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Clinical Features

The American Heart Association (AHA) has recommended the revised Jones Criteria as a guide for ARF diagnosis. The same have been approved by WHO study group for the diagnosis of initial attack of ARF (Table 1.1). The salient clinical features for the diagnosis of ARF are given in Fig. Revised Jones Criteria are (a) Major Criteria (b) Minor Criteria.

Supporting Evidence of Antecedent GAS Infection such as positive throat culture or rapid streptococcal antigen test. Elevated or rising ASO or other streptococcal antibodies titre.

Major Criteria
 
1)Carditis

It has been shown by prospective studies that Rheumatic heart disease (RHD) is linked to the occurrence of carditis during the first episode of ARF. If the first episode is accompanied with carditis, the recurrences also manifest carditis. Around 40 per cent cases of ARF develop carditis and 66 per cent of ARF patients with carditis develop RHD on follow up. During carditis (which is always pancarditis),Carey-Coombs murmur of acute valvulitis is audible. Since mitral regurgitation is the commonest valvular lesion during ARF, one hears a pansystolic murmur and middiastolic flow murmur at mitral area. Basal early diastolic murmur due to aortic regurgitation may be audible. The pulmonary and tricuspid valves are rarely involved. Pericarditis, pericardial effusion and arrhythmias (1st and 3rd degree heart blocks) are other features of rheumatic carditis.
Clinical feature of Rheumatic Fever
Clinical feature of Rheumatic Fever
(Reproduced with permission from API Text Book of Medicine edited by G.S. Sainani) Pericarditis is diagnosed by characteristic chest pain, pericardial rub, typical ECG changes or presence of pericardial fluid on 2D-Echo. Myocarditis presents as tachycardia, distant heart sounds, heart enlargement or signs of congestive cardiac failure (CCF).

2)Polyarthritis

It is the most common (occurring in 75 per cent cases of ARF) manifestation of ARF. It involves large joints, it is typically fleeting in character shifting from one large joint to another. Knees,ankles, elbows and wrists are the common joints involved. There is synovitis and synovial fluid shows polymorph cells. Joint swelling and pain usually resolves in 4-6 weeks and there is no residual deformity of joints.

3)Chorea
It is found in around 20 per cent cases of ARF and it is a late manifestation occurring even 3 months after GAS pharyngeal infection. Chorea is triggered by emotional disturbances with quasi-purposive involuntary movements involving mostly face and extremities. At times, chorea may be the only manifestation of ARF. Chorea may last for weeks to months.

4)Subcutaneous Nodules
These are found in about 3-6 per cent of cases of ARF. These nodules are typically subcutaneous, firm, painless, freely movable (0.5-2 cm size) and their presence indicates that the patient has carditis. These should be looked on external surfaces of the joints like elbows, knees and spine. These nodules last for about 1 month.

5)Erythema Marginatum

This is a rare manifestation seen in less than 5 per cent of ARF patients. It is erythematous,macular, evanescent, non-pruritic rash with pale centre and serpiginous or rounded borders. The rash occurs mostly on trunk and arms but never on face.

Minor Criteria

These are arthralgia, fever, prolonged PR interval, raised ESR and C-reactive protein levels. In some cases abdominal pain and epistaxis may occur. Other non specific laboratory findings are leucocytosis and anaemia.

Supportive Evidence
One must always look for supportive evidence for antecedent GAS infection in form of positive throat culture, rising ASO titres, and rapid streptococcal antigen tests. Various antibody tests carried out are anti-streptolysin O, anti-deoxyribonuclease B (ADNaseB), anti-nicotinamide adenosine dinucleotidase (ANA Dase),anti-hyaluronidase and anti-streptokinase.

When two serum samples taken at 2-4 weeks intervals show a two-fold rise, antibody tests are considered positive. The ASO titres of > 250 Todd units in adults and > 333 Todd Units in children are considered positive. The ASO titres may take upto 4-6 months to return to normal,hence by the time chorea or carditis develops after ARF, ASO titres may have returned to normal.

In such situation, one may rely on ADNase B levels, as it remains elevated even beyond 6 months after ARF. Diagnosis of ARF is confirmed if 2 major or one major and 2 minor Jones criteria are present along with a supporting evidence of GAS pharyngitis.

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