Medical Director, SVCMC Physician Assistant Program

Medical Director, SVCMC Physician Assistant Program
Vasculities, Polymyalgia Rheumatica, Giant Cell Arteritis (Temporal Arteritis) Victor Politi, M.D. FACP Medical Director, SVCMC Physician Assistant Program

Vasculitis “Vasculitis” is a general term for a group of diseases that involve inflammation in blood vessels. Blood vessels of all sizes may be affected, from the largest vessel in the body (the aorta) to the smallest blood vessels in the skin (capillaries). The size of blood vessel affected varies according to the specific type of vasculitis.

Overview There are many different types of blood vessels in the body.
Any particular vessel is part of a large vascular "tree" that includes large and medium sized arteries and smaller and smallest arterial branches (arterioles).

Overview These branches eventually reach all the tissues of the body, delivering oxygen and nutrients to a network of tiny vessels, called capillaries, that also remove wastes.

Overview The capillaries drain into the venous system.
The smallest veins are venules; these connect like the tributaries of a river to form larger and larger veins.

Overview Vasculitis can affect any of these different types of blood vessels. Inflammation can affect the lining of the vessels (endothelium) or the wall of an artery or vein. The damaged vessel does not function normally, and tissues that the vessel normally serves may then be affected.

Overview The effects of vasculitis that result from damage to the blood vessel include decreased function due to decreased blood flow (ischemia), death of some or all of an organ due to absent blood flow (infarction), or bleeding into the skin or other part of the body due to rupture of the blood vessel wall.

Overview Because vasculitis is a process that involves inflammation, it is usually accompanied by other features such as fever, or symptoms of involuntary weight loss and fatigue.

Overview Fortunately, available treatments for vasculitis are helpful, especially in the acute phase. Long term therapy presents challenges because of side effects of the medications typically used to treat the disorder.

WHAT CAUSES VASCULITIS?
In most cases, the cause is unknown. It is likely that a combination of factors causes the inflammatory process to be set in motion.

Vasculitis can occur in conjunction with another illness, such as lupus erythematosus or rheumatoid arthritis.

Sometimes, it is precipitated by a reaction to a drug or other substance. (This is known as hypersensitivity vasculitis.)

In still other cases, it occurs in conjunction with a viral illness, such as hepatitis B or C, HIV, cytomegalovirus, Epstein-Barr virus, and Parvo B19 virus.

WHAT ARE THE SYMPTOMS? Symptoms vary from patient to patient, and are dependent in part on the type of vasculitis.

Some common symptoms include:
Fatigue Weakness Fever Joint pains Abdominal pain Kidney problems (bloody urine, dark urine) Nerve problems (numbness, weakness, pain)

Begin with a careful history and physical exam
The diagnosis of vasculitis is often difficult, as the patient's symptoms may suggest many other illnesses. Begin with a careful history and physical exam You may be able to detect signs of organ problems suggestive of a vasculitic process.

Laboratory tests can help pinpoint the areas of the body affected.
Tests may include those that examine muscle, liver, or kidney function.

Other common tests that may provide useful information are additional blood tests, urinalysis, chest x-ray, and electrocardiogram. Tests of lung function may be needed in some cases.

Patients with evidence of nerve or muscle involvement may undergo nerve conduction studies and an electromyogram (a test of muscle function).

Tissue biopsy is a critical component of the diagnostic process.
The tissue sample is taken from an area thought to be involved in the vasculitis. In vasculitis affecting the larger vessels, an arteriogram may be useful. This test involves injecting dye into the arteries, which makes them visible on x-ray.

The diagnostic tests that are used vary widely depending on the type of vasculitis that is suspected.

TYPES OF VASCULITIS There are many different types of vasculitis.
They are classified according to the type and location of the blood vessels that are generally involved.

Large vessel vasculitis
Large vessel vasculitis — The types of vasculitis that affect large arteries include Takayasu arteritis and giant cell (temporal) arteritis.

Large vessel vasculitis
Involvement of the main artery of the body, the aorta, can sometimes occur in association with other illnesses such as ankylosing spondylitis, rheumatoid arthritis, and relapsing polychondritis.

Large vessel vasculitis Takayasu arteritis
Takayasu arteritis primarily affects the main artery that receives blood from the heart (aorta) and its branches. The inflammation may be localized to a portion of the aorta in the chest or abdomen and branches.

