Also known as post angina sepsis, it is an infectious syndrome.
It is a rare and life-threatening complication of bacterial infections that generally affects healthy adolescents and young adults.
Most often, it develops in association with a bacterial throat infection but can grow in association with a condition affecting the ears, salivary glands ( mumps ), sinuses, or teeth, or in association with a disease of the Epstein-Barr.
In people with Lemierre syndrome, the initial infection spreads to the neck’s deep spaces, leading to a transfer clot (septic thrombophlebitis).
It is sometimes made up of pus in the internal jugular vein (the blood vessel that carries blood to the brain, face, and neck).
The infected clot then circulates in the blood (septicemia), causing the infection to also spread to the lungs (most commonly), the skeletal system, and other parts of the body such as the spleen, liver, kidney, the heart, or the brain.
This can lead to life-threatening complications, such as respiratory distress syndrome due to a pulmonary embolism (blood clots in the lung), damage to other affected organs, and septic shock (in about 7 percent of cases).
Because most throat infections in young, healthy people do not cause serious health problems, diagnosis and treatment can be delayed.
Causes of Lemierre’s syndrome
Most of the time, Lemierre’s syndrome is due to a complication of a bacterial throat infection. Still, it has also been reported to be due to conditions that affect other areas of the head and neck, such as the ears, salivary glands, sinuses, and teeth.
The most common culprit for Lemierre’s syndrome is Fusobacterium necrophorum. This is the primary pathogen and is a pleomorphic, anaerobic gram-negative rod.
This bacteria is usually present in healthy people in various body parts (including the throat, digestive tract, and female genitalia).
Bacteria can cause invasive disease by releasing toxins into the surrounding tissue.
It has also been speculated that in some cases, other bacteria or a virus may be responsible for the initial infection before the onset of Lemierre’s syndrome, leading to conditions that favor the growth and invasion of F. necrophorum into the surrounding tissues.
Bacteria other than F. necrophorum that have been reported in case studies include Streptococcus species, Bacteroides species, Staphylococcus aureus, and Klebsiella pneumonia.
The Epstein-Barr virus has also been reported in people before the onset of Lemierre’s syndrome.
Symptoms of Lemierre’s syndrome
Lemierre’s syndrome is an acute, rare, and life-threatening anaerobic oropharyngeal infection, the classic presentation of human necrobacillosis.
In addition to worsening symptoms of the initial infection, symptoms at this stage of the disease typically include persistent fever and chills (rigors), as well as pain, tenderness, and swelling of the throat and neck.
The primary infection is in the head in a young, previously healthy person who subsequently develops a persistent high fever and disseminated metastatic abscesses, often including septic thrombophlebitis of the internal jugular vein.
Lemierre’s syndrome is often complicated by septic pulmonary embolism and distant metastatic infections.
Lemierre syndrome diagnosis
Lemierre syndrome can be diagnosed based on signs and symptoms and various blood tests and imaging studies.
Blood tests may reveal various abnormalities that suggest the diagnosis, such as a high white blood cell count, a low platelet count, or some other evidence of clotting problems and abnormal liver or kidney function.
Blood cultures should be collected, and the results commonly indicate that the blood is infected with F. necrophorum bacteria, which often raises a high suspicion for Lemierre syndrome.
In some cases, blood cultures are harmful due to the difficulties associated with culturing anaerobic bacteria.
Imaging studies may include a chest x-ray, detecting septic emboli, abscesses, or other pulmonary complications.
Imaging studies to evaluate septic thrombosis of the internal jugular vein may include ultrasound, such as a CT scan of the neck with contrast, and magnetic resonance imaging.
Magnetic resonance venography has the highest sensitivity for detecting internal jugular thrombosis.
Lemierre syndrome treatment
Treatment recommendations for Lemierre syndrome are primarily based on clinical experience and in vitro studies and are supported by limited data from observational studies and case reports.
The main treatment components include intravenous antibiotic therapy, therapy, and drainage at the injection sites.
A combination of antibiotics (at least one beta-lactamase resistant antibiotic and one beta-lactam antibiotic) is recommended to cover all possible responsible bacteria, as there have been reports of treatment failure with single antibiotics (monotherapy).
Intravenous antibiotics are indicated. F. necrophorum is usually susceptible to penicillin, cephalosporins, metronidazole, clindamycin, tetracyclines, and chloramphenicol. Some strains of F. necrophorum producing beta-lactamases have been described.
Ideally, the specific antibiotics used should depend on the available culture results.
Antibiotic therapy is usually continued for up to 6 weeks to allow the drug to penetrate the infected clots.
The primary treatment consists of intravenous antibiotic therapy for several weeks. Still, surgery may be necessary when there is abscess formation, shortness of breath, or severe clotting in the chest or brain.
When patients have dyspnea, stridor, or an inability to manage secretions, an artificial airway should be established.
Airway obstruction is more likely to occur in submandibular space infections.
Surgically obtained specimens should be cultured aerobically and anaerobically.
For patients with peritonsillar abscess, high-dose intravenous penicillin is the therapy of choice.
These must be incised and drained to prevent spontaneous rupture, aspiration pneumonia, airway obstruction, or dissection of infection into the lateral retropharyngeal space.
Surgical removal of infected clots is generally reserved for cases where antibiotic therapy and drainage are ineffective.
About half of Ludwig’s angina cases in the submandibular space can be cured without surgical intervention.
Surgical treatment may also be necessary for respiratory problems due to pulmonary embolism, severe blood clotting in other parts of the body (particularly the brain), mediastinitis, or to remove dead, damaged, or infected tissues with the use of the debridement technique.
The role of the use of anticoagulants) for blood clots in Lemierre’s syndrome is uncertain and has been controversial.
While anticoagulation can help prevent new clots and complications associated with thrombosis, it can cause a high risk of bleeding and help spread infected material.
Lemierre’s syndrome without evidence of extensive clotting usually resolves with appropriate antibiotic treatment and does not require anticoagulation.
However, it may be recommended in severe or progressive cases with persistent sepsis, extensive clotting, spreading to the cerebral sinuses, and when a patient does not begin to improve within 72 hours of appropriate antibiotics or surgical treatment.
Lemierre syndrome prognosis
The long-term prognosis and likelihood of survival in people with Lemierre syndrome vary depending on the syndrome’s progression, but even with proper treatment, it is fatal in some cases.
Advanced Lemierre’s syndrome is a life-threatening condition. Current mortality (death from syndrome) is estimated to be between 5% and 18%, depending on the data source.
However, as the mortality in the pre-antibiotic. Reportedly the 90% era, the prognosis for people with Lemierre’s syndrome has improved significantly due to advances in antibiotic therapy and high-level intensive care.
Receiving the diagnosis as quickly as possible and starting appropriate treatment increases the chance of survival.
Lemierre syndrome complications
Severe complications of Lemierre syndrome include acute respiratory distress syndrome, septic shock, pneumonia, empyema, meningitis, osteomyelitis (bone infection), brain abscess, and vocal cord paralysis.
This infection can complicate a routine case of infectious mononucleosis.