Public Health Priority: Routine
PHU response time: Respond to confirmed and probable cases within 3 working days. Enter confirmed and probable cases on NCIMS within 5 working days.
Case management: Work with key contacts in the LHD to enrol cases onto the Rheumatic Heart Disease Register to enable effective long-term management.
Contact management: None
Appendix 5: NSW case management roles and responsibilities
Acute rheumatic fever and rheumatic heart disease are sentinel conditions of poverty and of health inequality; their persistence mark the failure of our health and other systems to address the non-communicable diseases of the disadvantaged. While this document does not attempt to address all the social determinants of health there are important considerations for a public health response which should be taken into consideration when planning public health interventions (including but not limited to Environmental Health) specifically in addressing the Closing the Gap National targets. Recommendations made with regards to the social determinants of health, including Environmental Health may be beyond the scope of the Public Health workforce responding to an individual case or an outbreak, but have been included for consideration.
The priorities in the control and prevention of acute rheumatic fever (ARF) are:
The aim of this guideline is to summarise the public health aspects of ARF case management, including mitigation of progression to RHD, the chief complication of ARF. It is based on the existing Australian National Guideline (Second Edition, 2012).
Respond to confirmed and probable cases within 3 working days. Enter confirmed and probable cases on NCIMS within 5 working days.
ARF is a sporadic condition in the majority of instances. It has been estimated that a minority of individuals (approximately 3-5 per cent) infected with the causative organism, i.e. rheumatogenic strains of group A streptococcus (GAS), have an inherent susceptibility to developing the autoimmune sequelae which constitute ARF.
Although rare, ARF case clusters or outbreaks can occur and a public health response is required (refer to Section 13 - References and additional sources of information).
Based on the Australian National Guideline and 2015 Revised Jones Criteria, case management includes:
* Notification to Communicable Disease Control Branch in SA with inclusion on the RHD Register upon patient consent.
a The 2012 National Guidelines1 use the terminology ‘probable - highly suspected’ for probable, and ‘probable – uncertain’ for possible. These terms are interchangeable.
b See Section 9 for details including antibiotic option in confirmed penicillin allergy.
ARF is a preventable disease of socioeconomic disadvantage. Repeated episodes of ARF lead to cumulative cardiac valve damage, resulting in RHD. RHD is an important cause of premature morbidity and mortality in selected Australian populations, particularly Aboriginal and Torres Strait Islander, Maori and migrants from the Pacific nations. In addition to cardiac involvement other manifestations include: carditis, arthritis, Sydenham's chorea, erythema marginartum, and subcutaneous nodules. Minor manifestations include; arthralgia, fever and elevated acute-phase reactants.
The infectious agent is Group A-haemolytic streptococcus (Streptococcus pyogenes or GAS).
An abnormal autoimmune host response to GAS is required for the development of ARF.
Humans are the sole reservoir for GAS.
GAS is transmitted human-to-human via:
The importance of crowded living conditions to facilitate transmission has been well documented; e.g. the risk of GAS pharyngeal infection has been shown to be inversely proportional to the distance between a subject’s bed and that of a colonised or infected case (see review). Poor skin health and household crowding is thought to contribute to the overall burden of GAS infection and carriage.
Poverty, household crowding, poor household and community hygiene as risk factors for ARF has recently been confirmed in a large ecological study of over 1000 rheumatic fever cases in New Zealand.
The interval between GAS exposure and pharyngitis (which may be asymptomatic) or clinically apparent impetigo/pyoderma is 1-10 days.
The interval between GAS infection and onset of ARF varies depending on:
In general, the following applies:
A diagnosis of ARF can be made (a) after exclusion of differential diagnoses, such as disseminated gonococcal infection or systemic lupus erythematosis, and (b) if the clinical and laboratory features fulfil the Australian modification of the Jones Criteria (now largely incorporated into the 2015 Revised Jones criteria) (Refer to Table 1).
