and legs, kidney damage with severe hypertension, strokes, or heart attacks. Many other symptoms and problems can be seen in TAK including joint pains, fevers, fatigue, and others.

Who gets Takayasu’s Arteritis?

Takayasu's arteritis generally first strikes people when they are young (teens, 20s or 30s), is much more common in women than men, and is more common in Asia. However, the disease is seen all over the world and most patients with Takayasu's arteritis in non-Asian countries are not of Asian ancestry.

What causes Takayasu’s Arteritis?

The cause of Takayasu’s arteritis is unknown.

How is Takayasu’s Arteritis diagnosed?

The diagnosis of TAK is based on a combination of symptoms and laboratory tests. These usually include angiography, a study of the blood flow in arteries. The angiogram can be done with dye injected into the arteries, by MRI, or by CT scan. The study shows the characteristic changes of blockage and widening of the arteries affected by TAK. Physical examination of patients with TAK often demonstrates reduced blood pressure readings in the arms with blockages and reduced pulses (hence another name of the disease is “Pulseless Disease”).

How is Takayasu’s Arteritis treated?

Treatment of TAK almost always involves use of glucocorticoids in high doses that are slowly reduced over many months. Often an immunosuppressive drug such as cyclophosphamide, methotrexate, and azathioprine, is added to the glucocorticoids. If diagnosed early, treatment can bring about early remission and prevent organ failure. Unfortunately, while remission is usually achieved, relapses occur frequently and TAK is often a chronic problem.

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Giant Cell (Temporal) Arteritis (GCA)

What is Giant Cell Arteritis?

Giant Cell Arteritis (GCA), also known as “Temporal Arteritis” or “Cranial Arteritis”, is a form of vasculitis affecting medium and large sized blood vessels, especially those of the aorta and arteries going from the aorta to the arms, legs and the head. GCA most frequently affects the arteries in the head, leading to narrowing and sometimes to complete blockage of the blood vessel. This results in the surrounding tissues being deprived of an adequate blood supply. When GCA involves the arteries that supply blood to the eyes, blindness in one or both eyes may develop suddenly. Along with visual changes, the most common symptoms in GCA include headaches, pain in the jaw or tongue muscles when eating or talking, tenderness of the scalp, fevers, and arthritis, particularly pain and stiffness of the shoulders and hips. This pain and stiffness of the shoulders and hips is called Polymyalgia rheumatica. Polymyalgia rheumatica can occur without giant cell arteritis.

Who gets Giant Cell Arteritis?

Although still a relatively rare disease, GCA is one of the most common types of vasculitis. GCA only affects people older than 50 years of age and especially those older than 65. Women are somewhat more likely to get the disease than men.

What causes Giant Cell Arteritis?

The cause of GCA is unknown.

How is Giant Cell Arteritis diagnosed?

The diagnosis of GCA depends on both clinical signs and symptoms and a combination of laboratory tests and tissue biopsy. At the time of diagnosis, most patients (90%) with GCA have an elevated erythrocyte sedimentation rate (ESR or “sed rate”), a non-specific blood marker of inflammation, and many patients have anemia. Biopsy of the temporal artery, located above and front of the ear, is a simple and relatively safe procedure that does not usually require staying in a hospital, is the most reliable method of diagnosing GCA. Some patients with negative biopsies can still have the disease.

How is Giant Cell Arteritis treated?

Treatment with glucocorticoids should begin as soon as the diagnosis is assumed to be likely. Glucocorticoids are often started even before a biopsy is performed to help prevent blindness or other problems. Glucocorticoids are given in high doses for several months and then slowly reduced. Many experts also prescribe a low-dose aspirin each day for patients with GCA. There are no other drugs, including the chemotherapy and other drugs which affect the immune system which are clearly proven to be of benefit in the treatment of GCA. Polymyalgia rheumatica without GCA is treated with doses of glucocorticoids lower than those used for treating GCA. The average duration of treatment is between two and three years, but this varied considerably for individual patients.

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Polymyalgia Rheumatica (PMR)

What is Polymyalgia Rheumatica?

Polymyalgia rheumatica (PMR) is a form of arthritis that affects older persons. It typically affects the shoulders and hips, as well as the neck and torso, causing aching and morning stiffness. PMR can cause puffiness with swelling of the hands and feet, but is different from rheumatoid arthritis. Rheumatoid arthritis can occur in people of any age, and typically causes swelling of the small joints of the hands and feet. Blood tests for rheumatoid arthritis are typically negative in PMR. PMR is often associated with Giant Cell Arteritis (GCA). About 15% of people who have PMR will develop Giant Cell Arteritis (GCA: put in link); therefore, patients with PMR need to be followed by physicians knowledgeable in both disorders. Patients with PMR also need to immediately report new symptoms to their doctors that might indicate the onset of GCA, especially headaches or visual changes.

The links between PMR and GCA are of great interest to researchers studying vasculitis. Some investigators think of the two disorders as part of the same disease spectrum, rather than two distinct diseases.

Who gets Polymyalgia Rheumatica?

PMR is relatively uncommon compared to rheumatoid arthritis or osteoarthritis. PMR only affects people older than 50 years of age and especially those older than 65. Women are somewhat more likely to get the disease than men. About 50% of people with GCA will also have PMR.

What causes Polymyalgia Rheumatica?

The cause of PMR is unknown.

How is Polymyalgia Rheumatica diagnosed?

The diagnosis of PMR depends on both clinical signs and symptoms, especially aching and stiffness of the shoulders, hips, torso and neck, often accompanied by malaise, weight loss, low grade fever, poor appetite, and fatigue. There is no diagnostic laboratory test for PMR, but at the time of diagnosis, as in GCA, most patients have an elevated erythrocyte sedimentation rate (ESR or “sed rate”), a non-specific blood marker of inflammation, and many patients have anemia. Routine X-rays of affected joints are generally normal for the age of the patient.

How is Polymyalgia Rheumatica treated?

Treatment with glucocorticoids results in prompt improvement of symptoms. The dose of glucocorticoids is lower than those used for GCA, usually between 15 and 20 mg a day initially, and then slowly reducing the dose. No other drugs have been clearly proven to be of benefit in the treatment of PMR. In most patients, the duration of the disease is about 2 ½ years, but this varies greatly in individuals.

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Other Types of Vasculitis

There are many different forms of vasculitis, and the VCRC is focusing on studying six different types:  Wegener’s Granulomatosis (WG), Microscopic Polyangiitis (MPA), Churg-Strauss Syndrome (CSS), Polyarteritis Nodosa (PAN), Takayasu’s Arteritis (TAK), and Giant Cell Arteritis (GCA).  Although it is not possible to study each individual type of vasculitis separately with limited resources, our hope is that information learned in studying several types of vasculitis will be beneficial for understanding and treating other types of vasculitis as well.

If you wish to learn about other forms of vasculitis that are not discussed on this website, please visit the Vasculitis Foundation at www.vasculitisfoundation.org.

  
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