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 [1]
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
- Pneumonia
- 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
- Arthritis
- GI
- Lupus enteritis (mesenteric vasculitis)
- Most common cause of acute abdominal pain
- Pancreatitis
- PUD
- Lupus enteritis (mesenteric vasculitis)
- 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
- ↑ Lisnevskaia L, et al. Systemic Lupus Erythematosus. Lancet. 2014 May 29. Epub ahead of print.