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Home , Rheumatic fever

Rheumatic Licks the , bites the

Clare O’Donnell – Paediatric Cardiologist with thanks to Marion Hamer Nurse Practitioner - Paediatric Paediatric and congenital cardiac services Starship Hospital Action of Chambers and Valves

Diastole (relaxation) Systole (contraction) Rheumatic fever - Epidemiology • Acute rheumatic fever (ARF) remains the most common cause of acquired heart disease in children around the world

• The highest documented rates in the world have been in in Maori and Pacific people in NZ, Aboriginal Australians and those in the Pacific nations.

• ARF incidence is a sensitive marker of childhood disadvantage

• ARF tends to run in families and is more common in females

• Incidence peaks in the 5 – 14 year age group Acute Rheumatic Fever

• Acute rheumatic fever starts after an illness with the Group A streptococcal bacteria (GAS )

• Usually from a - pyogenes

• In some children this causes an autoimmune response (body attacks own tissue)

• Affects mainly the heart, joints, and

• Damage to the heart: mitral and /or

• Reoccurrences common if no preventative measures and may cause further cardiac valve damage. RF rate across NZ Government response

• Rheumatic fever target as part of the Better Public Services initiative

• Stop it: Prevent transmission of Strep A sore throats

• Treat it: Treat Strep A throats quickly and effectively Sore throats matter

• Sore throat in children are common – infection by virus is the most common cause – Infection by Group A streptococcus bacteria (strep throat) is a less frequent cause

• Strep throat requires treatment with

• Strep throat can lead to Rheumatic fever and heart damage

• Sore throats matter Treatment

http://www.heartfoundation.org.nz Primary prevention

• Alleviating child poverty • Education – Sore throats matter – get seen and get treatment – Hand hygiene – Safe sneezing / coughing into the elbow Faith - 6 years

old History Previously well and active Symptoms • No regular medicines, • – left side • immunised • occasional sore throat, • short of breath while lying • About 4-6 weeks ago had sore throat, flat and on exertion mild cold / sniffles and a bit sore and • Cough achy and got better . • Unwell about 2 weeks ago – left sided • Vomiting chest pain. Also had a sore ankle and couldn’t walk • • One week ago - breathless, chest pain, • Achy and tired fever, nausea, vomiting lethargy. Had a sore ankle. • Murmur • Sore left knee, swollen / hot On arrival in hospital: • cough, left upper quadrant pain and chest pain, breathless, can’t lie flat , fevers, feel miserable and achy • no sore throat, no diarrhoea, no • Mum says sore knee came on that morning

Signs and symptoms: Arthritis MAJOR manifestation

• Very painful swollen joints (usually ankles, knees, elbows, and wrists)

• Often first symptom

• Migratory – disappears from one and starts in another (poly- arthritis)

• May be present in one joint (mono-arthritis) : MAJOR manifestation • ARF almost always affects the valves especially the aortic and mitral

• Early disease - valvular regurgitation

• Recurrent RHD – valve regurgitation and stenosis

• May present as heart “murmur”

• Chest pain and/or difficulty breathing Mitral regurgitation Aortic regurgitation Left Coronary Artery

Right Coronary Artery Thickened rolled edges

Fusion

S & S: Sydenham's MAJOR manifestation

• Jerky , twitchy uncoordinated, involuntary and random movements, muscle weakness (most obvious in the face, hands and feet)

• Emotional instability. – irritability & anger, depression & sadness

• Clinical features – May begin 3-4 months after strep throat infection and often without other symptoms – Usually resolves in 6 months – More common in teenagers ( rare after 20 years) – Affects females more than males – May recur during pregnancy

• Chorea has a strong association with carditis S & S: Subcutaneous Nodules MAJOR manifestation

• Rare but highly specific

• Nodules more common when carditis is also present

• Painless lumps (groups of up to 12 over the elbows, wrists, knees and ankles )

• Skin not red or inflamed

• 1-2 weeks S & S: MAJOR manifestation

• Rare (difficult to see if dark skin) • Painless flat pink patches that spread outward in circular pattern (usually on back or front of body, almost never on face) • Not itchy or painful • May recur for months Signs and symptoms: MINOR manifestation • : – Joint pain ( non inflammatory), Usually large joints, mild or severe

• Fever: – Occurs in majority of cases, usually with onset of symptoms – Quick response to NSAID / salicylates

• Prolonged PR Interval: – Extreme 1st degree can sometimes lead to 2nd degree HB

