Revision - Kawasaki Disease & Rheumatic Fever Flashcards

1
Q

What is the diagnosis of Kawasaki disease based on?

A

The American Heart Association diagnostic criteria

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2
Q

What is the AHA diagnostic criteria for Kawasaki disease?

A

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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3
Q

Location of lymphadenopathy in Kawasaki disease?

A

Cervical

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4
Q

What can happen to hands and feet in Kawasaki disease?

A

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

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5
Q

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

A

Subacute phase (2-4w from fever onset)

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6
Q

What investigation can be done to exclude Streptococcus infection?

A

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

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7
Q

Mx of Kawasaki disease?

A

High dose aspirin + IV immunoglobulins

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8
Q

Role of aspirin in Kawasaki disease?

A

Reduce risk of coronary artery aneurysms & thrombosis

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9
Q

How long is aspirin given for in Kawasaki disease?

A

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

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10
Q

What is Reye’s syndrome?

A

A rare acute encephalopathy associated with liver failure.

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11
Q

Role of IVIG in Kawasaki disease?

A

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

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12
Q

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

A

Within 36 hours.

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

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13
Q

What are some complications of Kawasaki disease?

A

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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14
Q

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

A

Can rupture –> cardiac tamponade

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15
Q

What bacteria is implicated in rheumatic fever?

A

GAS

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16
Q

What is rheumatic fever?

A

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

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17
Q

How soon after Strep infection can rheumatic fever develop?

A

2-6 weeks

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18
Q

How does initial Strep. pyogenes infection typically present?

A

1) Pharyngitis (sore throat)

or

2) Cellulitis

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19
Q

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

A

As skin infection instead of pharyngitis

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20
Q

What criteria is used for diagnosing rheumatic fever?

A

Jones criteria

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21
Q

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

A

There must be evidence of recent GAS infection plus either;

  • 2 major criteria
  • 1 major criteria and 2 minor criteria
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22
Q

Give some evidence of a recent group A strep infection

A

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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23
Q

What are the 5 major criteria for rheumatic fever?

A

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)

24
Q

typical symptoms of rheumatic fever?

A
  • fever
  • joint pain
  • rash
  • chorea
  • SOB
  • nodules
25
Q

How can rheumatic fever affect the joints?

A

Affects large joints - hot, swollen, painful joints.

‘Migratory polyarthritis’ - different joints become inflamed and improve at different times.

26
Q

What is the most common symptom of rheumatic fever?

A

Polyarthritis (80%)

27
Q

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

A

Sterile inflammation with no organisms present

28
Q

What is the most significant manifestation of rheumatic fever?

A

Carditis

29
Q

What clinical features can carditis caused by rheumatic fever cause?

A

1) pericarditis

2) myocarditis

3) endocarditis

30
Q

How can myocarditis present?

A

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

31
Q

What is the most commonly affected valve in rheumatic fever?

A

Mitral valve

32
Q

How does rheumatic fever typically affect valves?

A

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.

33
Q

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

A

2-6m later

34
Q

What is Sydenham’s chorea sometimes preceded by?

A

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

35
Q

What are the 2 key skin findings in rheumatic fever?

A

1) SC nodules (only present with severe carditis)

2) Erythema marginatum rash –> look up pic!

36
Q

Where are subcutaneous nodules typically found in rheumatic fever?

A

Extensor surfaces

37
Q

What are the 4 minor criteria for rheumatic fever?

A

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

38
Q

Arthritis vs arthralgia?

A

Arthritis is an inflammatory condition that causes joint pain.

Arthralgia is joint pain.

39
Q

Mneumonic for rememebering major criteria for rheumatic fever: JONES

A

J - joint arthritis

O - organ inflammation such as carditis

N - nodules

E - erythema marginatum rash

S - sydenham’s chorea

40
Q

What 2 ECG findings may be present in rheumatic fever?

A

1) Prolonged PR
2) Tachycardia

N.B. may also be signs of pericarditis or myocarditis

41
Q

Investigations in rheumatic fever?

A

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

42
Q

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

A

1) anti-streptolysin (ASO)

2) anti-DNAse B

43
Q

When may troponin be raised in rheumatic fever?

A

Myocarditis

44
Q

Role of CXR in rheumatic fever?

A

Rule out HF

45
Q

Management of rheumatic fever can be divided into acute management and prophylaxis.

What does acute management of rheumatic fever involve?

A

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)

46
Q

What Abx are indicated in acute rheumatic fever?

A

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

To kill GAS

47
Q

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

A

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

48
Q

Importance of bed rest in rheumatic fever?

A

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.

49
Q

Is bed rest recommended in myocarditis?

A

Yes

50
Q

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?

A

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

51
Q

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

A

Mitral regurgitation

52
Q

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

A

Mitral stenosis

53
Q

What 3 factors can trigger a relapse of rheumatic fever?

A

1) reinfection with GAS

2) pregnancy

3) COCP

54
Q

Which medication can trigger a relapse of rheumatic fever?

A

COCP

55
Q

What is the most common valvular abnormality in Kawasaki disease?

A

Mitral regurg

56
Q

What 3 conditions can a strawberry tongue indicate?

A

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

57
Q
A