Internal Medicine

Rheumatic fever

Synonyms in a broader sense

Streptococcal secondary disease
Streptococcal Arthritis
Streptococcal-associated endocarditis

definition

Rheumatic fever is an inflammatory reaction of the body.
Toxins (bacterial poisons), which are formed by bacteria from the group of streptococci, cause this second disease to occur after a bacterial infection of the upper respiratory tract. Patients typically had streptococcal tonsillar angina 10-20 days before the onset of rheumatic fever
(Tonsillitis) or pharyngitis (sore throat).

causes

In this 10-20 day symptom-free interval, in which the patient does not feel sick, the body develops antibodies (body's own defense substances) against the bacteria that previously caused the inflammation of the upper airways:
The immune system forms antibodies against the invading bacteria. The body's own structures such as joints, heart muscle, skin or brain cells have proteins that are similar to bacteria protein, so that a cross reaction takes place between the body cells and the antibodies that are formed. This means that the antibodies originally formed as an immune response against the bacteria are now among others Direct against the body's own joint components or heart muscle cells. This results in rheumatic fever in patients with arthritis (joint inflammation) or endocarditis.

Frequency and occurrence

The peak of the disease frequency is between the ages of 5 and 15.
Rheumatic fever occurs only rarely in industrialized countries, as treatment of tonsillitis (angina tonsillaris) with penicillin prevents the second disease.

Symptoms

After a period of 10-20 days after a strep angina tonsillaris or streptococcal pharyngitis, during which the patient has no symptoms, various complaints appear. These affect the joints, the skin, the heart and the central nervous system:

The patient has a fever, headache and sweats more often.
- These are unspecific symptoms, but very typical in the presence of rheumatic fever.
Several large joints such as the hip, ankle or knee joints are painful, overheated and swollen (Joint inflammation, arthritis). The involvement of the joints is referred to as acute migratory polyarthritis, as the symptoms appear one after the other and in several joints, ie "jump over" from one joint to the other.
Skin changes occur in the context of rheumatic fever.
- The so-called rheumatoid nodules are nodular changes in the subcutaneous tissue, which are preferably located on the extensor sides of the arms and legs, but which can also occur on the heart valves.
- In addition, 10% of the patients have circumscribed, reddened areas of the skin (Erythema annulare), which are mainly located on the trunk of the body.
The so-called erythema nodosum occurs as a raised, tender knot formation on the lower leg.
Rheumatic fever also affects the heart:
- All parts of the heart wall can be affected by the inflammatory reaction, whereby one can distinguish between an inflammation of the heart muscle (Myocarditis), Inner layer of the heart (Endocarditis) and pericarditis (Pericarditis) differs.
Myocarditis proceeds with an increased heart rate (Tachycardia) and a non-rhythmic heartbeat (arrhythmia) hand in hand.
The inflammation of the inner layer of the heart (endocarditis) is an important factor in the prognosis of the course of the disease, as the heart valves are covered by theEndocardium) are formed.
- As part of the body's immune reaction, wart-shaped deposits form on the edge of the heart valves, which change the shape and function of the valves. Intact heart valves, however, are of crucial importance for the normal pumping function of the heart:
- The heart valves changed by the rheumatic fever lead to a restricted pumping function of the heart.
Symptoms caused by inflammatory changes in the central nervous system can occur even after a symptom-free interval of months, but are generally rare. Patients notice uncontrolled hand movements, clumsiness, or their own involuntary grimacing of the face. These symptoms are collectively referred to as chorea minor; they are an expression of an inflammation of the brain (Encephalitis).
If the symptom complex of chorea minor occurs, an examination for an inflammatory process of the heart should also be carried out.

Is there a rheumatic fever without a fever?

A rheumatic fever can occur without the person suffering from an elevated temperature. According to the underlying classification criteria (Jones criteria), the diagnosis of rheumatic fever is even possible without the presence of fever.

High fever is more common in children and adolescents. It can be a sign of a persistent bacterial infection (often with streptococci) in the upper respiratory tract, which is often the cause of rheumatic fever. The fever can also indicate severe inflammation in the human body.

diagnosis

Although the signs of inflammation in the blood are unspecific for rheumatic fever, they are typically present. The lowering of blood cells (Blood Cell Sedimentation Rate, ESR) is accelerated and the C-reactive protein (CRP) is increasingly formed in the course of inflammation.

