Updated: 11/22/2017

Systemic Lupus Erythematosus

Topic
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Questions
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Evidence
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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
      • 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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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.MK.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? Review Topic

QID: 106746
FIGURES:
1

Pericarditis

62%

(60/96)

2

Myocarditis

10%

(10/96)

3

Conduction arrhythmias

4%

(4/96)

4

Valvular disease

18%

(17/96)

5

Coronary artery disease

4%

(4/96)

M1

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PREFERRED RESPONSE 1
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(M1.MK.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? Review Topic

QID: 100456
FIGURES:
1

Anti-acetylcholine receptor

3%

(4/137)

2

Anti-Ro

3%

(4/137)

3

Anti-La

1%

(1/137)

4

Anti-ssDNA

3%

(4/137)

5

Anti-dsDNA

89%

(122/137)

M1

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PREFERRED RESPONSE 5
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