Rheumatic fever and rheumatic heart disease: updates for clinical practice

SUMMARY

Rheumatic heart disease is the most common cause of acquired childhood cardiac disease globally and, in Australia, affects First Nations peoples at disproportionately high rates.

Australian guidelines recommend that skin and throat infections, which can be due to Group A Streptococcus, should be managed with antibiotics in high-risk individuals to prevent rheumatic fever and rheumatic heart disease.

Regular long-acting intramuscular penicillin is recommended for secondary prevention of rheumatic fever. Injections are painful and should be administered by competent clinicians using strategies and techniques for minimising pain and distress.

The Australian Guideline for the Prevention, Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease (2025) includes recommendations for clinical care delivered within a cultural framework that puts the person and their family at the centre of care.

 

Introduction

Rheumatic fever is a serious outcome following infection with Streptococcus pyogenes (Group A Streptococcus, ‘Strep A’). In some individuals, superficial (throat or skin) Strep A infection triggers rheumatic fever, which is an autoimmune response 2 to 3 weeks after infection. Historically it was considered that this occurred in only about 3% of people¹ but more recent data from high endemic regions suggest 5 to 10% may develop rheumatic fever after Strep A infection.² Recurrent episodes of rheumatic fever can result in progressively severe rheumatic heart disease from cumulative heart valve damage. Rheumatic heart disease may also result from a single, severe episode of rheumatic fever.

Almost 12,000 people in Australia have had rheumatic fever and approximately 7500 live with rheumatic heart disease.³ First Nations communities in central and northern Australia and some migrant groups, particularly from the Pacific region, are disproportionately affected.^3,4 Many healthcare providers have little experience with rheumatic fever and rheumatic heart disease, and may be unaware of the large burden and devastating impact of these conditions in affected communities in Australia.

Prevention of rheumatic fever and rheumatic heart disease can be implemented at all stages of the disease trajectory. Primordial prevention addresses underlying socioeconomic disadvantage. Primary prevention comprises early treatment of Strep A infections (pharyngitis, tonsillitis and impetigo) to avert subsequent rheumatic fever, and secondary prevention focuses on preventing recurrent rheumatic fever to avert morbidity from rheumatic fever and development of rheumatic heart disease.

This article provides a brief overview of the epidemiology, prevention, diagnosis and management of rheumatic fever and rheumatic heart disease, with a focus on changes clinicians need to apply (Box 1), based on the 2025 Australian Guideline for the Prevention, Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease (the Australian guideline).⁵

 

Risk of rheumatic fever

Rheumatic fever is a disease of socioeconomic disadvantage.

Who is at risk

In Australia, rheumatic fever is a disproportionate burden for First Nations peoples. This is primarily a product of the ongoing impact of colonisation with marginalisation and systemic racism driving socioeconomic disadvantage, inadequate housing (including limited sanitation infrastructure) and limited healthcare access. From 2016 to 2021, rates among First Nations peoples ranged from 52 to 69 per 100,000 compared to 2.7 per 100,000 among all Australians.³ Aotearoa New Zealand Māori and other Pacific peoples are also over-represented on Australian rheumatic heart disease registers, particularly in eastern Australia.

Rheumatic fever is most common in children aged 4 to 15 years but continues to be seen in young adults aged into their 30s.³ Cases aged less than 4 years are less common but documented.⁶ The incidence is similar between males and females during childhood, although females experience a higher incidence after adolescence.

Rheumatic fever and rheumatic heart disease are notifiable conditions in the Northern Territory, Western Australia, Queensland, South Australia, New South Wales and Victoria. Dedicated control programs in these jurisdictions (excluding Victoria) provide clinical education and are central to disease notification, patient care coordination and epidemiological reporting.

Assigning risk level

Determining an individual’s risk for developing rheumatic fever (their risk category) is an important first step in guiding the decision to treat a potential Strep A infection and in considering a diagnosis of rheumatic fever. For an individual at high risk of rheumatic fever, a low threshold to use antibiotics when managing potential superficial Strep A infection (sore throat, impetigo) is needed. Similarly, rheumatic fever should be considered for a young person in a risk group presenting with one or more of unexplained fever, joint pain, breathlessness, chest pain or chorea, which may all characterise rheumatic fever.. However, determining risk is imprecise and requires an understanding of the context of the patient’s life and informed judgement from the healthcare provider. Factors that increase risk of rheumatic fever for an individual include a family history of rheumatic fever or rheumatic heart disease, and recurrent streptococcal infections during early childhood. Recurrent streptococcal infections are more common for children living in crowded households, with reduced access to sanitation and reduced access to or utilisation of health care. These factors may be difficult to ascertain in the clinic, so consideration of risk can be guided by knowledge of current epidemiology, which highlights highest-risk age, sex and ethnic groups. Ethnicity is not itself a risk factor but may correlate with socioeconomic disadvantage in colonised or migrant settings.

