Lemierre’s syndrome (LS) is a rare disease characterized by septic thrombophlebitis of the internal jugular vein. LS usually starts with pharyngitis and is followed several days later by the development of fever, rigors, swelling and neck pain and in many cases evidence of other organ involvement. Mortality was 90% in Lemierre’s original series but is now much lower (7%) with effective antibiotic use.
A previously healthy 24 year old female with no significant past medical history presented to her primary physician with complaints of sore throat and fever. A throat culture was negative and she was diagnosis with a presumed viral infection. One week later she presented to the emergency department with significant shortness of breath. In the ED, she was in severe respiratory distress with a room air PaO2 of 44. She was intubated and transferred to the intensive care unit. Upon arrival at our ICU, the patient required 100% oxygen and high PEEP. Vital signs showed a temperature of 36.1, blood pressure of 100/38, and heart rate of 130. On physical exam, the neck showed no JVD, nuchal rigidity, swelling or adenopathy. There were crackles in both lung bases. Exam was otherwise unremarkable. The initial laboratory results were notable for an elevated white count of 15.8 with 49% bands, platelets of 31,000, and creatinine of 1.5. A chest x-ray showed bilateral alveolar infiltrates. The patient was initially treated for presumed severe community acquired pneumonia with Levoquin and vancomycin. Blood, urine and sputum cultures were obtained. Her clinical status deteriorated rapidly and she developed oliguric renal failure due to ATN, severe DIC, septic shock and possible TTP within 48 hours of hospitalization. She was treated with CVVH, plasmaphoresis and vasopressors. Her oxygenation continued to decline and she developed acute respiratory distress syndrome. A lung protective strategy was employed. On hospital day four, fusobacterium necrophorum was isolated from her admission blood cultures. This raised concern about Lemierre’s Syndrome so an ultrasound of her internal jugular veins was obtained. There was a clot in the right jugular vein, confirming the diagnosis of LS. A CT scan of her chest showed extensive septic emboli bilaterally. Clindamycin, Metronidazole and Lovenox were added to her regimen. Fortunately, her condition slowly improved and she was able to make a full recovery.
This case highlights several aspects of LS. As in our case, LS usually affects previously healthy adolescents and young adults. The usual causative organism of LS is f. necrophorum, a gram negative anaerobe which is part of the normal oropharyngeal flora. F. necrophorum is somewhat slow growing so cultures may not turn positive until 5 to 8 days. Spread to other organs is common, with the lung (85%) and joints (26%) being the most frequently affected. The heart and GI tract are occasionally involved. CXR findings can include nodules, which may cavitate, and pleural effusions. Our patient showed extensive lung involvement. As was seen in our patient, the neck exam may be unrevealing. Diagnosis is based upon appropriate imaging studies, usually either CT or ultrasound of the neck, although MRI is also sometimes used. Metronidazole, clindamycin and penicillins with anaerobic activity are the mainstays of treatment. Duration of treatment is variable, but is generally between 2-6 weeks. The role of anticoagulation in LS is uncertain but some authors advocate its use. Surgical ligation of the IJV was utilized prior to the use of antibiotics, however, such surgery is now rarely required. Severe DIC and ARDS, as in our patient, are very rare.
Lemierre’s disease generally affects healthy patient with significant morbidity resulting from the septic emboli.
G.L. Schumaker, None.