Far Eastern University – Nicanor Reyes Medical Foundation o Human leukocyte antigen (HLA) class II

IM 3B: RHEUMATIC HEART DISEASE - Immune Response:

o Recurrent infection à further develop additional
RHEUMATIC HEART DISEASE antibodies à attack heart valves à development of
- Multisystem disease resulting from an autoimmune reaction to RHD
infection with Group A streptococcus o Exaggerated immune response associated with
- Pathophysiology: molecular mimicry— autoimmune reaction repeated infection à ARF à RHD (chronic sequela)
to the heart valves and other tissues of the body (joints); all o Repeated infection à valvular inflammation à
structures of the heart can be involved (pancarditis) healing process à stenosis and other valvular lesions
- Almost all of the manifestations resolve completely except for o M proteins present in streptococcus are recognized as
cardiac valvular damage similar to the lining of the valve à attack heart valves
- ARF and RHD are diseases of poverty
à valvulitis, carditis àRHD
- Decline was largely attributed to improved living conditions:
o less crowded housing
o better hygiene
- “Hot spots”:
o Sub-saharan Africa
o Pacific nations— including Philippines
o Australia
o Indian subcontinent

CLINICAL FEATURES
- Latent period of 3 weeks (1-5 weeks) between the
precipitating group A streptococcal infection and the
appearance of the clinical features of ARF
o Exceptions: can occur even after 6 months from the
- Mainly a disease of children aged 5-14 years
previous streptococcal infection
- Recurrent episodes remain relatively common in
o Chorea
ARF adolescents and young adults
o Indolent carditis
- No clear gender association for ARF
- Many patients report a prior sore throat
- Preceding group A streptococcal infection is commonly
- Peaks between 25 and 40 years
subclinical
- disease of adult because it is a chronic
RHD o In these cases it can only be confirmed using
complication of ARF
streptococcal antibody testing
- More commonly affects females (2x)
- The most common clinical presentation of ARF is
polyarthritis and fever (prolonged recurrent fever
PATHOGENESIS
associated with migratory arthritis)
- Organism Factors:
- Polyarthritis is present in 60-75%
o ARF is exclusively caused by infection of the upper
- Carditis in 50-60%
respiratory tract with group A β hemolytic
- Chorea <2%-30% — specifically indicative of ARF
streptococci—cough, cold, tonsillitis (MC)
o Chorea + History of previous streptococcal infection is
o Certain M-serotypes (particularly types 1, 3, 5, 6, 14,
already diagnostic
18, 19, 24, 27, and 29)
- Erythema marginatum and subcutaneous nodules <5%
o Potential role of skin infection and of groups C and G
streptococci— still investigated
HEART INVOLVEMENT
- Host factors:
- 60% of patients with ARF progress to RHD
o 3-6% of the population may be susceptible to ARF
- Valvular damage is the hallmark of rheumatic carditis
o Familial clustering: concordance in monozygotic twins
- Mitral valve is almost always affected, sometimes together
(Chorea)
with the aortic valve

