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Acute Rheumatic Fever

Queensland Health Guidelines for Public Health Units

 Revision History

 Version  Date  Changes
 1.0 February 2010  Full revision of guideline. 

Infectious Agent

Acute rheumatic fever (ARF) is an immune-mediated multi-system inflammatory disease that follows an infection by a bacterium, the group A streptococcus (GAS).

Notification Criteria

[Adapted from: Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia: an evidence-based review.  Applies principally to high-risk groups – for further information on criteria for other groups refer to the source document].

Clinical Evidence

The presence of two major manifestations OR one major and two minor manifestations.

Major manifestations:

  • Carditis (including subclinical carditis detected by echocardiography) 
  • Polyarthritis (classically, but not necessarily, migratory in nature) or monoarthritis
  • Chorea (can be used as sole criterion for ARF, ie. in the absence of other manifestations and without evidence of a recent GAS infection, provided other causes excluded)
  • Erythema marginatum
  • Subcutaneous nodules

Minor manifestations:

Clinical findings

  • Arthralgia (must not be considered a minor manifestation when arthritis is present)
  • (usually ≥38° at some time during acute illness)

 

Laboratory/Electrocardiographic findings

  • Elevated acute phase reactants (CRP ≥30 mg/L and / or ESR ≥30 mm/hr)
  • Prolonged PR interval

Laboratory Suggestive Evidence:

Supporting evidence of preceding Group A streptococcal infection

  • GAS isolated from a throat swab
  • Elevated or rising streptococcal antibody (ASOT, anti-DNase B) titres. The following titres are the upper normal limits in the given age groups of children:

 

 Age group (years)  Elevated ASOT titre (IU/mL)  Elevated anti-DNase B titre (IU/mL)

 4-5

 120

 100

 6-9

 480

 400

 10-14

 320

 380

Notification Procedure

ARF is a clinical diagnosis. As there is no diagnostic test for ARF, notifications must come from clinicians. All new episodes of ARF, even if recurrences, must be notified.

Attending Medical Practitioners/Medical Superintendents (or Delegates)

To notify upon diagnosis.

Reporting to NOCS

Report only confirmed cases.

Confirmed case: A confirmed case requires clinical evidence plus laboratory suggestive evidence.

Objectives of surveillance

  1. To monitor the epidemiology of ARF in Queensland.
  2. To coordinate management of ARF/RHD cases in conjunction with the Queensland RHD register and control program.

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Public Health Significance and Occurrence

ARF is a non-suppurative complication following infection with GAS and is characterised by acute inflammation of a variety of tissues. Long term effects of concern are fibrosis and scarring of cardiac valves, which can lead to rheumatic heart disease (RHD). Without effective management, at least 60% of ARF patients develop RHD, which can be severe.

ARF has virtually disappeared from most industrialised countries. However, ARF remains a substantial public health problem in the developing world and is a persisting problem in Aboriginal and Torres Strait Island populations in central and northern Australia. ARF is also a major public health problem in South Pacific island nations such as Fiji, Samoa and the Cook Islands, and in South Pacific island migrant populations in New Zealand. The extent of the ARF burden in South Pacific island migrant populations in Queensland is unknown, but clinicians serving these populations should be encouraged to have a high index of suspicion for ARF.

The recent (mid-2004 to mid-2009) annual incidence of ARF in school-aged Indigenous children in north Queensland, 155 episodes/100,000 persons 5-14 years of age, is of particular concern. This incidence indicates that these children are at extremely high risk of developing ARF, as ‘high-risk’ has been defined by an incidence of >30 episodes/100,000 persons 5-14 years of age. A consequence of this extreme risk is the high prevalence of RHD in the region.

Clinical Features

See clinical criteria and public health significance above.

Reservoir

Humans

Mode of Transmission

Group A streptococci are spread by large respiratory droplets or direct contact with infected individuals or carriers, and rarely via indirect contact through objects. Individuals with acute upper respiratory tract (especially nasal) infections are particularly likely to transmit infection.

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Incubation Period

Group A streptococcal infection: Usually 1 to 3 days, rarely longer.

Acute rheumatic fever: 1 to 5 weeks after the initial GAS infection.

Period of Communicability

GAS infection - 10 to 21 days in untreated, uncomplicated cases.
Individuals with untreated streptococcal pharyngitis may carry the organism for weeks or months. Contagiousness decreases sharply 2 to 3 weeks after onset of infection. Adequate penicillin therapy will generally terminate transmissibility within 24 hours.

