Rheumatic fever

Rheumatic fever is an autoimmune reaction that can occur after certain bacterial infections. Read all about it!

Rheumatic fever

On rheumatic fever is an autoimmune reaction that can occur several weeks after infection with certain bacteria. The immune system attacks the body's own cells and causes inflammation of the joints, skin and heart. In Germany, rheumatic fever has become rare, but in countries with poor medical care, it is a major health problem. Left untreated, a rheumatic fever can even be fatal. Read all about rheumatic fever here.

ICD codes for this disease: ICD codes are internationally valid medical diagnosis codes. They are found e.g. in doctor's letters or on incapacity certificates. I00I01I02

Product Overview

Rheumatic fever

  • description

  • symptoms

  • Causes and risk factors

  • Examinations and diagnosis

  • treatment

  • Disease course and prognosis

Rheumatic fever: description

Rheumatic fever is an autoimmune reaction triggered by certain bacteria called beta-hemolytic streptococci. When infected with these pathogens, the body's immune system attacks them and targets specific surface structures of the bacteria. For this purpose, the immune system forms so-called antibodies, small proteins that bind specifically to the surface of pathogens and make them recognizable to immune cells. The immune cells finally make the invaders harmless.

After the immune system has formed antibodies against a specific pathogen, they remain in the body for a longer time, even if the actual disease has already recovered. Re-infection with the same pathogen can counteract the immune system so quickly and effectively.

However, sometimes it happens that antibodies not only recognize foreign material, but also falsely bind to the body's own structures, such as the surface of the heart valves. This tissue is thus marked as alien to the rest of the immune system and it comes to a defense reaction against the body. This is called an autoimmune reaction, a reaction against oneself.

In general, this happens only rarely, certain pathogens, such as beta-hemolytic streptococci, however, are known to be able to trigger autoimmune reaction. They have a surface texture that is similar to certain body structures. The antibodies "confuse" their goal, so to speak, ("molecular mimicry"). In the context of rheumatic fever, especially the heart, joint and skin cells are affected by the misdirected immune response.

How common is rheumatic fever?

Only a very small proportion of people infected with beta-hemolytic streptococci subsequently develop a rheumatic fever. In countries with good medical care, this complication can often be prevented by the right treatment. However, in many developing countries, rheumatic fever is much more common and the most common cause of heart disease in children. Worldwide, nearly half a million people suffer from rheumatic fever each year, including, in particular, children and adolescents between the ages of 3 and 16 years.

Rheumatic fever: symptoms

In rheumatic fever, there are acute complaints that occur days to weeks after the streptococcal infection. Long-term effects of rheumatic fever continue to cause problems for years to decades after infection with streptococci. These long-lasting and later onset symptoms are mostly due to the structural damage to the organs, which is difficult to prevent.

Acute rheumatic fever

The acute rheumatic fever usually occurs several weeks after the streptococcal infection. The disease can present itself very differently and is not easy to recognize, because not all symptoms always appear the same. Many sufferers come to the doctor with fever, weakness and fatigue. In addition, small children additionally complain of abdominal pain. Also, pain in the large joints, such as the knee, hip or shoulder are typical complaints that occur in rheumatic fever. The joints often not only hurt, but are also red and swollen.

A fast pulse, often with chest pain on light loads, may be an indication of inflammation of the heart. A truncated, non itchy rash and small nodules under the skin are other signs of rheumatic fever. These skin conditions are not always present, but can additionally occur when the heart is affected.

Finally, in a rheumatic fever, the immune system can also attack the nervous system. It sometimes comes to personality changes, muscle weakness, balance problems and disorders of fine motor skills. Infestation of the brain can lead to a special movement disorder, the so-called Chorea Sydenham. Children are much more affected by this neurological syndrome than adult patients.

Typical of the Chorea Sydenham are uncontrolled, aimless movements. The children behave awkwardly, for example, they spill soup or break plates. Unlike with the heart inflammation, the neurological symptoms heal usually without consequences. For example, the Chorea Sydenham is usually only for a few months.

Long-term consequences of rheumatic fever

Of the late effects of rheumatic fever, adults over 30 are usually more likely to be affected. These chronic complaints are expected when patients became ill with a severe rheumatic fever during their childhood. Even at an older age, they can then repeatedly suffer from relapses with increasing physical limitations. It is unlikely, however, that a rheumatic fever will affect adults for the first time without having occurred in childhood.

