Lemierre's syndrome
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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. Fusobacteria are normal inhabitants of the oropharyngeal flora. The infection leads to inflammation of and formation of a thrombus (blood clot) in the internal jugular vein. Many of the resulting symptoms are due to the septic emboli that break off from this initial area and travel through the blood to other areas of the body. Symptoms of Lemierre's syndrome include sore throat, extreme lethargy, fever, generalized body weakness, rigors, and swollen cervical lymph nodes. The mainstay of therapy for Lemierre's syndrome is antimicrobial therapy. Lemierre's disease is a very rare disease with only approximately 160 cases in the last 40 years.
Historial Perspective
Sepsis following from a throat infection was described by Scottmuller in 1918.[1] 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.[2]
Pathophysiology
Microscopic Pathology
- It is a nonmotile, filamentous, nonfusiform pleomorphic non-spore-forming obligate anaerobic gram negative rod.
- Normal microbiota of the oropharynx, GI tract, and genitourinary tract.
- It is not known why F. necrophorum penetrates mucosa in some patients.
- Reduced host defense (EBV); synergistic, pathogenic complex with other bacteria.
Differentiating Lemierre's Syndrome from Other Diseases
Lemierre's disease needs to be differentiated from the following diseases:
- Descending necrotizing mediastinitis
- Meningitis (mostly in children with otitis media)
- Beware false positive chlamydia PCR
Epidemiology and Demographics
Age
- Children: otitis media
- Young Adults: tonsillitis or peritonsillar abscess
- Adults: tooth infections
- All ages: mastoiditis, sinusitis
Diagnosis
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.
Physical Examination
Throat
Ipsilateral neck tenderness parallel with sternocleidomastoid muscle
Lungs
Metastatic abscesses mainly to lungs (85%), but also joints (26%)
Abdomen
Abdominal pain 2nd to microabscesses vs. thrombophlebitis of abdomino- pelvic veins
Medical Therapy
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'.[3] 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.
Antimicrobial Regimen
- Septic jugular thrombophlebitis (Lemierre's syndrome)[4]
- 1. Causative pathogens
- Fusobacterium
- Viridans and other streptococci
- Staphylococcus
- Peptostreptococcus
- Bacteroides
- Other oral anaerobes
- 2. Empiric antimicrobial therapy
- 2.1 Immunocompetent host
- Preferred regimen (1): Penicillin G 2–4 MU IV q4–6h
- Preferred regimen (2): Metronidazole 0.5 g IV q6h
- Preferred regimen (3): Ampicillin-Sulbactam 2 g IV q4h
- Preferred regimen (4): Clindamycin 600 mg IV q6h
- 2.2 Immunocompromised host
- Preferred regimen (1): Cefotaxime 2 g IV q6h
- Preferred regimen (2): Ceftizoxime 4 g IV q8h
- Preferred regimen (3): Piperacillin 3 g IV q4h
- Preferred regimen (4): Imipenem 500 mg IV q6h
- Preferred regimen (5): Imipenem 500 mg IV q6h
- Preferred regimen (6): Gatifloxacin 400 mg IV q24h
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.[3]. 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,[2] but is now generally quoted as 15% with proper medical treatment, although one series of cases reported mortality as low as 6.4%.[7]
References
- Shah SA, Ghani R (2005). "Lemierre's syndrome: a forgotten complication of oropharyngeal infection". Journal of Ayub Medical College, Abbottabad : JAMC. 17 (1): 30–3. PMID 15929523.
- Cheung WY, Bellas J (2007). "Case report: Lemierre syndrome presenting with fever and pharyngitis". American family physician. 75 (7): 979–80. PMID 17429891.
Footnotes
- ↑ Schottmuller H (1918). "Ueber die Pathogenität anaërober Bazillen". Dtsch Med Wochenschr (in German). 44: 1440.
- ↑ 2.0 2.1 Lemierre A (1936). "On certain septicemias due to anaerobic organisms". Lancet. 1: 701–3.
- ↑ 3.0 3.1 UK Chief Medical Officer Update 29 Feb 2001 (CMO Update29 Feb 2001)
- ↑ Hall, Jesse (2015). Principles of critical care. New York: McGraw-Hill Education. ISBN 978-0071738811.
- ↑ Sibai K, Sarasin F (2004). "[Lemierre syndrome: a diagnosis to keep in mind]". Revue médicale de la Suisse romande (in French). 124 (11): 693–5. PMID 15631168.
- ↑ Weesner CL, Cisek JE (1993). "Lemierre syndrome: the forgotten disease". Annals of emergency medicine. 22 (2): 256–8. PMID 8427443.
- ↑ 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.