ACUTE RHEUMATIC FEVER

ACUTE RHEUMATIC FEVER.

An acute systemic immune mediated disease that may develop after an infection with Group A beta- hemolytic Streptococcal infection of the pharynx..

This disease can affect the HEART, JOINTS, SKIN, SUBCUTANEOUS TISSUE, BRAIN, RESPIRATORY SYSTEM, VESSELS, SEROSAL MEMBRANES, TENDONS AND FASCIAL SHEATHS..

Usually affects children and young adults between the ages of 5 -15 years. Peak at the age of 8 years. More common in low socio-economic class. Rare in high-income countries in Western Europe and North America, where the incidence is about 0.5 cases per 100000. endemic in South Asia, Africa and South America. Affects both sexes equally. But more common in Female. More common in temparate zone(cold) More frequent in january-february & october-november..

Two stages of the rheumatic process: *First stage is exudative & non specific – this stage is an example of Arthus Phenomena & consists of oedema,hyperaemea & capillary haemorrhage,leukocytic inflammation & collagen fragmentation. *Later stage is proliferative & specific- this phase is the Aschoff body. This is usually microscopic, but may be just visible to the naked eye. It arises in the adventitia of the blood vessels & is therefore to be paravascular, panarteritis may occur..

The condition is triggered by an immune-mediated delayed response to infection with specific strains of group A streptococci, which have antigens that cross-react with cardiac myosin and sarcolemmal membrane proteins. Antibodies produced against the streptococcal antigens cause inflammation in the endocardium, myocardium and pericardium, as well as the joints and skin. Histologically, fibrinoid degeneration is seen in the collagen of connective tissues. Aschoff nodules are pathognomonic and occur only in the heart. They are composed of multinucleated giant cells surrounded by macrophages and T-lymphocytes, and are not seen until the subacute or chronic phases of rheumatic carditis..

usually presents with fever, anorexia, lethargy and joint pain  2–3 weeks after an episode of streptococcal pharyngitis. although there may be no history of sore throat. Others features include Arthritis rashes, subcutaneous nodules, carditis and neurological changes ..

Rheumatic Fever Diagnostic Criteria Mnemonics | Medical Junction.

Positive throat swab culture Raised ASO titre Raised anti DNase-B Raised other streptococcal antibodies Streptococcal antigens H/O recent scarlet fever.

The diagnosis is based on – Two or more major manifestations, or one major and two or more minor manifestations. along with evidence of preceding streptococcal infection..

This is the commonest major manifestation and occurs early when streptococcal antibody titres are high. An acute painful, asymmetric and migratory inflammation of the large joints typically affects the knees, ankles, elbows and wrists. The joints are involved in quick succession and are usually red, swollen and tender for between a day and 4 weeks..

Rheumatic fever causes a pancarditis involving the endocardium, myocardium and pericardium to varying degrees. Its incidence declines with increasing age, ranging from 90% at 3 years to around 30% in adolescence. It may manifest as breathlessness (due to heart failure or pericardial effusion), palpitations or chest pain (usually due to pericarditis or pancarditis). Other features include tachycardia, cardiac enlargement and new or changed murmurs. A soft systolic murmur due to mitral regurgitation is very common. A soft mid-diastolic murmur (the Carey Coombs murmur) is typically due to valvulitis, with nodules forming on the mitral valve leaflets. Aortic regurgitation occurs in 50% of cases but the tricuspid and pulmonary valves are rarely involved. Pericarditis may cause chest pain, a pericardial friction rub and precordial tenderness. Cardiac failure may be due to myocardial dysfunction or valvular regurgitation. ECG evidence commonly includes ST and T wave changes. Conduction defects, including AV block, sometimes occur and may cause syncope..

Sydenham’s chorea, also known as St.Vitus dance, is a late neurological manifestation that appears at least 3 months after the episode of acute rheumatic fever, when all the other signs may have disappeared. It occurs in up to one-third of cases and is more common in females. Emotional lability may be the first feature and is typically followed by purposeless, involuntary, choreiform movements of the hands, feet or face. Speech may be explosive and halting. Spontaneous recovery usually occurs within a few months. Approximately one-quarter of affected patients will go on to develop chronic rheumatic valve disease..

Erythema marginatum occurs in less than 5% of patients. The lesions start as red macules that fade in the centre but remain red at the edges, and occur mainly on the trunk and proximal extremities but not the face. The resulting red rings or ‘margins’ may coalesce or overlap. Subcutaneous nodules occur in 5%–7% of patients. They are small (0.5–2.0 cm), arm and painless, and are best felt over extensor surfaces of bone or tendons. They typically appear more than 3 weeks after the onset of other manifestations and therefore help to confirm rather than make the diagnosis..

[image]. Erythema marginatum. [image].

• Uncommon except in children • Small (<2cm in diameter) firm & nontender, freely movable. • Attached to fascia, or tendon sheaths over bony prominences • Persist for days or weeks • Are recurrent • Indistinguishable from rheumatoid nodules.

Subcutaneous nodules.

