Diagnosis of ARF in children Welcome to Rheumatic Heart Disease Australia’s Health Provider Education series. Educational resources developed for clinicians by clinicians. Rheumatic Heart Disease Australia is an initiative of Baker IDI, James Cook University, and Menzies School of Health Research and is supported by the Commonwealth Department of Health and Ageing. This module will discuss the paediatric diagnosis of acute rheumatic fever with a focus on the Australian perspective.

Speakers Alan Ruben FRACP, FAFPHM Paediatrician and Public Health Physician, Apunipima Cape York Health Council, Cairns and Hinterland Health Service District, Queensland Health. Alan is a paediatrician and public health physician who has worked in Aboriginal health for over 20 years. Ben Reeves MBBS, FRACP Paediatric cardiologist, Cairns and Hinterland Health Service District, Queensland Health. Ben is a paediatric cardiologist based in Cairns, providing outreach paediatric cardiology services to Cape York and the Torres Strait. This presentation was developed with the assistance of Alan Ruben, and Ben Reeves, who are both based in Cairns in far northern Queensland, Australia. Both specialists have a longstanding interest in acute rheumatic fever and rheumatic heart disease, and remote health care delivery. Alan is a paediatrician and public health physician who has worked in Aboriginal Health for over 20 years. He has also spent a significant amount of time in the Pacific, working with WHO, UNICEF and AusAID. Ben is a paediatric cardiologist, providing outreach paediatric cardiology services to Cape York and the Torres Strait Islands. His interest in rheumatic heart disease began in Fiji, where he worked for two years as a general paediatrician before completing his paediatric cardiology training at the Mater Children’s hospital in Brisbane. November 2012

Learning objectives Appreciate the pathway to ARF and then RHD Recognize who is at risk for ARF/RHD Understand the Jones criteria used for diagnosis Present the recommended investigations Outline current management guidelines The learning objectives of this presentation are to provide an understanding of the sequence of events leading to acute rheumatic fever and subsequently rheumatic heart disease. You will learn how to recognize those most at risk for acute rheumatic fever and rheumatic heart disease, and gain an understanding of the Jones criteria used for diagnosis. The recommended investigations and current treatment guidelines will be outlined. November 2012

Take home messages Incidence in Aboriginal Australians and Torres Strait Islander people amongst the highest in the world Predominantly affects children aged 5 to 15 Largely affects disadvantaged populations High index of suspicion in high risk populations Diagnosis needs clinical criteria and investigation results Diagnosis often requires hospital admission The take home messages include that the incidence of acute rheumatic fever and rheumatic heart disease in Australian Aboriginal and Torres Strait Islander peoples is amongst the highest recorded in the world. Acute rheumatic fever predominantly affects children from disadvantaged populations aged between 5 to 15 years. A high index of suspicion is needed for those at greatest risk of disease, which means people living in high incidence populations. Rheumatic fever diagnosis demands that clinical criteria be met, and that relevant investigation results are compatible. Because the diagnosis can be complex, inpatient admission is often required. November 2012

Abbreviations AR aortic regurgitation ARF acute rheumatic fever BPG benzathine penicillin G CRP C-reactive protein ESR erythrocyte sedimentation rate GAS group A beta-haemolytic streptococcus MR mitral regurgitation RHD rheumatic heart disease Before proceeding further, it is worthwhile defining the terms and abbreviations that will used during this presentation. AR is used for aortic regurgitation. This is when there is a backward flow of blood from the aorta into the left ventricle, because the aortic valve does not close properly. Acute rheumatic fever is often termed ARF. This is a complication of Group A streptococcal infection, a common bacteria found in the throat and on the skin. In susceptible individuals, and particularly children, immune mimicry results in inappropriate cross reactivity between streptococcal and patient tissue antigens associated with joints, skin, brain and, most importantly here, the heart. BPG, or benzathine penicillin G, remains the most effective antibiotic for both the prevention and management of GAS pharyngitis and ARF. CRP is C-reactive protein, an acute phase protein produced by the liver during an inflammatory reaction. The erythrocyte sedimentation rate, or ESR, is the rate at which red blood cells settle. It is a non-specific measure of inflammation. Group A beta haemolytic streptococcus, otherwise known as ‘G’ ‘A’ ‘S’ or GAS, causes approximately twenty to forty per cent of symptomatic pharyngitis or sore throat. GAS-related pharyngitis is thought to be the major driver of ARF. Mitral regurgitation, or MR, occurs when blood leaks back from the left ventricle into the left atrium due to incomplete closure of the mitral valve Rheumatic heart disease, or RHD, is the chronic sequelae of recurrent episodes of ARF. Recurrent carditis with valve inflammation leads to scarring, distortion and eventual valve dysfunction. November 2012