 

An updated national guideline

The Australian Guideline on Best Practice Standards of Care and Guidance for Prevention, Diagnosis and Treatment of Rheumatic Fever and Rheumatic Heart Disease was updated in 2025.⁵ A First Nations cultural safety framework has been incorporated to support successful implementation of the Australian guideline at individual, family, community and health system levels. Several important updates for clinical practice are included in the revised guideline (Box 1). A Clinical Support App, which includes key information and a rheumatic fever diagnosis calculator, provides a useful point-of-care tool.⁷

 

Primary prevention in the Australian context

Primary prevention comprises early identification and treatment of Strep A infections to reduce the risk of rheumatic fever.

Identification and management of sore throat

The majority of sore throats are caused by viruses and do not require antimicrobial treatment. However, because rheumatic fever prevention depends on prevention or early treatment of streptococcal infections, and avoidance of recurrent infections that 'prime' the immune response towards autoimmunity, sore throat presentations are managed differently in high-risk populations, where high presence of Strep A is assumed.⁵ A throat swab should be collected and antibiotics administered immediately. One dose of intramuscular benzathine benzylpenicillin G is recommended because it is long acting and there is lower risk of missed doses compared with oral therapy. However, if injection is not possible, oral phenoxymethylpenicillin, cefalexin or amoxicillin for 10 days, or azithromycin for 5 days, are acceptable alternatives depending on circumstances, availability and potential drug intolerance.

For individuals who are not at high risk of rheumatic fever and have signs of tonsillitis, the throat should be swabbed and antibiotic treatment offered only if Strep A positive.

Swabbing for Strep A during community outbreaks of respiratory disease, such as COVID-19, helps prevent Strep A infection being missed during viral epidemics featuring sore throat.

Identification and management of skin infection

A skin sore, consistent in appearance with impetigo, should be assumed to be caused by Strep A and treatment is required in populations at high risk for rheumatic fever. Strep A skin sores are often round or linear, 1 to 2 cm in size with pus or a crust in early stages (Figure 1). Other bacteria, especially Staphylococcus aureus, may also be present. Oral trimethoprim+sulfamethoxazole (cotrimoxazole) or intramuscular benzathine benzylpenicillin G are considered equally suitable treatment options. The reason for this choice, rather than more narrow-spectrum oral options such as flucloxacillin, is that trimethoprim+sulfamethoxazole can be dosed twice daily, is palatable for children and has been shown to be non-inferior to penicillin for skin sores in a randomised trial.⁸ Patients and caregivers should be involved in all decisions about treatment.^9,10

 

Presentation and diagnosis of rheumatic fever

Rheumatic fever is clinically diverse, often subtle and mimics other conditions. Commonest presentations comprise polyarthritis or polyarthralgia with or without carditis, with fever and raised inflammatory markers (Table 1). Carditis is usually asymptomatic but detectable through specific electrocardiogram or echocardiogram abnormalities¹¹ and, in more severe cases, may be evident as a heart murmur, chest pain (pericarditis) or features of heart failure. Skin manifestations are rare, but specific, and include erythema marginatum or subcutaneous nodules. Sydenham chorea is a neurological manifestation that occurs in 12 to 28% of people with rheumatic fever, particularly adolescent girls.^12,13 It can appear weeks to months after the streptococcal infection has resolved and can last many months (Table 2).