A high index of suspicion for ARF is required, especially in at-risk individuals. The Revised Jones Criteria recognise the need for a lower diagnostic threshold in high-risk groups (see Table 1). In Australia, the high-risk populations are:
Although the recorded primary ARF episode is by definition the first episode to have been recognised and notified, many individuals have already had previous unrecognised ARF, evidenced by the finding of established RHD (as seen on echocardiogram) at the time of ‘primary’ ARF, or by performing a retrospective chart review and finding presentations consistent with ARF where the diagnosis was missed. Prior episodes may also have been inadequately symptomatic for the individual to have sought medical care. (See Table 1)
Carditis: active inflammation of the myocardium, endocardium and pericardium (i.e. pericarditis). The predominant manifestation of rheumatic carditis is involvement of the mitral and/or aortic valve endocardium presenting as a valvulitis. Echocardiography is the gold standard diagnostic modality, and echocardiogram criteria for rheumatic carditis diagnosis (and RHD diagnosis) are clearly defined.[1,13]
Arthritis: swollen and hot joint(s) with pain on movement. This is the most common presenting symptom of ARF. Rheumatic arthritis may have an abrupt onset and last for a few days to weeks and commonly involves the large joints either as a mono-arthritis (single joint) or polyarthritis (multiple joints). The inflammation is typically migratory (moves from one joint to another) with possible resolution of pain in one joint before onset in the next. Mono-arthritis is a major manifestation in high risk groups (see below) but lower-risk groups require polyarthritis to be a major manifestation; for them monoarthritis is classified as a minor manifestation.
Sydenham’s chorea: abrupt, jerky, involuntary movements +/- associated muscular weakness and emotional lability. These uncoordinated movements especially affect the hands, feet and facial muscles, are often more severe on one side of the body and disappear during sleep. Chorea has the latest onset of timing of all ARF manifestations, with onset from weeks to months after initial streptococcal infection.
Erythema marginatum: rare (<2%); highly specific for ARF (considered pathognomonic), but other rashes can be easily mistaken for it. The rash can be difficult to detect in dark-skinned people. The rash appears early in the course of ARF and appears as bright, pink macules or papules that are non-pruritic, blanch under pressure, spread outwards in a circular pattern and may wax and wane over the course of a day. The rash may be found on the trunk or limbs but not on the face.
Subcutaneous nodules: also a rare (<2% of cases) but highly specific manifestation of ARF in Aboriginal people. They are 0.5–2 cm in diameter, round, firm, mobile, painless nodules occurring in crops of up to 12 over the elbows, wrists, knees, ankles, Achilles tendons, occiput and posterior spinal processes of the vertebrae. The nodules usually appear in a symmetric distribution, usually present in the first weeks of illness and may be associated with more severe forms of carditis.
Arthralgia: pain on joint movement without evidence of swelling or heat. It usually occurs in the same pattern as rheumatic polyarthritis (migratory, asymmetrical, affecting large joints). Polyarthralgia is a major manifestation in high risk groups (see below) but classified as a minor manifestation in others. Monoarthralgia is a minor manifestation but only for high risk groups.
Fever: accompanies most manifestations of ARF, with the exception of chorea.
Elevated acute-phase reactants: e.g. CRP, ESR: typically seen in ARF; less so in chorea.
Prolonged P-R interval on electrocardiogram (ECG): transient first-degree heart block or other conduction abnormalities (e.g. junctional rhythm) are hallmarks of rheumatic carditis; however, only P-R prolongation is included as a minor criterion. For P-R interval upper limits of normal for different age groups, see Table 1, last footnote. An ECG is required in all cases of suspected ARF.
†High-risk groups are those living in communities with high rates of ARF (incidence >30/100,000 per year in 5 to 14 year olds) or RHD (all-age prevalence >2/1000). Aboriginal people and Torres Strait Islanders living in rural or remote settings are known to be at high risk. Data are not available for other populations, but Aboriginal people and Torres Strait Islanders living in urban settings, Maori and Pacific Islanders, and immigrants from developing countries, may also be at high risk.
‡Elevated or rising antistreptolysin O or other streptococcal antibody, or a positive throat culture or rapid antigen test for GAS.
††A definite history of arthritis is sufficient to satisfy this manifestation. Note that if polyarthritis is present as a major manifestation, polyarthralgia or aseptic mono-arthritis cannot be considered an additional minor manifestation in the same person.
§Chorea does not require other manifestations or evidence of preceding GAS infection, provided other causes of chorea are excluded.
