Lupus has been characterized as a multidimensional, unique, complex, challenging, unpredictable, and often elusive disease [2]. It is a non-organ-specific systemic disease with a varying prognosis that can be mild, serious, life-threatening, or even fatal. The disease is characterized by recurring remissions and exacerbations, often called flares, that occur most commonly in the spring and summer [1,5]. Periods of remission vary considerably among those diagnosed with lupus [2].

Lupus is more common than other better-known disorders, such as leukemia, multiple sclerosis, cystic fibrosis, and muscular dystrophy, affecting more individuals than all of these diseases combined [3]. The number of reported cases of lupus varies based on different sources; it is believed that there are at least 16,000 patients diagnosed with lupus each year, and more than 1.5 million individuals affected at any given time in the United States. Worldwide, it is estimated that more than 5 million individuals have some form of lupus [6,8]. However, because lupus is relatively uncommon, difficult to diagnose, and not a reportable disease, epidemiologic studies are often expensive and tend not to produce reliable results on a large scale. Therefore, the CDC notes that recent national estimates of prevalence and incidence are not available for SLE [6]. Further research is required to determine if lupus prevalence and incidence are changing over time [6].

More than 90% of SLE cases occur in women, with most women developing symptoms in their childbearing years (15 to 45 years of age) [11]. New diagnoses of SLE in women older than 45 years of age are uncommon [3]. SLE is most common among Black individuals, with Black women having three times the incidence of White American women. The incidence of lupus is also greater in Hispanic, Asian, and Native American women when compared to White women. Statistics show that Black and Hispanic women tend to develop the disease at a younger age, are more likely to develop more serious complications (particularly cardiovascular complications and kidney disease), and tend to have a higher mortality rate from the disease as compared to White women [11].

More than 90% of SLE cases occur in women, with most women developing symptoms in their childbearing years (15 to 45 years of age) [11]. New diagnoses of SLE in women older than 45 years of age are uncommon [3]. SLE is most common among Black individuals, with Black women having three times the incidence of White American women. The incidence of lupus is also greater in Hispanic, Asian, and Native American women when compared to White women. Statistics show that Black and Hispanic women tend to develop the disease at a younger age, are more likely to develop more serious complications (particularly cardiovascular complications and kidney disease), and tend to have a higher mortality rate from the disease as compared to White women [11].

Four different forms of lupus have been identified: cutaneous lupus erythematosus (CLE), drug-induced lupus, neonatal lupus, and SLE [2]. CLE mainly affects the skin and can be acute or chronic, with skin eruptions that, if left untreated, can lead to scarring and permanent disfigurement. Drug-induced lupus is associated with ingestion of various drugs that result in lupus-like symptoms. Neonatal lupus is a rare, non-systemic condition affecting infants of women with lupus. SLE, which affects multiple organ systems as well as the skin, is considered the most common of the four forms, accounting for 70% of all cases of lupus.

CLE is associated with various types of acute and chronic lupus, but the most prevalent form is discoid lupus erythematosus (DLE). DLE is often considered a mild form of lupus, while SLE is the most severe form of the disease [14]. About 20% of individuals with DLE will develop SLE [10].

Infants born to women with lupus are at risk for a rare condition referred to as neonatal lupus, which is not a true form of lupus. It occurs when an infant passively acquires autoantibodies from a mother with SLE [14]. Approximately 3% of infants born to women with lupus will have this temporary condition [2]. Women with diagnosed lupus should be screened for neonatal lupus during pregnancy by having a maternal blood test between 18 and 24 weeks gestation [2]. Infants with neonatal lupus may experience a rash, blood abnormalities, and liver problems. Most resolve by 6 months of age [14]. Some infants develop potentially serious complete heart block [5,6,14]. The heart block is treated with a pacemaker insertion [6]. In addition, approximately 25% of these infants are born prematurely [2].

Immune dysregulation, in the form of autoimmunity, is thought to be the prime cause of lupus. In patients with lupus, the body produces an accelerated inflammatory response, resulting in the production of autoantibodies (antibodies to one's self), causing immune complexes (antigens combined with antibodies) [3,16]. These autoantibodies and complexes assault the body's own healthy cells and tissues [2,3,4,5]. Antigen-antibody complexes can attack or suppress the body's normal immunity and cause damage to tissues. Symptoms of lupus are the result of the damage to the body's tissues secondary to the immunologic response.

A shortage or functional failure of T lymphocytes is believed to be partially responsible for this autoimmune reaction. Red blood cells, neutrophils, platelets, lymphocytes, or almost any organ or tissue in the body may be attacked. One of the hallmark indicators of lupus is the formation of autoantibodies, and the presence of autoantibodies in the blood is a key factor to the diagnosis of lupus [2,3,5].

The strong hereditary component of lupus is supported by the fact that first- and second-degree relatives of patients with lupus are at a greater risk for developing lupus [17]. Estimates indicate that 5% to 13% of relatives will develop lupus, but only 5% of children whose mothers had lupus will develop the disease [17]. An immediate blood relative with a history of other autoimmune disorders, such as rheumatoid arthritis or scleroderma, also appears to place individuals at greater risk for developing lupus [4].

