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Vasculitis—Rare, debilitating, and deadly

Phillips, Regina M. PhD, RN, CNE

doi: 10.1097/01.NME.0000420367.24093.fd
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Vasculitis is a rare and potentially deadly inflammation of the blood vessels that causes constriction or occlusion, ultimately limiting blood supply to tissues or organ systems. Although the cause of vasculitis is unknown, early recognition of this disease and prompt treatment can limit its devastating effects. We show you how.

Assistant Professor • Towson University • Towson, Md.

The author and planners have disclosed that they have no financial relationships related to this article.

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Ms. C is a 78-year-old, active, independent woman living in an adult apartment community. She's generally healthy except for some chronic problems, including osteoarthritis, hypertension controlled with diet and medication, and atrial fibrillation for which she takes warfarin to prevent clot formation. About 2 months ago, Ms. C started to feel unusually tired, but decided it was probably because her schedule had included several extended trips and outings with friends. The tiredness increased dramatically in the next few weeks; Ms. C was sleeping more during the day than ever before. In addition, her neck and hips ached more than usual and she had a headache that wouldn't go away.

She wondered if some of the pain was arthritis, but this pain was different. It wasn't the same joint each day; sometimes it was her neck, other times it was in her hip and knees. She took her usual medications for pain with no relief. Ms. C made an appointment with her physician, who sent her to the ED immediately with a diagnosis of vasculitis.

Let's take a closer look at this chronic and potentially deadly disease.

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Inflammation confirmation

Also known as angiitis or arteritis, vasculitis is a group of rare disorders that cause inflammation and necrosis to the walls of the blood vessels (see Selected types of vasculitis). The inflammatory process causes the involved vessels to narrow or become completely occluded, limiting blood flow to the tissues. As a result of the diminished blood flow, end-stage organ damage can result. In some severe cases, the inflammatory process stretches and weakens the vessel, increasing the potential for an aneurysm or a ruptured aneurysm. Inflammation of blood vessels can lead to serious health problems—and even death—depending on the particular vessels, organs, or body systems affected.

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Although the cause of vasculitis is unknown, recent or chronic infections may be involved. Patients infected with hepatitis B or C are at higher risk for developing the disease. Vasculitis is often triggered by an autoimmune response in the body that causes antibodies to attack and damage cells or tissues. Some patients may have an autoimmune disease, such as rheumatoid arthritis or scleroderma, for years before vasculitis develops. Certain cancers, such as leukemia or lymphoma, have also been linked to vasculitis.

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Vasculitis in children and middle age adults

Patients of any age or sex may be affected by this disease, but certain types of vasculitis are more prevalent in one age group than another. For example, children are more often affected by Kawasaki disease and Henoch-Schönlein purpura (HSP).

Kawasaki disease is most often seen in children under age 5, but can occur in older children. The inflammation of the blood vessels in this disease is similar to other forms of vasculitis; however, there's no specific diagnostic test for differential diagnosis. Therefore, the clinical picture is the key to identifying this type of vasculitis. Symptoms include a fever for 5 or more days, bloodshot eyes, a rash on the groin, and swollen hands and feet. The symptoms can lead to more severe coronary artery damage if diagnosis and treatment are delayed.

HSP can occur at any age, but is usually seen in children ages 2 to 11. In HSP, the inflammation of the small blood vessels of the skin causes them to leak, leading to the distinctive look of bruises on the legs. Patients may also experience arthritis and abdominal pain. In some cases, HSP leads to kidney damage, but this usually has no lingering effects. It can, however, reoccur during the patient's lifetime.

Takayasu arteritis is most often found in women under age 40. In this disease, vasculitis affects the aorta and other large vessels of the heart. As with other types of vasculitis, inflammation can block the arteries because of thickening vessel walls or weaken the vessel wall and predispose the patient to an aneurysm. As with many forms of vasculitis, the specific cause of this disease is unknown.

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Signs and symptoms

The symptoms of vasculitis vary depending on the type, the specific organ affected, and the extent of damage. Typically, a patient with any type of vasculitis will complain of general aches and pains, fever, and malaise.