*Care should be taken not to label other rashes, particularly non-specific viral exanthemata, as erythema marginatum.
‡‡Oral, tympanic or rectal temperature ≥38°C on admission, or a reliably reported fever documented during the current illness.
§Upper limit of normal P-R interval is: ages 2-12 years: 0.16 seconds; ages 13-16 years: 0.18 seconds; ages 17+ years: 0.20 seconds. If carditis is present as a major manifestation, a prolonged P-R interval cannot be considered an additional minor manifestation.
This can comprise positive antistreptolysin O titre (ASOT) or anti-DNAseB titre or isolation of group A-haemolytic streptococcus from throat swab; Refer to Section 9 Case Management.
Although ARF symptoms may be subtle, and the key importance of ARF is the potential for progression to RHD, ARF itself is often a very burdensome illness.
After primary ARF, outcomes range from full recovery with no permanent sequelae, to a fulminant disease course resulting in death. A recent NT study demonstrated that after a first ARF diagnosis, 61 per cent of people developed RHD within ten years. After RHD diagnosis, 27 per cent developed heart failure within five years. Careful pregnancy planning in women with RHD of child-bearing age is an important consideration, given elevated foetal and maternal risks.
The range of outcomes after a primary ARF episode includes:
ARF outcomes with regards to treatment requirements include:
Definition of ARF recurrence: new ARF episode occurring >90 days after a prior episode.
Diagnosis of ARF recurrence: there is a lower threshold for diagnosis of a recurrence compared with primary episode, as per line 3 of Table 1.
Timing of recurrences: most recurrences occur within the first 10 years after the primary ARF diagnosis which is the basis for the recommendation to give benzathine penicillin G for this duration. The recurrence rate is highest in the first year, and drops annually thereafter.
In Australia, the high-risk populations are:
People at risk of ARF are:
Australian ARF rates are available for jurisdictions in which ARF is notifiable or where specific research on this question has been conducted.
There are several reasons why ARF and RHD are leading causes for public health concern. Firstly, ARF and RHD are indicators for socioeconomic disadvantage and highlight the requirement for major improvements in the social determinants of health. Secondly, ARF and RHD cause a major burden of premature morbidity and mortality for young Indigenous people in Australia. In the 2006 Australian Institute of Health and Welfare report on mortality in Australia, rheumatic and other valvular disease had the highest differential mortality ratio between Indigenous and non-Indigenous Australians for any clinical category; 19.1, in comparison to 18.2 for nephritis and nephrosis, 18.1 for diabetes, 4.3 for ischaemic heart disease and overall ratio 3.9.
* NSW Admitted Patient Data Collection, 2003 – 2012, ICD-10 AM codes (100-102), excludes repeat admissions for individuals.
Source: SaPHaRI Centre for Epidemiology and Evidence, NSW Ministry of Health. (excludes recurrences).
The current cornerstone of ARF/RHD preventive activities in Australia currently 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, shown Table 3 include:
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 past ARF than in those of similar age and living in the same community/circumstances, but without prior ARF.
Level of prevention: Primordial
Level of prevention: Primary
Refer to endorsed therapeutic guideline - antibiotic.
Management of streptococcal pharyngitis:
Management of streptococcal impetigo in high-risk communities:
Encourage regular handwashing to reduce risk of transmission of GAS.
Level of prevention: Secondary
This is the most studied and effective preventive measure with 1a level evidence.
Secondary prophylaxis with intra muscular injections of benzathine penicillin G every 28 days significantly reduces ARF recurrence rates compared with placebo or oral penicillin and is the treatment of choice. This strategy forms the basis of the WHO's ARF recommendations and Australia's National Guidelines.
900mg ( >=20kg)=1,200,000 units
450 mg (<20kg) =600,000 units
Following documented penicillin allergy:
Duration of secondary prophylaxis: Minimum 10 years after most recent episode of ARF or until age 21 years (whichever is longer), then re-evaluate. If echocardiogram then shows no or mild RHD, cease secondary prophylaxis. If echo shows moderate RHD, continue until age 35. If echo shows severe RHD or valve surgery has been performed, continue until age 40 or longer.
