Rheumatic Fever Associate professor Masyuta D. I icon

Rheumatic Fever Associate professor Masyuta D. I

НазваRheumatic Fever Associate professor Masyuta D. I
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Rheumatic Fever

  • Associate professor Masyuta D.I.



  • Group A β-hemolytic Streptococcus is the inciting agent leading to the development of acute rheumatic fever, although the exact pathogenetic mechanisms remain unexplained. Certain serotypes of group A streptococci (e.g., M types 1, 3, 5, 6, 18, 24) are more frequently isolated from patients with acute rheumatic fever than are other serotypes. However, because the serotype is unknown at the time of clinical diagnosis of streptococcal pharyngitis, clinicians must assume that all group A streptococci have the capacity to cause rheumatic fever, and of all episodes of streptococcal pharyngitis should be treated accordingly.


  • Rheumatic fever is most frequently observed in the age group most susceptible to group A streptococcal infections, children from 5-15 yr. of age. The increased incidence of group A streptococcal pharyngitis in winter and early spring is associated with an increased number of cases of acute rheumatic fever during these same periods of the year.

  • The major epidemiologic risk factor for development of acute rheumatic fever is group A streptococcal pharyngitis. Group A. streptococcal impetigo does not result in acute rheumatic fever.

  • The attack rate of acute rheumatic fever following group A upper respiratory tract infection is approximately 3% of individuals with untreated or inadequately treated infection.


The group A streptococcus is a complex microorganism producing a large number of somatic and extracellular antigens that evoke brisk immune responses. Two streptococcal antigens are excellent examples of how an abnormal immunologic response might cause the clinical manifestations.

  • First, the group-specific polysaccharide of the group A β-hemolytic streptococcal cell wall is antigenically similar to the glycoprotein found in human and bovine cardiac valves (antigenic mimicry).



  • Antibodies produced against the group A Streptococcus cross react with the myofibrils and the smooth mussels of the vessel walls leading to fibrinoid changes in the heart.

  • In some patients a similar cross reaction occurs with synovial tissue to produce arthritis.

  • In patients with Sydenham chorea, common antibodies to antigens are found in the group A streptococcal cell membrane and the caudate nucleus of brain.


  • Non-specific lesions result from the fibrinoid degeneration of the connective tissue, inflammatory edema, and inflammatory cell infiltration. The classical feature is Ashoff nodules.

  • The inflammatory reaction is of two types, exudative and proliferative. The joint involvement represents the first one and lesion of the heart represent the second.


  • Rheumatic fever affects the three layers of the heart (endocarditis, myocarditis, and pericarditis) leading to pancarditis.

  • Endocarditis mainly affects the mitral and aortic valves. The endocardium, including the valves becomes hyperemic, edematous and infiltrated. Adhesion of cusps due to fibrosis leads to stenosis of valves, and scarring. Similar changes shortening of the chordae tendinae. In case of severe inflammation, the cusps are damaged and insufficiency develops. At this stage, small vegetations are formed which consists of platelet thrombi. These changes continue after the acute stage has subside.

  • In the myocardium there is round cell infiltration, muscle swelling, and fibrinous exudate.

  • Similar lesions may also be found in the walls of blood vessels, subcutaneous tissues (rheumatic nodule) and other connective tissues sites.

Clinical manifestations

  • The clinical manifestations appear 1 to 6 weeks after upper respiratory tract infection. In some cases, history of a preceding streptococcal infection may be not be present.

  • There is no single specific clinical manifestation or specific laboratory test that unequivocally establishes the diagnosis of rheumatic fever. Rather, there are a number of selective clinical findings, called Jones criteria, that make the diagnosis of acute rheumatic fever highly probable. Although the Jones criteria have been changed several times since their original publication, they have remained basically stable and are the accepted method by which the diagnosis of this disease is confirmed.

