Lemierre's syndrome

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Lemierre's syndrome
Classification and external resources
DiseasesDB 31108

Lemierre's syndrome (or Lemierre's disease) is a disease usually caused by the bacterium Fusobacterium necrophorum, and occasionally by other members of the genus Fusobacterium (F. nucleatum, F. mortiferum and F. varium etc.) and usually affects young, healthy adults. Lemierre syndrome develops most often after a strep sore throat has created a peritonsillar abscess, a crater filled with pus and bacteria near the tonsils. Deep in the abscess, anaerobic bacteria (microbes that do not require oxygen) like Fusobacterium necrophorum can flourish. The bacteria penetrate from the abscess into the neighboring jugular vein in the neck and there they cause an infected clot (thrombosis) to form, from which bacteria are seeded throughout the body by the bloodstream (bacteremia). Pieces of the infected clot break off and travel to the lungs as emboli blocking branches of the pulmonary artery bringing the heart's blood to the lungs. This causes shortness of breath, chest pain and severe pneumonia. Fusobacteria are normal inhabitants of the oropharyngeal flora. This is a very rare disease with only approximately 160 cases in the last 100 years.[1]

Sepsis following from a throat infection was described by Scottmuller in 1918.[2] However it was Andre Lemierre, in 1936, who published a series of 20 cases where throat infections were followed by identified anaerobic septicemia, of whom 18 patients died.[3]

Contents

[edit] Symptoms

The first symptoms are a sore throat, extreme lethargy, fever, and general body weakness, but after a week or two these symptoms are followed by a spiked fever, rigors, swollen cervical lymph nodes and septicemia (infection of the blood) which can cause complications in other parts of the body including abscesses of lung, brain, and other organs, kidney failure and also effects on liver and joints if untreated.

[edit] Treatment

Lemierre's syndrome is easily treated with antibiotics, but because sore throats are most commonly caused by viruses, for which antibiotic treatment is unnecessary, such treatment is not usual in the first phase of the disease. Lemierre's disease proves that, rarely, antibiotics are sometimes needed for 'sore throats'.[4] If a persistent sore throat, with the symptoms are found, physicians are cautioned to screen for Lemierre's syndrome. Fusobacterium necrophorum is generally highly susceptible to Beta-lactam antibiotics, metronidazole, clindamycin and third generation cephalosporins while the other fusobacteria have varying degrees of resistance to beta-lactams and clindamycin. The disease can often be un-treatable, especially if other negative factors occur,i.e. various diseases occurring at the same time, such as; meningitis, pneumonia etc.

[edit] Incidence

Lemierre's syndrome is currently a very rare disease, but was quite common in the early 20th century before the discovery of penicillin. The reduced use of routine antibiotics for sore throats by doctors may have increased the risk of this disease, with 19 cases in 1997 and 34 cases in 1999 reported in the UK.[4]. The incidence rate is currently 0.8 cases per million in the general population,[5] leading it to be termed the "forgotten disease".[6] The mortality rate was 90% prior to antibiotic therapy,[3] but is now generally quoted as 15% with proper medical treatment, although one series of cases reported mortality as low as 6.4%.[7]

[edit] References

[edit] Footnotes

  1. ^ O'Brien Sr, WT; Lattin GE Jr, Thompson AK (Sep 2006). "Lemierre syndrome: an all-but-forgotten disease". American Journal of Roentgenology 187 (3): W324. doi:10.2214/AJR.06.0096. 
  2. ^ Schottmuller H (1918). "Ueber die Pathogenität anaërober Bazillen" (in German). Dtsch Med Wochenschr 44: 1440. 
  3. ^ a b Lemierre A (1936). "On certain septicemias due to anaerobic organisms". Lancet 1: 701-3. 
  4. ^ a b UK Chief Medical Officer Update 29 Feb 2001 (CMO Update29 Feb 2001)
  5. ^ Sibai K, Sarasin F (2004). "[Lemierre syndrome: a diagnosis to keep in mind]" (in French). Revue médicale de la Suisse romande 124 (11): 693-5. PMID 15631168. 
  6. ^ Weesner CL, Cisek JE (1993). "Lemierre syndrome: the forgotten disease". Annals of emergency medicine 22 (2): 256-8. doi:10.1016/S0196-0644(05)80216-1. PMID 8427443. 
  7. ^ Chirinos JA, Lichtstein DM, Garcia J, Tamariz LJ (2002). "The evolution of Lemierre syndrome: report of 2 cases and review of the literature". Medicine (Baltimore) 81 (6): 458-65. PMID 12441902.