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

PHARYNGITIS

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

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
MAYOR MANIFESTATION

CARDITIS
POLYARTRITIS MIGRANS

SYDENHAM’S CHOREA (St. VITUS’ DANCE)

ERYTEMA MARGINATUM

SUBCUTANEUS NODULE
 Clinical Manifestations
10 MINOR MANIFESTATION

 Fever
 Arthralgia

 Acute-phase reactant ↑

(LED & CRP, leukocyte)

 ECG showed: prolong 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 nodules
14

 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 nodules
15
 Erythema marginatum
16

 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
 Other Clinical Features
19

 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
20
 2002–2003 WHO Criteria for The Diagnosis of ARF &
RHD (Based on The Revised Jones Criteria)
21

DIAGNOSTIC CATEGORIES CRITERIA

Primary episode of RF 2 Mayor/1 Mayor+2 Minor

Recurrent attack of RF in a patient without
established RHD
Recurrent attack of RF in a patient with
established RHD
Rheumatic chorea
Insidious onset of rheumatic carditis
manifestations + evidence of a
preceding GAS infection
2 Mayor/1 Mayor+2 Minor
manifestations + evidence of a
preceding GAS infection
2 Minor plus evidence of a
preceding GAS infection
Other major manifestations or evidence of
GAS infection not required

Chronic valve lesions of RHD Do not require any other criteria to be

diagnosed as having RHD
 Diagnosis: Differential Diagnosis of ARF
22

 Juvenile rheumatoid arthritis

 Systemic lupus erythematosus
 Other connective tissue diseases, including vasculitidies
 Bacterial endocarditis
 Reactive arthritis
 Sarcoidosis
 Medical Management
23

 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
24
Arthritis Mild Carditis Moderate Severe
Carditis Carditis

Bed rest 1-2 weeks 2-3 weeks 4-6 weeks 2-4 months
(Hospitalization) (up to 4 weeks) (CHF -)
Indoor 1-2 weeks 2-3 weeks 4-6 weeks 2-3 months
ambulation (up to 4 weeks)

Outdoor activity 2 weeks 2-4 weeks 1-3 months 2- 3 months

Full activity After 6-10 After 3 (6-10) After 3-6 Variable

weeks weeks months

These guidelines should be individualized by clinician(s) according to

patient and family circumstances.
 Eradication of The Pharyngeal Streptococcal Infection
25

 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
26

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
27
Arthritis Mild carditis Moderate Severe
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
 Potential Preventive Measures for Rheumatic Fever and
Rheumatic Heart Disease
28
 Primary vs Secondary Prevention
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GAS PHARYNGITIS
PRIMARY
XXX

ARF
SECONDARY
XXX

RHD
 Primary vs Secondary Prevention
PRIMARY SECONDARY
30
BENZATHINE 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 BID (maximum SULFONAMID po
250 mg BID) Oral 10 days Wt <30 kg 0.5 gr once daily
Wt ≥ 30 1 gr once daily
First Generation Cephalosporine
(cephalexin, cephadroxil) 15-20 mg/kg/dose bid
 Suggested Duration of Secondary Prophylaxis
31
Category of patient
Patient without proven carditis
Patient with carditis
(mild MR or healed carditis)
More severe valvular disease
After valve surgery
Duration of prophylaxis
For 5 years after the last attack, or until
18-21 years of age (whichever is
longer)
For 10 years after the last attack, or
until 21-25 years of age (whichever is
longer)
10 years or until 40 years of age
(whichever is longer), sometimes
lifelong prophylaxis
Lifelong