Lemierre's syndrome
Encyclopedia
Lemierre's syndrome is a form of thrombophlebitis
usually caused by the bacterium Fusobacterium necrophorum
, and occasionally by other members of the genus Fusobacterium (F. nucleatum, F. mortiferum and F. varium etc.) and usually affects young, healthy adults. Lemierre's syndrome develops most often after a sore throat caused by some bacterium of the Streptococcus
genus has created a peritonsillar abscess, a pocket filled with pus and bacteria near the tonsils. Deep in the abscess, anaerobic bacteria (microbes that do not require oxygen) like Fusobacterium necrophorum can flourish. The bacteria penetrate from the abscess into the neighboring jugular vein in the neck and there they cause an infected clot (thrombosis
) to form, from which bacteria are seeded throughout the body by the bloodstream (bacteremia). Pieces of the infected clot break off and travel to the lungs as emboli blocking branches of the pulmonary artery bringing deoxygenated blood from the heart to the lungs. This causes shortness of breath, chest pain and severe pneumonia
. Fusobacteria are a normal part of the oropharyngeal flora.
Sepsis
following from a throat infection was described by Scottmuller in 1918. However it was André Lemierre
, in 1936, who published a series of 20 cases where throat infections were followed by identified anaerobic septicemia, of whom 18 patients died.
s and a swollen, tender or painful neck. Often there is abdominal pain, diarrhea, nausea and vomiting during this phase. These symptoms usually occur several days to 2 weeks after the initial symptoms.
Symptoms of pulmonary involvement can be shortness of breath, cough and painful breathing (pleuritic chest pain
). Rarely there is hemoptysis
. Painful or inflamed joints (arthralgia
or arthritis
, respectively) exist when the joints are involved.
Septic shock
can occur which presents as hypotension
, tachycardia
, oliguria
and tachypnea
. Nuchal rigidity
, headache and photophobia
occur in case there is meningitis
.
Hepatomegaly
and splenomegaly
(enlarged liver and spleen, respectively) can be found, but are not always associated with hepatic or splenic abscesses.
Other signs and symptoms that may occur:
is the causative agent in most people with Lemierre's syndrome. However only 1 in 400 cases of Fusobacterium necrophorum results in Lemierre's syndrome. and 81% of cases of Lemierres's syndrome have been infected with Fusobacterium necrophorum, while in 11% of people it was caused by other Fusobacterium species. MRSA might also be an issue in Lemierre infections. Rarely Lemierre's syndrome is caused by other (usually Gram-negative
) bacteria, which include Bacteroides fragilis
and Bacteroides melaninogenicus
, Peptostreptococcus
spp., Streptococcus microaerophile, Staphylococcus aureus
, and Eikenella corrodens.
, a mastoiditis
, a sinusitis
or a parotitis
.
During the primary infection, F. necrophorum colonizes the infection site and the infection spreads to the parapharyngeal space
. The bacteria then invade the peritonsillar blood vessels where they can spread to the internal jugular vein
. In this vein, the bacteria cause the formation of a thrombus
containing these bacteria. Furthermore, the internal jugular vein
becomes inflamed. This septic thrombophlebitis
can give rise to septic microemboli
that disseminate to other parts of the body where they can form abscesses and septic infarctions. The first capillaries
that the emboli encounter where they can nestle themselves are the pulmonary capillaries. As a consequence, the most frequently involved site of septic metastases are the lungs, followed by the joints (knee, hip, sternoclavicular joint
, shoulder and elbow). In the lungs, the bacteria cause abscesses, nodulary and cavitary lesions. Pleural effusion is often present. Other sites involved in septic metastasis and abscess formation are the muscles and soft tissues, liver, spleen, kidneys and nervous system (intracranial abscesses, meningitis).
Production of bacterial toxins such as lipopolysaccharide
leads to secretion of cytokines by white blood cells which then both lead to symptoms of sepsis
. F. necrophorum produces hemagglutinin
which causes platelet aggregation that can lead to diffuse intravascular coagulation and thrombocytopenia
.