Table 1 Australian criteria for diagnosis of rheumatic fever [NB1]

Presentation	High-risk groups [NB2]	Low-risk groups
Major manifestations	Carditis (including subclinical evidence of rheumatic valvulitis on echocardiogram) Polyarthritis or aseptic monoarthritis or polyarthralgia Sydenham chorea [NB3] Erythema marginatum [NB4] Subcutaneous nodules	Carditis (including subclinical evidence of rheumatic valvulitis on echocardiogram) Polyarthritis Sydenham chorea Erythema marginatum Subcutaneous nodules
Minor manifestations	Fever ≥38°C Monoarthralgia ESR ≥30 mm/h or CRP ≥30 mg/L Prolonged P-R interval or advanced conduction abnormalities on ECG	Fever ≥38.5°C Polyarthralgia or aseptic monoarthritis ESR ≥60 mm/h or CRP ≥30 mg/L Prolonged P-R interval or advanced conduction abnormalities on ECG
Definite initial episode of rheumatic fever	2 major manifestations + evidence of preceding Strep A infection [NB5], or 1 major + 2 minor manifestations + evidence of preceding Strep A infection
Definite recurrent episode of rheumatic fever in a patient with a documented history of rheumatic fever or rheumatic heart disease	2 major manifestations + evidence of preceding Strep A infection, or 1 major + 2 minor manifestations + evidence of preceding Strep A infection, or 3 minor manifestations + evidence of a preceding Strep A infection
Probable or possible rheumatic fever (first episode or recurrence)	A clinical presentation in which rheumatic fever is considered likely but does not meet diagnostic criteria by either: 1 major or 1 minor manifestation, or no evidence of preceding Strep A infection (streptococcal titres within normal limits or titres not measured). Such cases should be categorised according to the level of diagnostic confidence, either: probable rheumatic fever (previously termed ‘probable: highly suspected’), or possible rheumatic fever (previously termed ‘probable: uncertain’).
CRP = C-reactive protein; ECG = electrocardiogram; ESR = erythrocyte sedimentation rate NB1: Refer to guidelines for more details about diagnostic criteria. NB2: Those living in communities with high rates of rheumatic fever (incidence greater than 30 per 100,000 per year in 5–14-year-olds) or rheumatic heart disease (all-age prevalence greater than 2 per 1000). First Nations peoples living in rural or remote settings; data are not available for other populations but First Nations peoples living in urban settings, Māori and Pacific Peoples, and potentially immigrants from developing countries, may also be at high risk. NB3: Sydenham chorea does not require other manifestations or evidence of preceding Strep A infection to diagnose rheumatic fever, provided other causes of chorea are excluded. NB4: Distinguish from nonspecific viral exanthems. NB5: Elevated or rising antistreptolysin O or anti-DNase B, or a positive throat culture or rapid antigen or nucleic acid test for preceding Strep A infection. Adapted with permission from reference 5

Table 2 Summary of Sydenham chorea presentations and management strategies

Severity	Presentation	Step-wise management [NB1]
Mild	Mild involuntary movements, incoordination or neuropsychiatric features Mild hypotonia or weakness Minimal functional impairment	Supportive measures: Calm environment, avoidance of over stimulation, rest, education about the condition
Moderate	Moderate functional impairment, slightly unsteady gait, some difficulty feeding and other self-care activities Inability to maintain or sustain actions such as handgrip, tongue protrusion or outheld hand/arms (motor impersistence) Mood change, anxiety, reduced attention, hyperactivity, obsessive compulsive behaviour Reduced or altered speech	Supportive measures plus: Anticonvulsant therapy usually with carbamazepine or sodium valproate (if no risk of pregnancy)
Severe	Severe functional impairment, unsteady gait, significant difficulty feeding and other self-care activities Difficulty sitting Dysarthria (markedly reduced or altered speech) Marked mood change, anxiety, reduced attention, hyperactivity, obsessive compulsive behaviour Chorea paralytica (bedbound, aphasic)	Supportive measures plus anticonvulsant therapy plus: There is some evidence for corticosteroid use (prednisone, prednisolone, or methylprednisolone) in Sydenham chorea or other autoimmune encephalopathy, but optimal dose and duration is not established Haloperidol has historically been used, but is now largely discouraged because of adverse effects of excessive sedation and extrapyramidal adverse effects In very unwell children, intravenous immunoglobulin or plasma exchange may be considered
NB1: Refer to guidelines for more details about management including drug doses. Adapted with permission from reference 5

There is no diagnostic test for rheumatic fever, and it can be difficult to achieve diagnostic confidence. Diagnosis is guided by the Jones Criteria,^5,14 which vary slightly for first and recurrent episodes and for high- and low-risk groups; definite diagnosis requires evidence of preceding streptococcal infection (positive serology or swab result) (Table 1). Diagnostic workup includes documentation of temperature, throat swab, blood tests (C-reactive protein, erythrocyte sedimentation rate, streptococcal serology), echocardiogram, electrocardiogram and exclusion of alternative causes for the clinical presentation. Echocardiography is an essential part of diagnostic workup to determine the presence of carditis and identify underlying, undiagnosed rheumatic heart disease. Chorea that is not attributable to another cause is enough to confirm rheumatic fever.

