pediatric alquds university

1. Rheumatic fever
Presented by: Musab saeed

2. • Rheumatic fever is a delayed, nonsuppurative,
autoimmune complication occurring 2-4 weeks
after an upper respiratory infection with group A
β-hemolytic streptococcus bacteria (GABHS) or
Scarlet fever
• Its characterized by inflammation of the heart, blood
vessels, joints, CNS and skin.
• The risk of developing rheumatic fever after an episode
of streptococcal pharyngitis has been estimated at
0.3-3%.

3. Epidemiology
Most common in children 5-15 years of age (
most susceptible to streptococcal throat infection
)
Rare < 3 years
No gender predilection ( some references f:m 2:1

4. Risk Factors
• The major risk factor is pharyngitis caused by
certain strains of GABHS (streptococcus pyogens)
• Children and young people
• Developing countries, lower socioeconomic
status
• Overcrowded and poor hygiene places
• Family history of rheumatic fever

5. ETIOLOGY
• Pathophysiology molecular mimicy
• Streptococcus pyogenes is a gram-positive cocci and it
produces two cytolytic toxins: streptolysin O and S.
• Rheumatogenic strains of GAS contain M proteins in their
cell wall and are immunogenic. B cells are stimulated to
produce anti-M protein antibodies against the infection
which also cross react with other tissues e.g. that of
the heart (causing rheumatic heart
disease), brain, joints and skin leading to a constellation of
multiorgan signs and symptoms. This is also exacerbated by
production of activated cross reactive T cells.

7. Clinical features
• Multisystem disorder that usually presents with
– Fever
– Anorexia
– Lethargy
– Joint pain
• Latentperiod: 2–4 weeks after an episode of streptococcal
pharyngitis.
• Diagnosis is made using ( Jones criteria )
– 2 or more major manifestations,
– Or 1 major and 2 or more minor manifestations,
– along with evidence of preceding streptococcal infection

9. Major features
• Carditis occurs in 30%-60% of cases. It is more common in
younger children
• Cardiac involvement is the hallmark and most important
complication of rheumatic fever, the inflammatory process may
involve all layers of the heart (pancarditis)
• Valvulits is frequent and typically causes insufficiency in left sided
valves (mitral and aortic) mitral regurgitation is the most common
early valvular manifestation.(tricups and pulmonary valves rarely
affected
• Carey-Coombs mid-diastolic murmur associated with mitral valve
inflammation.
• Myocarditis : is the most common cause of death in RF
• Pericarditis may cause chest pain, a pericardial friction rub.

10. Aschoff bodies are granulomatous lesions believed to be pathognomonic of
rheumatic fever. Most histologic descriptions have been limited to locations
adjacent to or within the myocardium

11. • Severe inflammation can cause congestive
heart failure (CHF).
• Patients with carditis may present with
shortness of breath, dyspnea upon exertion,
cough, paroxysmal nocturnal dyspnea, chest
pain, and/or orthopnea. Carditis may also be
asymptomatic and may be diagnosed solely
via auscultation or echocardiography.

12. Arthritis
• Most common and early manifestation its occurs
in 70% of patients
• Classicallymigratory,asymmetric and painful
• Typically affects large joints knee, ankle, elbow and
wrist but rarely small or unusual joints, such as
vertebrae.
• It does not result in chronic joint disease and
respond quickly to NSAIDs and Aspirin
• If not, the diagnosis is in doubt

13. • Sydenham chorea
• Late neurological manifestation at least 3
months after infection.
• This occurs in up to 25% of ARF cases in children
but is very rare in adults
• More common in female
• has both neurologic and psychological features.
• antineuronal antibody positive
.
• Its reflect involvement of the basal ganglia
and caudate nuclei
• Purposeless sudden movements

14. • Sydenham chorea usually resolves without
permanent damage within months
• May lasts for 2-3 years (medscape

15. Erythema marginatum
• occurs in approximately 5 -10%., it is very rare in
adults.
• Patients or parents may report a nonpruritic,
painless rash on the trunk and proximal limbs.
Red to pink macules and spread centrifugally with
central clearing
• It is usually noted only in fair–skinned patients.
• The lesions may persist intermittently for weeks
to months.

16. Never on face

17. Subcutaneous nodules
– Uncommon; associated with repeated episodes and severe
carditis; located over extensor surface of elbows, knees,
knuckles, and ankles, or scalp and spine;
– firm,
– nontender

19. Minor feactures
• Fever (of 101-102°F [38.2-38.9°C])
• Arthralgias
• Previous rheumatic fever
• Leukocytosis
• elevated erythrocyte sedimentation rate/C-
reactive protein
• and prolonged PR interval

20. • Arthralgia cannot be used as a minor
manifestation if arthritis is used as a major
manifestation (nelson)

21. Investigation
1. specific inflammatory markers
1. ESR and CRP
2. elevated WBCs
2. Serelogy
1. antistreptolysin O tities
abnormally elevated in 70- 80 % of cases
evidence of a recent GABHS infection.
The antistreptolysin antibodies peak in your system
between three to eight weeks after a strep
infection. Levels can remain high for several months
2. Anti –Dnase

23. Management
• Treatment strategies can be divided into
management
– Eradication of GABHS infection (primary prevention)
– Control of inflammation
– Supportive therapy
– Long term management (secondary prevention)

24. • Managementofthe acuteattack
– Single dose of benzylpenicillin1.2 million U i.m. or
– Oral penicillin250 mg 6-hourly for 10 days
– Penicillin-allergic: erythromycin or a cephalosporin
– Analgesia:optimally achieved with high dosesof Salicylates
• Treatment is then directed towards limiting cardiac
damageand relievingsymptoms

25. • Control of inflammation
• NSAIDS recommend only after the diagnosis of
rheumatic fever is certain.
• Corticosteroid in severe cardiac involvement
(CHF)

26. • Supportive
CHF : Diuretic, salt dietary restriction, digoixin
And bed rest
Sydenham chorea: if severe may be treated with
haloperidol

27. • Long term mangement
• Include continious antimicrobial prophylaxis to
prevent recurent episodes of rheumatic fever
• Duration depends on severity of heart disease

28. CATEGORY DURATION
Rheumatic fever without carditis At least for 5 yr or until age
21 year, whichever is longer
Rheumatic fever with carditis but
without valvular disease
At least for 10 yr or well into
adulthood, whichever is longer
Rheumatic fever with carditis &
persistent valvular disease)
At least 10 yr since last episode
& at least until age 40 yr;
sometime lifelong

29. Prognosis
• 1. there is no chronic sequelae of the joint, skin
and CNS manifestation of R fever
• 2. cardiac inflammation often leads to severe
valvular dysfunction, which may require
intervention immediately or many years after the
event. Valvular insufficiency or stenosis is usually
delayed ( more than 3 years after RF) and if
severe may require valve replacement of
valvuloplasty

30. • If rheumatic fever is not treated promptly,
long-term heart damage (called rheumatic
heart disease) may occur. Rheumatic heart
disease weakens the valves between the
chambers of the heart. Severe rheumatic
heart disease can require heart surgery and
result in death.