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Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) frequently affect the same types of people. The diseases may occur independently or in the same patient, either together or at different times. What follows is an explanation of the two diseases and how they are similar.

People over 50 years old are typically affected with PMR and GCA. The average age of patients is 70. One-third of patients with GCA also have PMR. These diseases are more common among women, and there is a higher incidence among Caucasians than any other ethnic group.

The exact cause of these illnesses is unknown.

Giant Cell Arteritis (GCA)

Giant cell arteritis causes inflammation that damages the arteries (blood vessels that carry blood and nutrients from the heart to tissues in the body). The large and medium-sized arteries are affected. Because some of the affected arteries provide blood to the head (cranium), including the temples, the condition may also be called cranial or temporal arteritis.

Symptoms of GCA:

• Atypical severe headaches are the most common symptom and occur in over 80 percent of patients
• Scalp tenderness, fatigue, fevers and a general sense of illness is common in about 50 percent of patients
• Jaw or facial soreness, especially with chewing, occur in about 50 percent of patients
• Vision changes or distorted vision caused by decreased blood flow occur in 15 to 50 percent of patients; blindness occurs in 5 to 15 percent of patients
• Stroke may occur in less than 5 percent of patients and is caused by decreased blood flow
• The large blood vessels may become narrowed or enlarged (aneurysm). If narrowing occurs in the blood vessels leading to the arms or legs, patients may notice fatigue or aching in the limbs, due to a reduced blood supply. Weak or absent pulses may be noticed by the doctor. These symptoms occur in 15 to 20 percent of patients.
• Other symptoms may include fever, weight loss, night sweats, depression, fatigue and a general feeling of being ill

The diagnosis of GCA is based on the presence of previously noted symptoms and/or the finding of abnormal blood flow in the arms, legs, or aorta; tenderness of the scalp or temples; visual abnormalities; and a high ESR.

Once the diagnosis has been made, treatment should be started as soon as possible.

If the diagnosis is suspected, but less convincing features are present, a temporal artery biopsy may confirm the diagnosis. The biopsy is taken from a part of the artery located in the hairline, in front of the ear. The biopsy is helpful in most cases, but in some individuals it may be negative or normal, even though the disease is present.

Polymyalgia Rheumatica (PMR)

Polymyalgia literally means “many muscle pains.” Rheumatica means “changing” or “in flux.”

Pain or aching is usually felt in the large muscle groups, especially around the shoulders and hips. Other symptoms may include:

• Stiffness, especially in the morning and after resting
• Weakness
• Fatigue
• Generally feeling ill
• Mild fevers (occasionally)
• Weight loss

There are other illnesses that may be confused with PMR. These include:

• Rheumatoid arthritis
• Infections
• Inflammation of blood vessels (vasculitis)
• Metabolic (chemical and hormone) abnormalities
• A variety of muscle diseases
• Cancer and many other diseases

Since there are so many illnesses that mimic PMR, how is it diagnosed? PMR is diagnosed after:

• Careful evaluation of a person’s medical history with an emphasis on the presence of pain, aching and stiffness in the shoulder, pelvic and hip regions
• A complete physical exam – during the exam, the presence of common PMR features and the effects of other illnesses are evaluated
• Excluding the possibility of other illnesses (blood test results may show distinct abnormalities typical of other diseases that would suggest different diagnoses)
• Evaluating the results of blood tests – a high erythrocyte sedimentation rate (ESR) is common among patients with PMR
• Quick recovery and disappearance of symptoms after treatment with low-dose corticosteroids

There is no known cure for PMR and GCA, but these diseases can be treated and controlled.

CORTICOSTEROIDS

Corticosteroid treatment helps rapidly relieve symptoms of both PMR and GCA. Treatment with corticosteroids is mandatory for GCA to prevent serious vascular complications, such as blindness. Low doses of corticosteroids are often successful in treating PMR. Higher doses are often required to control GCA.

The excellent response to treatment is so uniform that the lack of dramatic improvement, within days, would make the diagnosis of GCA or PMR doubtful.

