1. Acute Rheumatic Fever: Diagnostic and Management
SymCARD 4 th
2014
Acute Rheumatic Fever: Diagnostic and Management
Didik Hariyanto
Indry Putri Festari
Pediatric Cardiology Subdivision
Division of Cardiology and Vascular Medicine
Faculty Medicine Universitas Andalas
General Hospital dr. M. Djamil Padang

2. Introduction
Rheumatic fever (RF) is nonsuppurative complications of Group A streptococcal pharyngitis due to a delayed immune response
Continues to be problem worldwide
Underdiagnosed and undertreated
Estimated 30 million people suffer from ongoing heart disease from ARF, 70% dying at average age 35 years old
RHD developed in 44% of patients who initially had no clinical evidence of carditis
Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013
Lioyd T et all, Pediatrics 2003: 112:

3. IS IT ACUTE RHEUMATIC FEVER?
Case:
A 11 year-old girl, brought to hospital because she has pain in her right knee that is preventing her from walking
There’s breathlessness during activity
History of sorethroat 2 weeks before
ARTRITIS and DISPNEU
Diff Dx?
Septic arthritis
Rheumatic fever
Juvenille Rheumatoid Arthritis
Congenital Heart DIsease
etc
IS IT ACUTE RHEUMATIC FEVER?

4. Arthritis in Acute Rheumatic Fever
Most common feature: present in 80% of patients
Painful, migratory, short duration, excellent response of salicylates
Usually affected and large joints preferred knees, ankles, wrists, elbows, shoulders
Small joints and cervical spine less commonly involved
Differenciate with athralgia 4
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1 WHO. Rheumatic Fever and Rheumatic Disease. 2001
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

5. Carditis 4 SymCARD 2014 Most serious manifestation
May lead to death in acute phase or at later stage
Any cardiac tissue may be affected
Valvular lesion most common: mitral and aortic
Clinical manifestations:
Breathlessness
Tachycardia
Murmur (MR and AR)
Cardiomegaly
Heart failure th 4
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1 Park MK. Pediatric Cardiology for Practitioners. 2008
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

6. Major Manifestation Minor Manifestation
WHO Criteria for diagnosis of rheumatic fever (based on revised Jones criteria)
Major Manifestation
Minor Manifestation
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Clinical : fever, poliathralgia
Laboratory: elevated acute phase reactans (erythrocyte sedimentation rate or leucocyte count)
Supporting evidence of a preceding streptococcal infection within the last 45 days
Electrocardiogram: Prolonged P-R interval
Elevated or rising antistreptolysisn-O or other streptococcal antibody, or
A positive throat culture, or
Rapid antigen test for group A streptococci, or
Recent scarlet fever
1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

7. Criteria Diagnosis ARF
Two mayor manifestation, or
Combination 1 mayor and 2 minor manifestations and
Supporting evidence of a preceding streptococcal infection
1 WHO. Rheumatic Fever and Rheumatic Disease. 2001
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

8. 2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart disease (based on the revised Jones criteria)
Diagnostic categories
Criteria
Primary episode of RF
Two major *or one major and two minor**
manifestations plus evidence of a
preceding group A streptococcal
infection***.
Recurrent attack of RF in a patient without established rheumatic heart disease
Two major or one major and two minor manifestations plus evidence of a preceding group A streptococcal infection.
Recurrent attack of RF in a patient with established rheumatic heart disease.
Two minor manifestations plus evidence of a preceding group A streptococcal infection
Rheumatic chorea.
Insidious onset rheumatic carditis
Other major manifestations or evidence of group A streptococcal infection not required
Chronic valve lesions of RHD (patients presenting for the first time with pure mitral stenosis or mixed mitral valve disease and/or aortic valve disease).
Do not require any other criteria to be diagnosed as having rheumatic heart disease

9. Syndenham’s Chorea Extrapyramidal disorder
Fast, clonic, involuntary movements (especially face and limbs)
Muscular hypotonus
Emotional lability
First sign: difficulty walking, talking, writing
Usually a late manifestation: months after infection
Often the only manifestation of ARF
1 Park MK. Pediatric Cardiology for Practitioners. 2008
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

10. Subcutaneous Nodules Usually 0.5 - 2 cm long
Firm, non-tender, isolated or in clusters
Most common: along extensor surfaces of joint knees, elbows, wrists
Also: on bony prominences, tendons, dorsi of feet, occiput or cervical spine
1 Park MK. Pediatric Cardiology for Practitioners. 2008
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

11. Erythema Marginatum Present in 7% of patients Highly specific to ARF
Cutaneous lesion:
Reddish pink border
Pale center
Round or irregular shape
Often on trunk, abdomen, inner arms, or thighs
Highly suggestive of carditis

12. Arthralgia without objective findings is common in RF Less common
Minor Manifestation
Supporting evidence
Fever
Occurs in almost all rheumatic attacks at the onset, usually ranging from 38.4–40.0 °C
Diurnal variations are common, but there is no characteristic fever pattern.
Athralgia
Arthralgia without objective findings is common in RF
Less common
abdominal pain and epistaxis
ECG  Prolong PR interval
1 Park MK. Pediatric Cardiology for Practitioners. 2008
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

13. Therapy General guideline for bed rest and indoor ambulation
Arthritis alone
Mild Carditis
Moderate Carditis
Severe Carditis
Bed rest
1-2 week
3-4 week
4-6 week
As long as CHF is present
Indoor ambulation
2-3 month
Recommended anti-inflammatory therapy
Arthritis Alone
Mild Carditis
Moderate Carditis
Severe Carditis
Prednisone
2-6 week
Aspirin
1-2 week
3-4 week
6-8 week
2-4 month
Dosages: Prednisone, 2 mg/kg/day, in four divided doses; aspirin, 100 mg/kg/day, in four to six divided doses
1 Park MK. Pediatric Cardiology for Practitioners. 2008

14. Therapy…
Primary prevention of rheumatic fever: recommended treatment for streptococcal pharyngitis
1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

15. Therapy…. Antibiotics used in secondary prophylaxis of RF
1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

16. ARF and Heart Failure Management: When and How to Use it? Diuretic
ACE-inhibitor
Aldosterone antagonist
Inotropic
When and How to Use it?
1 WHO. Rheumatic Fever and Rheumatic Disease. 2001
2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

17. Monitoring and Evaluation ARF
ARF could become Rheumatic Heart Disease
Monitoring:
Echocardiography
Check inflammation marker if needed
1Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

18. Complication Rheumatic Heart Disease Heart Failure Other issues:
When the patient need to perform surgery?
Repair/replacement?
1Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

19. Take Home Message
Acute Rheumatic Fever leading to Rheumatic Heart Disease is a major problem world wide.
Appropriate treatment of group A strep pharyngitis necessary to prevent disease.
Preventing recurrences causing chronic heart disease simple, universally available, and costeffective.

20. terimakasih 4
SymCARD 2014 th