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).

In this article, we'll tell you how to assess a patient for SLE and what to teach her about managing this chronic disorder.

Unknown stimulus

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.

* 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.

* 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.

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.

Many drugs help

Complementary therapies that may be beneficial include relaxation, meditation, and massage.

Your role

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.

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.

Patient teaching

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.

Outlook improving

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.

Understanding the complement system

"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.

Figure. No caption available.

Dealing with flares

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.

REFERENCES

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Pullen RL. Managing cutaneous vasculitis in a patient with lupus erythematosus. Dermatol Nurs. 2007;19(1):21-26.

Rooney J. Systemic lupus erythematosus: unmasking a great imitator. Nursing. 2005;35(11):54-60.