Rheumatic fever diagnosis is classified as definite (meeting all criteria), probable (not meeting all criteria but highly suspected) or possible. For probable and possible diagnoses, expert clinical judgement is required to categorise whether rheumatic fever is considered to be the most likely diagnosis (Table 1).

 

Management of rheumatic fever

Goals of initial management include eradicating any residual Strep A infection and managing symptoms. Individuals suspected to have rheumatic fever should be admitted to hospital where possible for specialist review, investigation, education and multidisciplinary management planning. However, it may be reasonable for some people at low risk of complications, including those with mild symptoms and no persistent fever or evidence of carditis, to be initially managed in the community with support from a medical specialist, as long as echocardiography can be arranged within 2 weeks.⁵

Eradication of Strep A

Streptococcal infection may not be obvious at rheumatic fever presentation; however, eradication therapy, with one dose of intramuscular benzathine benzylpenicillin G or an oral alternative, is highly recommended. Strep A is sensitive to penicillin and related compounds (e.g. cephalosporins). Resistance to macrolide antibiotics (e.g. azithromycin) is found in less than 5% of skin isolates in Northern and Central Australia, but at slightly higher rates of 5 to 10% in Central Australia, southern Queensland and the Kimberley when considering all Strep A isolates.¹⁵

Management of symptomatic arthritis

Paracetamol is recommended for analgesia until a diagnosis is confirmed, to avoid the masking effect that anti-inflammatories can have on migratory joint symptoms, fever and inflammatory markers. Once the diagnosis is confirmed, nonsteroidal anti-inflammatory drugs (NSAIDs), such as naproxen, ibuprofen and aspirin, are acceptable alternatives, although naproxen may be safer and more convenient than aspirin. Note that, while rare, Reye’s syndrome is associated with aspirin use in children. Tramadol is no longer recommended for management of severe pain because of its safety concerns in children.^16,17

Management of carditis and heart failure

Chest pain, attributable to rheumatic pericarditis, may respond to NSAIDs or corticosteroids. Some cardiologists use colchicine, but evidence of effectiveness in rheumatic fever is lacking. In severe carditis, especially rheumatic fever recurrences with pre-existing rheumatic heart disease, pharmacological management of heart failure may be required. This may include use of loop diuretics and aldosterone antagonists in the acute phase, and an angiotensin converting enzyme (ACE) inhibitor (in children, preferably enalapril) and beta blockers once clinically stabilised. Corticosteroid use for severe acute carditis requires expert guidance.

Management of Sydenham chorea

Mild chorea should be managed with supportive measures, and pharmacological options should be considered if movement or mood changes interfere with daily activities. New recommendations for management are based on the degree of functional impairment, mood changes and severity (Table 2).⁵

Pharmacological therapy usually comprises carbamazepine or sodium valproate (if no risk of pregnancy). Corticosteroids can be added for severe chorea, and immunoglobulin and plasma exchange can be considered for very unwell children. Haloperidol is no longer recommended for suppression of choreiform movements.⁵

 

Secondary prevention

The duration of secondary antibiotic prophylaxis to prevent recurrent rheumatic fever is determined by the presence and severity of underlying cardiac involvement. Long-acting benzathine benzylpenicillin injections are favoured globally over oral penicillin; however, they are painful and adherence rates vary considerably. The Australian guideline includes a range of strategies to minimise pain and support patient autonomy over how injections are delivered.⁵ Rheumatic heart disease control programs use registers to help coordinate timing and duration of secondary prophylaxis and clinicians can support patients with reminders and flexible options for injection delivery.