Only 1-3 cases are diagnosed in a year among 1 million people in North America. The disease most often affects women during the years from age 10 to about 40. There is a 9:1 female predominance in this disease. Although the disease has a worldwide distribution, it appears to occur more often in Asian women.

The involvement of large arteries may lead to symptoms such as pain and weakness with use of the arms or legs (claudication). Other organs such as the intestines (abdominal pain after eating), heart (chest pain with exertion), or brain.

On the right is an example of an abnormal aortic arch in a patient with Takayasu's, with obvious dilation of the ascending aorta on the left side of the picture

The narrowing and irregularities that occur at several sites, and the “corkscrew” configuration of one vessel segment near the junction of the two arteries. These changes, caused by inflammation in the blood vessel wall, sometimes cause complete blockage of the artery

Large vessel vasculitis Giant cell arteritis
Giant cell arteritis may also affect the aorta and its branches. Frequent involvement of the arteries of the face and scalp, particularly those near the temples, accounts for the other common name for this disorder, temporal arteritis.

Giant cell arteritis is a disease that nearly always affects people older than 50 years of age. Among a million people 50 or older, approximately 2000 may be affected at any one time.

In addition to general symptoms associated with inflammation, headache, tiring of jaw muscles during chewing, and visual changes or loss of vision are suggestive of this disease.

The diagnosis is suspected based upon symptoms, the finding of an elevated level of a blood test (erythrocyte sedimentation rate or C-reactive protein), and a confirmatory biopsy of an artery (usually one or both temporal arteries).

Medium sized vessel vasculitis
Some types of vasculitis appear to spare the aorta and affect medium sized arteries instead. Polyarteritis is the term used for this disorder when it occurs in the absence of any other disease.

But similar involvement of blood vessels can occur when vasculitis develops in some people with rheumatoid arthritis, systemic lupus erythematosus, scleroderma (systemic sclerosis), hairy cell leukemia, and infectious forms of hepatitis (hepatitis B and C).

Medium sized vessel vasculitis Polyarteritis nodosa (PAN)
Polyarteritis nodosa is a term that refers to inflammation of medium to small arteries. In the skin the inflammation results in thickened nodular (nodose) vessels that can be felt or sometimes seen.

Medium sized vessel vasculitis Polyarteritis nodosa (PAN)
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.

Medium sized vessel vasculitis Polyarteritis nodosa
General symptoms due to inflammation, and specific symptoms due to bleeding, decreased blood flow (ischemia), or irreversible damage to organs due to the absence of blood flow (infarction), suggest the presence of polyarteritis.

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.

Damage to the nerves of the arms or legs, to the kidneys, the intestines, and the heart may occur. The diagnosis is suspected when several organs of the body are being damaged at the same time.

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 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.

Testing for the presence of antineutrophil cytoplasmic antibodies (ANCA) in the blood is helpful because these are frequently present in patients with polyarteritis or polyangiitis.

Arteriography or biopsy of an involved blood vessel is often necessary to confirm the diagnosis.

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.

Other diseases that can affect the medium sized arteries include Kawasaki disease and isolated central nervous system vasculitis

Small vessel vasculitis
Several different types of vasculitis can affect small vessels such as arterioles, capillaries, and small veins (venules). These disorders may appear very similar based upon biopsy results, but are distinguished from one another by other features.

Small vessel vasculitis Churg-Strauss Vasculitis
Churg-Strauss vasculitis occurs almost exclusively in people who have asthma. It is likely to cause lung damage. ANCA testing is valuable. Biopsy is useful to confirm the diagnosis.

Small vessel vasculitis Wegener's granulomatosis
Wegener's granulomatosis characteristically affects the nose and sinuses, the lungs, and the kidneys. Almost all those with Wegener's granulomatosis have a positive ANCA blood test. Biopsy of the lining of the nose, a sinus, part of a lung, or kidney may confirm the diagnosis.

Small vessel vasculitis Henoch-Schönlein purpura
Henoch-Schönlein purpura most often affects children but can occasionally cause disease in adults. Hallmarks of this illness are abdominal and joint pain, a skin rash consisting of small, red to purple, slightly raised areas, and kidney involvement that causes the urine to appear bloody or darkly colored, like tea or coffee.

Small vessel vasculitis Henoch-Schönlein purpura
The diagnosis of Henoch-Schönlein purpura is suggested by the symptoms and characteristic skin rash. Skin or kidney biopsy can confirm the diagnosis, especially if there are increased amounts of a specific class of antibody proteins (immunoglobulin A or IgA) in affected blood vessels or within the kidney.

