ARF

Question 1

• multisystem disease resulting from an autoimmune reaction to infection with GAS
• cardiac valvular damage (rheumatic heart disease [RHD]), which may persist after the other features have disappeared

Answer 1

ARF

Question 2

GLOBAL CONSIDERATIONS

Answer 2

• ARF and RHD are diseases of poverty
• RHD is the most common cause of heart disease in children in developing countries
• major cause of mortality and morbidity in adults
• 95% of ARF cases and RHD deaths now occur in developing countries

Question 3

• ARF is mainly a disease of children age
• peaks between

Answer 3

• 5-14 years
• 25 and 40 years

Question 4

ARF is exclusively caused by infection of the upper respiratory tract
with

Answer 4

GAS

Question 5

appear to be associated with susceptibility

Answer 5

HLA-DR7 and HLA-DR4

Question 6

class I] alleles have been associated with protection

Answer 6

• HLA-DRS,
• HLA-DR6,
• HLA-DR51,
• HLA-DR5S2,
• HLA-DQ

Question 7

Associations have also been described with polymorphisms at the ___, high levels of circulating mannose-binding lectin, and Toll-like receptors

Answer 7

tumor necrosis factor a locus (TNF a-308 and TNF-a-238)

Question 8

latent period of ARF

Answer 8

3 week (1-5 weeks)

Question 9

Clinical Features of ARF

Answer 9

• Polyarthritis (60-75%)
• carditis (50-60%)

Question 10

Erythema marginatum and subcutaneous nodules are now rare, being found in

Answer 10

<5% of cases

Question 11

Up to 75% of patients with ARF progress to

Answer 11

RHD

Question 12

hallmark of rheumatic carditis

Answer 12

Valvular damage

Question 13

is almost always affected

Answer 13

mitral valve

Question 14

characteristic manifestation of carditis in previously
unaffected individuals is ____, sometimes
accompanied by ____

Answer 14

• mitral regurgitation
• aortic regurgitation

Question 15

WHF Criteria for Echocardiographic Dx of RHD in Individuals <20 y.o

DEFINITE RHD

Answer 15

• Pathologic MR and at least two morphologic features of RHD of the mitral valve
• MS mean gradient 4 mmHg (note: congenital MV anomalies must be
  excluced)
• Pathologic AR and at least two morphologic features of RHD of the AV note: bicuspid AV and dilated aortic root must be excluded
• Borderline disease of both the MV and AV

Question 16

WHF Criteria for Echocardiographic Dx of RHD in Individuals <20 y.o

BORDERLINE RHD

Answer 16

• At least two morphologic features Of RHD of the MV without pathologic MR or MS
• Pathologic MR
• Patholorgic AR

Question 17

WHF Criteria for Echocardiographic Dx of RHD in Individuals <20 y.o

NORMAL ECHOCARDIOGRAPHIC FINDINGS

Answer 17

ALL 4

• MR that does not meet all 4 Doppler criteria (physiologic MR)
• AR that does not meet all 4 Doppler criteria (physiologic AR)
• An isolated morphologic feature of RHD of the MV (e.g valvular thickening without any associated pathologic stenosis or regurgitation
• Morphologic feature of RMD of the AV (e.g valvular thickening) w/o any associated Pathologic Stenosis or regurgitation

Question 18

most common form of joint involvement in ARF

Answer 18

Arthritis

Question 19

• migratory, moving from one joint to another over a period of hours
• ARF almost always affects the large joints
• most commonly the knees, ankles, hips, and elbows—and is
  asymmetric

Answer 19

Polyarthritis

Question 20

commonly occurs in the absence of other manifestations

Answer 20

Sydenham’s chorea

Question 21

• follows a prolonged latent period after group A streptococcal infection
• found mainly in females
• The choreiform movements affect particularly the head (causing characteristic darting movements of the tongue) and the upper limbs
• More than 50% of patients presenting with chorea will have carditis, for which reason echocardiography should be part of the workup

Answer 21

CHOREA

Question 22

classic rash of ARF

Answer 22

erythema marginatum

Question 23

painless, small (0.5—2 cm), mobile lumps beneath the skin
overlying bony prominences

