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Updated: Jun 18 2021

Systemic Lupus Erythematosus (SLE)

  • summary
    • Systemic Lupus Erythematosus is a chronic autoimmune disorder of unknown etiology that leads to accumulation of autoimmune complexes in joints, skin, kidneys, lungs, heart, blood vessels, and nervous system. Patients presents with polyarthralgia, butterfly malar rash, renal abnormalities, and cardiac disease.
    • Diagnosis is made based on a constellation of clinical and physical examination findings along with laboratory studies such ANA and anti-dsDNA antibodies.
    • Treatment is medical management with NSAIDS, methotrexate, sulfasalazine, cyclosporine, antimalarials and DMARDs.
  • Epidemiology
    • Demographics
      • common in 15-25 year old African-American women
  • Etiology
    • Genetics
      • multiple genes involved
      • multiple HLAs involved
        • HLA class II, HLA class III, HLA-DR, HLA-DQ
    • Systemic manifestations
      • joint involvement is most common feature (75%)
      • pancytopenia
      • pericarditis
      • kidney disease is most common cause of mortality
      • hip osteonecrosis is common in patients taking glucocorticoids
      • Raynaud's phenomenon
  • Presentation
    • Symptoms
      • fever
      • pain in multiple joints
        • hip pain (osteonecrosis)
    • Physical exam
      • butterfly malar rash
      • large joint swelling and synovitis
      • hand and wrist manifestation are common (90%)
        • swelling and synovitis of PIPs, MCPs, and carpus
        • ligamentous laxity
        • Raynaud's phenomenon
        • dorsal subluxation of ulna at DRUJ
  • Imaging Studies
    • Radiographs
      • usually no evidence of joint destruction
      • osteonecrosis of hips is common
  • Labs
    • Usually positive for
      • ANA (95%)
      • anti-dsDNA antibodies
      • HLA-DR3
      • few are RF positive
  • Treatment
    • Nonoperative
      • NSAIDS, methotrexate, sulfasalazine, cyclosporine, antimalarials, DMARDs
        • treatment is similar to RA
    • Operative
      • digit fusion vs resection arthroplasty for hand
        • indicated in advanced joint disease
        • soft tissue procedures have high failure rates
        • arthrodesis is treatment of choice for PIP or DIP deformities
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