Rheumatic Fever and Post-Streptoccocal Reactive Arthritis

What is Rheumatic Fever?
Rheumatic fever is a disease which is caused by the streptococcus bacteria. The disease can cause irreparable damage to the heart. Symptoms include: fever, joint inflammation, pericarditis and nodules on the skin.

In the past, outbreaks which would occur in closed communities led people to (incorrectly) believe that the disease was contagious. There was a dramatic decrease in the disease incidence after people began using penicillin to treat pharyngitis and strep throat.

Most children with rheumatic fever are between the ages of 5 and 15. The disease still affects people in developing countries and is a leading cause of heart problems among young people. In Israel, a few dozen children are diagnosed with rheumatic fever annually.

What causes the disease?
The disease is an abnormal response of a child’s immune system to an angina (caused by streptococcus. In this instance, the immune system attacks the bacteria as well as certain tissues in the body. The connection between the disease and the infection also helps doctors treat the condition and prevent it. Strep throat is a common infection which affects a large part of the population. There is a higher risk for people who have previously had the disease.

Is it hereditary?
There are genetic factors that affect a person’s chances of getting the disease however, it is not hereditary disease.

Why did my child get the disease? Can it be prevented?
A person’s environment and the bacteria are important factors, but in reality it’s hard to predict who will get sick. The disease is the result of an abnormal immune system reaction. There are different strains of streptococcus which are more likely to cause the disease. Prevention is dependent on early detection.

Is it contagious?
The disease itself is not contagious, but strep throat is. These bacteria are transferred from person to person.

What are the main symptoms of the disease?
The disease exhibits a number of symptoms, which vary from person to person. The disease develops after strep throat is left untreated or is not treated properly. Strep throat symptoms include: fever, headaches, sore throats, red tonsils. Additionally, the lymph nodes in the neck are enlarged. Symptoms may go undetected.

Common Symptoms of Rheumatic Fever

There are several common symptoms of rheumatic fever.

  • Arthritis
    Arthritis affects a number of joints all over the body. The inflammation of the joints spreads from one joint to another (less likely to affect the hands and neck). The pain usually passes after taking aspirin or other anti-inflammatory medications.
  • Carditis
    This is the most severe and serious symptom of rheumatic fever. Carditis is suspected when a child’ heart continues to race even when they are resting or sleeping. Listen to the heart in order to detect any murmurs. The severity of the murmurs varies from light to severe, which can indicate damage of the heart valves (endocarditis). In case of pericarditis, fluids may accumulate around the heart. In most cases, the problem goes away on its own, but severe cases (myocarditis), the heart’s pumping capability is damaged leading to heart failure. Symptoms of heart failure include coughing, chest pains, racing pulse and heavy breathing.
  • Chorea
    Chorea is an abnormal involuntary movement disorder, resulting from damage to parts of the brain due to the infection. Chorea affects between 10% and 30% of rheumatic fever patients. Unlike arthritis and carditis, chorea appears relatively late (between one and six months after the original infection). Symptoms include difficulty writing, difficulty performing everyday tasks such as getting dressed, walking and eating (due to involuntary movements). Sometimes, a person can control these movements for a short while. The movements go away during sleep and get worse during stress. At school, chorea can adversely affect your child’s capabilities and achievements (it affects a person’s attention and concentration). The disease itself goes away in about two months but it requires supportive treatment and monitoring.

There are a number of less-common symptoms, which affect the skin:

  • Erythema Marginatum
    These are red rings which on the inner surfaces of the limbs which come and go.
  • Nodules
    These affect less than 5% of the patients and depending on severity, may be missed entirely.

Does Rheumatic Fever manifest the same way for all children?
The most common manifestations of the disease among children are heart murmurs, arthritis and fever. Younger patients may present with carditis without arthritis. Chorea may present by itself without arthritis.

