Acute rheumatic fever is an auto immune disease, triggered by infection with specific strains of Streptococcus pyogenes, i.e. group A Streptococcus (GAS) It affects the various organs like heart, joints, blood vessels , brain and connective tissues

1. ACUTE RHEUMATIC FEVER
Ms. SANDHIYA. R M.Sc
CLINICAL INSTRUCTOR
CARE

2.  DEFINITION Acute rheumatic fever is an auto immune disease, triggered
by infection with specific strains of Streptococcus pyogenes, i.e. group A
Streptococcus (GAS), occurring after a latent period of approximately 3
weeks.
 ETIOLOGY Pharyngitis with Lancefield group A Streptococcus; This
causes attack of rheumatic fever 2-3 wk later in >3% of children.

3.  PATHOGENESIS
• Antigenic mimicry explains the pathology.
• Antigens on bacillary cell wall mimics glycoproteins in cardiac tissues. Hence,
cross reaction/an autoimmune reaction.
• Auto antibodies also cross react with cardiac myosin and myocardial
sarcolemma → myocarditis.
• Antibodies against caudate nucleus → chorea.
Latent period: Period of building up antibody response (3 wk).

4. PATHOPHYSIOLOGY
 Causative agent ( Group A beta hemolytic streptococci)
↓
Untreated sore throat
↓ Antigenic mimicry
Abnormal reaction - Auto immune disease
Rheumatic fever
↓
All layers of the heart & the MV become inflammed/thickened
↓
Vegetation forms
↓
Valvular stenosis & regurgitation
↓
Heart Failure

5.  HISTOLOGY AND PATHOLOGY
The inflammation causes exudative and proliferative reaction, with damage to collagen
fibers and connective tissue ground substance and typical lesion is called fibrinoid
degeneration which produces Aschoff’s bodies, which are paravascular submiliary
granulomas; found mainly in the heart.

6.  Organs Predominantly Affected by Rheumatic Fever
Heart—Pancarditis
1. Extracardiac organs – joints.
2. Subcutaneous tissue—nodules.
3. Brain – chorea.
4. Less frequently – pleura–pleural effusion

8.  Rheumatic Endocarditis
 Heals with fibrous thickening – adhesion of valve cusps
 Commonest valve involved – mitral valve:
 Next aortic (AI then AS)
 Last tricuspid;
 Pulmonary by 15th year of age

9.  Joints - Exudative/proliferative lesion; Capsule and synovium affected.
Healing occurs without much scarring so, no residual deformity.
 Subcutaneous Nodules - Aggregates of Aschoff’s nodules with
predominant fibroblasts.
 Brain - Non-specific lesions in cerebral cortex, cerebellum and basal
ganglion.

11.  DIAGNOSIS
Mainly clinical and is based on revised Jones criteria:
• Evidence of preceding streptococcal infection (GAS) (is required for revised
criteria) with
• Two major criteria or
• One major and two minor criteria
• In India skin lesions and rheumatic nodules are rare.
• Cannot be used to define diagnosis with absolute certainty; only a guide to
determine high risk group

13.  Incidence
 Usually affects children and young adults
 Most common age-between 5-15 years
 Rare under age of 5 and initial attacks are rare after 20 years
 If not prevented 3 or more attacks recur by 20 years
 Incidence is declining in western countries though it is quite prevalent in
India and in other developing countries

14.  Epidemiology
 Over crowding, dampness and poor socioeconomic conditions predispose.
 Clinically carditis and arthritis are only common.
 Rheumatic Polyarthritis
• Acute painful inflammation of multiple large joints
• Exudative then proliferative
• Characteristically migrating or flitting and fleeting
• Clinically shows pain, tenderness, redness and sometimes effusion
• Joints affected are—of limbs (Rarely of spine, temperomandibular joint,
costoclavicular joint)
• Order of frequency—knee, ankle, elbow, wrist (hip and small joints rare)
• No X-ray changes appear even in prolonged arthritis

