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National guide to a preventive health assessment for Aboriginal and Torres Strait Islander people


Chapter 10: Acute rheumatic fever and rheumatic heart disease 
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☰ Table of contents


Recommendations: Acute rheumatic fever and rheumatic heart disease

Preventive intervention type

Who is at risk?

What should be done?

How often?

Level/ strength of evidence

References

Immunisation

People with a history of acute rheumatic fever (ARF) or known rheumatic heart disease (RHD) Administer routine childhood and adult vaccinations plus annual influenza vaccination as per the National Immunisation Program Schedule (refer also to Chapter 3: Child health)

Provide pneumococcal vaccination
As per national guidelines II 9

Screening

Individuals coming from high-risk groups or living in high-risk settings for ARF/RHD
All pregnant women
Take a comprehensive medical history, and family history for cardiovascular disease

Cardiac auscultation to screen for RHD is not recommended due to poor sensitivity and specificity. The diagnosis of RHD must be made by echocardiography

Echocardiography is not currently recommended for population-based screening for RHD
Opportunistic and as part of routine health assessment GPP 9
All individuals with a past history of ARF, or cardiac murmurs suggestive of valve disease Refer for echocardiography and subsequent follow-up.

Refer to management guidelines for specific advice
As per management guidelines GPP 9

Behavioural

People with a past history of ARF or known RHD Emphasise the importance of early treatment for sore throat and prevention of skin infections (refer to Chapter 3: Child health, ‘Childhood kidney disease’)

Advise about healthy lifestyle (smoking, diet, exercise, dental health) and the need for regular clinical reviews (refer to Chapter 1: Lifestyle, and Chapter 8: Oral and dental health)

Offer contraceptive advice to females of child-bearing age in order to avoid unintended pregnancy (refer to Chapter 4: The health of young people)

Provide community-based health promotion about ARF/RHD
Opportunistic and annually GPP 39

Chemo-prophylaxis

All people in highrisk communities where Group A streptococcus (GAS) infections are common and ARF is prevalent Maintain a high index of clinical suspicion of streptococcal pharyngitis in people presenting with a sore throat

Take a throat swab to confirm a diagnosis of streptococcal pharyngitis and consider empirical treatment with single-dose intramuscular benzathine penicillin G or the less-preferred option of 10 days of oral penicillin V while awaiting test results
As presented GPP 39
All people with confirmed GAS pharyngitis Treat as above

There is no evidence to support treating family contacts of those with GAS pharyngitis
As presented IA 40
All people with ARF/RHD A new diagnosis of ARF or RHD should be notified to the communicable disease control unit in jurisdictions where these conditions are notifiable diseases

Recommend long-term prophylactic antibiotics (either benzathine penicillin every 21–28 days or the lesspreferred option of daily oral penicillin V) for the prevention of recurrent rheumatic fever attacks

Explain the importance of longterm antibiotics to both the affected individual and their family/carers

Include patient details in local patient information or medical record recall systems and, with consent, send details to the ARF/RHD centralised register
Opportunistic and as presented GPP 9
  Categorise patients according to the severity level of their disease (priority levels 1–4) (Box 1). This is necessary to plan the review and follow-up frequency tailored to patients As per individual recall plan IA 9
People with established RHD Provide antibiotic prophylaxis for dental and other high-risk procedures As required GPP 22

Environmental

People living in communities where GAS infections are common and ARF is prevalent Assess for overcrowding and refer to social support services for housing assistance if indicated (refer also to Chapter 7: Hearing loss)

If high rates of impetigo and underlying scabies, manage as per local healthy skin guidelines (refer to ‘Resources’)
Opportunistic IIIB 41

 

 

Box 1. Priority classifications for developing management plans9

Classification

Criteria

Priority 1 (severe)

People with any of the following:

  • severe valvular disease
  • moderate or severe valvular lesion with symptoms
  • mechanical prosthetic valves, tissue prosthetic valves and valve repairs including balloon valvuloplasty

Priority 2 (moderate)

Any moderate valve lesion in the absence of symptoms and with normal LV function

Priority 3 (mild)

ARF with no evidence of rheumatic heart disease (RHD), or trivial to mild valvular disease

Priority 4 (inactive)

Patients with a history of acute rheumatic fever (ARF; no RHD) for whom secondary prophylaxis has been ceased

For more detailed information on specific management plans for each priority area, consult RHD Australia guidelines (refer to ‘Resources’)9.


