Lemierre’s syndrome is a rare disease that occurs when a bacterial infection (usually a throat infection) spreads into the tissues and deep spaces within the neck and forms a blood clot in the jugular vein.

The infected clot then travels in the blood to other parts of the body, typically the lungs, which can cause life-threatening complications, notes the National Institutes of Health’s Genetic and Rare Disease Information Center (GARD).
In the era before the availability of antibiotics, the mortality rate for Lemierre’s was extremely high. According to an article published in July 2020 in Cureus, widespread use of antibiotics led to no cases being reported in the 1950s or 1960s, prompting Lemierre’s to be dubbed “the forgotten disease.”

That began to change in more recent decades, with studies showing about 0.8 to 1.5 million cases a year being reported worldwide between the years 1970 to 2007, per the Cureus article. Some theories for the reasons behind this uptick include increases in antibiotic resistance and changes in prescription patterns due to the awareness of this growing problem.

Today, Lemierre’s syndrome may not be as rare as once thought, and it's considered a reemerging infection. An article published in August 2020 in the Journal of Microbiology, Immunology, and Infection explains that Lemierre’s is more commonly seen in young adults, and doctors should consider the possibility of this disease in certain cases of persistent neck pain, deep neck infections, septicemia (a serious bloodstream infection), and other signs of Lemierre’s syndrome.

Causes and Risk Factors of Lemierre’s Syndrome

Lemierre’s syndrome is typically caused by Fusobacterium necrophorum, a bacteria that lives in the throat, digestive tract, and female genitals, according to GARD.

F. necrophorum may cause Lemierre’s syndrome by releasing toxins into the surrounding tissue. It’s also been suggested that in some cases, a different bacteria or virus may be responsible for an initial infection, which can then set the stage for Lemierre’s to develop. These possible initial viruses and infections include Epstein-Barr virus and streptococcal infection.

Risk Factors

Per the Journal of Microbiology, Immunology, and Infection, risk factors for Lemierre's syndrome include:
  • Being immunocompromised
  • Environmental conditions

Duration of Lemierre’s Syndrome

How long you are affected by Lemierre’s syndrome depends greatly on when you get a diagnosis and begin treatment and how far your disease has progressed. Typically, doctors will treat patients with antibiotics for up to six weeks.

For severe disease that has progressed, hospital admission to an intensive care unit may be necessary; according to StatPearls, the average length of hospitalization for Lemierre’s is approximately three weeks.

Complications of Lemierre's Syndrome

When an infected blood clot caused by Lemierre's syndrome travels in the bloodstream (septicemia), it can spread infection to the lungs, skeletal system, and other organs such as the spleen, liver, kidney, heart, or brain.

According to the Journal of Microbiology, Immunology, and Infection, other complications may include:
  • Pneumonia, lung lesions, abscesses, and pleural effusions (water around the lung)
  • Empyema
  • Epidural abscess (an abscess between your skull and your brain)
  • Brain abscess
Per GARD, serious complications may include:
  • Respiratory distress syndrome due to pulmonary emboli (blood clots in the lung)
  • Damage to other affected organs
  • Septic shock (in about 7 percent of cases)
  • Bone infection
  • Meningitis
According to the Journal of Microbiology, Immunology, and Infection, less common complications may include:
  • Soft tissue abscesses
  • Pyomyositis (bacterial infection of the skeletal muscle)
  • Abscesses in the spleen and liver
  • Endocarditis (inflammation of the heart’s inner lining)
  • Pericarditis (inflammation of the membrane surrounding the heart)
  • Renal abscess (kidney abscess)
  • In rare cases, eye complications

Related Conditions and Causes of Lemierre’s Syndrome

In addition to F. necrophorum, other viruses and infections linked to Lemierre’s syndrome include Epstein-Barr virus and streptococcal infection (strep), according to GARD.
Lemierre’s syndrome is also frequently mistaken for mononucleosis, notes Cureus.

Resources We Love

Genetic and Rare Disease Information Center (GARD)

Funded by the National Institutes of Health, this site lays out the key facts about Lemierre’s syndrome and other rare diseases, from causes to diagnosis to treatments.

StatPearls

This source from the National Center for Biotechnology Information (NCBI) offers information about how Lemierre’s syndrome can affect the body and why early diagnosis is important.

Editorial Sources and Fact-Checking

  • Lemierre Syndrome. Genetic and Rare Disease Information Center.
  • Dasari S, Jha P. A Systematic Review of Lemierre’s Syndrome With a Focus on Ophthalmologic Complications. Cureus. July 21, 2020.
  • Lee W, Jean S, et al. Lemierre's Syndrome: A Forgotten and Re-Emerging Infection. Journal of Microbiology, Immunology, and Infection. August 2020.
  • Allen B, Anjum F, et al. Lemierre Syndrome. StatPearls. December 4, 2020.

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