Major Manifestations:

• Role of echocardiography
  
  Echocardiography is the only new diagnostic tool, which significantly contributes in confirming the presence of rheumatic carditis.
  
  Initially echocardiography was used to differentiate the murmur of mitral regurgitation from systolic murmur caused by VSD, obstructive cardiomyopathy and mitral valve prolapse. It is now established that more than leaflet edema, the mitral annular dilation and secondary chordal elongation permits the apical portion of the anterior leaflet to prolapse back into the left atrium resulting in characteristic jet of regurgitant flow that passes over the posterior leaflet striking on posterior left-atrial wall.
  
  Recent experience suggests that silent mitral regurgitation can be demonstrated by doppler evaluation in patients presenting with isolated rheumatic polyarthritis. Silent but pathologic mitral regurgitation that cannot be heard can be differentiated from physiologic if
  
  
  • Regurgitant flow is holosystolic
    
    
  • Regurgitant flow should extend back to left-atrial wall.
    
    
  • Regurgitant flow should have mosaic pattern on color flow.
    
    
  • Regurgitant flow should be confirmed in more than one plane.

There now appears to be enough experience to add echocardiographic demonstration of silent-valve regurgitation as an additional minor manifestation. It is also important to demonstrate this echocardiographic evidence of pathologic regurgitation in patients presenting with polyarthritis to decide about future penicillin prophylaxis.

• Polyarthritis

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.