Corticosteroids (or “steroids”) are man-made drugs that closely resemble cortisol, a hormone that your adrenal glands produce naturally. Some corticosteroid medications include cortisone, prednisone and methylprednisolone. Prednisone is the most commonly used steroid to treat certain rheumatic diseases.

Steroids reduce the numbers of inflammatory cells and chemicals that cause these illnesses. Consequently, steroids minimize tissue damage. Steroids also reduce the normal activity of the immune system by affecting the protective functions of white blood cells.

The decision to prescribe steroids is always made on an individual basis. Your doctor will consider your age, presence of other illnesses and medications you are taking. Your doctor will also make sure you understand the potential benefits and risks of steroids before you start taking them.

You will have frequent blood tests while taking steroids to monitor possible side effects and to evaluate the effectiveness of therapy. These blood tests can usually detect problems before you are aware of any symptoms. Your doctor will also frequently evaluate your heart and lung function and blood sugar level, which may be increased secondary to steroids.

While taking steroids, it is important to keep all appointments with your doctor and the laboratory and have your blood pressure checked regularly. Because steroids increase your chance for developing an infection, report symptoms such as a cough, fever or shortness of breath to your doctor.

Long-term steroid treatment (for a few months to several years) requires additional testing and monitoring. The potential side effects caused by long-term steroid therapy should be discussed with your doctor.

With careful monitoring and appropriate treatment, most patients with PMR or GCA have a normal life span and lifestyle. The success of therapy is related to prompt diagnosis, aggressive treatment and careful follow-up to prevent or minimize side effects from the medications.

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Wegener’s granulomatosis is a rare disease of uncertain cause. It is characterized by inflammation in a variety of tissues, including blood vessels (vasculitis). Inflammation damages vital organs of the body.

Wegener’s granulomatosis primarily affects the upper respiratory tract [sinuses, nose, trachea (upper air tube)], lungs and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart and skin.

Wegener’s granulomatosis can affect people of all ages from childhood to adulthood. It affects men and women equally.

The symptoms of Wegener’s granulomatosis and their severity vary among patients. General signs of the disease may include:

• Loss of appetite
• Weight loss
• Fever
• Fatigue
  

Most patients first notice symptoms in the respiratory tract. Symptoms may include:

• Persistent runny nose (also called rhinorrhea) or the formation of nasal crusts and sores
• Nasal or facial pain
• Nose bleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
• Cough which may include bloody phlegm caused by upper airway or lower airway (lung) inflammation
• Chest discomfort
• Middle ear inflammation (also called otitis media), pain or hearing loss
• Voice change, wheezing or shortness of breath caused by inflammation of
  the trachea
  

Other possible symptoms include:

• Eye inflammation
• Joint pain (arthritis) or muscle pain
• Rashes or skin sores
• Kidney inflammation*
  *Although kidney inflammation is common, it is not usually associated with symptoms, such as pain.

Wegener’s granulomatosis has symptoms similar to a number of other disorders, which may make it difficult to diagnose. However, for the most effective and successful treatment, early diagnosis is critical.

It is the combination of symptoms, results of physical examinations, laboratory tests, x-rays and sometimes a biopsy (sample) of affected tissue (skin, nose, sinus, lung or kidney) that together prove the diagnosis of Wegener’s granulomatosis. Following treatment, these factors are also critical in judging whether the disease is active or in remission.

A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not by itself prove the diagnosis of Wegener’s granulomatosis or determine disease activity.

Other tests that influence a physician’s judgment of disease activity include:

• Measures of anemia (red blood cell count)
• Sedimentation rate (the speed in which blood cells settle in a vertical glass tube)
• Urinalysis
• Chest or sinus x-rays
  

Sometimes the lungs may become abnormal even though there are no symptoms, such as cough or shortness of breath. Therefore, it is important to periodically have lung x-rays if you are diagnosed with Wegener’s granulomatosis – even if you don’t have any symptoms of lung disease.

Because Wegener’s granulomatosis is often a life-threatening disease, it is treated with a variety of powerful medications that have been shown to be lifesaving.

