Brief overview
- Symptoms: Including fever, weakness, fatigue and pain in the large joints
- Causes and risk factors: Certain bacteria, so-called beta-hemolytic group A streptococci
- Diagnosis: Using the Jones criteria, throat swab, blood test, among others
- Treatment: Antibiotic therapy, anti-inflammatory and pain-relieving medication, steroids
- Course of the disease and prognosis: If treated at an early stage, the prognosis is good. Consequential damage (e.g. to the heart) may be irreversible.
- Prevention: Timely antibiotic treatment for streptococcal infection
What is rheumatic fever?
Rheumatic fever is an autoimmune reaction triggered by certain bacteria known as beta-hemolytic streptococci. When infected with these pathogens, the body’s own immune system attacks them and targets certain surface structures of the bacteria.
Once the immune system has formed antibodies against a certain pathogen, these remain in the body for a longer period of time, even if the actual illness has already been cured. The immune system can thus quickly and effectively counter new infections with the same pathogen.
However, it sometimes happens that antibodies not only recognize foreign material, but also mistakenly bind to the body’s own structures, for example the surface of the heart valves. This tissue is thus marked as foreign to the rest of the immune system and a defensive reaction against the patient’s own body occurs. This is called an autoimmune reaction, i.e. a reaction against oneself.
In rheumatic fever, heart, joint and skin cells are particularly affected by the misdirected immune reaction.
How common is rheumatic fever?
Only a very small proportion of people who become infected with beta-hemolytic streptococci go on to develop rheumatic fever.
In countries with good medical care, this complication can often be prevented with the right treatment. In many developing countries, however, rheumatic fever is much more common and is the most frequent cause of heart disease in children.
Worldwide, just under half a million people contract rheumatic fever every year, particularly children and adolescents between the ages of three and 16.
What are the symptoms?
These long-lasting and later-onset symptoms are usually caused by structural damage to the organs, which is difficult to prevent.
Acute rheumatic fever
Acute rheumatic fever usually occurs a few weeks after the streptococcal infection. The disease presents very differently and is not easy to recognize, as not all symptoms always appear equally obvious.
Many sufferers come to the doctor with a fever, headache, weakness and tiredness. Young children sometimes also complain of abdominal pain. Pain in the large joints, such as the knee, hip or shoulder, are also typical symptoms of rheumatic fever. The joints often not only hurt, but are also reddened and swollen.
Finally, the immune system may attack the nervous system during a rheumatic fever. This can lead to personality changes, muscle weakness, balance problems and fine motor skill disorders.
If the brain is affected, a special movement disorder may result, known as Sydenham’s chorea. Children are affected by this neurological syndrome much more frequently than adult patients.
Uncontrolled, aimless movements are typical of Sydenham’s chorea. The children behave clumsily, spilling soup or breaking plates, for example. Unlike heart inflammation, the neurological symptoms usually heal without any consequences. Sydenham’s chorea, for example, usually only lasts for a few months.
What late effects are possible?
Even at an older age, they may then suffer from recurrent attacks with increasing physical limitations. However, it is unlikely that rheumatic fever will affect adults for the first time without having occurred in childhood.
Damage to the heart as a result of rheumatic fever is relatively common and often lasts a lifetime. Up to 60 percent of all those affected show long-term damage to the heart.
This particularly affects patients who are diagnosed too late or have not received treatment. The immune system primarily attacks the heart valves. These function like a valve and ensure that the heart continuously pumps the blood in one direction. If the heart valves become damaged, this leads to chronic overload and ultimately to pumping failure of the heart.
Rheumatic fever: causes and risk factors
The result is a bright red mucous membrane in the throat with small yellow plaques (streptococcal angina). Streptococci are also responsible for the childhood disease scarlet fever, as well as for various skin infections.
Why rheumatic fever occurs in some people after a streptococcal infection and not in others is not fully understood. It is assumed that a certain susceptibility to such a false reaction of the immune system is inherited.
Age is also an important risk factor. Rheumatic fever is much more common in children than in older people. This risk is particularly high between the ages of five and 15, as throat infections with streptococci are more frequent during this period.
Examinations and diagnosis
The doctor always thinks of rheumatic fever when a child or adolescent comes in with a high temperature and joint pain and has also had a sore throat in recent weeks. However, it is not always easy to recognize rheumatic fever, as the symptoms present themselves very differently in many patients.
The so-called Jones criteria, which were developed back in 1944, serve as a diagnostic aid for doctors. They describe symptoms that together indicate rheumatic fever. The main criteria include
- Joint pain due to joint inflammation (arthritis)
- Carditis (inflammation of the heart muscle)
- Skin 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 inflammation levels in the blood, fever, electrocardiographic changes or evidence of streptococci in recent weeks.
If symptoms of rheumatic fever are already present but the acute throat infection has already been cured, there are other ways of detecting the pathogen. With the so-called antistreptolysin titer (ASL titer) and the anti-DNase B titer (ADB titer), signs of an immune reaction against the triggering bacteria can be found in the blood.
The diagnosis of rheumatic fever is made according to a specific decision catalog using the Jones criteria. In general, the more factors that are fulfilled, the more likely it is that rheumatic fever is present, with the main criteria carrying more weight.
Further clinical and imaging examinations help to establish the diagnosis. The doctor uses ultrasound and electrocardiography (ECG) to assess possible heart damage.
Rheumatic fever: treatment
The most important antibiotic in the fight against rheumatic fever is penicillin. Depending on the case, other antibiotics such as cephalosporins or macrolides may also be used. The doctor may also prescribe pain-relieving medication (analgesics).
If the heart is involved, the doctor will also prescribe anti-inflammatory drugs such as ibuprofen or naproxen as soon as the diagnosis has been confirmed. If the heart is severely affected, the doctor will also prescribe steroids. Whether they bring long-term improvement or only combat the symptoms acutely is controversial. It is also important that patients avoid any physical exertion.
If the heart valves become blocked in the long term, an operation may be necessary to either reopen the valve or replace it completely. However, doctors do not perform such an operation until at least one year after the acute inflammatory phase.
It may also be necessary for those affected to take antibiotics for the rest of their lives during invasive, i.e. surgical procedures (for example in the nasopharynx, on the teeth or on the skin). This is to prevent bacteria that temporarily enter the bloodstream from attaching to the heart.
Course of the disease and prognosis
The course and prognosis of rheumatic fever depend in particular on how quickly a doctor recognizes and adequately treats it.
If rheumatic fever is still at an early stage, the prognosis is good. It usually heals without any further problems. The joint pain also subsides over a longer period of time.
However, if heart damage has already occurred, this can usually no longer be repaired. In addition, there is an increased risk of suffering a further attack of rheumatic fever, which may exacerbate the damage.
Prevention
If, in the case of a streptococcal infection, antibiotic treatment is given while the throat is still inflamed, rheumatic fever can usually be avoided.
