Rheumatic Fever Flashcards
What is rheumatic fever?
- 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
Epidemiology?
- 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
Organism causes?
When do you see the clinical features - latent period?
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
Cases of rheumatic fever are often associated with?
Name the URTI?
All cases associated with recent infection (e.g. pharyngitis)
- Antibody and cellular immune response cross-reacts with human connective tissue
Pathogenesis of rheumatic fever?
- Rheumatic fever affect the peri-arteriolar connective tissue
- It is believed to be caused by antibodycross-reactivity
- This cross-reactivity is a Type II hypersensitivity reaction and is termedmolecular mimicry
- Delayed immune response to infection with group. A beta hemolytic streptococci. - After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves, joints, subcutaneous tissue & basal ganglia of brain
Group A beta strep strains that produces rheumatic fever?
M types l, 3, 5, 6,18 & 24
Pharyngitis?
produced by GABHS can lead to:
1. acute rheumatic fever
2. rheumatic heart disease
3. post streptococcal glomerulonephritis
Skin infection?
produced by GABHS leads to post streptococcal glomerulonephritis only
- It will not result in Rheumatic Fever or carditis
Clinical manifestation?
Diagnosis of RHD?
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
Jones major criteria?
JONES
J - joint involvement
O - myocarditis
N - nodules, subcutaneous
E - erythema marginatum
S - sydenham chorea
Jones minor criteria?
CAFE PAL
C - CRP increased
A - arthralgia
F - fever
E - elevated ESR
P - prolonged PR interval
A - anamnesis of rheumatism
L - leukocytosis
Absolute diagnostic evidence?
- throat cultures growing GABHS
- elevated anti-streptolysin O titers
Describe arthritis?
- 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
Describe carditis?
- 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
Phases of carditis?
- Acute phase - valvulitis
- Chronic phase - fibrosis, calcification & stenosis of heart valves
Features of carditis?
- Consists of either isolated mitral valvular disease or combined aortic & mitral valvular disease
- Valvular insufficiency: characteristic of both acute & convalescent stages of acute rheumatic fever
Sydenhams chorea?
- 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