Rheumatic fever with manifestations, lab investigations, diagnosis and treatment

1. Acute Rheumatic
Fever
Shyala Chand
Group 3

2. Introduction
• Acute rheumatic fever (ARF) is a multisystem disease resulting from
an autoimmune reaction to infection with group A streptococcus.
• It is a delayed non-suppurative sequelae to URTI with GABH
streptococci.
• It is a diffuse inflammatory disease of connective tissue, primarily
involving heart, blood vessels, joints, subcutaneous tissue and CNS.

3. Epidemiology
• Common in children age 5- 15 years.
• Rare <3 yrs.
• Girls > boys (esp cardiac manifestations)
• Common in 3rd world countries
• Risk Factors: Poverty, overcrowded living conditions

4. Pathogenesis
• Manifestations occur after latent period of 1- 3 weeks.
• Delayed immune response to infection with group. A beta haemolytic
streptococci.
• Concept of molecular mimicry, whereby an immune response targeted at
streptococcal antigens also recognizes human tissues.
• These cross reactive antibodies bind to epithelial surfaces and initiate T
cell immune complexes.

6. Clinical Manifestation
• There is a latent period of ~3 weeks (1-5 weeks) between the
precipitating group A streptococcal infection and the appearance of
the clinical features of ARF.
• Common Clinical Features:
 Polyarthritis (present in 60-75% of cases)
 Carditis (50-60%)
 Chorea (<2- 30%)
 Erythema marginatum and subcutaneous nodules (<5% of cases)

7. Polyarthritis
• Migratory polyarthritis moving from one joint to another over a period of
hours.
• 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.
• The pain is severe and usually disabling until anti-inflammatory
medication is commenced.

8. • Highly responsive to salicylates and other
nonsteroidal anti-inflammatory drugs (NSAIDs).
• Joint involvement that persists for more than 1 or
2 days after starting salicylates is unlikely to be
due to ARF.
• Arthritis do not progress to chronic disease.

9. Cardiac
• Up to 60% of patients with ARF progress to RHD.
• Manifest as pancarditis (endocarditis, myocarditis and
pericarditis),occur in 40-50% of cases
• Valvular damage is the hallmark of rheumatic carditis.
• Mitral valve is most commonly affected followed by aortic valve.

10. Cont’
• Damage to the pulmonary or tricuspid
valves is usually secondary to increased
pulmonary pressures resulting from left-
sided valvular disease
• Characteristic manifestation of carditis
in previously unaffected individuals is
mitral regurgitation, sometimes
accompanied by aortic regurgitation

11. Cardiac Symptoms
• Signs: new heart murmur, cardiomegaly, CHF, perciardial friction
rub, effusions
• Characteristic murmur or Rheumatic heart disease:
 mitral regurgitation
 Low-pitched mid diastolic flow murmur at the apex (Carey Coombs
murmur)
 Aortic regurgitation
 AV conduction delays

12. Cardiomegaly

13. Prolonged PR interval

14. Sydenham’s Chorea (St. Vitus Dance)
• 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

15. Skin manifestations
• Subcutaneous Nodules: usually associated with severe
carditis and occur several weeks after onset.
• Firm, painless nodules (up to 2cm) found over bony surfaces
and tendons
• Occur near elbows, knees, wrists, achilles tendon, vertebral
joints
• Usually persist for 1-2 weeks

17. Cont’
• Erythema Marginatum:
nonpruritic, painless
erythematous rash on trunk
and/or proximal extremities
• Macular lesions with raised
margins and central clearing
• May last from weeks to months

18. Other Features
• Fever – Low grade
• Arthralgia
• Pallor
• Anorexia
• Loss of weight

19. Differential Diagnosis
• Poststreptococcal reactive arthritis: is non-migratory
• Rheumatoid Arthritis
• SLE
• Infective endocarditis
• Drug reactions
• Serum Sickness

20. Lab investigations

21. Diagnosis
• Rheumatic fever is mainly a clinical diagnosis
• JONES CRITERIA
– Need 2 major criteria or 1 major and 2 minor criteria in the
presence of a prior strep infection to make the diagnosis
– Evidence of prior strep infection with positive throat
culture or antigen test, elevated streptococcal antibody
titer, or history of rheumatic fever/heart disease

23. 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)

24. STEP I 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)

25. Step II: Anti inflammatory
treatment• Arthritis
 NSAIDS - Aspirin
- 75- 100mg/kg/day, QID for 6 weeks
- Attain blood level 20-30mg/dL
• Carditis
 Corticosteroids – Prednisolone
- 2- 2.5mg/kg/day, BID for 2 weeks
- Taper over 2 weeks, add aspirin 75mg/kg/day for 2 weeks
- Continue aspirin alone 100mg/kg/day for another 4 weeks.

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

27. 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

28. Prophylaxis
• WHO GUIDELINES
– At least 5 years of prophylaxis or if child until age
18 if not cardiac involvement
– 10 years prophylaxis or if child until age 25 if has
mild mitral regurgitation
– Lifelong prophylaxis if has severe valve disease

29. 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.

30. Thank you