Acute Rheumatic
Fever (RF)• It is a delayed sequela of group A beta-hemolytic streptococcal (GAS) pharyngitis as immunological reaction to the infection.
• It is rarely followed skin infection by GAS
• Commonly affect children between 6 and 15 years of age.
• It is the most common acquired heart diseases in all age groups, accounting for about 50% of all cardiovascular diseases.
• Its commonly occurs in overcrowded and poor communities and there might be genetic predisposition for rheumatic fever.
• Acute RF typically develops 2-4 wk after the acute GAS pharyngitis
• Disease characterized by high rate of recurrence after the initial attack.• The clinical features consist of 5 major and 4 minor criteria, which are called revised Jones criteria.
Clinical features:
1. Migratory polyarthritis:
• Is the earliest manifestation of acute RF, occurs in about 75% of all patients.• It typically involve the large joints (knees, wrists, and elbows) while the involvement of spine, small joints of hands and feet or hip is uncommon.
Major criteria:
• The affected joint is hot, red, swollen and extremely tender.
• These manifestations may preceded by sever joint pain (arthralgia).
• The arthritis is non-deforming (completely resolve without residual joint destruction) and have dramatic response to NSAID (salicylates).
• The arthritis is characteristically migratory in nature.
• Monoarticular arthritis is unusual unless the salicylate is prematurely used during the early course of acute RF which stop the progression of migratory arthritis.
2. Carditis:
• It occurs in about 50% of all cases.• It’s either:
• Subclinical carditis (echocardiac evidence of valvulitis but without murmur)
• Or clinical carditis (echocardiac evidence of valvulitis plus new cardiac murmur).
• It pancarditis (inflammation of pericardium, myocardium and endocardium “valvulitis”)
• Presence of myocarditis and\or pericarditis without clinical evidence of endocarditis (Valvular disease) it is almost never rheumatic heart disease.
• The most common rheumatic heart disease is isolated mitral regurgitation or combined aortic and mitral regurgitations.
• Clinical manifestations of carditis:
• Tachycardia• New murmur (aortic or mitral regurgitation)
• Pericarditis
• Cardiomegaly
• Signs of congestive heart failure.
3. Chorea “Sydenham Chorea”:
• Occurs in 10-15% of patients.
• Usually presents as isolated, subtle movement disorders.
• It characterized by emotional liability, incoordination, poor school performance, uncontrollable movements and facial grimacing.
• It exacerbated by stress and disappear with sleep.
• The latent period between the acute GAS infection and chorea usually longer than that for arthritis or carditis and it might be as long as months (so it usually presents as isolated manifestation)
• It rarely lead to permanent neurological sequelae.
• Milkmaid’s grip: irregular contractions and relaxations of fingers muscle while squeezing the examiner’s fingers.
• Spooning and pronation of the hand while the arms extended.
• Warmian darting movement of the tongue on protrusion.
• Examination of hand writing for fine motor movements.
Examination methods of chorea:
• It is rare manifestation of acute RF occurs in about 1% of all cases.
• Consist of nonpruritic erythematous macular rash with pale centers and serpiginous borders.• Occurs on the trunk and extremities (but not on the face)
• It accentuated by local heat application.
4. Erythema Marginatum:
• It’s rare manifestation (<1%) seen predominantly in chronic or recurrent RF.
• They are firm, painless, nonpruritic, mobile nodules found on the extensor surfaces of the large and small joints, scalp and spine.• There’s a correlation between the presence of these nodules and significant rheumatic heart disease.
5. Subcutaneous nodules:
Subcutaneous nodules
Subcutaneous nodules
Subcutaneous nodules
• Arthralgia, only if the arthritis is not used as major criteria.• Fever (38.2-38.9 C).
• Elevated acute phase reactant (ESR, C-reactive protein or leukocytosis).
• Prolonged PR-interval on ECG ( unless the carditis is a major criteria).
