Disease Definitions

Aortitis

What is aortitis?

Aortitis is a rare form of vasculitis characterized by inflammation that predominantly affects the aorta. This type of vasculitis has been recognized as a separate entity relatively recently, and its definition continues to be the subject of debate. Generally, cases of aortitis include patients with aortitis with or without vasculitis of other large vessels who cannot be classified as having another type of large vessel vasculitis (such as giant cell arteritis or Takayasu’s arteritis) or another systemic condition known to be associated with aortitis.

Who gets aortitis?

Aortitis tends to affect individuals who are older than 60, and affects women more often than men.

What causes aortitis?

Aortitis is believed to be an autoimmune disease. A trigger for aortitis has not been found so far.

How is aortitis diagnosed?

Aortitis is diagnosed in one of 2 ways: 1) when an imaging test (such as a CT, MRI, or PET scan) shows evidence of inflammation of the aorta or 2) when examination of aortic tissue obtained following removal of an aortic aneurysm or another type of cardiac or vascular surgery shows inflammation in the aorta. Aortitis is then diagnosed when detailed history and investigations fail to reveal evidence of another systemic condition, such as giant cell arteritis, Takayasu’s arteritis, or another condition that is known to be associated with aortitis.

What is the treatment for aortitis?

Optimal treatment of aortitis is not known. Indeed, it is not known at this time whether aortitis needs to be treated, and if so what the treatment should be and how long it should be continued. Surgical removal of a segment of aorta may be sufficient to treat localized aortitis found on pathologic examination from aortic surgery when no additional areas of inflammation are present. In cases where medical treatment is deemed necessary, glucocorticoids (prednisone) are primarily used and immunosuppressive medications may be added to suppress the inflammation in the aorta.

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Behçet’s disease

What is Behçet’s Disease?

Behçet’s disease (or Behçet’s syndrome) is a chronic inflammatory disease that affects multiple organ systems, some but not all of which are from vasculitis. Oral ulcers (aphthous ulcers or “canker sores”) are seen in almost all patients, and genital ulcers in many. Eye inflammation (uveitis or iritis) is both common and potentially serious. Arthritis and skin disease (which commonly resembles either acne or a painful inflammatory nodule referred to as erythema nodosum) are common and bothersome symptoms. Less commonly, Behçet’s disease can involve the intestines, the brain, and large arteries (with risk of aneurysm) or veins (with risk of blood clots).

Who gets Behçet’s Disease?

Behçet’s disease is most commonly diagnosed in young adults of both sexes. In most ethnic groups, Behçet’s is a rare disease, but it is much more common in persons of Turkish ancestry in particular, and is also relatively common in other areas of the Middle East, Central Asia, Korea, and Japan.

What causes Behçet’s Disease?

The cause of Behçet’s disease is not known, but genetic background plays an important role. A gene called HLA-B51 has been associated with Behçet’s disease in multiple ethnic groups and has a far greater influence than any other gene. Close relatives of patients with Behçet’s disease are at increased risk of develop the disease themselves (compared to the general population), but the likelihood remains small.

How is Behçet’s Disease diagnosed?

There is no blood test or imaging test to diagnose Behçet’s disease. The disease is diagnosed based on the clinical syndrome, which almost always includes oral ulcers, and some combination of genital ulcers, uveitis, or typical skin lesions. Frequent episodes of oral ulcers are common in the general population and are not sufficient to make the diagnosis of Behçet’s disease. Other diseases can resemble Behçet’s disease, so it is important for physicians to consider and “rule out” other causes.

What is the treatment for Behçet’s Disease?

Because the severity of Behçet’s disease varies widely, the aggressiveness of treatment varies widely as well. Oral and genital ulcers, skin disease, and arthritis do not always require treatment, but colchicine (a non-immune-suppressive drug) is often used, and prednisone is sometimes needed if symptoms are disabling. Mild cases of uveitis can be treated with prescription eyedrops, but severe cases require high doses of prednisone and/or other immune-suppressive drugs. Immune-suppressive drugs such as azathioprine, cyclosporine, anti-TNF drugs (infliximab, etanercept, and others), and cyclophosphamide are used for severe eye disease, brain disease, gastrointestinal disease, and large artery or large vein disease, and for patients with other symptoms severe enough to require high doses of prednisone.

