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CC: CVS and Blood vessels > Rheumatic fever > Flashcards

Rheumatic fever Flashcards

1
Q

What is rheumatic fever?

A

Autoimmune inflammatory process

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2
Q

What is the primary cause of rheumatic fever?

A

Sequelae of streptococcal infection

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3
Q

What group of streptococcus can cause rheumatic fever?

A

Group A

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4
Q

What is the primary strep species in group A strep?

A

Streptococcal pyogenes

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5
Q

What other conditions can Strep pyogenes (group A strep) cause?

A

Strep pharyngitis (strep throat)

Cellulitis

Scarlet fever

Post-streptococcal glomerulonephritis

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6
Q

What presentation of group A strep infection does acute rheumatic fever most commonly follow?

A

Strep pharyngitis (strep throat)

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7
Q

What is the risk of developing rheumatic fever after an episode of strep pharyngitis?

A

0.3-3%

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8
Q

Where, in australia, is rheumatic fever still a large concern?

A

Central aboriginal populations

500 per 100,000 children, 50x higher than non-indigenous

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9
Q

What is the pathophysiological cause of the development of acute rheumatic fever following group A strep infection?

A

Molecular mimicry with the M protein of the bacterial cell wall

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10
Q

Where is the largest global burden of acute rheumatic fever? How many cases are there, and how many new cases per year?

A

Developing countries

15.6 million people with it and 470,000 new cases per year

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11
Q

What percentage of people with acute rheumatic fever will likely, without intervention, go on to develop rheumatic heart disease?

A

60%

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12
Q

Does acute rheumatic fever show any gender bias?

A

No

Though mitral stenosis and Sydenham chorea are more common in post-puberty females

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13
Q

Which age group has a higher incidence of acute rheumatic fever?

A

Those aged 5-15 years. Pretty rare in adults past 35 years

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14
Q

What are the 5 main presentations of acute rheumatic fever?

A

Polyarthritis

Carditis

Erythema Marginatum

Sydenham chorea

Subcutaneous nodules

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15
Q

Of the 5 main presentations of acute rheumatic fever, what are the three that present the earliest? (around 1 month)

A

Polyarthritis

Carditis

Erythema marginatum

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16
Q

How long is it before Sydenham chorea and subcutaneous nodules typically present in acute rheumatic fever?

A

2.5 months

A great deal of variability though, particularly with the chorea. Might not appear, might appear very early, etc

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17
Q

What is the normal length of a bout of acute rheumatic fever?

A

3 months, if left untreated

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18
Q

Are the small joints of the fingers typically involved in the polyarthritis picture of acute rheumatic fever?

A

No

Only happens in post-streptococcal arthritis, a controversial related syndrome that doesn’t carry a risk of carditis

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19
Q

How does the polyarthritis of acute rheumatic fever typically present?

A

Ranges from arthralgia to frank polyarthritis

Swelling, redness, warmth and joint tenderness

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20
Q

What joints are typically involved in the polyarthritis of acute rheumatic fever?

A

Knees

Ankles

Elbows

Wrists

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21
Q

How long does it take for the polyarthritis of acute rheumatic fever to subside?

A

Typically within a few days up to a week

Fully disappears within 2-4 weeks

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22
Q

Does the polyarthritis of acute rheumatic fever ever move to new joints?

A

Yes

Typically described as migratory, though new joints are affected before the previously involved joints improve

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23
Q

Does the polyarthritis of acute rheumatic fever ever leave lasting damage?

A

Not normally

Can very rarely leave a Joccoud joint, where there is periarticular fibrosis

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24
Q

Does imaging of the joints in the polyarthritis of acute rheumatic fever typically show many gross changes?

A

No, aside from a slight effusion there are usually no changes

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25
Q

If a patient with migratory polyarthritis suspected to be secondary to acute rheumatic fever is started on NSAIDs, how does that change the progression of the polyarthritis?

A

Usually prevents it from migrating further, and reduces the severity and clinical timeframe

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26
Q

In the carditis of acute rheumatic fever, is the inflammation normally a pericarditis?

A

No

Usually it is a pancarditis, involving the pericardium, epicardium, myocardium and the endocardium

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27
Q

What are some of the signs that indicate a patient with acute rheumatic fever could have a pancarditis?

A

New murmurs, cardiac enlargement, pericardial rub and/or effusion

S3

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28
Q

What two murmurs are most common in the pancarditis of acute rheumatic fever?

A

MR

AR

Carey-Coombs flow murmur

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29
Q

What are the features of MR?

A

High pitched

Pan-systolic

Apical

Radiating into the axilla

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30
Q

What are the features of aortic regurgitation?

A

High pitched

Decrescendo

Diastolic

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31
Q

How often do patients with the pancarditis of acute rheumatic fever present with solitary aortic murmurs?