More information – Guidelines For those who would like more information regarding the prevention, diagnosis and management of ARF and RHD, this can be found in the Australian Guidelines. These guidelines were substantially updated and revised in 2012, and are available at the RHD Australia website. www.rhdaustralia.org.au November 2012

More information – Quick reference There is also a Quick Reference Guide which can be a useful tool for busy clinicians, population health staff and managers. www.rhdaustralia.org.au November 2012

More information – other modules Further education modules are available at the RHD Australia Health Provider Education website. November 2012

ARF: some basics 3-6% of any population susceptible Incidence and prevalence in females >males ARF/RHD can run in families Specific genetic markers have been identified There is no racial predisposition Let’s commence by highlighting a few basic facts regarding acute rheumatic fever. Approximately 3 to 6% of any population is susceptible to ARF, with the incidence and prevalence in females greater than in males. ARF and rheumatic heart disease can run in families, and although specific genetic markers have been identified, there is no racial predisposition to disease. Thus the higher incidence of ARF in Aboriginal and Torres Strait Islander people is not due to an inherently higher susceptibility to ARF but rather a greater exposure to GAS infection.   November 2012

Australian setting Amongst the highest rates in the world ARF commonest in remote and disadvantaged areas Some Australian medical staff unfamiliar with ARF So what in particular ought we be concerned about in the Australian setting? Australian rates are amongst the highest recorded in the world, with ARF most frequently occurring in remote and disadvantaged areas and populations. Because ARF has largely disappeared in Australian urban areas, Australian health care providers are often unfamiliar with the diagnosis, treatment and management of this condition. November 2012

Environment If Aboriginal and Torres Strait Islander people are not inherently more susceptible to ARF, then what is driving this greater burden of disease? Improved living standards resulted in reduced transmission of GAS in the early 20th century, and almost the disappearance of ARF and RHD in the high income and industrialised world. It is a different story in low and middle income nations, where exposure to GAS infection remains high, and surveillance, diagnosis, secondary prophylaxis and treatment remain under resourced. A greater risk of ARF is associated with overcrowding, poor sanitation, and other conditions that may easily result in the rapid transmission or multiple exposures to GAS. For more information on the primary and primordial prevention of ARF and RHD, please refer to the module in this health provider education series. November 2012

Risk factors Established clear link with poverty household overcrowding poor sanitation housing quality and appropriateness educational disadvantage Limited access to health services variability of health infrastructure and follow up Geographically remote So what might be some of the underlying social and environmental determinants driving greater exposure to GAS?. History has taught us that ARF has a clear link with poverty, household overcrowding, poor sanitation, housing quality and appropriateness, and educational disadvantage. Almost half of Aboriginal and Torres Strait Islander households earn an income in the bottom quarter of the national average. The average weekly household income for Aboriginal and Torres Strait Islander peoples is only 62% that of non-Indigenous Australians. Remoteness is also another driving of disadvantage. This reflects the distance people have to travel to obtain services. 68% of Aboriginal and Torres Strait Islander peoples live outside major cities, with 43% in regional areas, 9% in remote, and 15% in very remote areas. The combination of economic disadvantage and remoteness in turn translates to environmental disadvantage with housing quality and household overcrowding being more common for Aboriginal and Torres Strait Islander people. Australian communities at greatest risk of this inter-related nexus of remoteness, poverty and environmental disadvantage include those in remote northern and Central Australia ,extending from the Torres Strait adjacent to Papua New Guinea through to the desert regions of southern WA. November 2012

