Lemierre's syndrome

Introduction: Lemierre's syndrome is a rare entity characterized by oropharyngeal symptoms (usually consisting of either pharyngitis, a peritonsillar abscess or both), local septic thrombophlebitis, bacteremia, and multiple metastatic abscesses frequently involving the lungs. This infection is caused by the Gram-negative anaerobic bacillus, Fusobacterium necrophorum. Despite the broad susceptibility of this organism, resistant isolates have been identified and mortality from this infection still occurs. In the setting of meningitis or multiple pulmonary abscesses, mortality can reach 30% despite appropriate antibiotics.

Case Presentation: A previously healthy 21-year-old male was referred to our institution after developing a sore throat, generalized malaise, nausea, arthralgias and myalgias. Initially, he was evaluated at a local clinic where he was noted to have a leukocytosis (12.3 x [10.sup.3] with 53% segmented neutrophils), a negative mononucleosis slide agglutination spot test and a nonreactive rapid streptococcal screen. He received intravenous saline and was given trimethobenzamide suppositories for nausea before being sent home. He was hospitalized less than 24 hours later with orthopnea, fevers to 39.8 degrees Celsius, and right-sided cervical adenopathy. He was tachycardic with an S4 and a 2/6 systolic ejection murmur at the apex. No carotid bruits were present. Decreased breath sounds were auscultated bilaterally with egophony and fremitus appreciated in the left lower lung field. Mild palpable tenderness was present in the right upper quadrant associated with hyperbilirubinemia (total/direct 5.3 mg/dL//4.3 mg/dL), and elevated liver function serologies (aspartate aminotransferase, 250 U/L, alanine aminotransferase 108 U/L, alkaline phosphatase 170 U/L). His white blood cell count had increased to 17.8 x [10.sup.3] with 80% segmented neutrophils and 11% band cells, and he was transferred to our facility after receiving intravenous ceftriaxone. A chest

radiograph revealed a left lower lobe infiltrate and bilateral pleural effusions (left side greater than the right). Chest computed tomographic imaging showed multiple peripheral nodular infiltrates and confirmed the left-sided pleural effusion. Thoracentesis produced an exudative fluid consistent with empyema (pH 7.1, ghlcose 23 mg/dL, lactate dehydrogenase 4,999 U/L and white cell count 25.4 x [10.sup.3] with 93% neutrophils) and subsequently a chest tube was inserted. Transthoracic and transesophageal echocardiograms revealed a tiny pericardial effusion, but showed no valvular vegetations and normal ventricular size and function with estimated ejection fraction of 60%. Abdominal ultrasound revealed no specific abnormalities, but a carotid doppler study demonstrated a thrombus in the right internal jugular vein. Blood cultures drawn locally then grew Gram-negative bacilli consistent with Fusobacterium necrophorum that were resistant to penicillin. The patient's antibiotic regimen was changed to ampicillin/sulbactam and metronidazole. Although the patient described a sore throat on admission, detailed imaging studies, dental examination and direct laryngoscopy failed to identify a specific oropharyngeal source for the infection. The patient responded quickly to intravenous antibiotic therapy and was discharged home on oral antibiotics on the ninth day after admission. After a total of 12 weeks of oral antibiotics, the patient achieved complete recovery and returned to work.

Discussion: Since the advent of antibiotics, Lemierre's syndrome is extremely uncommon in most clinical practices, causing some to call it the "forgotten disease." It is also known as "post-anginal sepsis" from the term "Vincent's angina," a painful membranous ulceration with edema and hyperemic patches of the oropharynx caused by spreading acute necrotizing gingivitis. It is probably more common than currently recognized, and with development of resistant bacteria, familiarity with the syndrome may be life-saving at some point in practice. Infections involving this anaerobe were first reported in veterinary literature in the late 1800s, and the first human infection was reported by Courmont and Cade in 1900. Lemierre was the first to summarize all that was known about the human form of the disease in 1936. Fusobacterium necrophormn is a slow-growing pleomorphic anaerobic Gram-negative bacillus that is found as a normal commensal in the body but may enter the circulation through the palatine tonsils. The illness typically occurs in a previously healthy teenager or young adult, beginning with pharyngeal inflammation, followed by recurrent fevers and rigors. The finding of jugular venous thrombophlebitis is the hallmark of the infection, implying an infectious source from the tonsillar veins. The most frequent site of infectious metastasis is the lung where multiple infarcts or nodules may be identified. Endocarditis, meningitis, hepatic abscesses and septic arthritis from this syndrome has been described previously in the literature. Because of its rarity, there is no standard treatment for this syndrome. F. necrophorum is susceptible to penicillin, but nearly half of all isolates produce [Beta]-lactamase and alternative antibiotics such as metronidazole given over extended periods (several weeks) have been recommended. Anticoagulants are not recommended for septic thrombi, and surgical debridement is indicated only for persistent abscesses.

Conclusion: Lemierre's syndrome involves sepsis caused by an anaerobic pharyngitis from the organism Fusobacterium necrophorum. Although it is rare in the antibiotic era, early recognition of oropharyngeal symptoms followed by prolonged fever, septic thrombophlebitis of the internal jugular veins, bacteremia, and frequent metastatic abscesses to the lungs and other organs is vital to minimize morbidity and mortality.

References

[1] Moller K, Dreijer B. Post anginal sepsis (Lemierre's Disease): A persistent challenge. Presentation of 4 cases. Stand J Infect Dis 1997; 29:191-194

[2] Lee BK, Lopez F, Genovese M, et. al. Lemierre's syndrome. South Med J 1997; 90:640-643

[3] Sinave CP, Hardy GJ, Fardy PW. The Lemierre syndrome: suppurative thrombophlebitis on the internal jugular vein secondary to oropharyngeal infection. Medicine 1989; 11:319-324

Clayton T. Cowl, MD, MS, J. R. Jett, MD--Division of Pulmonary & Critical Care Medicine, Mayo Clinic, Rochester, MN USA

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