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Acute rheumatic fever


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Acute rheumatic fever

  1. 1. ACUTE RHEUMATIC FEVER RHEUMATIC FEVER is an inflammatory disease that follows pharyngeal infection by specific strains of group A beta haemolytic streptococci (serotypes 1,3,5,6,18 etc). The major organs and tissues affected are the heart, joints, skin and central nervous system. In the heart, this inflammation tends to cause permanent damage. In the Indian sub-continent, rheumatic fever remains significant health problem and the most important cause of valvular heart disease, whereas in the developed world the incidence of rheumatic fever and therefore rheumatic valvular heart disease has declined progressively to fairly low levels. Contributory factors to this decline have been better socio economic conditions, early access to medical care, use of antibiotics and decreased overcrowding. ETIOLOGY: Following pharyngitis due to group A beta haemolytic streptococci , about 1-3 % of untreated cases develop rheumatic fever 3 to 4 weeks later. The attack rate may be higher in epidemics of streptococcal pharyngitis. The precise pathogenic role of the streptococcus is uncertain but is likely to be an immunological process as evidenced by latency, demonstration of gamma globulins in myocardial sarcolemma and circulating heart reactive anti bodies in cases with rheumatic carditis. It is postulated that an antibody to the carbohydrate call wall cross reacts with protein in valve tissue (antigenic mimicry) and may cause permanent damage to the heart valves. Similar cross reactivity with connective tissue in joints and with caudate nucleus may lead to arthritis and chorea respectively. EPIDEMIOLOGY: In developed countries the prevalence of rheumatic fever is less than one per thousand rising to 1% of school going children in the developing world. Most initial attacks occur between the ages of 5 to 15 years, but about 20% of cases occur in adults. Female to male ratio is 1:1. Contributory factors include overcrowding, poor socioeconomic status – these encourage the spread of streptococcal infection –malnutrition and poor access to health care. Host susceptibility also appears to play a role as there is a high incidence of recurrence of rheumatic fever. Recurrent attacks may occur even in patients in the age group 40-50 years. The disease may tend to occur in families suggesting a genetic predisposition in a few patients.
  2. 2. PATHOLOGY: Pancarditis. All 3 layers of the heart are affected. Pericarditis is usually transient and may produce a fibrinous effusion but cardiac tamponade and constriction are uncommon. Myocarditis is characterised by presence of Aschoff bodies which are perivascular foci of eousinophilic interstitial collagen surrounded by lymphocytes, plasma cells, macrophages and fibroblasts. These persist long after the acute illness has subsided. Endocarditis is marked by inflammation and edema of the valve cusps associated with verrucous nodules which develop along the lines of valve closure. Acutely, this may cause severe valve damage but more commonly the valve function remains little affected during this phase of illness. During healing however, progressive scarring of the valve apparatus with retraction and fusion can occur. If adhesion and fusion are predominant, stenosis of the valve results. If fibrosis and retraction (shrinkage) take place, regurgitation occurs. A combination of the two processes may also occur. Endocarditis most commonly affects the mitral valve – 60 to 70%, aortic valve in about 25% and less commonly the tricuspid valve in less than 10%. The pulmonary valve is rarely affected by rheumatic fever. Non cardiac tissues Arthritis – there is an exudative synovitis but no long term damage to joints results. Subcutaneous nodules – these are granulomatous lesions which disappear following the acute illness. Chorea – the cerebral pathology has not been identified but may involve the caudate nucleus. CLINICAL MANIFESTATIONS: Rheumatic fever is a systemic illness which occurs 2 to 3 weeks after an attack of acute pharyngitis ( ‘sore throat’) . The onset is with fever, anorexia, lethargy, and joint pains. Abdominal pain and epistaxis may sometimes occur. In some children fever and arthralgia may be the only symptoms and maybe trivial enough to be forgotten by the patient and the parents.
  3. 3. Polyarthritis – a migratory polyarthritis is seen in approximately 75% of patients. And is characterised by severe and tenderness in the affected joint. The arthritis is usually asymmetrical involving the large joints of the extremities – the ankle, the knee, the wrist, and elbow. Sometimes the spine, the temporo- mandibular and costo-clavicular are affected. Involvement of the small joints of hands or feet and of the hip is unusual. The arthritis is characteristically ‘migratory’ or ‘flitting’ in which as one joint improves another gets involved. Some may have only arthralgia. The duration of arthritis is less than 4 weeks at each site. And there is no residual disability (Rarely, recurrent attacks of rheumatic fever may result in a permanent disability involving ulnar deviation of the fingers and lateral deviation of the toes. This is termed Jaccoud’s arthritis.) Carditis – this is the most important manifestation of rheumatic fever affecting about 50% of all patients with first attack of rheumatic fever. Pancarditis occurs characterised by : Percarditis with central chest pain - A pericardial rub may be heard . Pericardial effusion may occur. The ECG may show concave upwards ( saddle –shaped) ST segment elevation. Myocarditis - clinical features include breathlessness, tachycardia, cardiac enlargement, soft S1 and presence of S3 and frank evidence of cardiac failure. ECG may show conduction defects (prolonged PR interval or second degree heart block), chest x-ray shows pulmonary congestion. Endocarditis – causes valvulitis which may result in cardiac murmurs. The following murmurs should be looked for: Pansystolic murmur at the apex Mid diastolic murmur at the apex (Carey Coombs murmur) Early diastolic murmur at aortic area or third left interspace parasternally. Chorea (Sydenhams chorea or St. Vitus’dance) – this is a late manifestation of rheumatic fever and may occur up to 1 to 6 months after a n attack of pharyngitis. It may be seen in 10 to 30% of patients with rheumatic fever and is commoner in girls. It may be followed by chronic cardiac disease. The child may show emotional lability, depression, uncharacteristic behaviour,
