Rheumatic Fever

(A disease of poverty)
Susana LEITU
Tuisese ILAITIA
Trainee Interns FSM
2008
 Acute rheumatic fever
is a multi-system auto-immune
disease that follows group A
streptococcal infection
(GAS)
 Background Information
• ARF & RHD – most common heart
disease in children & young adults
• Major health problem in developing
countries – Pacific Islands
• ≈ 15.6 million ppl are affected worldwide
– 2.4 million – are children (5-14yrs) in
developing countries
• Almost ≈ 0.5 million new cases declared
every year
 Group A Streptococcal
• Beta hemolytic
• Common culprit of pharyngitis, impetigo
and scarlet fever
• Virulent due to
– M protein
– F protein
– Acid (lipoprotein & hyaluronic) – cell and
capsule
– Produces toxin - streptolysin O
 Pathogenesis
• Abnormal humoral and cellular
immune response occurs
–Antigenic mimicry btw antibodies to
Strept M protein and human tissues
• Valves, myosin, tropomysin of heart
• Brain protein
• Synovial and cartilages
–Causing inflammation and tissue
damage
www.health.gov.mt
 Manifestations
Resolved sore throat – 1-2wks b4 symptoms

• Arthritis
– Pain & swelling in more then one large jnt
(ankle, knees, wrist)
– Usually ‘migratory’

• Fever
• Carditis – inflammation of heart
– Commonly present as a heart murmur
– Chest pain +/- difficult breathing

• Chorea (Sydenham’s)
– Twitching, jerking movement and muscle
weakness (most obvious in the face, hands
and feet)
– Usually resolves within 6 weeks (may last 6
months or more)
 Less common
• Subcutaneous Nodules
– Painless lumps – outside
surface of elbow, wrist, knees,
ankle in grps of 3-4-12)
– Skin not red/inflamed
– Last 1-2 weeks

• Erythema Marginatum
– Painless, flat pink patches on skin
– Usually on the trunk
– Hard to see on dark skin ppl

• Cough and Abdominal pain

 Diagnosis - Modified JONES

MAJOR CRITERIA MINOR CRITERIA

I. Carditis I. Fever
II. Polyarthritis II. Arthralgia
(migratory) III.Prolonged PR interval
III.Chorea IV.Elevated ESR, CRP
IV.Subcutaneous Nodules
V. Erythema
Marginatum
 1st episode of ARF (WHO)

< 2 MAJOR + Evidence of preceding

Group A Streptococcal Infection
OR

< 1 MAJOR + 2 MINOR + Evidence of

preceding Group A Streptococcal
Infection
• Untreated Grp A strept infxn
(eg pharyngitis) Æ lead to ARF

• Repeated / recurrent infxns lead

to chronic heart valve damage
(RHD)
 Differential diagnosis
• Systemic lupus eruthromatosus
• Infetive endocarditis
• Reactive arthritis
• Drug reaction
• Other connective tissue disease
 Baseline assessment
• ARF criteria
• ECG
• Swab throat and any infected skin sores –
usually negative
• FBC, ESR, ASOT, Blood culture
• Echocardiography – cardiac functions
and monitor progression
 Management (no RHD)
1. Admit for clinical care & education for
prevention further episodes
2. Benzathine Penicillin G (single dose) OR
Oral Penicillin V x 10 days
3. Paracetamol / asprin – fever and
arthritis
4. Severe Chorea – Carbamazepine or
Valporic acid
5. Carditis
• bed rest
• Anti failure medication – diuretics, ACEI,
digoxin (anticoagulants if AF)

When stabilized -
• Give 1st dose of 2° prophylaxis
• Educate pt & family - preventions
– Recurrent episodes, endocarditis, &
compliance
– Occasional follow ups
 Long-term Management
• Regular prophylaxis

1.Benzathine Penicillin G 600,000 –

1,200,000 units IM 3 weekly
– Penicillin V 250mg oral BD (if 1 is
contraindicated/not tolerated)
– Erythromycin 250mg oral BD (if penicillin
alergic)
Rheumatic Heart
Disease
 Rheumatic heart disease

is the chronic damage to heart

valves that follows acute
rheumatic fever
1. Mitral valve is affected in >90%
cases

