Rheumatic Fever

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This is an inflammatory disease that occurs in children and young adults (5 - 15 years) as a result of infection with group A streptococcus. It affects the heart, skin, joints and central nervous system. Pharyngeal infection with group A streptococcus may be followed by the clinical syndrome of rheumatic fever. This is thought to develop because of an autoimmune reaction triggered by the infective streptococcus and not due to direct infection of the heart or the production of a toxin.

Clinical features Revised Jones criteria for the diagnosis of rheumatic fever

Major

  • Carditis
  • Polyarthritis
  • Sydenham’s chorea
  • Erythema marginatum
  • Subcutaneous nodules


Minor

  • Arthralgia
  • History of rheumatic fever
  • Fever
  • Increased P-R interval on ECG
  • Raised ESR
  • Increased C-reactive protein

Evidence of streptococcal infection
Raised ASO titre (or increased titre of other specific antistreptococcal antibodies) Positive throat culture

Diagnosis Investigations

  • Throat swab
  • Serology
  • ESR
  • ECG
  • Echocardiography

Diagnosis is made based on two or more major criteria or one major plus two or more minor criteria plus evidence of antecedent streptococcal infection.

Treatment Drugs

  • Benzathine penicillin 0.6–1.2 mega units IM stat
    OR
  • Phenoxymethylpenicillin 500mg orally 4 times daily for 7 days. For recurrences, 250mg daily until the age of twenty or for 5 years after the latest attack
    OR
  • Erythromycin, 250 – 500mg orally 4 times daily for 7 days. For recurrences, 125 – 250mg once daily until the age of twenty or for 5 years after the latest attack
  • Prednisolone 1 – 2mg/kg per day divided into 4 equal doses for 10 days (in severe carditis)

Chronic rheumatic heart disease

More than 50% of those who suffer acute rheumatic fever with carditis will later develop chronic rheumatic valvular disease predominantly affecting the mitral and aortic valves.

Complications

  • Congestive cardiac failure
  • Pulmonary oedema

Management

Investigations

  • Chest X-Ray
  • ECG
  • Echocardiography

Treatment

  • Treat underlying complications
  • Give prophylaxis against recurrent rheumatic fever with Benzathine Penicillin 1.2 – 2.4 MU monthly for life
  • Give prophylaxis against infective endocarditis

Refer

  • For further evaluation, if the patient has significant heart murmurs
  • All patients with increasing cardiac symptoms.

Acute Rheumatic Fever (ARF) In Children

Description

An acute autoimmune process following pharyngitis caused by streptococcal infection. Diagnostic criteria
for rheumatic fever are based on the modified Jones criteria. Has defined high risk population recognizing variability in clinical presentation and had included Echocardiography as a tool to diagnose for subclinical carditis.

Table 45 Modified 2015 Jones’ criteria

Major

Minor

Carditis (clinical or subclinical)

Monoarthralgia

Arthritis – monoarthritis or polyarthritis

Fever (≥ 38.0ºC)

Polyarthralgia

ESR ≥ 30 mm/hour and/or CRP ≥ 3.0 mg/dl

Chorea

Prolonged PR interval (after considering the differences related to age: if

there is no carditis as a major criterion)

Erythema marginatum

 

Subcutaneous nodules

 

ESR – erythrocyte sedimentation rate; CRP – C-reactive protein (Adapted from ref 13). All patients with ARF should have an Echocardiography done even in the absence of clinical suspicion of valvular done.


Two major manifestations plus evidence of preceding streptococcal infection OR one major and two minor
manifestations plus evidence of preceding streptococcal infection are required to make a diagnosis

Investigations

• FBC/DC
• ESR/CRP
• Throat swab culture for Streptoccal
• Anti Streptolysin O titres (ASOT)
• ECG
• ECHO
• CXR

Treatment

• Benzathine penicillin IM injection 600 000 IU - 1.2 MU stat OR
• Oral phenoxymethyl penicillin 10-12.5mg/kg/dose twice daily for ten days maximum 500mg every 6
hours.
• Patients with hypersensitivity to penicillin can be treated with oral first generation cephalosporins
for 10 days.
• Treat chorea (if severe)
• Anti- heart failure medication (see section of CCF)
• Relieve symptoms, Bed rest, Relief of arthritis, pain and fever with Anti-inflammatory Agent

