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Clinical Summary

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any organ. It is nine times more common among woman, typically occurring during the reproductive years. The most common manifestations include constitutional symptoms, skin rash, oral ulcers, polyarthralgias/arthritis, and renal and central nervous system involvement. In addition, there is a high prevalence of antiphospholipid antibodies among patients with SLE, resulting in hypercoagulability with an increased risk of deep vein thrombosis and pulmonary embolism. Patients with SLE are at significant risk for cardiovascular complications including ischemic stroke and myocardial infarction. In fact, the most common cause of premature death in patients with SLE is accelerated atherosclerosis.

Management and Disposition

The SLE patient with a possible lupus flare does require a comprehensive evaluation in order to identify life- or organ-threatening disease. Serious manifestations such as worsening lupus nephritis, autoimmune hemolytic anemia, or lupus cerebritis require rapid rheumatology consultation, aggressive treatment with high-dose corticosteroids, and hospital admission. While select patients with lupus flares that are limited to skin, joints, and pleuritis/pericarditis may be discharged with a steroid taper and close rheumatology follow-up, this should ideally be done after discussion with the treating rheumatologist.

Distinguishing between lupus flare and infection can be challenging. When in doubt, empiric antibiotic treatment with close observation and further workup may be required.

FIGURE 26.7

Systemic Lupus Erythematosus—Butterfly Rash. Appearance of the malar “butterfly” rash in a dark-skinned patient. Note scales on the cheeks and frontal thinning hairline. (Photo contributor: Lawrence B. Stack, MD.)

Given the hypercoagulability and accelerated atherosclerosis in patients with SLE, chest pain in this patient population needs to be approached with a high level of suspicion for serious complications such as acute coronary syndrome and pulmonary embolism. Prediction tools such as the PERC score, Wells score, or HEART score have been found to underestimate the risk in these patients.

FIGURE 26.8

Systemic Lupus Erythematosus—Butterfly Rash. Erythematous malar rash with slight edema and minimal scaling (“butterfly pattern”). (Reproduced with permission from Wolff K, Johnson RA, Suurmond D. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology. 5th ed. New York, NY: McGraw Hill; 2005, p. 385.)

Pearls

  1. New-onset or worsening psychosis and/or seizures in a patient with lupus should raise suspicion for lupus cerebritis. Of note, magnetic resonance imaging (MRI) can be normal in patients with neuropsychiatric lupus.

  2. Hypersensitivity to sulfa is more common in patients with SLE, and sulfonamides can induce disease flares. Thus, sulfa-containing medications such as trimethoprim-sulfamethoxazole should be avoided.

  3. Lupus patients are immunosuppressed, even when not on immunosuppressive medications, and have a higher incidence of infections such as shingles, candidiasis, and pneumonia.

  4. The serum creatinine may be within normal limits early during a ...

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