ARF & RHD SIMPLIFIED

1. ACUTE RHEUMATIC FEVER
&
RHEUMATIC CARDITIS
Dr. Murtaza Kamal
MBBS, MD, DNB
Division of Pediatric Cardiology
Department of Pediatrics
Safdarjung Hospital & VMMC, New Delhi
DOP- 06/08/2016
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2. OBJECTIVES
 To know about the epidemiology of the
disease
 To understand the pathogenesis of rheumatic
heart disease
 To know about the clinical features: cardiac &
non-cardiac manifestations
 To learn about the laboratory studies of RHD
 To understand the principles of management
2

3. ACUTE RHEUMATIC FEVER
Autoimmune consequence of infection with
Group A streptococcal infection
Results in a generalised inflammatory response
affecting brains, joints, skin, subcutaneous
tissues, heart, respiratory system, vessels,
serosal membranes, tendons and fascial sheaths
Clinical presentation can be vague and difficult
to diagnose
Currently, the Jones criteria form the basis of the
diagnosis of the condition
3

4. Epidemiology
• Non suppurative complications of group A
streptococcal pharyngitis
• Certain serotype of GAS (M type 1,3,5,6,18,24)
• A delayed immune response caused by antibody
cross reactivity that can involve the heart, joints,
skin and brain
• Latent period of 1-3 weeks
• Gram positive cocci rich in M protein is a
virulence factor
4

5. Epidemiology
• Skin infection does not causes rheumatic fever
or carditis because skin lipid cholesterol inhibit
antigenicity.
• Incidence 5.3/1000 in Indian population*
• Incidence of RF following streptococcal throat
infection is 0.3%*
• Commonest age group 5- 15 yr
• First episode rare before 3 yr and after 30 yrs
• Male and female both equally affected
*(ICMR survey result)5

6. Epidemiology
• Mitral valve disease and chorea are more
common in girls
• Aortic valve involvement more common in boys
• Poor socioeconomic condition, unhygienic living
conditions, overcrowded household predispose
to streptococcal infections
• Common in tropics and subtropics
• Common in colder months
6

7. Patho-physiology
• The cytotoxicity theory- GAS toxin produces
enzyme streptolysin O
• The immune mediated theory- Immunological
cross reactivity between the GAS components
and mammalians tissue
• M protein M1,M5,M6, M19 share epitopes with
human tropomyosin and myosin
7

8. Patho-physiology
• Infection leads to rheumatic fever several
weeks after the sore throat has resolved
• The organism spreads by direct contact with
oral or respiratory secretions, and spread is
enhanced by crowded living conditions
• Patients remain infected for weeks after
symptomatic resolution of pharyngitis and
may serve as a reservoir for infecting others
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9. Patho-physiology
Fibrinoid degeneration of connective tissue
Inflammatory edema
Inflammatory cell infiltration and proliferation of
specific cells resulting in formation of ASHCOFF
NODULES
• Resulting in-
– Pancarditis in heart
– Arthritis in joints
– Nodules in subcutaneous tissue
– Chorea
9

10. Clinical presentation
• Modified Jones criteria (revised in 1992) provide
guidelines for the diagnosis of rheumatic fever
• The Jones criteria require the presence of:
2 major
Or
1 major and 2 minor criteria
• At least one essential criteria must be there in
diagnosis of rheumatic fever
• A diagnosis of rheumatic heart disease is made
after confirming antecedent rheumatic fever
10

11. Major criteria
(1) Migratory poly arthritis
(2) Pancarditis
(3) Chorea
(4) Sub cutaneous nodules
(5) Erythema marginatum
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12. Minor crietria
(1) CLINICAL CRITERIA-
(A) Fever
(B) Arthralgia
(2) LABORATORY CRITERIA-
(a) Acute phase reactants ( CRP, ESR)
(b) Prolonged PR interval in ECG
12

13. Essential Criteria
Evidences of recent streptococcal infections
(1) Elevated ASLO titre
(2) Positive throat swab culture
(3)Rapid antigen test for group A streptococci
13

14. ARTHRITIS
• Most common manifestation. (70%)
• Large joints (knee, elbow, ankle, wrist)
• Poly arthritis- succession or simultaneous
• Migratory in nature
• Swelling, heat, redness, severe pain, tenderness
,limitation of movement
• Responds drammatically with salicylates
• Subsides without residual deformity
• Lasts 1-5 weeks
14

15. CARDITIS
• Occurs in 50% of patients
• Tachycardia ( out of proportion to fever)
• Heart murmur of MR or AR or both
• Pancarditis ( pericarditis, myocarditis, endocarditis)
1 Endocardial-
- MR or AR murmurs indicative of dilatation of valve
with or without associated valvulitis
- Short mid-diastolic murmur (Carey-Coombs) may be
present
- Changing quality of heart sounds
2. Myocardial-
- Tachycardia even at rest.
- Arrhythmias or ectopic beats
- Cardiomegaly- on physical exam, CXR or ECHO
- Congestive cardiac failure – right or left sided 15