A

Very unusually

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32
Q

How can you make the diagnosis of acute rheumatic fever?

A

Two major JONES criteria (Joint arthritis, pancarditis, Nodules, Erythema marginatum, Sydenham chorea)

Or, one major JONES criteria and two minor

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33
Q

What are the minor JONES criteria?

A

Fever

Raised ESR or CRP

Leukocytosis

^ PR interval (can’t be included if pancarditis is used as major criteria)

Arthralgia (can’t be used if migratory arthritis used as major criteria)

Previous episode of rheumatic fever

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34
Q

How would you describe erythema marginatum?

A

Evanescent, pink or faintly red, non-pruritic

Central clearing

Erythematous

Can come and go very quickly

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35
Q

Where are the lesions of erythema marginatum of acute rheumatic fever found?

A

On the trunk and proximal aspects of the extremities

36
Q

What is an interesting thing about erythema marginatum?

A

Aside from coming and going within minutes to hours, they may become more evident after a hot bath or shower

37
Q

How are the nodules of acute rheumatic fever normally described?

A

Firm, painless lesions

Normally a few mm’s up to 2cm

38
Q

How do the nodules of acute rheumatic fever compare with the nodules of rheumatoid arthritis?

A

Smaller and more short-lived

Both conditions feature nodules on the elbow, but the nodules of acute rheumatic fever will be more over the olecranon whereas the nodules of rheumatoid arthritis will be 3-4cm distally

Overlying skin shows no signs of inflammation

39
Q

T/F the nodules of acute rheumatic fever are one of the more common features of acute rheumatic fever

A

False

They are the least occurring of the JONES major criteria

40
Q

When do the nodules of acute rheumatic fever typically present?

A

In the first week of the illness

41
Q

What can be said about a rheumatic fever patient if they develop subcutaneous nodules?

A

The development of the nodules of acute rheumatic fever is usually associated with a worse pancarditis

42
Q

Where are the nodules of acute rheumatic fever most commonly found?

A

Over bony surfaces (eg olecranon) and tendon sheaths

Elbow, knee, wrists, ankles, calcaneal tendon, spinous processes of vertebrae

43
Q

If you are assessing a patient for a pancarditis of acute rheumatic fever, what would you look for on examination?

A

A friction rub

New AR, MR, Carey-Coombs flow murmur

They will say the pain gets better when they lean forwards

44
Q

How would you describe the Sydenham chorea as part of acute rheumatic fever?

A

Uncoordinated, involuntary, purposeless movements

45
Q

Is Sydenham chorea as part of acute rheumatic fever associated with muscular weakness?

A

Yes

46
Q

What is a classical thing that happens to the patients grip strength when they have Sydenham chorea?

A

Will increase and decrease sporadically

Known as a relapsing grip, or milk maids sign

47
Q

Other than muscular weakness, what is the other major part of Sydenham chorea?

A

Emotional disturbance, with often abrupt changes in disposition (crying, restlessness etc)

Can have irritability, OCD symptoms, up to a transient psychosis

48
Q

Can Sydenham chorea be reduced with sedation?

A

Yes

Including sleep

49
Q

Is Sydenham chorea unilateral or bilateral?

A

Normally worse on one side

50
Q

What is the normal time of onset of Sydenham chorea after the initial presentation of acute rheumatic fever?

A

1-8 months

51
Q

What are some DDx’s of acute rheumatic fever?

A

Gonococcal arthritis

SLE

Juvenile rheumatoid arthritis

Lyme disease

Post-streptococcal reactive arthritis

52
Q

What are three good ways to diagnose acute rheumatic fever?

A

+ve throat culture for group A strep

+ve rapid strep antigen test

Elevated or rising antistreptolysin O antibody titer

53
Q

How useful are throat cultures in the diagnosis of acute rheumatic fever?

A

They are positive about 75% of the time by the time manifestations of ARF are present

54
Q

What is the unit of ASO titers?

A

Todd units per mL

55
Q

When do ASO titers peak in acute rheumatic fever?

A

Around 4-5 weeks post strep pharyngitis

56
Q

What week after the presentation of symptoms of acute rheumatic fever does the peak of ASO titer correspond to?

A

Week 2-3

57
Q

If a patient with suspected acute rheumatic fever is -ve for ASO (anti-streptolysin O), what do you do next?

A

Test for other strep antibodies

58
Q

What are some other strep antibodies that you can test for?

A

anti-DNAse B

Streptokinase

Antihyaluronidase

59
Q

What percentage of patients with acutee rheumatic fever have positive antibody tests if all 4 strep antibodies are tested for?

A

95%

60
Q

When does testing for acute phase things (ESR, CRP) become not useful?