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Figure. Va

Each form of vasculitis causes inflammation in blood vessels of varying sizes. Some forms affect the smaller blood vessels, such as those in the skin. When skin is affected, the patient may experience a variety of rashes, petechiae, purpura, or ecchymosis. Other forms attack larger vessels; for example, a form of arthritis develops from vasculitis that affects the joint cartilage. In this case, the patient experiences swelling, pain, decreased range of motion, and altered joint function.

Vasculitis may also affect the brain and nervous system, causing headaches, confusion, personality changes, seizures, and even coma. Depending on the area of the brain affected, vasculitis can lead to altered vision, hearing, or balance. Patients with vasculitis in the nerves to the arms or legs may experience tingling, paresthesia, or pain caused by diminished blood supply.

Vasculitis in the coronary arteries weakens the heart muscle, causing enlargement and myocardial infarction. In some cases, aneurysms develop from weakened vessel walls. Vasculitis in the gastrointestinal system will cause symptoms such as pain, diarrhea, constipation, or vomiting. If vasculitis affects lung tissue, the patient will experience shortness of breath, wheezing, coughing, and chest pain. In the kidneys, vasculitis leads to hypertension and potential renal failure.

Whatever the specific type of vasculitis, the inflammatory process damages the blood vessels to varying degrees and may affect blood flow to multiple areas or body systems.

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Diagnosis and treatment

Timely treatment for the specific type of vasculitis is imperative. The process starts with a comprehensive history and physical exam. Vasculitis may be suspected based on the patient's signs and symptoms, especially physical signs that indicate particular organ systems are affected. Diagnostic lab tests are also completed (see Common diagnostic tests).

A lower than normal hemoglobin and hematocrit level often indicates anemia, a complication of vasculitis. A higher than normal erythrocyte sedimentation rate (ESR) generally indicates the presence of inflammation. A high C-reactive protein (CRP) level is another indication of inflammation. This lab study is important because it indicates the formation of antibodies that are attacking the cytoplasm of white blood cells.

Blood work will also assess antineutrophil cytoplasmic antibodies (ANCAs), which are present in certain types of vasculitis, such as Wegener granulomatosis and Churg-Strauss syndrome. Wegener granulomatosis is rare and occurs more often in middle-aged individuals. It can affect blood vessels of the nose, ears, and sinuses, as well as the lungs, kidneys, and other organs. Churg-Strauss syndrome, a type of vasculitis affecting the respiratory tract, is also rare; asthma is the most common symptom of this disorder. Although their specific role is unclear, the presence of ANCAs is an important diagnostic tool for vasculitis.

Depending on the suspected type of vasculitis, a biopsy is often performed to confirm the diagnosis, looking for signs of inflammation or tissue damage. Other tests, such as a computed tomography scan, may be performed to help identify the specific type of vasculitis. An angiogram, if used, will help show the blood flow through the circulatory system. Magnetic resonance imaging will provide detailed images of internal organs, looking for signs of damage from diminished blood supply.

Early detection and initiation of treatment is important to limit tissue damage. Vasculitis is most often treated with glucocorticoids such as prednisone or prednisolone. The specific dosage and duration of the treatment will depend on the severity and chronicity of the disease. In severe vasculitis, other immunosuppressive drugs are prescribed because either the glucocorticoids aren't effective enough or the long-term adverse reactions of the glucocorticoids are too severe for the patient's condition or comorbidities.

Cyclophosphamide is one of the strongest of the immunosuppressive drugs used to treat severe vasculitis, especially when vital organs are in danger. Medications that are used for other rheumatic diseases, such as methotrexate or azathioprine, may also be prescribed for nonlife-threatening vasculitis or when steroids aren't optimal for the patient. Medical treatment is usually monitored by a rheumatologist over several months or longer.

In severe cases in which the cause has been isolated to circulatory autoantibodies, plasma exchange (plasmapheresis) and I.V. immunoglobulin have also been used. Surgical intervention, such as a kidney transplant or vascular grafting, may be required when permanent damage from severe vasculitis occurs.

