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Information

Classification criteria used to confirm SLE in studies can provide a basis in individual patients for estimating the probability that a disease is SLE. Four or more published criteria carry a 93% specificity and 92% sensitivity for SLE (Table 378-3, p. 2127, in HPIM-19).

Treatment: Systemic LUPUS Erythematosus

Choice of therapy is based on type and severity of disease manifestations. Goals are to control acute, severe flares and to develop maintenance strategies whereby symptoms are suppressed to an acceptable level. Treatment choices depend on (1) whether disease is life threatening or likely to cause organ damage; (2) whether manifestations are reversible; and (3) the best approach to prevent complications of disease and treatment (Fig. 378-2, p. 2128, and Table 378-5, p. 2130, in HPIM-19).

Conservative Therapies for Non-Life-Threatening Disease

  • NSAIDs (e.g., ibuprofen 400-800 mg three to four times a day). Must consider renal, GI, and cardiovascular complications.
  • Antimalarials (hydroxychloroquine 400 mg/d): may improve constitutional, cutaneous, articular manifestations. Ophthalmologic evaluation required before and during treatment to rule out ocular toxicity.
  • Belimumab (10 mg/kg IV at weeks 0, 2, 4 then monthly). B-lymphocyte stimulator (BLyS)-specific inhibitor. Should not be used in severe SLE such as nephritis or CNS disease and limited to pts with mild to moderate active disease.

Treatments for Life-Threatening SLE

  • Systemic glucocorticoids.
  • Cytotoxic/immunosuppressive agents: added to glucocorticoids to treat serious SLE.
    1. Cyclophosphamide: administered as IV pulse 500-1000 mg/M2 IV × 6 months followed by maintenance with mycophenolate mofetil or azathioprine. European studies have found cyclophosphamide 500 mg every 2 weeks for 6 doses may be effective, but it remains unclear whether these data will apply to U.S. populations.
    2. Mycophenolate mofetil: 2-3 g/d; efficacy data limited to nephritis. A higher proportion of African-American pts appear to respond to mycophenolate mofetil compared with cyclophosphamide.
    3. Azathioprine: may be effective but is slower in inducing therapeutic response.

Outline

Section 12. Allergy, Clinical Immunology, and Rheumatology