Treatment usually includes corticosteroid medications such as prednisone and chemotherapy drugs such as cyclophosphamide or methotrexate.

These drugs suppress the immune system and usually induce remission (the complete absence of all signs of the disease). Improvement usually occurs within days to weeks. When the disease is in remission, patients will reduce the dosage of these medications, but will continue treatment until the disease has been in continuous remission for one year.

The treatment used for Wegener’s granulomatosis has also been successfully applied to other vasculitic diseases.

Because the treatment drugs suppress the immune system, there is an increased risk of developing serious infections. Prednisone can also cause weight gain, cataracts, brittle bones, high blood pressure, diabetes and changes in mood and personality. Cyclophosphamide can cause sterility, bladder irritation and bleeding, and even cancer of the bladder. Methotrexate can cause liver irritation. Cyclophosphamide and methotrexate can each cause changes in blood counts and sometimes lung inflammation.

Because the medications used to treat Wegener’s granulomatosis can have serious side effects, patients are monitored closely by their doctor. The dosage of medication is adjusted as needed throughout the course of treatment.

After treatment and improvement occurs, relapses of the disease may occur in half of all patients. Flare-ups may follow reductions of the doses of prednisone, methotrexate or cyclophosphamide. Flare-ups can usually be controlled by increasing these medications.

It is obvious from the description of Wegener’s granulomatosis and its treatment that this is a very serious disease. It is also clear that treatment carries significant risks. However, treatment is lifesaving for almost everyone when the diagnosis is timely and proper medications are begun.

Prior to recognizing effective therapy in the 1970s, half of all patients with this illness died within five months of diagnosis. Today, over 75% of treated patients are alive at least 8 years later. Many of these patients have not experienced relapses and have been able to lead relatively normal lives.

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Churg Strauss vasculitis (CSV) is an extremely rare disease that results from inflammation causing injury to many organ systems. CSV may damage small and medium-sized blood vessels. CSV is also know as Churg Strauss syndrome, allergic angiitis and granulomatosis.

CSV is distinguished from other types of vasculitis because CSV patients:

• have asthma or have had asthma in the past
• have an increased number of 'allergic type' blood cells called eosinophils

Because many different organ systems may be involved, an enormous number of symptoms are possible.

Patients who have CSV may feel generally ill, have fevers, shortness of breath, cough, wheezing, increased nasal discharge, facial pain from sinusitis, shortness of breath from lung or heart disease, rashes, abdominal pain, blood in the stools, or muscle and joint pain (arthritis). CSV in the nerves may cause an abnormal sensation, followed by loss of sensation or loss of strength. Any combination of these symptoms may be present.

Although allergies have been thought to play a role in the development of CSV in some patients, the exact cause of CSV is unknown.

CSV can affect people of all ages, from children to the elderly. The average age of CSV patients at diagnosis is about 35 - 45 years old.

The diagnosis of CSV is based on a combination of factors, including:

• Complete medical history and physical exam to exclude other illnesses that may have similar symptoms
• Presence of typical CSV symptoms, especially the presence of asthma, the most constant feature of CSV
• Blood tests that indicate abnormal blood counts with an increase in eosinophils
• X-rays which show tissue damage or inflammation in areas such as the lungs or sinuses
• Urinalysis which may indicate excessive protein or abnormally high number of red blood cells

Once the diagnosis of CSV is suspected, a biopsy (tissue sample) of the affected area can confirm that inflammation of blood vessels is present. This is most easily done if there is a suspicious rash. If easily accessible areas, such as the skin, are not affected, the diagnosis may be supported by abnormal findings on a kidney or lung biopsy. These organs would only be recommended for biopsy if there were abnormal findings during an examination or on X-rays. If the results of blood or urine tests imply kidney involvement, a biopsy may be useful to show how the kidney was damaged.

CSV is generally a progressive, serious and sometimes life-threatening disorder. It always requires treatment with immunosuppressive drugs that suppress abnormal cells of the immune system. This is intended to minimize or prevent damage to normal tissues.

