Pediatric Systemic Lupus Erythematosus Treatment & Management

  • Author: Marisa S Klein-Gitelman, MD, MPH; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Sep 28, 2011
 

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.

Newer biologic therapies are looming as potential treatments for lupus, including B-cell directed therapies,[11] one of which has been approved for use in adult lupus.[12] 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.

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.

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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.

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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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Contributor Information and Disclosures
Author

Marisa S Klein-Gitelman, MD, MPH  Associate Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Head, Division of Rheumatology, Children's Memorial Hospital

Marisa S Klein-Gitelman, MD, MPH is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Barry L Myones, MD  Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital

Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References
  1. Osler W. On the visceral manifestations of the erythema group of skin diseases. Am J Med Sci. 1904;27:1.

  2. Suzuki M, Ross GF, Wiers K, Nelson S, Bennett M, Passo MH, et al. Identification of a urinary proteomic signature for lupus nephritis in children. Pediatr Nephrol. Dec 2007;22(12):2047-57. [Medline].

  3. Yurasov S, Wardemann H, Hammersen J, et al. Defective B cell tolerance checkpoints in systemic lupus erythematosus. Journal of Experimental Medicine. 2005;201:703-711. [Medline].

  4. Armstrong DL, Reiff A, Myones BL, Quismorio FP Jr, Klein-Gitelman M, McCurdy D. Identification of new SLE-associated genes with a two-step Bayesian study design. Genes Immun. May 14 2009;[Medline].

  5. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Sep 1997;40(9):1725. [Medline].

  6. Male C, Foulon D, Hoogendoorn H, Vegh P, Silverman E, David M, et al. Predictive value of persistent versus transient antiphospholipid antibody subtypes for the risk of thrombotic events in pediatric patients with systemic lupus erythematosus. Blood. Dec 15 2005;106(13):4152-8. [Medline].

  7. Ross GS, Zelko F, Klein-Gitelman M, et al. A proposed framework to standardize the neurocognitive assessment of patients with pediatric systemic lupus erythematosus. Arthritis Care Res (Hoboken). Jul 2010;62(7):1029-33. [Medline]. [Full Text].

  8. [Guideline] Brunner HI, Ruth NM, German A, Nelson S, Passo MH, Roebuck-Spencer T. Initial validation of the Pediatric Automated Neuropsychological Assessment Metrics for childhood-onset systemic lupus erythematosus. Arthritis Rheum. Oct 15 2007;57(7):1174-82. [Medline].

  9. Brunner HI, Mina R, Pilkington C, et al. Preliminary criteria for global flares in childhood-onset systemic lupus erythematosus. Arthritis Care Res (Hoboken). Sep 2011;63(9):1213-23. [Medline]. [Full Text].

  10. Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int. Feb 2004;65(2):521-30. [Medline].

  11. Podolskaya A, Stadermann M, Pilkington C, Marks SD, Tullus K. B cell depletion therapy for 19 patients with refractory systemic lupus erythematosus. Arch Dis Child. May 2008;93(5):401-6. [Medline].

  12. Navarra SV, Guzmán RM, Gallacher AE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet. Feb 26 2011;377(9767):721-31. [Medline].

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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.
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.
 
 
 
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