ACUTE RHEUMATIC FEVER -1–

Rheumatic Fever is an inflammatory disease that follows infection with

Group A Streptococcus.

Actually it is a poorly understood

“ Auto immune Reaction ”

to Group A Streptococcus.

It is a self limited disease that involves the

Joints, Skin and Subcutaneous Tissues, Brain, Serous surfaces and the HEART.

The disease would be of little practical consequences except for the cardiac

valve damage.
 -2–
Incidence

Prevalence of Rheumatic Heart Disease

2 -- 2.5 /1000 population

ICMR - Incidence 5.5 / 1000

Rheumatic Fever following Streptococcal infection

Sporadic - 0.3 %
Epidemic - 3%

Incubation period following Streptococcal infections to Rh. Fever is

10 days - 3wks.
 Etiology -3–

The disorder is a sort of hypersensitivity reaction to Group A beta

hemolytic Streptococcus. The exact etiopathogenesis is not clear.
Even though in 50% of the Rheumatic Fever, there may be a history
of preceding Streptococcal Pharyngitis.

Pathogenesis

A. Rheumatic Fever is mainly found in poverty stricken population.

Poverty, Poor nutrition, Over crowding predisposes it.

B. This disease tends to run in families. There may be a genetic

predisposition to Rh. Fever.

C. H L A – DR Antigen is said to be the marker of Rheumatic Fever .

D. Age : Common in 5- 15yrs

peak Age – 8yr. Rare below 3yrs.
 -4-

D. Climate: Seen mainly in Temperate Sub tropical & Tropical areas.

E. Season : High incidence – Winter months.

F. Previous attacks : An attack of URI gives 0.3% risks of developing

R. Fever. previous history of Rheumatic Fever gives 50% risk of
recurrence.

G. There is an Incubation Period from the onset of streptococcal

Pharyngitis to the onset of Rheumatic Fever.

Average -- 18 days.
PATHOLOGY

A. Non Specific :- Exudative Changes

B. Specific :- Proliferative Changes.

Exduative Changes :- Characterized by Oedema, Swelling, infiltration

seen classically in Joints, serous cavities & blood vessels.

 -5-
Specific Proliferative Changes

These Changes Consist of Scar formation, & production of specific

granulomas & thrombosis.
These are seen in Heart, blood vessels lungs, periarticular spaces, hands &
CNS.

Endocardial Changes:- Verrucous Endocarditis seen on the atrial surface of MV &

Ventricular surface of MV

Valves are swollen & Oedematous and distorted.

Myocardium :- is the site of perivascular round cell infiltration and specific

Aschoff bodies. Seen especially in the left atrium and the ventricles.

Pericardium : Serofibrinous pericarditis varying from “Bread – and - butter”

type to the pre dominantly exudative form.

 CLINICAL FEATURES

There is no single specific clinical manifestation that

establishes the diagnosis of Rheumatic Fever.

There are a number of Selective Clinical findings called as

JONES CRITERIA

that makes the diagnosis of Acute Rheumatic

Fever highly probable.

 - 8-
Poly Arthritis

Most Confusing Diagnostic Errors

Acute Migratory Polyarthritis with fever
Red, Hot, Swollen, Severe tenderness, Movement painful
Major Jts – Knee, Elbow, Ankle, Wrist
Several Jts involved simultaneous

Flitting & Fleeting Jt pain

Jt involvement - in 70% cases
Knees alone - 75%
Small Jts - < 1%
Need not be symmetric

No sequelae in the Joints

Resolves in a week
Never more than 2 – 3 Wks
Chorea & Poly arthritis never seen together – but exceptions possible.
 CARDITIS -9–
Seen in 50% of Rheumatic Fever
Doppler Studies shows up to 90%
It is seen after arthritis.
Within 1 – 2 Wks, not more than that
Pericarditis

Pancarditis Myocarditis

Endocarditis

Carditis is the only residual of Acute Rheumatic Fever that results

in Chronic Changes.
Initially Insufficiency Later Stenosis
M R - 100%
then MR + AR - 20% MS
A R alone rare AS
T R - Very rarely Not
P R - not Seen Seen
 Signs of Pericarditis - 10 –

May appear suddenly

Associated with precordial pain
Pericardial Friction rub
Striking in heart size
Sometimes asymptomatic
It is seen in association with myocarditis & Endocarditis
.
Never seen isolated

Pericardial effusion - rare if present minimal

Massive pericardial effusion is rare as a result of
Rheumatic Pericarditis.
 - 11 –
Signs of Myocarditis

1 Tachycardia Disproportionate to fever.

2 Gallop rhythm.

3 Muffling of heart sounds.

4 Arrhythmias - 1* Block/ 3 * Block / CHB may be seen

5 Congestive Heart Failure

6 Carey Coombs murmur-

Apical Soft Mid diastole murmur.

