Rosen & Barkin's 5-Minute Emergency Medicine Consult (695 page)

• Neurosyphilis requires IV antibiotics
  
• Pregnant women allergic to penicillin requiring desensitization
  

Follow-up care:

• Measure for falling titers in 6 mo and 1 yr after treatment.
  
• Tertiary/latent (>1 yr):
  
  • Measure for falling titers in 3, 6, 12, and 24 mo after treatment.
    

Infectious disease consultation for secondary and tertiary syphilis as well as congenital and neurosyphilis

Titers must be monitored.

• Syphilis is known as the “great imitator.”
  
• In patients presenting with unknown rash, think of syphilis and ask about history of genital lesions.
  
• Be sure to examine mucous membranes of all patients presenting with rash.
  
• Think of tertiary syphilis with neurologic symptoms of unknown etiology.
  

• Centers for Disease Control and Prevention (CDC). Primary and secondary syphilis–United States, 2003–2004.
  MMWR Morb Mortal Wkly Rep.
  2006;55(10):269–273.
  
• Domantay-Apostol GP, Handog EB, Gabriel MT. Syphilis: The international challenge of the great imitator.
  Dermatol Clin.
  2008;26(2):191–201, v.
  
• Grange PA, Gressier L, Dion PL, et al. Evaluation of a PCR test for detection of treponema pallidum in swabs and blood.
  J Clin Microbiol.
  2012;50:546–552.
  
• Hook EW 3rd, Peeling RW. Syphilis control – a continuing challenge.
  N Engl J Med.
  2004;351:122–124.
  
• Schacter J. Classification of latent syphilis.
  Sex Transm Dis.
  2005;32:143.
  
• Sparling PF. Diagnosis of neurosyphilis: New tools.
  Sex Transm Dis.
  2010;37:288–289.
  
• Timmermans M, Carr J. Neurosyphilis in the modern era.
  J Neurol Neurosurg Psychiatry.
  2004;75:1727–1730.
  
• Tramont EC. Treponema pallidum (syphilis). In: Mandell GL, Bennett JE, Dolin R, eds.
  Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases
  . 7th ed. Philadelphia, PA: Churchill Livingstone; 2009, Chapter 238.
  

CODES

• 091.2 Other primary syphilis
  
• 091.9 Unspecified secondary syphilis
  
• 097.9 Syphilis, unspecified
  

BASICS

• Chronic autoimmune disease; peak onset between ages 15 and 40 yr; characterized by flares and remissions
  
• Multisystem disease with diverse clinical manifestations:
  
  • Mucocutaneous:
    
    • Most commonly involved system
      
    • 4 specific skin rashes
      
  • Arthritis
    
  • Cardiac:
    
    • Endocarditis
      
    • Myocarditis
      
    • CHF
      
    • Conduction abnormalities
      
    • Atherosclerosis
      
    • MI
      
  • Renal:
    
    • Glomerulonephritis
      
    • Renal failure
      
  • Pulmonary:
    
    • Pleural effusion (usually exudative)
      
    • Pneumonitis/pleuritis
      
    • Pulmonary hemorrhage
      
    • Pulmonary embolism
      
    • Pneumonia
      
    • Pulmonary edema
      
    • Pulmonary hypertension
      
  • Neurologic:
    
    • Lupus cerebritis
      
  • Vascular:
    
    • Vasculitis
      
    • Thrombosis
      
    • Atherosclerosis
      
  • GI:
    
    • Peritonitis
      
    • Mesenteric vasculitis and ischemia
      
    • Pancreatitis
      

• Neonatal lupus may occur when maternal autoantibodies cross the placenta:
  
  • Associated with transient anemia and thrombocytopenia
    
• Congenital heart block is the most serious complication
  

• 10 times greater risk of MI due to atherosclerosis
  
• High incidence of osteoporosis related to chronic steroid use
  

• More common in females than males (9:1 ratio)
  
• More common in women of childbearing age
  
• More common in African Americans
  
• Higher frequency of systemic lupus erythematosus (SLE) and other autoimmune diseases among 1st-degree relatives
  

• Autoantibody production against cell nucleus and cytoplasmic structures, leading to inflammatory changes, vasculitis, and immune complex deposition in multiple organ systems
  
• A significant percentage of patients have an associated antiphospholipid syndrome:
  
  • Characterized by antibodies against cellular phospholipid components
    
  • Tendency toward recurrent vascular thrombosis
    
• Lupus is a chronic disease with several exacerbating factors:
  
  • Infection
    
  • Sun exposure
    
  • Fatigue
    
  • Trauma
    
  • Medications (sulfonamides)
    
  • Stress
    
  • Diet
    
• Drug-induced lupus is a milder disease that eventually resolves once the drug is discontinued. Usually presents with skin and joint manifestations while renal and neurologic involvement is rare.
  
