pediatric rheumatic fever

1. RHEUMATIC FEVER

2. OUTLINE
 Definition
 Epidemiology
 Aetiology
 Clinical features
 DX
 Complications
 Mx
 RHD

3. Rheumatic Fever
 Is a delayed non suppurative systemic
illness. That occur as a sequele
following group A beta haemolytic
streptococcal (GAS) pharyngitis.
Commonly occurs
 Principally occurs 2-3weeks after a GAS
pharyngitis

4. Epidemiology
 Principally a disease of childhood, a
median age of 10yrs (5-15 yrs);
however, RF also occurs in adults
(20% of cases).
 Endemic in developing countries. An
annual incidence of100-300 per
100,000 population
 Risk factors attributed to low
socio-economic status

5.  Poor hygiene, overcrowding, Throat
infections
 Genetic predisposition- specific HLA
markers (HLA-DR7) & specific B-cell
alloantigen
 High concordance in monozygous
twins than dizygotic.

6. Etiology
 Follows pharyngitis with GAS (ie,
Streptococcus pyogenes).
 The associated aetiologic subtypes
include M type 1,3,5,6,18,24
 Abt 2/3 RF linked to prior pharyngitis.

7. Pathophysiology
 Two theories; Cytotoxic and immunologic
theories
 Cytotoxic theory
 GAS produces toxins e.g streptolysin O
 This is cytotoxic to human heart, brain, joints.
 But why delayed response then?
 Immunologic theory
 GAS shares some antigenic properties(M protein,
protoplast membranes, cell wall carbohydrates,
capsular hyaluronate)
 with specific mammalian tissue-brain, heart, joint

8.  And so the antibodies produced
against the GAS
 Will also cross react with the human
the type 1V collagen
 Result is nonexudative inflammatory
reaction
 Degeneration of the collagen in the
heart, brain, joints, subcutaneous
tissue
 Causing the clinical features of the
disease

9. Clinical Manifestations
 History
 Risk factors
 Fever
 Previous sore throat 40-60%
 Examination
 Arthritis 75%
 Carditis 30%-60%
 Chorea 10%-15%
 Erythema marginatum 3%
 Subcutaneous nodules

10. Clinical features cont…
 Migratory Polyarthritis: Most common
symptom and frequently is the earliest
manifestation of acute RF (70-75%). Affected
joints are painful, swollen, warm, erythematous,
and limited in their range of motion. The
arthritis reaches maximum severity in 12-24
hours and persists for 2-6 days (rarely> 4 wk)
at each site and is migratory but not additive.
more common and more severe in teenagers
and young adults than in younger children.
Characteristicaly reponds well to even small
doses of salicylates.

11. Cont….
 Carditis: occurs in about 50–60%.
Pancarditis is the most serious.
 Presents with chest pain.
 complication and 2nd most common
complication of RF. In advanced cases
have features of heart failure. There’s
characteristic new or changing murmurs
necessary for a diagnosis of rheumatic
valvulitis. Murmurs of acute RF are from
valve regurgitation, and those chronic RF
are from valve stenosis. Mainly mitral
valve

12. chorea
 Sydenham chorea, Vitus dance): occurs in 10–
15% of RF patients, 1-6months after
pharyngitis. Usually presents as an isolated,
non rythmic body movements
 usually unilateral
 That cease during sleep
 Neurologic behavior disorder. Emotional lability,
incoordination,. Complete resolution typically
occurs, with improvement in 1-2 weeks and full
recovery in 2-3 months; but symptoms may
wax and wane for several years.

13. Cont…
 Erythema Marginatum. Rare (<3%
of RF patients) but characteristic rash
of acute rheumatic fever. It consists of
erythematous, macular lesions with
pale centers. It occurs primarily on the
trunk and extremities, but not on the
face, and it can be accentuated by
warming the skin.

14. Cont…..
 Subcutaneous Nodules. Rare (1% of
RF patients). Consist of firm nodules
that are painless not erythematous
approximately 1cm in diameter along
the extensor surfaces of tendons near
bony prominences.