Patients with some types of vasculitis may experience remission of the disease, which indicates that the inflammatory process in the vascular system is no longer detectable. If this occurs, the steroid or immunosuppressive drug dosing will be reduced and some medications replaced. When the disease is in remission, it isn't cured and relapses are possible. Often, the relapse is milder than the original disease process. However, the flare-up may not have the same signs and symptoms as the original disease. The relapse may occur months or years after the original disease process was detected.

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Table C

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Nursing considerations

Nursing care focuses on a careful and accurate assessment of signs and symptoms. These presenting signs and symptoms are often keys to identifying the specific type of vasculitis experienced by the patient. In addition, you must provide ongoing education to the patient and his or her caregivers regarding the types of diagnostic procedures the patient may undergo. Furthermore, you can help the patient and caregivers develop strategies to successfully manage individual symptoms at all stages of this chronic disease.

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Vasculitis in older adults

Older adults are more likely to experience giant cell arteritis (GCA), a form of vasculitis also called temporal or cranial arteritis. Although the disease is relatively rare, this particular form is one of the most common types of vasculitis. GCA almost always affects people over age 50 and is particularly prevalent in individuals over age 65. In fact, the incidence of GCA increases with advancing age.

As with many other types of vasculitis, there's no known cause for GCA, but some association with an impaired immune response or severe infections is apparent. Women are more likely to develop GCA than men, and GCA is more prevalent in White patients, especially those of northern European background.

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GCA affects medium- and large-size blood vessels, particularly the aorta and other vessels leading from the aorta to the arms, legs, and head. Often, GCA affects the blood vessels in the head, particularly the temporal artery, causing the vessels to narrow or completely occlude. Other commonly affected vessels are the ophthalmic, posterior ciliary, and central retinal artery. Inflammation of these vessels can lead to irreversible visual impairment or blindness in one or both eyes—potentially the most devastating complication of GCA.

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Signs and symptoms

Patients with GCA experience a variety of symptoms, such as excessive sweating, fever, weakness, severe tiredness, and general malaise. For some, symptoms include decreased appetite that can lead to weight loss. Many patients with GCA complain of throbbing headaches on one side of their head or the back of their head. Some patients complain of intermittent jaw pain, jaw pain associated with chewing, or pain in the tongue. Patients may experience muscle aches or pain and stiffness in the neck, upper arms, shoulders, and hips. Sometimes there's scalp sensitivity, particularly when the scalp is touched. Depending on the vessels involved, the patient may complain of blurred or double vision, or reduced vision in one or both eyes.

GCA often develops with polymyalgia rheumatica (PMR). About half the patients who have GCA will develop PMR. These conditions are closely related, with slightly different manifestations. PMR is a syndrome most often seen in women and rarely occurs in patients younger than age 50. Symptoms of PMR are systemic and characterized by pain in the shoulders, hips, and back that's usually present in the morning. Headaches are often present along with low-grade fever, malaise, and weight loss.

PMR can develop in the absence of GCA; in other cases, the patient will be diagnosed with PMR and later develop GCA. This sequence occurs in 10% to 20% of the cases.

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Diagnosis and treatment

The diagnosis of GCA is made by considering the patient's history, signs and symptoms, lab tests, and tissue biopsy. The physical exam will include touching the patient's head, which may show scalp sensitivity or a tender, thick artery on one side of the head. The affected artery may have a diminished pulse or no palpable pulse. Lab studies will be completed, including liver function studies. Most patients with GCA have an elevated ESR, elevated immunoglobulins, and changes to CRP; many have anemia.

Blood tests don't provide a definitive diagnosis, so a biopsy of the temporal artery is necessary to confirm the diagnosis of GCA. This procedure is relatively simple, safe, and often performed in a physician's office or outpatient center. The biopsy is the most reliable method of diagnosing GCA.

The goal of treatment is to reduce tissue damage caused by altered blood flow; treatment is often started before the diagnosis is confirmed by biopsy. GCA is treated with glucocorticoids, such as prednisone or prednisolone, given at high doses for several months and then gradually reduced. For some patients, low-dose aspirin is prescribed. The dosage and duration of the medication treatment varies, depending on the severity of the disease. No other drugs, including chemotherapy, have shown effectiveness against GCA.