Exaggerated immune response Acute rheumatic fever – ARF Chorea Carditis GAS pharyngitis Exaggerated immune response So how does ARF develop? This flowchart provides a graphic depiction of the disease process. Pharyngitis caused by GAS infection results in an exaggerated immune response in susceptible individuals, causing any or all of the symptoms listed here. Chorea, carditis, arthritis and fever. Recognition and diagnosis of ARF can reduce progression to RHD through the use of prophylactic antibiotics which prevent further episodes of ARF. A high level of suspicion in target populations will facilitate early diagnosis and commencement of prophylaxis. There is clear evidence that prophylaxis with benzathine penicillin reduces GAS infections, prevents recurrent attacks of ARF, and progressive RHD. Arthritis Fever November 2012

ARF progression * Valve damage is cumulative and silent How does ARF progress to rheumatic heart disease? Repeated episodes of ARF and recurrent heart inflammation eventually result in scarring and permanent damage to heart valves. These damaged heart valves do not function properly, and can eventually lead to stroke, heart infection, called endocarditis, and heart failure. ARF and RHD are a preventable cause of disability and early death, and are associated with an increased need for surgical heart and other interventions. Rheumatic heart disease (RHD) Cardiac failure, early death ARF recurs - often many times November 2012

Jones criteria So how do we accurately diagnose acute rheumatic fever? ARF diagnosis is based on the modified Jones criteria, seen here as presented in the Australian guideline for the prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. These criteria have been modified and are slightly different in high risk and low risk populations, with the changes in high risk populations being made to increase sensitivity and avoid under-diagnosis. They rely on looking for and confirming a combination of major and minor criteria. The diagnosis of an initial episode of ARF requires two major manifestations, or one major and two minor manifestations, with evidence of recent GAS infection. November 2012

Diagnosis and GAS Definite initial or recurrent ARF diagnosis requires: 2 major plus evidence GAS infection 1 major plus 2 minor plus evidence of GAS infection Throat swab ASOT Anti DNAse B No other probable diagnosis As a confirmed diagnosis of both initial and recurrent ARF requires evidence of recent GAS infection, let’s look at how that is confirmed. Remember in all cases of ARF, except those associated with chorea, evidence of recent GAS infection is essential. Evidence can be demonstrated by a positive throat swab OR serological or antibody evidence of exposure to GAS based on a raised ASOT or anti-DNAse. If serology is negative AND ARF continues to be suspected, and if an alternate diagnosis is not made, serology should be repeated after two weeks, because an increase in titer can be delayed. Since antibodies directed at GAS are produced as a delayed hypersensitivity reaction, there is no normal value, and the presence of these antibodies only indicates an exposure to GAS. Many people exposed to GAS remain asymptomatic, so the mere presence of positive GAS serology does not in itself indicate ARF. Once evidence of recent GAS infection is confirmed, diagnosis of an initial episode of ARF relies on also having 2 major criteria or 1 major and 2 minor criteria, with no other probable diagnosis. It is worthwhile to note the latest Australian guidelines have included additional categories and requirements for recurrent ARF and probable ARF. November 2012

Major manifestations This chart from the Australian Guidelines summarises and describes the major manifestations required for the diagnosis of ARF in a high risk population. They are arthritis, polyarthralgia, Sydenham’s Chorea, carditis, subcutaneous nodules and erythema marginatum. As we progress through this module, a more detailed description will be provided for each of these major manifestations. November 2012

Polyarthritis or aseptic mono-arthritis or polyarthralgia Major manifestations High risk groups Polyarthritis or aseptic mono-arthritis or polyarthralgia Carditis (including subclinical evidence of rheumatic valvulitis on echocardiogram) Chorea Erythema marginatum Subcutaneous nodules Low Risk groups Polyarthritis Carditis Chorea Let’s look at high risk groups, and start with polyarthritis, aseptic mono-arthritis and polyarthralgia. Erythema marginatum Subcutaneous nodules November 2012

