PATHOPHYSIOLOGY A.

Ideal Group A Hemolytic Streptococcal Infection

Cross reacting antibody formation

Binding to connective and heart tissue

Inflammation damage

Involvement of joints/ skin/ nervous system

Cardiac involvement

Endocarditis

Myocarditis

Faulty mitral and aortic semilunar valve function

Aschoff bodies

Pulmonary and Right heart involvement

Myocardial dysfunction

Chronic heart failure

Acute rheumatic fever can develop only as a sequel to pharyngeal infection by group A -hemolytic streptococcus. Streptococcal skin infections do not progress to acute rheumatic fever, although both skin and pharyngeal infections can cause acute glomerulonephritis. This is because the strains of the microorganism that affect the skin do not have the same antigenic molecules in their cell membranes as those that cause pharyngitis and, therefore, do not elicit the same kind of immune response. Acute rheumatic fever affects the heart, joints, central nervous system, and skin through an abnormal humoral and cellmediated immune response to group A streptococcal cell membrane antigens called M proteins. These antigens can bind to receptors on heart, muscle, and brain cells and have an affinity for membrane receptors within synovial joints, where they trigger an autoimmune response. Diffuse, proliferative, and exudative inflammatory lesions develop in the connective tissues, especially in the heart, joints and skin. The inflammation may subside before the treatment leaving behind damage to the heart, valves and increasing the individuals susceptibility to recurrent acute rheumatic fever after any subsequent streptococcal infections. Repeated attacks of acute rheumatic fever cause chronic proliferative changes in the previously mentioned organs as a result of scarring, granulomas, and thromboses. Approximately 10% of individuals with rheumatic fever develop rheumatic heart disease. It begins as carditis, or inflammation of the heart, called rheumatic heart disease. Rheumatic heart disease (RHD) continues to be a common health problem in the developing world, causing morbidity and mortality among both

children and adults. Even mild cases of rheumatic fever can cause carditis in all three layers of the heart wall. The primary lesion usually involves the endocardium, which lines the heart chambers and includes the heart valves. Endocardial inflammation causes of swelling of the valve leaflets, with secondary erosion along the lines of leaflet contact. Small, beadlike clumps of vegetation containing platelets and fibrin are deposited on eroded valvular tissue and on the chordate tendineae cordis. These lesions can become progressively adherent. Scarring and shortening of the involved structures occur over time. The valves lose their elasticity, and the leaflets may adhere to each other. If inflammation penetrates the myocardium, localized fibrin deposits develop that are surrounded by areas of necrosis. These fibrinoid necrotic deposits are called Aschoff bodies. Pericardial inflammation is usually characterized by serofibrinous effusion within the pericardial cavity.

Cardiomegaly and left heart failure may occur during episodes of untreated acute or recurrent rheumatic fever. Conduction defects and atrial fibrillation often are associated with rheumatic heart disease.