Lemierre syndrome is an uncommon but life-threatening condition defined by oropharyngeal infection leading to internal jugular vein thrombophlebitis with evidence of septic emboli1,2
The condition is most often seen in patients younger than 40 years, with a reported incidence of 3.6 cases/million population-years and a mortality rate of 4% to 9%, even with treatment.2,3 Overall, 6% to 19% of cases are culture negative, and Fusobacterium species remain the most common isolate in 58% to 86% of cases.1,4 Increasingly, non-Fusobacterium organisms such as Streptococcus, Staphylococcus, and polymicrobial infections are also recognized.1,4
Patients typically present with sore throat and fever but may have red-flag symptoms of neck swelling, pleuritic chest pain, and dyspnea5
Pulmonary manifestations of septic emboli are seen in 92% to 97% of patients with Lemierre syndrome, and untreated patients deteriorate rapidly owing to septic shock and respiratory failure.1,5
Patients with a compatible history and red-flag symptoms should be evaluated in the emergency department, and those with a confirmed diagnosis should be admitted to hospital
Key investigations include 2 sets of blood cultures and imaging of the neck and chest.1,2,4 Neck ultrasonography or computed tomography (CT) with contrast is used to confirm jugular vein thrombosis, whereas CT pulmonary angiography identifies pulmonary embolism, necrotizing pneumonia, or empyema.5
Patients with Lemierre syndrome require early antibiotic therapy and consultation with infectious disease specialists
Patients should be treated with empiric therapy to cover β-lactamase-producing organisms until susceptibilities are known, typically beginning with intravenous piperacillin–tazobactam (4.5 g every 8 hours).5 Prolonged antibiotic duration and procedural source control are often required. Depending on the location of septic emboli, incision and drainage of abscesses or chest tube insertion with or without fibrinolytics for empyema may be necessary.2,5
Anticoagulation should be considered for patients with extensive or symptomatic thrombophlebitis
Decisions regarding anticoagulation should be made on a case-by-case basis with hematology consultation. Retrospective studies have not definitively shown that anticoagulation improves outcomes or reduces recurrent thromboembolism, and patients often face increased bleeding risk from necrotizing infection, thrombocytopenia, and required source control procedures.2,5
Footnotes
Competing interests: Siraj Mithoowani reports receiving authorship royalties from UpToDate, payment for medicolegal expert testimony from the Canadian Medical Protective Association, and an honorarium from CSL Behring. No other competing interests were declared.
This article has been peer reviewed.
References
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