Further laboratory tests can determine whether a streptococcal infection has occurred:
A throat swab can be used to determine whether there is a colonization of the upper airways with streptococci. A streptococcal antigen rapid test and the option to culture the smear are available for this test.

The concentration of antibodies directed against the bacteria (Antistreptolysin and Anti-DNAse-B) can be determined in a blood sample.

The concentration of the antibody against streptolysin (Antibody titer) only indicates from a value of over 300 IU (IE = international units) indicates acute inflammation. The titer rises preferentially with streptococcal infections of the nasopharynx, which is why it is of particular importance in the diagnosis of rheumatic fever.

The Jones criteria, which were formulated by the American Heart Association in 1992, are used to diagnose rheumatic fever.
The presence of the disease is likely if evidence of a previous streptococcal infection is possible or two main criteria or 1 main and 1 secondary criterion of the Jones criteria are met.

The main criteria

migratory polyarthritis (Joint inflammation, affecting multiple joints)
Carditis (Inflammation of the heart)
Rheumatoid nodules
Erythema annulare (circumscribed, reddened areas of skin, especially on the trunk of the body)
Chorea minor (Central nervous system involvement with motor symptoms)

The secondary criteria include:

fever
Joint pain (Arthralgia)
Accelerated sedimentation rate and / or increased C-reactive protein
Change in the conduction of excitation in the heart

What changes can be found in the blood values?

In addition to the clinical symptoms, the laboratory examination is an important criterion for the diagnosis of rheumatic fever. When a rheumatic fever is present, the inflammation parameters in the blood are increased. These are the sedimentation rate (ESR) and the CRP value.

For further diagnosis, antibodies against metabolic products of the causative bacteria (streptococci) can be detected in the blood. An increased titer of antibodies against "streptolysin O" is a sign of a previous infection in the throat area, an increased titer of antibodies against the enzyme "DNase B" indicates an infection in the skin area.

More on this: Levels of inflammation in the blood

Duration

The duration of the illness is cannot be clearly established. On the one hand, rheumatic fever is itself one Sequelae of a bacterial infection, on the other hand, it also has some long-term complications.

The previous streptococcal infection can about 1-3 weeks last for. The subsequent symptom-free phase is also about 2 weeks, whereas that acute rheumatic fever up to 12 weeks persists.
This means that the period from infection to subsidence of the fever symptoms lasts approximately on average 14 weeks.

Some Secondary diseases however, the no limited duration of illness have. If left untreated, the symptoms exist for years. The length of the drug treatment varies depending on the severity of the rheumatic fever. In most cases the Medication over 5 years or be taken up to the age of 21, less often over 10 years Relapses and prevent chronic courses.

course

The course of the disease can be divided into 4 stages:

Streptococcal Infection
Latency period (= period without symptoms) of 1-3 weeks
rheumatic fever, duration approx. 6-12 weeks
Valve defects and scars with cardiac involvement

Treatment of rheumatic fever

The drug of choice for strep infections is the antibiotic penicillin, as the bacterial species is sensitive to this drug, i. the bacteria die under penicillin therapy.
The first step in the therapy of rheumatic fever is the administration of penicillin for 10 days and the aim is to kill still living streptococci. If there is an allergy to this antibiotic, macrolides such as erythromycin are prescribed.

Accompanying anti-inflammatory treatment with acetylsalicylic acid (e.g. Aspirin ®) or corticosteroids (e.g. cortisone) if the heart is involved.
Following this initial treatment, patients must take a lower dose of penicillin for a period of 10 years in order to avoid relapse (Relapse) to prevent rheumatic fever.
If the heart is affected by the inflammatory process, this intake period can be extended.
The antibiotic is usually administered every four weeks with an intramuscular injection (administration of the drug into the muscle by means of a syringe) so that the drug does not have to be taken daily in tablet form.

After the end of long-term therapy, penicillins should be administered during diagnostic or surgical interventions (e.g. dental examinations, operations in hospital) in order to avoid inflammation of the inner layer of the heart (Endocarditis prophylaxis).

This can occur if e.g. During dental treatment, bacteria from the mouth, nose and throat, including streptococci, enter the bloodstream and cause an inflammatory reaction. The antibiotic protection before, during and after the examination or operation serves to prevent the recurrence of rheumatic fever with cardiac valve involvement or a worsening of the cardiac valve changes after a rheumatic fever has taken place.