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Central Nervous System (CNS) Vasculitis

What is central nervous system vasculitis?

Central nervous system (CNS) vasculitis refers to vasculitis in the brain. The condition can be primary, with manifestations of vasculitis limited to the brain, or secondary, with brain involvement with a systemic form of vasculitis such as polyarteritis nodosa (PAN), granulomatosis with polyangiitis (Wegener’s), or others. The most typical symptoms of either primary or secondary CNS vasculitis include headache and cognitive dysfunction but may also include any of the following: seizure, stroke and coma.

Who gets central nervous system vasculitis?

Primary CNS vasculitis is extremely rare (less than 1-2 persons per million people per year. Men and women of all ages can get the disease although it is slightly more frequent in middle-aged men (45-65). Five to 15 percent of patients with PAN and granulomatosis with polyangiitis (Wegener’s) will get central nervous system manifestations (secondary vasculitis).

What causes central nervous system vasculitis?

The cause of primary CNS vasculitis is unknown as are the causes of the diseases associated with secondary CNS vasculitis.

How is central nervous system vasculitis diagnosed?

Since primary CNS vasculitis is so rare, a high index of suspicion is required to make the diagnosis. Mimickers must be ruled out, including drug use (ephedrine, cocaine, etc.), migraine, infections and some unusual malignancies. A combination of tests, including magnetic resonance imaging (MRI), cerebral angiography and spinal fluid examination (spinal tap) may help. In many cases, a brain biopsy is the only way to confirm the diagnosis.

What is the treatment for central nervous system vasculitis?

Depending on the clinical manifestations and extent of the disease, patients with CNS vasculitis will usually require treatment with corticosteroids combined with immunosuppressive agents, such as cyclophosphamide or azathioprine, for a period varying between 6 and 12 months.

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Cryoglobulinemic Vasculitis

What is cryoglobulinemic vasculitis?

Cryoglobulins are immune complexes - collections of antibodies bound to other proteins during the course of a response to infection or in some autoimmune diseases – that are particularly large and that fall out of solution from the blood and become solid when stored in the cold in the laboratory. In the body, immune complexes can stick to the cells that line small blood vessels, attracting white blood cells that damage blood vessel walls. The most common organs involved are the skin, nerves, and kidneys, with a wide range of other organs involved much less commonly. Most patients also feel ill, with fatigue and pain in the muscles and joints. Only a small percentage of patients have damage specifically to parts of the body that are exposed to the cold. These patients have “type I” cryoglobulins that can block blood flow without necessarily causing vasculitis, as opposed to the “type II” or “type III” cryoglobulins that typically destroy blood vessels through inflammation.

Who gets cryoglobulinemic vasculitis?

About 70-80% of patients have chronic infection with the hepatitis C virus. Many other patients have either lupus or Sjogren’s syndrome, and some others have lymphoma. In a small percentage of patients, the cause is completely unknown, and the term “essential cryoglobulinemia” is sometimes used.

What causes cryoglobulinemic vasculitis?

Deposition of immune complexes in the walls of small blood vessels causes white blood cells to stick to them and then act as they would if they were fighting a serious infection. “Activation” of white blood cells, particularly neutrophils, within blood vessels is destructive, and the organs supplied by those blood vessels are at risk of not receiving enough oxygen and nutrients. In patients with hepatitis C virus infection, the virus is contained in the immune complex and is clearly the cause. In patients with lupus, Sjogren’s syndrome, or lymphoma, the disease is thought to be autoimmune, meaning the immune system is mistakenly attacking one of its own components. Essential cryoglobulinemia is suspected to be autoimmune also, but chronic infection is also a possibility, and causes may differ among patients.

How cryoglobulinemic vasculitis diagnosed?

