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SLE is a chronic multisystem inflammatory disease → symptoms result from autoantibody production against cellular components of organ systems, inflammation, & tissue damage → variable clinical presentation ∴ individualized treatment

  • Connective tissue disorder → heart, joints, skin, lungs, blood vessels, liver, kidneys, nervous system
  • Disease course unpredictable → acute illness (flares) alternate with symptom remission
  • Autoimmune proliferation → ↑ production of auto-antibodies & immune complexes; defective immune system down-regulation
  • Etiology is largely unknown → ♀:♂ ratio 9:1 usually appears during child-bearing years; prevalence ↑ 2–3× in minorities; African American, Asian, Hispanic vs Caucasian; genetic link → ↑ prevalence in those with family members with SLE
    • Hormonal link → estrogen interferes with B-cell tolerance
    • Triggers → infection, Epstein-Barr virus (EBV), UV radiation, stress, drugs, hormones, others

Diagnostic criteria developed by American College of Rheumatology (ACR)

  • SLE → ≤4 of 11 criteria met (serially or simultaneously during any interval of observation)
    • If <4 criteria met, SLE diagnosis can still be made based on clinical assessment (Arthritis Rheum 1997;40:1725)
  • Other systemic effects
    • Muscles → myalgias, tenderness, proximal muscle weakness; may see ↑ CPK &/or myoglobin; no cases of rhabdomyolysis reported in SLE patients (J Rheumatol 1994;21:1967)
    • Cardiovascular → premature atherosclerosis → chest pressure or pain, dyspnea, diaphoresis, dizziness/lightheadedness
  • Antiphospholipid Antibody Syndrome (APS) → defined by anticardiolipin antibodies (ACA [or ACL]—IgG, IgA, IgM), lupus anticoagulant antibodies (LA–laboratory prolongation in clotting times) & β2-glycoprotein I antibodies (β2GPI—IgG, IgM)
    • Activate endothelial cells, disrupt normal physiologic coagulation processes; LA (but not ACA or β2GPI) can falsely elevate INR; ∼40% with SLE have APS (secondary APS) → not limited to those with lupus diagnosis; APS without lupus or other autoimmune disorder is 1° APS
    • Venous & arterial thrombosis → APS detected in 24% of VTE patients tested for thrombophilia (Thromb Res 2009;124:174)
    • Fetal mortality → 10–15% ♀ with recurrent miscarriage → APS; ASA & heparin ↓ fetal loss (Obstet Gynecol 1992;166:1318)
    • Treatment → anticoagulation–INR: 2–3; for those with clot at documented therapeutic INR, INR goal 3–4 (Hematology 2005;1:462); primary prophylaxis in SLE if + LA/ACA → hydroxycholoroquine plus ↓ dose ASA (Lupus 2011;20:206)
  • SLE prognosis → no cure; variable course → mild to rapidly progressive leading to organ failure; 5y survival >90%
    • Cause of death → active disease: cardiovascular disease & infection (immune system suppression) most common; also CNS, renal, cancer: SLE patients @ ↑ risk of some malignancies

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Clinical Pearl 37-1

Use cytotoxic agents as steroid-sparing agents to reduce impact of steroids on development of atherosclerosis

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Clinical Pearl 37-2

Lupus cerebritis: must exclude steroid-induced necrosis (typically presents during 1st wk of steroid initiation) to diagnose lupus cerebritis

Table 37-1 SLE Diagnosis/Treatment

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