Corticosteroids are the most common medications used to treat CSV. The most frequently used drugs in this category are prednisone or prednisolone. Patients whose nervous system, heart, kidneys or intestinal tract are affected might do extremely well with prednisone therapy alone.

Patients who have critical organ system involvement are also generally treated with corticosteroids and 'chemotherapy' medications such as cyclophosphamide (Cytoxan), methotrexate (Rheumatrex) or azathioprine (Imuran).

These medications are considered to be chemotherapy because they were first used to treat cancer.

In cancer treatment, chemotherapy refers to the use of any of a group of drugs whose main effect is either to kill or slow the reproduction of rapidly multiplying cells.

In rheumatology, chemotherapy is designed to decrease the abnormal behavior of cells, rather than kill cells. The doses of medication used for rheumatic or autoimmune conditions, such as CSV, are sometimes 10 to 100 times lower than the doses used for cancer treatment. The lower doses do not produce the same side effects as cancer therapy. However, the continued use of these medicines should be closely monitored to prevent harmful side effects. The fact that someone with CSV may initially tolerate treatment with chemotherapy-type medications does not guarantee that over a long time the patient will sustain the same degree of tolerance. Sometimes the patient’s blood counts will become lower within weeks, months or years of continued treatment. The goal of therapy is to stop all damage that is continuing to occur as a result of vasculitis. Once it is apparent that the disease is under control, doctors slowly reduce the dose of prednisone and eventually hope to discontinue it entirely. If the patient is also taking a form “chemotherapy,” over several months to one or two years, that medicine is slowly reduced and also discontinued.

Because CSV is a rare disease, accurate statistics on the effects of the disease on function and mortality are only approximate. On average, after five years of illness, 80 percent of patients have survived the effects of CSV. When treatment has been promptly initiated and carefully monitored by a physician knowledgeable about CSV, the likelihood of successful treatment and minimal organ damage is best. Patients who have had severe kidney, heart, intestinal tract or brain involvement have not done as well as patients with milder forms of CSV that may have only affected the skin, joints, lungs, nose, sinuses and ears. However, when treated promptly, even patients with the most severe forms of CSV can expect to achieve remission.

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Takayasu’s arteritis (TA) is an uncommon form of vasculitis. Inflammation damages large and medium-sized blood vessels. The vessels most commonly affected are the branches of the aorta (the main blood vessel that leaves the heart), including the blood vessels that supply blood to the arms and travel through the neck to provide blood to the brain. The aorta itself is also often affected.

Less commonly, arteries that provide blood flow to the heart, intestines, kidneys and legs may be involved.

Inflammation of large blood vessels may cause segments of the vessels to weaken and stretch, resulting in an aneurysm. Vessels can also become narrowed or even completely blocked (called an occlusion).

Approximately half of all patients with TA will have a sense of generalized illness. This may include fevers, swollen glands, anemia, muscle aches or arthritis.

Narrowed vessels cause decreased blood flow to the areas that are supplied “down stream” from the narrowed area. The changes that occur in TA are often gradual, allowing alternate (or collateral) routes of blood flow to develop. These alternate routes are often smaller “side roads.” The collateral vessels may or may not be adequate to carry as much blood as was present normally.

In general, the blood flow that occurs beyond an area of narrowing is almost always adequate to allow tissues to survive. In rare cases, if collateral blood vessels are not available in sufficient quantity, the tissue that is no longer supplied by blood and oxygen will die. This is called an “infarction.”

Narrowing of blood vessels to the arms or legs may cause fatigue, pain or aching due to reduced blood supply – especially during activities such as shampooing the hair, exercising or walking. It is much less common for decreased blood flow to cause a stroke or a heart attack (myocardial infarction). In some patients, decreased blood flow to the intestines may lead to abdominal pain, especially after meals.

Decreased blood flow to the kidneys may cause high blood pressure, but rarely causes kidney failure.