• Common medications include:
  
  • Chlorpromazine, methyldopa, procainamide, hydralazine, isoniazid, quinidine, minocycline
    

DIAGNOSIS

• 4 of the 11 criteria in the following list are needed to make the diagnosis:
  
  • Malar rash
    
  • Discoid rash
    
  • Photosensitivity rash
    
  • Oral ulcers
    
  • Arthritis
    
  • Serositis
    
  • Neurologic disorders
    
  • Hematologic disorders
    
  • Immunologic disorders
    
  • Renal disorders
    
  • Antinuclear antibodies
    

• Systemic:
  
  • Fatigue
    
  • Fever
    
  • Weight loss
    
  • Dyspnea
    
• Skin:
  
  • Malar rash (butterfly maculopapular facial)
    
  • Discoid rash (raised red patches)
    
  • Photosensitivity rash (subacute cutaneous lupus)
    
  • Bullous rash (large blisters)
    
• Musculoskeletal:
  
  • Myalgias
    
  • Joint pain
    
  • Arthritis:
    
    • Defined as 2 or more peripheral joints
      
    • Polyarthritis, symmetric, or migratory
      
• Heart:
  
  • Chest pain
    
  • Pericardial rub
    
  • Murmur
    
• Vascular:
  
  • Vasculitis
    
  • Thrombosis
    
  • Atherosclerosis
    
  • Peripheral vascular disease
    
• Lungs:
  
  • Dyspnea
    
  • Tachypnea
    
  • Pleural rub
    
  • Rales
    
• Nervous system:
  
  • Psychosis/depression
    
  • Headache
    
  • Seizures
    
  • Peripheral neuropathies
    
  • Stroke/cranial nerve deficits
    
  • Cerebritis
    
• GI:
  
  • Painless oral ulcers
    
  • Abdominal pain
    
  • Positive stool guaiac suggests mesenteric ischemia
    

• Symptoms commonly accumulate and exacerbate over years, with flares and remissions. A history of fatigue, rashes, and joint pain may point to the diagnosis.
  
• Patients describe arthralgias out of proportion to physical findings
  

• Check for fever
  
• Carefully evaluate skin for rashes and vasculitis
  

• Thorough history and physical exam needed to distinguish between major and minor flare-ups
  
• Major flare-ups:
  
  • CBC
    
  • Electrolytes, BUN, creatinine, glucose
    
  • UA
    
  • ESR
    
  • Chest radiograph, ECG, and pulse oximetry for cardiorespiratory symptoms
    

• CBC:
  
  • Leukopenia, thrombocytopenia, normochromic normocytic anemia
    
  • Degree of hematologic disorders suggests degree of disease activity
    
• ESR:
  
  • May be elevated during acute exacerbations
    
  • Not a good indicator of active disease
    
• CRP may also be elevated; marked elevation may be a sign of infection
  
• PTT:
  
  • May be elevated in patients with lupus anticoagulant
    
• UA:
  
  • Protein (persistent proteinuria > 0.5 g/day or 3+ persistently)
    
  • Casts (red blood cell)
    
  • Hematuria
    
  • WBCs
    
• Amylase is elevated in mesenteric ischemia and pancreatitis
  
• Send antinuclear antibody, rheumatoid factor (RF), antistreptolysin O (ASO) titer if diagnosis unclear
  
• Anti-Sm and anti-dsDNA are diagnostic
  
• A false-positive Venereal Disease Research Laboratory (VDRL) test is supportive of the diagnosis
  
• Joint aspirate typically shows fluid with fewer than 3,000 WBCs
  
• LP if suspicion for meningitis or encephalitis