Updated: 11/28/2019

Systemic Lupus Erythematosus

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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
  • 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
  • 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
        • form of type 3 hypersensitivity reaction 
      • 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
  • 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
  • Prognosis
    • often have recurrent flares
Presentation
  • Symptoms 
    • constitutional symptoms
      • fatigue, fever, or weight loss
    • arthralgias
    • serositis
      • pericarditis, pleural effusion, or myocarditis
  • Physical exam
    • cutaneous findings
      • malar rash (raised or flat erythematous butterfly rash on cheeks/nose and spares nasolabial fold)
      • discoid lesions (erythematous raised plaques with keratotic scale and follicular plugging)
      • photosensitive rash
      • oral ulcers
    • neurologic findings
      • behavioral changes
      • stroke
      • seizures
      • headaches
      • chance in psychiatric status
    • renal findings
      • hematuria
      • proteinuria
Studies
  • Labs
    • antibodies
      • antinuclear antibody (ANA)
        • best initial test
        • high sensitivity but low specificity
      • 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
    • ↓ complement levels during a flare
      • ↓C3, C4, and CH50
    • ↑ erythrocyte sedimentation rate
    • pancytopenia
      • leukopenia, thrombocytopenia, or hemolytic anemia
    • elevated partial thromboplastin time (PTT)
      • lupus anticoagulant increases the risk for thrombi and miscarriages
      • associated with antiphospholipid syndrome
  • 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
  • Sarcoidosis
    • distinguishing factors
      • adenopathy
      • restrictive lung disease
      • 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
  • Medical
    • non-steroidal anti-inflammatory drugs (NSAIDs)
      • indication
        • arthralgias
    • antimalarials
      • indications
        • dermatologic findings and joint pain
        • often used in conjunction with other medications, including steroids
      • drugs
        • hydroxychloroquine
        • chloroquine
      • side effects
        • risk of retinopathy
    • steroids
      • indication
        • acute flares
      • drugs
        • prednisone
    • 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
    • belimumab
      • indication
        • patients not responsive to steroids or other immunosuppressants
      • mechanism
        • 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
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(M1.MK.15.75) A 27-year-old woman presents to her primary care physician for evaluation of malaise, joint pains, and rash. Over the last 1-2 months, she has felt generally unwell with fatigue and low-grade fever. More recently, she has started to experience joint pains in her hands and noticed a rash over her face after sun exposure. She is otherwise healthy and takes no medications. Examination reveals an erythematous rash with a small amount of underlying edema (Figure A). Which of the following is the most common cardiac manifestation of this patient's underlying condition? Tested Concept

QID: 106746
FIGURES:
1

Pericarditis

60%

(92/153)

2

Myocarditis

14%

(21/153)

3

Conduction arrhythmias

4%

(6/153)

4

Valvular disease

17%

(26/153)

5

Coronary artery disease

4%

(6/153)

M 4 C

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(M1.MK.13.35) A 40-year-old Caucasian female presents to your office with fever, fatigue, cold fingers, and the rash shown in Figure A. The presence of which of the following antibodies has the highest specificity for diagnosis of this patient's condition? Tested Concept

QID: 100456
FIGURES:
1

Anti-acetylcholine receptor

2%

(4/172)

2

Anti-Ro

4%

(7/172)

3

Anti-La

1%

(1/172)

4

Anti-ssDNA

3%

(5/172)

5

Anti-dsDNA

89%

(153/172)

M 2 D

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