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File 64 Immune complications Acute Rheumatic Fever and Rheumatic Heart Disease Recommend Treat streptococcal throat and skin infections with a single injection of Benzathine Penicillin Any case of polyarthritis and fever in children should be considered as possibly acute rheumatic fever (ARF) and transferred to hospital for investigation and diagnosis 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 Background Acute Rheumatic Fever (ARF) is an auto-immune response to bacterial infection with group A streptococcus (GAS)  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 valves 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  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 1. 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 2. Immediate management – not applicable 3. Clinical assessment: Obtain complete patient history including File 64 Immune complications past episodes of ARF / RHD – is patient up to date with monthly penicillin injections? 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  Accurate diagnosis of ARF requires a combination of clinical and laboratory criteria and laboratory evidence of a recent group A streptococcal (GAS) infection 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  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 echocardiogram) 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 control 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  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. 4. Management: 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 made 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) Schedule 4 Penicillin Benzathine (Bicillin LA) DTP IHW / IPAP / RIN / NP 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 Form Strength Recommended Dosage Duration Administration Disposable 900 mg in IM 3 kg to under 6 kg 225 mg (0.5mL) Stat syringe 2mL 6 kg to under 10 kg 337.5 mg (0.75mL) (1.2million 10 kg to under 15.kg 450 mg (1mL) units = 15 kg to under 20 kg 675 mg (1.5mL) 900mg) 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 . If Penicillin allergy not reliably documented arrange for testing in hospital DTP RIN / NP / IHW / IPAP 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 Recommended Form Strength Duration Administration Dosage Capsule 250 mg Oral 500mgs bd 10 days Suspension 200mg per 5 mls Oral 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 Schedule 5. 4 Erythromycin 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 bacteraemia. 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 help 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 6. Referral / Consultation: Consult MO on all occasions of suspected ARF Consult MO for anticoagulation therapy / INR range References 1. 2. 3. 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.