Rheumatic fever causes inflammation of the heart, joints, skin, or brain. The condition occurs as a result of an untreated bacterial infection with group A streptococcus.
What is rheumatic fever?
Rheumatic fever, also called streptococcal rheumatism, is a secondary disease of an upper respiratory tract infection that has become rare in our latitudes. The disease occurs predominantly in childhood. The damage often only becomes apparent decades later. The symptoms of rheumatic fever appear a few weeks after a throat infection has been overcome and manifest themselves in high fever and pain and swelling in the knee, foot or elbow joints. A reddish skin rash also indicates rheumatic fever. Furthermore, conjunctivitis or tenosynovitis may occur. If the heart muscle is affected, there is chest pain and shortness of breath.
Causes
Rheumatic fever is caused by streptococcal infection, which occurs as a late consequence of strep throat. Tissue damage occurs because of an exaggerated defensive reaction by the body’s immune system. The antibodies produced by the body react with the body’s own cells. A few decades ago, the disease was relatively widespread. Many elderly people suffer from mitral valve stenosis as a result of an infection in childhood. The narrowing of the mitral valve results in reduced blood flow in the heart. Those affected suffer from fatigue, shortness of breath and palpitations. Even today, the disease is widespread in developing countries. Inferior nutrition, a concentration of many people in a small space, and untreated pharyngitis and tonsillitis favor the occurrence of rheumatic fever.
Symptoms, complaints, and signs
Symptoms of rheumatic fever can affect several areas. There may be discomfort in the skin, joints, heart, or brain. It often begins with a flu-like condition. Patients suffer from fever and a general feeling of weakness. In children, abdominal pain is often added. Various symptoms are possible on the skin. Relatively often, erythema nodosum develops on the lower leg. These are inflamed and painful red spots with nodules that form on the shin. In appearance, they resemble bruises. It is also possible for skin patches to appear on the abdomen and back. In addition, so-called rheumatoid nodules may form on the arms and legs. Arthritis in the large joints is another symptom. It usually begins symmetrically at the knee and ankle joints, then jumps to other joints and triggers severe pain. If the heart is affected, inflammation develops either in the heart muscle tissue (myocardium) or in the inner lining of the heart (endocardium). This can lead to dysfunction of the heart valves, arrhythmias or tachycardia. The death of microscopic tissue particles or the formation of so-called Aschoff nodes on the heart muscle are also possible. On listening, this is noticeable in altered heart murmurs. Eventually, encephalitis may develop in the brain, manifesting as apathy, anxiety, and undirected, involuntary movements (Sydenham’s chorea).
Diagnosis and course
In adults, rheumatic fever is difficult to diagnose because it presents with a rather atypical clinical picture. A physical examination is necessary. If rheumatic fever is suspected, a throat swab is taken and examined for streptococcal infestation. A chest x-ray will show whether there has been any enlargement of the heart as a result of the inflammation. Echocardiography uses ultrasound to visualize the heart valves and the inside of the heart. The physician will order a blood draw. The laboratory results will reveal an elevated leukocyte level and an elevated number of antibodies to streptococci if an infection is present. The Jones criteria published by the American Heart Association in 1992 are used to diagnose rheumatic fever. The course of the disease begins with tonsillitis or pharyngitis that has not been treated. This is followed by one to three symptom-free weeks. After that, the rheumatic fever occurs, which lasts up to twelve weeks. The disease is very protracted. If the heart valves are inflamed, scarring can occur years later.About half of all patients develop chronic rheumatic heart disease.
Complications
Due to rheumatic fever, malfunction of the heart valves is the main complication. The disease is the most common cause of permanent heart disease such as valvular heart disease and, by extension, heart failure. In the classic course, which usually occurs in childhood, inflammation of the inner lining of the heart is possible. In a severe course, cardiac arrest and death of the patient may occur. The joints are usually also affected, becoming inflamed, swollen and causing severe pain. In general, rheumatic endocarditis is accompanied by severe physical malaise. The affected person usually suffers from chronic pain, sweating and high fever – each of these symptoms is associated with further complications. In the long term, the disease impairs psychological well-being and can promote conditions such as depression or anxiety disorders. Treatment with the antibiotic agent penicillin can cause skin rashes, nausea and vomiting, and loss of appetite. Mucosal inflammation, dry mouth, and impaired sense of taste are common. Occasionally also anemia and transient liver dysfunction. Acetylsalicylic acid, used to inhibit inflammation, can cause heartburn, dizziness, and gastrointestinal bleeding, among other symptoms. The use of cortisone and immunosuppressants can also trigger a range of side effects.