  4. 4. restlessness and decreased school performance. The chorea may manifest uniaterally or bilaterally and consist of irregular, jerky, non repetitive, brief, semi- purposive movements of the extremities, face or trunk. Facial grimacing may occur. Chorea may last for 3 to 4 months and then subside. Occasionally it may last longer. The inability to sustain a given posture leads to the demonstration of certain signs:  Pronator sign – if the hands are raised above the shoulder level, they tend to assume an attitude of pronation.  Milkmaid’s grip – on squeezing the examiner’s fingers, irregular contractions result in a milking movement.  Jack-in-the-box tongue – rapid protusion and withdrawal of the tongue.  Piano-playing sign - on extending the arms irregular movements of the fingers are observed.  Spooning sign- on extension of the fore arm and hand, hyperextension of the fingers is observed. Subcutaneous nodules – these are small (less than 0.5cms), firm, painless, mobile nodules seen in about 2 to 10% of cases and associated with more severe carditis. They may be found over the extensor tendons of the hands, feet, knees, elbows, spinal column, sub-occipital region and tendo Achilles. They usually subside within 1 to 2 weeks. Erythema marginatum – this is a evanescent, red, macular rash with a serpiginous outline and a clear centre usually seen over the trunk, thighs and the arms. It tends to fade and reappear. It is reported in 10 -20% of cases but observed in less than 5% of patients in our population. Other manifestations- these include fever upto 38degrees C, pleurisy, pleural effusion. Diagnosis of acute rheumatic fever - there is no diagnostic test for rheumatic fever which tends to be over diagnosed in children with fever, arthralgia and soft ejection murmurs, all of which may occur in a viral fever. This can lead to expensive investigations, needless prophylaxis and prolonged follow up. Duckett Jones laid down the criteria for diagnosis of rheumatic fever in 1994. These have been subsequently modified by AHA in 1992 and revised in2001. The modified Jones criteria are:
  5. 5. Major Manifestations Minor Manifestations  Carditis  Polyarthritis  Chorea  Erythema marginatum  Sub cutaneous nodules  Fever  Polyarthralgia (if polyarthritis is not a major criteria)  Raised ESR or C reactive protein  Prolonged PR interval on ECG(if carditis is not a major criteria) Essential Criteria : Supporting evidence of preceding recent streptococcal infection ie positive throat culture or raised anti streptococcal antibody titres – ASO, AHT, and ADN ase B. Diagnosis of rheumatic fever requires :  Two major manifestations plus or essential criteria  One major and two minor manifestations plus essential criteria Jones criteria have a specificity of about 97% and sensitivity of 77%. MANAGEMENT OF ACUTE RHEUMATIC FEVER There is no specific treatment which cures or changes the course of illness. Treatment is aimed at limiting cardiac damage, relieving symptoms and eliminating any residual streptococcal infection.  Bed rest and supportive therapy- bed rest is advised during the acute phase of the illness or during active carditis and is continued till the temperature, leucocytosis, ESR and CRP come down. If carditis is present, bed rest is continued for 2 – 6 weeks after ESR and temperature have returned to normal. In such cases the total period of bed rest may be upto 3 months.  Salicylates – these are highly effective for fever and arthritis. Aspirin is given in a dose of 60 – 70 mg per kg body weight per day in 4-6 divided doses. Toxic effects include head ache, tinnitus, deafness, vomiting, hyperventilation and metabolic acidosis. Where possible, salicylate level should be monitored. Salicylates are continued till ESR and CRP have fallen to normal – usually 4 – 6 weeks – and then tapered gradually to
  6. 6. prevent a rebound. When carditis is present, aspirin may be required for a longer duration.  Corticosteroids – in patients who cannot tolerate salicylates, or in moderate to severe active carditis with congestive heart failure, corticosteroids are recommended. But there is no evidence that they are more beneficial than salicylates nor do they have a major impact on sequelae. Prednisilone is given in dose 1-2 mg per kg body weight daily till ESR is normal and then gradually tailed off. Salicylates are added while tapering steroids to prevent rebound.  Antistreptococcal therapy – this is given to eradicate streptococci and to prevent recurrence (secondary prophylaxis). Inj Benzathine Penicillin – 1.2 million units is administered intramuscularly on diagnosis followed by oral Phenoxymethyl Penicillin 500mg tid for 10 days. Subsequently, inj Benzathine Penicillin is given once in 3 weeks. In patients allergic to Penicillin, Erythromycin may be used.  Chorea – this self limiting condition may respond to Diazepam, oral Haloperidol or Valproate.  Myocarditis with cardiac failure – responds to bed rest with salt restriction, diuretics and ACE inhibitors. PROGNOSIS Acute rheumatic fever subsides within 6 weeks to 3 months. Carditis occurs within 2 weeks of onset of arthritis and is more common in children. There is a 1 to 10% mortality in the acute phase. Recurrent attacks of rheumatic fever can occur.Overall, 50% of patients develop chronic valvular heart disease. SECONDARY PROPHYLAXIS To prevent recurrences, prophylactic antibiotic therapy should be continued for the period indicated below:  Rheumatic fever without carditis - for 5 years or till the age of 21, whichever is later.  Rheumatic fever with carditis but no residual valvular lesion – for 10 years or till the age of 25 years , whichever is later.  Rheumatic fever with carditis and residual valvular lesion – life-long prophylaxis is indicated. However, in most patients, prophylaxis is
  7. 7. discontinued by age of 40years, since the chance of recurrence is low after that age.