A biopsy showing vasculitis plus a blood test for cryoglobulins is the typical way to diagnose cryoglobulinemic vasculitis. Skin is by far the most common site for biopsy, but muscle or nerve are other common sites. Damage to the kidney by cryoglobulins looks different than in other diseases and thus is diagnostic also. In some cases, a biopsy may not be necessary; for example, a patient with purpura (the typical skin damage in vasculitis) and new neuropathy and infection with hepatitis C may not need a biopsy. In addition to the blood test for cryoglobulins, several other blood tests that are abnormal in 80% of patients are often sent, because the cryoglobulin test requires different processing than other blood tests and is often performed improperly.

What is the treatment for cryoglobulinemic vasculitis?

For patients with hepatitis C, eradication of the virus cures vasculitis in most patients. Patients who have severe disease may need immune-suppressive treatment to control vasculitis before or during anti-viral treatment. Rituximab is probably the most effective treatment in such patients, but other medications such as prednisone, cyclophosphamide, and azathioprine have often been used. Sometimes plasma exchange, which directly removes cryoglobulins directly from the blood temporarily, is used in the small number of patients who have life-threatening disease. For patients without hepatitis C, there is no known cure, so immune-suppressive drugs are required in most patients. The effectiveness of different drugs has not been studied as extensively as it has in the setting of hepatitis C.

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Cutaneous Vasculitis

What is cutaneous vasculitis?

Vasculitis means inflammation inside the walls of blood vessels. Cutaneous vasculitis refers to inflammation in blood vessels found within layers of skin. When blood vessels become inflamed, they can become leaky or can close off. This may lead to symptoms such as purple spots on the skin (purpura), lumps (nodules), and breaks in the skin (ulcers).

Who gets cutaneous vasculitis?

Cutaneous vasculitis occurs in both men and women and can affect children and adults. People all over the world can get cutaneous vasculitis.

What causes cutaneous vasculitis?

There are many different causes of cutaneous vasculitis, and sometimes the cause is unknown. Certain medications or toxins can trigger cutaneous vasculitis. Some people experience cutaneous vasculitis as an isolated symptom, or cutaneous vasculitis can occur in the context of a disease that may cause other symptoms throughout the body.

How is cutaneous vasculitis diagnosed?

Cutaneous vasculitis can have a distinct appearance. A doctor with knowledge about cutaneous vasculitis can often examine your skin to determine if an abnormality looks like cutaneous vasculitis. Often, a skin biopsy will be performed to confirm a suspected diagnosis of skin vasculitis. During a skin biopsy, a small piece of tissue is removed from the skin. A pathologist will examine the tissue under a microscope to determine if there is inflammation within the blood vessels contained within the tissue specimen. Cutaneous vasculitis can involve blood vessels that are just underneath the skin as well as deeper vessels in skin. Skin biopsies should be performed by a doctor with experience in diagnosing cutaneous vasculitis to make sure an adequate sample of tissue is collected.

What is the treatment for cutaneous vasculitis?

Triggering factors for cutaneous vasculitis can sometimes be identified, such as some drug or food intake, alcohol ingestion, long periods of standing up, especially in a warm and humid environment. Avoiding these factors can help and limit the risk of recurrence. In other cases, cutaneous vasculitis is a component of a more systemic disease, whose treatment will, most of the time, also be effective for the skin lesions. When there is no obvious cause or in case of recurrent, isolated cutaneous vasculitis, systemic treatments can be considered. Different agents can be tried, including short courses of oral corticosteroids, colchicine, hydroxychloroquine, dapsone, pentoxifylline, indomethacin, azathioprine, methotrexate, mycophenolate mofetil and leflunomide. The efficacy of each of these agents varies between patients and, in the absence of large studies on cutaneous vasculitis, one cannot predict which one may be the best, without trying them successively until finding one that limits the frequency and/or intensity of the flares. All these medications can cause side effects and their use must be balanced with the relative benignity of isolated cutaneous vasculitis. Local treatments with ointments or dermocorticoids have no place, but can help when lesions are itchy.