Heart damage from rheumatic fever is relatively common and often lasts a lifetime. Up to 60 percent of those affected show long-term damage to the heart. This applies in particular to late-diagnosed or untreated patients. The immune system attacks especially the heart valves. These work like a valve and guarantee that the heart pumps the blood continuously in one direction. If the heart valves break, this leads to a chronic overload and finally to pumping failure of the heart.

Rheumatic fever: causes and risk factors

The cause of the autoimmune reaction is beta-hemolytic streptococci of group A. Behind this complex name hides a relatively common pathogen, which settles especially in the pharynx and there leads to inflammation. A crimson pharyngeal mucosa with small yellow coverings is the result (streptococcal angina). Also, the childhood disease scarlet fever is caused by streptococci, as well as various skin infections.

Why some people have a rheumatic fever after a streptococcal infection and not in others is not completely clear. It is thought that some susceptibility to such a malfunction of the immune system is inherited.

Another important risk factor is age. Rheumatic fever in children is much more common than in the elderly. This risk is particularly high between the ages of five and fifteen, as throat infections with streptococci are more frequent during this period.

Other risk factors include poor hygienic and poor living conditions, along with inadequate medical care. Until the 1950s, rheumatic fever was also the most common inflammatory rheumatic disease among schoolchildren and adolescents in Germany. Thanks to consistent and timely antibiotic therapy, the number of cases has fallen sharply.

Rheumatic fever: examinations and diagnosis

The doctor always has to think of a rheumatic fever when a child or adolescent with a high fever and joint pain comes along and also had a sore throat in recent weeks. However, it is not always easy to recognize the rheumatic fever, as the symptoms may be very different in many patients.

As a diagnostic aid serve the doctor the so-called Jones criteria, which were developed in 1944. They describe symptoms that together indicate a rheumatic fever. Main criteria include:

  • Joint pain (arthritis)
  • Carditis (heart muscle inflammation)
  • Rash (especially on the trunk)
  • Small nodules under the skin (especially on the elbows, wrists, knees and Achilles tendons)
  • Chorea Sydenham (movement disorder)

In addition, there are some secondary criteria, such as increased levels of inflammation in the blood, fever, ECG changes or evidence of streptococci in recent weeks.

To prove the pathogen, the doctor can perform a special rapid test for acute sore throat. He makes a throat swab that makes it clear within a few minutes whether beta-hemolytic group A streptococci are responsible for the inflammation.

If there are already symptoms of rheumatic fever, but the acute pharyngeal infection has already been cured, there are other ways to detect the pathogens. The so-called antistreptolysin titer (ASL titer) and the anti-DNase B titer (ADB titer) allow the blood to look for signs of an immune reaction against the triggering bacteria.

After a certain decision-making catalog, one can use the Jones criteria to diagnose rheumatic fever. As a general rule, the more factors are met, the more likely a rheumatic fever is, with more emphasis on key criteria. Further clinical and imaging investigations help on the way to the diagnosis. To estimate possible heart damage, the doctor uses ultrasound and electrocardiography (ECG).

Rheumatic fever: treatment

The main element of treatment of rheumatic fever is antibiotic therapy. On the one hand, it significantly reduces the risk of rheumatic fever after infection with beta-hemolytic streptococci. On the other hand, if there are already the first signs of this, one can reduce the further progression of rheumatic fever and a risk of long-term damage by up to 80 percent.The most important antibiotic in the fight against rheumatic fever is penicillin. Depending on the case, other antibiotics such as cephalosporins or macrolides are used.

If the heart is involved, anti-inflammatory drugs such as ibuprofen or naproxen are also used as soon as the diagnosis is confirmed. In addition, steroids are administered if the heart is severely impaired. Whether they bring long-term improvement, or only acutely fight the symptoms is controversial. It is also important that the patients avoid any physical stress.

If there is a long-term closure of the heart valves, surgery may be needed to either reopen the valve or replace it completely. However, such an intervention can be performed at the earliest one year after the acute inflammatory phase.

Depending on the degree of damage to the heart, the patients must also take a long-term antibiotic protection, in severe heart damage even life. This can be given in tablet form, or by syringe every few weeks. The long-term therapy prevents a renewed flare-up of the disease and thus protects against more serious long-term damage.

Rheumatic fever: disease course and prognosis

Disease progression and prognosis of rheumatic fever depend in particular on how fast it is recognized and adequately treated. If an antibiotic treatment already during the pharyngitis, the rheumatic fever can usually be avoided. Even though the rheumatic fever is still in its early stages, the prognosis is good. It usually heals without further problems. The joint complaints sound over a longer period of time.

However, if heart damage has already developed, it can usually no longer be repaired. In addition, the risk of another episode of rheumatic fever increases, which can aggravate the damage. It is therefore advisable to go to the doctor in case of severe sore throat, or fever with joint pain with the ill child and to have a quick streptococcal test.

