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A CHRONIC INFLAMMATORY disease, systemic lupus erythematosus (SLE) causes a wide range of signs and symptoms. One characteristic sign lends SLE its name: a malar or "butterfly" rash over the cheeks and the bridge of the nose that may resemble the markings on a wolf's face (lupus is Latin for wolf).
Most patients with SLE can expect to live a normal lifespan with appropriate treatment and lifestyle changes, but exacerbations (flares) can be life-threatening. More than 90% of patients with SLE live at least 10 years after diagnosis, but those with hypertension, thrombocytopenia, renal disease, and severe anemia have a worse prognosis and require aggressive disease management.1
In this article, we'll tell you how to assess a patient for SLE and what to teach her about managing this chronic disorder.
Considered an autoimmune disease, SLE occurs when the body's immune system attacks its own tissues and cells for unknown reasons. In people with SLE, antibodies form against self (autoantibodies) and nonself (antigens). Some autoantibodies combine to form immune complexes (each immune complex consists of one autoantibody and one antigen), and B cells become hyperreactive. Both autoantibodies and immune complexes can damage tissues.2
A combination of factors can trigger an exaggerated immune response, including hormonal changes; some medications, such as quinidine, methyldopa, isoniazid, and phenytoin; stress; immunologic factors; genetic factors; or environmental influences such as exposure to ultraviolet light, chemicals, and toxins.2
Although the risk is very small, close relatives of people with SLE, especially identical twins, are more likely than the general population to develop the disease.2 This suggests that a specific gene or set of genes may predispose a person to develop SLE.
Before puberty, one boy is diagnosed with lupus for about every three girls. From puberty to the age of 50, one teenage boy or man is diagnosed with lupus for about every 10 teenage girls or women of the same age. One man over the age of 50 is diagnosed with lupus for about every eight females who are over 50.1,3 SLE is also found more often in women of African-American, Asian, American Indian, and Hispanic descent than in white women.
Because SLE occurs much more often in women than in men during the years that women menstruate, researchers suspect that female hormones play a role. Recent studies have shown that men with lupus have the same masculine features as men who don't have the disease.1,3
Criteria for an SLE diagnosis were developed by the American College of Rheumatology (ACR).4 Any person with 4 or more of the 11 criteria, at any time in her medical history, is considered to have SLE. See Does your patient have SLE?
Signs and symptoms of SLE range from mild to severe and vary unpredictably among patients. Early signs and symptoms, which are nonspecific, include fever, fatigue, and weight loss. Your patient's signs and symptoms must be correlated with lab analysis and imaging studies. Here's a system-by-system breakdown:
* Dermatologic. Skin problems including photosensitivity rashes, oral ulcers, and alopecia are a common feature of lupus. Besides the characteristic butterfly-shaped facial rash, patients may develop flat or raised rashes or lesions anywhere on the body. A definitive diagnosis requires a biopsy.Although skin problems may not be life-threatening, they can be uncomfortable or painful and decrease the patient's quality of life due to disfigurement and altered body image. Skin conditions may occur alone or accompany a more serious inflammatory response that affects vital organs.
* Musculoskeletal. Most patients with SLE have nonerosive arthritis, including joint tenderness, edema, and effusions that are symmetric, and usually nondeforming.4 The nondeforming arthritis of SLE differentiates it from rheumatoid arthritis, which causes progressive joint destruction. The arthritis of SLE frequently involves the proximal interphalangeal and metacarpophalangeal joints of the hands as well as the wrists and knees.5
* Neurologic. Your patient may be depressed because of cerebral vasculitis or because she's trying to cope with the stress of a chronic illness. In some patients, central nervous system involvement causes strokes, seizures, cognitive impairment, headaches, visual disturbances, or psychosis.
* Gastrointestinal (GI). Your patient may have abdominal discomfort or pain, diarrhea, anorexia, or nausea. Vasculitis of the intestine can cause acute abdominal pain with cramps, vomiting, and diarrhea.5 These problems may result from peritonitis, pancreatitis, mesenteric vasculitis, and bowel infarction secondary to lupus vasculitis.
* Hematologic. Autoantibodies may be produced against cell surface antigens of blood cells, including red blood cells, white blood cells, and platelets. This results in hemolytic anemia, leukopenia, or thrombocytopenia.2
* Renal. About 50% of patients with SLE have renal disease, such as glomerulonephritis, usually from deposition of immune complexes and resultant inflammation and tissue damage.