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Drug-Induced Vasculitis (DIV)

What is drug-induced vasculitis (DIV)?

DIV is a form of vasculitis that affects small blood vessels. The disease can affect many parts of the body but especially involves the skin, causing rash. A skin biopsy may show blood vessel inflammation termed leukocytoclastic vasculitis. Some patients can have inflammation in other organs, including nerve involvement. Systemic manifestations can include fever, weight loss and joint pain.

Who gets DIV?

Drug-induced vasculitis occurs in men and women of all races.

What causes DIV?

A variety of medications have been linked to drug-induced vasculitis. Commonly reported associations are with minocycline, an antibiotic used to treat acne, and propylthiouracil, a drug for hyperthyroidism. A variety of other drugs can cause leukocytoclastic vasculitis.

How is DIV diagnosed?

The diagnosis depends initially on the medical history and physical examination. A skin biopsy is commonly performed, showing leukocytoclastic vasculitis.

What is the treatment for DIV?

Treatment depends on the extent of involvement by vasculitis. For patients with only skin involvement, discontinuation of the causative medication is all that may be needed. Some patients will require corticosteroids to control more bother symptoms. Rarely immunosuppressive agents will be required.

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Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss)

What is eosinophilic granulomatosis with polyangiitis?

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), also known as “allergic granulomatosis”, is a rare form of vasculitis which, like Granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis, affects small and medium-sized vessels. Most patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss) have asthma, nasal and sinus allergies. Patients which EGPA often have unusually high numbers of eosinophils, a type of white 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 EGPA on. Other problems caused by EGPA include lung infiltrates, rashes, peripheral nervous system disease, abdominal pain, kidney, and cardiac disease.

Who gets eosinophilic granulomatosis with polyangiitis?

EGPA affects people of all ages and both sexes.

What causes eosinophilic granulomatosis with polyangiitis?

The cause of EGPA is not known.

How is eosinophilic granulomatosis with polyangiitis diagnosed?

EGPA 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.

What is the treatment for eosinophilic granulomatosis with polyangiitis?

Treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) 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 EGPA are treated in the same way as other patients with asthma. Worsening of asthma in patients with EGPA does not necessarily mean that the EGPA is active in other organs. Unfortunately, while remission of symptoms is usually achieved, the relapse rate remains high.

Is asthma part of vasculitis?

Not exactly. Even though asthma is present in almost all patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and corticosteroids represent the cornerstone treatment for both conditions, asthma it is not part of the vasculitis. In eosinophilic granulomatosis with polyangiitis (Churg-Strauss) asthma is more to be considered as an underlying predisposing condition, and it usually persists even after effective treatment for vasculitis.

What are the chances that my newly diagnosed vasculitis will relapse?

It can be hard to determine relapses in eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA). A return of sinus symptoms and asthma is very common, but these may not represent active vasculitis. These symptoms may require treatment with prednisone and are commonly the rate-limiting features in being able to reduce the prednisone dose in EGPA. Relapses of vasculitis in EGPA are less common and have been reported in ranges from 10-30%.

In Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) do increases in the eosinophil count predict relapse?

We do not have very good data to tell us what the most reliable markers of disease activity or predictors of relapse in eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) are. Eosinophils are one of the subtypes of white blood cells and the level of eosinophils can be measured in the blood. In addition to a careful clinical evaluation, the eosinophil count and other markers of inflammation such as the erythrocyte sedimentation rate (ESR, sed rate) and C-reactive protein (CRP) are usually followed in patients with EGPA. In patients with EGPA who have positive ANCA assays, we also monitor the ANCA level (MPO-ANCA). Some patients with EGPA have elevated IgE levels at the beginning of their disease. In these patients IgE levels can be useful in follow-up to help predict relapse.

Disease activity is absent in most patients with EGPA when eosinophil levels are low. Prednisone suppresses eosinophil counts quickly and effectively. Therefore, eosinophil counts may drop faster than disease activity disappears. On the other hand, when disease activity recurs, this is usually associated with - or preceded by - increases in eosinophil counts. If increasing eosinophil counts are not addressed by changes in therapy, worsening disease activity often follows.