Background


‘While acute rheumatic fever has become a rare curiosity in Australia’s non-Indigenous population, its incidence in Indigenous Australians living in remote areas remains among the highest reported in the world. It is unlikely that such a stark contrast between two populations living within the same national borders exists for any other disease or on any other continent.’1
Acute rheumatic fever (ARF) is an illness caused by an abnormal and exaggerated immune response to infection with Group A beta-haemolytic streptococcus (GAS or Streptococcus pyogenes). The role of preceding GAS throat infection is undisputed, although preceding symptomatic GAS pharyngitis is infrequently identified in Aboriginal and Torres Strait Islander populations.2 The role of preceding skin infection in ARF is less certain.3 Immune priming is possibly important but the exact pathogenic mechanisms remain
elusive.4,5

ARF remains the commonest cause of acquired heart disease in children worldwide.6 The community incidence of ARF is a sensitive marker of childhood disadvantage.1 In Australia and nearby Pacific nations, the incidence of ARF varies widely: children in remote Aboriginal and Torres Strait Islander communities of Northern and Central Australia, Pacific Islanders, Maori people and refugees are in the highest risk groups. In some remote communities, reported prevalence rates of greater than 300 per 100,000 children aged 5–14 years are more than 100 times that of the wider Australian population.7

ARF tends to run in families and is slightly more common in females. It has a low incidence rate in wealthier societies.5 The incidence of ARF peaks in the 5–15-year age group; it occurs endemically in tropical high incidence settings, as opposed to outbreaks that have occasionally been reported in other parts of the world.
Individuals with a first episode of ARF are at high risk of recurrences, especially in the first year; however, this increased risk extends over the next 10 years or so.8 The concern is that ARF, especially recurrent ARF, leads to rheumatic heart disease (RHD) in a high proportion of people (likely to be >50%).7 RHD is characterised by cumulative scarring and distortion of heart valves – primarily the mitral and aortic valves. The peak prevalence of RHD is in the 15–35-year age group.8 Progression causes valve regurgitation, stenosis or both – with a substantial risk of subsequent heart failure, atrial fibrillation, embolic stroke, infective endocarditis, disability and early death. The RHD-attributable mortality rate for Aboriginal and Torres Strait Islander peoples is substantially higher than that for the equivalent non-Indigenous population. ARF and RHD are preventable.


Diagnosis of ARF


ARF is said to ‘lick the joints and bite the heart’ (Ernest-Charles Lasègue, 1816–83). It is a clinical diagnosis; there are no definitive diagnostic laboratory tests. For more than half century, clinicians relied on the Jones criteria; they were revised in 1992, but still appeared to be too specific for high-risk populations. This prompted development of the Australian guideline9 and the World Health Organization guideline.10 However, the most recently revised Jones criteria, from 2015,11 are again regarded as the Gold Standard. In moderate- to high-risk populations, mono-arthritis, polyarthralgia and subclinical, echocardiographically diagnosed carditis are major criteria (Table 1). There is also the ability to diagnose ‘probable ARF’, so as to include manifestations that do not satisfy the criteria for definite ARF but for which the clinician feels ARF is the most likely diagnosis. One common-sense diagnostic approach is to suspect ARF in any child with acute fever and joint pain or any child with new onset movement disorder, especially in a high-risk setting, and have a low threshold for consulting an experienced colleague.12 A further concern is that mild to moderate acute carditis can present without arthritis, particularly with ARF recurrences. This is commonly asymptomatic and, as a consequence, it may go undetected and potentially cause cumulative valve damage.13
 

Table 1. Australian guideline criteria for acute rheumatic fever9

 