Criteria in the Jones System for Acute Rheumatic Fever

  • Major Criteria

  • Carditis

  • Polyarthritis migratory

  • Erythema marginatum

  • Chorea

  • Subcutaneous nodules

Two major criteria or one major and two minor criteria plus evidence of a preceding streptococcal infection indicate a high probability of rheumatic fever. In the three special categories listed below, the diagnosis of rheumatic fever is acceptable without two major or one major and two minor criteria. However, only for a and b can the requirement for evidence of a preceding streptococcal infection be ignored.

  • Two major criteria or one major and two minor criteria plus evidence of a preceding streptococcal infection indicate a high probability of rheumatic fever. In the three special categories listed below, the diagnosis of rheumatic fever is acceptable without two major or one major and two minor criteria. However, only for a and b can the requirement for evidence of a preceding streptococcal infection be ignored.

  • a. Chorea, if other causes have been excluded.

  • b. Insidious or late-onset carditis with no other explanation.

  • c. Rheumatic recurrence: In patients with documented rheumatic heart disease or prior rheumatic fever the presence of one major criterion, or of fever, arthralgia, or elevated acute-phase reactants suggests a presumptive diagnosis of recurrence. Evidence of previous streptococcal infection is needed here.


  • This important finding in acute rheumatic fever is a pancarditis that involves the pericardium, epicardium, myocardium, and endocardium.

  • Carditis is the only residual of acute rheumatic fever that results in chronic changes.

  • Tachycardia, cardiac dilatation, feeble apical impulse, muffled first heart sound and conduction defects leading to varying degrees of heart block may occur due to myocarditis.

  • The carditis of rheumatic fever may be mild or very severe, leading to intractable heart failure


  • The electrocardiogram may indicate a Ist-degree heart block (prolonged PR interval), and on rare occasions, 2nd- or 3rd-degree block may also be present. In first attacks, electrocardiograms are otherwise usually unremarkable. There are also prolongation of QT interval and T wave changes.

  • No specific findings are revealed by the common, chest roentgenogram, but cardiomegaly is common, especially in individuals with significant carditis.

  • Some individuals with subclinical evidence of valvular disease may show valvular regurgitation on two-dimensional Doppler echocardiography.

Rheumatic carditis presenting as mitral and aortic insufficiency in a 14-year-old girl with an initial attack of ARF. Frontal view of the chest demonstrating severe cardiomegaly, particularly of the left atrium and left ventricle. There is mild prominence of the central pulmonary vasculature.

  • Rheumatic carditis presenting as mitral and aortic insufficiency in a 14-year-old girl with an initial attack of ARF. Frontal view of the chest demonstrating severe cardiomegaly, particularly of the left atrium and left ventricle. There is mild prominence of the central pulmonary vasculature.


  • Later, in the chronic stage, scarring of the valve with either typical "fishmouth" abnormality or even calcified valve tissue may lead to stenosis. Often there is a combination of insufficiency and stenosis. Once the signs of activity of acute rheumatic fever has subsided but signs of residual valvular damage are present then it is called rheumatic heart disease.

  • Acute rheumatic mitral valvulitis superimposed on chronic rheumatic heart disease. Small vegetations (verrucae) are visible along the line of closure of the mitral valve leaflet (arrows). Previous episodes of rheumatic valvulitis have caused fibrous thickening and fusion of the chordae tendineae.


  • Pericarditis occurs in acute rheumatic fever as a component of pancarditis.

  • The first symptom of pericardial disease is often precordial pain. The major complaint is a sharp, stabbing sensation over the precordium and often the left shoulder and back. The pain may be exaggerated by lying supine and relieved by sitting, especially leaning forward.

  • Cough and dyspnea may also occur.


  • On physical examination, many of the finding relate to the degree of fluid accumulation in the pericardial sac.

  • The presence of a friction rub is a helpful but may be a variable sign in acute pericaditis, becoming apparent only after the effusion is reduced. Pericardial rub is best head over the base of the heart with the patient sitting up and leaning forward with breath held at the end of expiration. It may be heard in both phases of the cardiac cycle. When the effusion is lager, muffled heart sounds may be the only auscultatory finding.


  • Physical examination reveals increased area of cardiac dullness.

