Acute Rheumatic Fever

Original Author: Dr Khuen Ng and Trainee Doctor Alisha Burman
Last Updated: 29th July 2018
Revisions: 11

Introduction

Acute rheumatic fever (ARF) is a systemic illness that occurs 2-4 weeks after pharyngitis in some people, due to cross-reactivity to group A β-haemolytic streptococcus (GAS), also called Streptococcus pyogenes.

Epidemiology

  • 4 million children affected worldwide
  • 94% of cases are in developing countries
  • Most common in tropical countries with no seasonal variation
  • More common in females

Pathophysiology

Streptococcus pyogenes is a gram-positive cocci and it produces two cytolytic toxins: streptolysin O and S.

Rheumatogenic strains of GAS contain M proteins in their cell wall and are immunogenic. B cells are stimulated to produce anti-M protein antibodies against the infection which also cross react with other tissues e.g. that of the heart (causing rheumatic heart disease), brain, joints and skin leading to a constellation of multiorgan signs and symptoms. This is also exacerbated by production of activated cross reactive T cells.

Risk Factors

  • Children and young people
  • Poverty
  • Overcrowded and poor hygiene places
  • Family history of rheumatic fever
  • D8/17 B cell antigen positivity

Clinical Features

The Revised Jones Diagnostic Criteria (below) describes the key clinical features that may be present. In addition to this, you may elicit from the history that they had a recent sore throat or scarlet fever. Also, in severe acute rheumatic failure, a heart murmur might be heard on examination and it is most commonly the mitral valve, which is affected.

Diagnostic Requirements

Positive throat culture for Group A β-haemolytic streptococcus or elevated anti-streptolysin O (ASO) or anti-deoxyribonuclease B (anti-DNASE B) titre.

AND

2 major criteria OR 1 major and 2 minor criteria present for initial ARF. (Same criteria for recurrent ARF plus can also be just 3 minor criteria)

Major Criteria (SPECS)

  • Sydenham’s chorea
  • Polyarthritis
  • Erythema marginatum (figure 1: more examples at dermnetnz)
  • Carditis
  • Subcutaneous nodules

Minor Criteria (CAPE)

  • CRP or ESR – Raised acute phase reactant
  • Arthralgia
  • Pyrexia/Fever
  • ECG – Prolonged PR interval

*Joint (arthritis or arthralgia) and cardiac (carditis or prolonged PR interval) manifestations can only be counted once, not twice, as either a major or a minor criterion.

*Slight variation of criteria for high risk population

Figure 1: Erythema Marginatum 

Differential Diagnosis

Thera are numerous differential diagnoses to ARF, but below are examples of a few differentials and their distinguishing features.

Septic Arthritis
  • ·Usually only one joint involved
  • ·Positive gram strain, culture and elevated WBC of aspirated synovial fluid
Reactive arthropathy
  • Commonly males and often associated with urethritis and conjunctivitis
Infective endocarditis
  • Positive blood culture
  • Echocardiogram shows vegetations on the valves
  • Signs: Janeway lesions, Osler nodes, splinter haemorrhages
Myocarditis
  • Troponin and creatinine kinase elevated
  • ECG: saddle ST segments or T wave changes

 

Investigations

  • Bloods: ESR, CRP, FBC (WBC),
  • Blood cultures to exclude sepsis
  • Rapid Antigen Detection Test
  • Throat culture: may be negative by the time rheumatic fever symptoms occur
  • Anti-streptococcal serology: ASO and anti-DNASE B titres
  • ECG: prolonged PR interval
  • CXR if carditis is suspected: congestive heart failure may be seen in ARF due to valvular damage
  • Echocardiography

Management

Initial management in confirmed rheumatic fever

  1. Antibiotics e.g. benzathine benzylpenicillin (1st choice due to its long acting property, serving the purpose of GAS eradication and secondary prophylaxis), phenoxymethylpenicillin , amoxicillin. In confirmed penicillin allergy, alternatives include cephalosporins (avoid in IgE mediated penicillin allergy and anaphylaxis), macrolides and clindamycin (4)
  2. Aspirin or NSAIDs e.g. naproxen or ibuprofen
  3. Assess for emergency valve replacement
  4. In severe carditis (e.g. congestive cardiac failure or 3rd degree heart block) glucocorticoids and diuretics may be required

Definitive and Long‐term management

Secondary prophylaxis with intramuscular Benzathine benzylpenicillin every 3-4 weeks, oral Phenoxymethylpenicillin twice daily, oral sulfadiazine daily, or oral azithromycin (in penicillin allergy) (4)

Complications and Prognosis

  • 2% of the population can get permanent damage to heart valves and chronic rheumatic heart disease
  • With treatment ARF should resolve within 2 weeks but cardiac inflammation may take months to resolve fully and thus, it is common for patients to relapse within this time

References

[1] [Online]. Available: http://bestpractice.bmj.com
[2] [Online]. Available: emedicine.medscape.com
[3] American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015 May 19;131(20):1806-18.
[4] Prevention of rheumatic fever and diagnosis and treatment of acuteStreptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. 2009 Mar 24;119(11):1541-51.

 

Quiz

Question 1 / 5
How long after pharyngitis does acute rheumatic fever typically present?

Quiz

Question 2 / 5
Which one of these is not a risk factor for ARF?

Quiz

Question 3 / 5
Which of the following is a minor criteria for ARF?

Quiz

Question 4 / 5
What is the initial management?

Quiz

Question 5 / 5
Which virulence factor causes B cells to produce a substance that cross reacts with tissues in the heart, brain, joints and skin?

Results

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