Laboratory investigations reveal signs of a bacterial infection with elevated C-reactive protein
, erythrocyte sedimentation rate
and white blood cells (notably neutrophils). Platelet count can be low or high. Liver function tests and renal function tests are often abnormal.
Thrombosis of the internal jugular vein can be displayed with sonography
. However, thrombi that have developed recently have low echogenicity and thus will not show up on ultrasound. A CT scan or an MRI scan is more sensitive in displaying the thrombus.
Chest X-ray
and chest CT may show pleural effusion, nodules, infiltrates, abscesses and cavitations.
Bacterial cultures
taken from the blood, joint aspirates
or other sites can identify the causative agent of the disease.
Other illnesses that can be included in the differential diagnosis
are:
es, for which antibiotic treatment is unnecessary, such treatment is not usual in the first phase of the disease. Lemierre's disease proves that, rarely, antibiotics are needed for 'sore throats'. Fusobacterium necrophorum is generally highly susceptible to beta-lactam antibiotic
s, metronidazole
, clindamycin
and third generation cephalosporins while the other fusobacteria have varying degrees of resistance to beta-lactams and clindamycin. Additionally, there may exist a co-infection by another bacterium. For these reasons is often advised not to use monotherapy in treating Lemierre's syndrome. Penicillin and penicillin-derived antibiotics can thus be combined with a beta-lactamase inhibitor
such as clavulanic acid
or with metronidazole. Clindamycin can be given as monotherapy.
If antibiotic therapy does not improve the clinical picture, it may prove useful to drain any abscesses and/or perform ligation of the internal jugular vein where the antibiotic can not penetrate.
There is no evidence to opt for or against the use of anticoagulation therapy. The low incidence of Lemierre's syndrome has not made it possible to set up clinical trials to study the disease.
The disease can often be un-treatable, especially if other negative factors occur,i.e. various diseases occurring at the same time, such as meningitis
, pneumonia
.
. The reduced use of antibiotics for sore throats may have increased the risk of this disease, with 19 cases in 1997 and 34 cases in 1999 reported in the UK
. The incidence rate is currently 0.8 cases per million in the general population, leading it to be termed the "forgotten disease". The disease is known to affect healthy young adults. The disease is becoming less rare with many cases being reported, however it is still known as "the forgotten disease" as many doctors are unaware of its existence, therefore often not even diagnosed which might conisderably change the above mentioned statistics. The mortality rate was 90% prior to antibiotic therapy, but is now generally quoted as 15% once this illness is correctly diagnosed and cured with proper medical treatment, although one series of cases reported mortality as low as 6.4%.
following from a throat infection was described by Scottmuller in 1918. However it was Andre Lemierre, in 1936, who published a series of 20 cases where throat infections were followed by identified anaerobic septicemia, of whom 18 patients died.
Thrombophlebitis
Thrombophlebitis is phlebitis related to a thrombus . When it occurs repeatedly in different locations, it is known as "Thrombophlebitis migrans" or "migrating thrombophlebitis".-Signs and symptoms:...
usually caused by the bacterium Fusobacterium necrophorum
Fusobacterium necrophorum
Fusobacterium necrophorum is the species of Fusobacterium that is responsible for Lemierre's syndrome, and appears to be responsible for 10% of all acute sore throats, 21% of all recurring sore throats, and 23% of peritonsillar abscesses with the remainder being caused by Group A streptococci or...
, and occasionally by other members of the genus Fusobacterium (F. nucleatum, F. mortiferum and F. varium etc.) and usually affects young, healthy adults. Lemierre's syndrome develops most often after a sore throat caused by some bacterium of the Streptococcus
Streptococcus
Streptococcus is a genus of spherical Gram-positive bacteria belonging to the phylum Firmicutes and the lactic acid bacteria group. Cellular division occurs along a single axis in these bacteria, and thus they grow in chains or pairs, hence the name — from Greek στρεπτος streptos, meaning...
genus has created a peritonsillar abscess, a pocket filled with pus and bacteria near the tonsils. Deep in the abscess, anaerobic bacteria (microbes that do not require oxygen) like Fusobacterium necrophorum can flourish. The bacteria penetrate from the abscess into the neighboring jugular vein in the neck and there they cause an infected clot (thrombosis
Thrombosis
Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot to prevent blood loss...
