ACUTE RHEUMATIC
FEVER AND
RHEUMATIC HEART
DISEASE
RIA NOVA
Definition
Rheumatic Fever (RF) and Rheumatic Heart
Disease (RHD) are nonsuppurative complications
of Group A streptococcal (GAS) pharyngitis due to
a delayed immune response
Acute Rheumatic Fever (ARF) is a constellation of
symptoms that stems from a nonsuppurative,
auto-inflammatory multi-system response
following infection by GAS, or Streptococcus
pyogenes
RHD remains the most serious sequelae of RF
and causes considerable global morbidity and
mortality
INCIDENCE
4
PHARYNGIT
IS
GAS VIRAL
15-20% >80%
ARF
RESOLVED
0,3-3%
• Prevalence of RHD: 0,2 – 77,8/1000 children
RISK FACTORS
6
Family History of ARF
Genetic predisposition
(HLA-DR1, HLA-DRW6, twin)
Low socioeconomic
Age 6 -15 years (mostly 8 years)
Health System-related factors
Group A beta hemolytic
Streptococcus
7
STRUCTURE
Capsule: hyaluronic acid
Cell Wall: outer, middle and inner layer
Outer layer: proteins M, T and R
M component is the most potent & antigenic
Middle layer: specific carbohydrates eg N acetyl glucoamin
Inner layer:peptidoglycan –responsible for cell wall rigidity
Cytoplasm
Pathogenesis pathway for ARF
and RHD
8
Clinical Manifestations
9
CARDITIS
ON
ATI
EST POLYARTRITIS MIGRANS
NIF
MA SYDENHAM’S CHOREA (St. VITUS’
OR DANCE)
MAY ERYTEMA MARGINATUM
SUBCUTANEUS NODULE
Clinical Manifestations
10
ON Fever
TATI Arthralgia
IFES Acute-phase reactant ↑
(LED & CRP, leukocyte)
MAN
OR
ECG showed: prolong
MIN
interval PR
Rheumatic Carditis
11
Occur in 40-50% cases
Common in the first 3 weeks
The single most important prognostic
factor in RF; only valvulitis leads to
permanent damage
Valves affected:
Mitral (60%),
Aortic (10%);
Mitral and Aortic (30%),
Tricuspid & pulmonary valve rare
Rheumatic Carditis (cont’)
12
Clinical features:
Endokarditis/valvulitis :
Apical holosystolic murmur of MR
Children with previous RHD, a definite change in
the character of any of these murmurs or the
appearance of a new significant murmur
Miokarditis: Unexplained CHF or
cardiomegaly
Pericarditis: friction rub, chest pain,
effusion, ECG changes
Congestive Heart Failure
Rheumatic Arthritis
13
The most frequent major manifestation
Occurring up to 75% of patients in the first attack
Typically present as migratory polyarthritis
Most often in the larger joints, small joints of the
hands, feet and neck are rarely affected
Inflamed joints are characteristically warm, red
and swollen, & aspirated sample of synovial fluid
may reveal a high leukocyte count
Subcutaneous rheumatic
14
nodules
The incidence varies; reported in up to 20%
of cases
Round, firm, freely movable, painless lesions,
size 0.5–2.0 cm
They occur in corps over bony prominences
or extensor tendons
Common locations: the elbows, wrists, knees,
ankles & Achilles tendons
Similar lesions occur in SLE and rheumatoid
arthritis.
Subcutaneous rheumatic
15
nodules
Erythema
16
marginatum
Present in 7-15% of patients
Usually occurs early in the course of a rheumatic
attack & highly specific to RF
The lesions are multiple, nonpruritic & nonpainful,
blanch under pressure, and are only rarely raised.
