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Diseases

Genetic and Rare Diseases Information Center (GARD)

Lemierre syndrome


Other Names for this Disease
  • Lemierre postanginal sepsis
  • Necrobacillosis
  • Oropharyngeal infection leading to secondary septic thrombophlebitis of the internal jugular vein
  • Postanginal sepsis secondary to orophyngeal infection
  • Septic phlebitis of the internal jugular vein
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My daughter has been diagnosed with Lemierre syndrome. Please provide me with information.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Lemierre syndrome?

Lemierre syndrome is a rare and potentially life-threatening illness. The bacterium responsible for this disease is typically Fusobacterium necrophorum, although a wide variety of bacteria have been reported as causing the disease. 

The bacterial infection begins in the oropharynx then spreads through the lymphatic vessels. Following this primary infection, thrombophlebitis of the internal jugular vein (IJV) develops. The final phase of the disease occurs when septic emboli (pus-containing tissue) migrate from their original location in the body to various organs. The lungs are most commonly involved, however other sites may include the joints, muscle, skin and soft tissue, liver, and spleen. 

The symptoms of Lemierre syndrome include fever, sore throat, neck swelling, pulmonary involvement and joint pain. It is an uncommon disease that occurs in about one person per million per year. The disease primarily affects healthy young people before age 40.

Diagnosis of Lemierre syndrome rests on the presence of a blood clot (or clots) in the IJV and blood cultures that show the presence of Fusobacterium necrophorum. Intravenous antibiotics are the mainstay of treatment. 
Last updated: 6/9/2015

What causes Lemierre syndrome?

In about 90% of cases, Lemierre syndrome is caused by Fusobacterium necrophorum; however, the syndrome has also been reported with other bacteria, including Stapylococcus aureus and Streptococcus pyogenes.[1]
Last updated: 11/12/2008

What are the symptoms reported in children who have Lemierre syndrome?

In children and adolescents, Lemierre syndrome usually begins with a severe sore throat, persistent fever, and possibly chills. Some cases begin with acute otitis media. As the syndrome progresses, there is neck pain and tender swelling along the internal jugular vein. If undiagnosed, the next stage is the "metastasis" of septic emboli to the lungs, abdominal organs, brain or heart. Lung involvement typically results in a productive cough (a cough that brings up mucus or phlegm) and chest pain. Girls may report abdominal pain and have enlargement of the liver (hepatomegaly) and jaundice, all of which indicate involvement of the liver.[1]
Last updated: 2/17/2009

How is Lemierre syndrome diagnosed?

After performing blood cultures and complete blood counts, contrast computed tomography (CT) of the neck provides the definitive diagnosis. Ultrasound can also confirm internal jugular vein thrombosis.[1]
Last updated: 11/12/2008

How is Lemierre syndrome treated?

Most cases of internal jugular thrombophlebitis can be managed medically without the need for surgery of the infected vein. Prolonged courses of intravenous antibiotics (3 to 6 weeks) is usually required. Anticoagulants have sometimes been used, but efficacy is unconfirmed. Surgery of the internal jugular vein may be required only in the rare patient who fails to respond to antibiotic treatment alone.[1]
Last updated: 11/12/2008

Why do several recent reports indicate a rise in the number of cases of Lemierre syndrome?

Possible explanations for the reported rise might be: (1) a decrease in antibiotic prescription, (2) more selective use of antibiotics, and/or (3) prescription of antibiotics that are not as effective against an infection with Fusobacterium necrophorum.[2]
Last updated: 11/12/2008

How can I find additional information and supportive resources on Lemierre syndrome?

To locate information and supportive resources on Lemierre syndrome, click here.
Last updated: 11/12/2008

References
  • Schwartz RH. Chapter 30: Infections Related to the Upper and Middle Airways. In: Long SS, Pickering LK, Prober CG. Long: Principles and Practice of Pediatric Infectious Diseases, 3rd ed. PA: Churchill Livingstone, An Imprint of Elsevier; 2008;
  • Aspesberro F, Siebler T, Van Nieuwenhuyse JP, Panosetti E, Berthet F. Lemierre syndrome in a 5-month-old male infant: Case report and review of the pediatric literature. Pediatr Crit Care Med. 2008;
  • Srivali N, Ungprasert P, Kittanamongkolchai W, Ammannagari N. Lemierre's syndrome: An often missed life-threatening infection. Indian J Crit Care Med. 2014 Mar; 18(3):170-2. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3963201/. Accessed 6/9/2015.
  • Gupta N, Kralovic SM, McGraw D. Lemierre syndrome: not so forgotten!. Am J Crit Care. 2014 Mar; 23(2):176-9. http://ajcc.aacnjournals.org/content/23/2/176.long. Accessed 6/9/2015.
Other Names for this Disease
  • Lemierre postanginal sepsis
  • Necrobacillosis
  • Oropharyngeal infection leading to secondary septic thrombophlebitis of the internal jugular vein
  • Postanginal sepsis secondary to orophyngeal infection
  • Septic phlebitis of the internal jugular vein
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.