15.  Acute Rheumatic Carditis
 It is the most important manifestation of rheumatic fever.
 Earliest evidence: Tachycardia disproportionate to degree of fever; CHF in severe
cases.
 Valvular involvement: Occurs in 50% of cases with rheumatic fever
• First evidence is a soft apical systolic murmur due to edema of the valves and or a soft
pericardial rub.
• If disease regresses (murmur) disappears completely
• If disease progresses—it is replaced by a soft low pitched mid diastolic murmur –
“Carey-Coombs murmur”—more specific, said to be due to nodules forming on mitral
valve leaflets; may not progress to mitral stenosis
• Mild pericardial effusion —may occur; no constriction/tamponade
• In fulminant forms — severe aortic and mitral incompetence -severe CHF.

16.  Frequency of Valvular Involvement
 Mitral valve is most frequently involved 80%– either alone or with aortic and tricuspid.
 Aortic valve next 30% – usually with mitral or alone.
 Mitral involvement, perhaps due to greater pressure on left side.
 Next – tricuspid but never alone.
 Initially edema of cusps occur (Soft murmur). In later course fusion of cusps,
destruction, rupture of chordae take place.
 Involvement of myocardium: Clinicaly manifests with tachycardia, cardiac
enlargement, and in ECG as T wave inversion and prolonged PR interval.
 Involvement of pericardium: Pericarditis—serofibrinous pericarditis – clinically
pericardial rub – pericardial effusion has grave prognosis, myocarditis or pericarditis
without valvular involvement is unlikely to be rheumatic fever.
 Conduction tissue involvement – arrhythmias and syncope.

17.  Subcutaneous nodules:
• Significance: It is presence indicates acute activity of rheumatic fever
• Description: Small, painless, >pea size, transient swellings – skin over them
moves freely; appear in crops and disappear; occur in conjunction with
carditis, last for 1-2 weeks
• Location: Extensor tendons of hands and feet, elbow, wrist, ankle, margins
of patellae, over ulnar border of forearm, scalp posterior occipital line, over
scapulae, spines of vertebrae
•Number: Vary from 1 to 200, rare;
 Age :common in children than in adults

18.  Rheumatic chorea or Sydenham’s chorea (Chorea minor, St. Vitus dance)
• This is a delayed manifestation of rheumatic fever
• Occurs about 6 months after streptococcal infection
• Other manifestations may or may not be present with it; may be the 1st
• Commoner in females
• Clinically manifests as – quasi purposive, non-repetitive, sudden jerky involuntary movements involving the
extremities –uni/bilateral
• Often accompanied by muscle weakness, emotional instability
• Exaggerated by excitement, effort, fatigue
• Subsides during sleep
• Can also be associated with movements of lips, and facial grimacing
• Tongue movements are specially characteristic— “ jack in the box tongue” or “lizard tongue”—suddenly
protruded and suddenly shot back into the mouth with lightening speed
• Associated muscle weakness and hypotonia bring about certain signs.
Pronator sign — on putting arms up – forearm is pronated with flexion at the wrist hyperextension at MP
joints.
Milk maid’s grip – grip is waxing and waning – due to inability to maintain tonic contraction.

20.  Types of Chorea
• Chorea mollis chorea associated with severe hypotonia simulating paralysis
• Pure chorea – when no previous rheumatic manifestations are noted.
•Chorea gravidorum: Rheumatic chorea precipitated during pregnancy-
common in primi; does not require termination of pregnancy.

21.  Skin Rashes in Rheumatic Fever
 Erythema Marginatum
1. Incidence: Occurs in 10 to 20% of children with rheumatic fever Morphology of
lesion: have pale center, red serpiginious margins, painless, evanescent Blanches on
pressure
2. Location: Prominent parts of limbs and trunk never on face
3. Spread: Concentrically
Brought on by application of heat or warm bath
Significance–does not indicate rheumatic activity but characteristic of rheumatic fever.
 Erythema Nodosum
1.Dusky red raised papules, over shin of tibia;
2. less specific and less commoner than erythema marginatum .

