Rheumatic fever

Synonyms in the broader sense

  • Streptococcal allergic second disease
  • Streptococcal associated arthritis
  • Streptococcal associated endocarditis

definition

Rheumatic fever is an inflammatory reaction of the body.
Toxins (bacterial toxins), which are formed by bacteria from the group of streptococci, cause after a bacterial infection of the upper airways, this second disease occurs. Patients were typically 10-20 days prior to rheumatic fever on streptococcal tonsillitis
(Tonsillitis) or pharyngitis (pharyngitis).

causes

In this approximately 10-20-day symptom-free interval, when the patient does not feel ill, the body develops antibodies (the body's own antibodies) against the bacteria that previously caused upper airway inflammation:
The immune system forms antibodies against the invading bacteria. Internal structures such as joints, heart muscle, skin or brain cells have proteins that are similar to the bacterial protein, so that a cross reaction between the body cells and the antibodies formed takes place. This means that the antibodies originally formed as an immune response against the bacteria are now directed, inter alia, against the body's own joint components or cardiac muscle cells. This results in the patients in the context of rheumatic fever arthritis (joint inflammation) or endocarditis.

Frequency and occurrence

The disease frequency peak is between the 5th and 15th year of life.
Rheumatic fever is rare in industrialized countries, as treatment of tonsillitis (angina tonsillaris) with penicillin prevents the second disease.

symptoms

After a period of 10-20 days after tonsillar streptococcal tonsillitis or streptococcal pharyngitis, in which the patient has no symptoms, various complaints occur. These affect the joints, the skin, the heart and the central nervous system:

  • The patient has a fever, a headache and sweats more.
    • These are nonspecific symptoms, but very typical in the presence of rheumatic fever.
  • Several large joints such as the hip joints, ankle or knee joints hurt, are overheated and swell ( joint inflammation, arthritis ). The involvement of the joints is referred to as acutely migratory polyarthritis, as the symptoms occur sequentially and on multiple joints, so "jump" from one to the other joint.
  • There are skin changes in 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 patients have circumscribed erythematous areas ( erythema annulare ), which are mainly localized to the trunk of the body.
  • The so-called erythema nodosum occurs as a sublime, tender knot formation on the lower leg.
  • The rheumatic fever also affects the heart:
    • All cardiac wall constituents may be affected by the inflammatory response, with a distinction between myocarditis, inner lining ( endocarditis ) and pericarditis ( pericarditis ).
  • Myocarditis is associated with increased heart rate ( tachycardia ) and non-rhythmic heartbeat ( arrhythmia ).
  • Inflammation of the inner lining of the heart (endocarditis) is an important factor in the progression prognosis of the disease, since the heart valves are formed by the inner lining of the heart ( endocardium ).
    • At the edge of the heart valves arise during the immune reaction of the body wart-like deposits, which change the shape and function of the valves. However, intact heart valves are crucial for the normal pumping function of the heart:
    • The heart valves altered by the rheumatic fever lead to a limited pumping function of the heart.
  • Symptoms caused by inflammatory changes in the central nervous system may also occur after a symptomless interval of months, but they are altogether rare. The patients notice uncontrolled movements of the hands, awkwardness or their own involuntary grimacing of the face. These symptoms are collectively called chorea minor; they are an expression of inflammation of the brain ( encephalitis ).
  • If the symptom complex of the chorea minor occurs, an examination of the inflammatory process of the heart should also be carried out.

Is there a rheumatic fever even without a fever?

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

High fever is more common in children and adolescents. It may be a sign of persistent bacterial infection (often streptococcal) in the upper respiratory tract, which is often the cause of rheumatic fever. In addition, the fever may indicate a strong inflammation in the human body.

diagnosis

The inflammatory signs in the blood are unspecific for the rheumatic fever, but typically present. The lowering of the blood corpuscles ( blood cell lowering rate, BSG ) is accelerated and the C-reactive protein ( CRP ) is increasingly formed in the course of the inflammation.