1. Evidence of a systemic illness (Non specific) Leucocytosis, raised ESR and CRP. 2. Evidence of preceding streptococcal infection(Specific) * Throat swab culture: group A β-haemolytic streptococci (also from family members and contacts) * Antistreptolysin O antibodies (ASO titres): rising titres, or levels of > 200 U (adults) or > 300 U (children) 3. Evidence of carditis => Chest X-ray: cardiomegaly; pulmonary congestion => ECG: 1st- and, rarely, second-degree atrioventricular block; features of pericarditis; T-wave inversion; reduction in QRS voltages => Echocardiography: cardiac dilatation and valve abnormalities Blood culture- if febrile.

The aims of treatment are- To suppress the inflammatory response, so as to minimize cardiac damage. To provide symptomatic relief. To eradicate pharyngeal streptococcal infections..

1) Bed rest & general measure- * Complete bed rest * Daily examination-particularly during 1st 2-3wks to see carditis or HF developed or not. #Duration of bed rest- No carditis, only polyarthritis- Bed rest 2 wks & gradual ambulation over 2 wks. Carditis, but no cardiac enlargement- Bed rest 4 wks & gradual ambulation over 4 wks. Carditis with cardiac enlarge ment- Bed rest 6 wks & gradual ambulation over 6 wks. Carditis with HF- Complete bed rest as long as HF present & gradual ambulation over 3 months..

# Ambulation is usually started- once *Fever has subsided *Joint pain controlled *Heart failure controlled.

2) Analgesic & anti inflammatoty treatment- Both salicylates & corticosteroids have dramatic effect on fever & polyarthritis of RF. Salicylates: Aspirin 60 mg/kg/day for children & 100mg/kg/day for adults in 6 divided doses. Initial dose is given for the 1st 2 wks, then reduced to 60 mg/kg/day for another 6 wks. Usual therapy lasts for 6-12 wks til normalization of ESR,CRP & clinical symptoms has subsided. Corticosteroids: prednisolone 1-2 mg/kg/day for the 1st 3 wks, the dose then gradually tapered..

Arthralgia – Paracetamol Only arthritis- salicylates 100 mg/kg/day for 6 divided doses for 2 wks. Then 75 mg/kg/day for 4-6 wks. Arthritis &/or carditis without cardiomegaly- steroid and above. Carditis with cardiomegaly with HF- prednisolone 2 mg/kg/day for 2 wks, taper over 2 wks but overlap with salicylates 75 mg/kg/day, then only salicylates continued for further 6 wks in same dose..

Patients with carditis. Patients with progressive cardiac enlargement. Patients with hyperpyrexia. Failure of salicylates to control symptoms of arthritis despite adequate serum levels (20-25 mg/dl)..

3) Antibiotic(anti-streptococcal) therapy: Inj. Benzyle Penicillin IM single dose- Wt - <30 kg – 0.6 million unit Wt - >30 kg – 1.2 million unit Oral phenoxy methyl penicillin- 250mg 3 times per day for 10 days for child. 500mg 3 times per day for 10 days for adult. If penicillin allergy- Erythromycin 20-40mg/kg/day in 2-4 divided doses(max 1g/day).

4) If heart failure present: Anti failure treatment like Diuretics & digoxin should be used. 5) When carditis complicated by marked valvular regurgitation causes severe haemodynamic compromises- Valve replacement is life saving..

1. Premodial prevention: Taking measures to minimize future hazards to health by influencing factors like environment, economy, society, behavior & culture. 2. Primary prevention: * Treatment of proven or presumed streptococcal pharyngitis(initial attack) 3. Secondary prevention: * Prevention of recurrent attack- Inj. Benzyl penicillin- single dose of IM Injection every 3-4 wks Wt - ≥30 kg – 1.2 million unit Wt - <30 kg – 0.6 million unit.

b) Oral penicillin- 250 mg twice daily. c) Oral Sulphonamide- Wt - ≥30 kg – 1 gm daily Wt - <30 kg – 500 mg daily d) Oral Erythromycin- 250 mg twice daily..

RF without proven carditis- Until 21 years of age or 5 years after the last attack (which one is longer) RF with carditis without valvular heart diasease- Until 25 years of age or 10 years after the last attack (which one is longer) RF with carditis with residual valvular disease- Until 40 years of age or at least 10 years post episode. Lifelong prophylaxis may be required. Lifelong prophylaxis- 1) Severe valve disease. 2) After valve replacement surgery.

Penicillin prophylaxis for RF does not prevent sub-acute infective endocarditis. Routine prophylaxis for patient with Rheumatic valvular disease undergoing dental or other procedures. Antibiotic prophylaxis is recommended only for patient with- * Prosthetic valves. * Previous endocarditis * Certain forms of congenital heart diseases like VSD, Congenital AS. * For heart transplant patient with vasculopathy.

Full recovery Recurrence Chronic carditis, Heart failure Rheumatic valvular disease Mural thrombosis Embolization Infective endocarditis.

Chronic valvular heart disease develops in at least 50% of those affected by Rheumatic fever with carditis..

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