Rheumatic Fever Flashcards

1
Q

What is rheumatic fever?

A
  • Acute rheumatic fever is a systemic disease of childhood, often recurrent that follows group A beta hemolytic streptococcal infection
  • It is a delayed non-suppurative sequelae to URTI with GABH streptococci.
  • It is a diffuse inflammatory disease of connective tissue, primarily involving heart, blood vessels, joints, subcutaneous tissue and CNS
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2
Q

Epidemiology?

A
  • Ages 5-15 yrs are most susceptible
  • Rare <3 yrs
  • Girls>boys
  • Common in 3rd world countries
  • Environmental factors
    e.g. overcrowding, poor sanitation, poverty
  • Incidence more during fall, winter & early spring
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3
Q

Organism causes?

When do you see the clinical features - latent period?

A

group A beta-streptococcus
- There is a latent period of ~3 weeks (1–5 weeks) between the group A streptococcal infection and the appearance of the clinical features of RF

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4
Q

Cases of rheumatic fever are often associated with?

Name the URTI?

A

All cases associated with recent infection (e.g. pharyngitis)
- Antibody and cellular immune response cross-reacts with human connective tissue

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5
Q

Pathogenesis of rheumatic fever?

A
  1. Rheumatic fever affect the peri-arteriolar connective tissue
  2. It is believed to be caused by antibodycross-reactivity
  3. This cross-reactivity is a Type II hypersensitivity reaction and is termedmolecular mimicry
    - Delayed immune response to infection with group. A beta hemolytic streptococci.
  4. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves, joints, subcutaneous tissue & basal ganglia of brain
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6
Q

Group A beta strep strains that produces rheumatic fever?

A

M types l, 3, 5, 6,18 & 24

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7
Q

Pharyngitis?

A

produced by GABHS can lead to:
1. acute rheumatic fever
2. rheumatic heart disease
3. post streptococcal glomerulonephritis

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8
Q

Skin infection?

A

produced by GABHS leads to post streptococcal glomerulonephritis only
- It will not result in Rheumatic Fever or carditis

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9
Q

Clinical manifestation?

Diagnosis of RHD?

A

Jones criteria for the diagnosis of acute rheumatic fever
- 2 major criteria
- 1 major & 2 minor criteria along with the absolute requirement
Note: There are 5 major and 4 minor criteria & an absolute requirement for evidence (microbiologic or serologic) of recent GABHS infection

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10
Q

Jones major criteria?

A

JONES
J - joint involvement
O - myocarditis
N - nodules, subcutaneous
E - erythema marginatum
S - sydenham chorea

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11
Q

Jones minor criteria?

A

CAFE PAL
C - CRP increased
A - arthralgia
F - fever
E - elevated ESR

P - prolonged PR interval
A - anamnesis of rheumatism
L - leukocytosis

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12
Q

Absolute diagnostic evidence?

A
  1. throat cultures growing GABHS
  2. elevated anti-streptolysin O titers
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13
Q

Describe arthritis?

A
  • Migratory polyarthritis, involving major joints
  • Commonly involved joints-knee, ankle, elbow & wrist
  • Occur in 80%, involved joints are exquisitely tender
  • In children below 5 yrs arthritis usually mild but carditis more prominent
  • Arthritis do not progress to chronic disease
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14
Q

Describe carditis?

A
  • Manifest as pancarditis
    e.g. endocarditis, myocarditis and pericarditis - occur in 40-50% of cases
  • Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ
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15
Q

Phases of carditis?

A
  1. Acute phase - valvulitis
  2. Chronic phase - fibrosis, calcification & stenosis of heart valves
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16
Q

Features of carditis?

A
  1. Consists of either isolated mitral valvular disease or combined aortic & mitral valvular disease
  2. Valvular insufficiency: characteristic of both acute & convalescent stages of acute rheumatic fever
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17
Q

Sydenhams chorea?

A
  • The choreiform movements affect particularly the head and the upper limbs
  • They may be generalized or restricted to one side of the body (hemi-chorea)
  • Clinically manifest as-clumsiness, deterioration of handwriting, emotional lability or grimacing of face
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18
Q

Epidemiology of Sydenhams chorea?

A

occurs in children, rare in adults

19
Q

Causes of Sydenhams chorea?

A

likely due to molecular mimicry, with autoantibodies reacting with brain ganglioside in the basal ganglia
- Chorea eventually resolves completely, usually within 6 weeks

20
Q

Erythema marginatum?

A
  • Unique, transient lesions of 1-2 inches in size
  • It consists of erythematous, serpiginous, macular lesions with pale centers that are not pruritic
  • Pale center with red irregular margin
  • More on trunks & limbs & not on the face & it can be accentuated by warming the skin
21
Q

Erythema marginatum is associated with?

A

Often associated with chronic carditis

22
Q

Subcutaneous nodules?

A
  • Painless, pea-sized, palpable nodules
  • Mainly over extensor surfaces of joints, spine, scapulae & scalp
23
Q

Subcutaneous nodules are associated with?