  • Narrow pulses,

  • tachycardia,

  • neck vein distention,

  • enlarger liver,

  • edema,

  • and increased pulsus paradoxus

  • suggest significant fluid accumulation.


  • Electrocardiogram shows low voltage waves caused by a damping effect of the pericardial fluid.

  • Pressure on the myocardium by fluid or exudate produces a current of injury that results in mild elevation of ST segments.

  • Generalized T wave inversion occurs as a consequence of associated myocardial inflammation.

  • Echocardiography shows an echo-free zone surrounding the heart.


  • Chest roentgenogram shows an enlarged, rounded, cardiac shadow (like "water bottle") will a narrow base and obliteration of the right cardiophrenic angle. This is characteristically associated in most cases with pulmonary oligemia. It is perhaps the only point which helps in differentiating this condition from myocarditis with heart failure where the lung fields are invariable congested.


  • This is the most confusing of the major criteria and probably leads to more diagnostic errors than any of the other manifestations. Rheumatic arthritis is more common in older children.

  • The arthritis develops acutely (hours to overnight).

  • The arthritis is migratory and affects several different large joints: the elbows, knees, ankles, and wrists. It rarely occurs in the fingers, toes, or spine.

  • The involved joints are red, hot, and swollen.

  • The involved joints are so acutely inflamed, swollen and tender that even a mere touch or the weight of a bed sheet over the joint causes unbearable pain.

  • Movement of the joint is extremely painful.



  • The arthritis does not result in chronic joint disease.

  • After initiating anti-inflammatory therapy, the arthritis may disappear in 12-24 hr.

  • Untreated, it may persist for a week or more.

  • After recovery, there is no residual damage.

  • In many patients with early arthritis of rheumatic fever, because of treatment with anti-inflammatory drugs, the classic migratory polyarthritis does not develop, confusing the diagnosis.

  •   Described by Lasegue in 1884: “Rheumatic fever is a disease that licks the joints but bites the heart.


  • Sydenham's chorea (or St Vitus'dance), a unique part of the rheumatic fever syndrome, occurs much later than other manifestations.

  • Benign manifestation reflects involvement of basal ganglia and caudate nuclei

  • Chorea is more common in prepubertal girls.

  • It is characterized by sudden purposeless non-repetitive involuntary movements of extremities (particularly of the face and limbs), hypotonia and emotional instability with abrupt alterations between laughter and tears.

  • These choreoathetoid movements may begin very subtly. It usually appears 2 to 6 months after an acute streptococcal sore throat.

  • The movements are often very difficult to detect at the onset. However, careful questioning of parents and teachers usually reveals evidence of increased clumsiness. One of the best signs of this in school-aged children is a marked deterioration in their handwriting.


  • These movements cause facial grimacing and lead to inability to write and inability to hold the tongue still when protruded (Filatov’s sign)

  • Sydenham's chorea may affect all four extremities or may be unilateral.

  • Occasionally hypotonia is of such extreme degree that the patient remains absolutely bed ridden.

  • The intensity of the involuntary movements increases during purposeful activities, excitement, and fatigue, and is lessened during sleep.

  • Speech also becomes defective.

  • Association of chorea and carditis is more common.

  • An attack may persist for a few weeks to a few months. Recurrence is not uncommon but eventual recovery is complete with no neurologic sequelae.

Erythema marginatum

  • The unique rash seen in patients with rheumatic fever is another of the major manifestations that can be very difficult to diagnose. It occurs very infrequently.

  • Although early in the disease it may manifest as nonspecific pink macules that are usually seen over the trunk, later in its fully developed form, there is blanching in the middle of the lesions, sometimes with fusing of the borders.

  • This rash can be made worse with application of heat, but characteristically it is evanescent.

  • There is no itching or induration, but a tendency to recurrence.

  • The face is never involved.

  • A, Erythema marginatum. Annular plaques (complete rings) of varying sizes with flat, pale centers and fairly distinct, raised erythematous margins. B, Note the serpiginous borders formed by a coalescence of several partial rings.