• Elevated acute phase reactants: – CRP ≥ 30 & ESR ≥ 50

• Evidence of Group A streptococcal infection: – Group A betahaemolytic strep might be seen on throat swab since the infection may have finished by time of onset of the ARF symptoms – ASOT – serum reaches peak at around 3-6 weeks after infection and starts to fall around 6-8 weeks – Anti BNase B reaches peak level at 6-8 weeks after infection and starts to fall around 3 months Jones Criteria - summary

Major Criteria J Joints () ♥ Carditis (pancarditis and mid diastolic murmur) N Nodules E Erythema marginatum S Sydenham's chorea

Minor criteria Fever, ESR, Arthralgia, Long PR interval Difficulties with ARF diagnosis

• Child and family don’t always come to hospital or health clinic with their symptoms because; – Symptoms don’t seem serious enough – Access to health clinic is difficult

• Health staff don’t recognise the signs and symptoms of ARF – A sore throat can seem just like any other cold and sore throat – Lots of children get colds and sore throats

• ARF can look like other illnesses – Sore joints may get confused with a sports injury or “growing pains” – etc Treatment of Acute RF

Treat the acute illness – All cases of ARF should receive; – A single injection of Benzathine G, or – Oral Penicillin for 10 days ( if penicillin )

Relieve symptoms – Pain relief for arthritis (paracetamol or ) – Bed rest if cardiac failure – Anti-failure medication (, ACEi, +/- ) – Treat chorea (Carbamazepine or Valporic acid if severe) Secondary prophylaxis Key element in management of RHD

• Prevents further Group A streptococcal infection (stop strep throat !!!!) • Prevent repeated episodes of ARF ( where heart, joints, brain and skin become inflamed and swollen → repeated damage to valves in heart ) • Prevent development of RHD • Reduces the severity of RHD • Helps to reduce the risk of death form severe RHD Secondary prophylaxis

The standard dose • 1,200,000 units of Benzathine Penicillin G IM for ALL people ≥30kg • 600,000 units IM for children <30kg

• Monthly depot IM BPG (every 28 days) • 3 weekly (21-days) BPG for those with recurrent ARF despite full adherence to 28-day BPG.

Summary

• Acute rheumatic fever starts after an illness with the Group A streptococcal bacteria (GAS infection)

• Usually from a sore throat -

• Rheumatic fever is serious it can cause permanent damage to the heart

• Sore throats matter – Primary prevention – education families and health workers to take sore throats seriously – Treat with antibiotics

• Jones Criteria is used to guide diagnosis of ARF and RHD

• Need good long-term secondary prophylaxis to prevent further development of RHD Education

• Early evaluation and treatment of in children to help prevent RF – Sore throats matter !!! – Hand hygiene / sneeze into elbow

• Emphasize measures that minimize further damage to the valves of the heart. – Secondary prophylaxis of patients with previous RF and valve involvement with penicillin injections every 3-4 weeks decrease the recurrence of RHD.

• Additional prophylactic antibiotics prior to dental and surgical procedures decrease the likelihood of bacterial .

Rheumatic fever resources

• New Zealand www.heartfoundation.org.nz

• For NZ Guidelines for Rheumatic fever http://www.heartfoundation.org.nz/uploads/Rheumatic%20fever%20guide line%201(5).pdf

• World Heart federation – Resources for Health Professionals www.worldheart.org/rhd

• For training resources and data base development resources http://www.world-heart-federation.org/what-we-do/rheumatic-heart- disease-network/for-health-professionals/world-heart-federation- rheumatic-heart-disease-resources/

• RHD Australia www.rhdaustralia.org.au

• http://www.youtube.com/watch?v=tWMGNG61SRA Faith - 6 years old

History of present illness Symptoms • Chest pain – left side • Previously well and active • short of breath while lying flat and on exertion • No regular medicines, • Cough • immunised • Vomiting • occasional has a sore throat, • Fevers • About 4-6 weeks ago had very sore throat, mild • Achy and tired cold / sniffles and was a bit sore and achy and • Sore left knee, swollen / hot got better. • Sore tummy – liver enlargement • Unwell about 2 weeks ago – left sided chest pain. Had a sore ankle - couldn’t walk • Murmurs heard • One week ago - breathless, chest pain, fever, PSM radiating to axilla (MR) & diastolic murmur heard nausea, vomiting, lethargy…felt miserable at base of heart (AR) • Enlarged heart - Apex displaced to 6th intercostal space anterior axillary line. • On arrival in hospital: • cough, left upper quadrant pain and chest pain, breathless, can’t lie flat , fevers, feel miserable Investigations and achy • CXR – • no sore throat, no diarrhoea, no rash • ECG: prolonged PR interval on 12 lead ECG • Mum says sore knee came on that morning • Labs: Raised ESR and CRP • Strep titres elevated *ASOT and antiDNase high • ECHO: severe mitral regurgitation, mild aortic regurgitation