Snapshot
- A 26-year-old woman presents to the dermatology clinic for fatigue, weakness, and fevers for the past month. She reports significant weight loss despite eating a normal diet. She reports that she sunburns very easily and has a facial rash that is hard to cover with makeup. On physical exam, she has a butterfly rash with nasolabial sparing on her face, several discoid lesions on her fingers, and a erythematous rash on her chest in a V-neck distribution. On laboratory exam, she has a highly positive antinuclear antibody and positive anti-double-stranded DNA antibody.
Introduction
- Clinical definition
- systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by acute flares, commonly presenting with rash, joint pain, and fever
- multiple organ systems are involved, including renal, neurologic, dermatologic, cardiovascular, and hematologic
- systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by acute flares, commonly presenting with rash, joint pain, and fever
- Epidemiology
- demographics
- more common in female patients of reproductive age
- African American, Asian, or Hispanic descent
- risk factors
- family history
- oral contraceptive use
- hormone replacement therapy
- other autoimmune diseases
- demographics
- Pathogenesis
- unknown but thought to be an interaction among immune dysfunction, genetic factors, and environmental factors
- autoantibodies precipitate immune complexes in multiple organs, including kidneys, skin, and brain
- polyclonal activation of B-cells with the production of autoantibodies against DNA
- complement factors and cytokines also play a key role
- environmental triggers include sunlight (photosensitive rash), infection, and drugs (HIP)
- Hydralazine
- Isoniazid
- Procainamide
- unknown but thought to be an interaction among immune dysfunction, genetic factors, and environmental factors
- Associated conditions
- antiphospholipid syndrome
- increased risk of thrombosis
- increased risk of atherosclerosis
- lupus nephritis
- anti-DNA immune complexes deposition in glomeruli
- nephritic or nephrotic syndrome
- diffuse proliferative is the most common and most severe type
- drug-induced lupus
- typically positive for antinuclear antibody and antihistone antibody
- typically without renal or neurologic involvement
- complement level is typically normally
- Libman-Sacks endocarditis (LSE)
- noninfectious endocarditis characterized by thrombi on the mitral or aortic valves (LSE in SLE)
- Raynaud phenomenon
- antiphospholipid syndrome
- Prognosis
- often have recurrent flares
Presentation
- Symptoms
- constitutional symptoms
- fatigue, fever, or weight loss
- arthralgias
- constitutional symptoms
- Physical exam
- cutaneous findings
- malar rash (raised or flat erythematous butterfly rash on cheeks/nose and spares nasolabial fold)
- photosensitive rash
- oral ulcers
- neurologic findings
- behavioral changes
- stroke
- seizures
- headaches
- chance in psychiatric status
- renal findings
- hematuria
- proteinuria
- cutaneous findings
- thrombocytopenia
Studies
- Labs
- antibodies
- anti-double-stranded DNA (dsDNA) antibody
- often rises during flares
- high specificity but low sensitivity
- poor prognostic factor
- often indicates renal disease
- anti-Smith antibody (antibody to snRNPs)
- high specificity (more than anti-dsDNA) but low sensitivity
- antihistone antibody
- high sensitivity for drug-induced lupus
- anti-double-stranded DNA (dsDNA) antibody
- ↓ complement levels during a flare
- ↓C3, C4, and CH50
- ↑ erythrocyte sedimentation rate
- pancytopenia
- leukopenia, thrombocytopenia, or hemolytic anemia
- antibodies
- Urinalysis
- proteinuria or hematuria may indicate renal disease
- Making the diagnosis
- based on clinical presentation and laboratory studies
- diagnosis confirmed with 4 or more criteria from RASHNIA4
- Renal disease
- Arthralgias
- Serositis
- Hematologic abnormalities
- Neurologic abnormalities
- Immunologic derangements
- Antinuclear antibodies
- 4 types of rashes
- malar
- discoid
- photosensitive
- oral ulcers
Differential
- Acne rosacea
- distinguishing factors
- erythematous papules and pustules on face without nasolabial sparing
- no other systemic findings
- distinguishing factors
- Sarcoidosis
- distinguishing factors
- adenopathy
- restrictive lung disease
- distinguishing factors
- skin findings of lupus pernio, rather than malar rash, discoid lesions, or ulcers
Treatment
- Management approach
- antimalarials are often used alongside steroids for acute flares
- management is often dictated by specific organ involvement
- Conservative
- use sunscreen and avoid sun exposure
- indication
- for all patients
- indication
- use sunscreen and avoid sun exposure
- Medical
- non-steroidal anti-inflammatory drugs (NSAIDs)
- indication
- arthralgias
- indication
- antimalarials
- indications
- dermatologic findings and joint pain
- often used in conjunction with other medications, including steroids
- drugs
- hydroxychloroquine
- chloroquine
- side effects
- risk of retinopathy
- indications
- steroids
- indication
- acute flares
- drugs
- prednisone
- indication
- immunosuppressants
- indications
- patients not responsive to steroids
- patients unable to tolerate steroid taper
- lupus nephritis
- drugs
- azathioprine
- methotrexate
- mycophenolate
- for patients with lupus nephritis
- cyclophosphamide
- for patients with lupus nephritis
- indications
- belimumab
- indication
- patients not responsive to steroids or other immunosuppressants
- mechanism
- indication
- non-steroidal anti-inflammatory drugs (NSAIDs)
- inhibits B-cells
Complications
- Causes of death in SLE
- infections
- renal disease
- cardiovascular disease
- Lupus nephropathy
- can be fatal
- Cardiovascular disease
- leading cause of death in patients with SLE
- includes Libman-Sacks endocarditis, hypertension, and cardiac tamponade
- Thrombosis