Lemierre syndrome or Lemierre’s disease refers to thrombophlebitis of the jugular veins with distant metastatic sepsis in the setting of initial oropharyngeal infection such as pharyngitis/tonsillitis with or without peritonsillar or retropharyngeal abscess.
Lemierre’s syndrome is also called postanginal shock including sepsis and human necrobacillosis. It most often develops as a complication of a bacterial sore throat infection in young, otherwise healthy adults..
It is a serious condition and may lead to further systemic complications such as sepsis.
Lemierre’s syndrome was relatively common before the discovery of antibiotics. In the 1960s, however, antibiotics almost eliminated the disease. For this reason, Lemierre’s syndrome is sometimes called the “forgotten disease.” Fewer than 4 in every 1 million people develop Lemierre’s syndrome worldwide each year. However, cases have steadily increased since 1998.
It is named after Andre Lemierre who first described it in 1936.
Lemierre’s syndrome can affect a person at any age. However, people under 30 years old account for most cases and are more likely to develop the disease than older adults.
Most cases presented in the 2nd decade of life (51%), followed by the 3rd decade (20%) and then the 1st decade (8%).
There is no clear gender predilection.
Fusobacterium species, most commonly Fusobacterium necrophorum, are responsible for the majority of bacteremia in cases of Lemierre’s syndrome.
Over one third patients would have a polymicrobial bacteremia formed by anaerobic streptococci and other miscellaneous gram-negative anaerobes.
Metastatic infections following the internal jugular thrombophlebitis occur in 63%-100% of patients The lungs are by far the most common site of metastatic infection in Lemierre’s syndrome, followed by the major joints. Metastatic infections of the liver, muscle, pericardium, brain and skin have also been described.
Thrombosis may propagate from the IJ vein inferiorly into the subclavian vein or superiorly into the cavernous, sigmoid or transverse sinuses. Meningitis may also complicate up to 3% of cases.
Any person with a sore throat or upper respiratory infection runs a slight risk of developing Lemierre syndrome. Several cases of Lemierre’s Syndrome have occurred in people with strep throat or infectious mononucleosis.
Pathophysiology and Causes
Lemierre’s syndrome occurs most often when a bacterial (e.g., Fusobacterium necrophorum) throat infection progresses to the formation of a peritonsillar abscess. As the anerobic bacteriae flourish, the abscess ruptures internally, the drainage carrying bacteria seeps through the soft tissue and infects the nearby structures.
Spread to the nearby internal jugular vein provides a gateway for the spread of bacteria through the bloodstream. Thrombophlebitis of internal jugular vein occurs leading to blood clot formation. Pieces of the potentially infected clot can embolize blocking branches of the pulmonary artery.
F. necrophorum is an anaerobic gram-negative anaerobic rod that is part of the normal flora of the oropharynx. The factors for becoming invasive pathogen are not known.
Some authors have proposed an alteration in the pharyngeal mucosa by a viral or bacterial pharyngitis might playing a role. In up to one third of patients polymicrobial bacteremia is demonstrated, anaerobic streptococci and other miscellaneous gram-negative anaerobes are also found frequently. Reports contain methicillin-resistant Staph. aureus (MRSA) as well.
The palatine tonsils and peritonsillar tissue are the primary sources of infection in most cases.
Other primary sources of infection include the lungs, middle ear, mastoid, teeth and sinuses. Following the primary infection, there is local invasion of the lateral pharyngeal space and septic thrombophlebitis of the internal jugular vein.
Patients typically present unwell, with trismus[difficulty in opening mouth] and pain behind the angle of the jaw. Neck swelling may be present.
Early symptoms of Lemierre’s syndrome may appear similar to common viral infections, as they can include a sore throat, fever, and muscle weakness.
Sore throat is the most common symptom and typically precedes all other symptoms by 4–5 days [ up to 12 days]. Some patients will have complete resolution of pharyngitis symptoms prior to the onset of symptoms from the IJ thrombophlebitis or metastatic infection.