Small vessel vasculitis- Cryoglobulinemia
Cryoglobulins are complexes of the body's infection fighting proteins (antibodies, immunoglobulins) with the proteins that are their targets (antigens). When the serum of the blood of patients with cryoglobulinemia is cooled, the complexes become so large that they form visible clumps (precipitates, cryoglobulins).

Among people with cryoglobulinemic vasculitis, many have chronic infections. The most common is caused by the hepatitis C virus.

Two features of this type of vasculitis are the appearance of crops of raised red bumps on the legs and inflammation of the kidneys (glomerulonephritis).

A blood test for cryoglobulins and a characteristic appearance of a skin or kidney biopsy specimen confirms the diagnosis.

the hand from the same patient at different times
the hand from the same patient at different times. The image on the left is normal and the one on the right shows the patient in the midst of a flare of cryoglobuinemic vasculitis.

Small Vessel Vasculitis- Hypersensitivity vasculitis
Inflammation of small blood vessels, that cannot be classified as any of the previous disorders, and which occurs after someone has been exposed to a medication that could cause an allergic (hypersensitivity) reaction may lead to a diagnosis of hypersensitivity vasculitis.

Small vessel vasculitis
Small vessel vasculitis may also be seen in some patients with rheumatoid arthritis, systemic lupus erythematosus, inflammatory muscle diseases (polymyositis and dermatomyositis), Sjögren's syndrome.

HOW IS VASCULITIS TREATED?
In hypersensitivity vasculitis, removal of the offending substance is often enough. Some patients may need a short course of steroid therapy. Others benefit from nonsteroidal antiinflammatory drugs such as ibuprofen.

The exact treatment of the other types of vasculitis will be dependent on the specific type of vasculitis and the areas/organs that are involved.

Some measures that may be necessary include: Use of steroids, such as prednisone. Steroids may be taken orally in some cases or high doses may be needed and given intravenously.

For more serious types of vasculitis, or when steroids cannot be tapered because of recurrent vasculitis, other "cytotoxic" medications are used. These medicines suppress the immune system and interfere with the function of cells that participate in the vasculitic process.

The use of one such drug, cyclophosphamide, has dramatically improved the outlook for patients with some types of vasculitis.

Behcet’s disease is most common along the “Old Silk Route”, which spans the region from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran.

Behcet’s disease Although the disease is rare in the United States, sporadic cases do occur in patients who would not appear to be at risk because of their ethnic backgrounds (e.g., in Caucasians or African–Americans).

Behcet’s disease In Japan, Behcet’s disease ranks as a leading cause of blindness.

Behcet’s disease Behcet’s disease is virtually unparalleled among the vasculitides in its ability to involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones, and involving veins as well as arteries.

Behcet’s disease Because of the diversity of blood vessels it affects, manifestations of Behcet’s may occur at many sites throughout the body.

Behcet’s disease Behcet’s is one of the few forms of vasculitis in which there is a known genetic predisposition. The presence of the gene HLA–B51 is a risk factor for this disease- many people possess the gene, but relatively few develop Behcet’s.

Behcet’s disease Diagnosis is based on the occurrence of symptoms and signs that are compatible with the disease, the presence of certain features that are particularly characteristic (e.g., oral or genital ulcerations), elimination of other possible causes of the patient’s presentation, and — whenever possible — proof of vasculitis by biopsy of an involved organ.

Buerger’s Disease characteristic pathologic findings — acute inflammation and thrombosis (clotting) of arteries and veins — affecting the hands and feet. Another name for Buerger’s Disease is thromboangiitis obliterans.

Buerger’s Disease The classic Buerger’s Disease patient is a young male (e.g., 20–40 years old) who is a heavy cigarette smoker. More recently, however, a higher percentage of women and people over the age of 50 have been recognized to have this disease.

Buerger’s Disease Buerger’s disease is most common in the Orient, Southeast Asia, India and the Middle East, but appears to be rare among African–Americans.

Buerger’s Disease Initial symptoms often include Claudication
numbness and/or tingling in the limbs Raynaud’s phenomenon Skin ulcerations and gangrene of the digits

Buerger’s Disease The association of Buerger’s Disease with tobacco use, particularly cigarette smoking, cannot be overemphasized. Most patients with Buerger’s are heavy smokers, but some cases occur in patients who smoke “moderately”

Buerger’s Disease Buerger’s disease can be mimicked by a wide variety of other diseases that cause diminished blood flow to the extremities.