High-risk groups*

All other groups

Initial episode of acute rheumatic fever (ARF)

  • Two major or one major and two minor manifestations plus
  • Evidence of a preceding Group A streptococcus (GAS) infection†

Recurrent attack of ARF in a patient with known past ARF or rheumatic heart disease (RHD)

  • Two major or one major and one minor or three minor manifestations plus
  • Evidence of a preceding GAS infection†

Probable ARF (first episode or recurrence)

A clinical presentation that falls short by either one major or one minor manifestation, or in the absence of streptococcal serology results, but one in which ARF is considered the most likely diagnosis. Such cases should be further categorised according to the level of confidence with which the diagnosis is made:

  • highly suspected ARF
  • uncertain ARF

Major manifestations

  • Carditis (including evidence of rheumatic valvulitis on echocardiogram)
  • Polyarthritis or aseptic mono-arthritis or polyarthralgia‡
  • Chorea§
  • Erythema marginatumII
  • Subcutaneous nodules
  • Carditis (excluding subclinical evidence of rheumatic valve disease on echocardiogram)
  • Polyarthritis‡
  • Chorea§
  • Erythema marginatum
  • Subcutaneous nodules

Minor manifestations

  • Mono-arthralgia
  • Fever ≥38°C#
  • Erythrocyte sedimentation rate (ESR)≥30 mm/hr or C-reactive protein (CRP) ≥30 mg/L
  • Prolonged P-R interval on electrocardiogram (ECG)**
  • Fever ≥38°C#
  • Polyarthralgia or aseptic mono-arthritis‡
  • ESR ≥30 mm/h or CRP ≥30 mg/L
  • Prolonged P-R interval on ECG**


*High-risk groups are those living in communities with high rates of ARF (incidence >30 per 100,000 per year in 5–14-year-olds) or RHD
(all-age prevalence >2 per 1000). Aboriginal and Torres Strait Islander peoples living in rural or remote settings are known to be at high risk.
Elevated or rising anti-streptolysin O (ASO) or other streptococcal antibody, or a positive throat culture or rapid antigen test for GAS. The ASO must be interpreted according to age and the background ‘streptococcal burden’ of the community.
A definite history of arthritis is sufficient. Other causes of arthritis/arthralgia should be carefully excluded.
§Rheumatic (Sydenham’s) chorea does not require other manifestations or evidence of preceding GAS infection, provided other causes of chorea are excluded.
IIErythema marginatum is a distinctive rash. Care should be taken not to label other rashes, particularly non-specific viral exanthemas, as erythema marginatum.
#Oral, tympanic or rectal temperature ≥38°C on admission or documented during the current illness.
**Note that if carditis is present as a major manifestation, prolonged P-R interval cannot be considered an additional minor manifestation in the same person.


Clinicians may not always appreciate the distinction between the two most common forms of the illness. Table 2 outlines the differences between the acute febrile illness with joint manifestations and, often, carditis; and the neurological/behavioral disorder, which often also has accompanying carditis. Clinicians should also be aware that erythema marginatum and subcutaneous nodules are both rare manifestations of ARF.
 

Table 2. Clinical manifestations of acute rheumatic fever

Acute febrile illness

Neurological illness (25–30%)

  • Onset: 2–4 weeks after Group A streptococcus (GAS)infection
  • Fever is common
  • Acute joint symptoms and signs
  • Carditis: clinical and subclinical
  • Skin manifestations and subcutaneous nodules  – these are rare
  • Raised inflammatory markers 
  • (C-reactive protein, erythrocyte sedimentation rate)
  • Evidence of preceding GAS infection  (anti-streptolysin O [ASO] and anti-DNase B)
  • Dramatic symptomatic response to aspirin and  non-steroidal anti-inflammatory drugs
  • Duration: usually <6 weeks
  • The clinical features of recurrent acute rheumatic fever may be less prominent
  • Later onset: 2–6 months after GAS infection
  • Most common in females aged 10–24 years
  • No fever
  • Often occurs as isolated manifestation: joint manifestations are usually not a feature
  • Distinctive chorea and behavioural disorder
  • Carditis > 30%: often subclinical
  • Often normal inflammatory markers
  • Because may occur as delayed manifestation, ASO and anti-DNase B not helpful
  • Duration ≥3 months, with later recurrence ~30%
  • Followed by rheumatic heart disease in ~50%