Rheumatic fever

FAQ - 💬

❓ What is rheumatic fever signs and symptoms?

👉 Symptoms of rheumatic fever redness, pain and swelling of your joints (arthritis), usually ankles, knees, wrists or elbows. pain in your chest, breathlessness and a fast heart rate. jerky, uncontrollable movements in your hands, feet and face. tiny bumps under your skin.

❓ Can rheumatic fever be cured?

👉 Rheumatic fever doesn't have a cure, but treatments can manage the condition. Getting a precise diagnosis soon after symptoms show up can prevent the disease from causing permanent damage. Severe complications are rare. When they occur, they may affect the heart, joints, nervous system or skin.

❓ What are the 5 major criteria in diagnosing rheumatic fever?

👉 The 5 major manifestations of acute rheumatic fever are carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, of which the most common are carditis and arthritis. The Jones criteria were revised in 2015 to include separate criteria for low-risk and moderate- to high-risk populations.

❓ Is rheumatic fever for life?

👉 Proper treatment of strep throat can prevent this life-threatening disease. Rheumatic fever is a complication of untreated strep throat that can be life-threatening. Thanks to antibiotics, rheumatic fever is rare in the U.S. and other developed countries. It can cause permanent damage to the heart and heart failure.

❓ How do you catch rheumatic fever?

👉 Rheumatic fever is not contagious People cannot catch rheumatic fever from someone else because it is an immune response and not an infection. However, people with strep throat or scarlet fever can spread group A strep to others, primarily through respiratory droplets.

❓ Who is at high risk for rheumatic fever?

👉 The incidence of acute rheumatic fever is highest in children between the ages of 5 and 15 years. Acute rheumatic fever is very rare in children 3 years of age and younger in the United States. First-onset acute rheumatic fever is rare in adults, although recurrence may occur through adulthood.

❓ How do they test for rheumatic fever?

👉 Tests for rheumatic fever might include: Blood tests. Blood tests can be done to check for signs (markers) of inflammation in the blood. These tests include C-reactive protein and the erythrocyte sedimentation rate.

❓ What happens when you have rheumatic fever?

👉 Symptoms of rheumatic fever can include: Fever. Arthritis (painful, tender joints), most commonly in the knees, ankles, elbows, and wrists. Symptoms of congestive heart failure, including chest pain, shortness of breath, fast heartbeat.

❓ What is the most common cause of rheumatic fever?

👉 Rheumatic fever is an inflammatory disease that can develop when strep throat or scarlet fever isn't properly treated. Strep throat and scarlet fever are caused by an infection with streptococcus (strep-toe-KOK-us) bacteria. Rheumatic fever most often affects children ages 5 to 15.

❓ How do you test for rheumatic fever?

👉 Tests for rheumatic fever might include:

  1. Blood tests. Blood tests can be done to check for signs (markers) of inflammation in the blood. ...
  2. Electrocardiogram (ECG or EKG). This test records the electrical activity of the heart. ...
  3. Echocardiogram. Sound waves are used to create pictures of the heart in motion.
19 Apr 2022

❓ What part of the body does rheumatic fever affect?

👉 About 5% of those with untreated strep infection will develop rheumatic fever. Rheumatic fever may affect the heart, joints, nervous system and/or skin. In more than half of all cases, it leads to serious inflammatory disease of the valves of the heart.

❓ What causes rheumatic fever?

👉 Rheumatic fever may develop after strep throat or scarlet fever infections that are not treated properly. Bacteria called group A Streptococcus or group A strep cause strep throat and scarlet fever.

❓ What is rheumatic fever (RSF)?

👉 Rheumatic fever is an inflammatory disease that can develop when strep throat or scarlet fever isn't properly treated. Strep throat and scarlet fever are caused by an infection with streptococcus (strep-toe-KOK-us) bacteria.

❓ Can rheumatic fever be prevented?

👉 Rheumatic fever can develop if strep throat and scarlet fever infections are not treated properly. Early diagnosis of these infections and treatment with antibiotics are key to preventing rheumatic fever. Rheumatic fever may develop after strep throat or scarlet fever infections that are not treated properly.

❓ How long does it take to get rheumatic fever?

👉 How You Get Rheumatic Fever. Rheumatic fever may develop after strep throat or scarlet fever infections that are not treated properly. Bacteria called group A Streptococcus or group A strep cause strep throat and scarlet fever. It usually takes about 1 to 5 weeks after strep throat or scarlet fever for rheumatic fever to develop.


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