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• What causes vasculitis?
• What is going to happen to me?
• Is vasculitis curable?
• Is vasculitis hereditary?
• Does diet affect vasculitis?
• Will my vasculitis return?
• How should I guard against the occurrence of a disease flare?
• Why do I have to have bloodwork checked frequently?

What causes vasculitis?

The causes of most forms of vasculitis remain unknown. Infections are strongly suspected of playing a role in in forms such as the association of hepatitis B (a virus) and polyarteritis nodosa, and hepatitis C (another virus) and cryoglobulinemic vasculitis. Bacterial infections have been suspected of playing a possible role in granulomatosis with polyangiitis (GPA, formerly known as Wegener’s) which is the reason that some patients with GPA that is limited to the upper respiratory tract are treated only with an antibiotic, Bactrim (trimethoprim/sulfamethoxazole). A general theory that applies to many types of vasculitis is that the disease results from the occurrence of a particular infection in a person whose genes (and other factors) make him/her susceptible to developing vasculitis.

What is going to happen to me?

The course of vasculitis is often difficult to predict. Some types of vasculitis may occur only once and do not return. Other types are prone to recurrences. For all patients with vasculitis, it is essential to be evaluated by physicians who are experienced in the treatment of these diseases. Vasculitis is treatable, and many patients achieve remissions through treatment. It is important to balance the types of medications necessary to control the disease and the risk of side effects that those medicines often bring. A primary aim of several ongoing new studies in vasculitis is to find drugs that help maintain remission.

Is vasculitis curable?

Most forms of vasculitis are treatable if detected early enough, before substantial organ damage has occurred. While often effective, however, the treatments remain imperfect and require improvement. Further research is needed in all forms of vasculitis. Greater knowledge of these diseases will lead to better treatments and, some day, to cures.

Will my children or other family members get it?

Vasculitis is not contagious. One cannot acquire vasculitis from contact with a vasculitis patient. In addition, despite the fact that genes probably play a role in susceptibility to some forms of vasculitis, it is unusual for vasculitis to occur in more than one member of the same family. Thus, vasculitis is not a heritable disorder. All of these points illustrate the fact that the causes of vasculitis are complex. In all likelihood, patients develop vasculitis because of the simultaneous occurrence of multiple risk factors, most of which remain poorly understood.

Does diet affect vasculitis?

This is one of the most commonly-asked questions by patients with vasculitis. All patients want to do whatever is within their power to help treat their disease. Unfortunately, there is presently no evidence that a person’s diet affects susceptibility to vasculitis, or that consuming or avoiding certain foods or beverages affects the course of the disease. In general, we advocate eating a balanced healthy diet rich in protein and vegetables. Avoidance of excessive empty calories, processed foods, and sugars may be very important, particularly in patients on steroids who are at risk for weight gain.

Will my vasculitis return?

After patients achieve remission from their vasculitis, it is logical for them to wonder if their disease will ever return. The answer, which is often difficult to give with certainty, depends in large part on the patient’s specific type of vasculitis. For example, some types of vasculitis, such as Henoch-Schönlein purpura (HSP) or vasculitis caused by a medication, are often self-limited and resolve on their own. Other forms of vasculitis (e.g., Buerger’s disease, a disease strongly associated with cigarette smoking) resolve with institution of the definitive treatment: smoking cessation.

However,  other forms of vasculitis behave less predictably and never come back in some patients but recur frequently in others. Granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA), Takayasu arteritis, microscopic polyangiitis, and many other types of vasculitis fall into the category of diseases that have periods of quiescence and periods of flare. Disease flares in vasculitis can be mild (rash, minor joint pains) or severe (renal failure, skin ulcers). Flares may occur if medications are discontinued or dosage is lowered. Flare may occur in the context of infection. Often the reason for disease flare is unknown.

At the present time, the ability of doctors to predict who will suffer disease flares and who will maintain in long-term remissions (or be cured) needs refinement. Progress in this area will come through research.

How should I guard against the occurrence of a disease flare?

We believe that several points are worth keeping in mind:

First, the symptoms of flares are usually very similar those experienced at the onset of disease. If headaches signaled the beginning of giant cell arteritis, then the recurrence of headaches may indicate a disease flare. If leg ulcers began as painful red lumps on the leg the first time, then the return of painful red lumps may mean that vasculitis is back. Patients must become experts about their own manifestations of vasculitis so that they can recognize them immediately, consult their doctors, and begin appropriate treatment before serious damage occurs.

