Revision - Kawasaki Disease & Rheumatic Fever Flashcards by Esme Cosham

What is the diagnosis of Kawasaki disease based on?

The American Heart Association diagnostic criteria

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What is the AHA diagnostic criteria for Kawasaki disease?

Presence of fever (usually ≥39°C) for at least 5 days, plus at least 4 out of 5 key features:

1) Mucositis: erythema and cracking of the lips, strawberry tongue and/or oral erythema

2) Conjunctivitis: bilateral conjunctivitis without exudate

3) Rash: maculopapular, erythroderma or erythema multiforme

4) Peripheral changes: erythema, oedema and/or desquamation of the hands and feet

5) Cervical lymphadenopathy

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Location of lymphadenopathy in Kawasaki disease?

Cervical

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What can happen to hands and feet in Kawasaki disease?

Red palms of the hands and the soles of the feet which later peel

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Which phase of Kawasaki disease is the highest risk period for developing cardiac complications?

Subacute phase (2-4w from fever onset)

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What investigation can be done to exclude Streptococcus infection?

Anti-streptolysin O titre (ASOT): to exclude group A streptococcal infection

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Mx of Kawasaki disease?

High dose aspirin + IV immunoglobulins

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Role of aspirin in Kawasaki disease?

Reduce risk of coronary artery aneurysms & thrombosis

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How long is aspirin given for in Kawasaki disease?

High dose aspirin given until fever has resolved for 48h, then low dose given for approx 6 weeks

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What is Reye’s syndrome?

A rare acute encephalopathy associated with liver failure.

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Role of IVIG in Kawasaki disease?

Infusion within the first 10 days of illness can reduce the incidence of coronary artery aneurysms .

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When should improvement be seen after intiating treatment in Kawasaki disease?

Within 36 hours.

Note - A second dose of IVIG may be given if an improvement is not seen.

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What are some complications of Kawasaki disease?

1) Coronary artery aneurysms

2) Myocarditis/pericarditis

3) Arrhythmias

4) Valvular disease (e.g. mitral regurgitation)

5) Coronary artery thrombosis/myocardial infarction

6) Sudden cardiac death

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What is the danger of a coronary artery aneurysm in Kawasaki disease?

Can rupture –> cardiac tamponade

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What bacteria is implicated in rheumatic fever?

GAS

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What is rheumatic fever?

A systemic inflammatory disorder that arises as a result of infection with GAS.

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How soon after Strep infection can rheumatic fever develop?

2-6 weeks

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How does initial Strep. pyogenes infection typically present?

1) Pharyngitis (sore throat)

2) Cellulitis

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How is strep infection more likely to present in hot countries?

As skin infection instead of pharyngitis

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What criteria is used for diagnosing rheumatic fever?

Jones criteria

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What does the Jones criteria state about the diagnosis of rheumatic fever?

There must be evidence of recent GAS infection plus either;

2 major criteria
1 major criteria and 2 minor criteria

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Give some evidence of a recent group A strep infection

1) Positive throat swab

2) Positive rapid streptococcal antigen test

3) Raised streptococcal antibody titre (ASO or DNAse)

4) Recent episode of scarlet fever

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What are the 5 major criteria for rheumatic fever?

1) Polyarthritis (joint involvement)

2) Carditis & valvulitis (heart involvement)

3) Sydenham’s chorea (nervous system involvement)

4) Erythema marginatum (skin involvement)

5) SC nodules (skin involvement)

typical symptoms of rheumatic fever?

fever
joint pain
rash
chorea
SOB
nodules

How can rheumatic fever affect the joints?

Affects large joints - hot, swollen, painful joints. ‘Migratory polyarthritis’ - different joints become inflamed and improve at different times.

What is the most common symptom of rheumatic fever?

Polyarthritis (80%)

What may aspiration of synovial fluid in polyarthritis caused by rheumatic fever show?

Sterile inflammation with no organisms present

What is the most significant manifestation of rheumatic fever?

Carditis

What clinical features can carditis caused by rheumatic fever cause?