Arthritis Monoarthritis present in 17% of ARF presentations Migratory asymmetric polyarthritis Affects peripheral large joints Often intense pain – will not tolerate passive movement Limited duration: 2 days to 3 weeks Dramatic response to salicylates rapid response assists diagnosis Arthritis and arthralgia are the most common presentations of ARF in children. Classically, the arthritis is a polyarthritis, usually affecting peripheral large joints, with joints in the lower limbs affected more frequently than the upper limbs. The polyarthritis is often extremely painful, with movement avoided and passive movement not tolerated. Sometimes, other significant signs of inflammation are absent. Usually asymmetrical and migratory, resolution in one joint is often followed by reappearance in another shortly afterwards. When this happens in an adjacent joint, the arthritis appears to be migrating. Sometimes however the arthritis is additive. The arthritis is usually of limited duration, two days to 3 weeks, although it can occasionally last longer. Treatment with aspirin or other non-steroidal anti-inflammatory drugs results in dramatic improvement, sometimes with relief of symptoms occurring overnight, and definitely within 2 to 3 days. This rapid response can assist with diagnosis. If anti-inflammatory treatment is ceased, arthritis may recur, generally within one to 2 months of the original episode. November 2012

Can a monarthritis be ARF? In high risk populations: aseptic monoarthritis can be a major manifestation monoarthritis often associated with carditis if joint aspirate sterile, prior to treatment for septic arthritis, investigate for ARF In high risk populations, a monoarthritis can be considered as a major manifestation of ARF, as it is often associated with carditis. One important point to remember is that aseptic monoarthritis should always be investigated and followed up as possible ARF even if preemptive antibiotic treatment is given. Collaboration between physicians and orthopaedic surgeons is valuable in this setting. November 2012

Polyarthralgia A major criteria ONLY in high risk populations: Multiple painful joints Can be migratory Unlike arthritis lacks: Effusions Heat Morning stiffness Polyarthralgia can be less dramatic than the classical polyarthritis and monoarthritis seen in ARF. In high risk populations the modified Australian Jones criteria for ARF classify this as a major manifestation whilst in other populations in remains a minor manifestation of ARF. The differentiation of arthralgia from arthritis is clinical and is based on painful joints without clinical signs such as effusions and heat, along with no history of morning stiffness. November 2012

Carditis Can involve all layers of the heart Pericardium – can cause effusions Myocardium – affects heart function and conduction Endocardium – the classic valve lesions MR then AR most common lesions Right sided valves rarely involved Stenosis is a late finding Let’s now describe the second major manifestation, carditis. Carditis can involve all the layers of the heart. If the pericardium is affected, pericardial effusions can result. Heart function and conduction will be impacted if the myocardium is involved. The classic valvular lesions seen ARF are caused when the endocardium is involved, with the most common being mitral regurgitation or MR and then aortic regurgitation or AR. The right sided heart valves, the tricuspid and pulmonary valves are rarely involved. It is important to remember that subclinical carditis, that is heart valve inflammation demonstrated on echocardiography without an associated murmur, is now classified as a major criteria in the diagnosis of ARF in high risk populations November 2012 22

Carditis: investigations Early echocardiography essential repeated at 2 to 6 weeks Chest x-ray Electrocardiogram So what are the recommended investigations if carditis is suspected? Echocardiography is key. It can distinguish innocent murmurs and detect subclinical carditis. However it is often difficult to access echo services, especially in remote areas with high incidence of ARF and this is one reason why cases of suspected ARF should be admitted to hospital. Determining valvular disease can be difficult and often requires follow-up echocardiogram examination, as initial findings may be normal with changes only appearing after 2 to 6 weeks. Carditis does not always occur in ARF and incidence varies between 30 to 80%. Because the changes of carditis can be delayed, it is important that all patients with suspected ARF have follow-up echocardiography. Remember a normal echocardiogram does not exclude a diagnosis of ARF. November 2012

Carditis: treatment Often requires inpatient bed rest and care if : moderate/severe carditis suspected by clinical findings Consider steroids for severe carditis If signs of heart failure or cardiomegaly consider diuretics and ACE inhibitors An what about the management of acute ARF-related carditis? The key is admission to hospital, close observations and investigation, and consideration of bed rest along with diuretics and ACE inhibitors if heart failure develops. Steroids may be considered in myocarditis with heart failure but usually heart failure in this setting is due to valve dysfunction. November 2012