16. Jones Criteria
 For Diagnosis: 2major criteria or 1major +
2minor criteria. Evidence of recent GAS infection
(+ve throat swab, ASOT)
Major criteria Minor criteria
Carditis
Polyarthritis
Chorea
Subcutaneous nodules
Erythema marginatum
Fever
Arthralgia
Prolonged PR interval on
electrocardiogram
Elevated acute-phase
reactants (APRs): ESR/CRP

17. Investigations
 Throat culture (done early)
 Anti-Streptolysin O titres (ASOT): peaks 2-
3wks of RF
 Acute-phase reactants: Raised ESR, CRP
 Heart reactive antibodies: Tropomyosin is
elevated in persons with acute RF.
 Chest radiography (cardiomegally, features
of congestion. Helps differentiate
pneumonia)
 Echocardiograph
 ECG

18. Differential Diagnoses
Arthritis
 Rheumatoid arthritis
 Reactive arthritis (e.g., Shigella,
Salmonella, Yersinia)
 Sickle cell disease
 Malignancies
 Systemic lupus erythematosus
 Lyme disease

19. DD Cont…
Carditis
 Viral myocarditis
 Viral pericarditis
 Infective
Endocarditis
 Kawasaki disease
 Congenital heart
disease
 Mitral valve
prolapse
 Innocent murmurs
Chorea
 Huntington chorea
 Wilson disease
 Systemic lupus
erythematosus
 Cerebral palsy
 Tics
 Hyperactivity

20. Treatment
 Bed Rest: more with Carditis.
 Primary prevention: treat pharyngitis with
penicillin
 Secondary Prevention: (Monthly Benzathine
Penicillin 1.2MU IM), To prevent I.E
 Anti-inflammatory agents (ASA,
corticosteroids): withheld if arthralgia or
atypical arthritis is the only manifestation
of presumed acute RF so as not to obscure
migratory Arthritis and diagnosis.

21. Cont…..
 ASA: Used in patients with typical
migratory polyarthritis and those
with carditis WITHOUT
cardiomegaly or congestive heart
failure
 Patients with carditis and
cardiomegaly or congestive heart
failure should receive
corticosteroids. ASA introduced
later.

22. Rheumatic Heart Disease (RHD)
 Is the most serious and second
most common complication of
rheumatic fever.
 Contributes to 50% of acquired
heart lesions in developing world
 Acute rheumatic heart disease often
produces a pancarditis
characterized by endocarditis,
myocarditis, and pericarditis.

23. Cont…
 Endocarditis: manifested as valve
insufficiency in the order- mitral valve (65-
70% of patients), aortic valve (25%),
tricuspid valve (10%), pulmonary valve is
rarely affected. Severe valve insufficiency
during the acute phase may result in CHF and
even death (1% of patients).
 Pericarditis: rarely affects cardiac function or
results in constrictive pericarditis.
 Myocarditis may cause myocardial
dysfunction compounding the effect of CHF

24. Cont…
 Chronic RHD occur in 9-39% of adults
with previous rheumatic heart disease.
Fusion of the valve apparatus resulting
in stenosis or a combination of stenosis
and insufficiency develops 2-10 years
after an episode of acute rheumatic
fever, and recurrent episodes may
cause progressive damage to the
valves
 Diagnosis is with RF or h/o RF.(i.e
Jones Criteria)

25. RHD Clinical Features
 Dyspnea, chest discomfort, pleuritic
chest pain, edema, cough, or
orthopnea.
 New changing murmurs: can be apical
PSM; low pitched, rumbling apical
diastolic murmur (carey coombs)
 Pericardial friction rub indicates
pericarditis
 Physical features of CHF

26. RHD Investigations
 As in RF but specifically can do
 Echocardiography: MR, MS, other valvular
dysfunction
 CXR: Features of congestion,
cardiomegally.
 Histology of affected valves: Aschoff
Bodies (perivascular foci of eosinophilic
collagen surrounded by lymphocytes,
plasma cells, and macrophages), “bread
and butter” pericarditis.
 ECG: Dysrhthmias and AVblocks

27. RHD Treatment.
 Manage CHF, and RF accordingly
 Prophylaxis with Benzathine
Penicillin.
 Surgical Valve Replacements.

28. Complications of RHD:
 Infective Endocarditis
 Chronic and/or intractable CHF
 Cardiac Cirrhosis

29. Thank you