Buerger’s Disease Diseases with which Buerger’s Disease may be confused include atherosclerosis,endocarditis, other types of vasculitis, severe Raynaud’s phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma), clotting disorders of the blood, and others.

Buerger’s Disease Angiograms of the upper and lower extremities can be helpful in making the diagnosis of Buerger’s disease.

Buerger’s Disease Certain angiographic findings are diagnostic of Buerger’s. These findings include a “corkscrew” appearance of arteries that result from vascular damage, particularly the arteries in the region of the wrists and ankles. Angiograms may also show occlusions (blockages) or stenoses (narrowings) in multiple areas of both the arms and legs.

Buerger’s Disease On the right, is an abnormal angiogram of an arm demonstrating the classic “corkscrew” appearance of arteries to the hand. The changes are particularly apparent in the blood vessels in the lower right hand portion of the picture (the ulnar artery distribution).

Buerger’s Disease It is essential that patients with Buerger’s disease stop smoking immediately and completely. This is the only treatment known to be effective in Buerger’s disease. Patients who continue to smoke are generally the ones who require amputation of fingers and toes.

Polymyalgia Rheumatica (PMR)
An inflammatory disorder that causes widespread muscle aching and stiffness, especially in the neck, shoulders, thighs and hips.

Overview Although some people develop these symptoms gradually, PMR can literally appear overnight. People with the condition may go to bed feeling fine, only to awaken in pain the next morning.

Overview Just what triggers PMR isn't known, but the cause may be a problem with the immune system, perhaps involving both genetic and environmental factors. Aging also appears to play a role — the disease almost always occurs in people age 50 and older.

Overview PMR usually goes away on its own in a year or two — often as mysteriously as it came. Mild symptoms - NSAIDs Severe pain - corticosteroids (prednisone)

Signs/Symptoms PMR causes moderate to severe aching and stiffness in the muscles in the hips, thighs, shoulders, upper arms and neck. Most patients have pain in at least two of these areas. Initially, pain may be on just one side of the body, but as the disease progresses, symptoms are likely to occur on both sides.

Signs/Symptoms Stiffness is usually worse in the morning or after sitting or lying down for long periods and may last up to an hour.

Signs/Symptoms The aching and stiffness of PMR often occur suddenly, but sometimes may develop gradually. PMR can cause other signs and symptoms including: Fatigue Unintentional weight loss Weakness or a general feeling of being unwell Sometimes, a slight fever

In the United States, approximately 15 percent of people with PMR have a related condition called giant cell arteritis, which causes the arteries in the temples and sometimes in the neck and arms to become swollen and inflamed.

In PMR, the aching is located primarily around the shoulders and hips

Causes PMR is an arthritic syndrome that causes the muscles to feel achy and stiff due to mild inflammation of the joints and surrounding tissues. Most of the inflammation occurs in the hip and shoulder joints, but it may develop elsewhere in the body as well.

Causes In general, the inflammation isn't as severe as that in inflammatory types of arthritis, such as rheumatoid arthritis.

Causes In PMR, inflammation occurs when white blood cells attack the lining of the joints (synovium). Researchers aren't sure what causes this abnormal immune system response, but they suspect that as with many disorders, both genetic and environmental factors are involved.

Causes Some evidence suggests a link between PMR and certain viruses, such as adenovirus, which causes respiratory infections ranging from the common cold to pneumonia; human parvovirus B19, the source of an infection that primarily affects children; and human parainfluenza virus.

Polymyalgia rheumatica Screening and diagnosis
The signs and symptoms of PMR are similar to those of a number of other conditions, including rheumatoid arthritis and polymyositis — a disease that causes muscle inflammation and weakness.

Polymyalgia rheumatica Screening and diagnosis
Take a complete medical history Thorough physical exam Lab Studies CBC, Sed Rate, RF, C-reactive protein

Polymyalgia rheumatica Complications
The most serious complication of PMR is giant cell arteritis. If left untreated, giant cell arteritis may lead to vision loss, a stroke or an aortic aneurysm

The exact relationship between Polymyalgia Rheumatica and Giant Cell Arteritis isn't clear, but about 15 % of people with PMR also develop giant cell arteritis nearly 50% of those with giant cell arteritis have PMR.

PMR itself causes few other serious problems, but the corticosteroid drugs used to treat the disease can cause a number of complications. the risk of developing diabetes or bone fractures is 2-5 times higher than the risk for people not taking steroids.