ARF is not communicable.

Susceptibility and Resistance

Susceptibility to GAS infections is general.  Type specific immunity may develop through inapparent infection.

Most ARF cases in Australia occur in Indigenous children 5-14 years of age. There is a significant risk of recurrence of ARF with further cardiac damage following subsequent GAS infections in an individual who has already had at least one episode of ARF.

 

Management

Cases

Hospitalisation is normally advised for clinical assessment, diagnostic interventions and formulation of a long-term management plan. Any concerns or uncertainties should be discussed with a paediatrician or physician familiar with the disease.

Investigation
FBC, ESR, CRP, ASOT and anti-DNAase B
Throat swab 
ECG
Echocardiography should be included in the assessment of every case.

Treatment
Asprin or NSAID therapy (if there is not marked improvement of arthritis within 3 days of commencing this therapy, another diagnosis should be considered).
Penicillin to eliminate streptococci (even if GAS are not isolated on culture).  If past history of severe allergy to penicillin, treat with erythromycin.

Bed rest (for those with severe acute valvular disease or heart failure).

(NB: Steroids are only indicated if there is severe acute carditis with cardiac failure or pericarditis).

Chemoprophylaxis
Those with a history of ARF are at increased risk of further episodes and the development of RHD. Therefore antibiotic chemoprophylaxis (to prevent further GAS infections) should be given regardless of whether rheumatic carditis was diagnosed in the acute episode.
Benzathine penicillin 900mg (1,200,000 U) IMI monthly is the recommended chemoprophylaxis for children ≥ 20 kg body weight and adults. (450 mg (600,000 U) for those weighing less than 20 kg).

Oral penicillin is not recommended, as compliance can be a problem resulting in an increased risk of recurrence. However, if there is absolute refusal to have benzathine penicillin IMI, then penicillin V 250 mg orally twice daily (for all ages) can be considered.

Oral erythromycin is recommended in those allergic to penicillin (250mg twice daily for all ages).

The duration of chemoprophylaxis depends on the severity of cardiac involvement. For example, in those with no carditis or evident valve disease, it should continue for 10 years or until 21 years of age (whichever is longer).


Follow-up
See Chemoprophylaxis above. Regular clinical follow-up is required for all patients with ARF and/or RHD.

Influenza and pneumococcal vaccination is recommended for those with rheumatic heart disease, regardless of age.

Extra antibiotic coverage will be required prior to any dental or surgical procedure, insertion of a catheter or intra-uterine contraceptive device etc.

Pregnant women with rheumatic heart disease need to be assessed early in pregnancy and monitored closely. Antibiotic cover will be required during labour.

 

A coordinated approach is essential for the effective long-term management of ARF/RHD patients. For this reason, a RHD register and control program was recently established in Queensland. This program has five coordinators situated across Queensland whose role is to provide the necessary coordination and training of clinical staff.

The program should be informed of all confirmed cases of ARF.
A scanned copy of the completed ARF case report form should be emailed by the PHU to:
Arf&RhdRegister@health.qld.gov.au

Counselling
The program coordinators will up-skill local clinical staff about ARF/RHD so that they can provide appropriate information and support to cases (and parents/guardians) and monitor compliance with chemoprophylaxis.

Contacts

Contact Tracing
Not necessary

Other Control Measures

Low threshold for antibiotic treatment of throat infections in Indigenous children.

Preventive Measures

  • Educate the public and health workers about the modes of transmission, and about the need for prompt presentation when someone (particularly a school-aged child) develops a sore throat.
  • Many medical practitioners in Australia have never seen a case of ARF, because the disease has disappeared from the populations among which they train and work. Every opportunity should be taken to inform practitioners new to rural and remote Queensland about the essential features of the disease, how to make the diagnosis, how each case should be subsequently managed, and how ARF is to be notified.

Summary

Summary reports of ARF notifications in Queensland are included in the notifiable disease reports prepared from time to time by the Communicable Diseases Branch, Queensland Health.

References

RHDAustralia, an initiative of the Menzies School of Health Research. The Australian guidelines for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. 2nd Edition. 
(accessed November 2012). (The Heart Foundation and the Cardiac Society of Australia and New Zealand originally produced the 2006 guidelines)

Steer AC, Carapetis JR. Acute rheumatic fever and rheumatic heart disease in Indigenous populations. Pediatr Clin N Am 2009; 56: 1401-1419.

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Last updated: 21 November 2012