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HEART DISEASE AD ASTRA PER ASPERA
 - Previously unaffected individuals à mitral regurgitation
o Recurrent ARF à Mitral stenosis
- Myocardial inflammation à affect electrical conduction
pathways à P-R interval prolongation (normal is 0.12-0.2
s)
o 1st degree AV block or rarely higher-level block
o Softening of the first heart sound (due to prolongation
of PR interval)
o Mild mitral stenosis will produce loud S1 because
leaflets are thickened and restricted,
o Severe mitral stenosis — S1 is soft because the valve
doesn’t close anymore due to severe hardening
JOINT INVOLVEMENT
- Must be arthritic not arthralgic (arthritis= inflammation;
arthralgia= pain)
- Objective evidence:
o Inflammation
o Hot, swollen, red and/or tender joints
o Involvement of more than one joint
o Typically migratory— moving from one joint to
another over a period of hours
o Usually affecting the large joints: knees, ankles, hips,
elbows
o Asymmetric
- Pain is severe and usually disabling until anti-inflammatory
medication is commenced
- Less severe joint involvement- minor criteria
- Aseptic monoarthritis may be a presenting feature of ARF
- Highly responsive to salicylates and other NSAIDs:
o if given NSAID or aspirin— pain will be relieved within
24-48 hours
- Joint involvement that persists more than 1 or 2 days after
starting salicylate is unlikely due to ARF
o Salicylates and other NSAIDs should be withheld—
and pain managed with acetaminophen or codeine—
until the diagnosis is confirmed
CHOREA
- Sydenham’s chorea: follows a prolonged latent period
after a GABHS infection—found mainly in females
- Choreiform movements: affect particularly the head
(causing characteristic darting movements of the tongue)
and the upper limbs
- Eventually resolves completely, usually within 6 weeks
SKIN MANIFESTATIONS
- Erythema marginatum: classic rash of ARF
o Pink macules that clear centrally, leaving a
serpiginous, spreading edge
o Rash is evanescent, appearing and disappearing
before the examiner’s eyes
INTERNAL MEDICINE 3B: CARDIOLOGY – RHEUMATIC
HEART DISEASE
SUBCUTANEOUS NODULES
- Painless
- Small (0.5-2 cm)
- Mobile lumps beneath the skin
- Overlying bony prominences (hands, feet, elbows, occiput,
and occasionally the vertebrae)
- They are delayed manifestation, appearing 2-3 weeks after
the onset of disease, last for just a few days up to 3 weeks,
and are commonly associated with carditis
OTHER FEATURES: (minor criteria)
- Fever: occurs in most cases of ARF
o high-grade fever (39 C) is the rule
o lower grade temperature— not uncommon
- Elevated acute phase reactants: also present in most cases
- C-reactive protein (CRP) and erythrocyte sedimentation
rate (ESR) are often dramatically elevated — nonspecific,
also used in monitoring response of patient to treatment
- Occasionally the peripheral leukocyte count is mildly
elevated
EVIDENCE OF A PRECEDING GROUP A STREPTOCOCCAL
INFECTION:
- Essential in making the diagnosis of ARF
- Serologic evidence is usually needed
- Most common serologic tests are the anti-streptolysin O
(ASO) and anti-DNase B (ADB) titer
OTHER POST-STREPTOCOCCAL SYNDROMES THAT MAY BE
CONFUSED WITH RF:
- Post-streptococcal reactive arthritis (PSRA):
o Small-joint involvement that is often symmetric
o A short latent period following streptococcal infection
(usually <1week)
o Occasional causation by non group A-hemolytic
streptococcal infection
o Slower responsiveness to salicylate
o The absence of other features of ARF, particularly
carditis
- Pediatric autoimmune neuropsychiatric disorders
associated with streptococcal infection (PANDAS):
o Is a term that links a range of tic disorders and
obsessive-compulsive symptoms with group A
streptococcal infections
o At risk of carditis
CONFIRMING THE DIAGNOSIS:
- No definitive test
- The diagnosis of ARF relies on the presence of a
combination of the following:
o Typical clinical features AND
o Evidence of the precipitating Group A streptococcal
infection
VISION 20|20
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AD ASTRA PER ASPERA
 JONES CRITERIA Minor manifestations - Clinical: Polyarthralgia, fever
- 2 Major OR - Laboratory: Elevated ESR or
- 1 major + 2 minor + evidence of previous strep infection leukocyte count
MAJOR - Carditis - Prolonged PR interval
CRITERIA - Migratory polyarthritis Supporting evidence of - Elevated or rising ASO or other
- Sydenham’s chorea preceding streptococcal streptococcal antibody, or
- Erythema Marginatum infection within the last - A positive throat culture or
- Subcutaneous nodules
45 days - Rapid antigen test for group A
MINOR - Arthralgia
or
CRITERIA - Fever
- Elevated Acute phase - Recent scarlet fever
- reactants
- Prolonged PR interval RECOMMENDED TESTS IN CASES OF POSSIBLE ACUTE
Evidence of - (+) throat culture or rapid strep-antigen test RHEUMATIC FEVER:
antecedent - Elevated or rising strep TESTS FOR ALTERNATIVE
Group A strep - antibody test RECOMMENDED FOR ALL CASES: DIAGNOSIS:
infection - WBC - If possible
Mnemonic: - ESR endocarditis: repeated
J- joint involvement - CRP blood cultures
♥- Carditis - Blood cultures if febrile: - Possible septic
N- Neurologicà Sydenham’s o To rule out presence of arthritis: Joint aspirate
E- Erythema Marginatum other infection such as IE (microscopy and
S- Subcutaneous nodules o ARF blood culture is culture)
usually (-) - For choreiform
DIAGNOSTIC CATEGORIES CRITERIA - Electrocardiogram movements: copper,
Primary episode of - 2 major OR o repeat in 2 weeks and 2 ceruloplasmin, anti-
Rheumatic Fever - 1 major, 2 minor, evidence of months if prolonged P-R nuclear antibody, drug
preceding group A interval or other rhythm screen
streptococcal infection abnormality - For arboviral, auto-
Recurrent attack of - 2 major OR - CXR if clinical or immune or reactive
rheumatic fever in a - 1 major, 2 minor, evidence of echocardiographic evidence of arthritis: serology and
patient without preceding group A carditis auto-immune markers
established RHD streptococcal infection - Echocardiogram (consider
Recurrent attack of - 2 minor plus evidence of repeating after 1 month if
rheumatic fever in a preceding group A negative):
patient with established streptococcal infection o to screen for presence of
RHD VHD and myocarditis
Rheumatic chorea - Other major manifestations or - Throat swab (preferably before
insidious onset rheumatic evidence of group A giving antibiotics)— culture for
carditis streptococcal infection not Group A Strep
required - Anti-streptococcal serology:
Chronic valve lesions of - Do not require any other both ASO and anti DNase B
rheumatic heart disease criteria to be diagnosed as titers, if available (repeat 10-14
(patients presenting for having rheumatic heart disease days later if not confirmatory)
the first time with pure
mitral stenosis or mixed TREATMENT
mitral valve disease - Antibiotics
and/or aortic valve o All patients with ARF should receive antibiotics
disease) sufficient to treat the precipitating group A
Major manifestations: - Carditis streptococcal infection
- Erythema o Penicillin— DOC
- Polyarthritis marginatum § Can be given orally phenoxymethyl penicillin,
- Chorea 500 mg (250 mg for children <27 kg) PO twice
- Subcutaneous nodules daily or