A

When you have started antirheumatic drugs

61
Q

Is testing for acute phase reactants ever useful after starting on anti-rheumatic drugs?

A

Yes

Useful when you are tapering treatment down, because you can look for acute rebound of inflammation

62
Q

Is ESR or CRP more useful in acute rheumatic fever?

A

CRP

Because it normalises faster

63
Q

What kind of anaemia can chronic inflammation lead to?

A

Normocytic normochromic

64
Q

What is an investigation that you could order that could separate acute rheumatic fever from post-streptococcal glomerulonephritis?

A

Complement levels

Usually normal in ARF, but hypocomplementaemia is common in poststreptococcal glomerulonephritis

65
Q

What are 5 points of difference that mark acute rheumatic fever from post-streptococcal reactive arthritis?

A

Latent period between strep infection and the migratory arthritis is 1-2/52 with PSRA, cf 2-3/52 with ARF

PSRA has worse response to aspirin and NSAIDs cf ARF

No pancarditis in PSRA, and arthritis is much worse

Extra-articular manifestations such as tenosynovitis and renal abnormalities are often seen in PSRA

ESR and CRP tend to be lower in PSRA

66
Q

Does post-streptococcal reactive arthritis and acute rheumatic fever typically start around the same time?

A

No

Acute rheumatic fever will start about a week later than post-streptococcal reactive arthritis

67
Q

Is it thought that NSAIDs are very useful in post-streptococcal reactive arthritis?

A

Not as much as they are helpful in acute rheumatic fever

68
Q

T/F there is pancarditis in post-streptococcal reactive arthritis

A

F

69
Q

How important is it, to differentiate between post-streptococcal reactive arthritis, and acute rheumatic fever?

A

Not important because there is evidence that lots of people with post-streptococcal reactive arthritis can still go on to develop valvular damage

70
Q

What is the relationship between post-streptococcal reactive arthritis patients and the Jones criteria?

A

They usually fulfill the Jones criteria (one major, two minor)

71
Q

If a patient with post-streptococcal reactive arthritis is found to be Jones criteria positive, what should be done for treatment?

A

They should be treated as though they have acute rheumatic fever with antibiotics, NSAIDs

72
Q

What are the three cornerstones of management of acute rheumatic fever?

A

Antibiotic therapy

Heart failure management

Anti-inflammatory therapy

73
Q

Why are rheumatic fever patients put on antibiotics?

A

To reduce the carriage of group A streptococcal (GAS)

74
Q

Should household contacts be tested for GAS if the patient is infected?

A

Yes

And they should also be started on antibiotics even if they have no symptoms

75
Q

How should the heart failure of acute rheumatic fever be managed?

A

With standard heart failure drugs

Valve repair/replacement when the HF is due to regurgitant lesions

76
Q

Is valve replacement or repair generally preferred?

A

Repair

Avoids the long-term need for anticoagulation

77
Q

What is the anti-inflammatory of choice in acute rheumatic fever?

A

Asprin, 4-8g/day in adults, 80-100mg/kg/day in children

78
Q

When should you stop administering anti-inflammatories in acute rheumatic fever?

A

When there are no more symptoms

Or, when acute phase reactants are normal, but this should mainly be CRP not ESR which can be +ve for much longer

79
Q

What are some options for oral prophylactic antibiotics for acute rheumatic fever?

A

Penicillin V

Sulfadiazine

Macrolides

80
Q

Despite there being oral antibiotics available for acute rheumatic fever prophylaxis, what is the preferred antibiotic?

A

Benzathine penicillin G

Because it is given IM every four weeks, which prevents issues with compliance

81
Q

How should we change the administration of prophylactic antibiotics in high incidence rheumatic fever populations?

A

Increase the frequency of administration of benzathine penicillin G to every 2-3 weeks

82
Q

What are some side effects to benzathine penicillin G?

A

Life threatening allergic reaction

Pain at site of injection

83
Q

For the oral antibiotics used in preventing rheumatic fever, how do you decide which to administer?

A

Penicillin V is the preferred agent

Sulfadiazine if allergic to penicillin V

Macrolide (azithromycin) if allergic to penicillin and sulfadiazine

84
Q

What is the big factor that prolongs the time required for prophylactic antibiotics in the setting of acute rheumatic fever?

A

The presence or absence of pancarditis

85
Q

If a rheumatic fever patient has pancarditis and residual heart disease, how long should they take antibiotics?

A

10 years or until 40 years of age, whichever is longer

Sometimes lifelong prophylaxis

86
Q

If a rheumatic fever patient has pancarditis but no residual heart disease, how long should they take antibiotics?

A

10 years or until 21 years of age, whichever is longer

CC: CVS and Blood vessels (9 decks)

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