What is Lemierre's disease?

Lemierre's Syndrome

Lemierre's syndrome is a rare and potentially life-threatening condition characterized by oropharyngeal infection, internal jugular vein thrombophlebitis, and septic emboli with Fusobacterium necrophorum as the primary causative organism.

Definition and Etiology

Lemierre's syndrome, also known as post-anginal septicemia or necrobacillosis, typically follows an oropharyngeal infection that progresses to septic thrombophlebitis of the internal jugular vein with subsequent metastatic spread 1. The condition most commonly affects previously healthy adolescents and young adults 2.

The primary causative organism is:

• Fusobacterium necrophorum (most common) 2
• Other potential pathogens include:
  • Fusobacterium nucleatum
  • Actinomyces meyeri 1
  • Various anaerobic bacteria including streptococci, staphylococci, and Klebsiella pneumoniae 2

Clinical Presentation

The typical clinical presentation includes:

• Initial symptoms:

  • Pharyngitis or tonsillitis (present in >85% of cases) 2
  • Fever
  • Sore throat
  • Neck pain and tenderness
  • Fatigue 3

• Progressive symptoms:

  • Persistent high fever
  • Rigors
  • Night sweats
  • Tender cervical lymphadenopathy
  • Swelling along the sternocleidomastoid muscle 4

Complications

Metastatic infections can affect multiple organs:

1. Pulmonary (most common): Pneumonia, pleural empyema, cavitating lung lesions 2, 3
2. Neurological: Cerebral venous sinus thrombosis, subdural empyema, cavernous sinus thrombosis 1, 3
3. Vascular: Carotid artery narrowing 3
4. Other: Joint infections, hepatic involvement 5

Diagnosis

A high index of suspicion is crucial for early diagnosis, especially in adolescents or young adults with severe pharyngitis and persistent neck pain 2. Diagnostic approach includes:

• Blood cultures: Essential for isolating the causative organism 6
• Imaging:
  • CT scan with contrast: Gold standard for visualizing internal jugular vein thrombosis 5
  • Ultrasound: May be used as an initial screening tool

Treatment

Treatment should be initiated promptly to avoid significant morbidity and mortality 1:

1. Antibiotics:

   • Prolonged course (3-6 weeks) of antibiotics with anaerobic coverage 2, 3
   • Recommended regimens include:
     • Metronidazole plus β-lactam antibiotics 2
     • Clindamycin
     • Ampicillin-sulbactam 6

2. Anticoagulation: May be considered in cases with extensive thrombosis 1

3. Surgical intervention:

   • Drainage of abscesses when present
   • Rarely, ligation of the jugular vein may be necessary in severe cases 3

Clinical Significance and Prevention

The incidence of Lemierre's syndrome has increased in recent years, possibly due to decreased antibiotic prescription for upper respiratory tract infections as part of antibiotic stewardship programs 2. Clinicians should maintain vigilance when evaluating patients with severe pharyngitis, especially when symptoms persist or worsen despite initial treatment.

Key Points for Clinicians

• Consider Lemierre's syndrome in patients with persistent pharyngitis and neck pain, especially if accompanied by septic symptoms
• Early diagnosis and aggressive treatment are essential for good outcomes
• Include anaerobic coverage in antibiotic regimens
• Monitor for metastatic complications, particularly pulmonary involvement
• Complete the full course of antibiotics (3-6 weeks) to prevent relapse

Lemierre's syndrome represents a serious complication of what initially appears to be routine pharyngitis, highlighting the importance of vigilance and prompt intervention in cases of persistent or worsening oropharyngeal infections.