Systemic lupus erythematosus

Background

• Autoimmune disorder affecting all systems

Epidemiology

• Female:Male 10:1
• Peak in 20s-30s
• More common in African Americans

Clinical Features

SLICC Classification Criteria 2012

Requirements: >4 of the following criteria (at least 1 clinical and 1 laboratory) OR biopsy proven lupus nephritis with positive ANA or Anti-dsDNA

• Clinical criteria
  • Malar rash, bullous lupus, photosensitivity
  • Discoid rash, hypertrophic lupus
  • Oral ulcers or nasal ulcers
  • Non-scarring alopecia
  • Synovitis
  • Serositis
  • Nephritis
  • Cerebritis, myelitis, neuropathy
  • Hemolytic anemia
  • Leukopenia or lymphopenia
  • Thrombocytopenia

• Immunological criteria

• • ANA
  • Anti-dsDNA
  • Anti-Sm
  • Antiphospholipid antibody
  • Low complement C3, low C4
  • Direct Coombs' test in the absence of haemolytic anaemia

Organ system affected:

• Cardiopulmonary
  • Pneumonia
    • Cover for Listeria and Pseudomonas
  • 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
• Urine pregnancy
• ANA
• ESR
• UA
• Bedside echo if ill or hypotensive
• (Consider anti-DNA, anti-Smith, anti-Nuclear, anti-phospholipid, C3,C4, direct Coombs')

Flare

• Bedside echo if ill or hypotensive
• CBC
• Chem
• UA
• Urine pregnancy
• 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
• Musculoskeletal symptoms can usually be managed as out patients
• Chest pain requires urgent ACS evaluation
• Infections usually require admission for antibiotics and systemic corticosteroids

See Also

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

Sources

• Rosen's
• Up to date