There have been case series of non-anaphylactic death following intramuscular benzathine benzylpenicillin G administration in individuals with severe rheumatic heart disease, predominantly in low- and middle-income countries.^18,19 A small number of cases have been reported in Australia. The hypothesised mechanism is vasovagal syncope resulting in fulminant cardiovascular collapse due to the severe rheumatic heart disease and/or heart failure preventing haemodynamic recovery of the vagal response. As such, oral antibiotics may be preferred for a small subset of people in Australia with severe rheumatic heart disease at greatest risk of such complication.⁵

 

Rheumatic heart disease diagnosis, staging and management

Rheumatic heart disease diagnosis is often delayed, and new diagnoses are seen across all ages. In 2024, nearly 80% of Australian First Nations peoples newly diagnosed with rheumatic heart disease did not have a previous episode of rheumatic fever recorded.³

The diagnostic and staging criteria in the Australian guideline are consistent with the 2023 World Heart Federation Guidelines for the Echocardiographic Diagnosis of Rheumatic Heart Disease.¹¹ Stages A to D reflect risk of disease progression, severity and subsequent clinical complications, as well as informing priority classification to guide the frequency of clinical follow-up and duration of secondary antibiotic prophylaxis. Priority classification can be varied and tailored to the individual in consultation between primary care and specialist teams.

People with established rheumatic heart disease are at risk of heart failure, stroke, atrial fibrillation and infective endocarditis. Regular clinical assessment, including opportunistic electrocardiography and routine echocardiography, is recommended to screen for the risk or development of clinical complications. Antibiotic prophylaxis against infective endocarditis is recommended for all people with rheumatic heart disease when undergoing high-risk procedures that can lead to bacteraemia, such as invasive procedures of the mouth and skin, and musculoskeletal, respiratory, genitourinary and gastrointestinal systems.⁵ Detailed information on the classification of dental and other surgical procedures by invasiveness, and considerations for risk management, is available in Therapeutic Guidelines.²⁰

 

Conclusion

Rheumatic fever and rheumatic heart disease can be largely prevented and managed. People working with high-risk populations need to consider the possibility of these diagnoses and follow national guidelines and local protocols, particularly to: treat potential Strep A infections (sore throats, impetigo) early with antibiotics, suspect rheumatic fever early and order relevant investigations, notify public registers and check local requirements, use echocardiography, support BPG adherence and provide culturally safe healthcare.For more information visit the ARF and RHD Guidelines.

 

Key practice points

• Antibiotic treatment of sore throat (pharyngitis and tonsilitis) and skin sores (impetigo) is recommended for people at high risk of rheumatic fever. These conditions may be caused by Group A Streptococcus, and prompt treatment decreases the risk of developing rheumatic fever and subsequent rheumatic heart disease.
• Penicillin injections should be administered within a framework of culturally safe, patient-centred care using appropriate techniques and strategies to minimise pain and distress.
• The priority classification of rheumatic heart disease considers severity based on clinical and echocardiography findings, and guides the duration of secondary antibiotic prophylaxis and follow-up requirements.
• Complications of established rheumatic heart disease include heart failure, atrial fibrillation, stroke and infective endocarditis. Regular clinical review to identify new signs and symptoms is imperative.
• Antibiotic prophylaxis against infective endocarditis is recommended for individuals with rheumatic heart disease undergoing invasive dental, skin, gastro-intestinal or urogenital procedures.
• Rheumatic fever and rheumatic heart disease are notifiable conditions in New South Wales, the Northern Territory, Queensland, South Australia, Victoria and Western Australia.

This article was finalised on 11 June 2026.

Conflicts of interest: Sara Noonan was production editor for the Australian Guideline for the Prevention, Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease as per her employment with Menzies School of Health Research and National Heart Foundation. James Marangou, Bart Currie and Anna Ralph were editors on the Australian guideline and Vicki Wade was an author, reviewer and cultural lead.

Anna Ralph has received funding from the Australian Government via the National Health and Medical Research Council (NHMRC) and the Medical Research Futures Fund (MRFF) for research and a fellowship relevant to rheumatic fever. She is a board member and non-executive director of the Timor-Leste Hearts Fund.

James Marangou has received funding from the Australian Government via the NHMRC and MRFF for research and a fellowship relevant to rheumatic fever. He has also received research funding from the Heart Foundation. James is the secretary of the Strep A Vaccine Global Consortium (SAVAC) echocardiography in group A Strep vaccine trials technical advisory working group. He has received funding from SAVAC toward registration, accommodation and travel for meeting attendances.

This article is peer reviewed.

 

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