Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

1. Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

2. Acute Rheumatic FeverDiagnosis and Management

3. This presentation is intended to support the Curriculum for training health workers and others involved in the diagnosis and management of acute rheumatic fever and rheumatic heart disease. It has been made possible thanks to the support of the Vodafone Group Foundation and the International Solidarity, State of Geneva, and the ongoing support of Menzies School of Health Research, Caritas Australia, Fiji Water Foundation, Cure Kids and Accor Hospitality.

4. Revised Jones Criteria ARF can be confirmed if certain signs and symptoms are present. The Revised Jones Criteria (below) can help guide the diagnosis. MAJOR Criteria - signs and symptoms more often associated with ARF MINORCriteria- signs and symptoms that help support the diagnosis Evidence of recent GAS Infection is required

5. Revised Jones Criteria The World Health Organisation set the international standard for diagnosis of ARF. First episode or recurrent episode of ARF (no RHD): • 2MAJORmanifestations or1MAJORand2MINORmanifestations and • Evidence of preceding Group A streptococcal infection … (within 3 weeks before ARF symptoms) ARF (with existing RHD): • 2MINORmanifestations and • Evidence of preceding Group A streptococcal infection … (within 3 weeks before ARF symptoms) ** Individual country guidelines also exist **

6. Signs and Symptoms MAJOR Manifestations Arthritis • Painful, swollen joints (usually knees, ankles, wrists, elbows) • Very common with ARF, often the first symptom • Usually ‘migratory’- disappears from one joint as it starts in another (poly-arthritis), however may just be present in one joint (mono-arthritis). • Carditis • May present as a heart ‘murmur’ • Chest pain and/or difficulty breathing may be present in more severe cases

7. Signs and Symptoms Sydenham’s chorea • Twitchy, jerking movements and muscle weakness (most obvious in the face, hands and feet) • May occur on both sides or only one side of body • More common in teenagers and females (rare after age 20) • May be associated with irritability and or depression • May begin up to 3-4 months after the streptococcal throat infection, and often occurs without other symptoms • Usually resolves within 6 weeks (may last 6 months or more) • May recur in females during pregnancy

8. Signs and Symptoms Subcutaneous nodules • Painless lumps on the outside surfaces of elbows, wrists, knees, ankles in groups of 3-4 (up to 12) • The skin is not red or inflamed • Last 1-2 weeks (rarely more than 1 month) • Nodules are more common when Carditis is also present. Erythema marginatum • Painless, flat pink patches on the skin that spread outward in a circular pattern • Usually occurs early, may last months, rarely lasts years • Usually on the back or front of body, almost never on the face • Hard to see in dark-skinned people.

9. Signs and Symptoms MINOR Manifestations Fever • Occurs in the majority of cases, usually with the onset of symptoms • Usually ranging from 38.4 – 40.0º C (101-104º F) Arthralgia • Usually involves large joints • May be mild or severe Group A streptococcal infection • Group A beta-haemolytic streptococci may not be seen on a throat swab since the infection may be resolved at the time of onset of ARF symptoms. • ASOT – serum reaches a peak level around 3-6 weeks after infection and starts to fall at 6-8 weeks • Anti BNase B – reaches a peak level up to 6-8 weeks after infection and starts to fall at around 3 months after the infection. ** Normal antibody titre ranges vary with age and geography **

10. Difficulties with ARF Diagnosis A combination of signs and symptoms is required to confirm ARF People with ARF do not always present to the health system with symptoms because • Symptoms may not be considered serious • Transport to the health facility may be difficult Health staff may not recognise the signs and symptoms of ARF ARF may be confused with other illnesses, for example • Sore joints may be confused with a sports injury or ‘growing pains’

11. Treatment for ARF Treat the acute illness • Benzathine penicillin G injection or • Oral Penicillin for 10 days Relieve symptoms • Bed rest • Relief of arthritis, pain and fever (Paracetamol or Aspirin) • Treat chorea (use Carbamazepine or Valproic acid if severe) • Anti-heart failure medication (e.g. Diuretics, ACEi, Digoxinif required)

12. ARF Management Plan First dose of Benzathine penicillin G (start secondary prophylaxis) Baseline echocardiogram (if available) ARF alert on medical notes & computer systems (if applicable) Education for person and family Refer to local doctor / health facility Dental examination Long-term secondary prophylaxis plan

13. Management of Probable ARF • Treat the symptoms } • Dose of Benzathine penicillin G } as for ARF • Echocardiogram(if available) } Medical officer review after one month, and Repeat echocardiogram(if available) • If NOT ARF…cease Benzathine but monitor for ARF symptoms • If ARF… continue Benzathine and manage as for ARF

14. Summary The Jones Criteria is used to guide the diagnosis of ARF with a combination of MAJOR Manifestations, MINOR Manifestations and evidence of recent GAS Infection A long-term Management Plan should be established to prevent recurrence of ARF and development or worsening of RHD Probable ARF cases should also be monitored