16. CARDITIS
3. Pericardial-
- Pericarditis
- Pericardial effusion
ECG Changes-
- Changing contour of P waves
- Inversion of T waves
- Prolongation of PR interval
• Sign of CHF (gallop rhythm, cardiomegaly, distant
heart sound)
• Maybe self limiting or may lead to slowly
progressive valvular deformity
• Mitral valve attacked in 75% cases, aortic in 30% (
but rarely as the sole valve), tricuspid and
pulmonary in < 5% cases
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17. Thick valves, small vegetations Fish mouth mitral valve opening
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18. CHOREA
• Sydenham’s chorea (St vitus’ dance) in 15%
• More common in prepubertal girls (8-12 yrs)
• Neuro psychiatric disorder
• Neurological - Choreic movement and hypotonia
• Psychiatric - emotional lability, hyperactivity,
separation anxiety, OCD
• Begins with emotional lability replaced by
choreic movement and then motor weakness
• Elevated titre of anti neuronal antibody
18

19. ERYTHEMA MARGINATUM
• In less than 10 % cases.
• Non-pruritic ,serpiginous
or annular erythematous
rashes.
• Trunk and inner proximal
portion of extremities
• Never seen on face
• Evanescent, disappears on
exposure to cold
• Shape of rings or crescents
with clear centers
19

20. SUBCUTANEOUS NODULES
• 2- 10 % of cases
• Commonly in cases with recurrences
• Hard, painless, nonpruritic,freely movable
swelling of 0.2 to 2 cm
• Extensor surface of both legs, small joints, scalp,
spine
• Not transient, lasting for weeks
• Are recurrent
• Indistinguishable from rheumatoid nodules
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21. 21

22. Exception of jones criteria
(1) Chorea may occurs as the only manifestation
of rheumatic fever
(2) Indolent carditis can be the only
manifestation who comes one month after the
onset of RF
(3) Some time recurrences of rheumatic fever
may not fulfill the Jones criteria
22

23. Clinical course of disease
• Only carditis can causes permanent cardiac
damage
– Sign of mild carditis disappears rapidly in weeks
but severe carditis may last for 2 to 6 months
• Arthritis subsides with in a few days to several
week
– Even without treatment does not causes permanent
damage
• Chorea gradually subsides in 6-7 months or
longer and does not causes permanent
neurological sequele
23

24. LABORATORY INVESTIGATIONS
• Rapid antigen detection test
• Throat culture
• Antistreptococcal antibodies
◦ The elevated level of antistreptococcal
antibodies is useful, particularly in patients
that present with chorea as the only
diagnostic criterion
◦ 333 Todd Units
◦ Antibody titers should be checked at 2-week
intervals in order to detect a rising titer
24

25. LABORATORY INVESTIGATIONS
• Ratio of antibodies to extracellular streptococcal
antigens rises during the first month after
infection and then plateaus for 3-6 months
before returning to normal levels after 6-12
months
• The anti-DNAse B has a slightly higher sensitivity
(90%)-240 Todd Units
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26. LABORATORY INVESTIGATIONS
• Antihyaluronidase results are frequently
abnormal in rheumatic fever patients with a
normal level of ASO titer and may rise earlier
and persist longer than elevated ASO titers
during rheumatic fever
• Acute phase reactants- Both tests have a high
sensitivity but low specificity for rheumatic fever
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27. INVESTIGATIONS
• CHEST X-RAYS:
– Cardiomegaly
– Pulmonary congestion
– Other findings consistent with heart failure
• ECHO :
– Annular dilatation
– Elongation of the chordae to the anterior leaflet
– A postero laterally directed mitral regurgitation jet
The left ventricle is frequently dilated in association
with a normal or increased fractional shortening
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28. INVESTIGATIONS
• ECG:
Sinus tachycardia
Sinus bradycardia
First-degree atrioventricular (AV) block
(prolongation of the PR interval)
ST segment elevation may be present and is
marked most in lead II, III, aVF, and V4 -V6
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29. INVESTIGATIONS
• Heart catheterization-
In acute rheumatic heart disease, this procedure
is not indicated
With chronic disease, heart catheterization has
been performed to evaluate mitral and aortic
valve disease and to balloon stenotic mitral
valves
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30. HISTOLOGIC FINDINGS
Aschoff bodies:
Perivascular foci of eosinophilic collagen
surrounded by lymphocytes, plasma cells, and
macrophages
Found in the pericardium, perivascular regions
of the myocardium, and endocardium
Anitschkow cells:
Plump macrophages within Aschoff bodies
Bread and butter pericarditis:
In the pericardium, fibrinous and serofibrinous
exudates
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31. TREATMENT AND MANAGEMENT
 Therapy is directed towards:
Eliminating the group A streptococcal pharyngitis (if
still present)
Suppressing inflammation from the autoimmune
response
Providing supportive treatment for congestive heart
failure
 Following the resolution of the acute episode,
subsequent therapy is directed towards:
Preventing recurrent rheumatic heart disease (in
children)
Monitoring for the complications and sequelae of
chronic rheumatic heart disease (in adults)
31