Early diagnosis and treatment are the keys to limiting the duration and severity of vasculitis. Patients usually begin to feel markedly better just a few days after treatment is started. Patients may need to continue with glucosteroids for 1 to 2 years, and the dosage needs to be reduced gradually before medication is discontinued. PMR, if present, is also treated with glucocorticoids.

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Nursing considerations

The older adult with vasculitis, such as Ms. C, faces a unique set of challenges that other age groups may not experience. Complications from GCA include damage to other blood vessels in the body and the potential development of aneurysms. For the older adult, this is especially an issue because age-related changes may have already weakened the cardiovascular system.

The treatment regimen may pose special challenges for this age group. Glucocorticoids taken for long periods of time can cause osteoporotic changes in a population already at risk. This adverse reaction increases the patient's chance of fracture. Patients should be encouraged to take extra calcium with vitamin D, engage in weight-bearing exercise, and monitor bone density.

Other medication that the patient takes may also be affected by glucocorticoids. For example, Ms. C takes warfarin to control atrial fibrillation. The prednisone she was prescribed for vasculitis may affect the action of warfarin, so closer monitoring of international normalized ratio levels is required.

The older adult with vasculitis may be too tired in the early stages to exercise or walk even a short distance. However, administration of glucocorticoids usually helps the patient feel better in a short period of time. Older patients need to be aware that the adverse reactions of glucocorticoids may contribute to weakness and muscle atrophy, putting them at greater risk for falls.

You can help patients and their families understand the need to carefully assess the environment to prevent falls. For example, the patient may benefit from a cane or walker to help maintain balance. The healthcare provider may recommend a physical therapy referral to help maintain muscle strength and balance. Patients with GCA are often monitored by a rheumatologist in addition to their usual healthcare provider for months or years. Maintaining regular appointments with all providers is imperative. Patients also need to understand that glucocorticoid dosing must be reduced before the medication is discontinued completely.

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Early + accurate = success

Successful treatment of vasculitis depends on early and accurate diagnosis, and prompt initiation of treatment. With some types of vasculitis, the patient experiences a remission; however, the symptoms may return at any time. Some people have several remissions and flares over their lifetime.

Thanks to prompt diagnosis and treatment, Ms. C's symptoms subsided in a few months and the glucocorticoids were gradually discontinued. Remember, however, that vasculitis is a chronic condition. Ongoing healthcare assessment and monitoring is important to address any symptoms that may return. Your support will help Ms. C continue to enjoy healthy aging.

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On the web

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American College of Rheumatology:http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/vasculitis.asp

Johns Hopkins Vasculitis Center:http://www.hopkinsvasculitis.org

National Heart Lung and Blood Institute:http://www.nhlbi.nih.gov/health/health-topics/topics/vas/signs.html

Rare Diseases Clinical Research Network's Vasculitis Clinical Research Consortium:http://rarediseasesnetwork.epi.usf.edu/vcrc/

Vasculitis Foundation:http://www.vasculitisfoundation.org

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Learn more about it

Black JM, Hawkes JH. Medical-Surgical Nursing: Clinical Management for Positive Outcomes. St. Louis, MO: Saunders; 2009.
    Johns Hopkins Vasculitis Center. What is vasculitis? http://hopkinsvasculitis.org.
      Lee CH, Hammel JM. Temporal arteritis in emergency medicine. http://emedicine.medscape.com/article/809492-overview.
        National Heart, Lung and Blood Institute. What is vasculitis? http://www.nhlbi.nih.gov/health/health-topics/topics/vas/.
          National Kidney and Urologic Diseases Information Clearinghouse. Henoch-Schönlein purpura. http://kidney.niddk.nih.gov/kudiseases/pubs/HSP/.
            National Library of Medicine. Temporal arteritis. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001484.
              Rare Diseases Clinical Research Network. Vasculitis clinical research consortium. http://rarediseasesnetwork.epi.usf.edu/vcrc/.
                Vasculitis Foundation. Polymyalgia rheumatica. http://vasculitisfoundation.org/polymyalgiarheumatica.
                  © 2012 Lippincott Williams & Wilkins, Inc.