* Cardiopulmonary. Pericarditis and pleuritis, the most common cardiopulmonary disorders in SLE, occur when immune complexes are deposited in the pericardial and pleural spaces, producing an inflammatory response.
SLE is often called the "great imitator" because signs and symptoms may be vague and nonspecific and mimic other disorders. Listen carefully to your patient and her concerns. When you perform a head to toe assessment, note the presence of any flat or raised skin lesions. Remember that not every patient with SLE develops the butterfly rash. Ask your patient if she's sensitive to light, feels fatigued, or has pain in her joints. Correlate your initial assessment with diagnostic lab and imaging studies to determine the priority of nursing interventions. Review the lab values that might suggest that the inflammatory effects of SLE are targeting vital organs.
Most patients with SLE demonstrate elevated serum levels of antinuclear antibody (ANA). Although the ANA level isn't specific for SLE, a positive ANA is one of the ACR criteria for SLE diagnosis. About 2% of patients with SLE will test negative for ANA but will have antibodies to the nuclear antigen anti-Ro (SSA).1
Autoantibodies may wax and wane during the course of the disease, and levels of autoantibodies don't always correlate with disease severity. You must evaluate the patient's signs and symptoms along with the presence of autoantibodies.
A complete blood cell (CBC) count may reveal anemia or leukopenia. In a patient already diagnosed with SLE, the CBC can also reveal lymphopenia from immunosuppressive therapy. Liver function tests may be mildly elevated in active SLE or in response to immunosuppressive medications or nonsteroidal anti-inflammatory drugs (NSAIDs).
The erythrocyte sedimentation rate and C-reactive protein are tests commonly used to identify the presence of inflammation. The serum C3 and C4, which are complement components, can also help the clinician evaluate inflammation. (See Understanding the complement system.)
A patient with SLE should have renal function studies, including a 24-hour urine collection for protein and creatinine clearance. The results of the 24-hour urine collection should be correlated with the patient's serum creatinine, total protein, and albumin levels.
Chest radiographs may show pleural effusion, pulmonary infiltrates, and cardiomegaly. An echocardiogram may be indicated to evaluate a pericardial effusion or valvular pathology. It may also be needed to confirm a diagnosis of pulmonary hypertension, a complication of SLE.
Now let's look at treatment options for patients with SLE.
Fever and joint pain and edema may be controlled by NSAIDs, most often ibuprofen initially. Fenoprofen, flurbiprofen, mefenamic acid, ketoprofen, indomethacin, and piroxicam may also be used.5 The antimalarial medication hydroxychloroquine (Plaquenil) may help treat fatigue, joint pain, skin rashes, and pleuritis.
Glucocorticoids such as prednisone (Deltasone) may be used for their anti-inflammatory, immunosuppressive, and metabolic effects. These medications modify the body's immune response to stimuli. Large doses may be needed for severe complications affecting the hematologic or central nervous system, kidneys, blood vessels, or membranes.5
Immunosuppressive drugs and cytotoxic agents such as azathioprine (Imuran) are frequently ordered to reduce inflammation in severe corticosteroid-resistant disease that involves the kidneys, central nervous system, and cardiopulmonary system. Only physicians who are specialists and experienced with these drugs should administer them.5 See Teaching your patient about medications for SLE.
Complementary therapies that may be beneficial include relaxation, meditation, and massage.
When developing the plan of care, focus on teaching your patient how to prevent disease flares, manage them when they occur, and limit complications. (See Dealing with flares.)
Carefully assess your patient's skin for lesions, such as rashes, and inspect her mouth. Assess her joints for edema, tenderness, and limited range of motion.
Cognitive disorders are common in patients with SLE.1 Changes in mood and depression may be due to the disease process, the stress of chronic illness, or adverse reactions to medications. Encourage her to try relaxation techniques, which may help alleviate anxiety and depression. Note any changes in her mental status, mood, or behavior, which may indicate an increase in disease activity. Assess her speech pattern, swallowing ability, eye movement, vision, and motor movement because lupus can cause vasculitis of the central nervous system, resulting in a strokelike syndrome.
Auscultate her lungs for adventitious breath sounds, such as a pleural friction rub or crackles, which may indicate pleuritis. Auscultate for heart murmurs from valvular dysfunction or pericardial friction rub from pericarditis.
