Approach Considerations
The most important tool in the medical care of the patient with systemic lupus erythematosus (SLE) is careful and frequent clinical and laboratory evaluation to tailor the patient’s medical regimen and to provide prompt recognition and treatment of disease flare, which is the cornerstone of successful intervention. Because lupus is a lifelong illness, patients must be indefinitely monitored.
Consideration should be given to the prevention of atherosclerosis and osteoporosis, because these are long-term consequences of SLE and its treatment. Moreover, studies have brought attention to the need for the preservation of gonadal function when gonadotoxic therapies are used to treat severe disease.
Recently, consensus treatment plans have been published for pediatric lupus proliferative nephritis class III and IV. These consensus treatment plans are currently being studied in a comparative effectiveness trial. [21, 22]
Newer biologic therapies are looming as potential treatments for lupus, including belimumab, a B-cell directed therapy, [23] which was approved for use in adult lupus in 2011. [24] Belimumab was approved for children with SLE in April 2019.
Belimumab is the first drug approved for pediatric SLE. Belimumab is a B-lymphocyte stimulator (BLyS)-specific inhibitor. BLyS is a naturally occurring protein required for survival and development of B-lymphocyte cells into mature plasma B cells that produce antibodies. In autoimmune diseases, elevated BLyS levels are thought to contribute to production of autoantibodies.
The FDA approval for belimumab for pediatric SLE was based on the PLUTO phase 2 study (n=93). Patient received standard therapy plus belimumab IV or placebo every 4 weeks. The primary endpoint was the SLE Responder Index 4 (SRI4). At 52 weeks, more patients treated with belimumab had responded compared with placebo. Severe flares were 62% less frequent with belimumab. Median time to first severe flare was 159.5 day with belimumab compared with 82 days for placebo. [25]
Others include T-cell–directed therapies, anticomplement therapies, anticytokine therapies, and peptide manipulation to promote tolerance. Stem cell transplantation and high-dose immunoablative therapies are also being studied, but it is unclear whether these therapies confer an advantage. The most recent therapies under trial are monoclonal antibodies directed again interferon-alpha, which are in phase I and II clinical trials, while plasmacytoid dendritic cell inhibitors are in development and in phase I trials.
The need for surgical care depends on the severity of organ involvement and the need for tissue diagnosis. Usually, SLE is not a surgical condition. If surgery is necessary, closely monitor the patient for healing and evidence of infection.
Activity
Encourage patients with systemic lupus erythematosus to maintain a normal lifestyle. Exercise is important in maintaining bone density and an appropriate weight. Caution patients that fatigue and stress have been associated with disease flares. Caution patients to avoid sunlight and to liberally apply waterproof sunblock every 2 hours when exposed to the sun.
Deterrence/prevention
Disease flares lead to poor outcome because of reinjury to vital organs. A poor outcome can be prevented with meticulous medical surveillance and attention to the chronic nature of the disease. Patient and family education is extremely important in this regard. Some flares are the result of excessive sun exposure. These can be avoided using sun protection. (Fluorescent lights may also cause increased rash in patients with SLE.)
Consultations
A rheumatologist should be an integral part of the medical care team supporting the lupus patient. Other consultations depend on the type of organ involvement. Consider consultation with a nephrologist for severe end-organ disease.
Patient transfer
Consider transfer to a tertiary care facility for all children with SLE.
Diet
Dietary restrictions are driven by the patient’s medical therapy. Most patients require a course of corticosteroids and should be on a no-added-salt, low-fat, calcium-sufficient diet. Recognize that patients frequently try nontraditional medical remedies and food supplements. These remedies should be met with an open and supportive response. Monitoring nontraditional remedies and food supplements is important, because they may alter metabolism of more traditional medications, such as warfarin sodium, or they may have a negative effect. Of note, L-canavanine in alfalfa sprouts has been implicated in causing lupus, and excess use should be avoided.
Inpatient Treatment
Inpatient care in patients with systemic lupus erythematosus (SLE) is required for severe hematologic, nephrologic, neurologic, or psychiatric disease or for complications from these (eg, severe anemia, renal failure, stroke, seizure), including the use of intravenous (IV) high-dose corticosteroids or chemotherapy as required. Hospitalization may also be required for severe hypertension.
Inpatient care is appropriate for the patient with unexplained fever to provide sepsis evaluation and treatment, as well as to evaluate the patient for disease flare and to treat him or her accordingly.
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The classic malar rash, also known as a butterfly rash, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds.
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In systemic lupus erythematosus (SLE), many genetic-susceptibility factors, environmental triggers, antigen-antibody responses, B-cell and T-cell interactions, and immune clearance processes interact to generate and perpetuate autoimmunity.