2. Aortic valve

3. Tricuspid & pulmonary – rarely

(only in severe RHD when all valves
are affected)
 Valvular lesions
1. Mitral Regurgitation
1. Most common lesion
2. Find commonly in children & adolescent
2. Mitral Stenosis
1. Longer chronic damage to mitral valve
2. Commonly seen in adults
3. Complication – Atrial fibrillation
3. Aortic Stenosis
1. Rarely seen as an isolated lesion
2. Usually develop as a long-term complication
of AR
 Symptoms – depends on valve lesion & severity
• May not show for years until disease
become severe
– SOBOE
– Feeling tired
– General weakness
• As Lt ventricular failure advances (later)
– Orthopnea (SOB on lying down)
– Paroxysmal Noctural Dyspnoea (PND) –
waking up at night with SOB
• Rt ventrical may also fail
– Peripheral oedema
• If Atrial fibrillation (in MS)
– Palpitation
• Risk of thromboembolic strokes
• Patients with Aortic valve lesion may
experience (in addition to SOB)
– Angina
– Syncope
 Examination & Investigations
Clinical assessment should be conducted
CAREFULLY - early detection makes a
big different in life saving
• Careful auscultation to pick out
murmurs – refer for echocardiography
• Thorough clinical examination to assess
severity and complication
– Ventricular failure
– AF or stroke
– IE
♥ECG - Determines sinus rhythm &
ventricular failure

♥Chest X-ray - Aids in assessing

chamber size & detecting signs of
failure – pulmonary congestion

♥Echocardiography - Detects valve

damage, assessing the severity & Lt
ventricular function
 Management (RHD)
GOAL – prevent disease progression & to
avoid, or delay valve surgery
Mx depends on severity of disease

1. 2° prophylaxis (paeds 3wkly, adults

4wkly)
2. Regular clinical review (3wkly – yearly)
3. Follow up echo – follow progression
 Complications of RHD

• Congestive cardiac failure

• Infective endocarditis
• Atrial fibrillation – mostly in MS
• Stroke – 2˚ to AF, LA dilatation
 Case
13 yo Fijian male referred from
Sigatoka hospital for:

• Cough & fever – 2/7

• SOBOE & on rest – 1/52
• HPC:
– Previously well until 1/52 (developed SOB on
exertion and on rest)
– 2 days ago developed productive cough and
fever.
– Uses to play rugby before
• No chest-pain
• No PND – uses 1 pillow at night
• Gets tired easily
• No history of sore throat
• ROS:
9PU
9BO
• No vomiting, no dizziness and no fitting
• PMH
–Currently the 1st admission

• SH
–3rd eldest of 5 children
–Parents separated – children living
with mothers family
–Pt lives with maternal grandmothers
sister
 Clinical Findings
• O/E:
Well built and in dyspnoeic
– SaO2: 91%RA
– HR: 126
– BP: 104/88
– T: 36.7° C
– MAP: 95
 Chest
• Hyperdynamic precordium
• Apex beat @ 6th ICS/MCL
• Parasternal heave
• S1S2 – grade 4 Pansystolic murmur radiating
up to axilla
• Soft diastolic murmur
• Coarse crepitation bibasally
Abdomen:
• Soft, not distended
• Liver – 5-6cm BRCM
• Spleen not palpable

Extremities:
• Mild bipedal pitting oedema

Skin:
• Generalized tinea versicolour lesions
 Investigations
• Urea 2.9 (1.7-8.3) mmol/l
• Creatinine 47 (62-106) L µmol/l
• Sodium 137 (135-148) mmol/l
• Potassium 4.9 (3.5-5.3) Hmmol/l
• Chloride 107 (90-110) mmol/l
• Glucose/RBS 3.9 (3.9-9.9) mmoll
• Total Bilirubin 19 (0-17) µmol/l
• ALP 151 (40-129) 1H U/I
• ALT 33 (10-41) U/I
• AST 22 (10-37) U/I
• Total Protein 74 (66-87) H g/l
• Albumin 31 (34-48) g/l
ÓChest X-Ray

ÓBlood culture – R/o IE – 3 sets of blood

ÓNo growth 24hrs, 48hr and 72hrs

ÓEchocardiography
 Assessment
1. Rheumatic Heart Disease
2. Mitral regurgitation
3. ?? AR
4. Heart failure
5. DDx Infective Endocarditis
 Management
• Supportive – O2 etc

• Anti-failure
– Frusemide 67mg IV 6hourly
– Spironolactone 25mg orally 8hourly
– El Enalapril 1mg orally BD
– Digoxin 250mcg orally BD

• Crytalline Penicillin
 Follow up
☺For 3 weekly prophylaxis

☺Follow up - SOPD

☺Follow up echocardiography

☺IE prophylaxis