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File 64 Immune complications
Acute Rheumatic Fever and Rheumatic Heart Disease
 Treat streptococcal throat and skin infections with a single injection of Benzathine
 Any case of polyarthritis and fever in children should be considered as possibly
acute rheumatic fever (ARF) and transferred to hospital for investigation and
 Since treatment involves painful monthly injections for at least 10 years, it is
important to make as accurate a diagnosis as possible on presentation by admitting
to hospital for observation without aspirin or steroid treatment and arranging for an
echocardiogram. If this is not done on presentation, it can be very difficult to be sure
about the diagnosis at a later time
 Penicillin prophylaxis is critical to prevent recurrences of acute rheumatic fever
with heart disease
 Acute Rheumatic Fever (ARF) is an auto-immune response to bacterial infection
with group A streptococcus (GAS) [3] in the throat or possible skin, it affects the
joints, nervous system and skin and still occurs in Aboriginal and Torres Strait
Islander communities
 Often the first episode is not diagnosed
 Any episode may cause heart damage -Rheumatic Health Disease (RHD)
 Involvement of the heart can be fatal during the acute stage or lead to rheumatic
heart disease, a chronic condition resulting in scarring and deformity of the heart
 ARF is predominantly a disease of children aged between 5 and 14 years although
recurrent episodes may continue well into the fourth decade of life [3]
 Patients with recurrent episodes of ARF have a higher risk of developing RHD
Related topics:
 Upper respiratory tract infection (child), page 527
 Upper respiratory tract infection (adult), page 227
 Bacterial skin infections, page 279
May present with:
 Fever and generally unwell
 Painful tender swollen joints. May involve one or more joints and often moves
from one joint to another (migratory polyarthritis). Any joint can be involved but
most commonly affects the large joints of the limbs – knees, ankles, elbows
 Abdominal pain
 May be unable to walk and have to be carried
 Strange jerky movements of the trunk and/or limbs which the patient cannot
control (chorea), which disappears when asleep
 Skin rash – not often seen in Aboriginal and Torres Strait Islander peoples
 Recent history of a sore throat or skin infection
 Breathlessness if cardiac involvement has resulted in heart failure; chest pain
 May have a past history of acute rheumatic fever
Immediate management – not applicable
Clinical assessment:
 Obtain complete patient history including
File 64 Immune complications
past episodes of ARF / RHD – is patient up to date with monthly penicillin
 any history of sore throat and length of time since present
 any history of skin infections and length of time since present
 any measures taken to treat presenting concern
 current medications
Perform standard clinical observations + oxygen saturations
Perform physical examination
 inspect throat for signs of throat infection
 inspect and palpate all skin surfaces for signs of skin infection and skin rash
– pink with definite rounded borders, occurring mainly on the trunk, never on
the face; blanches under pressure (erythema marginatum)
 inspect and palpate joints for swollen, tender joints and small nodules (pea
sized), painless, overlying bony prominences
 auscultate the heart, for heart murmur
 look for indications of heart failure - increased heart rate or irregular (heart
block), increased respiratory rate, basal crackles in chest, liver enlargement,
ankle oedema
Diagnostic criteria ARF [3]
 Accurate diagnosis of ARF requires a combination of clinical and laboratory
criteria and laboratory evidence of a recent group A streptococcal (GAS)
 It takes an experienced Medical Specialist to review the clinical presentation with
pathology results to confirm the diagnosis to determine ongoing management.
This is most easily achieved by admission to a tertiary facility.
 The diagnosis of ARF requires special interpretation of the Jones Criteria and is
notifiable – contact the Rheumatic Fever / Rheumatic Heart Disease register in
your area and the Public Health Unit (Unlike most other notifiable disease,
ARF is not based upon a laboratory diagnosis, and notification therefore
has to be done by the health care worker)
Diagnosis RHD
 Diagnosis of RHD is based on the degree of damage to the heart
 This is confirmed through the use of echocardiogram by an experienced clinician
 Serial echocardiography plays a critical role in the diagnosis and management
Australian guidelines for the diagnosis of Acute Rheumatic Fever in high risk
groups [3]
File 64 Immune complications
An initial episode of ARF is diagnosed when the Jones Criteria are fulfilled. This requires
the presence of two major manifestations or one major and two minor manifestations, in
the presence of evidence of a recent group A streptococcal infection.
However chorea may occur as the only manifestation of ARF.