) to form, from which bacteria are seeded throughout the body by the bloodstream (bacteremia). Pieces of the infected clot break off and travel to the lungs as emboli blocking branches of the pulmonary artery bringing deoxygenated blood from the heart to the lungs. This causes shortness of breath, chest pain and severe pneumonia
Pneumonia
Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
. Fusobacteria are a normal part of the oropharyngeal flora.
Sepsis
Sepsis
Sepsis is a potentially deadly medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection. The body may develop this inflammatory response by the immune system to microbes in the blood, urine, lungs, skin, or other tissues...
following from a throat infection was described by Scottmuller in 1918. However it was André Lemierre
André Lemierre
André-Alfred Lemierre was a French bacteriologist.He studied in Paris where he became an externe in 1896, interne in 1900. He obtained his doctorate in 1904, became Médecin de Hôpitaux in 1912 and later worked in the Hôspital Bichat. He was habilitated in 1913 and in 1926 was promoted to professor...
, in 1936, who published a series of 20 cases where throat infections were followed by identified anaerobic septicemia, of whom 18 patients died.
Signs and symptoms
The symptoms vary, but usually start with a sore throat, fever, and general body weakness. These are followed by extreme lethargy, spiked fevers, rigors, swollen cervical lymph nodeLymph node
A lymph node is a small ball or an oval-shaped organ of the immune system, distributed widely throughout the body including the armpit and stomach/gut and linked by lymphatic vessels. Lymph nodes are garrisons of B, T, and other immune cells. Lymph nodes are found all through the body, and act as...
s and a swollen, tender or painful neck. Often there is abdominal pain, diarrhea, nausea and vomiting during this phase. These symptoms usually occur several days to 2 weeks after the initial symptoms.
Symptoms of pulmonary involvement can be shortness of breath, cough and painful breathing (pleuritic chest pain
Pleurisy
Pleurisy is an inflammation of the pleura, the lining of the pleural cavity surrounding the lungs. Among other things, infections are the most common cause of pleurisy....
). Rarely there is hemoptysis
Hemoptysis
Hemoptysis or haemoptysis is the expectoration of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis is the expectoration (coughing up) of blood or of blood-stained sputum from the bronchi, larynx, trachea, or lungs Hemoptysis or haemoptysis ...
. Painful or inflamed joints (arthralgia
Arthralgia
Arthralgia literally means joint pain; it is a symptom of injury, infection, illnesses or an allergic reaction to medication....
or arthritis
Septic arthritis
Septic arthritis is the purulent invasion of a joint by an infectious agent which produces arthritis. People with artificial joints are more at risk than the general population but have slightly different symptoms, are infected with different organisms and require different treatment. Septic...
, respectively) exist when the joints are involved.
Septic shock
Septic shock
Septic shock is a medical emergency caused by decreased tissue perfusion and oxygen delivery as a result of severe infection and sepsis, though the microbe may be systemic or localized to a particular site. It can cause multiple organ dysfunction syndrome and death...
can occur which presents as hypotension
Hypotension
In physiology and medicine, hypotension is abnormally low blood pressure, especially in the arteries of the systemic circulation. It is best understood as a physiologic state, rather than a disease. It is often associated with shock, though not necessarily indicative of it. Hypotension is the...
, tachycardia
Tachycardia
Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...
, oliguria
Oliguria
Oliguria is the low output of urine, It is clinically classified as an output below 300-500ml/day. The decreased output of urine may be a sign of dehydration, renal failure, hypovolemic shock, HHNS Hyperosmolar Hyperglycemic Nonketotic Syndrome, multiple organ dysfunction syndrome, urinary...
and tachypnea
Tachypnea
Tachypnea means rapid breathing. Any rate between 12-20 breaths per minute is normal. Tachypnea is a respiration rate greater than 20 breaths per minute. - Distinction from other breathing terms :...
