Systemic lupus erythematosus

Background

• Autoimmune disorder affecting all systems

Epidemiology

• Female:Male 10:1
• More common in African Americans

Clinical Features

Diagnostic Criteria: 4 out of 11 of the following:

1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis (polyarticular)
6. Serositis (Pericarditis or pleuritis)
7. Renal disorder (renal failure, protenuria, casts)
8. Neurologic or psychotic symptoms
9. Hematologic (anemia, thrombocytopenia, or leukopenia)
10. Immunologic (+antibofy testing)
11. ANA (positive ANA)

Organ system affected:

• Cardiopulmonary
  • Pneumonia
    • Cover for Listeria and Pseudmonas
  • CAD
    • More common and more complications post-PCI
  • PE
  • Pericarditis
  • Endocarditis
    • Infectious and Libman-Sachs

• Neuropsychiatric/Altered mental status
  • Non-convulsive status epilepticus
  • CNS vasculitis
  • Stroke
  • Encephalitis
  • Meningitis

• Musculoskeletal
  • Arthritis
    • Usually symmetric
    • Consider septic arthritis if there is a single inflamed joint
      • Cover for ''Salmonella'' in addition to standard coverage

• GI
  • Lupus enteritis (mesenteric vasculitis)
    • Most common cause of acute abdominal pain
  • Pancreatitis
  • PUD

• Dermatologic
  • Malar rash across bridge of nose
  • Discoid rash, erythematous with scale
  • Treat with topical 1% hydrocortisone

• Renal
  • Usually a nephritis
  • Can cause a glomerulonephrosis

Differential Diagnosis

• Rheumatoid arthritis
• Sjogren's syndrome
• Dermatomyositis
• Polymyositis
• Stevens-Johnson syndrome
• Toxic Epidermal Necrolysis
• Septic arthritis
• Lyme disease
• Vasculitis
• Acute Rheumatic Fever
• Toxic Shock Syndrome
• TTP
• ITP
• DIC

Workup

Undiagnosed

• CBC
• Chem 10
• ANA
• ESR
• UA
• Bedside echo if ill or hypotensive
• (Consider anti-DNA, anti-Smith, anti-Nuclear, anti-phospholipid)

Flair

• Bedside echo if ill or hypotensive
• CBC
• Chem
• UA
• As directed by organ system involved

Management

• Inflammatory complications
  • Methylprednisolone 1-2mg/kg in most cases
• Infectious
  • Stress dose steroids with hydrocortisone 100mg IV Q8hr if on or recently on steroids

• Dermatologic
  • Hydrocortisone 1% cream

Disposition

• Suspected new diagnosis can have out patient workup if well appearing
• Mild flairs can have expedited out patient management
• High-risk population, low threshold to admit

See Also

• Arthritis
• Fever and Rash
• Lupus Anticoagulant
• Pericarditis
• Pericardial Effusion and Tamponade
• Acute Renal Failure
• Adrenal Crisis

Sources

• Rosen's
• Up to date