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Lemierre’s syndrome (or Lemierre’s disease, also known as postanginal sepsis and human necrobacillosis) 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. Lemierre syndrome develops most often after a strep sore throat has created a peritonsillar abscess, a crater filled with pus and bacteria near the tonsils. Deep in the abscess, anaerobic bacteria (microbes that do not require oxygen) like Fusobacterium necrophorum can flourish. The bacteria penetrate from the abscess into the neighboring jugular vein in the neck and there they cause an infected clot (thrombosis) to form, from which bacteria are seeded throughout the body by the bloodstream (bacteremia). Pieces of the infected clot break off and travel to the lungs as emboli blocking branches of the pulmonary artery bringing the heart’s blood to the lungs. This causes shortness of breath, chest pain and severe pneumonia. Fusobacteria are normal inhabitants of the oropharyngeal flora. This is a very rare disease with only approximately 160 cases in the last 100 years.[1]
Sepsis following from a throat infection was described by Scottmuller in 1918.[2] 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.[3]
Fusobacterium necrophorum is the causative pathogen in most of the patients with Lemierre’s syndrome. One literature review reported that F. necrophorum had caused the disease in 81% of the patients, while in 11% of the patients with Lemierre’s syndrome the disease was caused by other Fusobacterium species.[4] Rarely Lemierre’s syndrome is caused by other (usually Gram-negative) bacteria, which include Bacteroides fragilis and Bacteroides melaninogenicus, Peptostreptococcus spp., Streptococcus microaerophile, Staphylococcus aureus, and Eikenella corrodens.[4][5]
Lemierre’s syndrome is initiated by an infection of the head and neck region. Usually this infection is a pharyngitis (which occurred in 87,1% of patients as reported by a literature review[6]), but it can also be initiated by an otitis, a mastoiditis, a sinusitis or a parotitis.
During the primary infection, F. necrophorum colonizes the infection site and the infection spreads to the parapharyngeal space. The bacteria then invade the peritonsillar blood vessels where they can spread to the internal jugular vein.[7] In this vein, the bacteria cause the formation of a thrombus containing these bacteria. Furthermore, the internal jugular vein becomes inflamed. This septic thrombophlebitis can give rise to septic microemboli[8] that disseminate to other parts of the body where they can form abscesses and septic infarctions. The first capillaries that the emboli encounter where they can nestle themselves are the pulmonary capillaries. As a consequence, the most frequently involved site of septic metastases are the lungs, followed by the joints (knee, hip, sternoclavicular joint, shoulder and elbow[9]). In the lungs, the bacteria cause abscesses, nodulary and cavitary lesions. Pleural effusion is often present.[6] Other sites involved in septic metastasis and abscess formation are the muscles and soft tissues, liver, spleen, kidneys and nervous system (intracranial abscesses, meningitis).[7]
Production of bacterial toxins such as lipopolysaccharide leads to secretion of cytokines by white blood cells which then both lead to symptoms of sepsis. F. necrophorum produces hemagglutinin which causes platelet aggregation that can lead to diffuse intravascular coagulation and thrombocytopenia.[10][11]
The symptoms vary per person, but usually start with a sore throat, fever, and general body weakness. These are followed by extreme lethargy, spiked fevers, rigors, swollen cervical lymph nodes and a swollen, tender or painful neck. Often there is abdominal pain, diarrhea, nausea and vomiting during this phase. These symptoms usually occur several days to 2 weeks after the initial symptoms.
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