RHEUMATIC FEVER

RF
 INTRODUCTION
In 1930s strong association between
tonsillopharyngitis and scarlet fever epidemics
with acute RF lead to the diagnosis and
management protocol
Leading cause of acquired heart disease in
children/young adults
Now classified as CT/collagen vascular disease
Incidence Developing countries 100/100000
USA/Europe 2/100000
 DEFINITION

RF is a multisystemic inflammatory disease

secondary to delayed sequel to pharyngeal
infection to Group A beta hemolytic
streptococci
 ETIOPATHOGENESIS

Evidence of Group A beta hemolytic

streptococci infection (GAS)
1. Clinical/epidemiological studies show the
close relationship between GAS and RF
2. Increased titres antibodies to Streptococcal
Antigens
3. Both primary and secondary attacks are
prevented by prompt anti-microbial treatment
CONTINUED

Rheumatogenic strains -3,5,18,19,24

Antibodies are produced against cell wall
antigen of GAS and surface proteins [M] to
cross react with cardiac /other tissues
Now RF is considered as autoimmune disease

No direct relationship between RF and GAS

EPIDEMIOLOGY
More common in 5-15 years age group
Pharyngitis epidemics causing RF -3%
Environmental,bacterial and host factors
important
Overcrowding ,poor sanitation,poor
immunity,close contacts in families/colonies
predispose
Sporadic pharyngitis less severe to cause RF
compared to epidemics
 20022003 World Health Organization Criteria
Diagnostic Categories Criteria
Primary episode of rheumatic fevera Two major or one major and two minor manifestations
plus evidence of preceding group A streptococcal
infection

Recurrent attack of rheumatic fever Two major or one major and two minor manifestations
in a patient without established plus evidence of preceding group A streptococcal
rheumatic heart disease infection

Recurrent attack of rheumatic fever Two minor manifestations plus evidence of preceding
in a patient with established group A streptococcal infectionc
rheumatic heart diseaseb

Rheumatic chorea Other major manifestations or evidence of group A

streptococcal infection not required
Insidious onset rheumatic carditisb
Chronic valve lesions of rheumatic heart disease Do not require any other criteria to be
(patients presenting for the first time with pure diagnosed as having rheumatic heart
mitral stenosis or mixed mitral valve disease disease
and/or aortic valve disease)d

Major manifestations Carditis

Polyarthritis

Chorea

Erythema marginatum

Subcutaneous nodules

Minor manifestations Clinical: fever, polyarthralgia

Laboratory: elevated erythrocyte

sedimentation rate or leukocyte counte

Electrocardiogram: prolonged P-R interval

Supporting evidence of a preceding Elevated or rising anti-streptolysin O or other
streptococcal infection within the last streptococcal antibody, or
45 days

A positive throat culture, or

Rapid antigen test for group A streptococcus, or

Recent scarlet fevere

FOOTNOTES
aPatients may present with polyarthritis (or with only polyarthralgia or monoarthritis)
and with several (3 or more) other minor manifestations, together with evidence of
recent group A streptococcal infection. Some of these cases may later turn out to be
rheumatic fever. It is prudent to consider them as cases of "probable rheumatic fever"
(once other diagnoses are excluded) and advise regular secondary prophylaxis. Such
patients require close follow up and regular examination of the heart. This cautious
approach is particularly suitable for patients in vulnerable age groups in high
incidence settings.
bInfective endocarditis should be excluded.

cSome patients with recurrent attacks may not fulfil these criteria.

dCongenital heart disease should be excluded.

e1992 Revised Jones criteria do not include elevated leukocyte count as a laboratory
minor manifestation (but do include elevated C-reactive protein), and do not include
recent scarlet fever as supporting evidence of a recent streptococcal infection.
RHEUMATIC CARDITIS