Signs of Endo carditis - 12 –
Manifested by organic murmurs
Most frequent - M R
Myocarditis
MR - Dilatation of L V
- Dilatation of MV ring
Endocarditis
- Swelling of
- Cellular MV
infiltration cusps
- Long systolic murmur.
- Filling most of systole
- High pitched blowing quality
- At least of grade 2/6 or >
- Best heard at the apex
- Transmitted to the axilla
- Not affected by position
 - 13 –
Two types of mid diastolic murmur possible in acute Rheumatic

Fever with carditis in addition to the established MS

1. In severe MR - flow through MV ed during diastole

a low pitched diastole rumble.

2. Carey - Coombs murmur - soft, short, mid diastolic murmur

heard only at the apex - due to mitral vegetations

3. Established MS - not seen in the acute phase - it take years

up to 20 yr
- seen as early as 2 - 3 yrs.
 - 14 –
AR -
Second most common murmur

Isolated AR rare

MR + AR most often seen

Early blowing diastolic murmur

MR may disappear eventually

But. AR may persist

TR & PR:

TR may be seen rarely

PR not seen
 - 15 –
Chorea - Sydenham Chorea, St. Vitus’ Dance

Quasi purposive, Non repetitive, Involuntary movement,

predominantly affecting the extremities & face

15% - in outbreaks up to 30%

CNS manifestation alone with involvement of Basal ganglia & caudate nucleus

Seen after 3 months or more

- Involuntary purposeless movement.

- Muscular weakness
- Emotional Lability
- More evident - Stress & Awake
- Disappears during Sleep
- All muscle affected,
- Grimace
- young , adolescent Girls
 - 16 –
Chorea - continuing

Signs i. Darting Sign, Bag of worms

Protruded tongue darts in & out of mouth
ii. Pronator sign
iii. Milking sign : Milk maid’s sign
iv. Spoon or Dishing sign
Types Pure Chorea
Hemi Chorea
Lump Chorea, Chorea Mollis
4 Beats of Chorea
Teacher
Mother
Grand mother
Siblings

Resolves in 1 – 2 wks
Severe cases 3 – 4 months up to 2yrs
Very rarely recurs.
Subcutaneous Nodules - 17 –

Now rarely seen

Now seen in Chr. RHD < 5%

Firm Nodules over hard bony

Surfaces – elbow, wrist, shine, knee, ankle, spine, occiput
0.5 – 2 cm in size
Painless
Freely mobile
Skin over it – normal
Several nodules may be seen

Structure Resembles Aschoff Nodule

It may persist for days to month after the onset of Rh. Fever.

It may occur in Rheumatoid Arthritis & S L E.

Erythema Marginatum - 18 –

Rare, but specific , only in 5%

Macular & Pink in color
Non pruritic
Serpiginous
Erythematous border
Normal skin in the centre
One inch diameter
Trunk & Proximal limb
Not on Face
Warmth – accentuates
Transient & Migratory
Blanches with pressure

Seen early in the disease.

MODIFIED JONES CRITERIA - 19 –
Major Manifestations :
1. Carditis
2. Polyarthritis
3. Chorea
4. Erythema Marginatum
5. Subcutaneous Nodules
Minor Manifestations :
Clinical
1. Arthralgia
2. Fever
3. Laboratory Findings, Increased Acute Phase reactants like
ESR
CRP
4. Prolonged PR interval - ECG
PLUS
Evidence of preceding Gp A Streptococcal Infection

Culture - Throat culture

Rapid Antigen
Antibody rise / Elevation
 Minor Manifestations - 20 –

1 Arthralgia: Less specific, Feels discomfort in the joints, but

no physical signs.
2 Fever: No higher than 101 – 102 F.
If more - careful re evaluation

3 E S R & C R P: Almost always elevated. values influenced by -

Salicylate , Steroid, drugs, Anemia & CHF

Returns to normal in the later part of Acute Rh. Fever.