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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.

What is the treatment for giant cell arteritis?

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.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in giant cell arteritis have ranged from 50-90%.

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Granulomatosis with polyangiitis (Wegener’s) (GPA)

What is granulomatosis with polyangiitis (Wegener's) (GPA)?

Granulomatosis with polyangiitis (Wegener's) (GPA) 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 everyone with GPA 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.

What is “granulomatosis with polyangiitis”?

Granulomatosis with polyangiitis (GPA) is a new term introduced to replace the name Wegener’s granulomatosis. As the term is being adopted, the terminology “granulomatosis with polyangiitis (Wegener’s)” has been proposed

Who gets granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (Wegener's) (GPA) 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 GPA.

What causes granulomatosis with polyangiitis?

Granulomatosis with polyangiitis (Wegener's) (GPA) is thought to be an auto-immune disease for which there is no known cause.

How is granulomatosis with polyangiitis diagnosed?

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

What is the treatment for granulomatosis with polyangiitis?

Treatment of GPA 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.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in granulomatosis with polyangiitis (Wegener’s) have ranged from 50-70%.

What is the difference between limited and severe granulomatosis with polyangiitis (Wegener’s)?

Not all patients with granulomatosis with polyangiitis (GPA) have similar disease. The term “limited” GPA was introduced in 1966 and was originally defined as GPA that did not involve the kidney. Unfortunately, this term has often been misinterpreted. “Limited” GPA is not equivalent to non-severe GPA as there are people who meet the definition of “limited” disease who may have very severe disease and people who do not meet the definition of limited GPA whose disease may be quite mild. Because of this, the term “limited” GPA is best avoided. It is far more helpful to view this disease in terms of the location of organ involvement and the degree of severity of the disease in each of these locations.

What is the difference between ANCA and ANA?

Both are blood tests used by doctors to help in the diagnosis of autoimmune disease. Antineutrophil cytoplasmic antibody (ANCA) is blood test commonly elevated in patients with diseases such granulomatosis with polyangiitis, microscopic polyangiitis, and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). Antinuclear antibody (ANA) is a blood test most often elevated in patients with systemic lupus erythematosus (“lupus”), Sjogren syndrome, scleroderma, and other types of autoimmune diseases.

Can you still be diagnosed with granulomatosis with polyangiitis (GPA, Wegener’s) if the ANCA is negative?

Yes. Although ANCA is elevated in a majority of patients with granulomatosis with polyangiitis (Wegener’s) it is not detectable in all patients and is not necessary to make the diagnosis.

In ANCA-associated vasculitis, including granulomatosis with polyangiitis (Wegener’s, GPA), do increases in the ANCA tests predict disease relapse?

A general and over-simplified answer is: no. This answer is based on the statistical analysis of large groups of patients followed prospectively in clinical trials. The results from these studies have led to the general opinion held by vasculitis experts that treatment decisions should not be made based on changes in ANCA levels alone.

There are some patients, however, for whom ANCA levels track the disease activity very well. In these patients, ANCA increases do predict relapses. To know how ANCA levels relate to disease activity in individual patients, their ANCA levels need to be monitored over time.

It should also be noted that there are different ways of measuring ANCA levels. To follow serial ANCA levels they need to be measured by the same method to be able to make comparisons.

What are anti-GBM antibodies and what do they have to do with my vasculitis?

Anti-GBM antibodies are antibodies directed against glomerular basement membranes. Patients with anti-GBM disease (Goodpasture’s syndrome) will present with involvement of lungs and kidneys, so-called pulmonary-renal syndrome. This can include bleeding in the lungs (hemoptysis) that can lead to respiratory failure and bleeding in the kidney (hematuria) that can lead to rapidly progressive loss of kidney function. Goodpasture syndrome has to be considered in patients who are thought to have ANCA-related vasculitidies (granulomatosis with polyangiitis and microscopic polyangiitis) as those diseases can also present with pulmonary-renal syndrome. Measuring anti-GBM antibodies and ANCA in these patients will usually help distinguish the two conditions although there are rare cases of patients with both ANCA and anti-GBM antibodies.