32. Drugs for primary prophylaxis of acute
rheumatic fever
Drugs Doses Sore throat
treatment
BENZATHINE
PENICILLIN G
Deep IM after
Sensitivity test
1.2 million unit (>27 kg)
0.6 million unit (<27 kg)
Single dose
PENICILLIN (oral) CHILDREN – 250 mg QID
ADULTS – 500 mg TID
10 days
AZITHROMYCIN
(oral)
12.5 mg/kg/day
Once daily
5 days
CEPHALEXIN (oral) 15-20 mg/kg/dose BD 10 days
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33. Prevention of rheumatic fever
Primary prevention-
• 10 days course of penicillin therapy for
streptococcal pharyngitis
• Patient sensitive to penicillin should advise
erythromycin 20-40 mg/kg in two divide dose
Secondary prophylaxis-
Patient with documented history of rheumatic
fever, isolated chorea, those without evidence
of rheumatic heart disease must receive
prophylaxis
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34. SECONDARY PROPHYLAXIS
• Benzathine penicillin G 1.2 million units given
intra muscularly every 21st day after sensitivity
testing
• Alternative method if any reaction to penicillin:
(1) Oral penicillin V 250 mg twice daily
(2) Oral sulfadiazine 1 gm once daily
(3) Oral sulfisoxazole 0.5 gm once daily
(4) Oral erythromycin ethyl succinate 250 mg BD
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35. Duration of prophylaxis for rheumatic fever
Category Duration
RHEUMATIC FEVER WITHOUT
CARDITIS
5 yr or until age 21 yr
Whichever is longer
RF WITH CARDITIS BUT
WITHOUT RESIDUAL HEART
DISEASE
(NO VALVULAR HEART DISEASE)
10 yr or well into adulthood
Whichever is longer
RF WITH CARDITIS AND
RESIDUAL HEART DISEASE
(PERSISTENT VALVULAR HEART
DISEASE)
At least 10 yr since last episode
and last until age 40 yr
Some time life long prophylaxis
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36. Management of rheumatic fever
• Bed rest:
Duration depends on type and severity
of manifestation
One week for isolated arthritis
Several weeks for severe carditis
Full activity is allowed when ESR becomes normal
• Anti -inflammatory drugs:
Mild to moderate carditis-
Aspirin 90-120 mg/kg/day in 4-6 divided doses
for 4-6 weeks then tapering of 75 mg /kg /day in
next 2 weeks
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37. Management of rheumatic fever
• Severe carditis-
Add steroid prednisone 2 mg /kg/day in four
divided doses for 2-6 weeks
(If weight > 20 kg,dose of steroid 60mg/day for 3 weeks then
50mg/day for one week then 40 mg/day for next week, then reduce dose 5mg per
week
If weight <20 kg,dose of steroid 40mg/day for 2 weeks then reduce
by 5 mg/week)
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38. Management of rheumatic fever
• Arthritis-
Aspirin therapy for 2 weeks then
gradually tapering over 2-3 weeks
• Treatment of CHF-
(1) Complete bed rest, oxygen
(2) Prednisone for severe carditis of recent
onset
(3) Digoxin or furosemide if indicated
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39. Arthritis
alone
Mild
carditis
Moderate
carditis
Severe
carditis
Bed rest 1-2 weeks 3-4 weeks 4-6 weeks As long as
congestive
heart failure
present
Indoor
Ambulatio
1-2 weeks 3-4 weeks 4-6 weeks 2-4 months
Prednisone 0 0 0 2-6 weeks
aspirin 1-2 weeks 3-4 weeks 6-8 weeks 2-4 months
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40. Management of rheumatic fever
• Management of chorea-
Usually self limiting
Reduce physical and mental stress
Anti inflammatory agents are not needed in
patient with isolated chorea
For severe cases: Phenobarbitone,
haloperidol
Plasma exchange
IVIG
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41. PROGNOSIS OF RHEUMATIC FEVER
• Presence or absence of permanent cardiac
damage
• Cardiac status at the start of treatment
• Recurrence of rheumatic fever
• Regression of heart disease
41

42. RHEUMATIC HEART DISEASE
• Results from single or repeated attacks of RF
• Rigidity and deformity of valves resulting in
stenosis or incompetence or both
• Mitral valve alone in 50%
• Mitral + Aortic in 25%
• Pure aortic uncommon
• History of RF obtained in 60%
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43. Treatment for patients following
rheumatic heart disease (RHD)
• Preventive and prophylactic therapy is
indicated after rheumatic fever and acute
rheumatic heart disease to prevent further
damage to valves
• Patients with rheumatic heart disease and
valve damage require a single dose of
antibiotics 1 hour before surgical and dental
procedures to help prevent bacterial
endocarditis
• Patients who had rheumatic fever without
valve damage do not need endocarditis
prophylaxis
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44. Surgical Care
 Indication:
Heart failure persists
or worsens after
aggressive medical
therapy for acute RHD,
surgery to decrease valve
insufficiency may be life-
saving
 40% of patients with
acute rheumatic heart
disease subsequently
develop mitral stenosis as
adults
 Procedures:
Mitral valvulotomy
Percutaneous balloon
valvuloplasty
Mitral valve
replacement
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45. THANKS FOR UR
PATIENCE
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