No two people with lupus will experience identical symptoms. The onset of lupus may be acute or insidious, vague, or even nonspecific. On average, individuals with lupus have symptoms of the disease for two to three years before a diagnosis is made [3]. Symptoms are the result of the inflammatory and immune response of the individual's body to the disease process [3]. Repetitive cycles of exacerbations and remissions of symptoms are a hallmark of the lupus disease process.

Common symptoms of lupus include fever, weight loss, malaise, fatigue, skin rashes, polyarthralgia, vasculitis, Raynaud syndrome (discussed in detail later in this course), patchy alopecia (hair loss), and painless ulcers of the mucous membranes [5]. Fatigue is probably the most universal symptom, described as a persistent complaint of a paralyzing fatigue that normal rest may not relieve [2]. Vague symptoms of lupus include aching, fatigue, low-grade or spiking fever, chills, and malaise. Episodic fever is reported by more than 80% of all patients with lupus, with a low-grade fever most often noted [2]. Infection is certainly a major concern and is a potential symptom for patients with lupus. Those with lupus are more susceptible to opportunistic infections due to alterations in their hematologic system, especially in white blood cells. Women with lupus may also experience irregular periods or amenorrhea due to the disease process [2,3].

Vascular symptoms may include vasculitis or Raynaud syndrome. Vasculitis may include necrotic ulcerations that occur most frequently on the lower legs, ankles, and dorsa of the feet [2]. Raynaud syndrome can develop, especially in the digits of the hands and feet, secondary to stress, cold, or vibratory stimuli [3]. This condition is caused by sudden onset of vasospasms of the fingers and toes. The vasospasms cause the digits to tingle and the extremities to turn red, blue, or white in color. In serious cases, it can lead to infarctive lesions, necrotic ulcers, or gangrene. This form of cold sensitivity occurs in approximately 40% of patients with lupus [23].

The antinuclear antibody (ANA) test is the most specific and sensitive test for lupus and is therefore the most commonly used autoantibody test. Ninety-seven percent of patients with lupus have a positive ANA blood test. The titer and patterns of the blood sample are reported. A titer greater than 1:80 is usually considered positive [24]. It is important to note that a positive ANA test is found in 97% of patients with lupus, but alone, it does not indicate a conclusive diagnosis of lupus [24]. A positive ANA test, although not always found, satisfies one of the four typical clinical characterizations required for a definitive diagnosis of lupus. ANA tests may also be positive in patients with other connective tissue diseases, chronic infectious diseases, and autoimmune diseases [24].

In cases of mild disease, little or no medication may be needed [30]. Medications used for mild lupus with little or no organ involvement may include NSAIDs that are used to control arthritis pain, inflammation, and fever. NSAIDs function to control and reduce inflammation secondary to the lupus. Examples of generic NSAIDs include ibuprofen, indomethacin, and naproxen. The major side effects of NSAIDs include gastrointestinal upset, ulcers, and a potential decline in renal function. Other NSAIDs available for the management of lupus symptoms include selective cyclooxygenase-2 (COX-2) inhibitors [30]. These drugs have been very effective in the reduction of inflammation by selectively controlling the chemicals that cause inflammation in the patient with lupus [2]. While some of the COX-2 inhibitors are considered to be safe, they may increase the chance of blood clots in some at-risk patients [2]. Topical treatment for skin lesions may include corticosteroid creams.

Hydroxychloroquine sulfate may also be used to treat lupus. This drug is an antimalarial but is used to treat lupus because it focuses on containing the immune response. In addition, it may act as an anticoagulant and cholesterol reducer [5]. Possible side effects of antimalarials include rash, nausea, and headache. A serious potential side effect of antimalarial drugs is retinal damage. An eye examination should be completed before treatment begins and annually thereafter [2].

Generally, those with lupus are in the young adult development stage of life when an initial diagnosis is made. Lupus may cause alterations in the individual's ability to achieve specific tasks fundamental to this developmental stage. Common tasks or activities for the young adult's developmental goal achievement generally include gaining independence, achievement of vocational goals, establishment of intimate relationships, social responsibility, childbearing, and childrearing [1]. Limitations in any of these developmental activities for this specific age group can result in challenges for the individual diagnosed with lupus.

Baker and Wiginton found that study participants "expressed concern that others in their lives did not understand lupus and failed to acknowledge the seriousness of their conditions due to the symptoms not being readily apparent" [44]. Family and friends may deny that the disease is a problem, fail to assist the individual, or fail to understand the disease, particularly if the individual shows no outward signs of the disease. Falvo terms this "invisible disability" [1]. Lupus is one such invisible chronic illness. Additionally, some people may be uncomfortable being in a relationship with an individual with a chronic illness like lupus. They may not know what to say, worry about saying the wrong thing, or fear that the chronic disease is contagious.

Tremendous stress may be associated with managing a chronic illness. The loss of control related to the chronic disease can exacerbate the stress level for an individual. Stress also may be elevated by the compromising of roles and changes in the level of functioning [1]. It is vitally important that an individual's personal perception of the stresses associated with the disease, as well as their capacity to cope, be considered, as perceptions of stress and coping abilities will vary. Ineffective coping and high stress levels in individuals with chronic illnesses can result in depression, which is the most common coping problem in patients with lupus [3].