Further laboratory investigations can determine whether a streptococcal infection has taken place:
With a throat swab can be determined whether there is a colonization of the upper airways with streptococci. For this test, a rapid streptococcal antigen test and the ability to create a culture of the smear are available.

The concentration of anti- bacterial antibodies ( antistreptolysin and anti-DNAse-B ) can be determined in a blood sample.

The concentration of the antibody against streptolysin ( antibody titer ) only indicates an acute inflammation above a value of more than 300 IU ( IE = international units ). The titer preferably increases in streptococcal infections of the nasopharynx, which is why it is of particular importance in the diagnosis of rheumatic fever.

Rheumatic fever is diagnosed by the Jones criteria formulated in 1992 by the American Heart Association.
The presence of the disease is likely if evidence of a previous streptococcal infection is possible or if two main criteria or one major criterion and one minor criterion of the Jones criteria are met.

The main criteria

  • migratory polyarthritis ( joint inflammation, multiple joints are affected )
  • Carditis ( inflammation of the heart )
  • rheumatoid nodules
  • Erythema annulare ( circumscribed reddened skin areas, especially on the trunk of the body )
  • Chorea minor ( involvement of the central nervous system with motor symptoms )

Additional criteria include:

  • fever
  • Joint pain ( arthralgia )
  • Rate of erythrocyte accelerates and / or increases C-reactive protein
  • Change in the transmission of excitement 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. In the presence of rheumatic fever, the inflammatory parameters in the blood are increased. These are the erythrocyte sedimentation rate (ESR) and the CRP value.

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

duration

The duration of the disease is not clear. On the one hand, rheumatic fever itself is a secondary disease of a bacterial infection, but on the other hand it also involves some lengthy sequelae.

The previous streptococcal infection can last for about 1-3 weeks. The subsequent asymptomatic phase also runs for about 2 weeks, whereas the acute rheumatic fever lasts up to 12 weeks.
Thus, the period from infection to the onset of fever symptoms lasts about 14 weeks on average.

However, some sequelae can occur that have no limited duration of illness. If left untreated, the symptoms persist for years. The drug treatment takes different lengths depending on the severity of the rheumatic fever. In most cases, the medication must be taken for 5 years or until the age of 21, more rarely over 10 years, to prevent relapses and chronic courses.

course

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

  1. Streptococcal infection
  2. Latency (= no period of time) 1-3 weeks
  3. Rheumatic fever, lasting about 6-12 weeks
  4. Flap defects and scars on cardiac involvement

Treatment of rheumatic fever

The drug of choice in streptococcal infections is the antibiotic penicillin, as the bacterial species is sensitive to this drug, ie the bacteria die off on penicillin therapy.
The first step in the treatment of rheumatic fever is the administration of penicillin over 10 days with the goal of killing living streptococci. If allergy to this antibiotic is present, macrolides such as erythromycin are prescribed.

An anti-inflammatory concomitant treatment with acetylsalicylic acid (eg Aspirin®) or corticosteroids (eg cortisone) in the involvement of the heart are performed.
Patients must take lower dose penicillin for 10 years following this initial treatment to prevent relapse ( recurrence ) of rheumatic fever.
If the heart is affected by the inflammatory process, this period of ingestion may be prolonged.
The antibiotic is usually given at four-week intervals with an intramuscular injection (administration of the drug by syringe into the muscle), so that the drug must not be taken daily in tablet form.

After the end of long-term therapy, penicillins should be administered during diagnostic or surgical procedures (eg dental examinations, operations in the hospital) in order to avoid inflammation of the heart's lining ( endocarditis prophylaxis ).