Carefully assess the patient's abdomen for distension and tenderness. Abdominal pain may be directly related to SLE. Also monitor your patient for signs and symptoms of infection such as fever secondary to immunosuppressive therapy.
Many drugs used to treat SLE have serious adverse reactions. Teach your patient how to reduce the risk of complications associated with these medications. Tell her to take medications as prescribed and promptly report adverse reactions to her healthcare provider rather than stopping the drug or skipping doses. Teach her to continue taking her medications even if she feels better.
Stress the importance of promptly reporting pain, fatigue, or a new rash because these may indicate that a flare is under way. She should also report other signs and symptoms of infection and have regular blood work.
Encourage your patient to ask her healthcare provider what treatments and strategies are available to manage SLE and how blood work can help keep track of her condition. Explain that good hygiene, including oral hygiene, is essential to help prevent infections.
Advise the patient and her family to be involved in self-help groups and to be aware of the most recent research and clinical trials about SLE.
Over the past three decades, improved diagnostic tools and medications for aggressively managing inflammation have remarkably improved the lives of people living with lupus. By staying up-to-date on lupus as new information becomes available, you can help give your patient a fighting chance against this stealthy, often unpredictable disease.
"The complement system provides one of the major effector mechanisms of both humoral and innate immunity. The system consists of a group of proteins (complement proteins C1 through C9) that are normally present in the plasma in an inactive form. Activation of the complement system is a highly regulated process, involving the sequential breakdown of the complement proteins to generate a cascade of cleavage products capable of proteolytic enzyme activity. This allows for tremendous amplification because each enzyme molecule activated by one step can generate multiple activated enzyme molecules at the next step. Complement activation is inhibited by proteins that are present on normal host cells; thus, its actions are limited to microbes and other antigens that lack these inhibitory proteins."
Source: Porth CM. Essentials of Pathophysiology: Concepts of Altered Health States. Philadelphia, PA: Lippincott Williams & Wilkins; 2004.
The malar rash, which can be flat or raised, forms a butterfly shape that covers the bridge of the nose and extends to both cheeks.
Source: Porth CM. Essentials of Pathophysiology: Concepts of Altered Health States. 2nd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2007.
Lupus flares are exacerbations of disease. A flare is diagnosed based on an increase in signs and symptoms that interfere with the patient's quality of life, lab indicators, and imaging studies. Sometimes, however, a flare may be under way when the lab and imaging studies are normal. At other times, your patient may not have an increase in symptoms, but her lab values may indicate increased inflammation.
Flares are common and can be triggered by many factors: too much work or not enough rest, stress, an emotional crisis, an infection, an injury, surgery, pregnancy, the postpartum period, ultraviolet light exposure, or sudden discontinuation of lupus medications.
Even a low-grade fever or a new rash or lesion can signal that a flare is under way. Encourage your patient to pay close attention to any signs or symptoms, however subtle, because these may indicate an increase in disease activity.
During a flare, your patient may need to use analgesics for pain syndromes associated with the joints, skin, or other areas of the body. For mild to moderate pain, an NSAID such as ibuprofen, propoxyphene (Darvon), and tramadol (Ultram) are often effective. If the flare causes more intense pain, your patient may require a more potent opioid analgesic such as hydrocodone, oxycodone, or fentanyl.
Explain to her that the goal is to improve the quality of her life, and these medications will be used for only a short time during the flare. If she's taking an opioid, encourage her to be as active as possible, drink plenty of water, and have sufficient fiber in her diet. To prevent constipation, she should use a stool softener combined with a stimulant laxative. Also encourage her to get plenty of rest.
To prevent future flares, teach your patient to get enough physical and emotional rest, maintain good nutrition, and avoid direct sunlight and other light sources. She should also see her healthcare provider when she has an infection so that it can be treated aggressively. Teach your patient to limit her exposure to fluorescent light and halogen lamps, overhead lights, computer screens, photocopiers, and slide projectors in her home and workplace and in public places. Encourage her to limit outdoor activity between 10 a.m. and 4 p.m. and to use a sunscreen with a sun protection factor of at least 30.6 Also teach her to protect herself from sunlight by wearing a wide-brimmed hat and long-sleeved clothing.
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Alliance for Lupus Research. http://www.lupusresearch.org.
American Autoimmune Related Diseases Association, Inc. http://www.aarda.org.
Arthritis Foundation. http://www.arthritis.org.
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SLE Lupus Foundation. http://www.lupusny.org.
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