The neurological illness (Sydenham’s chorea) is more common in girls and sometimes presents as refusal to go to school and self-isolation. The movement features of chorea can be subtle and intermittent, unilateral or bilateral, sometimes being seen only after a period (10–15 minutes) of quiet observation. The chorea disappears during sleep. Shame and embarrassment are common concerns for these children. They can also have halting and jerky speech patterns – this causes more embarrassment. Inappropriate behaviour occasionally includes obsessive-compulsive features.9
As mentioned, timely diagnosis of ARF at the time of presentation is critical and a high index of suspicion is needed, especially in populations at high risk. If ARF is suspected, immediate management steps include the following:

  • obtain a second expert opinion, usually by telephone
  • arrange admission to hospital within 24 hours, if possible
  • take a careful history, including a family history, and conduct a thorough physical examination
  • arrange ECG, mainly to check P-R interval (this is age dependent), and take a throat swab for GAS culture
  • take blood for baseline streptococcal serology (anti-streptolysin O [ASO] and anti-DNase B) and inflammatory markers (erythrocyte sedimentation rate and C-reactive protein)
  • administer intramuscular benzathine penicillin G (also called Bicillin LA) according to protocol with the aim of eradicating GAS from the throat
  • do not give aspirin or non-steroidal anti-inflammatory drugs immediately; the in-hospital observed response to these medications is diagnostically helpful. Give paracetamol for pain relief.

The final diagnosis of ARF should only be made by a specialist clinician with substantial experience in the diagnosis of ARF and/or in consultation with such a clinician.9


Diagnosis of RHD


The diagnosis of RHD usually occurs in one of three ways:

  1. Presentation with symptoms – cardiac (most common) and/or neurological (stroke, endocarditis). This indicates advanced disease.
  2. Finding a heart murmur or signs of rheumatic valve disease on routine examination, incidentally and/or by echocardiography for another matter. The person is often asymptomatic.
  3. Case detection as part of a RHD screening program. Such individuals are usually asymptomatic and may have no discernible heart murmur.

Any person with suspected or proven RHD must be immediately referred, initially by telephone, to a specialist service for assessment if any of the following are detected:

  • symptoms, particularly exertional dyspnoea
  • untreated atrial fibrillation
  • fever
  • a recent neurological event
  • current pregnancy (remember to test).

Echocardiography provides a definitive diagnosis of RHD and this may take some time to organise for people in remote settings. The World Heart Federation has now developed standardised and evidence-based criteria.14


Interventions


Preventing and treating ARF and RHD

A common strategy is to implement complementary interventions at each level of the preventive health spectrum.9

Primordial prevention refers to addressing social determinants of health such as adequate housing, education and employment. Ultimately, primordial prevention is the only sustainable way to prevent ARF and RHD.1,15

Primary prevention: Treatment of streptococcal pharyngitis with penicillin is effective in some controlled temperate-climate settings (eg the US military) but has largely been unsuccessful in poorly resourced, highrisk populations. This may be because a preceding symptomatic GAS pharyngitis is less likely to present to the community health service.3 Despite a lack of clear evidence of effectiveness in remote Aboriginal and Torres Strait Islander communities, treating symptomatic pharyngitis in high-risk populations with intramuscular benzathine penicillin G is currently recommended.16 Oral phenoxymethyl penicillin V for 10 days is a second-line alternative but is not preferred. Some innovative nurse-led, school-based strategies for primary prevention have recently been proposed, especially in New Zealand, and may stimulate renewed interest in this approach, although they are expensive and their effectiveness in Australian settings is yet to be demonstrated.17,18 Healthy skin programs in remote communities aimed at reducing community prevalence of scabies and impetigo have had challenges of sustainability.19 The production and distribution of an effective vaccine is not likely to be available for many years.