Some patients with TA may not have any symptoms. Their diagnosis may be stumbled upon by a doctor who has difficulty measuring blood pressure in one or both arms. Similarly, a doctor may notice that the strength of pulses in the wrists, neck or groin may not be equal, or the pulse on one side may be absent.

The exact cause of TA is unknown.

TA often affects young Oriental women, but it can affect children, women and men of all ages and ethnic backgrounds. At diagnosis, TA patients are often between 15-35 years old.

Every year in the United States, two to three new cases of TA per million Americans are diagnosed.

The diagnosis of TA is based on a combination of factors, including:

• Complete medical history and careful physical exam to exclude other illnesses that may have similar symptoms
• X-rays, which show location and severity of vessel damage
• Procedures to detect blood vessel narrowing or aneurysm, including:

~Magnetic resonance imaging (MRI): test that produces images of the human body without the use of X-rays. MRI uses a large magnet, electromagnetic energy waves and a computer to produce these images.

~ Computed axial tomography (CAT scan): X-rays and computers are used to produce images of internal organs, including large blood vessels.

~ Angiography: X-ray pictures of the inside of blood vessels. During angiography, a long slender tube called a catheter is inserted into a large artery (generally, in the groin area or arm). The catheter is slowly and carefully threaded through the artery until its tip reaches the segment of vessel to be examined. A small amount of contrast material is injected into the blood vessel through the catheter, and X-rays are taken. The contrast agent enables the blood vessels to appear on the X-ray pictures.

• Significant narrowing of blood vessels may result in turbulent blood flow through the narrowed area that creates an unusual sound called a bruit.

With most other forms of vasculitis, a biopsy (tissue sample) of the affected area confirms the presence of blood vessel inflammation. A biopsy is most appropriate when easily accessible areas, such as the skin, are affected. However, when large blood vessels are affected, a biopsy is often not practical because of the risks of surgery.

Corticosteroids are the most common treatment for TA. The most frequently used drug in this category is prednisone or prednisolone. Corticosteroids work within hours after the first dose is provided. While this medication is often dramatically effective, it may be only partially effective for some patients.

The goal of therapy is to stop all damage due to vasculitis. Once it is apparent that the disease is under control, doctors slowly reduce the dose of prednisone to sustain improvement, thereby trying to minimize treatment side effects. In some patients, it is possible to gradually discontinue medication without a relapse.

As the dose of prednisone is gradually reduced, about half of all patients will have recurrent symptoms or progression of illness. This has led to exploring additional therapies to produce remission. Among medications that have been tried, with varying degrees of success, are “chemotherapy” medications such as cyclophosphamide (Cytoxan) and methotrexate (Rheumatrex).

When these medications are added to prednisone to treat TA, 50 percent of patients who had previously relapsed will achieve remission and be able to gradually discontinue prednisone . Overall, about 25 percent of patients will have disease that is not entirely controlled without continued use of these therapies. This emphasizes the need for continuing research to identify better and less toxic treatments for TA and other forms of vasculitis.

Many patients with TA have high blood pressure (hypertension). Careful control of blood pressure is very important. Inadequate treatment of high blood pressure may result in stroke, heart disease or kidney failure.

In some instances, narrowing of arteries to the kidney may be the cause of hypertension. Whenever possible, it is desirable to stretch narrow vessel openings with a balloon (angioplasty) or to do a bypass operation to restore normal flow to the kidney. This may result in normal blood pressure, without the need to use antihypertensive medications.

Some patients may have serious disabilities because of narrowed blood vessels to other sites such as the arms or legs. Bypass operations may correct these abnormalities. Aneurysms can also be surgically repaired or corrected.

In the United States and Japan, only about 3 percent of patients with TA die after having the disease for an average period of 5 years. This encouraging statistic is the product of recognizing the disease and treating it appropriately. Reports from certain other parts of the world have been less optimistic. This may be the result of delayed recognition and treatment.

For patients who live long lives, in spite of having Takayasu’s disease, there are significant problems that must be recognized. Having a chronic illness requires periodic evaluation and adjustment of medications whenever necessary. The medications for TA have side effects, and these must be monitored by a physician, as well as by blood tests. The effects of illness on function may be significant.

