Authors: Rita Baron-Faust,Jill Buyon
Because many vasculitis symptoms are nonspecific, diagnosis can be difficult. Often a biopsy of the skin or other affected areas may help in confirming a
diagnosis. For example, a skin biopsy can reveal inflamed blood vessels beneath the second layer of skin (the
dermis
).
Erythrocyte sedimentation rate (ESR)
is one of the blood tests that measure inflammation. As you’ll recall, the ESR measures the speed at which red blood cells settle in a vertical glass tube. When inflammation is present, the cells settle to the bottom faster than in people who don’t have inflammatory conditions. Women with vasculitis may also have a slight anemia, which can be detected with a red blood cell count.
The diagnosis of giant cell arteritis is based on the symptoms noted on
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and a finding of abnormal blood flow in the arms, legs, or aorta. Your physician will look for tenderness of the scalp or temples and visual abnormalities; your SED rate will be high (greater than 50). A temporal artery biopsy involves taking a small tissue sample from a part of the temporal artery located in the hairline in front of the ear. When viewed under a microscope, the tissue from the temporal arteries reveals inflammation and unusually large macrophages, or “giant cells.” A biopsy can be helpful in most cases, but the disease can “skip” areas of the artery, so a biopsy on both sides of the head may be done, says Dr. Mitnick. The use of color Doppler ultrasound to image the temporal arteries has been proposed as a useful diagnostic test, but is not always accurate.
Polymyalgia rheumatica (PMR)
can mimic many illnesses, and it can only be diagnosed after these other illnesses are excluded. During your physical exam, your doctor will look for pain, aching, and stiffness in the shoulder, pelvic, and hip regions. A high ESR is common among patients with PMR. Sometimes a short course of low-dose corticosteroids is given; if symptoms disappear fairly quickly, then it’s likely to be PMR.
C-reactive Protein (CRP)
is also elevated in giant cell arteritis and polymyalgia rheumatica, but there are no established biomarkers for these or other forms of vasculitis.
23
The primary diagnostic test for Takayasu’s arteritis is
magnetic resonance angiography (MRA)
. Thickening of the aortic wall can be seen on MRA. In some cases, damage to the aorta, the aortic valve, or other large blood vessel is found and surgery is needed to repair it. In angiography, a special dye (which appears opaque on x-ray) is injected into an artery and x-rays are taken of key blood vessels. In many cases, the aorta will show long areas of narrowing (
stenosis
). But these narrowed areas can also be found in many or all of the large blood vessels.
GPA is diagnosed by the results of a physical exam, together with symptoms, lab tests, x-rays, and sometimes a biopsy of affected tissue (skin, nose, lung, or kidney). These factors are also used to judge whether the disease is active or in remission. Other tests include a blood test for
antineutrophil cytoplasmic antibodies (ANCAs)
, antibodies to enzymes located in the cytoplasm of white blood cells called
neutrophils
, a red blood cell count to test for anemia, an ESR (or SED rate), and urinalysis to detect protein in the urine (proteinuria), and x-rays of the sinuses or chest may also be done. Tests for ANCA in GCA and PMR can rule out other autoimmune diseases.
Lupus manifestations may represent vasculitis. “To a greater or lesser extent, lupus is a vasculitic illness,” remarks Dr. Mitnick. “There are frequent skin manifestations, but there may also be vasculitis affecting internal organs, such as the kidneys, lungs, or brain. When a patient with lupus nephritis becomes hypertensive, we worry the renal arteries are affected with vasculitis. Likewise, if a lupus patient has a stroke, we wonder about vasculitis in the brain, cerebral vasculitis.”
Vasculitis is treated according to the stage of disease, the level of disease activity, and whether there’s vessel damage. The initial treatment goal of ANCA-associated vasculitis is remission, and then the maintenance of remission, with maintenance treatments continuing for 18 to 24 months.
29
Corticosteroids
, most commonly prednisone, are the usual treatment for the most common types of vasculitis. Corticosteroids relieve symptoms of both giant cell arteritis and polymyalgia rheumatica within days and slow or stop the progression of the disease.
25
While low doses of corticosteroids are effective in treating PMR, higher doses (40 to 60 mg of oral prednisone a day) are often required initially to control temporal arteritis and Takayasu’s arteritis. Unfortunately, the blindness that can occur in rare cases of temporal arteritis may not be reversible with treatment. (This is why it is so important to diagnose the disease before permanent damage takes place.)
Steroid sparing agents
, such as low doses of
methotrexate
,
cyclophosphamide (Cytoxan)
, or
azathioprine (Imuran)
may be used so that lower doses of prednisone can be used. Cyclophosphamide can cause bladder irritation, bleeding, and cancer.
Angioplasty
may also be used to trast Takayasu’s and GCA. In angioplasty, a balloon-tipped catheter is threaded into blood vessels to widen narrowed arteries, such as in the kidneys. In some cases, damage from severe inflammation can require bypass procedures or aortic valve replacement, but the surgery must be done when the disease is inactive. Active GCA may last only a year or two, but continued low-dose treatment may be needed after remission.
Because GPA can be life threatening, it is treated with high doses of prednisone and azathioprine and methotrexate.
30
Extended treatment with corticosteroids may be needed, so eventually the dose of prednisone is slowly decreased. Some women may have a remission, but it’s difficult to predict how long it will last. Methotrexate and azathioprine can be effective in maintaining remission.
31
However, half of all GPA patients may have a relapse, so close monitoring is vital.
In clinical trials, infusions of the B-cell-depleting drug rituximab (Rituxan) every six months were shown to be more effective than azathioprine in maintaining remission in ANCA-associated vasculitis, especially GPA.
32
Rituxan may also be effective in a rare form of ANCA-associated vasculitis, EGPA.
33
Biologic drugs
, including those that block the inflammatory cytokine
tumor necrosis factor alpha (TNFα)
, are also being used to treat different forms of vasculitis.
34
These include
etanercept (Enbrel)
and
infliximab (Remicade)
.
35
However, anti-TNF drugs are apparently not effective in Wegener’s.
Intravenous immune globulin (IVIG)
may have benefit as an adjunctive therapy in ANCA-associated vasculitides, particularly in particularly in relapsing disease, recent studies suggest. One preliminary study from France found that just one course of IVIG in combination with corticosteroids or other immunosuppressants produced remission in over half of patients with GPA, EGPA, and another rare ANCA vasculitis called
microscopic polyangiitis (MPA)
.
36
Vasculitis in women of reproductive age does not affect pregnancy and fertility, but the medications use to treat it can.
As mentioned in previous chapters, prednisone can cause menstrual irregularities but can safely be used during pregnancy and breast-feeding. Because prednisone (and similar drugs) can lead to osteoporosis, women may need bone-building drugs and bone scans to monitor bone mineral density regardless of their age. However, the effects of vasculitis on pregnancy and fertility have not been studied.
Because vasculitis often arises in later life, when the risk of heart disease also increases, researchers are investigating connections between the two conditions. Blood vessel inflammation is known to be a component of cardiovascular disease, and people with vasculitis also have an increased risk of arteriosclerosis, or narrowing of blood vessels by fatty plaques. Blood clots can form at the site of active inflammation, and vasculitis itself can also cause heart attacks and strokes. For now, women with vasculitis would do well to take steps to reduce any of the accepted risk factors for coronary disease, such as obesity, high blood pressure, high cholesterol, and a sedentary lifestyle. And stop smoking!
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