. Nuchal rigidity
Meningism
Meningism is the triad of nuchal rigidity , photophobia and headache. It is a sign of irritation of the meninges, such as seen in meningitis, subarachnoid hemorrhages and various other diseases...
, headache and photophobia
Photophobia
Photophobia is a symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of actual physical photosensitivity of the eyes, though the term...
occur in case there is meningitis
Meningitis
Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...
.
Hepatomegaly
Hepatomegaly
Hepatomegaly is the condition of having an enlarged liver. It is a nonspecific medical sign having many causes, which can broadly be broken down into infection, direct toxicity, hepatic tumours, or metabolic disorder. Often, hepatomegaly will present as an abdominal mass...
and splenomegaly
Splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any...
(enlarged liver and spleen, respectively) can be found, but are not always associated with hepatic or splenic abscesses.
Other signs and symptoms that may occur:
- Headache (not related to meningitis)
- Muscle pain
- JaundiceJaundiceJaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...
- TrismusTrismus-Common causes:*Pericoronitis is the most common cause of trismus.*Inflammation of muscles of mastication. It is a frequent sequel to surgical removal of mandibular third molars . The condition is usually resolved on its own in 10–14 days, during which time eating and oral hygiene are compromised...
- CrepitationsRalesCrackles, crepitations, or rales are the clicking, rattling, or crackling noises that may be made by one or both lungs of a human with a respiratory disease during inhalation. They are often heard only with a stethoscope...
are sometimes heard over the lungs - Pericardial friction rubPericardial friction rubA pericardial friction rub, also pericardial rub, is an audible medical sign used in the diagnosis of pericarditis. Upon auscultation, this sign is an extra heart sound of to-and-fro character, typically with three components, two systolic and one diastolic. It resembles the sound of squeaky...
s as a sign of pericarditisPericarditisPericarditis is an inflammation of the pericardium . A characteristic chest pain is often present.The causes of pericarditis are varied, including viral infections of the pericardium, idiopathic causes, uremic pericarditis, bacterial infections of the precardium Pericarditis is an inflammation of...
(rare) - Cranial nerve paralysisParalysisParalysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...
and Horner's syndromeHorner's syndromeHorner's syndrome is the combination of drooping of the eyelid and constriction of the pupil , sometimes accompanied by decreased sweating of the face on the same side; redness of the conjunctiva of the eye is often also present...
(both rare)
Cause
Fusobacterium necrophorumFusobacterium necrophorum
Fusobacterium necrophorum is the species of Fusobacterium that is responsible for Lemierre's syndrome, and appears to be responsible for 10% of all acute sore throats, 21% of all recurring sore throats, and 23% of peritonsillar abscesses with the remainder being caused by Group A streptococci or...
is the causative agent in most people with Lemierre's syndrome. However only 1 in 400 cases of Fusobacterium necrophorum results in Lemierre's syndrome. and 81% of cases of Lemierres's syndrome have been infected with Fusobacterium necrophorum, while in 11% of people it was caused by other Fusobacterium species. MRSA might also be an issue in Lemierre infections. Rarely Lemierre's syndrome is caused by other (usually Gram-negative
Gram staining
Gram staining is a method of differentiating bacterial species into two large groups ....
) bacteria, which include Bacteroides fragilis
Bacteroides fragilis
Bacteroides fragilis is a Gram-negative bacillus bacterium species, and an obligate anaerobe of the gut.B. fragilis group is the most commonly isolated bacteriodaceae in anaerobic infections especially those that originate from the gastrointestinal flora. B. fragilis is the most prevalent organism...
and Bacteroides melaninogenicus
Bacteroides melaninogenicus
Bacteroides melaninogenicus is a normal flora found in the upper respiratory tract. it is an important human pathogen in various anaerobic infections often mixed with other aerobic and anaerobic bacteria....