Subcutaneous nodules

  • These lesions occur infrequently (seen in 3% to 5% of patients) and are most commonly observed in patients with severe carditis.

  • These pea-sized nodules are firm and nontender, freely movable, and there is no inflammation.

  • They are characteristically seen on the extensor surfaces of the joints, such as the knees and elbows, and also over the spine.

  • The overlying skin is normal.

  • Usually last for a shorter period of time; rarely may persist up to 4 weeks.

Minor Criteria

  • The minor manifestations are much less specific but are necessary to confirm a diagnosis of rheumatic fever. They include the clinical findings of fever and arthralgia.

  • Arthralgia is present if the patient feels discomfort in the joint in the absence of objective findings (e.g., pain, redness, warmth) on physical examination. (Arthralgia cannot be counted in satisfying the Jones criteria if arthritis is present.)

  • Fever, usually no higher than 38.3 or 38.8C, may be present. High fever of 39.4 or 40.0C requires careful re-evaluation and consideration of other diagnoses.

Minor Criteria

  • Included in the minor criteria are several laboratory tests.

  • Acute-phase reactants, such as the ESR or C-reactive protein, may be elevated. However, these test are non-specific and are useful index for monitoring activity and progress of the disease. They may remain elevated for prolonged periods of time (months) and are used by some clinicians as a guideline for modifying doses of anti-inflammatory drugs.

  • Occasionally, nonspecific elevations of serum gamma globulin may be seen.

  • A prolonged PR interval on the electrocardiogram is also included among the minor criteria. This also is a nonspecific finding and should be used only after careful consideration.

Evidence of Group A Streptococcal infection

  • There must be evidence of a preceding group A streptococcal infection documented by

  • a positive throat culture,

  • a history of scarlet fever,

  • or elevated streptococcal antibodies such as antistreptolysin O (ASO), antideoxynribonuclease B (anti-DNase B), or antihyaluronidase (AH). The most commonly used test is the ASO test. The diagnosis of rheumatic fever should not be seriously considered in patients without evidence of a recent group A streptococcal infection


  • Long periods of bed rest are not necessary for most patients. Bed rest is recommended for 2-4 weeks in cases of minimal carditis and several months (may be 3 months) in cases of moderate to severe carditis. Preferable to keep patients at bed rest until the ESR approaches normal and congestive heart failure has been controlled.

  • Salt is restricted if signs of heart failure are observed. The ration must contain elevated quantity of kalium (raisins, fig, dried apricots, potatoes, and curds).

  • Volume of liquids must be 200-300 ml less than diuresis.


  • Management of acute rheumatic fever can be divided into three approaches:

  • treatment of the group A streptococcal infection that led to the disease,

  • use of anti-inflammatory agents to control the clinical manifestations of the disease,

  • and other supportive therapy, including management of congestive heart failure, if that has occurred.


  • All patients presenting with acute rheumatic fever should be treated for a group A streptococcal infection at the time the diagnosis is made, whether or not the organism is initially isolated from the patient.

  • Antibiotics are started after a throat culture has been obtained. A 10 full days of an appropriate oral agent or a single intramuscular injection of benzyl penicillin is recommended.

  • Patients, who are allergic to penicillin, should receive erythromycin for 10 days.

  • Sulfadiazine is not an appropriate agent for treatment of acute streptococcal pharyngitis because a high percentage of organisms are resistant to these antimicrobial agents.


  • Salicylates provide prompt and dramatic relief for the patient with the arthritis of acute rheumatic fever.

  • Early administration of salicylates to a patient suspected of having rheumatic fever before the diagnosis is established with certainty may obscure the diagnosis by interrupting the development of migratory arthritis. Therefore, salicylates or other anti-inflammatory agents should be withheld until the clinical course of the disease has adequately defined itself.

  • Salicylates are given until the signs of activity have subsided (6-9 weeks).

  • Dose is decreased gradually to avoid the rebound after stoppage of the drug.


