Systemic lupus erythematosus (SLE)



Background

  • Chronic inflammatory disease of connective tissue with multi-organ involvement
  • More severe and more frequent in non-caucasions
  • F > M
  • ? Oestrogen as those on OCP more susceptible
  • Multiple anigens and auto-antibodies
  • 4 or more manifestations (below)

Clinical Manifestations

  • Serositis (pleuritis, pericarditis)
  • Oral ulcers
  • Arthritis (nonerosive)
  • Photosensitivity (exposure to UV radiation causes skin manifestations)
  • Blood dyscrasias (hemolytic anemia, leukopenia, thrombocytopenia)
  • Renal manifestations (proteinuria, casts)
  • Positive ANA finding
  • Immunologic disorders (anti-Smith antibodies positive, antiphospholipid antibody positive, false positive test for syphilis, presence of anti–double-stranded DNA antibodies)
  • Neurologic manifestations (seizures, focal signs, psychosis)
  • Malar (butterfly) rash (photosensitive)
  • Discoid rash

Complications

Renal disease (30%-60%)

  • Proteinuria
  • Active urinary sediment (> 5 RBC per high powered field)
  • Pyuria
  • Casts
  • Urinary monitoring in inactive disease
  • Renal biopsy in all with signs of kidney involvement
    • (glomerulonephritis clasification and grading)
    • May need repated Bx with flare ups

Respiratory

  • Pleural inflammation is common
    • Chest pain, SOB, cough.
    • Pleural effusions typical in ANA-positive exudates with low complement
    • Occasional pulmonary hypertension or interstitial disease

Labs

  • Anemia (normochromic normocytic or Fe++ deficient
  • Leukopenia (lymphopenia - not granulocytopenia), thrombocytopenia (disease or drug effects)
  • Antiphospholipid disorder (thrombotic disorder)
  • Anticardiolipin antibodies give a false positive test for syphilis

Treatment

  1. Suppress flares
  2. Maintenance prevent organ damage

Mild disease

( arthritis, dermatitis, and constitutional symptoms)

NSAIDs, hydroxychloroquine, and low-dose steroids

Severe disease

High dose steroids with cyclophosphamide to induce remission, followed by longer-term, immunosuppressive therapy


Content by Dr Íomhar O' Sullivan 10/09/2010. Last review Dr IOS 16/12/19.