Tertiary: This comprises medical and surgical managment of RHD which is outside the scope of this document
‡ BPG – benzathine penicillin G
‡‡ IMI – intramuscular
*Recommendations about school exclusion need to be considered carefully in communities characterized by low school attendance. Exclusion should also incorporate avoiding contact with at-risk individuals e.g. people with prior ARF.
§ or every 21 days for selected individuals (uncommon)
§§ only in exceptional circumstances; less effective than 1st line
** Based on the Canadian National Occupancy Standard which provides definitions of household crowing based on age and sex of occupants. In general they consider >2 people per bedroom to represent crowding.
To identify cases of ARF and RHD in a timely manner:
Depending on State/Territory requirements (see Table 4), cases should be notified to the jurisdictional notifiable diseases database and/or the RHD disease register (control program), in a timely fashion.
ARF is notifiable in QLD, NT, WA (2015), NSW (2015) and SA (2016). Possible cases are not notifiable.
RHD is notifiable in NSW (2015) [<35 years only], WA (2015) and SA (2016).
Jurisdictional ARF/RHD control programs exist in QLD, NT, SA, NSW and WA. Each of these jurisdictions maintains a register of ARF/RHD cases.
*Nil in VIC, ACT or TAS
Communicable Disease Directors (or their nominated delegate) should be notified if an ARF outbreak is suspected (see Section 12).
A confirmed case requires clinical evidence AND laboratory suggestive evidence.
Except in the case of rheumatic Sydenham's chorea which may occur alone without other manifestations or laboratory suggestive evidence, provided other causes of chorea are ruled out. Therefore Sydenham's chorea along, without laboratory suggestive evidence, is sufficient evidence for a confirmed case.
As for probable case, where the treating clinician has less confidence about ARF as the correct diagnosis, but other differential diagnoses have been excluded.
A diagnosis of RHD is made by a cardiologist based on echocardiographic findings. RHD is defined as the presence of specific echocardiographic features summarised in Appendix 3.
Example: a high-risk child with no prior ARF/RHD with monoarthralgia, fever, P-R prolongation (all minor criteria) and positive streptococcal serology where other causes have been excluded, may be deemed a probable case. A high-risk child with monoarthralgia, fever and elevated CRP only, where other causes have been excluded, may be deemed to be a possible case. The decision is at the discretion of the ARF specialist consulted, based on history, examination findings, history etc.
There is no diagnostic test for ARF. Required laboratory testing includes:
Numerous laboratory methods for testing ASOT exist, and results can vary significantly between test types. Laboratories may establish locally-appropriate reference ranges for a given ASOT test type and for their population, but if such data are unavailable, the cut-offs provided below are recommended, with advice to practitioners to be mindful of variability between test types. A rise in titre between paired acute and 14-28-day convalescent serum samples may help but also has limitations.
Roles and responsibilities for the management of cases in NSW is described in Appendix 5.
People with suspected ARF should be admitted to hospital within 24 hours or as soon as feasible for people living in remote communities. Admission to hospital is advised to:
Within 3 days of notification of confirmed and probable cases begin follow-up investigation.
Within 5 working days of notification enter confirmed and probable cases.
Notification of ARF and RHD should be made with the NSW notification form.
Cases should be managed by a doctor with expertise in the management of ARF/RHD. Treating doctors seeking specialist advice can be referred through the jurisdictional RHD control programs to an ARF/RHD specialist. Management is guided by the Australian National Guidelines which provide care plans based on the individuals priority status (P1, P2, P3). Some jurisdictional control programs provide education and adherence support directly to patients and their families; elsewhere this responsibility lies with the primary care team.
Admission to hospital for further investigation and follow-up is highly recommended.
Important components of case management include:
* Discharge should be delayed until resolution of carditis and inflammatory markers as early discharge has led to fatal decompensation in the community setting. Resolution of these markers should be determined and managed by an ARF/ RHD specialist clinician
Ensure the patient, their family and primary carers have access to culture and language-appropriate resources, and have been provided with culturally appropriate face to face educational sessions, using an interpreter where required to cover areas including: diagnosis and cause, increased potential risk to family members of ARF, long-term medical management and pharmacological and non-pharmacological secondary prophylaxis options (see ‘Additional sources of information’ for web addresses for patient resources.)