, Peptostreptococcus
Peptostreptococcus
Peptostreptococcus is a genus of anaerobic, Gram-positive, non-spore forming bacteria. The cells are small, spherical, and can occur in short chains, in pairs or individually. Peptostreptococcus are slow-growing bacteria with increasing resistance to antimicrobial drugs.The most frequently...
spp., Streptococcus microaerophile, Staphylococcus aureus
Staphylococcus aureus
Staphylococcus aureus is a facultative anaerobic Gram-positive coccal bacterium. It is frequently found as part of the normal skin flora on the skin and nasal passages. It is estimated that 20% of the human population are long-term carriers of S. aureus. S. aureus is the most common species of...
, and Eikenella corrodens.
Pathophysiology
Lemierre's syndrome is initiated by an infection of the head and neck region. Usually this infection is a pharyngitis (which occurred in 87.1% of patients as reported by a literature review), but it can also be initiated by an otitisOtitis
Otitis is a general term for inflammation or infection of the ear, in both humans and other animals.It is subdivided into the following:*Otitis externa, external otitis, or "swimmer's ear" involves the outer ear and ear canal. In external otitis, the ear hurts when touched or pulled.*Otitis media...
, a mastoiditis
Mastoiditis
Mastoiditis is an infection of mastoid process, the portion of the temporal bone of the skull that is behind the ear which contains open, air-containing spaces. It is usually caused by untreated acute otitis media and used to be a leading cause of child mortality. With the development of...
, a sinusitis
Sinusitis
Sinusitis is inflammation of the paranasal sinuses, which may be due to infection, allergy, or autoimmune issues. Most cases are due to a viral infection and resolve over the course of 10 days...
or a parotitis
Parotitis
Parotitis is an inflammation of one or both parotid glands, the major salivary glands located on either side of the face, in humans. The parotid gland is the salivary gland most commonly affected by inflammation.-Infectious parotitis:...
.
During the primary infection, F. necrophorum colonizes the infection site and the infection spreads to the parapharyngeal space
Parapharyngeal space
The parapharyngeal space is a potential space in the head and the neck. It has clinical importance in otolaryngology due to parapharyngeal space tumours and parapharyngeal abscess developing in this area....
. The bacteria then invade the peritonsillar blood vessels where they can spread to the internal jugular vein
Internal jugular vein
The two internal jugular veins collect the blood from the brain, the superficial parts of the face, and the neck.-Path:On both sides and at the base of the brain, the inferior petrosal sinus and the sigmoid sinus join to form the internal jugular vein...
. In this vein, the bacteria cause the formation of a thrombus
Thrombus
A thrombus , or blood clot, is the final product of the blood coagulation step in hemostasis. It is achieved via the aggregation of platelets that form a platelet plug, and the activation of the humoral coagulation system...
containing these bacteria. Furthermore, the internal jugular vein
Internal jugular vein
The two internal jugular veins collect the blood from the brain, the superficial parts of the face, and the neck.-Path:On both sides and at the base of the brain, the inferior petrosal sinus and the sigmoid sinus join to form the internal jugular vein...
becomes inflamed. This septic thrombophlebitis
Thrombophlebitis
Thrombophlebitis is phlebitis related to a thrombus . When it occurs repeatedly in different locations, it is known as "Thrombophlebitis migrans" or "migrating thrombophlebitis".-Signs and symptoms:...
can give rise to septic microemboli
Septic embolism
A septic embolism is a type of embolism that is infected with bacteria, resulting in the formation of pus. These may become dangerous if dislodged from their original location. Like other emboli, a septic embolism may be fatal....
that disseminate to other parts of the body where they can form abscesses and septic infarctions. The first capillaries
Capillary
Capillaries are the smallest of a body's blood vessels and are parts of the microcirculation. They are only 1 cell thick. These microvessels, measuring 5-10 μm in diameter, connect arterioles and venules, and enable the exchange of water, oxygen, carbon dioxide, and many other nutrient and waste...
that the emboli encounter where they can nestle themselves are the pulmonary capillaries. As a consequence, the most frequently involved site of septic metastases are the lungs, followed by the joints (knee, hip, sternoclavicular joint
Sternoclavicular articulation
The sternoclavicular articulation is a synovial double-plane joint composed of two portions separated by an articular disc. The parts entering into its formation are the sternal end of the clavicle, the upper and lateral part of the manubrium sterni , and the cartilage of the first rib, visible...