Guidelines

Medical guidelines provide doctors with assistance in treating certain clinical pictures. you are not legally binding, but systematic findings on the disease that have been summarized over the years. You give one Decision support for diagnostics, therapy and Prevention, but must always be adapted to the individual case.

The guidelines on rheumatic fever or post-streptococcal arthritis are published by various societies. The "German Society for Rheumatology e.V." published a general recommendation for the treatment of rheumatic fever in children and adults. The "German Society for Pediatric Cardiology", however, published a guideline explicitly for children and adolescents.

The guidelines comprise eight sub-points, in which the complete management of the disease is summarized. First, the rheumatic fever is defined biochemically and the disease stages are classified based on various criteria. Then the symptoms and accompanying symptoms of the disease are described and the optimal diagnostic procedure is shown. Differential diagnoses for exclusion are also mentioned. The therapy is listed step by step in the fifth sub-item. The guideline concludes with recommendations for aftercare, prevention and prophylaxis of rheumatic fever.

How contagious is a rheumatic fever?

Rheumatic fever is not contagious. In contrast, the underlying infection of the upper respiratory tract with bacteria (streptococci) is often contagious. These bacteria are transmitted from person to person by inhaling small droplets (droplet infection) or through close contact with affected persons (smear infection). To avoid infection, intensive hygiene measures (e.g. hand washing) or avoiding close contact with those affected are recommended. About 1 to 3% of people with a bacterial infection (streptococci) of the upper respiratory tract develop a rheumatic fever.

Read more on the subject at: How do I know if my fever is contagious?

Prognosis and complications

The prognosis is determined by the degree of inflammation in the inner layer of the heart. If the patient falls ill with rheumatic fever again, the probability increases that a heart valve defect will occur in the further course.
Therefore, it is important to carry out an early and consistent penicillin therapy as well as a prophylactic administration of penicillin before examinations and operations, before degenerative (= pathologically changed) and irreversible (= irreversible) valve damage occurs.

Inform yourself: Artificial heart valves

Rheumatic fever on the heart

The main symptoms of rheumatic fever occur, among other things, in the heart. All structures of the human heart can be involved: the outer skin ("Pericarditis"), The heart muscle tissue ("Myocarditis") And the inner skin ("Endocarditis"). Depending on the inflamed area of the heart, different symptoms and consequential damage occur. Involvement of the inner wall of the heart is dangerous Valve defects can lead. Often are above all insufficient heart valves in the left heart as a result of endocarditis.

If the heart muscle is involved, it can lead to Muscle cell death, Arrhythmias of the heart Formation of nodules and to Heart failure come. If the pericardium is affected, it leads to chest pain. This inflammation can too life threatening be when Pericardial effusions. The audible rubbing of the pericardium during auscultation is typical.

If heart involvement, especially with heart valve defects, has occurred, prophylactic medication must be continued for 5-10 years, in severe cases up to the age of 40. When treated, the acute inflammation in the heart heals within 4-8 weeks. Under certain circumstances it can also take on chronic courses.

Differences in rheumatic fever in adults and children

Rheumatic fever occurs more frequently in children between the ages of 3 and 16. A new occurrence is usually very rare in adulthood.

In adults, rheumatic fever manifests itself primarily in the joints. In addition to inflammation, there is severe reddening of the affected joint and severe pain. An asymptomatic course is also often possible. The symptoms usually resolve within a few months. Targeted therapy can lead to an improvement in symptoms after a few days to weeks.

While rheumatic fever typically has a milder course in adults, more severe courses are possible in children. This often involves the heart being involved. Inflammation of the inner lining of the heart and the heart valves (Endocarditis) is possible. There is a risk that the inflammation will lead to severe scarring of the tissue and that the function of the heart valves will be impaired. Without adequate therapy, permanent damage and severe impairment of the heart's activity are possible.

Rheumatic fever after scarlet fever

Approximately 1 to 3% of scarlet fever patients develop rheumatic fever within a few weeks of a scarlet fever infection. Children between the ages of 4 and 10 are particularly affected.
Scarlet fever is a bacterial infection with streptococci (group A) that manifests itself primarily in the mouth and throat and on the skin. In addition to a severe sore throat with difficulty swallowing, there is a strong reddening of the mouth and throat ("strawberry tongue") and a fine-spotted rash all over the body. Typically, the area around the mouth is spared from the rash and appears pale. In addition, the patient develops a high fever and swelling of the lymph nodes in the neck.

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