Acute rheumatic fever (ARF) and rheumatic heart disease (RHD)

Notification requirement for ARF and RHD

Medical practitioners who reasonably believe that a patient has, or may have, ARF or RHD must notify the Department of Health in writing within five business days. This is a Victorian statutory requirement.

The treating doctor may be asked to complete a confidential questionnaire by the department to collect additional information. This is used for the detection of disease trends and program development.

Primary school and children’s services centre exclusion for ARF and RHD

Exclusion is not required.

Infectious agent of ARF and RHD

ARF is primarily (but not exclusively) a disease of childhood that occurs as a complication following an infection with the Group A Streptococcus bacterium (GAS), also known as Streptococcus pyogenes). If untreated, GAS pharyngitis (‘strep throat’) can lead to ARF.

RHD develops after one or more bouts (episodes) of ARF during a person’s childhood or adolescence. However, RHD can also develop following subclinical or unrecognised ARF.

Both ARF and RHD are medical conditions caused by GAS but are not themselves infectious diseases.

Identification of ARF and RHD

Clinical features

Clinical features of ARF may include:

• Arthritis and/or arthralgia
• Sydenham chorea
• Carditis (murmur, cardiac enlargement, decompensation, pericardial rub, or echocardiographic changes)
• Prolonged P-R interval and other electrocardiographic rhythm abnormalities
• Subcutaneous nodules
• Erythema marginatum
• Fever
• Elevated acute-phase reactants (C-reactive protein or erythrocyte sedimentation rate)

Patients with RHD have chronic valvular heart disease. Early in the disease, RHD may not cause any symptoms. Later, patients may have a range of symptoms, including reduced exercise tolerance and breathlessness. Complications of RHD include stroke, heart failure, and arrhythmia.

People with RHD often need cardiac surgery to replace or repair their heart valves. Worsening of symptoms and both cardiac and obstetric complications can occur during pregnancy.

Diagnosis

Diagnosis of ARF is based on clinical features, pre-existing risk, and laboratory evidence of a preceding GAS infection, as described in the Revised Jones Criteria.

Diagnosis of RHD is based on echocardiographic findings and interpretation by a cardiologist.

Detailed information on diagnosis and management of these conditions can be found in The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3.2 edition, March 2022).

Public health significance and occurrence of ARF and RHD

Until the mid-20^th century ARF was a common cause of morbidity in the general child population in many countries. Subsequent reductions in ARF have been attributed to improvements in housing and socioeconomic conditions, better access to healthcare, and improved access to penicillin for treatment of streptococcal infections. However, higher rates of ARF and RHD persist in conditions of poverty and poor access to preventive services.

ARF and RHD are preventable conditions disproportionately affecting certain priority populations in Victoria, particularly Aboriginal and Torres Strait Islander people and Pacific Islander people. Children and adolescents from these communities are significantly overrepresented as patients. Immigrants from developing countries, including refugees and asylum seekers, are also at increased risk. Having a personal or family history of ARF or RHD also increases a person’s risk of developing these conditions.

In Victoria, ARF and RHD disproportionately impact Aboriginal and Torres Strait Islander children as well as Pacific Islander children, leading to health disparities and adverse outcomes. Recent research found that Aboriginal and/or Torres Strait Islander people were 10 times more likely, and Pacific Islander people 82 times more likely, to have an episode of ARF than people of other ethnicities.

ARF and RHD may lead to many years of disability over the course of a person’s lifetime, due to ARF in childhood, followed by heart disease and associated complications throughout adulthood. RHD is an important cause of premature mortality amongst affected populations.

Reservoir, mode of transmission, and period of communicability

See streptococcal disease (Group A beta-haemolytic streptococcus)

Incubation period of ARF and RHD

The interval between GAS infection and onset of ARF varies depending on:

• ARF type which can manifest as one or a combination of carditis, arthritis, chorea, erythema marginatum or subcutaneous nodules.
• factors such as host immune response and whether the episode is primary or a recurrence.

In general, the following applies:

• Rheumatic carditis or arthritis – 2-3 weeks after GAS infection but can be as early as 1 week in recurrent ARF
• Sydenham’s chorea – 6-9 weeks after GAS infection.

RHD often has a long asymptomatic phase and may go undetected for many years.

Susceptibility and resistance to ARF and RHD

In Australia, the populations at highest risk of these conditions include:

• Aboriginal and Torres Strait Islander people, particularly those from northern Australia
• People of Māori or Pacific Islander background
• Immigrants from developing countries.

People at risk of ARF are:

• Children aged 5–14 years, with a peak at around 8 years
  • It is rare for ARF to occur in 3 year-olds 18 or > 40 year-olds, although recurrent ARF can occur beyond 40 years-old.
• People living in a high-risk community
  • A high-risk community is one where high rates of ARF (incidence >30/100,000 per year in 5–14 year olds) or RHD (all-age prevalence >2/1000) are present (Table 2).
• Those with increased risk of exposure to GAS infection include those living
  • in crowded living conditions
  • with inadequate access to ‘health hardware’ within homes or communities (i.e., the physical equipment necessary for healthy, hygienic living, including access to bathing and facilities to wash clothes and bedding)
  • with lower levels of health literacy
  • with socioeconomic disadvantage.
• Those with an inherent susceptibility to the autoimmune response
  • evidence for genetic susceptibility is growing and remains under investigation in the Australian context and globally.

Both ARF and RHD are more common in females than males. RHD commonly presents during pregnancy due to symptoms resulting from added stress on the heart.

Control measures for ARF and RHD

Preventive measures

The current cornerstone of ARF/RHD preventive activities in Australia is secondary prophylaxis with an antimicrobial agent effective against GAS for those with diagnosed prior ARF/RHD.

Levels of prevention for ARF/RHD and its sequelae include:

• Primordial – improved social determinants of health
• Primary – treatment of GAS infection (no vaccination currently available)
• Secondary – antimicrobial prophylaxis after ARF e.g., intramuscular benzathine penicillin G every 21 – 28 days
• Tertiary – medical and surgical management of RHD, and consideration of echocardiographic screening in selected populations such as in pregnant women.

Primordial and primary preventive strategies classically refer to disease prevention in unaffected hosts and the general community however, these same messages are equally important to provide to individuals after ARF/RHD diagnosis to potentially reduce their risk of recurrences; a new episode of ARF is estimated to be 10 times more common in an individual with a past episode of ARF than in those of similar age and living in the same community/circumstances, but without prior ARF.

There is currently no vaccine against GAS.

Control of case

No precautions or exclusions are required.

Treatment

In general, patients with suspected ARF should be admitted to hospital as soon as possible to facilitate timely and accurate diagnosis, assess for RHD, provide treatment, and arrange appropriate follow-up.

ARF and RHD patients should be linked to appropriate ongoing care; this may include regular antibiotic treatment (‘secondary prophylaxis’) against GAS infection, follow-up echocardiography, and specialist and dental review, in accordance with national guidelines, ARF classification, and assigned priority status.

Family should be actively involved in care where appropriate. Age-, culture- and language- appropriate education and resources, including on strategies to reduce further GAS transmission, should be provided to patients, families, primary carers.

Control of contacts

No precautions or exclusions are required. Household contacts should be included in education around activities to reduce GAS transmission.

Control of environment

Individuals diagnosed with ARF and//or RHD should be supported to improve their living conditions where possible.

Outbreak measures for ARF and RHD

Outbreak management is dependent on the setting and specific disease. Seek advice from the relevant Local Public Health Unit or the department.