Sydenham’s chorea Rapid, uncoordinated jerking movements Primarily the face, feet and hands Female to male ratio of 2:1 Occurs up to 6 months after acute infection Mostly children, 5 to 13 years “Milkmaids” sign Tongue fasciculations Emotional lability We will move on to discuss the next major manifestation, Sydenham's chorea. This is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. Sydenham's chorea results from childhood infection with GAS and is reported to occur in 20-30% of patients with ARF. The disease is usually latent, occurring up to 6 months after the acute infection, but may occasionally be the presenting symptom of acute rheumatic fever. Sydenham's Chorea is more common in females than males and most patients are younger than 18 years of age. Adult onset of Sydenham's Chorea is comparatively rare and most of the adult cases are associated with exacerbation of chorea following childhood Sydenham's Chorea. Sydenham's chorea is characterized by the acute onset (sometimes a few hours) of neurologic symptoms, classically chorea, usually affecting all limbs. Other neurologic symptoms include behavior change, dysarthria, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache, slowed cognition, facial grimacing, fidgetiness and hypotonia. Also, there may be tongue fasciculations ("bag of worms"), and a "milk maids sign", which is a relapsing grip demonstrated by alternate increases and decreases in tension, as if hand milking. Emotional changes, such as easy crying or inappropriate laughing, may precede the development of chorea and, in some cases, deteriorating school performance can be the initial concern. The hypotonia may be severe and the extremities may appear paralysed. The cranial nerves are not involved, but speech often is abnormal and is described as "jerky" with sudden changes in pitch and loudness November 2012

Erythema marginatum Rare finding reported in less than 2% Australian Aboriginals difficult to see on dark skin Presence of rash diagnostic of ARF Pale center and darker margins Blanch under pressure Circular snake like pattern Occurs on trunk and extremities Not itchy or painful Erythema marginatum is the next major manifestation, and an example of the rash can be seen here. The rash is difficult to see on dark skin, and will blanch under pressure. This is a rare finding, reported in less than 2% of Aboriginal and Torres Strait Islander patients with ARF. It is an erythematous rash with a pale centre and a darker margin. It has a circular pattern and predominantly occurs on trunk and extremities, and is neither itchy nor painful. November 2012

Subcutaneous nodules Rare, only seen in 2% cases Highly specific of ARF Strongly associated with carditis Round firm and freely mobile 0.5 to 2.0 cm in diameter Appear 1 to 2 weeks after symptom onset Occur in crops of up to 12 over elbows knees, wrists, ankles, achilles tendons, occiput, and posterior spinal processes The final major manifestation that need description is subcutaneous nodules. Subcutaneous nodules are also rare but highly specific for ARF and strongly associated with carditis. The nodules are round, firm, and freely mobile, with a diameter of half to 2 cm. They occur predominantly over pressure areas including the extensor surface of joints, in particular the elbows, knees and ankles. They can also occur over the head and posterior spinal processes. November 2012

Polyarthralgia or aseptic monoarthritis Minor manifestations High risk groups Monoarthralgia Fever ESR≥30 mm/h or CRP ≥30 mg/L ECG changes Low Risk groups Polyarthralgia or aseptic monoarthritis Let’s now discuss the minor manifestations for ARF diagnosis. In high-risk groups these are monoarthralgia, or joint pain, fever, raised inflammatory markers and ECG evidence of heart block. November 2012

Fever Temperature greater than 38C In the absence of fever documentation reliable history if anti-inflammatory therapy given already given Fever can be either documented and greater than 38 degrees Celsius, or a reliable history of fever if anti-inflammatory therapy has been given, or in the absence of a documented fever. It is important that the temperature is measured and recorded at the time of initial presentation. November 2012