Table 46 Management of Acute Rheumatic Fever (ARF)

 

Arthritis

Carditis

Prednisolone 2 mg/kg/day

Nil

2 – 4 weeks

Aspirin 50 to 60 mg/kg/day in four to six divided doses

1 – 2 weeks

Until symptoms subside

Ibuprofen 30 mg/kg/day in 3 divided doses, where Aspirin not tolerated

Until symptoms subside

Until symptoms subside

NB: The dose of prednisolone should be 2 mg/kg/day (max 60 mg); then taper by 20–25% per week. Aspirin can be reduced to 25 to 30 mg/kg/day when symptoms improve. The dose of prednisolone should be tapered, and aspirin started during the final week.


Management of Sydenham’s chorea
• Reduce physical and emotional stress and use protective measures as indicated.
• Benzathine penicillin IM stat (Eradicate GAS), then every 28 days for secondary prophylaxis.
Anti-inflammatory agents not indicated.
For severe chorea, any of the following drugs may be used:
• Carbamazepine 7–20 mg/kg/day (7–10 mg/kg day usually sufficient) given TDS PO until chorea is
controlled for at least 2 weeks, then trial off medication
• Valproic acid Usually 15–20 mg/kg/day (can increase to 30 mg/kg/day) given TDS PO until chorea is
controlled for at least 2 weeks, then trial off medication.
• Phenobarbitone, Haloperidol and Chlorpromazine can be used when above not available.


Further management plan
• ARF register (cardiac clinic), issue Acute Rheumatic Fever (ARF)prophylaxis card
• Education of patient and family
• Dental examination
• Long term secondary prophylaxis plan


Secondary prophylaxis to prevent recurrent ARF is a long term, regular administration of antibiotics to:
• Prevent group A β-Haemolytic Streptococcal (GAS) pharyngitis.
• Prevent repeated development of ARF.
• Prevent development of Rheumatic heart disease (RHD)
• Reduce severity of RHD
• Help reduce the risk of death from severe RHD


Antibiotic regimens for secondary prophylaxis

Table 47 Antibiotic regimens for secondary prophylaxis

Antibiotic

Dose

Route

Frequency

First line

Benzathine Penicillin G

1,200,000 U (900 mg) ≥ 30 Kg

600,000 U (450 mg) ≤30 Kg

Deep IM Injection

4-weekly

Second line (If IM route is not possible or refused, adherence should be carefully monitored)

Phenoxymethylpenicillin (Pen V)

250mg

Oral

Once daily

Following documented penicillin allergy

Erythromycin

250mg

Oral

Twice daily

NOTE: Duration of prophylaxis in all persons with ARF is for a minimum of 10 years after the most recent episode of ARF or until age 21 years (whichever is longer). For RHD, the duration of prophylaxis is for life.

Rheumatic Heart Disease

Description

Rheumatic heart disease (RHD) is including a spectrum of lesions from pericarditis, myocarditis, and valvulitis during ARF, to chronic valvular lesions that evolve over years following one or more episodes of ARF. The common valvular lesions in RHD include mitral regurgitation/stenosis, aortic regurgitation/stenosis and tricuspid regurgitation/stenosis

Signs and Symptoms

Heart failure (as a result of valvular insufficiency or stenosis)

Medical:
• Prevent ARF (Elimination GAS pharyngitis as above)
• Supportive treatment for CCF (as above)
• Prevent recurrent ARF in children with RHD (see secondary prophylaxis above)
• Monitoring for the complications and sequelae of chronic RHD.
Surgery:
• Indicated in patients with persistent CCF
OR
• Worsening after aggressive medical therapy for RHD to decrease valve insufficiency/regurgitation

Complications

• Atrial arrhythmias,
• Pulmonary oedema,
• Recurrent pulmonary emboli,
• Infective endocarditis
• Intracardiac thrombus formation,
• Systemic embolism