Secondary prevention through ARF/RHD control programs: These are aimed at reducing recurrent ARF in people with known previous ARF or established RHD.20 Register-based programs endeavour to provide intramuscular benzathine penicillin G prophylaxis every 21 to 28 days for 10 years or more. Oral antibiotics are much less effective and not recommended for first-line use in prophylaxis.9 A comprehensive primary healthcare approach is key to effective service delivery of this treatment. ARF/RHD prevention programs also advocate regular clinical follow-up and echocardiograms, routine vaccinations,21 dental care, family planning advice and endocarditis prophylaxis.9,22 Delivery of effective secondary prophylaxis in remote settings has proven to be extremely challenging, especially in those communities at highest risk of ARF/RHD.23–26 A stepped-wedge randomised control trial of a multifaceted intervention to address barriers to effective control programs is currently underway in the Northern Territory.27
Several recent studies have investigated the role of echocardiographic screening for RHD in asymptomatic children. The rationale is that routine screening identifies children with previously undetected disease, including a substantially large group of children with ‘subclinical’ valvular changes.28–30 However, there is still no universally accepted approach to the interpretation and management of minor valvular changes and there are substantial resource limitations. Routine screening is yet to be widely advocated in Australia.29,31
As part of a coordinated control program, a structured care plan is recommended for all people with a history of ARF or established RHD based on a priority classification as outlined in Box 1.9

Tertiary prevention: This involves the treatment of people with established RHD and includes treatment of cardiac failure, cardiac dysrhythmias, other complications of RHD, and cardiac interventions including surgery, rehabilitation and long-term post-surgical anticoagulation. These topics are outside the scope of this chapter.

 

Practical considerations for clinical care

Practical considerations include the following.

  • Where ARF and/or RHD are notifiable conditions, clinicians should notify the relevant state and territory health authorities when they newly identify a patient with ARF or RHD.
  • Where there is an established regional and community register-based ARF/RHD prevention program, all patients should be included in the register and all subsequent related clinical ‘events’ and followup contacts recorded to keep it updated. This enables the register to function as a valuable, real-time clinical tool.32
  • Clinicians should recognise that minimising the stress and pain of prophylaxis injections is a key factor in people returning regularly.33 Administration of benzathine penicillin G requires proper training and experience (refer to ‘Resources’) and should not be performed by inexperienced or transient staff.26
  • Most people receiving secondary prophylaxis injections will be pre-teens, adolescents and young adults. Managing a chronic disease in this group requires special insight, patience and perseverance on the part of health professionals. Because most young people on an ARF/RHD program will be asymptomatic and prophylaxis is painful and frequent, extra attention to continuity of care is a critical component of a successful prevention program.26,33,34 (Refer to Chapter 4: The health of young people.)
  • Clinics may need to take a flexible approach to where injections are administered (eg clinic, home, clinic vehicle, school, opportunistic)33 and arrange clinical ‘fast tracking’ for young people who are attending clinic for their injections to reduce wait times.
  • Wherever possible, services such as echocardiography, specialist review and dental care should be provided in the community rather than having people travel, often long distances, to city-based tertiary care centres. People at highest risk of recurrent ARF/RHD are also often at increased risk of experiencing difficulties when travelling to a major centre. The reasons for this are multifactorial; people ‘coming to town’ must navigate a complex health system. They may also face culture shock, separation from family, strange surroundings, strange faces, perplexing paperwork, incomprehensible rules, complicated instructions, uncomfortable waiting rooms, transport misunderstandings, plus unfamiliar food and accommodation.
  • In clinical practice, true penicillin allergy is rare.35 If the label ‘penicillin allergy’ is in the clinical chart, it is important to invest additional effort to verify the type and severity of the allergic reaction as many reactions are mild, such as nausea or local injection irritation. Given a lack of prophylaxis can have disastrous sequelae, referral to an allergist or immunologist is recommended to determine if there is an absolute contraindication to penicillin.
  • Women of child-bearing age with known RHD who are taking anticoagulant medication for any reason require specialist family planning advice and careful follow-up because of the potential increase in medication-related fetal anomalies.9
  • ARF/RHD prevention programs sometimes refer to ‘monthly’, ‘4-weekly’ or ‘moon-cycle’ injections, but these are variably interpreted and can lead to inconsistent administration regimes. It is essential to emphasise that all prophylaxis injections must be given within a 21–28-day period. From the 29th day onwards, these are ‘days at risk’.36
  • A continuous quality improvement approach at the primary care level is recommended.37
  • A secondary prophylaxis program will also include regular GP and specialist review (physician, cardiologist and/or paediatrician), serial echocardiography to assess possible progression of valve disease, dental review as part of endocarditis prevention, influenza and pneumococcal vaccinations plus family planning advice for women of reproductive age. The program should provide ongoing family education and support.9
  • People having ARF/RHD secondary prophylaxis (and/or a responsible family member or carer) can be given a laminated card with relevant details of their treatment to assist with administration of prophylaxis when outside their community.
  • Regular mobile phone text message reminders for benzathine penicillin G injections have been used in some community clinics with good results (Marea Fittock, RHDAustralia, personal communication, March 2017), although this has not been formally evaluated and might not be acceptable or effective for everyone.
  • Addressing language and communication barriers is core to successful programs. As mentioned, continuity of care is an important factor, and this is best achieved through community-based health workers.34
  • The language used by healthcare providers is also important. Because ARF is a sensitive marker of childhood disadvantage, the highest risk settings are frequently households with difficulties of overcrowding, poverty, poor health literacy, unemployment and day-to-day domestic chaos. The consistent delivery of high-quality health services to this population poses many challenges for affected individuals, families and health service personnel. Terms such as ‘adherence’ or ‘compliance’ to treatment have implicit value judgements and are therefore best avoided.

