Rheumatic Fever Affects Children: Causes and Manifestations

Rheumatic fever is an acute multisystem inflammatory disease that affects and damages connective tissues. It is multisystemic because it affects multiple systems of the body, most commonly joints, heart, but also the nervous system and skin.

It occurs after an infection of the upper respiratory tract and the pharyngeal tonsils caused by streptococcus.

It most commonly occurs in pre-school and school-age children aged 5 to 15 years.

In the past, the disease used to be common, especially in children living in poor socio-economic conditions. In developed countries, the incidence rate is currently declining.

This is aided by better hygiene, especially by the availability of antibiotic treatment for streptococcal upper respiratory tract diseases and tonsillitis.

The first mentions of the disease appear as early as the 16th century.

However, it was not until William Charles Wells published information in 1812 that linked rheumatic fever to heart problems.

In 1889, Cheadle described the clinical course, and in 1904, the causative agent of the disease, Micrococcus rheumaticus (Streptococcus), was isolated from the patient.

Streptococcal infection is the cause of rheumatic fever.

It is caused by beta-hemolytic streptococcus from group A. Thus a rheumatogenic serotype of pyogenic streptococcus. Infection with this bacterium most often occurs in the upper respiratory tract and as streptococcal angina.

TIP: Learn more in the article on sore throat.

The course of this infection may sometimes be mild and unnoticed.

Complications with non-specific symptoms arise later on. Based on common signs, i.e. the course and symptoms, the doctor is able to make a diagnosis of rheumatic fever.

Rheumatic fever, also called febris rheumatica, is caused by a streptococcal infection. However, other risk factors also contribute to its emergence.

The emergence of the disease is affected by risk factors such as:

• upper respiratory tract and tonsil infection caused by streptococcus pyogenes
• immunopathological response to infection
• individual predisposition
• immunological factors
• toxic mechanisms

Antibodies produced by the immune system overreact to the presence of the pathogen, as a result of which the body's own tissues also negatively affect and destroy it. The autoimmune component thus most often acts on connective tissues and other tissues that are located throughout the body.

Therefore, this disease is referred to as systemic or multisystem.

Disability, ie also damage, is manifested on the cardiovascular, nervous system, but also on the joints and skin. The same is true for kidney damage, where the cause is a nephritogenic serotype.

Table: Rheumatogenic and Nephritogenic Serotypes of Streptococci

The disease is not heritable, although there may be some familial genetic predisposition in certain cases.

The disease as such is not infectious.

However, streptococcus is transmitted from person to person through the infected saliva and airways. These risk factors, and thus the individual's individual susceptibility, subsequently contribute to the outbreak of rheumatoid fever.

Poor hygiene or neglected treatment is the cause of the spread of the infection. The social environment, i.e. larger congregations of people, helps transmit the bacteria, especially in kindergartens and schools.

In this case, too, it is important to maintain a sufficient hygienic standard, and therefore to wash your hands thoroughly. Home treatment and avoiding contact with other people is of great importance in preventing the spread of streptococcal diseases.

The disease is multisystemic, so its manifestations can be found on several systems of the body.

It most commonly occurs in children from 5 to 15 years of age. It occurs 10 to 20 days after an upper respiratory tract infection or tonsillitis caused by streptococci.

General symptoms of the disease include:

• tiredness
• weakness
• sweating
• increase in body temperature to fever
• headaches
• stomach ache
• loss of appetite
• nosebleed, i.e. epistaxis

Subsequently, the disease manifests itself depending on the disability of the body system. The disease most commonly affects:

• joints
• cardiovascular system
• nervous system
• skin

Initially, the disease manifests itself mainly in the joints, such as polyarthritis, i.e. inflammation of the joints, in up to 75% of cases. Heart failure is present in 35-50% of cases. The neurological form may present with a late symptom sometimes called St Vitus' dance.

St Vitus' dance, also known as Sydenham's chorea, is manifested in 10-15% of cases in children. Involuntary movements, speech and swallowing disorders are typical. It is often confused with a behavioural disorder and tics, with difficulties subsiding and rarely lasting for months.

Table: Symptoms in Individual Systems

The diagnosis of rheumatic fever is based on the clinical picture, medical history of overcome streptococcal respiratory tract infection or tonsillitis.

Subsequently, physical examinations are performed, mainly by listening to heart sounds and the presence of murmurs.

CRP, sedimentation, or FW, and blood count or ASLO are performed as ancillary laboratory tests. Swab culture is important if upper airway inflammation persists.

ECG and echocardiogram are important in heart problems. However, there is no clear symptom or test for the diagnosis.

Table: Revised Jones Criteria for the Diagnosis of Rheumatic Fever

Rheumatic fever usually occurs 10 days to two weeks after angina or other streptococcal inflammation of the upper respiratory tract (throat, nasopharynx).

Especially in cases where the infection has not been adequately treated with antibiotics.

The combined risk factors give rise to an autoimmune response, which results in damage to the body's own tissues. It occurs as a sterile inflammation in the affected tissue.

Rheumatic fever usually occurs 10 to 20 days after inflammation.

Fever, weakness and fatigue, sweating come first. There may be some headache and abdominal pain. Later on, issues with joints and the cardiovascular system appear during the later course of the disease.

It is typical for joint involvement that inflammation affects one or more joints. Inflammation is migratory and short-lived. There is significant joint pain, impaired joint function, i.e. issues with joint mobility. But also fever, redness and swelling in the affected joint.

It responds very well to treatment with non-steroidal anti-inflammatory drugs.

Consequently, heart disease is common. And t manifests itself depending on which part of the inflammation damages. In pericarditis, there is friction murmur, which the doctor hears during the examination. In endocarditis, there is a murmur that results due to an issue with the heart valves.

Cardiac disorders also include irregularities in the heartbeat. There is tachycardia when a person resting or sleeping, not just when under strain or exertion.

Heart rhythm disturbance can be perceived as palpitations. A serious complication is heart failure. In some cases, permanent heart damage and thus rheumatic valve defect persists.

Skin manifestations are less common, especially in the form of rashes. The presence of erythema marginatum (erythema annulare) is typical. It involves pink or red rings, a circular rash, with a whitish centre.

When subcutaneous nodules occur, it is also associated with heart disease. The nodules are movable relative to their surroundings and are painless. The skin colour on the site is normal. They are most often found around the joints and on the nape.

Neurological symptoms occur as late symptoms of the disease, especially as chorea.

Chorea is also called St. Vitus' dance because "chorea" is a Greek word that means "a dance". Chorea is an abnormal involuntary movement disorder. It is associated with speech disorders, difficulty swallowing, issues with motor skills and walking.

These issues are exacerbated by fatigue and stress.

However, they recede during rest and sleep. Other manifestations include impaired concentration, mood and anxiety.

Over time, neurological problems resolve spontaneously. The course of the initial disease may be mild and unnoticed. The disease often relapses, i.e. returns.