ESR & CRP Repeat serology 10 to 14 days if not confirmatory To satisfy minor criteria: serum CRP ≥30mg/L ESR ≥30mm/hr Elevated WBC insensitive marker for ARF Inflammatory markers are non-specific indicators for ARF. Nonetheless ESR and CRP elevations are considered minor manifestations, with the criteria requiring that either of these be 30 or greater. The CRP tends to rise and fall more quickly than ESR., so that performing both can be useful. CRP is often easier to perform in a remote setting. November 2012

ECG If ARF suspected always ECG Check P-R interval Normal 0.16 sec if 3 to 12 years old If prolonged repeat ECG in 1 to 2 months If P-R interval returns to normal: ARF more likely An ECG should always be performed when ARF is suspected. A prolonged PR interval is the most common manifestation, although 2nd degree and complete heart block can also occur. The normal PR interval for a 3 to 12 year old is 0.16 of a second, and for 12 to 16 year old is 0.18 of a second. If the PR interval is prolonged, repeat the ECG after 1 to 2 months. The changes are more likely to be related to ARF if the ECG returns to normal, and this therefore increases the probability of diagnosis. November 2012

Diagnosis: key investigations Investigations are important both to confirm a diagnosis of ARF and for diagnosis of other conditions which may be mistaken for ARF Throat swab, antistreptococcal serology (ASOT and antiDNAse), white cell count, differential, ESR, CRP, ECG and echocardiogram are always necessary. If heart failure is suspected a chest x-ray may be useful both to confirm pulmonary oedema and to rule out other causes of shortness of breath, including pneumonia. If infective endocarditis or septic arthritis is considered a possibility , multiple sets of blood cultures should be taken prior to the commencement of antibiotics. Any patient from a high risk population presenting with an acute monoarthritis should be considered to have possible septic arthritis, and a joint aspirate should be performed. In children this may be a difficult and expert advice will assist with this decision. November 2012

Differential diagnosis Let’s now consider the differential diagnosis of ARF. This chart from the Australian guidelines outlines the differential diagnosis of the three most common major manifestations of acute rheumatic fever, polyarthritis with fever, carditis and chorea. Some of the diseases listed are extremely rare, and some, such as Lyme disease, do not occur in Australia. There are only a few important conditions which need to be considered in most patients presenting with possible ARF. These include septic arthritis and infective endocarditis, both of which have high levels of associated morbidity if missed. Gout and pseuodogout usually occur in older adults and leukaemia and lymphoma presenting with polyarthritis and fever is very uncommon. Conditions such as disseminated gonococcal infection however, are not uncommon especially in sexually active adolescents. November 2012

Diagnosis key points ARF remains a difficult diagnosis requires recommended tests to be performed High index of suspicion for populations at greatest risk Cardiology opinion recommended for suspected ARF In high risk populations also consider ARF if: child < 5 years of age presents with arthritis Monoarthritis is a common presentation Simple falls rarely cause joint effusions Hospital admission recommended for initial presentations So what are the key diagnostic points? ARF can be difficult to diagnose, as there are multiple, and sometimes repeated tests recommended for confirmation. A high index of suspicion is needed in high risk populations to avoid possible missed diagnosis. Specialist paediatrician input can be helpful if you suspect ARF. Consider the possibility of ARF in any child from a high risk population presenting with arthritis, especially if more than 5 years of age. Keep in mind that monoarthritis is a common presentation of ARF in Aboriginal and Torres Strait Islander populations. Remember that simple falls rarely cause joint effusions. And finally admission to hospital is recommended for all initial presentations, in order to confirm diagnosis, assess severity of disease and develop a treatment plan. November 2012

Probable ARF Unfortunately a definite diagnosis of ARF cannot be made in all cases even though no alternate diagnosis is reached. In this case a diagnosis of probable ARF should be considered. The Australian guidelines provide a reference flowchart for the management and follow up of probable ARF and subdivides this group into Uncertain and Highly Suspected groups. It should be noted that secondary prophylaxis is recommended for both these groups. A diagnosis of probable ARF can be made when a patient has a clinical presentation compatible with ARF but falls short of meeting the diagnostic criteria by either one major or one minor manifestation, or in the absence of definitive GAS serology. In this case close follow-up is essential both to determine a definite episode of ARF, and to ensure that another condition is not being missed. The Australian guidelines have also made the criteria for diagnosing a recurrent episode of ARF more sensitive by allowing this diagnosis to be made even if no major and only three minor criteria are met. November 2012