Most serious/specific manifestation of RF

Affect 41 to 83% of RF patients

Range of involvement asymptomatic to

pregressive CHF/death
Affect all the three structures endocardium
,myocardium and pericardium resulting in
pancarditis
RHEUMATIC CARDITIS CONTD

evidence of Rh. carditis are

Change /appearance of a new murmur

Cardiomegaly in X ray /fluoroscopy

Pericardial friction rub/effusion by ECHO

Signs of CCF

Prolonged PR in ECG
RHEUMATIC ARTHRITIS
Most common /non specific manifestation
Affect >80% of RF patients
Acute non suppurative migratory polyarthritis
Affect bigger joints
Knee/ankle/elbow/wrist/shoulder
Painful ,asymmetric ,transient and migratory
Usually benign self limiting
Usually no permanent sequelae
Inflammatory changes without infection joint fluid
CHOREA (ST VITUS DANCE/SYDENHAMS )
Extra pyramidal disorder characterised by sudden
irregular ,quasipurposive (dance like),non repetitive
involuntary movements
Movements increase in stress/awake ,disappear in
sleep
Present as involuntary movements of
hands/face/limbs with hypotonia ,difficulty in
writing /talking/walking
Delayed manifestations of RF (>3 m)in 80% RF
patients ;Most have benign course /complete
resolution
SUBCUTANEOUS NODULES

Small pea sized ,firm , painless, freely mobile

nodules
They are usually 0.5 to 2.0 long

Seen over the extensor surface of joints Knee

,elbow,wrist and bony prominences ,tendons,
dorsum of foot,occipital region
Seen in 20% of RF patients

Last for a few days

ERYTHEMA MARGINATUM
Erythematous macular rash
Have pale centre irregular shape
Transient migratory non pruritic
Disappear after few days
Highly specific - <5% of RF patients
Varies in size in trunk , abdomen, inner aspect of
arms and thigh not on face
Rash induced by heat( fair skin better seen)
Rash presence suggestive of co-existing carditis
DIAGNOSIS OF RF
Based on jones criteria
evidence of GAS infections +
2 major or 1 major+ 2 minor criteria
evidence of GAS infection
Increased ASLO titre (>330 children, > 250 adults )

+ve throat culture for GAS

Increased ASZ(Streptozyme) & increased AH (Anti

hyaluronidase )
HPE Aschoff s nodule- upto 30-40% of biopsies increased in
LV,IVS, LA appendage
OTHER TESTS

Leukocytosis
Thrombocytosis

Normochromic/Hypochromic anemia

Increased Acute phase reactants CRP,ESR

ECG prolonged PR interval and ST

Chest X ray - Cardiomegaly

TREATMENT OF RF
No specific cure of RF
Chemotherapy Penicillin Drug of choice
Injection 1.2 m units of Benzathine penicillin IM or
Injection Procaine penicillin 6L units IM *10 days
In penicillin allergic patients
Erythromycin 1 g OD *10 days
Azithromycin 500 mg followed by 250 mg od * 4
days
cephalexin/Cefadroxil *10 days
Sulfonamides /TC not recommended
OTHER TESTS

Leukocytosis
Thrombocytosis

Normochromic \Hypochromic anemia

Increased Acute phase reactants CRP ,ESR

ECG Prolonged PR interval ,Sinus tachycardia

ADJUVANT LINE OF TREATMENT
Rheumatic arthritis
Salicylates Aspirin 10 mg/kg QID
(If pain persists with Aspirin RF is questionable )
Rheumatic carditis

- Treatment of CCF
- Prevention of IE

- Steroids for acute carditis

Rheumatic chorea

-Haloperidol 1 mg first ,later 5 mg od/day

-Sodium Valproate 15 -20 mg/day
SECONDARY PREVENTION OF RF
Benzathine penicillin -6 Lac IU for pateints weight
< 27 Kg and 1.2 million IU for patients weighing
>27 kg
Once in 4 weeks or 3 weeks (endemic area)
RF +carditis + residual valve disease -ideally life
long or to 40 years of age ,10 years from last
attack
RF + carditis -10 years or till adulthood
RF 5 years or till 21 years (whichever is longer )
COMPLICATIONS OF RF

CCF
IE

VHD

Chronic arthritis (Jaccouds)

Chorea
IMPORTANT POINTS IN RF
5% RF persist for > 6 months (chronic RF ) and
they develop significant carditis /chorea
All cases of RHD manifest in < 6 months
Steroids in RF pericarditis ,CCF
Jaccouds arthritis Deformities in fingers and
toes
RF licks the joints and bites the heart
Recurrent fresh attacks of RF is common in
endemic areas