Usually on the trunk or proximal extremities, &
never on the face
Erythema marginatum
17
Sydenham’s Chorea
18
Primarily females, rare after 20 years old
Prevalence 5–36%
May occur alone
Has a longer latency period after GAS infection,
as long as 1–7 months
Characterized by emotional lability,
uncoordinated movements & muscular
weakness
First sign: difficulty walking, talking, writing,
then the movements are abrupt and erratic
Sydenham’s Chorea
19
Sydenham’s Chorea
20
Sydenham’s Chorea
21
Video chorea
22
Other Clinical Features
23
Less frequent or less specific to ARF:
Epistaxis
Abdominal pain (5%) due to peritonitis
Hematuria (5%)/renal involvement
When routine biopsy done, in up to 39%
Pneumonitis
Mild pleuritis (5 - 10%)
Encephalitis (extremely rare)
Diagnosis
24
2002–2003 WHO Criteria for The
Diagnosis of ARF & RHD (Based on The
Revised Jones Criteria)
25
DIAGNOSTIC CRITERIA
CATEGORIES
Primary episode of RF 2 Mayor/1 Mayor+2 Minor
manifestations + evidence of
a
preceding GAS infection
Recurrent attack of RF in a 2 Mayor/1 Mayor+2 Minor
patient without established RHD manifestations + evidence of
a
preceding GAS infection
Recurrent attack of RF in a 2 Minor plus evidence of a
patient with established RHD preceding GAS infection
Rheumatic chorea Other major manifestations or
Insidious onset of rheumatic evidence of GAS infection not
required
carditis
Diagnosis: Differential Diagnosis
of ARF
26
Juvenile rheumatoid arthritis
Systemic lupus erythematosus
Other connective tissue diseases,
including vasculitidies
Bacterial endocarditis
Reactive arthritis
Sarcoidosis
Medical Management
27
General measures: Bed rest
Antimicrobial therapy:
Eradication of the pharyngeal
streptococcal infection
Suppression of the inflammatory process
Management of heart failure
Management of chorea
General Measures
28 Arthritis Mild Carditis Moderate Severe
Carditis Carditis
Bed rest 1-2 2-3 weeks 4-6 weeks 2-4
(Hospitalizat weeks (up to 4 months
ion) weeks) (CHF -)
Indoor 1-2 2-3 weeks 4-6 weeks 2-3
ambulation weeks (up to 4 months
weeks)
Outdoor 2 weeks 2-4 weeks 1-3 months 2- 3
activity months
Full activity After 6- After 3 (6- After 3-6 Variable
10 weeks 10) weeks months
These guidelines should be individualized by clinician(s) according to
patient and family circumstances.
Eradication of The Pharyngeal
Streptococcal Infection
29
Benzathine benzylpenicillin
600.000 U IM: weight < 30 kg
1,2 juta U IM: weight > 30 kg
As a first dose of prophylaxis
Allergy to Benzathine
benzylpenicillin
Erythromisine 40-50
mg/Kg/day in 2-4 doses for 10
days
Suppression of The Inflammatory
Process
30
Clinical Manifestation Therapy
ATHRALGIA ANALGESIC
(PARACETAMOL)
ARTHRITIS SALICYLATES 90-100
mg/Kg/day for 2 weeks
→ 25 mg/Kg/day for 4-6
weeks
CARDITIS Prednisone 2 mg/Kg/day
for 2 weeks →tapp off 2
weeks →salisilate 75
mg/Kg/day for 2-6 weeks
Suppression of The
Inflammatory Process
31
Arthritis Mild Moderate Severe
carditis carditis carditis
Prednisone 0 0 2-4 weeks 2-6 weeks
Salicylates 1-2 weeks 2-4 weeks 6-8 weeks 2-4
months
Prednisone:1–2mg/kg-day, to a maximum of 80mg/day given
once daily, or in divided doses). After 2–3 weeks of therapy the
dosage may be decreased by 20–25% each week. While reducing
the steroid dosage, a period of overlap with aspirin is
recommended to prevent rebound of disease activity
Salicylates: 90-100 mg/kg/day/divided into 4-5 doses for 2
weeks
60–70mg/kg-day for 3–6 weeks
Treatment Chorea
32
sydenham
Potential Preventive Measures for Rheumatic
Fever and Rheumatic Heart Disease
33
Primary vs Secondary
Prevention
34
GAS
ARF
PHARYNGIT
IS
PRI
MA
RY
XXX
R
H
D
SEC
OND
ARY
XXX
Primary vs Secondary
Prevention
PRIMARY SECONDARY
BENZATHINE
35 BENZYHL PENICILLIN G BENZATHINE BENZYHL PENICILLIN G
600.000 U IM wt < 30 kg (every 3-4 weeks)
1,2 juta U IM wt > 30 kg 600.000 U IM wt < 30 kg
1,2 juta U IM wt ≥ 30 kg
PHENOXYMETHYL PENICILLIN (PENICILIN V) PENICILLIN V 2 x 125-250 mg
3-4x 250 mg for 10 days
ERYTRHOMYCIN ERITHROMYCIN 250 mg twice daily
20-40 mg/kg/day /2-4 doses/10 days
Azithromycine :12.5 mg/kg/day once daily
500 mg on first day, 250 mg per day for the
next 4 days
Clindamycin 20 mg/kg per day divided in 3
doses (maximum 1.8 g/d) Oral 10 days
Clarithromycin 15 mg/kg per day divided SULFONAMID po
BID (maximum 250 mg BID) Oral 10 days Wt <30 kg 0.5 gr once daily
First Generation Cephalosporine Wt ≥ 30 1 gr once daily
(cephalexin, cephadroxil) 15-20 mg/kg/dose
bid
Suggested Duration of Secondary
Prophylaxis
36
Category of patient Duration of prophylaxis
Patient without proven For 5 years after the last
carditis attack, or until 18-21 years
of age (whichever is longer)
Patient with carditis For 10 years after the last
(mild MR or healed carditis) attack, or until 21-25 years
of age (whichever is longer)
More severe valvular 10 years or until 40 years of
disease age
(whichever is longer),
sometimes
lifelong prophylaxis
After valve surgery Lifelong
