Unusually Severe Pharyngitis in a 19-Year-Old Woman
A previously healthy 19-year-old woman presents with a sore throat that has become progressively more painful over the course of 1 week. She also has left-sided neck swelling, light-headedness, and intermittent headaches. She denies recent sexual activity.
A previously healthy 19-year-old woman presents with a sore throat that has become progressively more painful over the course of 1 week. She also has left-sided neck swelling, light-headedness, and intermittent headaches. She denies recent sexual activity.
Self-medication with throat lozenges provided no relief. Fever and profuse sweating subsequently developed, and the pain eventually became so severe that she was unable to eat.
PHYSICAL EXAMINATION
The patient is diaphoretic. She has bilateral tonsillar exudate, submental and submandibular lymphadenopathy, decreased active and passive range of motion of the neck with tenderness on the left side, and trismus. Results of cardiac, pulmonary, abdominal, and neurological examinations are unremarkable.
LABORATORY AND IMAGING RESULTS
Laboratory studies reveal leukocytosis (white blood cell count, 21,800/µL) with bandemia (band count, 18%). A mononucleosis spot test yields a negative result. A chest radiograph shows multiple bilateral nodular opacities. A CT scan of the neck reveals left internal jugular thrombosis, palatine tonsillitis, and peritonsillar
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CORRECT ANSWER: C
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The patient has Lemierre syndrome (choice C), one of the life-threatening complications of streptococcal pharyngitis. This case is a classic presentation of a rare disease that begins with a sore throat yet can be potentially fatal if not diagnosed and appropriately treated.
Lemierre syndrome, also known as postanginal sepsis, results from an acute (commonly streptococcal) pharyngitis with secondary septic thrombophlebitis of the internal jugular vein and subsequent metastatic spread throughout the body. The usual causative agent is the gram-negative anaerobe, Fusobacterium necrophorum.1,2
Diagnosis. A high degree of clinical suspicion is necessary because the reported annual incidence of the disease is less than 1 case per million in the general population. Although rare now, this syndrome was not uncommon in the preantibiotic era. The condition most frequently affects previously healthy young adults.3
The initial presentation consists of sore throat with fever, malaise, trismus, exudative tonsillitis, tender neck swelling, and cervical lymphadenitis. Differential diagnoses include infectious mononucleosis, viral pharyngitis, staphylococcal pneumonia, tuberculosis, and endocarditis. Definitive diagnosis is made by contrast CT scanning of the neck.1,2
The most common complications are septic pulmonary emboli and septic arthritis. Other possible sequelae are disseminated intravascular coagulation, renal failure, empyema, cerebrovascular accident, and meningitis resulting from retrograde propagation of the cranial sinuses. Lengthy hospitalizations are common, and mortality is about 5% even today.
Treatment. The mainstay of treatment is prompt initiation of parenteral antibiotics. Herein lies a clinical challenge because most upper respiratory tract infections are caused by viruses. Surgical resection or ligation of the jugular vein is reserved for cases of septic emboli that persist despite appropriate antibiotic therapy. Anticoagulation is controversial and rarely indicated.2
Differential diagnoses. A variety of other causes of pharyngitis are in the broad differential here, including infectious mononucleosis, viral pharyngitis, and tuberculous pharyngitis. Infectious mononucleosis (choice A) frequently presents with a systemic illness and significant exudative pharyngitis in this age-group, and it could easily be considered in this case. However, the toxicity, degree of local neck symptoms and signs, character of the leukocytosis, and negative mononucleosis spot test result strongly suggest an alternative diagnosis.4
Acute HIV seroconversion (choice D) also commonly causes a prodromal systemic illness accompanied by pharyngitis and lymphadenopathy, although the illness would never be this severe. Further, subsequent tuberculous pharyngitis, which can be quite severe, does not occur so acutely or subacutely; it takes time for the HIV to effect its immune suppression of lymphocytes.
THE TAKE-HOME MESSAGE:
When pharyngitis has an unexpectantly severe clinical course with local and systemic signs and symptoms, suspect an unusual complication, such as the potentially life-threatening Lemierre syndrome. Prompt, aggressive therapy is required in such cases.
Viral pharyngitis (choice B) is extremely common and on occasion can be very severe (eg, herpangina). However, the severity, secondary complications, and duration of this patient's illness are well beyond the scope of essentially any viral pharyngitis, and a more serious cause must be considered.
Outcome of this case. The patient had a stormy hospital course. A throat culture was positive for group C streptococci. On day 9, pain developed in the right hip. MRI and arthrocentesis revealed septic arthritis of the hip. An echocardiogram showed no evidence of endocarditis.
Therapy consisted of parenteral clindamycin and metronidazole. The patient remained febrile for 14 days, but her condition gradually improved and she was discharged on day 18. At day 32, she was afebrile, asymptomatic, and doing well.
References:
REFERENCES:1. Golpe R, MarÃn B, Alonso M. Lemierre's syndrome (necrobacillosis). Postgrad Med J. 1999;75:141-144.
2. Karkos PD, Asrani S, Karkos CD, et al. Lemierre's syndrome: a systematic review. Laryngoscope. 2009;119:1552-1559.
3. Riordan T, Wilson M. Lemierre's syndrome: more than a historical curiosa. Postgrad Med J. 2004;80:328-334.
4. Luzuriaga K, Sullivan JL. Infectious mononucleosis. N Engl J Med. 2010;362:1993-2000.
