Rheumatic Fever

N C Joshi
Rheumatic Fever - Presentation
After a latent period of 1-5 weeks, the clinical manifestations of rheumatic fever becomes manifest. Certain manifestations have been designated as major manifestations & include carditis, arthritis, chorea, subcutaneous nodules & erythema marginatum. Other nonpathognomic signs & symptoms are called minor manifestations. The laboratory signs are included in this category.

It has always been the first & most important element to be considered in establishing the diagnosis of rheumatic fever since it may result in the only significant sequelae of the disease. Carditis is the rheumatic inflammatory process, which involves the endocardium, myocardium &pericardium. The incidence of carditis in rheumatic fever in developing parts of the world has been reported to be much higher varying from 64% to 80% compared to West (40-51%).

The confirmation of carditis during initial attack of rheumatic fever solely depends upon auscultatory recognition of mitral and/or aortic valvar incompetence.

The pansystolic murmur of high frequency [because of high systolic pressure gradient between Left ventricle (LV) & Left atrium (LA) more than 100 mm Hg] begins with first heart sound & as pressure gradient persists after aortic closure, murmur is detected beyond 2 nd heart sound. The murmur is heard at apex &extends towards axilla because it is the LV which transmits vibratory activity generated by the turbulent regurgitant flow to the chest wall. The murmur occasionally can propagate to the sternal border due to predominant involvement of posterior leaflet. This classical description may not be present if murmur is soft & then is better detected in left lateral position during full expiration.

In addition, often apical mid diastolic murmur is heard, due to additional volume of blood in left atrium contributed by regurgitant flow across mitral valve. This murmur originally was described by Dr. Carry Coombs of Bristol in 1924. This murmur is only heard in the presence of mitral regurgitation & is low pitched as turbulence is caused by the increased flow without a pressure gradient. The mid diastolic murmur as an isolated murmur would never be present as an isolated finding with the initial attack of rheumatic carditis but may be an earliest manifestation of mitral stenosis.

Rarely a basal diastolic murmur of aortic regurgitation can be encountered in first attack of acute rheumatic fever.

Myocarditis: Myocarditis in the absence of valvulitis is never rheumatic in origin. Myocardial involvement in acute rheumatic carditis unequivocally does exist but it is not significant from clinical stand point of view except for its probable role in mitral annulus dilation and it may lead to congestive heart failure. There is no way to prove presence of myocarditis except cardiomegaly on X-ray chest. Even myocardial biopsy has not shown to confirm the presence of myocardial involvement.

As with myocarditis in acute rheumatic fever, pericarditis is never encountered in the absence of valvar involvement. The precordial pain of pericarditis automatically directs attention to heart but from practical standpoint it is not hemodynamically significant and never causes cardiac tamponade.

The onset of carditis in majority of children older than 6 years of age is rather abrupt and in 76% of the cases occurs during first week. In children younger than 6 years the onset of carditis is often insidious and it may take several weeks until an unequivocal diagnosis can be made. During this period children are chronically ill with low grade fever and pain in joints. The incidence and severity of carditis in this group is often greater.

Polyarthritis has always been the "Achilles heel" of Jones criteria. The list of disorders which can mimic rheumatic polyarthritis includes juvenile rheumatoid arthritis, post viral arthritis, and systemic lupus. At the onset of polyarthritis, a given diagnosis is difficult to establish.

Rheumatic arthritis can be present with wide range of severity and duration. The pain can be so severe that patient may refuse to walk and will scream with pain when hardly touched, even by bed clothing. On the other hand, pain may be so mild that it is often not recalled a week later. The pain can last less than 24 hours or recur off and on for weeks.

