Rheumatic fever is an autoimmune disease that can occur weeks after a Group A streptococcal (GAS) infection such as a sore throat. It is characterized by migratory arthritis, heart inflammation (carditis), abnormal movements (Sydenham's chorea), skin nodules, and rash. If not properly treated, it can cause long-term damage to the heart valves known as rheumatic heart disease. Treatment involves antibiotics to treat the initial strep infection, anti-inflammatory drugs like aspirin to reduce symptoms, and ongoing preventative antibiotics to reduce the risk of recurrent episodes and further heart damage.

1. Rheumatic Fever
&
Rheumatic Heart Disease
Rheumatic fever: “Licks the
joints and Bites the heart”
Dr Deep and Dr Bhadra

2. STREPTOCOCCUS
• Pneumonia
• Necrotizing fasciitis
• Rheumatic fever
• Poststreptococcal glomerulonephritis
• Pharyngitis / tonsillitis
• Neonatal meningitis (Group-B)
• PANDAS / Tourette syndrome : Pediatric Autoimmune
Neuropsychiatric Disorders Associated with Streptococcal infections
2/#

3. Streptococcus
Types of Hemolysis

4. May-2015-CSBRP7/29/2019 HM Patel Centre For Medical Care & Education 4

5. •S M types l, 3, 5, 6,18 & 24
• Pharyngitis- produced by GABHS can lead to- acute rheumatic fever ,
rheumatic heart disease & post strept. Glomerulonepritis
• Skin infection- produced by GABHS leads to post streptococcal
glomerulo nephritis only. It will not result in Rh.Fever or carditis
Group A Beta Hemolytic Streptococcus

6. RHD - MATHS
GAS 24 = 2 X 3 X 4 2 X 3 X 4 12
Immune
Reaction
Heart 30 = 2 X 3 X 5 2 X 3 X 5 15 Carditis
Joint 36 = 2 X 3 X 6 2 X 3 X 6 18 Arthritis
Skin 42 = 2 X 3 X 7 2 X 3 X 7 21 Skin lesions

7. RHD - LITERATURE
MEAT (GAS)
APOLITICAL (HRT)
ASYNCHRONOUS
(JNT)
ATYPICAL (SKN)
Removal of Single
“a”
MET
POLITICAL
SYNCHRONOUS
TYPICAL

8. Epidemiology
•Ages 5-15 yrs are most susceptible
•Rare <3 yrs
•Girls>boys
•Common in 3rd world countries
•Environmental factors-- over crowding, poor sanitation,
poverty,
• Incidence more during fall ,winter & early spring

11. RHEUMATIC FEVER (RF)
• It is an acute, immunologically mediated
disease
• Occur a few weeks after group A
Streptococcal pharyngitis
• Multisystemic disorder
• May progress to chronic RHD (Valvular
heart disease)

12. Pathogenesis
“Damage Is Mediated Both By Abs And T-cells”
Streptococal Antigen
M Protein
B Cell
Antibodies
Complement
Activation
T Cell CD4+
Cytokines
M Cells
Activation

13. CF – CLINICAL FEATURES
RF is characterized by:
– Migratory polyarthritis of the large joints
– Pancarditis
– Subcutaneous nodules
– Erythema marginatum
– Sydenham’s chorea
The diagnosis of RF is established by the
“Jones criteria”

15. Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, Carapetis J, Remenyi B, Taubert KA, Bolger
AF, Beerman L, Mayosi BM. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in
the era of Doppler echocardiography: a scientific statement from the American Heart Association.
Circulation. 2015 May 19;131(20):1806-18.

17. Rheumatic Heart Disease:
Sreptococcal pharyngitis / tonsillitis

18. Erythema marginatum

19. Subcutaneous nodules
May-2015-CSBRP
7/29/2019 HM Patel Centre For Medical Care & Education 19

20. Sydenham's chorea: causes loss of muscle control,
leading to awkward gait and distorted hand gestures
20

21. Clinical Features
• Migratory polyarthritis, involving major joints
• Commonly involved joints-knee,ankle,elbow & wrist
• Occur in 80%,involved joints are exquisitely tender
• In children below 5 yrs arthritis usually mild but carditis more prominent
• Arthritis do not progress to chronic disease
1.Arthritis

22. Clinical Features (Contd)
• Manifest as pancarditis(endocarditis, myocarditis and
pericarditis),occur in 40-50% of cases
• Carditis is the only manifestation of rheumatic fever that leaves a
sequelae & permanent damage to the organ
• Valvulitis occur in acute phase
• Chronic phase- fibrosis,calcification & stenosis of heart valves.
2.Carditis