This can occur, for example, when the dental treatment bacteria from the nose and throat, which include the streptococci, enter the bloodstream and cause an inflammatory response. The antibiotic protection before, during and after the examination or surgery serves to prevent the recurrence of the rheumatic fever with a heart valve involvement or an exacerbation of heart valve changes after statttenemumem rheumatic fever.

guidelines

Medical guidelines help doctors to treat certain medical conditions. They are not legally binding, but over a period of years summarized systematic findings on the disease. They help with diagnosis, therapy and prevention, but always have to be adapted to the individual case.

The guidelines on rheumatic fever or also on the poststreptococcal arthritis are published by several companies. The "German Society of Rheumatology" published a general recommendation for the treatment of rheumatic fever in children and adults. The "German Society for Pediatric Cardiology", on the other hand, explicitly published a guideline for children and adolescents.

The guidelines include eight sub-headings summarizing the full management of the disease. First, the rheumatic fever is defined biochemically and the classification of disease stages based on various criteria. Subsequently, 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 subitem. The guideline concludes with recommendations for the follow-up, prevention and prophylaxis of rheumatic fever.

How contagious is a rheumatic fever?

The rheumatic fever is not contagious. On the other hand, the common underlying infection of the upper respiratory tract with bacteria (streptococci) is contagious. These bacteria are transmitted via the inhalation of small droplets (droplet infection) or by close contact with affected persons (smear infection) from person to person. To avoid infection, intensive hygiene measures (eg washing hands) or avoiding close contact with those affected are recommended. About 1 to 3% of those with a bacterial infection (streptococcus) of the upper respiratory tract develop a rheumatic fever.

Prognosis and complications

The prognosis is determined by the degree of expression of the inner-inner-lining inflammation. If the patient is again suffering from rheumatic fever, the likelihood of a valvular heart failure increases as the patient progresses.
Therefore, it is important to carry out an early and consistent penicillin therapy and to carry out a prophylactic penicillin administration before examinations and operations before degenerative (= pathologically changed) and non-reversible (= irreversible) valve damage occurs.

Rheumatic fever at the heart

The leading symptoms of a 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 secondary damages occur. The involvement of the inner wall of the heart, which can lead to heart valve defects, is dangerous. In most cases, insufficient heart valves are present in the left heart as a result of endocarditis.

Involvement of the heart muscle can lead to the death of muscle cells, arrhythmias of the heart, development of nodules and heart failure. If the pericardium is affected, it causes chest pain. This inflammation can be life-threatening when Perikardergüssen. Typical is the audible pericardia during auscultation.

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

Differences of rheumatic fever in the adult and the child

Rheumatic fever is more common in children between the ages of 3 and 16 years. In adulthood, a new appearance is usually very rare.

In adults, rheumatic fever manifests itself primarily at the joints. In addition to an inflammation, it comes to a strong redness of the affected joint and also to severe pain. Often an asymptomatic course is possible. As a rule, complaints develop within a few months. Targeted therapy can improve the symptoms after just a few days to weeks.

While in adults rheumatic fever is typically characterized by a milder course, more severe courses are possible in children. It often comes to a participation of the heart. Inflammation of the heart and heart valves ( endocarditis ) is possible. There is a risk that as a result of the inflammation to a strong scar tissue and the heart valves are impaired in their function. Without adequate therapy, permanent damage and severe impairment of the heart's activity are possible.

Rheumatic fever after scarlet fever

About 1 to 3% of scarlet fever patients develop rheumatic fever within a few weeks of scarlet fever. Especially children between the ages of 4 and 10 are affected.
Scarlet fever is a bacterial infection with streptococci (group A), which manifests itself mainly in the oral and pharyngeal area as well as on the skin. In addition to severe sore throat with difficulty swallowing it comes here to a strong redness in the mouth and throat ("strawberry tongue") as well as a fine blotchy rash on the whole body. Typically, the area around the mouth is recessed from the rash and appears pale. In addition, patients experience high fever and swelling of the lymph nodes of the neck.

  • How contagious is scarlet fever?
  • How often can you get scarlet fever?
  • Complications of scarlet fever

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