Lemierre syndrome is the late sequelae of bacterial infection with anaerobic bacteria in the throat, such as the pathogens that cause tonsillitis. The disease leads to phlebitis and periodic septic emboli. If diagnosed early, treatment is with high-dose broad-spectrum antibiotics, which is combined with the administration of anticoagulants in later stages.
What is Lemierre syndrome?
Bacterial infections of the oropharynx can cause foci of inflammation in the acute phase. However, many bacterial infections may continue to cause symptoms long after these acute symptoms have resolved, that is, they may be associated with late symptoms. Lemierre’s syndrome is one such late consequence of bacterial infections of the mouth and throat. A simply transmitted infection, i.e. one that has not broken out, can also be associated with the disease. The syndrome is also known as necrobacillosis, postanginal sepsis, postanginal septicemia, or postanginal sepsis. Sepsis is a systemic inflammatory response that affects the entire body. In most cases, Lemierre’s syndrome manifests as purulent phlebitis preferentially in the jugular vein and results in periodic septic emboli. In 1900, P. Courmant and A. Cade first described the basis of an infection of the oral pharynx recognized as a precipitating factor of subsequent sepsis. The name Lemierre syndrome is derived from the French physician André Alfred Lemierre, who published a related description and case study of 20 patients in 1936.
Causes
The cause of sepsis as defined by Lemierre’s syndrome is infection with anaerobic bacteria. Peritonsillar abscess is most commonly associated with the syndrome. Most often, young, apparently healthy adults are affected by abscess formation in the tonsil area. Anaerobic bacteria such as Fusobacteria require no oxygen to multiply in abscesses and penetrate the jugular veins via the abscess in Lemierre’s syndrome. Local inflammation sets in and blood clots form, which can cause jugular vein thrombosis and support the spread of bacteria through the blood system. In the periphery, blood vessels thus occlude, causing septic embolism. In principle, all anaerobic bacteria can cause the syndrome. It is not the direct result of a bacterial infection in the acute stage, but rather the result of a spread infection with anaerobic bacteria.
Symptoms, complaints, and signs
In the early stages, patients with Lemierre syndrome suffer from tension in the neck and sore throat. Within a short time, lethargy develops with severe episodes of fever and swollen neck lymph nodes. By then, the symptoms resemble those of a severe flu. A few days or a week after the onset of the first symptoms, liver and kidney dysfunctions set in due to the proliferation of anaerobic bacteria. These symptoms are often associated with diarrhea, vomiting, and skin rashes. Progressive bacteremia leads to sepsis with systemic inflammatory reactions and febrile episodes. Blood clot formation in the vessels is a common occurrence, as bacterial infestation of the veins increases the risk of thrombus formation. Pulmonary embolism is the most common complication of Lemierre’s syndrome in the late stages. Often, late-stage patients are in such poor condition that the pulmonary embolism is barely noticed. Of the 20 cases described by Lemierre, seven died as a result of the syndrome.
Diagnosis and course of the disease
The clinical picture of Lemierre’s syndrome resembles severe influenza and is therefore often treated too late with the necessary treatment. Because some infections of the heart valves are also associated with a similar course, making a timely and correct diagnosis is challenging. Crucial for the diagnosis of Lemierre’s syndrome is, above all, the combined observation of all clinical symptoms with the initiation of a blood culture. Laboratory tests may show initial signs of bacterial infection, such as elevated C-reactive protein or neutrophilia. Jugular vein thrombosis of Lemierre’s syndrome is detected by ultrasound, CT or MRI. The same is true for syndrome-associated thrombosis.Immediately after diagnosis, appropriate treatment must be started.
Complications
In most cases, Lemierre’s syndrome primarily results in the usual symptoms of influenza. Those affected suffer from fever and a cough. Furthermore, there may also be tension in the neck or back. Also the lymph nodes swell and the complaints develop thereby to a strong flu. Without treatment, Lemierre’s syndrome can also cause liver or kidney problems, so that in the worst case the affected person suffers from kidney failure. There are also rashes on the skin and sufferers have to deal with vomiting and diarrhea. It is not uncommon for a pulmonary embolism to occur, which can also lead to death if left untreated. The patient’s quality of life is considerably limited and reduced by Lemierre’s syndrome. In many cases, the symptoms of the syndrome are greatly underestimated, which is why treatment is often delayed. If treatment is initiated early, the symptoms can be well controlled. Consequential damage also does not occur, and Lemierre syndrome does not reduce the patient’s life expectancy.
When should you see a doctor?