24. Valves affected are: decreasing order
– Mitral
– Aortic
– Tricuspid
– Pulmonary
RHD is virtually the only cause of mitral stenosis
Mnemonic: MAT

25. Clinical Features (Contd)
• Occur in 5-10% of cases
• Mainly in girls of 1-15 yrs age
• May appear even 6 months after the attack of rheumatic fever
• Clinically manifest as-clumsiness, deterioration of
handwriting,emotional lability or grimacing of face
3.Sydenham Chorea

26. Clinical Features (Contd)
• Occur in <5%.
• Unique, transient lesions of 1-2 inches in size
• Pale center with red irregular margin
• More on trunks & limbs & non-itchy
• Worsens with application of heat
• Often associated with chronic carditis
4.Erythema Marginatum

27. Clinical Features (Contd)
• Occur in 10%
• Painless,pea-sized,palpable nodules
• Mainly over extensor surfaces of joints,spine,scapulae & scalp
• Associated with strong seropositivity
• Always associated with severe carditis
5.Subcutaneous nodules

28. Clinical Features (Contd)
• Fever – Low grade
• Arthralgia
• Pallor
• Anorexia
• Loss of weight
Other features (Minor features)

29. Laboratory Findings
• High ESR
• Anemia, leucocytosis
• Elevated C-reactive protien
• ASO titre >200. (Peak value attained at 3
weeks,then comes down to normal by 6 weeks)
• Anti-DNAse B test
• Throat culture-GABHstreptococci

30. Laboratory Findings (Contd)
• ECG- prolonged PR interval
• Echo - valve edema,mitral regurgitation, LA & LV dilatation,pericardial
effusion,decreased contractility

31. Chronic RHD
• Characterized by organization of acute
inflammation and subsequent fibrosis
• Valves show thickening, commissural fusion and
shortening,
• Cordae tendinae shows thickening and shortening
• Mitral valve: MS [Button hole, Fish mouth]

32. HM Patel Centre For Medical Care & Education
Complications
• Cardiac murmurs
• Cardiac hypertrophy and dilation
• Valvular heart disease
• Heart failure
• Arrhythmias (particularly AF in the setting
of mitral stenosis)
• Thromboembolic complications
• Infective endocarditis
7/29/2019 32

33. Sydenham’s Chorea
•
•
•
•
•
•
•
•
•
•
Extrapyramidal disorder:
Fast, clonic involuntary movements (especially face and
limbs)
Muscular hypotonus
Emotional lability
First sign: difficulty walking, talking, writing
Usually a late manifestation, can be months after
infection
May be the only manifestation of ARF
Often associated with carditis
Usually benign and resolves in 2-3 months
But can last for more than 2 years

34. Treatment
• Step I - primary prevention (eradication of streptococci)
• Step II - anti inflammatory treatment (aspirin,steroids)
• Step III- supportive management & management of complications
• Step IV- secondary prevention (prevention of recurrent attacks)

35. STEP I: Primary Prevention of Rheumatic Fever
(Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients Intramuscular Once
27 kg (60 lb)
1 200 000 U for patients >27 kg
or
Penicillin V Children: 250 mg 2-3 times daily Oral 10 d
(phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d
Estolate (maximum 1 g/d)
or
Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)

36. Arthritis only Aspirin 75-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis Prednisolone 2-2.5
mg/kg/day, give as two
divided doses for 2
weeks
Taper over 2 weeks &
while tapering add
Aspirin 75 mg/kg/day
for 2 weeks.
Continue aspirin alone
100 mg/kg/day for
another 4 weeks
Step II: Anti inflammatory treatment
Clinical condition Drugs

37. • Bed rest
• Treatment of congestive cardiac failure: -digitalis,diuretics
• Treatment of chorea: -diazepam or haloperidol
• Rest to joints & supportive splinting
3.Step III: Supportive management &
management of complications

38. STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular
or
Penicillin V 250 mg twice daily Oral
or
Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and
recommended

39. Prognosis
• Rheumatic fever can recur whenever the individual experience new
GABH streptococcal infection,if not on prophylactic medicines
• Good prognosis for older age group & if no carditis during the initial
attack
• Bad prognosis for younger children & those with carditis with valvar
lesions

40. Rheumatic fever:
“Licks the joints and
Bites the heart”