In our experience, 25% of patients have an entirely normal lifestyle. Another 25% have had to make some adjustments in their activities. About half of our patients had to modify their jobs and a small number of that group were occupationally disabled.

TA is clearly a treatable disease, and most patients improve. However, it is apparent that many patients have to deal with consequences of this illness that may be partially, or less often, completely disabling. These effects can be minimized by a team of physicians that includes specialists in vascular and immunologic diseases (rheumatology, immunology, radiology, vascular medicine, vascular and cardiac surgery). For best results, a team leader should coordinate diagnostic tests and the different forms of treatment that TA patients may require.

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Necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arteriols, capillaries or venules.

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Central Nervous System Vasculitis (Primary Angiitis of the Central Nervous System, Granulomatous Angiitis of the Central Nervous System, Isolated Angiitis of the Central Nervous System)

Vasculitis (meaning inflammation of the blood vessels) confined to the brain, the spinal cord and its covering is referred to as central nervous system vasculitis. Central nervous system (CNS) vasculitis is a rare disorder. Central nervous system vasculitis can be classified as primary (primary angiitis of the central nervous system or PACNS) when there is no other disease or condition present that may cause blood vessels to be damaged. Secondary vasculitis of the central nervous system is more common and can be one part of a variety of systemic illnesses including generalized autoimmune diseases such as systemic lupus erythematosus (SLE), Sjögren’s syndrome, and a variety of systemic vasculitides such as Wegener’s granulomatosis, polyarteritis nodosa and others. Secondary CNS vasculitis can also be caused by a reaction to drugs such as amphetamines or cocaine and even some over-the-counter cold preparations.

The signs and symptoms of CNS vasculitis are similar to those encountered in having a stroke because they result from a reduction or sudden stoppage of blood flow to the brain. In general, while the disease may present abruptly, it more commonly causes a waxing and waning illness where there are multiple neurological signs and symptoms which evolve over many weeks or months. There may be transient ischemic attacks or brief periods of visual loss, inability to use an arm or a leg or speech impairment. Severe headache that is unresponsive to conventional therapy is also a very common symptom. On occasion involvement of the spinal cord may also cause weakness of the arms and legs. Other symptoms that could suggest CNS vasculitis are far less specific and include profound loss of memory and concentration (i.e. dementia), an altered level of consciousness and problems with bowel or bladder function. The symptoms of CNS vasculitis may be extremely difficult to separate from more common forms of neurological disease such as multiple stokes from hardening of the arteries (atherosclerosis), emboli (dislodged blood clots that travel to the brain from the heart or large blood vessels in the neck), infections or even multiple sclerosis.

Perhaps no other form of vasculitis is as difficult to diagnose as CNS vasculitis. There are no simple tests that can secure the diagnosis. Tests that may provide clues, but not a certain diagnosis include analysis of cerebral spinal fluid (a spinal tap), CT scans or even MRI. All of these tests can be useful, but they are unable to separate CNS vasculitis from other forms of neurological disease.

Cerebral arteriography, which is performed by injecting dye directly into arteries that supply blood to the brain, can be helpful in detecting vascular disease within the central nervous system. Unfortunately, results from this technique are nonspecific. At times atherosclerosis (i.e. hardening of the arteries) within the brain circulation and spasm of the cerebral vessels due to a multitude of factors can mimic the changes seen in CNS vasculitis.

Biopsy of the brain is the most direct means of making the diagnosis of CNS vasculitis. Obviously, such a procedure should never be taken lightly, but it may be vitally important to secure the diagnosis, since the treatment for this condition is long-term and may have significant risks. This biopsy is done under general anesthesia in the operating room where a small piece of brain is taken and studied under the microscope and evaluated for possible infection. The procedure can generally be done with minimal adverse effects and the information provided can at times be lifesaving Many conditions can mimic vasculitis. These mimics have completely different treatments e.g. antibiotics for infections or surgery or radiation therapy or chemotherapy for brain tumors. Thus it is essential to rule out such diagnoses before embarking upon therapy for CNS vasculitis. Not surprisingly, patients are often reluctant to have a brain biopsy unless the reasons are adequately explained.

