RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE IN CHILDREN
What is acute rheumatic fever?
One of the most common infections a child may acquire is sore throat, also known as pharyngitis. There is a range of infectious agents that can cause sore throat, most frequently in children 5-15 years of age. Among these, group A streptococci (GAS) is unique: when left untreated, it can predispose some children to develop rheumatic fever. This is an illness characterized by fever and inflammation of various organs of the body, due to an untreated (or improperly treated) GAS infection. Around two thirds of patients with rheumatic fever previously had an upper respiratory tract infection.
Acute rheumatic fever occurs because some parts of GAS bacteria look similar to natural proteins found in the heart and other areas of the body. When the body’s immune cells encounter group A streptococci, they remember the unique protein structure of some parts of the bacteria, in order to protect the body from future attacks. Some of these immune cells may come into contact with normal (similar looking) proteins around the body and start attacking these structures, leading to inflammation.
What about rheumatic heart disease?
Patients who develop rheumatic fever are at risk for developing rheumatic heart disease (RHD) — an inflammation of the heart valves or the innermost layer of the heart, or both. In some children, rheumatic heart disease can develop very slowly, with symptoms showing up in as long as 20 years from the last episode of rheumatic fever. Rheumatic heart disease is the most common acquired heart disease in children.
In the following paragraphs, we’ll discuss more important information about acute rheumatic fever and rheumatic heart disease, such as signs, symptoms and possible management options for children with these conditions.
A. Acute Rheumatic Fever
Signs and symptoms
Acute rheumatic fever is diagnosed following a strict set of criteria. Some signs and symptoms weigh more than others, based on how frequent these were seen in previously affected children.
Major clinical signs and symptoms include inflammation of the heart (carditis), inflammation of joints (polyarthritis), involvement of the nervous system (Sydenham chorea), and skin inflammation (erythema marginatum and subcutaneous nodules).
Around half of patients diagnosed with rheumatic fever develop carditis, which is inflammation of the heart. Children may complain of chest pain or get tired easily, and present with an increased heart rate and unusual heart sounds. Chest x-rays may show an increased heart size, while an ECG may show an irregular heartbeat.
Multiple joint inflammation, also known as polyarthritis, is more commonly seen in acute rheumatic fever as compared to carditis. Children develop red, swollen, very painful joints that may be warm to touch. The knees, ankles, elbows, and wrists are the most frequently affected joints. Initially, symptoms last for 1-3 days. As one set of joints become much better, other joints in the body start to become affected in a similar way. This is the “migratory” nature of polyarthritis in acute rheumatic fever.
In Sydenham chorea, children have difficulty controlling spontaneous and involuntary movements, which occur only when they are awake. There may be difficulty in writing properly and in gripping objects (such as utensils and pencils). Unusual darting movements of the tongue and a spooning motion of the hands (as the arms are extended) may also be observed. These movements are accompanied by fleeting changes in emotions and inappropriate behaviors. Symptoms of Sydenham chorea can last for months, even as long as 2 years.
Skin involvement includes erythema marginatum or erythema annulare. This is a pinkish-red, non-itchy rash with a pale center, found at the trunk and arms. On the other hand, subcutaneous nodules are small, firm, painless masses that are commonly seen in areas near bony prominences.
Some minor signs and symptoms help support a diagnosis of acute rheumatic fever. These include painful but non-swollen joints (known as arthralgia), fever of at least 101 degrees Fahrenheit (or 38.5 degrees Celsius), elevated markers of inflammation (determined through blood tests), and certain abnormalities in an ECG tracing.
It is important to establish the connection between a previous case of pharyngitis and the subsequent development of acute rheumatic fever. In this case, there should be a documented previous episode of GAS pharyngitis, roughly 2-3 weeks prior, via a positive throat culture or a rapid streptococcal antigen test.
The main points of therapy for children with acute rheumatic fever include antibiotics and anti-inflammatory medications, on a case-to-case basis.
As mentioned earlier, children with acute rheumatic fever have a high chance of developing further inflammation in the heart. Recurrent attacks with group A streptococci may further increase this possibility. Long-term use of antibiotics as a form of prophylaxis reduces this risk. The duration of treatment may depend on several factors and is decided on by a qualified healthcare provider.
B. Rheumatic Heart Disease
Acute rheumatic fever can progress to rheumatic heart disease (RHD) in the form of small masses that form along the heart valves. These lesions, called verrucae, are a combination of blood cells and proteins that are found near borders of heart valves. As the inflammation dies down, areas affected by the verrucae become scarred, which in turn affect the mobility and efficiency of the heart. When secondary or recurrent attacks occur, new verrucae are prone to form near these scars, causing further damage. Overall, these lesions can lead to chronic heart conditions.
Signs and symptoms
RHD is confirmed using echocardiography, which is an ultrasound of the heart. Accompanying signs and symptoms include difficulty breathing, difficulty performing exercise, chest pain, swelling of the legs, increased heart rate and blood pressure. Examination by a healthcare provider may reveal unusual heart sounds (such as murmurs and extra heart sounds) that are heard through a stethoscope. An increased heart size may be seen on chest x-ray.
Treatment options for RHD include antibiotics, corticosteroids, heart medications for unusual heart rhythms, and surgery. Not all patients receive the same type of treatment. Qualified healthcare providers determine which treatment options may be given on a case-to-case basis.
Acute rheumatic fever is an inflammatory condition affecting multiple organs, especially the heart. As this is due to a specific bacterial infection, proper and timely clinical management of a child with a suspected GAS pharyngitis can help prevent the development of rheumatic fever. Similarly, prompt treatment of a child with acute rheumatic fever may halt the progression to rheumatic heart disease, which is often a chronic, lifelong condition.
- Kliegman, R.M. et al. (2020). Chapter 210 Group A Streptococcus. Nelson Textbook of Pediatrics 21st edition. Elsevier.
- Kliegman, R.M. et al. (2020). Chapter 465 Rheumatic Heart Disease. Nelson Textbook of Pediatrics 21st edition. Elsevier.
- Sika-Paotonu D, Beaton A, Raghu A, et al. (2017). Acute Rheumatic Fever and Rheumatic Heart Disease. In: Ferretti JJ, Stevens DL, Fischetti VA, editors. Streptococcus pyogenes: Basic Biology to Clinical Manifestations. University of Oklahoma Health Sciences Center. Taken from: https://www.ncbi.nlm.nih.gov/books/NBK425394/
- Langlois, D.M. & Andreae, M. (2011). Group A Streptococcal Infections. Pediatrics in Review October 2011, 32 (10) 423-430.
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