What are the chances of recovering lost hearing in a patient with granulomatosis with polyangiitis (GPA, Wegener’s)?

Hearing can be transiently decreased in patients with GPA with acute otitis, because of the presence of liquid in the inner ear(s), which will regress under appropriate treatment. However, when the inner ear damage is severe, because of prolonged or multiple recurrences of otitis, and/or when the auditory nerve(s) are involved by inflammation or compression, the hearing loss can be permanent.

Can a saddle nose deformity be repaired?

Yes, a saddle nose deformity can be repaired as long as the underlying vasculitis is not active. This would require consultation with a surgeon, often times an ear nose and throat (ENT) specialist who could discuss this option with you.

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IgA vasculitis (IgAV) (Henoch-Schönlein Purpura)

What is IgA vasculitis?

IgA vasculitis (IgAV) also known as Henoch-Schönlein Purpura (HSP), is a form of vasculitis (vessel inflammation) which affects small blood vessels. Often times IgAV starts out as a rash on the legs which is purplish and raised. It may cause joint aches. Serious complications like involvement of the intestines and the kidney can also occur.

Who gets IgA vasculitis?

IgA vasculitis most often affects children but it can occur at any age.

What causes IgA vasculitis?

The exact cause of this disease is unknown but is related to your immune system. People may have a cold (upper respiratory infection) or a gastrointestinal illness just before they develop the disease. However, no virus or other infection has been identified as a cause of the disorder.

How is IgA vasculitis diagnosed?

The diagnosis is based on your symptoms and examination findings. Your doctor will run some blood tests to look for inflammation, evaluate kidney function and rule out other forms of vasculitis. A urine sample will be needed to evaluate for kidney involvement. A biopsy from the skin or kidney may be needed.

What is the treatment for IgA vasculitis?

Often times no treatment may be needed and the disease can resolve on its own. Sometimes if there is kidney or gut involvement, prednisone may be started.

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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 for microscopic polyangiitis?

Like granulomatosis with polyangiitis (Wegener's) (GPA), 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.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in microscopic polyangiitis have ranged from 40-60%.

What is the difference between ANCA and ANA?

Both are blood tests used by doctors to help in the diagnosis of autoimmune disease. Antineutrophil cytoplasmic antibody (ANCA) is blood test commonly elevated in patients with diseases such granulomatosis with polyangiitis, microscopic polyangiitis, and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). Antinuclear antibody (ANA) is a blood test most often elevated in patients with systemic lupus erythematosus (“lupus”), Sjogren syndrome, scleroderma, and other types of autoimmune diseases.

In ANCA-associated vasculitis, including microscopic polyangiitis (MPA), do increases in the ANCA tests predict disease relapse?

A general and over-simplified answer is: no. This answer is based on the statistical analysis of large groups of patients followed prospectively in clinical trials. The results from these studies have led to the general opinion held by vasculitis experts that treatment decisions should not be made based on changes in ANCA levels alone.

There are some patients, however, for whom ANCA levels track the disease activity very well. In these patients, ANCA increases do predict relapses. To know how ANCA levels relate to disease activity in individual patients, their ANCA levels need to be monitored over time.

It should also be noted that there are different ways of measuring ANCA levels. To follow serial ANCA levels they need to be measured by the same method to be able to make comparisons.

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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.

Who gets polyarteritis nodosa?

People between the age of 40 years and 60 years are most often affected. Men may be affected more than women. In most cases, we do not know why people get PAN. Some cases of PAN occur in patients with chronic infection with the hepatitis B virus.

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 nodosa diagnosed?

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

What is the treatment for polyarteritis nodosa?

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.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in polyarteritis nodosa have ranged from 10-30%.

