RHEUMATIC FEVER

By Walter Uronu
MSc.Echocardiography
 DEFINITION


Rheumatic fever - is an autoimmunological mediated inflammatory
disorder which occurs due to delay response to /untreated Group A
Beta Hemolytic streptococcal pharyngeal infection in the tonsilo-
phargngeal area.

Complications of Group A Beta Hemolytic Streptococci (GABHS)
includes;

Supparatives - peritonsillar abscess, sinusitis, otitis media

Non- supparatives- ARF, Scarlet fever, Acute Glomerulonephritis.

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 EPIDEMOLOGY


Occurs most at age of 5-15yrs but also possible till 30yrs

16-25% of CVD pts have RF.

Prognosis is worse for females than for males.

More commonly in poor socio-economic strata of the society in
living in damp and overcrowded place.

India it’s range is 5-7/1000.

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 RISK FACTORS


Age- 5-15yrs

Untreated streptococcal infection

Overcrowding areas

Poverty, Poor hygiene

Lack of access to medical care

Family predisposition

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 CAUSES


Oral cavity disease eg; Gum disease

Weakened immune system

Certain dental procedures.

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 PATHOGENESIS


Elements of the classic triad of agent, host and environment
All play major roles in pathogenesis of RF

The agent responsible, GABHS has >100 subtypes defined by
M-protein surface molecules

Specific M –protein subtypes appear to be rheumatogenic,
Typically mucoid, strains that adhere well to pharyngeal
tissue

The antiphagocytic properties of M- protein allow persistence
of bacteria in tissues for up to 2 weeks, until specific
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Autoimmune response: molecular
mimicry

M-protein, N-acetylglucosamine, and several other epitopes
mimic
myocardium(myosin and tropomyosin),
heart valves (laminin),
synovia (vimentin),
skin (keratin) and
subthalamic and caudate nuclei in the brain (lysogangliosides)

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 GABHS pharyngitis

Activation of innate immune system

-Leads to GABHS antigens to be presented on B and T lymphocytes

Production of Antibodies against GABHS antigens (IgM &IgG)

Cross reactive of antibodies with Human tissues (heart, brain, joint etc)

Due to molecular mimicry (same amino acid sequence/chain btn human

Tissue & GABHS )

Immunological mediated inflammation & damage of human tissue

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 DIAGNOSIS


Acute rheumatic fever (ARF) occurs after GABHS pharyngitis

The postsuppurative pharyngitis phase is frequently followed
by migratory polyarthritis and carditis, the most common
manifestations of ARF, typically within 2 to 3 weeks after
infection

In contrast, Sydenham chorea is less frequent and occurs 1 to
6 month later

The cutaneous manifestations, erythema marginatum, and
subcutaneous nodules are far less common, typically do not
occur in isolation 9
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 MODIFIED JONES CRITERIA

(A). MAJOR CRITERIA (B). MINOR CRITERIA


Carditis 
Fever > 38.5 degree celcius

Arthritis 
Arthralgia without arthritis

Sydenham’s Chorea 
Elevated ESR and CRP

 For diagnosis; Nodules
Subcutaneous 
Prolonged PR-interval in ECG
2
 major + essential criteria
Erythema marginatum 
Previous Hx. of RHD or RF
1 major + 2minor criteria + essential criteria

Essential criteria +ve blood culture of GABHS (evidence of
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 Carditis


Early manifestation, as pancarditis affecting the endocardium,
myocardium, and pericardium simultaneously.
a. Cardiac involvement ranges from an asymptomatic
presentation to progressive congestive heart failure and
death.
b. The most typical manifestations include increased heart rate,
rhythm disturbances,new murmurs or pericardial friction
rub, cardiomegaly, and heart failure.
c. Heart failure is rare in the acute phase; if present, it is usually
the result of myocarditis.
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 
Typically manifests as valvulitis, detected by presence of
mitral regurgitation(MR) or less commonly AR on auscultation

Acute or chronic myocardial dysfunction, acute pericardial
disease

Early manifestation, within 2 weeks of RF

Approximately 40 to 60 % of RF episodes with carditis result
in RHD

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 Clinical findings

Pericarditis is evidenced by presence of a pericardial rub

Myocarditis by tachycardia, soft S1, presence of S3, and CCF

Endocarditis by the presence of Carey-comb’s murmur (Mitral
diastolic murmur)

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 Arthritis

Most frequent symptoms of RF
Flitting and fleeting type of polyarthritis
occurring in up to 75% of patients with acute symptoms.

Typically very painful, migratory, and limited to the major
joints of the arms and legs. It involves large joints, such as
the knees, ankles,elbows, wrists, and shoulders.

Earliest manifestation, 2 to 3 weeks after onset of RF

The arthritis is self limited
Symptoms varying from, minor arthralgia to severe arthritis
with erythema, warmth and swelling
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02/06/2024 16
 Jacod’s arthritis

Ulnar deviation of 4th and 5th finger with flexion at
metacarpophalangeal joints is the only residual deformity
seen in rheumatic polyarthritis

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 Subcutaneous nodules

These usually measure 0.5 to 2 cm and are firm,painless, and
freely mobile nodules that can be isolated or found in
clusters over the extensor surfaces of joints (knees, elbows,
and wrists), bony prominences,tendons, dorsum of foot,
occipital region, and cervical processes.

