Updated: 11/29/2020

Systemic Lupus Erythematous

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Snapshot
  • A 46-year-old African American woman with history of systemic lupus erythematosus presents with increasing fatigue, arthralgias, rash, and hematuria. She reports that she had previously been on hydroxychloroquine with success but had not seen a physician in 5 years. Physical exam shows a malar rash on her face. Laboratory exam reveals a highly positive antinuclear, anti-double-stranded DNA, and anti-Smith antibodies. She also has low complement levels. She is restarted on hydroxychloroquine and steroids.
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
      • 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
        • 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 and antihistone antibodies
      • 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
    • neonatal lupus erythematosus
      • associated with patients with anti-Ro or anti-La antibodies
      • neonates present with rashes and congenital heart block
  • 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
      • anti-Ro and anti-La
        • also seen in Sjögren syndrome
        • associated with higher risk of neonatal lupus erythematosus in pregnant women
      • 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
      • caused by immune-mediated destruction 
    • 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
      • indications
        • for all patients
  • Medical 
    • non-steroidal anti-inflammatory drugs (NSAIDs)
      • indications
        • 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
      • indications
        • 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
      • indications
        • patients not responsive to steroids or other immunosuppressants
      • mechanism
        • inhibits B-cells
Complications
  • Causes of death in SLE
    • infections
    • renal disease
    • cardiovascular disease
      • leading cause of death in these patients
      • includes Libman-Sacks endocarditis, hypertension, and cardiac tamponade
  • Thrombosis
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(M2.RH.17.4799) A 38-year-old woman comes to clinic complaining of general malaise and a facial rash. She reports that the rash began one week ago after she had been playing outside with her dog. The rash has not resolved nor improved since then. She denies pain or pruritus. When asked about new exposures, the patient denies any changes in lotions or soaps, but does report she was recently prescribed a new medication by her primary care physician. The patient denies tobacco or illicit drug use. She is sexually active with one male partner. She works as a school nurse in an elementary school, and she recently traveled to Botswana for two months for a missionary trip. The patient’s temperature is 99°F (37.2°C), blood pressure is 112/72 mmHg, pulse is 74/min, and respirations are 14/min with an oxygen saturation of 99% O2 on room air. On physical exam, you appreciate an oral ulcer, and mild joint stiffness and swelling of the patient’s hands and knees. A photograph of the patient’s facial rash is shown in Figure A. You feel her current presentation is drug-related. Which of the following could be the medication causing her symptoms? Tested Concept

QID: 109182
FIGURES:
1

Amiodarone

0%

(0/29)

2

Carbamezapine

0%

(0/29)

3

Doxycycline

24%

(7/29)

4

Hydralazine

69%

(20/29)

5

Methotrexate

7%

(2/29)

M 6 C

Select Answer to see Preferred Response

(M2.RH.17.4754) A 27-year-old female is brought to the emergency department by police when she attacked her date outside of a pizza restaurant. The patient refused to explain her behavior to the police. She seemed to be responding to internal stimuli when being initially interviewed. The patient has a past medical history of depression, anxiety, and obesity. The patient has a known history of medication non-compliance. Her temperature is 99.5°F (37.5°C), pulse is 112/min, blood pressure is 100/70 mmHg, respirations are 18/min, and oxygen saturation is 98% on room air. On physical exam the patient is poorly kempt and has an odd body odor. Inspection of her oropharynx reveals multiple ulcers. Cardiac exam is unrevealing with the exception of a friction rub. Since the incident, the patient has calmed down and is currently resting. She is no longer responding to internal stimuli and is more cooperative. Lab values are obtained and are below.

Serum:
Na+: 135 mEq/L
Cl-: 100 mEq/L
K+: 3.9 mEq/L
HCO3-: 24 mEq/L
BUN: 20 mg/dL
Glucose: 70 mg/dL
Creatinine: 1.7 mg/dL
Ca2+: 10.0 mg/dL
Mg2+: 1.5 mEq/L

Hemoglobin: 10 g/dL
Hematocrit: 35%
Reticulocyte count: 4.9%
Platelet count: 275,000/mm^3
Leukocyte count: 5,500/mm^3

Which of the following is the next best step in management for this patient's underlying condition?
Tested Concept

QID: 108972
1

IM haloperidol

2%

(1/42)

2

Haloperidol depot

10%

(4/42)

3

Clozapine

7%

(3/42)

4

Urine toxicology

43%

(18/42)

5

ANA and anti-dsDNA levels

36%

(15/42)

M 6 C

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(M3.RH.15.3) A 29-year-old woman presents to the emergency department with joint pain and a notable rash. She has had joint pain for the past 12 months but noticed the rash recently as well as generalized malaise. She states her joint pain is symmetric, in her upper extremities, and is worse in the morning. Her temperature is 97.6°F (36.4°C), blood pressure is 111/74 mmHg, pulse is 83/min, respirations are 14/min, and oxygen saturation is 98% on room air. Laboratory studies are ordered as seen below.

Hemoglobin: 10 g/dL
Hematocrit: 30%
Leukocyte count: 6,800/mm^3 with normal differential
Platelet count: 207,000/mm^3

Serum:
Na+: 140 mEq/L
Cl-: 101 mEq/L
K+: 4.9 mEq/L
HCO3-: 21 mEq/L
BUN: 30 mg/dL
Glucose: 120 mg/dL
Creatinine: 1.8 mg/dL

The patient is ultimately admitted to the hospital. Which of the following is the most appropriate test to monitor her disease progression?
Tested Concept

QID: 102721
FIGURES:
1

Anti-CCP

0%

(0/2)

2

Anti-dsDNA

100%

(2/2)

3

Anti-nuclear antibody

0%

(0/2)

4

Anti-topoisomerase

0%

(0/2)

5

Rheumatoid factor

0%

(0/2)

M 12 E

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