ARF diagnosis and management First requires diagnosis then secondary prophylaxis Inpatient assessment recommended Specialist review for ongoing management Bed rest NSAIDs Initial then follow up echocardiography Chest x-ray If heart failure: ACE inhibitors, diuretics Consider steroids for carditis We will now conclude by recapping the principles of diagnosis and management of ARF in children and adolescents. Any effective treatment first requires diagnosis, and then commencement of secondary prophylaxis. Remember that a diagnosis of probable ARF also warrant prophylaxis and close follow-up. Hospitalisation is generally necessary for a number of reasons. First to complete the recommended tests and investigations, second to access specialist opinion and review, and third to arrange and coordinate an appropriate treatment plan. Early specialist input is helpful to ensure alternate diagnoses are considered and to facilitate ongoing follow-up and management. Bed rest and aspirin or NSAIDs may be required. Initial and follow up echocardiography, is important. Medical management with ACE inhibitors and diuretics may be needed in the setting of heart failure and severe myocarditis may benefit with commencement of steroids. Finally chorea will often settle spontaneously but if troublesome should prompt specialist input. November 2012

Principles of secondary prevention Secondary prevention first requires the diagnosis of ARF/RHD Long term antimicrobial prophylaxis prevents recurrent ARF but significant challenges in service delivery Success requires: register-based program effective recall system functioning primary health care service As we conclude, it’s useful to cover the basic principles of secondary prevention. For more information on secondary prevention please see the appropriate module in this health provider education series. Secondary prevention first requires the diagnosis of acute rheumatic fever and/or rheumatic heart disease. Once the diagnosis has been made, the person must be given long term antimicrobial or antibiotic prophylaxis to prevent recurrent episodes of acute rheumatic fever, and associated valve inflammation which can progress to rheumatic heart disease. There are significant challenges in the delivery of antimicrobial prophylaxis, and successful delivery requires a register based secondary prevention program, effective recall systems, and a functioning primary health care service.       November 2012

Take home messages Incidence in Aboriginal Australians and Torres Strait Islander people amongst the highest in the world Predominantly affects children aged 5 to 15 Largely affects disadvantaged populations High index of suspicion in high risk populations Diagnosis needs clinical criteria and investigation results Diagnosis often requires hospital admission In closing, let’s revisit our take home messages: Remember the incidence of acute rheumatic fever and rheumatic heart disease in Australian Aboriginal and Torres Strait Islander peoples is amongst the highest recorded in the world. Acute rheumatic fever predominantly affects children from disadvantaged populations between 5 to 15 years of age. A high index of suspicion is needed for those at greatest risk of disease, which means people living in high incidence populations. Rheumatic fever diagnosis demands that clinical criteria be met, and that relevant investigation results are compatible. Because the diagnosis can be complex, inpatient admission is often required. November 2012

More? If you would like to know more about ARF and RHD please refer to the new Australian guidelines. Further education modules are available at the RHD Australia Health Provider Education website. November 2012

More? Register for… Downloadable PowerPoint presentations Additional resources Additional assessment items for training providers You can also register at the Health Provider Education website for additional resources to download this and other power-point presentations for your own use in your local practice and additional assessment items for training providers. If you would like to be notified about new modules and updates, please ‘Like’ us on Facebook at the provided below. www.facebook.com/RHDEd for notification about new modules and updates November 2012

How’d you go? Test your knowledge with a brief self-assessment quiz Finally for those of you who would like to test your knowledge regarding the information presented in this module, please go to the Brief Self-Assessment Quiz at the link provided on this website. November 2012

Diagnosis of ARF in children Welcome to Rheumatic Heart Disease Australia’s Health Provider Education series. Educational resources developed for clinicians by clinicians. Rheumatic Heart Disease Australia is an initiative of Baker IDI, James Cook University, and Menzies School of Health Research and is supported by the Commonwealth Department of Health and Ageing. This module will discuss the paediatric diagnosis of acute rheumatic fever with a focus on the Australian perspective.