What is the difference between systemic polyarteritis nodosa (PAN) and cutaneous PAN?

Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized blood vessel. In patients with "cutaneous polyarteritis nodosa", the findings of the disease are localized to the skin (purpura or subcutaneous nodules), but also sometimes the peripheral nerve(s) of the same limb (causing weakness and/or numbness). Patients with systemic polyarteritis nodosa have involvement of other organs, including the gastrointestinal tract, heart and/or kidney. The systemic form is therefore usually most severe and requires stronger treatment.

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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”).

What is the treatment for Takayasu’s arteritis?

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.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in Takayasu’s arteritis have ranged from 50-80%.

What is the difference between Takayasu’s and giant cell arteritis?

The key difference between Takayasu’s arteritis (TAK) and giant cell arteritis (GCA) is the age of the patients affected by the disorders. Takayasu’s arteritis affects younger patients, generally less than 40 years of age, while giant cell arteritis affects older patients, generally over 50 years of age.

Both Takayasu’s arteritis and giant cell arteritis affect large arteries, but the typical initial manifestations of the two diseases are also usually different. Patients with giant cell arteritis typically present with headaches affecting the temples, scalp tenderness, jaw pain and fatigue with chewing, shoulder or hip pain and stiffness, and/or sudden changes in vision (temporary or permanent blindness or partial vision loss). Patients with Takayasu’s arteritis usually do not have these symptoms but may have arm or leg pain with use, or chest pain, or be found to have no pulse in an arm or leg. However, many clinical findings may be similar between the two diseases, including the presence of constitutional symptoms (fatigue, fevers, chills, malaise, weight loss), muscle and joint pains, stroke-like symptoms, diminished or absent pulses, asymmetric blood pressures, and narrowed or blocked primary branches of the aorta found on imaging studies.

Giant cell arteritis is usually diagnosed by a temporal artery biopsy, whereas Takayasu’s arteritis is not, since the temporal artery is not classically affected in TAK. Diagnosis of Takayasu’s arteritis is usually made by imaging studies of arteries such as angiography.

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Urticarial vasculitis (UV)

What is urticarial vasculitis?

Urticarial vasculitis is a form of vasculitis that affects the skin, causing wheals or hives and/or red patches due to swelling of the small blood vessels. It has two forms: One with normal levels of proteins called complements; the other with low levels of complements; it’s called hypocomplementemic vasculitis.

Who gets urticarial vasculitis?

Both males and females get the disease.

What causes urticarial vasculitis?

The cause of most cases of urticarial vasculitis is unknown. It may be associated with a number of diseases, especially systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. Some cancers, including leukemias, colon and pancreatic, and infections like Hepatitis B and C can cause this form of vasculitis. So can some drugs, including antibiotics, ACE inhibitors used for treating high blood pressure, and certain diuretics.

How is urticarial vasculitis diagnosed?

Diagnosis is based on characteristic patches in the skin. Sometimes a biopsy is ordered to show inflammation in the skin and damage of small blood vessels with white blood cells. Since it’s often associated with a number of different diseases, it’s often necessary to do other tests and exams to rule out underlying conditions like lupus erythematosus or cancer. Tests of vital organs may also be indicated, especially when the blood levels of complement are low.

What is the treatment for urticarial vasculitis?

Treatment depends on the extent of symptoms and organ involvement. When levels of complement are normal and there is no internal organ involvement or underlying disease, the symptoms may improve on their own or with minimal treatment. In this case, antihistamines or nonsteroidal drugs such as ibuprofen or naproxen may be helpful. For more severe cases, other drugs which affect the immune system may be needed, such as corticosteroids (prednisone, others), hydroxychloroquine, colchicine, dapsone; and chemotherapies like azathioprine or cyclophosphamide. Treatment may be intermittent, although it is not uncommon for patients to need treatment for several year.

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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: eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (CSS), giant cell arteritis (GCA), granulomatosis with polyangiitis (Wegener's) (GPA) (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN), and Takayasu’s arteritis (TAK). 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.

Vasculitis Foundation

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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