Rheumatic Fever

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Acute rheumatic fever is still an important disease of children in certain developing countries.
Rheumatic fever is an immune disorder that follows an infection in children, usually a streptococcal tonsillitis or pharyngitis.
Certain strains of streptococci, especially group A -haemolytic streptococci, produce particular antigens to which antibodies are developed by certain susceptible individuals; these antibodies may cross-react with host cardiac antigens.
The disease occurs mainly in children between the ages of 5 and 15 years, and was once prevalent in the UK, Europe and USA.
It is now rare outside certain developing countries with low socioeconomic standards. The disease is a systemic disorder which, in the acute phase, presents with fever, malaise and, sometimes, synovitis and
polyserositis. However, the most important target organ is the heart.

Patients develop characteristic lesions (Aschoff's nodules).

Histology of the Aschoff's nodule.
The Aschoff's nodule is composed of an area of degenerate collagen, surrounded by activated histiocytic cells and lymphoid cells. These lesions stimulate fibroblast proliferation and lead to scarring.
Rheumatic fever causes a pancarditis in the acute phase
The components of the pancarditis are:

Rheumatic pericarditis. Aschoff's nodules form in the pericardium, associated with an acute pericarditis. The acute inflammatory exudate is often predominantly of the serous type (mainly fluid with comparatively little fibrin or neutrophil components). The serous exudate can produce a pericardial effusion, which may distend the pericardial cavity.

Rheumatic myocarditis. Aschoff's nodules developing in the myocardium are associated with interstitial oedema and mild inflammation, sometimes with muscle-fibre necrosis. The myocarditis is usually clinically mild, but may produce left ventricular failure.

Rheumatic endocarditis. Aschoff's nodules may form anywhere in the endocardium, producing slight
irregularity of the endocardial surface. However, Aschoff's nodules in the valves lead to greater
irregularity, and there may be erosion of the overlying endocardium, particularly at the points at which the valves contact each other at the line of closure.
In these sites, small aggregations of fibrin and platelets accumulate to form small vegetations. The aortic and mitral valves are most prone to develop severe lesions, probably because of the higher pressures to which they are exposed and the more vigorous and traumatic valve closure.
In the acute phase of rheumatic fever the greatest dangers to the patient are the pericarditis and myocarditis; however, the main morbidity of rheumatic fever is the long-term effects of the immune
damage causing chronic scarring of valves.
Numerous small vegetations are present on the mitral valve.
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