questions 5

Systemic Lupus Erythematous

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Topic updated on 06/10/17 7:24pm

Snapshot
  • A 21-yearPhoto-old African-American female presents to the clinic complaining of weakness and malaise, which has grown worse over the last month. She has lost 9 kg despite maintaining her regular diet. She reports that whenever she goes into the sun she gets a painful rash on her face. She has a fever of 40 degrees Celsius. Laboratory results reveal that she is ANA positive.
Introduction
  • A multi-system, autoimmune disorder
  • Characterized by 
    • polyclonal activation of B cells resulting in the production of anti-DNA antibodies
    • antibody-mediated cellular attack
    • deposition of antigen-Ab complexes
  • Triggers include
    • sunlight
    • drugs
      • specific agents known to produce SLE-like syndrome
      • usually resolves when drug is discontinued
      • most common agents include
        • hydralazine 
        • procainamide
        • chlorpromazine
        • isoniazid
        • methyldopa
        • quinidine
        • penicillamine
  • Usually affects women (90%) of child-bearing age, especially African-Americans
  • Neonatal Lupus Syndrome is one manifestation of the disease
Presentation
  • Symptoms
    • usually present with non-specific complaints including
      • fever
      • anorexia
      • weight loss
      • arthralgia
      • myalgia
  • Physical exam
    • malar rash
    • oral ulcers
    • photosensitivity
    • arthritis at large joints
    • muscle stiffness or pain
    • may identify neurologic changes
    • may identify psycho-social changes
    • thrombogenic state
Evaluation
  • Diagnosis made when 4 of the following criteria are met (remember "DOPAMINE RASH")
    • Discoid rash
      • erythematous, circular macules with scales
      • occur in 80% of cases
    • Oral aphthous ulcers
    • Photosensitivity
    • Arthritis of large joints
      • arthralgias and myalgias are the most common presentation (95% of cases)
      • symmetric morning stiffness
      • swelling of the hands involving MCP and PIP joints
        • occurs in 60% of cases
    • Malar rash (50% of cases)
    • Immunologic labs 
      • anti-ds-DNA 
        • very specific to SLE
      • anti-Sm antibody
        • very specific, but not sensitive to SLE
      • anti-Ro antibody
    • Neurologic changes
      • range from psychosis to seizures to personality change 
    • ESR elevated
    • Renal disease
      • 50-60% of cases
      • most common cause of death in SLE patients
    • ANA (antinuclear antibodies)
      • very sensitive, but not specific to SLE
      • also positive in other collagen vascular diseases
    • Serositis 
      • occurs in 30-50% of cases
      • the two most common manifestatoins are pericarditis and pleuritis
      • exudative effusion
      • any unexplained pleural effusion in a young woman is SLE until proven otherwise
         
    • Hematologic changes
      • including anemia or thrombocytopenia
  • Serum C4 level
    • may be deminished
      • secondary to activation of the classical complement system in SLE
  • RPR/VDRL test
    • may be falsely positive for syphilis in SLE patients due to cross-reactivity phenomenon
  • PT-INR, PTT
    • PTT usually elevated in SLE patients
 
Anti-ds DNA
Anti-
histone
Anti-
SM
Anti-
SSA (Ro)
Anti-
SSB (La)
Anti- centromere
Anti-
ribonucleo
proteins
Anti-
mitochondia
SLE
98%
 
100%
 
 
 
 
 

Drug-induced SLE

 
95%
 
 
 
 
 
 

Sjogren's

 
 
 
70-95%
60-90%
 
 
 
CREST
 
 
 
 
 
90%
 
 
Mixed CT Disease
 
 
 
 
 
 
95%
 
Primary Biliary Cirrhosis
 
 
 
 
 
 
 
90%

 

Differential
  • The differential diagnosis for SLE is very broad, given its system effects, and so its presentation often overlaps with many autoimmune, neoplastic, or infectious pathologies
  • A few common diagnoses to rule out in this age group would be lymphoma, leukemia, tuberculosis, HIV/AIDS, bacterial endocarditis among others
Treatment
  • Medical management 
    • NSAIDS
      • indicated in most SLE patients for mild joint symptoms
    • steroids
      • indicated for acute exacerbations
    • hydrochloroquine, cyclophosphamide, and azathioprine
      • indicated in progressive and refractory cases of SLE
  • Lifestyle modifications
    • avoid exposure to sunlight
Prognosis, Prevention, and Complications
  • Prognosis
    • ranges widely and is hard to predict, as individual patient courses differ
    • 10-year survival is excellent, however
  • Prevention
    • no preventive measures are available
  • Complications
    • secondary renal disease is most common cause of death in SLE patients
ADDENDUM: Neonatal Lupus Syndrome
  • Presents with
    • skin rash
    • thrombocytopenia
    • possible congenital heart block
    • usually anti-Ro positive
    • neonatal SLE is most common cause of congenital heart blocks

 



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Qbank (2 Questions)

TAG
(M3.RH.3) A 29-year-old woman with a 3-year history of systemic lupus erythematosus (SLE) presents to her rheumatologist for routine follow up. Her course has been complicated by lupus nephritis (baseline creatinine 1.1 mg/dL) and she has been on stable doses of methotrexate and hydroxychloroquine for the last 2 months following a flare. Which of the following lab values can be used to best monitor this patient's disease? Topic Review Topic

1. Anti-nuclear antibody
2. Anti-dsDNA antibody
3. Anti-Smith antibody
4. Anti-RNP antibody
5. CH50 level

PREFERRED RESPONSE ▶
TAG
(M2.RH.9) A 24-year-old African-American woman presents to her primary care physician complaining of weakness and malaise. She complains that she has unintentionally lost 10 pounds of weight in 2 weeks. Upon further questioning, it is found that she gets a painful rash on her face when she goes outside in the sun. Figure A is noted upon physical examination. Her hemoglobin level was found to be 8.1 g/dl. What is most likely to be found on laboratory testing? Topic Review Topic
FIGURES: A          

1. Positive anti-ds-DNA antibodies
2. Positive anti-La antibodies
3. Positive anti-centromere antibodies
4. Positive anti-cardiolipin antibodies
5. Positive anti-ganglioside antibodies

PREFERRED RESPONSE ▶
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