Lemierre�s Syndrome

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					Lemierre’s Syndrome

     Ryan Sanford
       11.16.08
                        History
• Human disease named after French physician
  Andre Lemierre
  – 18/20 of his cases died
• Pathogen first recognized in veterinary medicine
  –   Calf diphtheria
  –   Stomatitis of calves, lambs, pigs
  –   Foot rot in cattle, sheep
  –   Lung and liver abscesses in cattle/pig
• Necrotic abscesses  ‘Necrobacillosis’
               Definition

    ‘…disseminated abscesses and
  thrombophlebitis of IJV after infection
     of the oropharynx … [caused by]
       Fusobacterium necophorum’

• AKA: postanginal sepsis; necrobacillosis;
  The Forgotten Disease
             Epidemiology
• 0.6-2.3 cases per million population
  – Relatively more common in pre-ABX era
  – Prevalence currently increasing
• M:F = 2:1
• Most common in teenagers, young adults
• Healthy, fit individuals
                            The Bug
• Fusobacterium necrophorum
    – Commensal in OP, GU, GI flora
    – Anaerobic GNRs.
    – Fastidious anaerobic agar  yellow colored
      colonies with cabbage odor

‘The diagnosis of this infection may
be suggested by the peculiar odor –
like Limburger or overripe
Camembert cheese – of pus
produced by it’
              Alston 1955
             Pathogenesis
• Fit young adult > ‘routine’ pharyngitis >
  breakdown in mucosal integrity > F.
  necrophorum invasion
• Toxin production
  – LPS endotoxin
  – Leukocidin
  – Platelet aggregation > thrombophlebitis and
    anaerobic environment
Pathogenesis
          Clinical Features
                            ‘Be not deceived by a
                           comparatively innocent
                          appearing pharynx as the
                          veins of the tonsil may be
                         carrying the death sentence
                                of your patient’.
                                            Hall 1939




‘…a syndrome that is so characteristic that
      mistake is almost impossible’ Lemierre 1936
IJV Septic Thrombophlebitis
  Thrombophlebitis of EJV




Pleural Effusions + Pulmonary
 Consolidation w/ Cavitation
Septic Emboli, Atelectasis,
     Effusions, ARDS
           Beyond Head and Neck
               Manifestations
• LUNGS [>97%]: multiple nodular infiltrates, pleural
  effusions, cavitation, rapidly progress
   – Empyema in 10-15%
   – ARDS in <10%
• BONE/JOINT:
   – Large joints  septic arthritis, arthralgias.
   – OM: 3% of cases
• SOFT TISSUE: infections up to 25%
• INTRA ABDOMINAL
   – Liver ~50% w/ abnormal LFTs, many jaundiced
   – Spleen/Liver: multiple abscesses, less common
   – Peritonitis rare
          Beyond Head and Neck
              Manifestations
• CNS:
  – Retrograde propagation to cranial sinuses from IVJ thrombosis
  – Purulent meningitis, rare
  – CN palsies
• CV:
  – IE uncommon
  – Septic shock requiring inotropic support rare
• Renal
  – AKI/ARF requiring RRT <5%
• Heme
  – TCP often
  – DIC uncommon
Dr. Lemierre’s Thoughts
                   Signs
• IJV Thrombophlebitis: U/s, CT, MRI
• CXR: nodules, cavitation, nonspecific
  consolidation
• CT Chest: peripheral nodules +/- cavitation
• ↑WBC w/ L Shift, ↑CRP
• Abnormal LFTs
• Cx: F necrophorum grows in 48h – 7d.
  – Often misdiagnosed as Bacterioides sp.
                Treatment
• Slow response to ABX
  – Defervesce in 8-12d
    • Sequestered organisms in abscesses, thrombus
• ABX
  – Resistant to AMGs
  – FQs poor activity
  – Macrolides resistant in ~25%
  – Best choices are PCN [w/ β-Lactamase
    inhibitor], Clindamycin, Metronidazole
                     Treatment
• No RCTs to guide treatment
• Most recommend Metro + a PCN
  – All advise tailoring therapy to clinical situation
  – No consensus on duration
• Anticoagulation?
• Abscess drainage
• ~5% Mortality
Prognosis
      Lemierre’s and Pharyngitis
            ‘The Forgotten Disease’
• PMDs discouraged to use ABX in routine
  pharyngitis could contribute to the increased
  incidence of Lemierre’s
• Emerging macrolide resistance?
• Improved ability to culture anaerobes
               Who Gets ABX?




‘If we physicians are going to reserve antibiotic use in head
  and neck infections we must be aware of the lessons the
pre-antibiotic era taught us. Lemierre’s syndrome should be
  remembered as a deadly but preventable complication of
                          pharyngitis’.
                       References
1.   Lemierre A. On Certain Septicaemias Due to Anaerobic
     Organisms. Lancet 1936;1:701-703
2.   Syed MI. Lemierre Syndrome: Two Cases and Review.
     Laryngoscope, 117:1605-1610, 2007
3.   Alston JM. Necrobacillosis in Great Britain. BMJ 1955;II:1524-8.
4.   Hall C. Sepsis following pharyngeal infections. Ann Otol Rhinol
     Laryngol 1939;48:905-25.
5.   Clinton L. Lemierre’s Syndrome. NEJM 2004; 350:16, e14.
6.   Riordan T. Lemierre’s Syndrome: more than a historical curiosa.
     Postgrad Med J;80:328-334
7.   Jankowich, M. et al. Chest 2007;132:1706-1709
8.   Sinave CP. The Lemierre Syndrome: Suppurative
     Thrombophlebitis of the Internal Jugular Vein Secondary to
     Oropharyngeal Infection. Medicine. 68:2;1989 85-94
9.   Chirinos JA. The Evolution of Lemierre Syndrome. Medicine 81:
     458-65; 2002.

				
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