Simple explanations about the non-pharmacological measures associated with reduced risk of ARF episodes should be provided. While randomised trial evidence is lacking, observational studies have repeatedly indicated that household overcrowding correlates with ARF incidence,[25,26] and therefore strategies to mitigate this need to be developed with families. The definition of crowding used by the Australian Institute of Health and Welfare (no more than 2 people per bedroom) may appear excessively cautious and unfeasible, but nevertheless provides a target towards which to work. Randomised trials of the effect of washing children’s hands and bodies with soap and water have demonstrated a significant decrease in impetigo rates in the groups assigned to washing.[44,45] While a link between pyoderma and ARF has never definitively been established, washing with soap and water is an appropriate primordial preventive message to promote for families and communities affected by ARF.
All people with newly diagnosed ARF and RHD in NSW should be offered face-to-face education with a health care worker or environmental health officer on how to minimise the risk of future episodes of ARF.
The PHU should consider whether an environmental health assessment of the person’s home would be appropriate, particularly if more than one case has occurred in a community.
An environmental health assessment may be a useful adjuvant to treatment, to address social/environmental issues where this is appropriate and reasonable to do so. The capacity to conduct environmental evaluation varies by region. The appropriate response depends on the geographical setting and the nature of the affected person’s housing.
To assist in advising about strategies to avoid future ARF recurrences, the living conditions experienced by the patient could be assessed by an Environmental Health Officer/Unit and in conjunction with the relevant public housing government department or housing provider/funding agency for private housing. Consent should be provided by the family affected and where possible a community member should be present with the family prior to assessment. The following describes a suggested response for remote-dwelling Indigenous Australians living as tenants in public or private housing.
Issues to assess include:
Deficiencies in the health hardware which are beyond the capacity of the patient’s family to manage should be referred to the jurisdictional government housing authority if the patient lives in public housing or the housing provider if the patient lives in private housing.
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Not routine. Contact management in potential outbreak scenarios is addressed in Section 12 – Special situations. The secondary attack rate for sporadic ARF is well below the threshold required for instigation of contact treatment (e.g. see reference regarding transmission rates  ). ARF occurs in only a small proportion of individuals infected with GAS, perhaps as few as 3-5%.
Contacts living with the case person should be included in education about ARF/RHD, in particular the association of GAS transmission with over-crowding and the importance of skin health to reduce GAS transmission for all those living in a household with a person with ARF/RHD. Additionally, for family contacts, the possibility of genetic susceptibility can be raised.
ARF has been considered to be a sporadic condition in Australia. However, in recent years, a small number of situations consistent with possible outbreaks have been observed in parts of northern Australia with already underlying high rates of ARF. This section therefore provides guidance on management of such possible ARF outbreaks, with the caveats that:
As per definition provided above, Section 8 Laboratory testing
A greater than expected number of confirmed or probable ARF cases occurring during an approximately 4-week period within a defined region. The threshold number of cases and timing needs to be defined by Public Health Units at regional levels and will vary by background rate of ARF and community population size. For example, two cases in a community, especially if related (e.g. in same family, household or class) may be enough to trigger scrutiny
Examine at-risk contacts (e.g. aged <21 years, depending on resources) for features of ARF. Cardiac auscultation by an experienced clinician but where possible use of a portable echocardiogram will provide best sensitivity for diagnosis46
Collect samples for bacterial culture +/- typing of Group A Streptococcus from all cases (already routine practice), but additionally from all contacts; ideally both throat swabs and swabs of any skin sores should be collected in this setting.
Provide community education about ARF and ARF prevention strategies.
† Note that doses differ from doses required for ARF secondary prophylaxis, since the latter requires a large depot dose with longer half-life for prevention of future exposures, whereas the strategy for contact treatment is to eradicate any current GAS infection or carriage.
‡ Note that doses in mL differ from the CARPA manual as the 2015 preparation of Bicillin LA comes as 900mg in 2.3ml. This may well change again, so check current formulation being used.
*Those allergic to penicillin should receive azithromycin 12mg/kg up to 500mg orally, daily for 5 days.
Additional information, resources and RHD program materials can be found at RHD Program