4 P R Interval: Prolonged in > 20%, Non specific.

Seen even in the absence of carditis.
 Evidence of Gp. A Streptococcal Infection - 21 –
- Positive Throat Culture
- History of Scarlet Fever
- Elevated streptococcal antibodies

A S O > 333 U
Anti DNase B
AH
A 2 fold in Antibody titer more important than a single value

2 Major / 1 Major + 2 Minor + Evidence of Streptococcal infection.

---

Exceptions i. Rheumatic Chorea

ii. Late onset carditis
iii. Rheumatic Recurrence
[ 1 Major /Fever, Arthralgia / ed E S R , C R P/
+ Evidence of G A S infection]
 TREATMENT - 22 –

- Supportive treatment + CHF management

- Treatment of Streptococcal infection
- Use of Anti inflammatory Agents
- Prevention - Primary Prophylaxes
Secondary Prophylaxes

i. Supportive Treatment

Bed rest: Arthritis - normal activity as early as possible = 5 – 7 days.

Mild Carditis – At least 10 – 15 days.
Carditis with CHF – Strict bed rest till signs & symptoms of CHF
disappear

No need to wait for the murmur to disappear, ESR & CRP to come
back to normal.

In severe carditis with CHF & Cardiomegaly - Heart size has to

return to normal or is at least stable.
ii. Treatment of Streptococcal Infection - 23 –

12 L . Unit Benzathine Penicillin G single IM

6 L Unit Procaine Penicillin G Daily X 10 Day.
Erythromycin 1gm orally x 10 day.

Prophylaxis against recurrence should be instituted immediately.

12 L. Benzathine Penicillin evey 3Wk.

iii. Anti Inflammatory Agents

Arthritis, Carditis, Chorea

Arthritis: Aspirin 80 – 100mg/ kg/ day

Minimum pd 6wks
Tapered in the last 2 – 4 wks
Rebound – again 4 – 6 wks Rx
Response with in hours .
3 – 4 divided doses
Avoid gastric irritation.
 - 24 –

Carditis: Mild Carditis without CHF - Salicylate alone

Carditis if severe, Cardiomegaly or CHF – steroid + Salicylate.

Prednisone – 2mg/ kg/ day/ Bd 2 – 3 wks sufficient.

Then start tapering Steroid and add Salicylate – 100 mg/ day
in 4 divided dose.
Then continue for 3 – 4 wks.

Chorea: Mild Chorea - Diazepam

Severe - Haloperidol
( Phenobarbitone, Chlorpromazine)

CHF: Diuretics
Digitalis
Supportive Measures
 Prevention - 25 –
Primary Prophylaxis
Treatment of Streptococcal Pharyngitis to prevent a primary
attack of RF.
12 L - Benzathine Peni: IM - Single
6 L - Procaine Peni: IM - OD x 10 day.
Penicillin V - 250mg/day - BD x 10 day.
Erythromycin - 40mg /kg /day - tid / Qid x 10

Secondary Prophylaxis
Prevention of recurrence of RF
12 L – Benzathine P – IM – Every 3wks
Penicillin V - 250 mg Bid
Sulfadiazine – 500mg OD
Erythromycin – 250 mg Bid

Optimal duration of treatment – Uncertain

Safest - To continue lifelong
Alternative – Continue up to 21 years or 5 years – Which ever is late.
 Prognosis - 26 –

75% ARF well after 6wks.

< 5% Symptomatic even after 6month with Chorea &
Intractable Carditis
Do not rebound after 8wks of treatment

Carditis

70% recover without any residual heart disease.

Children with Severe Carditis, CHF & Pericarditis - 70% may have
permanent heart disease

Pts with recurrent RF have greater incidence of

permanent Heart Disease.

Pts with Chorea without initial Carditis may present years

later with Mitral Stenosis
 - 27 –
Acute Rheumatic Fever.

Acute Rheumatic Fever with Carditis.

Chronic Rheumatic Heart Disease

< 3% - Rh activity persist > 6 month.

Recurrence Rheumatic Fever.

Rebound

Mimetic Character.

Rheumatic Chorea.