Acute Rheumatic Fever: Diagnostic and

ManagementDidik HariyantoIndry Putri Festari

SymCARD 4 th 2014

Pediatric Cardiology SubdivisionDivision of Cardiology and Vascular Medicine

Faculty Medicine Universitas AndalasGeneral Hospital dr. M. Djamil Padang

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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. 2013Lioyd T et all, Pediatrics 2003: 112:1065-68

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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

Diff Dx?• Septic arthritis• Rheumatic fever• Juvenille Rheumatoid

Arthritis• Congenital Heart DIsease• etc

ARTRITIS and DISPNEU

IS IT ACUTE RHEUMATIC FEVER?

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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

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1 WHO. Rheumatic Fever and Rheumatic Disease. 20012Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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Carditis• 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

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1 Park MK. Pediatric Cardiology for Practitioners. 20082Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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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

WHO Criteria for diagnosis of rheumatic fever (based on revised Jones criteria)

1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

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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. 20012Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart disease (based on the revised Jones

criteria)Diagnostic categories CriteriaPrimary episode of RF Two major *or one major and two minor**

manifestations plus evidence of apreceding group A streptococcalinfection***.

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

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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. 20082Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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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. 20082Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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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

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• 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

Minor ManifestationSupporting evidence

1 Park MK. Pediatric Cardiology for Practitioners. 20082Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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TherapyArthritis 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

1-2 week 3-4 week 4-6 week 2-3 month

General guideline for bed rest and indoor ambulation

Arthritis Alone

Mild Carditis Moderate Carditis

Severe Carditis

Prednisone 0 0 0 2-6 weekAspirin 1-2 week 3-4 week 6-8 week 2-4 month

Recommended anti-inflammatory therapy

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

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Therapy…

Primary prevention of rheumatic fever: recommended treatment for streptococcal pharyngitis

1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

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Therapy….

Antibiotics used in secondary prophylaxis of RF

1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

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ARF and Heart Failure•Management:• Diuretic• ACE-inhibitor• Aldosterone antagonist• Inotropic

When and How to Use it?

1 WHO. Rheumatic Fever and Rheumatic Disease. 20012Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

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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

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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

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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.

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terimakasih4

SymCARD 2014th