The diagnosis and care of patients with CNS vasculitis is a team effort. This team needs to include a physician such as a rheumatologist or immunologist who is expert in vasculitic diseases of the nervous system. It should also include a neurologist who is expert in other forms of CNS vascular disease. An experienced neurosurgeon capable of performing a biopsy precisely suited to the patient’s needs is vital, as is a pathologist, skilled in interpreting such biopsies. Finally, a highly experienced neuroradiology department capable of providing the best possible images of brain abnormalities is essential.

Once the diagnosis of CNS vasculitis is secured, to best determine appropriate treatment, the clinician must attempt to classify this disease into one of several subcategories. These include primary granulomatous angiitis (GACNS) of the CNS and benign angiopathy of the CNS (BACNS), which has more recently been referred to as reversible vasoconstrictive (vessel spasm) disease.

Patients with true GACNS, proven by biopsy, invariably require aggressive therapy with drugs such as high doses of glucocorticoids (a cortisone-like drug such as Prednisone) and a second drug known as cyclophosphamide. These drugs are generally administered for six to twelve months and require meticulous follow-up to assess benefit and avoid side effects.

Possible side effects of high doses of glucocorticoids include abnormalities of blood sugar, blood pressure, weight gain, thinning of the bones, and increased risk of infections.

Cyclosphosphamide also has formidable toxicities including lowering of the white blood cells making individuals more prone to infections, bleeding from the bladder and even bladder cancer. Patients who take cyclophosphamide for more prolonged periods of time have a higher incidence of certain forms of cancer later in life.

Such a powerful combination of drugs is used because without them this disease is nearly uniformly fatal. With successful therapy, patients may have partial or complete recovery. Unfortunately, the brain is an organ that has a very limited potential to regenerate itself. Thus, if a patient has experienced a massive stroke, he or she will continue to suffer the deficit of that stroke even if the vasculitis is successfully treated. Many months may need to pass before one can tell what degree of recovery will ultimately occur.

This form of immunosuppressive therapy should be provided by a clinician skilled in its use. Regular blood counts and urinalysis are essential. These can be monitored by a local doctor and a consultant, who may be local or even a great distance away. It is generally advised that patients receive antibiotic prophylaxis to prevent certain forms of infectious disease such as pneumocystic carinii pneumonia, especially during the first few months of therapy.

A second form of PACNS has been referred to as benign angiopathy of the CNS (BACNS). This is different than GACNS. It is a condition that occurs mostly in young women who experience the sudden onset of a headache or a stroke and have been found to have a highly abnormal CNS angiogram (blood vessel picture). Only a skilled, experienced clinician can differentiate this form of vasculitis from others. This is a distinction that is vitally important. This point is emphasized because the outcome for BACNS is more favorable than GACNS, and patients with this condition do not generally require prolonged courses of glucocorticoids or cyclophosphamide. This disease is often treated with a short course of glucocorticoids as well as other drugs such as calcium channel blockers that relax “tight” blood vessels.

Here are some medical review articles that have been written in the last few years on CNS vasculitis. These are found in medical libraries of hospitals, medical centers and medical schools.
1. Calabrese LH, Duna GF, Lee JT: Vasculitis in the central nervous system. Arthritis & Rheumatism 40:1189-1201,1997.
2. Younger D, Calabrese L: Granulomatous angiitis of the central nervous system. Neurologic Clinics of North America 15:821-834,1997
3. Calabrese L: Therapy of systemic vasculitis Neurologic Clinics of North America 15:973-992.

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Vasculitis with cryoglobulin immune deposits affecting small vessels, i.e. capillaries, venules or arterioles, and associated with cryoglobulins in serum. Skin and glomeruli are often involved.

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Vasculitis with IgA-dominant immune deposits affecting small vessels, i.e. capillaries, venules, or arterioles. Typically involves skin, gut and glomeruli, and is associated with arthralgias or arthritis.