Second, we believe that patients truly know and understand their own bodies. It is important to discuss new or changing symptoms with your physicians. Together, patients and physicians can determine if new symptoms truly represent a vasculitis flare or if the cause is something equally as likely (medication side effect, infection, or other common medical issues).

Finally, because vasculitis treatments require careful monitoring by doctors, patients should discuss any changes in treatment with their physicians. Increasing or decreasing medications without consulting a physician may lead to trouble.

Why do I have to have bloodwork checked frequently?

Blood tests are helpful to monitor for the return of vasculitis by keeping a watchful eye on important parameters such as kidney function, liver tests, and markers of inflammation (ESR and CRP). Blood tests are also very important to ensure that medications are not causing any side effects such as liver irritation or low blood counts.

How often should my blood be checked?

This depends on the specific medicine or medicines that you take. Patients on cyclophosphamide (Cytoxan) should have their counts checked every week. Patients on most other kinds of medications used to treat vasculitis (Methotrexate, Azathioprine) usually only need to have their blood work checked monthly. If some laboratory tests are abnormal or nearly so, then more frequent monitoring may be required.

What type of tests do we check?

Regardless of the type of vasculitis and the exact type of medication that a patient takes, similar types of tests are monitored. These tests are:

1. a complete blood count;
2. tests of kidney function including a urinalysis; and
3. liver function tests.

The table below outlines the importance behind checking each of these tests.

Type of Test	What should be checked	Why?
Complete Blood Count (“CBC”)	White blood cells (WBC) Platelets Hematocrit	Low WBC count may lead to infections. Low platelets may cause bleeding. Low hematocrit means insufficient oxygen-carrying capacity of the blood.
Kidney Function	Creatinine Blood Urea Nitrogen (BUN)	High creatinine and BUN indicate that the kidneys are not performing their blood-cleansing function properly.
Urinalysis	Protein Level Red Blood Cells	Normal urinalyses have no protein and no blood. The presence of protein and/or blood in the urine may indicate active vasculitis in the kidneys (or damage to the bladder from cyclophosphamide).
Liver Function	Albumin Aspartate aminotransferase(AST) Alanine aminotransferase (ALT)	Often a good indication of overall health. Elevated AST/ALT levels indicate inflammation in the liver (usually caused by medications).

Vasculitis Center Doctors

Collaborators

Vasculitis can involve virtually any organ system within the body. Hence, our Vasculitis Center maintains close collaborative relationships with experts from other specialties. The Vasculitis Center includes collaborators from several medical disciplines who help provide the highest level of care for our patients. They have extensive experience managing vasculitis within their subspecialty and work closely with the Physicians in the Vasculitis Center to provide comprehensive care for our patients:

Otolaryngology (ENT):

Our ENT team includes specialists in inflammatory sinus disease, sensorineural hearing loss, and chronic middle ear disease. We are pleased to also have a Doctor of Audiology, Dr. Dinkes, who specializes in inflammatory process on our team as well.

• Dr. Jean Kim (sinus disease, middle ear manifestations)
• Dr. Alexander Hiller (upper airway disease)
• Dr. Roni Dinkes (audiology)

Neuro-ophthalmology:

• Dr. Andrew Carey

Endocrinology / Osteoporosis:

• Dr. Han Na Kim

• First Description
• Who gets Polyarteritis Nodosa (the “typical” patients)?
• Classic symptoms of Polyarteritis Nodosa
• What causes Polyarteritis Nodosa?
• How is Polyarteritis Nodosa diagnosed?
• Treatment and Course of Polyarteritis Nodosa
• In medical terms, by David Hellmann, M.D.

First Description

The first description of this disease dates back to 1866 when Kussmaul and Maier identified a condition that consisted of “focal, inflammatory, arterial nodules”. They termed this disorder “periarteritis nodosa” because of the inflammation they observed around the blood vessel wall. The name was changed to polyarteritis nodosa (PAN) to underscore the fact that inflammation throughout the entire arterial wall – not just around the wall – is a major disease feature. Polyarteritis nodosa is sometimes termed “systemic necrotizing vasculitis”, but this term is non-specific as other forms of vasculitis also have systemic and necrotizing features.

Who gets Polyarteritis Nodosa (the “typical” patient)?