Polymyalgia rheumatica Treatment
If the diagnosis of PMR is strongly suspected, a trial of low dose corticosteroids is given, usually in the form of mg of prednisone (Deltasone, Orasone, etc.) per day. If PMR is present, these medications will quickly control the pain. The response to corticosteroids can be dramatic – sometimes, patients experience improvement after only one dose – but the symptoms may improve more slowly.

However, if symptoms have not been completely relieved after 2 to 3 weeks of treatment, the diagnosis of PMR must be called into question and other diagnoses should be considered.

Unfortunately, nonsteroidal anti-inflammatory drugs, such as ibuprofen (Advil, Motrin, etc.) and naproxen (Naprosyn), are ineffective in the initial treatment of PMR.

When symptoms have been controlled, the dose of corticosteroid medication is gradually decreased. The goal is to find the lowest dose that keeps the individual patient comfortable. Some people can stop corticosteroids within a year, but most will need a small amount of this medication for 2 to 3 years, sometimes longer, to keep aching and stiffness under control. .

Because the symptoms of PMR are so sensitive to even small changes in corticosteroid dose, it is not unusual for some of the symptoms to return as this medication is decreased. So both the blood tests and the corticosteroid dose must be closely monitored

Points to Remember! Aching and stiffness come on quickly in PMR but there are often no visible signs of swelling and inflammation on examination, making it difficult to diagnose. Symptoms are worst at night and on rising in the morning. Symptoms respond briskly to low doses of corticosteroids, but some symptoms may recur as the dose is lowered.

Temporal Arteritis A systemic panarteritis affecting medium sized and large vessels in patients over the age of 50 This condition is also referred to as Giant Cell Arteritis

Giant Cell Arteritis Referred to as temporal arteritis since that artery is frequently involved Other extracranial branches of the carotid artery are also usually involved Approximately 50% of patients also have polymyalgia rheumatica

Classic Symptoms Headache scalp tenderness visual symptoms
jaw claudication throat pain

Classic Symptoms The temporal artery is usually normal on exam but may be nodular, enlarged, tender, or pulseless Occlusive arteritis of the posterior ciliary branch of the ophthalmic artery results in blindness

Classic Symptoms Ischemic optic neuropathy of giant cell arteritis may produce no funduscopic findings for the first hours after the onset of blindness

Classic Symptoms In patients in whom giant cell arteritis has affected the aorta or its major branches asymmetry of pulses in the arms a murmur of aortic regurgitation bruits head near the clavicle resulting from subclavian artery stenoses

Classic Symptoms In approximately 15% of patients -large vessel involvement, particularly thoracic aortic aneurysms, may occur years after the diagnosis Diff Dx Takayasu’s Arteritis

Non-classic Symptoms 40% of patients present with non-classic symptoms
respiratory tract problem (dry cough) mononeuritis multiplex (painful paralysis of shoulder)

Non-Classic symptoms Fever of unknown origin -
Giant cell arteritis accounts for 15% of all cases of FUO in patients > 65 frequently associated with rigors,sweats usually have normal WBC count (prior to prednisone)

In an older patient with FUO, an elevated ESR, and normal WBC count, giant cell arteritis must be considered even in the absence of well-known symptoms of headache and jaw claudication

Lab Findings In 90% of cases - elevated ESR
Elevated C-reactive protein Mild normochromic, normocytic anemia and thrombocytosis Elevated Alk Phos

Treatment Goal - prevention of blindness - once blindness develops it is usually permanent Therapy with prednisone, 60mg daily for one-two months before tapering Temporal artery biopsy - (positive findings may be present up to two weeks after starting prednisone)

Treatment When only symptoms of polymyalgia rheumatica present - temporal artery biopsy not necessary In adjusting prednisone dosage- ESR useful but not absolute reference Treat the patient not the ESR!

PMR Polymyalgia rheumatica age over 50
Stiffness and pain to shoulder and hip muscles inflammatory polyarthritis/joint effusions to knees and other joints normal CPK and elevated ESR

PMR DX--muscle biopsy shows atrophy without necrosis or inflammation
RX-- nsaids/ steroids Diff Dx include dermatomyositis/polymyositis

Follow-up Thoracic aortic aneurysms occur 17 times more frequently in giant cell arteritis cases - This can happen at any time but typically occurs approximately seven years after the diagnosis

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Medical Director, SVCMC Physician Assistant Program

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