When should you see a doctor?
If fever, rash, and joint pain occur, rheumatic fever may be underlying. A visit to the doctor is recommended if symptoms persist after one to two days. If other signs of illness appear, such as chest pain or the typical jerky movements, a medical professional should be consulted. Rheumatic fever mostly occurs in connection with a streptococcal infection. Children between the ages of five and fifteen also belong to the risk groups and should be treated by a physician when the above-mentioned symptoms occur. Other favoring factors are unhygienic living conditions and an unbalanced diet. Rheumatic fever is treated by the family doctor or an internist. In the case of any skin changes, the dermatologist is the right person to contact. If the condition is treated during the initial pharyngitis, rheumatic fever can often still be averted. In contrast, damage to the heart that has already occurred cannot be repaired and also increases the risk of further episodes of the disease. Therefore, in case of severe sore throat with fever and joint pain, the general practitioner or pediatrician should be involved immediately. The physician can perform a rapid streptococcus test and thereby quickly make the diagnosis.
Treatment and therapy
The patient is prescribed strict bed rest for about fourteen days. Drug treatment is started immediately. Penicillin is given to kill the streptococci and should be given over a period of ten days. If there is an allergy to penicillin, macrolides are given. Anti-inflammatory drugs reduce fever and relieve joint inflammation. Common active ingredients here are ibuprofen, indometacin or piroxicam. Corticosteroids are prescribed against the inflammation of the heart. These active ingredients are similar to the body’s own hormones. The inflammation is relieved by the active ingredient inhibiting the formation of the natural substances that ultimately trigger the inflammation. At this point, cortisone, prednisolone or dexamethasone should be mentioned. The duration of drug treatment depends on the severity of the disease. As a rule, medication is prescribed up to the age of 21 or up to five years after the last occurrence of rheumatic fever. In severe cases, drug treatment is recommended for up to ten years after the onset of rheumatic fever.
Prevention
Since sore throats have been treated with antibiotics, the occurrence of rheumatic fever has become a rarity in Western industrialized countries. As a preventive measure, febrile illnesses of the upper respiratory tract should not be taken lightly. The doctor will decide to what extent the administration of antibiotics is necessary.Recurrence of rheumatic fever can be prevented by taking small doses of antibiotics for several years after the last occurrence of the disease.
Follow-up
Generally, after the disease has resolved, long-term therapy with monthly, intramuscular benzathine penicillin is given as a follow-up treatment until the age of 25; however, penicillin can alternatively be given orally. If cardiac or joint impairments persist, rehabilitation in the form of physical therapy is also recommended. In any case, regular cardiac examinations are prescribed. In general, children and young adults with healed rheumatic fever are at high risk for recurrence (relapse) over the next five years or for life. Carditis could develop with any further upper respiratory illness caused by A streptococcus; without further follow-up, the risk is as high as 20 percent. Therefore, depending on whether rheumatic fever with or without carditis was already present, they are given antibiotic prophylaxis for five (without carditis) to ten years (with carditis) after completed therapy. In the case of a permanent valvular defect, antibiotic follow-up treatment is sometimes prescribed until the age of 40 or sometimes for life. In the case of surgical procedures, endocarditis prophylaxis should also be administered. For environmental prophylaxis, it is recommended that all family members of the affected person have a throat swab to detect the streptococcus A group. If positive, antibiotic treatment is also prescribed for the affected family members.
Here’s what you can do yourself
This disease, which has become rare in Germany, belongs in the hands of family physicians, pediatricians, or internists. If the skin is affected, patients should also consult a dermatologist. If rheumatic fever is diagnosed, it is extremely important for patients to take the medications prescribed by their doctors, otherwise it can irrevocably damage the heart or even be fatal. It is also essential to adhere to the prescribed bed rest. Because those affected are usually children, parents must pay close attention to following the doctor’s instructions here. This may be made more difficult by the fact that the drugs commonly used for treatment can trigger strong side effects that prevent compliance. However, children in particular have to take the medication for a longer period of time, usually until they are 21 years old. Because the disease is the result of streptococcal infection, hygienic measures are often required. This absolutely includes cleaning the environment, as the bacteria are found on surfaces that have not been cleaned, faucets and door handles. Food can also contain streptococci. Therefore, in a household where a patient has rheumatic fever, food should be washed thoroughly before use. Incidentally, the same applies to the hands of all family members. Frequent washing can prevent infection and reinfection.