Answer 23

Subcutaneous nodules

Question 24

The most common serologic tests for Evidence of a preceding GAS infection

Answer 24

• anti-streptolysin O (ASO)
• anti-DNase B (ADB) titers

Question 25

Diagnosis: initial ARF

Answer 25

2 major manifestations or 1 major plus 2 minor manifestations

Question 26

Diagnosis: recurrent ARF

Answer 26

2 major or 1 major and 2 minor or 3 major

Question 27

Major Criteria

Answer 27

- Low-risk populations + Carditis - Clinical and/or subclinical + Arthritis - Polyarthritis only + Chorea + Erythema marginatum + SC nodules

Question 28

Minor Criteria

Answer 28

- Low-risk populations + Polyarthralgia + Fever (238.5°C) + ESR =60 mm in the first hour and/or CRP 23.0 ma/dl + Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)

Question 29

Test to Always request :

Answer 29

- Electrocardiogram (ECG) - Echocardiogram - Complete blood count (CBC) - C-reactive protein (CRP) - Streptococcal serology (antistreptolysin and anti-DNase B)

Question 30

Tests to exclude alternative diagnoses, depending on clinical presentation and locally endemic infections for Neisseria gonorrhoeae

Answer 30

Urine molecular test

Question 31

Tests to exclude alternative diagnoses, depending on clinical presentation and locally endemic infections Chlamydia trachomatis

Answer 31

Urine molecular test

Question 32

Tests to exclude alternative diagnoses, depending on clinical presentation and locally endemic infections for viral hepatitis, Yersinia spp., cytomegalovirus (CMV), parvovirus 819, respiratory viruses, Ross River virus, Barmah Forest virus

Answer 32

Serologic or other testing

Question 33

is the drug of choice for ARF

Answer 33

Penicillin

Question 34

- no proven value in the treatment of carditis or chorea - At higher doses, the patient should be monitored for symptoms such as nausea, vomiting, or tinnitus

Answer 34

SALICYLATES AND NSAIDs

Question 35

Drug of choice for Carditis or chorea

Answer 35

Aspirin

Question 36

Is a suitable alternative to aspirin and has the advantage of twice-daily dosing

Answer 36

Naproxen

Question 37

In patients with severe chorea, ___ is preferred to haloperidol.

Answer 37

carbamazepine or sodium valproate

Question 38

Are effective and lead to more rapid symptom reduction in chorea. They should be considered in severe or refractory cases.

Answer 38

corticosteroids

Question 39

Untreated, ARF lasts on average

Answer 39

12 weeks

Question 40

With treatment, patients are usually discharged from hospital within

Answer 40

1-2 weeks

Question 41

inflammatory markers should be monitored every

Answer 41

1-2 weeks until they have normalized (usually within 4-6 weeks)

Question 42

should be performed after 1 month to determine if there has been progression of carditis.

Answer 42

echocardiogram

Question 43

if commenced within 9 days of sore throat onset, a course of ___ will prevent almost all cases of ARF that would otherwise have developed.

Answer 43

penicillin

Question 44

SECONDARY PREVENTION - best antibiotic for secondary prophylaxis - can be given every 3 weeks, or even every 2 weeks, to persons considered to be at particularly high risk

Answer 44

benzathine penicillin G

Question 45

AHA Recommendations for Duration of Secondary Prophylaxis CATERGORY OF PX - Rheumatic fever without carditis

Answer 45

For 5 years after the last attack or 21 years of age (whichever is longer)

Question 46

AHA Recommendations for Duration of Secondary Prophylaxis CATERGORY OF PX - Rheumatic fever with carditis but no residual valvular disease

Answer 46

For 10 years after the last attack, or 21 y.o (whichever is longer)

Question 47

AHA Recommendations for Duration of Secondary Prophylaxis CATERGORY OF PX - Rheumatic fever with persistent valvular disease, evident clinically or On echocardiography

Answer 47

For 10 years after the last attack, or 40 years of age (whichever is longer); Sometimes lifelong prophylaxis