  • For patients with mild carditis without evidence of congestive heart failure, salicylates alone are indicated. However, in patients with congestive heart failure or other significant manifestations of carditis, corticosteroids are required.

  • Administration of steroids should be limited both in amount and in duration to reduce their untoward side effects. For most children, a total dose of 2.5 mg/kg/24 hr of prednisone divided into two doses is appropriate. A short course of steroids over 2 wk is usually sufficient. As ESR falls to normal, prednisone is gradually withdrawn to minimize rebound phenomena.

  • Even with short courses of steroids in these doses, side effects may occur, including some cushingoid changes and hypertension.


  • Congestive heart failure should be treated by conventional techniques. Diuretics are indicated in patients with severe congestive heart failure. Cardiac glycosides such as digitalis also may be used.

  • The treatment of Sydenham's chorea has been controversial. Patients with chorea should remain in a quite atmosphere. Originally, Phenobarbital or other sedatives were used. Diazepam is prescribed for patients with mild chorea. In patients with severe chorea, haloperidol has been used successfully.

  • There is no specific therapy for erythema marginatum or the subcutaneous nodules of acute rheumatic fever.


  • Prevention and treatment of group A streptococcal infection can prevent rheumatic fever. There are two forms of prevention for acute rheumatic fever:

  • primary prophylaxis

  • and secondary prophylaxis.

Primary prophylaxis

  • Primary prophylaxis refers to antibiotic treatment of the streptococcal upper respiratory tract infection to prevent an initial attack of rheumatic fever. Appropriate diagnosis and adequate antibiotic therapy with eradication of group A streptococci from the upper respiratory tract reduce the risk of developing rheumatic fever to near zero.

  • Antibiotic therapy initiated up to approximately 1 wk after onset of the streptococcal sore throat can prevent rheumatic fever. However, antibiotic therapy must be adequate. Ten full days of oral therapy are essential if the oral method is used.

Secondary prophylaxis

  • Secondary prophylaxis refers to the prevention of colonization or infection of the upper respiratory tract with group A β-hemolytic streptococci in people who have already had a previous attack of acute rheumatic fever. Patients who receive antibiotics continuously and do not have group A streptococcal infections do not have recurrences of rheumatic fever.

  • The recommended methods of secondary prevention include regular monthly (every 3-4 wk) injections of intramuscular bicillin-5, or daily oral administration of erythromycin (for individuals who cannot take penicillin).

Secondary prophylaxis

  • The necessary duration of secondary prophylaxis in individuals with a documented history of rheumatic fever or with rheumatic heart disease is controversial.

  • Some clinicians think that patients without carditis may need secondary prophylaxis for 5 yr. after the most recent attack or when they reach their 18th birthday, whichever comes first.

  • Others recommend that, in patients who have significant rheumatic heart disease or who have a significant risk of contracting group A streptococcal upper respiratory tract infection (e.g., medical professionals, school teachers, those living in crowded conditions), the duration of secondary prophylaxis should be longer.

  • Some recommend that treatment be continued for life in patients with rheumatic valvular heart disease. Recommendations for each patient must be individualized, depending on the patient's condition and the environment in which he or she lives and works.


  • Of the five major clinical manifestations of ARF, all eventually disappear with or without treatment leaving no sequelae, with the important exception of carditis.

  • Up to 70% of patients with ARF who develop carditis during the initial episode recover with no residual valvular disease.

  • Between 50% and 100% of patients with two or more recurrences of ARF with carditis develop chronic rheumatic heart disease. This correlates with the severity of the carditis.  

  • Patients with chorea may present years later with mitral stenosis.


  • Acute attacks last an average of 3 months. A general sense of well being, no malaise or fever, no joint pain, gradual weight gain, return of appetite, improvement of pallor, return of sleeping pulse rate to normal are all clinical evidences pointing to the subsidence of rheumatic activity.

  • The clinical evidences may be corroborated by the estimation of E.S.R. and C-reactive protein, both of which should return to normal levels.

  • Recurrence may be precipitated by further streptococcal infections, pregnancy, or use of the Pill.


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