Most cases of PAN occur in the 4th or 5th decade, although it can occur at any age. Men are twice as likely to be affected than women. A minority of patients with PAN have an active hepatitis B infection. In the rest of the cases, the cause(s) is presently unknown, and the disease is said to be “idiopathic” in nature.

Classic symptoms and signs of Polyarteritis Nodosa

PAN is a multisystem disease that may present with fever, sweats, weight loss, and severe muscle and joint aches/pains. PAN may develop in a subacute fashion, over several weeks or months. Patients may have nonspecific complaints such as fever, malaise, weight loss, anorexia, and abdominal pain. The disease can affect nearly any site in the body, but it has a predisposition for organs such as the skin, kidney, nerves, and gastrointestinal tract. Many patients with PAN have high blood pressure and elevated erythrocyte sedimentation rates (ESR). The presentation of PAN may also include skin abnormalities (rash, ulcers) and peripheral neuropathy (pain, the sensations of burning, tingling, or numbness, or weakness in a hand or foot). However, the disease has a predilection for certain organs and tissues; these are described below.

• Nerve
• Skin
• Kidney
• Gastrointestinal tract
• Heart
• Eye
• Genitals

Nerve

• Peripheral neuropathies are very common (50 to 70%). This includes tingling, numbness and/or pain in the hands, arms, feet, and legs.
• Central nervous system (CNS) lesions may occur 2 to 3 years after the onset of PAN and may lead to cognitive dysfunction, decreased alertness, seizures and neurologic deficits.

Skin

• Skin abnormalities are very common in PAN and may include purpura, livedo reticularis, ulcers, nodules or gangrene.
• Skin involvement occurs most often on the legs and is very painful.

Kidney

• Renal artery vasculitis may lead to protein in the urine, impaired kidney function, and hypertension.
• Small percentage of patients go on to require dialysis.

Gastrointestinal Tract

• Abdominal pain, gastrointestinal bleeding (occasionally is mistaken for inflammatory bowel disease)
• Hemorrhage, bowel infarction, and perforation are rare, but very serious

Heart

• Clinical involvement of the heart does not usually cause symptoms.
• However, some patients develop myocardial infarctions (heart attacks) or congestive heart failure.

Eye

• Scleritis or inflammation in the sclera (white part of the eye)

Genitals

• Testicular infarction

What causes Polyarteritis Nodosa?

Hepatitis B causes a minority of cases of PAN. With the availability of hepatitis B vaccine now, cases of PAN caused by hepatitis B are now rare in the developed world. It is possible that other infections contribute to other cases of PAN, but links between other infections and this disease remain conjectural at the present time.

How is Polyarteritis Nodosa Diagnosed?

Routine laboratory tests may provide important clues to PAN, but there is no single blood test that is diagnostic of this disease. Most patients with PAN have elevated ESRs. Proteinuria (protein in the urine) is common among those with kidney involvement.

If there is skin or muscle/nerve involvement, a skin or muscle/nerve biopsy can be extremely helpful in coming to a definite diagnosis of PAN. Nerve conduction studies are a non-invasive way of identifying nerves that are involved by the inflammation. (These nerves can then be biopsied to confirm the diagnosis). The diagnosis is confirmed by a biopsy showing pathologic changes in medium-sized arteries. The biopsy site may vary. Most biopsies are taken from skin, symptomatic nerve, or muscle. An angiogram of the abdominal blood vessels may also be very helpful in diagnosing PAN. Aneurysms most often affect the arteries leading to the kidneys, liver or gastrointestinal tract.

The American College of Rheumatology (ACR) has established criteria that should be fulfilled if a patient is to be included in a research study of PAN. The criteria are designed to differentiate PAN from other forms of vasculitis. Not all patients have all criterion. Some, in fact, may have only 2 or 3 criteria, yet their physicians are still comfortable classifying their disease as PAN. A committee of ACR physicians selected 10 disease features (criteria) as being those that best distinguish PAN from other vasculitides. In order to be classified as a PAN patient – for the purpose of research studies – a patient should have at least 3 of the 10 ACR criteria.

