The “Forgotten Disease” Remembered: Lemierres’s Syndrome and the Severe Complications

Kareem Niazi, MD*, Hofstra North Shore-Long Island Jewish School of Medicine, Manhasset, NY and Alex Makhnevich, MD, Hofstra North Shore - Long Island Jewish School of Medicine, Manhasset, NY

Meeting: Hospital Medicine 2016, March 6-9, San Diego, Calif.

Abstract number: 711

Categories: Adult, Clinical Vignettes Abstracts

Keywords: , , ,

Case Presentation: A previously healthy 27 year old female was admitted with a severe cough and pleuritic chest pain after being treated as an outpatient for tonsillitis/pharyngitis 3 weeks prior to admission.  Her initial outpatient complaint was sore throat, which was thought to be viral.  However, after symptoms did not improve she was treated with a course of antibiotics and subsequently with oral steroids.  On presentation her chief complaints were a severe cough for a week, pleuritic chest pain, and odynophagia.  She was afebrile, BP 130/80, HR 90, RR20 with 100% SpO2 on RA. On exam she had decreased breath sounds on the left, and a tender neck on palpation.  Labs revealed a WBC of 30.7 with 5% bands and negative blood cultures. CT of the chest and neck revealed a large loculated left sided empyema containing numerous foci of gas and a right internal jugular (IJ) vein thrombus from skull base to the level of the thyroid gland.  She underwent surgical exploration of the neck that was negative for abscess and received a chest tube.  Pleural fluid grew Fusobacterium necrophorum that confirmed the diagnosis of Lemierre’s Syndrome.  After resolution of the empyema, her chest tube was pulled and she was discharged home with IV ertapenem for 4 weeks and aspirin 81mg daily, with outpatient follow up. 

Discussion: Coined the “forgotten disease” after the discovery of penicillin, Lemierre’s Syndrome refers to a thrombophlebitis of the internal jugular vein typically after bacterial tonsillitis/pharyngitis. Symptoms start with a sore throat and fever, then progress to a swollen, tender, and typically unilateral neck pain.  An increase in incidence has been reported, which may reflect antibiotic resistance or changes in antibiotic prescription patterns.  If patients are not treated early, the bacterial oropharyngeal infection fusobacterium, results in a suppurative thrombophlebitis of the IJ vein which gives rise to septic emboli, most often affecting the lungs. F. necrophorum has also been shown to produce hemagglutinin, causing platelet aggregation that can lead to diffuse intravascular coagulation and thrombocytopenia.  Early symptoms are nonspecific, thus patients presenting to the hospital after failed outpatient therapy need a high index of suspicion and prompt imaging with appropriate cultures.  Despite the present era of antibiotics, due to severe complications, mortality has been reported in about 5% of cases.  Treatment consists of long-term IV antibiotics and appropriate management of complications.  The role of anticoagulation remains controversial.

Conclusions: An increasing incidence of this albeit rare disease mandates a high index of suspicion for the hospital physician who admits a formally healthy young patient with symptoms of neck pain, fever and a recent pharyngitis.  Due to the morbidity and mortality associated with this condition, this is one entity that should never be forgotten.

To cite this abstract:

Niazi K, Makhnevich A. The “Forgotten Disease” Remembered: Lemierres’s Syndrome and the Severe Complications. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 711. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/the-forgotten-disease-remembered-lemierress-syndrome-and-the-severe-complications/. Accessed July 24, 2019.

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