Is Rheumatic Fever different for children than it is for adults?
Rheumatic fever affects young children and adults up until age 25. It is rare for a child to get it before age 3. Over 80% of all patients are between the ages of 5 and 19. The disease may recur later in life when antibiotic treatment isn’t sufficient.

How is Rheumatic Fever diagnosed?
The disease is diagnosed with the help of careful analysis of the clinical symptoms and tests. The clinical measurements needed to diagnose the disease are called “Modified Jones’ Criteria”, after the doctor who first put the criteria together. If a doctor suspects a child is suffering from rheumatic fever, the child is monitored to see if he fits the criteria.

Most of the examinations are vital the correct and timely diagnosis of the disease. Blood tests are done to verify the diagnosis. In most cases, the infection is visible unless chorea is the only symptom. Evidence of a streptococcal infection is important for a proper diagnosis. A throat culture is not enough to determine the existence of the streptococcal bacteria. There are a number of tests which can check if there are streptococcal antibodies in the blood. However, children who only exhibit chorea will not have an increase of streptococcal antibodies in the blood, and therefore this test is not always accurate.

Diagnosing Carditis
A new murmur, caused by an infection of the heart is the most common symptom of carditis and is generally discovered by a doctor. An EKG is effective in checking the involvement of the heart in the disease, and a chest x-ray can help evaluate the size of the heart. None of these exams are painful or cause any discomfort to your child.

Is the disease treatable?
The disease presents an important medical problem, which can be prevented in certain areas in the world. Treating strep throat can prevent the disease altogether. Once diagnosed, the doctor will prescribe antibiotics. The strep infection is also treated in case there are any bacteria still present in the body. The doctor will administer a shot of 1.2 million units of penicillin which will destroy the bacteria and protect the child’s body for 3-4 weeks. Children who’ve had rheumatic fever in the past must get the penicillin treatment every three weeks to prevent a recurrence of the disease.

Parents need to be extra-supportive when the child exhibits chorea. Most medications prescribed to fight the disease have side effects (drowsiness, and shaking). Sometimes, chorea can last for several months even with proper treatment.

Side Effects
Assuming the medication treatment of non-steroid medications does not need to last for an extended period of time, the side effects are minimal. Steroid-based medications, on the other hand, can carry more serious side effects, such as weight gain, swelling, acne, stretch marks and hair growth. It is important to monitor penicillin to determine if your child has an allergic reaction to it. Penicillin shots are painful and are generally accompanied with a local anesthetic.

A recurrence is most likely to happen the first three-five years after the disease. Additionally, the risk of heart disease is also higher. Therefore prevention methods are recommended to patients regardless of the severity of their disease. Most doctors agree that antibiotics should be administered for five years after the infection. Children who suffered from heart damage should take antibiotics until age 40.

Tests and Monitoring
Periodical monitoring and lab tests are required frequently. Close monitoring is recommended in cases of carditis and chorea. After the symptoms subside, we recommend you build a monitor plan for your child together with your doctor. The symptoms will subside eventually, but there is always the risk of recurrence. It is higher during the first five years following the original infection.

Recurrences tend to be unexpected in terms of timing and severity. The appearance of carditis during the first infection increases the risk of a more severe heart infection. There are cases where a carditis patient has a full recovery. Sometimes, surgery is required to repair damage caused to the heart. Full recovery is possible, unless carditis caused significant damage to the heart valves.

Living with Rheumatic Fever
Family support is very important during an outbreak of the disease, especially for children suffering from chorea and carditis. If the disease passes and there is no evidence of damage to the heart, there are no special recommendations for everyday activities. The main concern is the response to the long-term preventative antibiotics.

Post-Streptococcal Reactive Arthritis
This is a type of arthritis found among children and adults, which can appear after a strep infection and does not fulfill all of the Jones’ Modified Criteria for rheumatic fever. It appears early on during the disease and generally affects the hands. It does not respond well to anti-infection agents and can last several months. Some patients may develop carditis later on, and most doctors agree on the need to prescribe preventative antibiotics.