Lemierre’s Syndrome: A Rare Complication of Upper Respiratory Infections With Thrombophlebitis

Introduction:

Lemierre’s syndrome is a rare but serious complication with significant morbidity and mortality if not diagnosed and treated initially. A French physician and bacteriologist, Andre Lemierre, first described this syndrome in 1936 and named it after him. The condition can be very severe, but the survival rate can increase with timely diagnosis and prompt treatment. Lemierre’s syndrome is rare because of the extensive use of Penicillin, but it was widespread before the invention of antibiotics. Fortunately, with the advent of antibiotics, Lemierre's syndrome is rarely encountered—about one in a million.

What Is Lemierres Syndrome?

Lemierre’s syndrome is rare but can be potentially life-threatening, primarily from a sore throat. Lemierre’s syndrome is mainly caused by an acute primary oropharyngeal infection leading to the secondary development of thrombophlebitis (an inflammatory process causing a blood clot to block one or more veins) of the internal jugular vein (major blood vessel in the neck and head region) with subsequent septicemia and blood clot. It is also known as postanginal septicemia or necrobacillosis.

The lung is one of the most commonly affected organs in this syndrome. The causative species of Lemierre’s syndrome are Fusobacterium, specifically Fusobacterium necrophorum, but various bacteria have been implicated. In rare cases, it is reported that Gram-negative bacteria, like Peptostreptococcus spp., Bacteroides fragilis and Bacteroides melaninogenicus, Streptococcus microaerophile, Streptococcus pyogenes, Staphylococcus aureus, and Eikenella corrodens can also cause Lemierre’s syndrome.

What Is the Pathophysiology of Lemierres Syndrome?

Lemierre's syndrome typically begins with a bacterial infection of the upper respiratory tract, most commonly staphylococcal or streptococcal pharyngitis. In rare cases, the infection can begin in the ears, nose, or other sites inside the head.

Lemierre's syndrome occurs when the common sore throat develops into serious infections and causes complications. Following the initial upper respiratory tract infection, the causative microorganisms mainly Fusobacterium necrophorum can invade and spread to adjacent tissues, leading to the formation of abscess in the neck region. The infection progresses and spreads to the internal jugular vein (a major blood vessel in the neck). As the infection progresses, septic thrombophlebitis occurs, forming a blood clot within the vein due to severe infection. This infected clot can break off and spread throughout the body, causing septic emboli (obstruction of a blood vessel by an infected blood clot) in distant organs, like the lungs. If proper attention is not given, this condition can culminate in severe sepsis with multiple organ dysfunction, making this a hazardous condition.

In about 90 percent of the cases, the infection is caused by Fusobacterium necrophorum, which is a bacteria naturally found in the human body, such as in the throat, stomach, and colon. However, the researchers are not clear how these bacteria spread and cause severe infection. One theory suggests a weak immune system makes spreading and moving around easier for bacteria. Also, the misuse and overuse of antibiotics cause the bacteria to become more resistant and stronger.

What Are the Symptoms of Lemierres Syndrome?

The early signs and symptoms of Lemierre's syndrome usually begin with a sore throat, fever, fatigue, and muscle pain. Extreme lethargy, high fevers, swollen cervical lymph nodes, a painful and swollen neck, abdominal pain, nausea, vomiting, and diarrhea can follow it. If not properly treated, these symptoms will become severe within two weeks or several days and can spread to other organs or cause sepsis or meningitis.

Symptoms of pulmonary involvement:

• Shortness of breath.

• Cough.

• Painful breathing.

• Blood when coughed up.

• Clammy skin or excessive sweating.

• Wheezing.

Symptoms of sepsis:

• Increased rate of breathing.

• Incoherent speech.

• Low blood pressure.

• Irregular heartbeats.

• Decrease in urination.

• Stomach pain.

• Patches of discolored skin.

Symptoms of meningitis:

• Head swelling.

• Stiff neck.

• Seizure or stroke.

• Irritability or confusion.

• Sensitivity of the eyes to light.

How Is Lemierres Syndrome Diagnosed?

Lemierre's syndrome diagnosis is challenging, as it is rare, and the initial symptoms are nonspecific. So healthcare professionals should be very cautious and suspicious if the sore throat does not subside even after effective treatment. The following diagnosis procedure will be very handy in diagnosing Lemierre's syndrome:

1. Complete Blood Count - This blood test helps confirm the presence of harmful bacteria. The C-reactive protein, white blood cells, and erythrocyte sedimentation rate will be elevated. Also, there will be variations in the platelet count.

2. Blood Culture - This is important for identifying the causative pathogens and planning the appropriate antibiotic therapy. The sample can be collected from the blood, joint aspirates, or the infection site.

3. Imaging Tests - Thrombosis can be seen with sonography. However, an ultrasound will be more effective if the thrombosis has a low echogenicity or echogenicity like the flowing blood. Also, an MRI (magnetic resonance imaging) and CT (computed tomography) scan displace the thrombus of the intra-thoracic retrosternal veins effectively. Chest X-ray helps identify pleural effusion, nodules, abscesses, infiltrates, and cavitations.

What Is the Differential Diagnosis of Lemierres Syndrome?

The differential diagnosis of Lemierres syndrome includes

• Viral pharyngitis.

• Epstein–Barr virus.

• Streptococcal pharyngitis.

• Peritonsillar abscess.

• Retropharyngeal abscess.

• Diphtheria.

• Epiglottitis.

• Q fever.

• Pneumonia.

• Tuberculosis.

What Is the Treatment for Lemierres Syndrome?

After the diagnosis of Lemierre’s syndrome, the treatment should be immediate, as a delay can worsen the patient’s condition and progress into septicemia and organ dysfunction. The treatment should be a multifaceted approach that treats both the infection and its complications.

1. Antibiotics - Antibiotic therapy is the mainstay treatment for Lemmier’s syndrome, targeting the microorganisms involved. The courses of antibiotics should be taken for several weeks, and intravenous broad-spectrum antibiotics are provided instead of oral.

2. Anticoagulants (Blood Thinners) - In some cases, anticoagulants may significantly prevent clot extension and embolization. As there are complications like bleeding with the use of anticoagulants, in less severe cases of Lemierre’s syndrome that do not show signs of a prominent blood clot, antibiotics and supportive therapy without anticoagulation will work.

3. Surgery - Surgical drainage of abscesses may also be required to control the infection source.

Conclusion:

Lemierre’s syndrome is a life-threatening complication, but it is not common these days after the advent of antibiotics. An oropharyngeal infection and the formation of blood clots characterize it. Diagnosing this condition is challenging as the symptoms are not very specific. With the help of blood tests and cultures and using imaging tools such as CT or ultrasound, Lemierre’s syndrome can be easily identified. Also, immediate treatment with antibiotics and anticoagulants is needed to avoid complications.