Minor criteria:
Evidence of recent group A streptococcal (GAS) infection:
• Scarlet fever.• Positive throat swab culture for GAS ( rarely positive).
• Raised ASO (antistreptolysin O) titer and other anti-streptococcal Abs e.g. anti DNase B antibodies.
• 2 major criteria or 1 major and 2 minor criteria PLUS the evidence of recent GAS infection.
• Recurrent RF diagnosed by presence of 3 minor criteria plus evidence of recent GAS infection.
Diagnosis of Rheumatic fever:
• There are THREE conditions in which the diagnosis of RF done without strict adherent to Jones criteria, these are:
• When the chorea is only the major manifestation of RF.
• When the indolent carditis is the only manifestation.
• In limited number of patients with recurrent RF in high risk population.
• General measures: bed rest and closed monitoring for evidence of carditis.
• Antibiotics therapy: Regardless to the throat swab culture results to eradicate GAS from URT by:• 10 days course of oral penicillin or amoxicillin
• or single I.M. injection of benzathin penicillin G.
Treatment of RF:
• If the patient is allergic to penicillin:
• 10 days course of erythromycin• Or 5 days course of azithromycin or clindamycin.
• Aspirin or corticosteroid should be delayed if arthralgia or atypical arthritis is the only manifestation of RF.
• Acetaminophen (paracetamol) can be used for treatment of pain and fever while patient observed for more definite signs of acute RF.
3. Anti-inflammatory therapy:
• Typical migratory arthritis or those with
• Carditis without cardiomegaly or congestive heart failure.Dose:
• 50-70 mg\kg\day in 4 divided doses orally for 3-5 days
• followed by 50 mg\kg\day in 4 divided doses orally for 3 wk
• then half of the dose for another 2-4 wk.
Oral salicylate (aspirin): indicated in:
Corticosteroid (prednisone):
• indicated in carditis associated with cardiomegaly and\or CHFDose:
• 2mg\kg\day in 4 divided doses orally for 2-3 wk
• Followed by half of the dose for another 2-3 wk
• Then gradual tapering of the dose by 5 mg\day every 2-3 days.
• When prednisone is being tapered, the aspirin at dose of 50 mg\kg\day in 4 divided doses orally should be given for 6 wk to prevent rebound of inflammation.
• Treatment of congestive heart failure:
• Digoxin
• Fluid and salt restriction
• Diuretics
• Oxygen.
• Phenobarbital is the drug of choice.
• Haloperidol or chlorpromazine as alternative therapies.• Anti-inflammatory agents (corticosteroid)for few weeks if the other drugs are ineffective.
5. Treatment of Sydenham chorea:
• Primary prevention: Is prevention of first attack of acute RF by identification and eradication of GAS pharyngitis.
• Secondary prevention: Is prevention of recurrent attacks of RF in patients with previous attack of acute RF by continuous prophylactic antibiotic therapy to prevent any GAS infection of URT.
Prevention of RF:
• This secondary prevention should be started as soon as the diagnosis of acute RF made and immediately after a full course of antibiotic therapy.
Duration of antibiotic prophylaxis :
• CATEGORY• DURATION
• RF without carditis
• 5 yr or until 21 yr of age, whichever longer
• RF with carditis
• without valvular heart disease
• 10 yr or until 21 yr of age, whichever is longer
• RF with carditis and persistent valvular HDz
• For adulthood (40yr), sometimes lifelong prophylaxis
• Single I.M. injection of benzathin penicillin G every 4wk or every 3wk in populations with high incidence of RF.
• Oral penicillin V, 250 mg twice daily.
• Sulfadiazine or sulfasoxazole once daily.
• Macrolides (erythromycin or clarithromycin) or azithromycin for penicillin and sulfonamide-allergic patients.
Regimen of secondary prevention:
• It depend on the degree of permanent cardiac damage.
• Cardiac involvement may resolve completely especially in the 1st attack and followed by prophylactic therapy.• The severity of cardiac involvement worsens with each recurrent RF.
Prognosis of Rheumatic fever:
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