Symptoms such as tension, sore throat, and flu signs indicate Lemierre syndrome. Medical attention is needed if symptoms develop into the flu. If other symptoms develop, medical advice is also needed. If the symptoms have not subsided after three days, the illness must be taken to a medical professional. If kidney or liver complaints develop, medical advice must be sought the same day. Otherwise, a painful rash may develop, accompanied by vomiting and diarrhea. In severe cases, a life-threatening pulmonary embolism may develop. Patients who have been diagnosed with a peritonsillar abscess are particularly at risk for Lemierre’s syndrome. Local inflammation is also one of the risk factors. Persons who belong to corresponding risk groups are best advised to speak to the responsible physician immediately. Pregnant women, children, as well as sick and elderly people should have worrying symptoms clarified quickly anyway and treated if necessary. In addition to the family doctor, Lemierre syndrome can go to a gastroenterologist and a dermatologist.
Treatment and therapy
If the diagnosis of Lemierre’s syndrome is made early enough, the physician may then initiate conservative treatment with high-dose broad-spectrum antibiotics. This treatment only strikes in the early stages of Lemierre’s syndrome and therefore depends on diagnosing the disease as early as possible. Physicians often treat all patients with sore throat and signs of bacteremia with high-dose broad-spectrum antibiotics, even if Lemierre syndrome has not yet been confirmed as a diagnosis. This procedure is necessary because delayed treatment of the syndrome in most cases has life-threatening consequences and for this reason requires intensive medical therapy. The later the syndrome is detected, the higher the mortality. If appropriate treatment is given in time, mortality is again less than five percent. To date, there have been hardly any long-term studies on the treatment of Lemierre’s syndrome. For this reason, it is currently unclear whether surgical removal of the thrombosed jugular vein is a viable treatment option. Similarly, it is not yet clear whether patients could benefit from lifelong administration of anticoagulant medication. Administration of anticoagulants has been performed at least in the late stages of the disease to prevent complications such as pulmonary embolism.
Outlook and prognosis
The prognosis for Lemierre syndrome is based on an assessment of disease progression. Although due to medical developments, treatment measures have improved considerably, fatal disease progression may still occur. To date, the mortality rate is 10 percent. If the diagnosis is made late, the bacterium has already spread widely in the organism and caused damage, some of which is irreparable. The body’s immune system is weakened and can no longer successfully defend itself against the pathogens. If treatment is started in the early stages of the disease, the prognosis is usually favorable. In a drug treatment, the spread of the bacteria can be prevented.In addition, bactericidal agents succeed in causing the death of the pathogen. Within a few weeks, the patient is normally discharged from treatment as recovered. An increased risk arises if pus forms in the lungs or neck. Surgical interventions are performed to prevent sepsis. Otherwise, the affected person may die due to blood poisoning. Each operation is associated with various risks and side effects. When making a prognosis, this fact must be taken into account. Normally, it is a routine operation, which proceeds without further complications under disturbances. Accordingly, the patient is discharged from the treatment as cured after wound healing.
Prevention
Lemierre syndrome cannot be fully prevented. However, professional treatment of ear, nose, and throat infections probably helps prevent it. In particular, the causative agents of tonsillitis are associated with the syndrome. Therefore, timely treatment and curing of such inflammations and infections should be considered preventive measures in the broadest sense.
Follow-up
Often, the measures of aftercare in Lemierre syndrome are very limited. In this case, the first priority should be to see a doctor at an early stage so that no further complications can occur. This can also prevent further worsening of the symptoms, so the affected person should see a doctor at the first symptoms or signs of Lemierre’s syndrome. In most cases, the disease can be treated relatively well with the help of various medications. This also does not lead to further complications. The affected person should pay attention to the correct dosage when taking the medication and furthermore to a regular intake in order to alleviate the symptoms permanently. Regular checks by a doctor are very important in order to monitor the symptoms permanently. There is very rarely a reduced life expectancy. When taking antibiotics, it should be noted that they are not taken together with alcohol, as their effect is otherwise reduced. Often, those affected by Lemierre’s syndrome need support and care from family and friends, which can have a positive effect on the further course of the disease.
What you can do yourself
Lemierre syndrome cannot be cured by everyday self-help, but alleviation of the accompanying symptoms is certainly possible. Affected individuals tend to be lethargic, which must be overcome. Above all, regular exercise is important, even if the symptoms make it seem difficult. However, regular mobilization prevents blood clots, which is why it should become routine. The usual home remedies, such as calf wraps or a cold rag on the forehead, help against fever episodes. Plenty of rest and darkness may also be found pleasant by sufferers. Furthermore, those affected suffer from diarrhea and vomiting. Accordingly, adequate fluid intake is important, otherwise there is a risk of dehydration. With regard to diarrhea, a change in diet is suitable as an everyday aid. Before important appointments, food intake can be avoided. In general, patients should switch to a light diet. At the beginning of the disease, there is often neck pain, for which warmth is often perceived as pleasant and soothing. The occurring sore throat can be combated with honey. Gentle teas are also recommended, but care should be taken that they do not dehydrate the body. In general, the symptoms are similar to those of flu. For this reason, everyday self-help strategies for flu illness also lend themselves to reducing accompanying symptoms of Lemierre’s syndrome.