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HEART DISEASE AD ASTRA PER ASPERA
 § Single dose of 1.2 million units (600,000 units for § delivered every 4 weeks
children 27 kg) IM Benzathine pen G § 5 years from occurrence of ARF or up to 21 years
o Amoxicillin: 50 mg/kg (max 1 g) daily x 10 days old, whichever is longer
- Salicylates and NSAIDs: o Oral penicillin V (250 mg)
o Treatment of arthritis, arthralgia and fever § Twice daily
o No proven value in the treatment of carditis or chorea § Less effective than Benzathine penicillin G
o Aspirin is the DOC, an initial dose of 80-100 mg/kg per § (+) allergy à erythromycin (250 mg) twice daily
day given every 6-8 hours
§ According to doc, since Filipinos are smaller we AHA RECOMMENDATION FOR DURATION OF SECONDARY
can use 50 mg/kg instead of PROPHYLAXIS
§ 80-100 mg/kg; but 80-100 mg/kg is the RF without carditis - For 5 years after the last attack or
recommended 21 years old (whicever is longer)
§ Patient would be at risk for upper GI bleeding RF with carditis but no - For 10 years after the last attack,
o Salicylate toxicity: residual valvular or 21 years old (whichever is
§ Nausea disease longeR)
§ Vomiting RF with persistent - For 10 years after the last attack
§ Tinnitus valvular disease, or 40 years old (Whichever is
§ *dose can be reduced to 60-70 mg/kg per day evident clinically or on longer).
for 2-4 weeks echocardiography - Sometimes lifelong
- Glucocorticoids
o Still controversial
o Improving the short or longer term outcome of
carditis
o Reduce the acute inflammation and result in more
rapid resolution of failure
o Gastrointestinal bleeding and fluid retention

PROGNOSIS
- Untreated: average 12 weeks
- With treatment: discharged from hospital within 1-2 weeks
- Inflammatory markers should be monitored every 1-2
weeks until they have normalized (usually within 4-6
weeks)
- Echocardiogram: after 1 month to determine if there has
been progression of carditis

PREVENTION:
- Primary prevention:
o Elimination of the major risk factors for streptococcal
infection: overcrowded housing
o Mainstay of primary prevention for ARF remains
primary prophylaxis
§ the timely (within 9 days) and complete
treatment of group A streptococcal sore throat
with antibiotics (Penicillin)
- Secondary
o Mainstay of controlling ARF and RHD
o Long-term penicillin prophylaxis to prevent
recurrences
o Best antibiotic for secondary prophylaxis is
Benzathine Penicillin G:
§ 1.2 million units or USE AT YOUR OWN RISK!
§ 600,000 units if less than 27 kg Notes from Lecture PPT, Fist Bump Trans

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