The American College of Rheumatology 1990 criteria for the classification of Polyarteritis Nodosa

1. Weight loss of > 4 kg since beginning of illness
2. Livedo reticularis
3. Testicular pain or tenderness
4. Myalgias, weakness, or leg tenderness
5. Mononeuropathy or polyneuropathy
6. Development of hypertension
7. Elevated BUN or creatinine unrelated to dehydration or obstruction
8. Presence of hepatitis B surface antigen or antibody in serum
9. Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
10. Biopsy of small or medium-sized artery containing granulocytes

Treatment and Course of Polyarteritis Nodosa

Treatment of PAN has improved dramatically in the past couple of decades. Before the availability of effective therapy, untreated PAN was usually fatal within weeks to months. Most deaths occurred as a result of kidney failure, heart or gastrointestinal complications. However, effective treatment is now available for PAN. After diagnosis, patients are treated with high doses of corticosteroids. Other immunosuppressive drugs are also added for patients who are especially ill. In most cases of PAN now, if diagnosed early enough the disease can be controlled, and often cured.

In medical terms, by David Hellmann, M.D.

A discussion of Polyarteritis Nodosa written in medical terms by David Hellmann, M.D. (F.A.C.P.), for the Rheumatology Section of the Medical Knowledge Self–Assessment Program published and copyrighted by the American College of Physicians (Edition 11, 1998). The American College of Physicians has given us permission to make this information available to patients contacting our Website.

Polyarteritis nodosa is a small– and medium–sized arteritis affecting multiple organs, especially the skin, peripheral nerve, gut, kidney, and heart. The age of onset ranges from childhood to late adulthood but averages 40 years. Polyarteritis nodosa has been associated with active hepatitis B, hepatitis C, or both; therefore, the disease is more common in injection drug users.

Polyarteritis nodosa is probably mediated by deposition of immune complexes. Evidence includes the observation that patients with polyarteritis nodosa associated with hepatitis B or hepatitis C have immune complexes consisting of immunoglobulin and viral antigens circulating in the blood and deposited in inflamed vessels. Moreover, antiviral therapy can remit the vasculitis in some of these patients.

The onset is gradual over weeks to months, and the initial symptoms are often nonspecific. The earliest clues that the patient has vasculitis come usually from the skin (where vasculitis may appear as palpable purpura, livedo reticularis, digital gangrene, or tender nodules), or the peripheral nervous system (where infarction of one mixed motor and sensory nerve after another results in mononeuritis multiplex, one of the most specific clues that a patient has vasculitis). Renal involvement eventually develops in most and is accompanied by hypertension in half of patients, whereas Granulomatosis with Polyangiitis
rarely elevates the blood pressure. Polyarteritis nodosa also commonly involves the gut (abdominal angina, hemorrhage, perforation), heart (myocarditis, myocardial infarction), or eye (scleritis). Rupture of renal or mesenteric micoaneurysms can simulate an acute abdomen.

Confirming the diagnosis requires either biopsy specimen showing small– or medium–sized arteries, or mesenteric arteriography showing microaneurysms or alternating areas of stenosis and dilation. Biopsy of a symptomatic nerve or a symptomatic muscle is 65% sensitive, whereas biopsy of an asymptomatic site is less than 30% sensitive. Because mesenteric angiography is 60% sensitive, it should be done when there is not a symptomatic site to biopsy. Renal biopsy should be avoided unless angiography rules out microaneurysms susceptible to rupture.

Without treatment, almost all affected patients die within 2 to 5 years. Treatment with prednisone (starting at 1 mg/kg daily) and cyclophosphamide (2 mg/kg daily) appeared to revolutionize the outcome of polyarteritis nodosa by achieving 70% 10–year survivals and established this combination of agents as the standard therapy. However, newer studies suggest that prednisone alone may achieve the same high survival as prednisone and cyclophosphamide, although flares were less frequent in patients taking cyclophosphamide. Other studies indicate that the traditional therapy with prednisone and cyclophosphamide should be abandoned in patients with polyarteritis nodosa associated with hepatitis B. Patients treated with the traditional combination respond, but almost all survivors become chronic carriers of hepatitis B and may die later of cirrhosis or variceal bleeding. The newly propsed regimen consists of 2 weeks of prednisone to control the vasculitis, followed by plasmapheresis to remove immune complexes, and accompanied by antiviral therapy with lamivudine to rid the patient of the hepatitis B infection. The long–term value of anti–viral therapy for polyarteritis nodosa associated with hepatitis C is not established.

• How do we Diagnose Vasculitis?
• Skin Biopsy
• Kidney Biopsy
• Sural Nerve Biopsy
• Temporal Artery Biopsy
• Lung Biopsy
• Brain Biopsy
• Abdominal Angiogram
• Central Nervous System Angiogram
• Other Useful Tests

How do we diagnose Vasculitis?

