Perspective

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with widespread physical effects caused by the production of autoantibodies to components of cellular nuclei.¹ The term lupus (Latin for “wolf”) is attributed to the 13th-century physician Rogerius, who used it to describe the characteristic facial lesions that were reminiscent of a wolf’s bite.

In some patients the illness is mild. Other patients suffer early, catastrophic organ damage. Early deaths are frequently due to injury to the kidney and brain. Later deaths often occur as a result of acute myocardial infarction, stroke, or infection. Steroids used for treatment may cause or worsen complications.

Epidemiology

SLE is more common by a ratio of 12 : 1 in women aged 15 to 45 years and by a ratio of 2 : 1 in younger and older women. The overall prevalence of this disease is about 1 in 1000. In most studies of SLE, about 90% of enrollees are women. In the United States, the disease is three times more common in black women than in white women. In addition to genetic factors, age, sex, race, and socioeconomic status have an impact on disease expression and prognosis. With optimal management, the 20-year survival rate approaches 70% and the 1-year survival rate is about 90%.²

Pathophysiology

SLE is a prototypic autoimmune disease characterized by tissue damage from excessive antibody production and immune complex deposition. The chronic inflammation characteristic of the disease originates from overproduction of autoantibodies and failure of the body to suppress them.

Nearly every tissue in the body can be affected. Autoantibodies directly react to human antigens, immune complexes are deposited in tissue and blood vessels, and the complement cascade is activated, which results in inflammation and organ damage.

The exact cause is unknown, but genetic predisposition, viruses, ultraviolet light (including sunlight), and medications such as hydralazine, isoniazid, and procainamide are known to be involved in certain patients. There is a relationship to specific human leukocyte antigen genotypes.

Autoantibodies to lupus erythematosus are found in laboratory workers who handle lupus sera. Exposure to certain drugs can produce a SLE-like syndrome. Hormonal factors include an association with estrogens, which may explain the higher prevalence in women.

Presenting Signs and Symptoms

The triad of fever, joint pain, and rash in a woman of childbearing age suggests SLE. The most well-recognized cutaneous finding is the red, raised butterfly rash (Fig. 108.1), but malaise, fatigue, aches, fever, and weight loss are the most common symptoms. The rash, which does not cross the nasolabial fold, may be painful or pruritic. It may be precipitated by sunlight and can last from days to weeks.

Fig. 108.1 Erythematous malar rash of systemic lupus erythematosus.

Note that the rash does not cross the nasolabial fold.

(From Gladman DD, Urowitz MB. Clinical features. In: Hochberg MC, Silman AJ, Smolen JS, editors. Rheumatology. Philadelphia:, Mosby; 2003. pp. 1359–79.)

More than two thirds of patients have vague constitutional symptoms. A thorough evaluation is required before attributing such symptoms to lupus alone. Patients can have kidney failure, infections, adrenal failure, and other complications with similar symptoms (Box 108.1).

See Box 108.1

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