A recurrent attack of ARF in a patient with known past ARF or RHD in high risk groups
requires 2 major or 1 major and 2 minor or 3 minor manifestations plus evidence of a
preceding GAS infection
Major manifestations:
Carditis – (including subclinical evidence of rheumatic valve disease on
Polyarthritis or aseptic monoarthritis or polyarthralgia. Usually migratory ie finishes in
one joint, begins in another
Chorea - strange jerky movements of the trunk and or limbs which the patient cannot
Erythema marginatum – pink skin rash with definite rounded borders, occurring
mainly on the trunk, never of the face, and blanches under pressure
Subcutaneous nodules – small painless pea sized nodules overlying bony
prominences (e.g. elbows)
It is recommended that echocardiographically suggested valve damage (subclinical or
otherwise) diagnosed by a clinician with experience in enchocardiographys of patients with
ARF/RHD be included as a major manifestation [3]
Minor manifestations
Clinical findings - fever
Laboratory findings
elevated acute phase reactants – ESR ≥ 30 mm/hr or CRP ≥ 30mg/L
prolonged PR interval on ECG
Supporting evidence of Group A streptococcal infection
Positive throat culture
Elevated or rising streptococcal antibody titre
These serological titres are often high at baseline in Aboriginal and Torres Strait Islander
community children because of repeated skin infections with GAS. So acceptable
evidence for recent GAS infection are either 1) titres of > 2 x reference eg ASOT > 400
IU/mL or Anti-DNase B > 600 U/mL or 2) a rising titre when repeated after 10-14 days.
 Consult MO who will likely advise:
 evacuation / hospitalisation – confirmation and management of ARF should
occur in hospital (a wrong diagnosis either positive or negative will have
serious consequences)
 blood for FBC, ESR, C-reactive protein (CRP), ASOT, anti-DNase B
 throat swab and ECG
 Provide pain relief as required. Use Paracetamol as analgesic. Do not give
Aspirin as it may cause signs to disappear and prevent diagnosis being
 There is no specific cure for ARF. Treatment consists of:
 IMI Penicillin to eliminate streptococci (even if Group A streptococci not
isolated on culture)
 oral penicillin should not normally be used, as compliance with 10 days of
treatment cannot be guaranteed
See Simple Analgesia Protocol (back cover)
Penicillin Benzathine (Bicillin LA)
File 64 Immune complications
Authorised Indigenous Health Workers and Isolated Practice Area Paramedics must consult MO
Rural and Isolated Practice Endorsed Registered Nurses may proceed
Nurse Practitioners may proceed
Route of
Recommended Dosage
900 mg in
3 kg to under 6 kg 225 mg (0.5mL)
6 kg to under 10 kg 337.5 mg (0.75mL)
10 kg to under 450 mg (1mL)
units =
15 kg to under 20 kg 675 mg (1.5mL)
20 kg & over & adults 900 mg (2mL)
Provide Consumer Medicine Information if available:
Management of Associated Emergency: As for severe allergic reactions. See Anaphylaxis
Apply EMLA cream to the injection site 30 – 60 minutes prior to injection, allow to warm up to room temp
If reliably documented allergy to Penicillin treat with Erythromycin [3].
If Penicillin allergy not reliably documented arrange for testing in hospital
Authorised Indigenous Health Workers and Isolated Practice Area Paramedic must consult MO
Rural and isolated Practice Endorsed Registered Nurses may proceed
Nurse Practitioner may proceed
Route of
250 mg
500mgs bd
10 days
200mg per 5 mls
Child: 15mg/kg up to
10 days
500mg bd
Provide Consumer Medicine Information if available:
Management of Associated Emergency: as for severe allergic reactions see Anaphylaxis
Follow up:
 Follow up of patients with ARF / RHD essential
 Assign an individual management plan
 Place person on recall register and monitor closely
Recommended duration of secondary prophylaxis. The most effective
regime for continuous prophylaxis is a 4 weekly injection of Benzathine
Penicillin – see current edition of Chronic Disease Guidelines.
Consult MO for antibiotic prophylaxis for procedures expected to produce
 Provide education and support to patient and family, resources available include
as Strong Heart, Strong Body, (from Tropical Population Health Service) books,
DVD, reminder cards
 Contact the ARF / RHD register person in your district or Public Health Unit for
 Antenatal patients with RHD may deteriorate because of the increased cardiac
workload required during pregnancy. Patients need to be assessed early in
pregnancy and monitored closely with 2 weekly follow up. The woman will also
need antibiotic cover for labour
 Primary prevention
 have a low threshold for treating throat infections with Penicillin in Aboriginal
and Torres Strait Islander children, see URTI (child) and URTI (adult)
 reduce the prevalence of scabies and impetigo
 Give influenza and pneumococcal vaccine
File 64 Immune complications
Referral / Consultation:
 Consult MO on all occasions of suspected ARF
 Consult MO for anticoagulation therapy / INR range
National High Blood Pressure Education Program Working Group on High Blood
Pressure in Children and Adolescents, The Fourth Report on the diagnosis,
Evaluation and Treatment of High Blood Pressure in Children and Adolescents.
Pediatrics, 2004. 114(2): p. 555.
Therapeutic Guidelines, Pharyngitis and/or tonsillitis. 2006, Therapeutic Guidelines
Ltd: Melbourne.
National Heart Foundation Australia, Rheumatic Fever/Rheumatic Heart Disease
Guideline Development Working Group, and Cardiac Society of Australia and New
Zealand, Diagnosis and management of acute rheumatic fever and rheumatic heart
disease in Australia: An evidence based review. 2006, NHFA.