Late manifestation, Occurs 3 to 6 weeks after onset of RF

Patients who have subcutaneous nodules almost always have
carditis
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02/06/2024 19
 Erythema Marginatum

< 5% and Evanescent

This is an evanescent erythematous macular rash with a pale
center of irregular shape. It is usually nonpruritic and tends
to disappear after a few days

Pink Macular lesions with an erythematous rim .

The lesions vary in size and affect mainly the trunk, abdomen,
and inner aspect of arms and thighs, but not the face.

Worsen with heat application.

Often associated with chronic Carditis
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 Sydenham’s Chorea

Neurological manifestation of RF

Late manifestation ,2 to 30% of RF (3 month after onset)

Also known as Saint Vitus’ dance or chorea minor, this
extrapyramidal disorder is characterized by purposeless
and involuntary movements of face and limbs, muscular
hypotonia, and emotional lability.

Exacerbated by stress and disappears with sleep.

Clinical maneuvers to elicit chorea;
-Demonstrate milkmaid’s grip,Handwriting examination.

More common in females
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 CLINICAL INVESTIGATION


Lab Test and Diagnostic Test

Elevated ASO titers,serum (antistreptococcal antibody test).

Biopsies – for Aschoff’s nodules a form of granulomatous
inflammation, can be seen in the proliferative stage and are
considered pathognomonic for rheumatic carditis. Such
nodules are most often found in the interventricular septum,
the wall of the left ventricle, or the left atrial appendage.

CBC test for Elevation of ESR, CRP, Hb count(Anemia), Platelet
count (thrombocytosis)and WBC (Leukocytosis).
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 
Chest radiography - may identify increased cardiac size,
increased pulmonary vasculature, or pulmonary edema.

Echocardiography - show mitral regurgitation or aortic
insufficiency. Calcifications of the leaflets and subvalvular
apparatus are present in the chronic, not acute, phase of
rheumatic heart disease.

ECG - presence of PR prolongation and sinus tachycardia,
- Myocarditis may prolong the QT interval
- Pericarditis, low-voltage QRS complexes and ST-segment
changes in the precordial leads can be observed.
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 TREATMENT


ANTIBIOTIC THERAPY
Oral penicillin 500mg BD x 10days
Tab Erythromycin 250mg BD x 10days (in case ofpenicillin allergy)

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 TREATMENT


Arthritis and Arthralgia: Salicyclates or NSAIDS eg; aspirin 80-
100mg/kg/day in 4-5 divided doses x 3-5wks

Severe carditis: corticosteroids (prednisolone 1-2mg/kg/day max
60mg x 4-6wks

Sydenham’s Chorea ;
-Haloperidol -0.5mg/kg/day
-Carbamazepine or sodium valproate – 15-20mg/kg/day x 1-2wks

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
It is generally recommended that patients with suspected RF
be admitted for close observation and workup.
A. Carditis
1. Secondary prophylaxis with penicillin has been shown to
reduce not only streptococcal infections but recurrent attacks
of acute RF as well.

Patients with mild carditis should receive secondary
prophylaxis for 10 years after the most recent attack or at
least until the age of 25 years, whichever is longer.

More severe valvular damage necessitates lifelong secondary
prophylaxis
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 2. Congestive heart failure should be managed with standard
therapy
3.Steroids: if symptoms of RF and/or carditis persist despite
adequate aspirin therapy (corticosteroids)
4.A gradual reduction in steroid dosing is necessary to avoid
relapses. If symptoms are mild, they usually subside without
specific treatment. For severe symptoms, treatment with
salicylates should be tried before restarting corticosteroids.
5. Corticosteroids, despite relieving symptoms or carditis, do
not prevent valvular damage.
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 PREVENTION

Primary prevention. The most important step in the
management of RF is the eradication of GAS infection,
However, no treatment can eradicate GAS completely in all
patients because of high colonization rates.
1. Early therapy
2. Penicillin is the agent of choice primarily for its narrow
spectrum of activity,long-standing proven efficacy, and low
cost
a. single intramuscular dose of penicillin G benzathine.-
intramuscular- 10-day course of oral therapy b.The oral
antibiotic of choice is penicillin V (phenoxymethylpenicillin)
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 Drug Dosage Route Duration

Primary prevention
Benzathine (penicillin 600,000 U (≤ 27 kg) IM Once
G) 1.2 million U (≥ 27 kg)
or
Penicillin V (children) 250 mg (2–3 times/d) Oral 10 d

Penicillin V 500 mg (2–3 times/d) Oral 10 d

(adolescents
and adults)
Penicillin-allergic
patients
Erythromycin ethyl 40 mg/kg/d (2–4 Oral 10 d
succinate times/d
up to 1 g/d)

Erythromycin estolate 20–40 mg/kg/d (2–4 Oral 10 d

times/d up to 1 g/d)
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 Duration of Therapy for Secondary Prevention of Rheumatic
Fever

Disease state Duration of therapy

RF + carditis + residual valvular disease At least 10 y postepisode and at least until

the age of 40 y. Lifelong prophylaxis may
be required
RF + carditis without valvular disease 10 y or beyond adulthood, whichever is
longer

RF without carditis 5 y or until the age of 21 y, whichever is

longer

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REFERENCE

Dorothy R Morlow, Pediatric text book 6th edition

Black JM Hawks, Medical surgical nursing 8th edition
Suzanne C , Brenda G , Medical surgical nursing 2005
Wood S L, Cardiac Nursing

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 THANK YOU

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