1) pericarditis 2) myocarditis 3) endocarditis

How can myocarditis present?

Congestive heart failure: SOB, orthopnoea, PND, cardiogenic shock

What is the most commonly affected valve in rheumatic fever?

Mitral valve

How does rheumatic fever typically affect valves?

In an attack of rheumatic fever, valve incompetence (i.e. regurgitation) is more likely to develop than valve stenosis. Valve stenosis tends to develop as a feature of chronic disease many years later.

How soon after initial streptococcal infection does Sydenham’s chorea appear?

2-6m later

What is Sydenham’s chorea sometimes preceded by?

Emotional lability or behaviour which is out of character for the patient.

What are the 2 key skin findings in rheumatic fever?

1) SC nodules (only present with severe carditis) 2) Erythema marginatum rash --> look up pic!

Where are subcutaneous nodules typically found in rheumatic fever?

Extensor surfaces

What are the 4 minor criteria for rheumatic fever?

1) Raised ESR or CRP 2) Pyrexia (typically >39 degrees) 3) Polyarthralgia i.e. pain present in multiple joints (this is not included in the criteria if polyarthritis is already present) 4) Prolonged PR on ECG 5) History of rheumatic fever

Arthritis vs arthralgia?

Arthritis is an inflammatory condition that causes joint pain. Arthralgia is joint pain.

Mneumonic for rememebering major criteria for rheumatic fever: JONES

J - joint arthritis O - organ inflammation such as carditis N - nodules E - erythema marginatum rash S - sydenham's chorea

What 2 ECG findings may be present in rheumatic fever?

1) Prolonged PR 2) Tachycardia N.B. may also be signs of pericarditis or myocarditis

Investigations in rheumatic fever?

1) ECG 2) Vital signs (fever) 3) Throat swabs for GAS 4) Rapid streptococcal antigen test (RAST) 5) Anti-streptococcal antibodies 6) Blood tests: FBC, ESR, CRP, troponin, rheumatoid factor, anti-CCP

What are the 2 most common antibody tests used for rheumatic fever?

1) anti-streptolysin (ASO) 2) anti-DNAse B

When may troponin be raised in rheumatic fever?

Myocarditis

Role of CXR in rheumatic fever?

Rule out HF

Management of rheumatic fever can be divided into acute management and prophylaxis. What does acute management of rheumatic fever involve?

1st line --> supportive, bed rest Abx --> single dose of IV benzylpenicillin followed by oral penicillin V (phenoxymethylpenicillin) for 10d High dose aspirin Steroids (if carditis expected)

What Abx are indicated in acute rheumatic fever?

Single stat IV dose of benzylpenicillin, followed by 10d oral penicillin V. To kill GAS

Role of high dose aspirin or other NSAIDs in acute rheumatic fever?

To manage inflammation and reduce symptoms such as fever, joint pain, and swelling.

Importance of bed rest in rheumatic fever?

Rheumatic fever is one of the few conditions where bed rest is recommended as first-line treatment, even if the patient feels well. This is even more important in cases of suspected active myocarditis (indicated by abnormalities seen on the echocardiogram and a raised ESR), where limitation of exercise is strongly advised.

Is bed rest recommended in myocarditis?

Yes

After the initial attack, the child should be followed up regularly and prophylactic treatment started to reduce the chance of any future attacks. What is the current recommended first-line prophylactic treatment for rheumatic fever?

Benzathine penicillin G, given every 4 weeks as an IM injection.

What is the most common valve lesion in rheumatic fever overall?

Mitral regurgitation

What is the most common long-term cardiac complication of rheumatic fever?

Mitral stenosis

What 3 factors can trigger a relapse of rheumatic fever?

1) reinfection with GAS 2) pregnancy 3) COCP

Which medication can trigger a relapse of rheumatic fever?

COCP

What is the most common valvular abnormality in Kawasaki disease?

Mitral regurg

What 3 conditions can a strawberry tongue indicate?

1) Scarlet fever 2) Kawasaki disease 3) Toxic shock syndrome