Rheumatic arthritis is a migrating polyarthritis and involvement of single joint is extremely unusual. It usually involves larger joints- knees, ankles, elbow and wrist-joint. Characteristically the pain far exceeds the objective findings which may well be the reason Dr. Jones originally chose polyarthralgia rather than polyarthritis as a major manifestation. The 1955 modification which Dr. Jones personally endorsed has required only minimal objective findings to establish the presence of arthritis. Limitation of voluntary motion and tenderness to touch are acceptable and both are usually present. Though Feinstain described rheumatic joints as typically red, hot or swollen, this is not usually seen. Redness is uncommon and when present with rheumatic arthritis- usually only a small area of mild erythema is encountered. Any joint that is markedly inflamed and swollen should be considered septic. If this is associated with pericarditis the first consideration should be that it is infectious mandating immediate confirmation and intervention. If patient fails to respond to Aspirin on a dosage of 100 mg/kg within 48 hours, one can exclude rheumatic arthritis.

There is a lower incidence of carditis in patients with classical sever migratory polyarthritis as compared to those who have milder joint manifestation.

Arthralgia is an extremely common complaint in all patients with rheumatic fever especially during recurrence and is probably neglected because of its nonspecific nature. Nevertheless it does at times precede severe carditis and deserves better recognition.

Chorea (Sydenham chorea)
This is a late manifestation of acute rheumatic fever whose incidence has apparently shown a decline. Earlier reports showed an incidence of 52% as against 15-20% in the more recent surveys. This is of interest since in areas where rheumatic fever is still a common affliction, the incidence of chorea is equal to west where the disease has shown a decline.

The latent period of chorea varies from 1 to 6 months. It is more common in females and is characterised by non-repetitive, purposeless involuntary movement often associated with muscle weakness signs of incoordination, Nervous milking grip, positive pronator sign, hanging knee jerk, alternating contraction & relaxation of pupils and emotional instability. It may be associated with carditis but laboratory signs of rheumatic activity have usually subsided.

Erythema marginatum and subcutaneous nodules
Although erythema marginatum has been traditionally regarded as major manifestation of acute rheumatic fever - its validity is indeed questionable because of the following facts
It is an uncommon finding. It cannot be correlated with other signs of rheumatic activity. It is nonspecific and can occur with drug reactions, glomerulonephritis and sometimes without apparent reason. It is however often associated with carditis and in such instance it serves to confirm the diagnosis of rheumatic fever. If seen as an isolated finding it can by no means serve as an evidence of acute rheumatic fever. It is an irregular circinate evanescent red rash with normal central stain without itch.

Subcutaneous nodules are late and relatively uncommon manifestations of an active rheumatic fever and are almost always associated with severe carditis. These appear on wrist, elbow, knees, ankles and skull.

The inclusion of these two signs as major manifestations of acute rheumatic fever independent of other major manifestations are thus somewhat erroneous and it is preferable to designate them as secondary major manifestations of acute rheumatic fever.

Clinical manifestations
Fever: It is usually present during first week to ten days of rheumatic fever and is rarely above 39 0 C. If patient's temperature elevation persistently exceeds 39 0 C, other cause should be sought. In particular, in the presence of rheumatic heart disease superimposed infective endocarditis should be the first consideration.

Arthralgia: Arthralgia is an extremely common complaint in rheumatic fever. It commonly precedes an acute attack of carditis.

Laboratory minor manifestations
Laboratory data which can be helpful in the diagnosis of acute rheumatic fever are of two kinds :
- Evidence of Streptococcal infection
- Tests for the presence of rheumatic activity

The proof of streptococcal infection can be based on:
A positive throat culture for group A beta-hemolytic Streptococcus. Although old literature quotes throat culture to be positive in 25%, with the common use of antibiotics in almost every febrile child this figure is nowadays hardly ever attained.

The most useful and practical test for detecting a preceding streptococcal infection is a rise in antistreptolysin O titre. The rise occurs in a week, reaches a peak in 3-5 weeks and subsides in 2-6 months. It can be detected in about 80% of cases of acute rheumatic fever because it remains elevated far longer than other signs.

It must be understood that a low ASO titre does not exclude the diagnosis of rheumatic fever if other criteria are fulfilled and on the other hand too much reliance on a solely elevated ASO titre can result in unnecessary treatment.

Rheumatic Fever Rheumatic Fever 01/24/2001
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