23. Minor Manifestations of Rheumatic Fever
• Fever, tachycardia
• Arthralgia
• Abdominal pain
• Epistaxis
• Pleurisy
• Pleural effusion
• Rheumatic pneumonitis (rare)
• Long PR interval

24.  INVESTIGATIONS
 1. Evidence of systemic illness.
 2. Evidence of preceding streptococcal infection.
 3. Evidence of carditis.

25.  Evidence of Systemic Illness
Fever, leucocytosis, raised ESR (all non-specific but useful in follow – up)
 Evidence of Preceding Streptococcal Infection
1. Positive culture from throat swab for GrA Streptococcus.
2. Positive test does not differentiate recent infection and chronic carrier.
3. Rapid antigen test-useful for screening but culture is more definite.
4. Antibody test; done when culture and antigen tests are negative. Raised ASO
titer - especially raising titer is of value- levels >200 or 300 Tods unit in children is
diagnostic. ASO titer can be normal in adults and rheumatic chorea.
5. Other antibodies –anti-deoxyribo nucleus antibody >120 todd units,
antihyaluronidase >1:256. (false +ves occur) In 95% raised titer of at least one of
these antibodies indicates strepto infection.
6. Acute phase reactants-creative protein +ve, ESR raised.

26.  Evidence of Carditis
1. X-ray chest-cardiac enlargement, pulmonary congestion
2. ECG: I and II degree AV block, T↓ reduction in QRS voltage, features of
pericarditis
3. Echo: Valve abnormality and cardiac dilatation; very early change –
stretching of anterior mitral chordae

27.  MANAGEMENT
I. Bed rest II. Drug therapy.
 Bed Rest
(1) During acute phase of rheumatic fever.
(2) Until ESR and temperature comes to normal.
(3) In patients with carditis further 2-6 weeks rest.

28.  Drug Therapy
Note: Drugs do not shorten the duration of attack but suppress the inflammation and minimize
the damage.
Drug therapy consists of: (a) Aspirin, (b) steroids, (c) antibiotics
 Salicylates - Aspirin (acetyl salicylic acid).
Effective in symptomatically relieving arthritis
Start with 60-100 mg/kg/day in divided doses (4 or 5 divided doses in children).
In adults upto120 mg/kg/day- maximum 8 gm/day.
• Toxic effects: Nausea, deafness, vomiting, hyperventilation, acidosis.
• Duration: Approximately for a month/ (upto 3 mths in some) or until ESR is normal. But after 2
weeks dose is reduced to 60 mg/kg/day for remaining 4 weeks.

29.  Corticosteroids: Cortisone/ACTH/prednisone
Indications for Steroids
 Severe carditis
 Severe arthritis
 As additive to aspirin, if aspirin alone is not effective.
 STEROIDS Dose: Oral prednisolone 1-2 mg/kg/day in divided doses until ESR is
normal.
 Patients with severe carditis appear to respond more promptly to steroids. In
severe cases 2-3 months of steroid therapy may be needed.
 Some authors advise IV methyl prednisolone in extreme cases.
 For chorea
• Diazepam/haloperidol; neither salicylates nor steroids help.
• And penicillin prophylaxis even if it is isolated chorea.
• Haloperidol dose 0.5-2 mg bid/tds-PO

30.  PREVENTION OF RHEUMATIC HEART DISEASE
 Primary prevention: Prompt treatment of the initial attack.
 Secondary prevention: Prevention of recurrent attacks.

31. Exposure to Group A streptococcal bacteria
 (1 -4 days) ↓ Primordial prevention : Improving housing /hygiene
Group A streptococcal infection
(2-6 weeks) ↓ Primary prevention : Identification & treatment of
streptococcal sore throat
Acute Rheumatic fever
(Recurrences) ↓ Secondary prevention : Regular echocardiography to
(month to years) detect asymptomatic RHD cases , Secondary prophylaxis
with antibiotics ( regular penicillin )
Rheumatic Heart Disease
(month to years) ↓
Rheumatic heart disease morbidity
( CCF, Atrial fibrillation, IE, Stroke)
↓ Clinical Management (HF medication, Surgery, Anticoagulation)
Death