However, a sore throat may be particularly concerning if it does not improve with antibiotic medication.
The neck pain is typically unilateral and may be aggravated by turning the head away from the involved side as a consequence of irritation of the sternocleidomastoid muscle
Lungs are the most common site of metastatic infection, chest pain and pulmonary complaints are the most consistent indicators of metastatic disease.
The common presenting symptoms of Lemierre’s syndrome are
- Pleuritic chest pain – pain during breathing
- Neck mass
- Neck pain
- Ear pain
- Bone/joint pain
- Dental pain
- Abdominal pain
On examination, fever is the present in most of cases. There might be signs of pharyngitis or peritonsillar abscess. A mass in the neck may be palpable at the angle of the jaw or along the anterior margin of the sternocleidomastoid muscle.
Jaundice may be seen in 11%-49% with Lemierre’s syndrome will be jaundiced.
Without treatment, sepsis develops. Sepsis is suggested by fever above 101°F, dyspnoea, dizziness, increased heart rate and mental confusion. Patient looks ill and fatigues.
As sepsis advances, hallucinations, incoherent speech, irregular pulse and decrease in urine volumes and low blood pressure occurs.
Shock is a late finding in the disease process and a predictor of mortality.
Apart from generalized sepsis, pneumonia and meningitis features could be present marking the spread of infection.
Septic arthritis (most commonly hip or knee), pharyngitis/peritonsillar abscess are also seen.
Jaundice could be present.
Palsy of 10, 11, 12 cranial nerves may be present.
This illness should be suspected when any of the following are true
- Pharyngitis that does not resolve in 3 to 5 days
- Pharyngitis followed by systemic or respiratory symptoms such as fever, chills, rigors or dyspnea
- Pharyngitis associated with lateral cervical pain and dysphagia
- Pharyngitis followed by sepsis or multiple pulmonary abscesses.
Basic blood investigations like CBC and ESR would show presence of infection. Blood is sent for culture of anerobic bacteriae.But it may take from 2 to 7 days to get the complete picture] and, may be suppressed by the previous administration of antibiotics.
A contrast computed tomography scan or an ultrasound to check for a thrombophlebitis can be considered. Often this finding is the first evidence of Lemierre syndrome. Contrast-enhanced CT allows for visualization of surrounding structures and is the most readily available. It is the most preferred study and considerd gold standard. CT shows an intraluminal filling defect in the jugular venous wall, frequently superior to ultrasound due to better assessment of deeper venous segments, depict sites of septic emboli, most often encountered as pulmonary septic emboli.
Ultrasound may show thrombus within the jugular vein or other neck or facial veins.
A chest X-ray may also be used to identify whether the infection has spread to the lungs. Lung lesions typically begin as multiple, usually nodular infiltrates that progress rapidly to cavitary lesions and are frequently accompanied by pleural effusions.
Arthrocentesis may be considered in presence of septic arthritis.
Prolonged antibiotic therapy constitutes the mainstay of treatment of Lemierre’s Some authors have recommended against the use of monotherapy with metronidazole because of the frequent occurrence of mixed infection with other oral flora.. The duration of antibiotic therapy should be from 2 to 6 weeks.
The choice of antibiotics should be based on in vitro studies of the organism and drug sensitivity
- Ampicillin – sulbactam
- Ticarcillin – clavulanate
- Penicillin plus metronidazole
The use of anticoagulation is controversial, and there is limited evidence for their use.
Surgery for drainage of abscesses like neck abscess, peritonsillar or lateral pharyngeal abscesses may be required.
Abscesses may also develop in the neck, head, lungs, or brain.
Septic Arthritis should be drained.
A patient who receives medical advice in time have a high survival rate. Relief from symptoms may begin after several days of antibiotics with full recovery taking a few weeks.
The people who do not seek advice or ignorethe early symptoms may run into serious complications and have dismal prognosis.
Mortality was over 90% before antibiotics. It has been brought down by antibiotics but is reported as high as 18% in some reports/
Disseminated intravascular coagulation has been reported in up to 9% of cases
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