Patients with vasculitis learn that making the diagnosis is sometimes quite difficult. Many endure numerous doctors’ visits, tests, and hospitalizations before the pieces of the puzzle are assembled. The diagnosis of vasculitis usually requires a biopsy of an involved organ (skin, kidney, lung, nerve, temporal artery). This allows us to ‘see’ the vasculitis by looking under a microscope to see the inflammatory immune cells in the wall of the blood vessel. Although, making a diagnosis of vasculitis can be quite involved, this is very important for two main reasons:

# ONE:  Vasculitis has many MIMICKERS (other diseases that have similar features but require different treatments). It is important to rule out other causes of vascular inflammation, other than a primary autoimmune condition as the management could be different.

# TWO:  The treatments for vasculitis itself involve substantial risk. No physician should prescribe such treatment without making every effort to secure a firm diagnosis.

Blood tests, X–rays, and other studies may suggest the diagnosis of vasculitis, but often the only way to clinch the diagnosis is to biopsy  involved tissue, examine the tissue under the microscope in consultation with a pathologist (ideally one experienced at examining biopsies in vasculitis), and find the pathologic hallmarks of the disease.

If a patient’s symptoms, physical examination, and diagnostic testing suggest involvement of a particular organ, one of the procedures below may be used to confirm (or exclude) the diagnosis of vasculitis:

1. Skin Biopsy: One of the least invasive ways of making the diagnosis. A minor procedure performed under local anesthesia. The wound is closed with 1–2 stitches that are removed 7–10 days later.

The correct diagnosis of PAN (polyarteritis nodosa) was not confirmed by this biopsy because the biopsy was not deep enough. The biopsy specimen contains only the epidermis and superficial dermis. PAN classically affects medium–sized arteries located in the deep dermis.

In contrast to the biopsy above, the skin biopsy below was deep enough to include the deep dermis as well as some subcutaneous fat.

The white, oval–shaped areas are fat lobules. Just superficial to the subcutaneous fat, within the deep dermis, an inflamed medium–sized vessel is evident.

2. Kidney Biopsy: A kidney biopsy will be performed if there is evidence of kidney involvement by vasculitis (red blood cells or protein in the urine, for example). This procedure is done under local anesthesia while the kidney is visualized by ultrasound. Because of the small but significant risk of bleeding after this procedure, patients are usually monitored in the hospital for 24 hours after the biopsy.

3. Sural Nerve Biopsy: The sural nerve is a sensory nerve over the lateral aspect of the foot. Under local anesthesia in an operating room, a surgeon removes a small piece of the nerve, usually along with a piece of the adjacent muscle (the gastrocnemius). Because the sural nerve does not innervate muscles (remember: it is a sensory nerve, not a motor nerve), the patient does not lose any strength on the side of the foot and lower leg. There maybe, however, some residual numbness on the side of the foot. Patients generally tolerate this numbness well (if the vasculitis has involved the nerve severely enough, some patients already have numbness in that region).

4. Temporal Artery Biopsy: Performed to diagnose Giant Cell Arteritis, also known as Temporal Arteritis, because the temporal artery is often involved. The temporal artery courses up the temples, just in front of the ears. The biopsy, done under local anesthesia, is performed by making a small incision just above the hairline (sometimes shaving a small area of hair is required). The procedure is extremely well–tolerated by patients. Within several weeks, there is usually little or no sign that a biopsy was done. Complications of temporal artery biopsies are extremely rare. Sometimes, to increase the diagnostic yield, both temporal arteries (i.e., the ones on each side of the head) are biopsied.

5. Lung Biopsy : Often the best way to make a diagnosis of vasculitis that involves the lungs, such as granulomatosis with polyangiitis (GPA). A lung biopsy may be performed in one of two ways: 1) open lung biopsy, a sizable surgical procedure; or 2) thoracoscopic lung biopsy, a less invasive but still significant procedure. Even a thoracoscopic biopsy usually requires at least 48 hours in the hospital and the temporary placement of a chest tube to permit the lung to re–expand.

6. Brain Biopsy: Often necessary to confirm the diagnosis of Central Nervous System (CNS) Vasculitis. This is usually performed on the non–dominant side of the patient’s brain (that is, if the patient is right–handed — and therefore “left–brained” — the biopsy is performed on the right side of the brain). Biopsy of the brain’s covering, the meninges, is usually performed at the same time.