, shoulder and elbow). In the lungs, the bacteria cause abscesses, nodulary and cavitary lesions. Pleural effusion is often present. Other sites involved in septic metastasis and abscess formation are the muscles and soft tissues, liver, spleen, kidneys and nervous system (intracranial abscesses, meningitis).
Production of bacterial toxins such as lipopolysaccharide
Lipopolysaccharide
Lipopolysaccharides , also known as lipoglycans, are large molecules consisting of a lipid and a polysaccharide joined by a covalent bond; they are found in the outer membrane of Gram-negative bacteria, act as endotoxins and elicit strong immune responses in animals.-Functions:LPS is the major...
leads to secretion of cytokines by white blood cells which then both lead to symptoms of sepsis
Sepsis
Sepsis is a potentially deadly medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection. The body may develop this inflammatory response by the immune system to microbes in the blood, urine, lungs, skin, or other tissues...
. F. necrophorum produces hemagglutinin
Hemagglutinin
Influenza hemagglutinin or haemagglutinin is a type of hemagglutinin found on the surface of the influenza viruses. It is an antigenic glycoprotein. It is responsible for binding the virus to the cell that is being infected...
which causes platelet aggregation that can lead to diffuse intravascular coagulation and thrombocytopenia
Thrombocytopenia
Thrombocytopenia is a relative decrease of platelets in blood.A normal human platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. These limits are determined by the 2.5th lower and upper percentile, so values outside this range do not necessarily indicate disease...
.
Diagnosis
Diagnosis and the imaging (and laboratory) studies to be ordered largely depend on the patient history, signs and symptoms. If a persistent sore throat, with the symptoms are found, physicians are cautioned to screen for Lemierre's syndrome.Laboratory investigations reveal signs of a bacterial infection with elevated C-reactive protein
C-reactive protein
C-reactive protein is a protein found in the blood, the levels of which rise in response to inflammation...
, erythrocyte sedimentation rate
Erythrocyte sedimentation rate
The erythrocyte sedimentation rate , also called a sedimentation rate or Biernacki Reaction, is the rate at which red blood cells sediment in a period of 1 hour...
and white blood cells (notably neutrophils). Platelet count can be low or high. Liver function tests and renal function tests are often abnormal.
Thrombosis of the internal jugular vein can be displayed with sonography
Medical ultrasonography
Diagnostic sonography is an ultrasound-based diagnostic imaging technique used for visualizing subcutaneous body structures including tendons, muscles, joints, vessels and internal organs for possible pathology or lesions...
. However, thrombi that have developed recently have low echogenicity and thus will not show up on ultrasound. A CT scan or an MRI scan is more sensitive in displaying the thrombus.
Chest X-ray
Chest X-ray
In medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...
and chest CT may show pleural effusion, nodules, infiltrates, abscesses and cavitations.
Bacterial cultures
Microbiological culture
A microbiological culture, or microbial culture, is a method of multiplying microbial organisms by letting them reproduce in predetermined culture media under controlled laboratory conditions. Microbial cultures are used to determine the type of organism, its abundance in the sample being tested,...
taken from the blood, joint aspirates
Arthrocentesis
Arthrocentesis is the clinical procedure of using a syringe to collect synovial fluid from a joint capsule. It is also known as joint aspiration. Arthrocentesis is used in the diagnosis of gout, arthritis, and synovial infections....
or other sites can identify the causative agent of the disease.
Other illnesses that can be included in the differential diagnosis
Differential diagnosis
A differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...
are:
- Q feverQ feverQ fever is a disease caused by infection with Coxiella burnetii, a bacterium that affects humans and other animals. This organism is uncommon but may be found in cattle, sheep, goats and other domestic mammals, including cats and dogs...
- TuberculosisTuberculosisTuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...
- PneumoniaPneumoniaPneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
Treatment
Lemierre's syndrome is primarily treated with antibiotics given intravenously. However, because sore throats are most commonly caused by virusVirus
A virus is a small infectious agent that can replicate only inside the living cells of organisms. Viruses infect all types of organisms, from animals and plants to bacteria and archaea...
es, for which antibiotic treatment is unnecessary, such treatment is not usual in the first phase of the disease. Lemierre's disease proves that, rarely, antibiotics are needed for 'sore throats'. Fusobacterium necrophorum is generally highly susceptible to beta-lactam antibiotic
Beta-lactam antibiotic
β-Lactam antibiotics are a broad class of antibiotics, consisting of all antibiotic agents that contains a β-lactam nucleus in its molecular structure. This includes penicillin derivatives , cephalosporins , monobactams, and carbapenems...
s, metronidazole
Metronidazole
Metronidazole is a nitroimidazole antibiotic medication used particularly for anaerobic bacteria and protozoa. Metronidazole is an antibiotic, amebicide, and antiprotozoal....
, clindamycin
Clindamycin
Clindamycin rINN is a lincosamide antibiotic. It is usually used to treat infections with anaerobic bacteria but can also be used to treat some protozoal diseases, such as malaria...
and third generation cephalosporins while the other fusobacteria have varying degrees of resistance to beta-lactams and clindamycin. Additionally, there may exist a co-infection by another bacterium. For these reasons is often advised not to use monotherapy in treating Lemierre's syndrome. Penicillin and penicillin-derived antibiotics can thus be combined with a beta-lactamase inhibitor
Beta-lactamase inhibitor
A beta-lactamase inhibitor is a drug given in conjunction with a beta-lactam antibiotic. Although the inhibitor does not usually have significant antibiotic activity on its own, it inhibits activity of beta-lactamase, a protein that confers resistance of beta-lactam antibiotics to...
such as clavulanic acid
Clavulanic acid
Clavulanic acid is a competitive β-lactamase inhibitor combined with penicillin group antibiotics to overcome certain types of antibiotic resistance. It is used to overcome resistance in bacteria that secrete β-lactamase, which otherwise inactivates most penicillins...
or with metronidazole. Clindamycin can be given as monotherapy.
If antibiotic therapy does not improve the clinical picture, it may prove useful to drain any abscesses and/or perform ligation of the internal jugular vein where the antibiotic can not penetrate.
There is no evidence to opt for or against the use of anticoagulation therapy. The low incidence of Lemierre's syndrome has not made it possible to set up clinical trials to study the disease.
The disease can often be un-treatable, especially if other negative factors occur,i.e. various diseases occurring at the same time, such as meningitis
Meningitis
Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...
, pneumonia
Pneumonia
Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
.
Prognosis
When properly diagnosed, the mortality of Lemierre's syndrome is about 4.6%. Since this disease is not well known and often remains undiagnosed, mortality might be much higher.Epidemiology
Lemierre's syndrome is currently rare, but was more common in the early 20th century before the discovery of penicillinPenicillin
Penicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
. The reduced use of antibiotics for sore throats may have increased the risk of this disease, with 19 cases in 1997 and 34 cases in 1999 reported in the UK
United Kingdom
The United Kingdom of Great Britain and Northern IrelandIn the United Kingdom and Dependencies, other languages have been officially recognised as legitimate autochthonous languages under the European Charter for Regional or Minority Languages...
. The incidence rate is currently 0.8 cases per million in the general population, leading it to be termed the "forgotten disease". The disease is known to affect healthy young adults. The disease is becoming less rare with many cases being reported, however it is still known as "the forgotten disease" as many doctors are unaware of its existence, therefore often not even diagnosed which might conisderably change the above mentioned statistics. The mortality rate was 90% prior to antibiotic therapy, but is now generally quoted as 15% once this illness is correctly diagnosed and cured with proper medical treatment, although one series of cases reported mortality as low as 6.4%.
History
SepsisSepsis
Sepsis is a potentially deadly medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection. The body may develop this inflammatory response by the immune system to microbes in the blood, urine, lungs, skin, or other tissues...
following from a throat infection was described by Scottmuller in 1918. However it was Andre Lemierre, in 1936, who published a series of 20 cases where throat infections were followed by identified anaerobic septicemia, of whom 18 patients died.