Updated: 10/10/2016

Systemic Lupus Erythematosus (SLE)

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Introduction
  • Chronic autoimmune disorder of unknown origin
    • leads to accumulation of autoimmune complexes in joints, skin, kidneys, lungs, heart, blood vessels, and nervous system
  • Genetic & epidemiology
    • common in 15-25 year old African-American women
    • multiple genes involved
    • multiple HLAs involved
      • HLA class II, HLA class III, HLA-DR, HLA-DQ
  • Systemic manifestations
    • joint involvement imost 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-DNA 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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