7. Angiogram / angiography: Angiography is helpful in the diagnosis of Polyarteritis Nodosa (PAN). Similar to a heart catheterization,  after inserting a catheter into a large artery in the leg and advancing the catheter into the aorta, radiographic dye is injected into blood vessels supplying the gastrointestinal tract. In the proper clinical setting, the detection of aneurysms (small outpouchings of blood vessel walls) is diagnostic of PAN. This gives an accurate picture of the luminal (inside) anatomy of blood vessels.

8. Central nervous system angiogram Frequently part of the “work–up” of CNS vasculitis. The procedure is identical to an abdominal angiogram, except the catheter is advanced all the way up to the large vessels supplying the head and neck (for example, the carotid arteries). On angiography, CNS vasculitis is characterized by “beading” (dilated areas alternating with narrowing of the blood vessels). A strikingly abnormal angiogram may eliminate the need for a brain biopsy.

9. Other Useful Tests: There are many other tests that are helpful in the diagnosis of vasculitis, or in evaluating the activity of the disease:

• Erythrocyte sedimentation rate (ESR)
• C–reactive protein (CRP)
• Urinalysis
• CT Scan
• ANCA tests

Erythrocyte sedimentation rate (ESR):  Also known as the “sed rate”, for short. This is an old but useful test first employed by the ancient Greeks as a test for pregnancy. It is important to note that there are several influences on the ESR such as anemia and hypergammaglobulinemia which may have nothing to do with an inflammatory state.

C–reactive protein (CRP): CRP is a protein produced by the liver in response to inflammation within the body.

Urinalysis: Many forms of vasculitis affect the kidneys. A simple way of determining whether or not the kidneys are involved is to perform a urinalysis. By performing checks for several indicators of inflammation in a patient’s urine, the physician may determine if inflammation is present within the kidneys. These indicators include:

• Protein (“proteinuria”)
• Red blood cells (“hematuria”)
• Clumps of red blood cells (“casts”)

Pictured below is a urine specimen from a patient with Wegener’s granulomatosis and glomerulonephritis (inflammation in the kidneys).

From another Wegener’s granulomatosis patient’s urinalysis, “blebs” (identified by white arrows) protrude from the surface of the red blood cells that have been damaged in transit through the kidney.

CT Scan (a CAT scan, or computed tomography) — A type of radiology test that permits a non-invasive, cross–sectional view of a patient’s anatomy. On the illustration below (a chest CT scan from a patient with GPA), the view is up (looking toward the patient’s head, from his or her feet). The heart is the white, rounded object in the upper center of the picture. The black regions are the patient’s lungs. The large spot in the left lung (corresponding to the patient’s right lung) is a nodule caused by GPA. Other smaller nodules are also evident.

 

MRI / MRA: MRI is another imaging modality that can be useful for diagnosing and following systemic vasculitis; particularly large vessel vasculitis. MRI allows for visualization of the vessel wall. In vasculitis, the vessel wall may be thickened or edematous.

ANCA tests — ANCA is an abbreviation (acronym) for anti–neutrophil cytoplasmic antibodies. These antibodies are found in the blood of patients with several different types of vasculitis, including Wegener’s Granulomatosis, Microscopic Polyangiitis, and the Churg–Strauss Syndrome. ANCAs and their association with vasculitis were recognized in the mid–1980s, and their use has become increasingly widespread since the 1990s. ANCAs are detected by a simple blood test. These antibodies are directed against the cytoplasm (the non–nucleus part) of white blood cells. Their precise role in the disease process remains uncertain but is a topic of considerable research interest. ANCAs come in two primary forms: 1) the C–ANCA [C stands for cytoplasmic] and, 2) the P–ANCA [P stands for perinuclear]. C–ANCAs have a particularly strong connection to Wegener’s Granulomatosis (up to 80% of patients – and possibly more of those with active disease – have these antibodies). When C–ANCAs are present in the blood of a patient with symptoms or signs suggesting Wegener’s, the likelihood of the diagnosis increases considerably. Because of the long list of other conditions that are sometimes associated with ANCAs, however, in most cases it is still VERY IMPORTANT to biopsy an organ involved by vasculitis to verify the diagnosis.

 

All information contained within the Johns Hopkins Vasculitis Center website is intended for educational purposes only. Visitors are encouraged to consult other sources and confirm the information contained within this site. Consumers should never disregard medical advice or delay in seeking it because of something they may have read on this website.