A
  1. Associated with strong seropositivity
  2. Always associated with severe carditis
24
Q

Minor features of rheumatic fever?

A
  1. Fever – Low grade
  2. Arthralgia
  3. Pallor
  4. Anorexia
  5. Loss of weight
25
Q

Labaratory findings?

A
  1. High ESR
  2. Anemia, leucocytosis
  3. Elevated C-reactive protein
  4. ASO titre >200.
    - Peak value attained at 3 weeks, then comes down to normal by 6 weeks
  5. Anti-DNAse B test
  6. Throat culture-GABH streptococci
  7. ECG - prolonged PR interval
  8. Echo
26
Q

Echo results?

A
  1. valve edema
  2. mitral regurgitation
  3. LA & LV dilatation
  4. pericardial effusion
  5. decreased contractility
27
Q

Ddx for RHD?

A

Patients with infective endocarditis
- present with both joint and cardiac manifestations

28
Q

Differentiating between infective endocarditis vs RHD?

A
  1. blood cultures
  2. the presence of associated findings
    - hematuria, splenomegaly, splinter hemorrhages
29
Q

Treatment of rheumatic fever?

A
  1. bed rest
  2. antibiotic therapy
  3. anti-inflammatory therapy
30
Q

Antibiotic therapy?

A
  1. 10 days of orally administered penicillin or erythromycin
    or
  2. a single intramuscular injection of benzathine penicillin to eradicate GABHS from the upper respiratory tract
    - Afterwards, the patient should be started on long-term antibiotic prophylaxis
31
Q

Anti-inflammatory therapy?

A
  1. salicylates, corticosteroids
    - should be withheld if arthralgia or atypical arthritis is the only clinical manifestation of presumed acute rheumatic fever
  2. Acetaminophen can be used
32
Q

Treatment of Patients with typical migratory polyarthritis & with carditis without cardiomegaly or congestive heart failure?

A

Treatment with oral salicylates
- 100mg/kg/day in 4 divided doses PO for 3-5 days
- followed by 75mg/kg/day in 4 divided doses PO for 4-8wk

33
Q

Treatment of Patients with carditis & cardiomegaly or congestive heart failure?

A
  1. treatment with corticosteroids
  2. Prednisone 2mg/kg/day in 4 divided doses for 2-6wk followed by a tapering of the dose that reduces the dose by 5mg/24hr every 2-3 days
  3. At the beginning of the tapering of the prednisone dose, aspirin should be started at 75mg/kg/day in 4 divided doses to complete 12wk of therapy
34
Q

Supportive therapy for patients with moderate to severe carditis?

A
  1. digoxin
  2. fluid & salt restriction
  3. diuretics & oxygen
    Note: The cardiac toxicity of digoxin is enhanced with myocarditis
35
Q

Treatment of Sydenham Chorea?

A
  1. Anti-inflammatory agents are usually not indicated
  2. Sedatives: phenobarbital (16-32mg every 6-8hr PO) is the drug of choice
  3. If phenobarbital is ineffective, then haloperidol (0.01-0.03mg/kg/24hr divided bid PO) or chlorpromazine (0.5mg/kg every 4-6hr PO) should be initiated
  4. Long-term antibiotic prophylaxis
36
Q

Primary prevention in rheumatic fever?

A

10 days course of penicillin therapy
- about 30% of patients with acute rheumatic fever do not recall a preceding episode of pharyngitis

37
Q

Secondary prevention of rheumatic fever?

A

Secondary prevention is directed at preventing acute GABHS pharyngitis in patients at substantial risk of recurrent acute rheumatic fever

38
Q

Who should receive prophylaxis in secondary prevention?

A

Patients with documented history of rheumatic fever, including those with isolated chorea & those without evidence of rheumatic heart disease MUST receive prophylaxis

39
Q

Secondary prevention in rheumatic fever without carditis?

How long to give Prophylaxis treatment?

A

at least for 5 year or until age 21 year, whichever is longer

40
Q

Secondary prevention in rheumatic fever with carditis but without residual heart disease (no valvular disease)?

A

at least for 10 years or well into adulthood, whichever is longer

41
Q

Secondary prevention in rheumatic fever with carditis and residual heart disease (persistent valvular disease)?

How long to give prophylactic treatment for?

A

at least 10 years since last episode and at least until age 40 years: sometimes lifelong

42
Q

Drugs used in secondary prevention as prophylaxis?

A
  1. penicilin G benzathine
    - 600,000U for children, ≤27 kg
    -1.2 million U for children >27 kg, every 3wk
    - intramuscular
  2. penicilin V
    - 250mg twice a day
    - oral
  3. sulfadiazine or sulfisoxazole
    - 0.5g, once a day for patients ≤60lb; 1.0g, once a day for patients >60lb
    - oral
43
Q

Alternative for penicillin allergic patients?

A
  1. Cephalexin 500mg iv stat
  2. Azithromycin 500mg iv stat
  3. Clarithromycin 500mg iv stat or
  4. Clindamycin 900mg iv stat