 

Policy considerations for delivery of successful ARF/RHD control programs

Policy considerations include the following.

  • Secondary prophylaxis for ARF/RHD is a complex and demanding endeavour and should be provided within the framework of a formal register-based ARF program – preferably sited within a state or territory health system.32
  • An ARF/RHD prevention program should be guided by a steering (or advisory) committee with strong representation from practising clinicians experienced in the day-to-day management of ARF/RHD and from community representatives. The steering group should have clear goals and meet regularly to provide advice to program staff on priority activities, develop policy, publish new developments and report outcomes to clinicians and government.
  • An ARF/RHD prevention program should focus on reporting outcomes such as new cases of ARF, and recurrences; hospitalisations for conditions such as cardiac failure, stroke and endocarditis; cardiac interventions, including surgery; complications of cardiac interventions; and all-cause mortality. Appendix 3 of the Australian guideline provides a set of key performance indicators for ARF/RHD that cover these issues in more detail.9 In addition to reporting process measures such as numbers of injections administered, it is essential to provide data on real health outcomes to deliver a true assessment of program achievement.
  • The ARF/RHD register must be updated in real time for it to be clinically useful. As such, all relevant practising clinicians (doctors, nurses, health workers, allied health professionals) in both the government and the Aboriginal Community Controlled Health Service sectors should have password-protected online access to the register.38
  • Generally, routine secondary prevention is best provided in primary healthcare settings rather than by tertiary care organisations and hospital-based consultant specialists. However, communities with the highest incidence of ARF also experience a high turnover of medical and nursing staff. Thus, resident primary healthcare staff, particularly Aboriginal and Torres Strait Islander health workers, must be appropriately skilled and have ongoing training to maintain their skills. With a properly supported workforce, the vast majority of secondary prophylaxis can be delivered by local Aboriginal and Torres Strait Islander health workers.33,38
  • From the outset, strong community engagement to identify the optimal ways to deliver each community’s ARF/RHD prevention service is essential to enhance its effectiveness and sustainability. Communities should be regularly informed of the program’s outcomes, and interventions to enhance the health literacy of people with ARF/RHD and their families is an essential and ongoing task.

 

National guide to a preventive health assessment for Aboriginal and Torres Strait Islander people

 





 
 
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