Acute rheumatic fever

Description:

The Acute Rheumatic Fever (ARF)  – the postinfectious complication of tonsillitis (quinsy) or pharyngitis caused by a β-hemolitic streptococcus of group A, developing in the form of a general inflammatory disease of connecting fabric at predisposed persons, preferential children and teenagers (7-15 years) is more rare – at young people up to 23 years.

Symptoms of Acute rheumatic fever:

Outstanding scientist-pediatrician A.A. Kissel (1939) gave the brilliant description of the main displays of rheumatism, having called them  "an absolute symptom complex of a disease". Treat them:
• polyarthritis;
• damage of heart;
• chorea;
• annulyarny erythema;
• rheumatic small knots.
Polyarthritis, carditis, chorea can proceed separately, but is more often – in various combinations with each other.
Rheumatic polyarthritis – the leading symptom of 2/3 cases of the first attack of ORL.
Main signs:
• acute beginning;
• fever;
• joint pains;
• a swelling (at the expense of a sinoviit and defeat of periartikulyarny fabrics);
• limitation of movements;
• fervescence and erubescence over joints is possible.
Distinctive features:
• involvement in process of large and average joints, most often knee and talocrural, radiocarpal and elbow;
• dissociation between scanty clinical data and sharply expressed subjective symptomatology – the painful affected joint pains is characteristic, especially at the movements;
• symmetry of defeat;
• volatility of a joint syndrome;
• lack of deformations;
• bystry involution of pathological process (especially against the background of antiinflammatory therapy of pain disappear within several days or even hours).
At 10-15% of patients polyarthralgias (unlike arthritis are not followed by restriction of movements, morbidity at a palpation and other symptoms of an inflammation) are noted.
Rheumatic arthritis is most often combined with a rheumatic carditis, however can proceed separately (at 15% of patients).
Rheumatic carditis – the main symptom of ORL (90-95% of cases) which defines weight of a course of a disease and its outcome.
Manifestations of a rheumatic carditis:
• valvulitis;
• myocarditis;
• pericardis.
The rheumatic carditis is always followed by emergence at auscultation of the noise testimonial of involvement in pathological process of valves of heart.
The systolic noise which is reflection of mitral regurgitation – the leading symptom of a rheumatic valvulitis:
• on character – long, blowing;
• happens different intensity, does not depend on a postural change of a body and a phase of breath;
• it is connected with І tone;
• occupies the most part of a systole;
• it is best of all listened in the field of a heart top;
• it is usually carried out to the left axillary area.
The basal protodiastolic noise characteristic of aortal regurgitation can be one of symptoms of an acute rheumatic carditis:
• begins right after ІІ tone;
• on character – high-frequency, blowing, decreasing;
• it is best of all listened along the left edge of a breast after a deep exhalation at the patient's inclination forward.
Myocarditis can be focal or diffusion.
Subjective symptoms:
• increased fatigue;
• heartaches, heartbeat;
• asthma.
Objective symptoms:
• easing of sonority of the I tone (loss of a muscular component);
• systolic noise of functional character on a heart top (as the tone of papillary muscles which are attached to valve shutters is lowered);
• disturbances of a rhythm and conductivity (on an ECG – dysfunction of a sinus and atrial node in the form of tachycardia, bradycardia or a respiratory arrhythmia);
• shift of borders of heart (preferential to the left);
• symptoms of heart failure (the last two symptoms are not characteristic of focal myocarditis).
Quite often (45-75% of cases) at primary rheumatic damage of heart it is possible to find additional III and less often (15-25%) – the IV tones. At the same time the frequency of their identification, as a rule, correlates with weight of a carditis.
Systolic noise at myocarditis usually happens weak or moderate, is better listened in the V point, is more rare – on a pulmonary artery; out of borders of the cordial area, as a rule, it is not carried out. According to FKG-data, for patients with myocarditis the most characteristic is systolic noise of an ovalnostikhayushchy form, sredneamplitudny and mid-frequency, registered immediately behind the I tone, is preferential in the V point and in a pulmonary artery.
The pericardis can be dry or exudative (with a small amount of an exudate). Emergence of a pericardial rub is possible.
The Rheumatic Heart Disease (RHD) forms as a rheumatic carditis outcome. The isolated RPS forms prevail:
• mitral insufficiency (most often);
• insufficiency of the aortal valve;
• mitral stenosis;
• mitral and aortal defect.
The maximum quantity (75%) of cases of RPS is observed within 3 years from an onset of the illness. Frequency of development of RPS after the first attack of ORL makes:
• children have 20-25%;
• teenagers have 33%;
• adults have 39-45%.
The repeated attacks of ORL, as a rule, aggravate expressiveness of valve pathology of heart.
Features of formation of RPS in modern conditions:
• slower rate of emergence;
• moderate degree of manifestation;
• permanent compensation for a row of years.

Hysterical chorea  – typical manifestation of ORL in 6-30% of cases. As a rule, the chorea is combined with other clinical syndromes of ORL (a carditis, polyarthritis), but it can be 5-7% of patients only symptom of a disease. Girls aged from 6 up to 15 years are ill more often.
The disease begins gradually with emergence of unstable mood, astenisation of the child, tearfulness, irritability. Later there are hyperkinesias (twitchings) of muscles of a trunk, extremities, mimic face muscles, a diskoordination of movements, decrease in a muscle tone. The doctor is able to catch even insignificant hyperkinesias if he long holds the child's brushes in the hand.
Hyperkinesias amplify at nervousness, disappear during sleep, more often happen bilateral, is more rare unilateral (hemochorea). Performance of coordination tests is complicated. Having narrowed eyes, the child cannot precisely concern after cultivation of hands an index finger of a tip of a nose and long hold put out tongue (more than 15 c), experiences difficulties at inflation of cheeks and a grin of teeth. Handwriting is broken, the speech becomes muffled, the movements – awkward.
At the expressed chorea form – a positive symptom of "flabby shoulders" (when lifting the patient for armpits the head is deeply hung in shoulders); retraction of epigastric area at a breath is noted, a delay of the return bending of a shin when checking a knee jerk. Patients meet the expressed hypomyotonia.
The small chorea is often accompanied by symptoms of vegetative dystonia (perspiration, a resistant red dermographism).
Against the background of adequate treatment symptoms of a chorea disappear in 1-2 months.
The ring-shaped erythema (annulyarny rash) is observed in 4-17% of cases of ORL at height of the rheumatic attack. Clinically: light pink ring-shaped enanthesis of a trunk or extremities. Never happens on a face, does not tower over skin level, disappears when pressing; there is no itch or other subjective feelings. The ring-shaped erythema usually quickly, within a day, completely disappears.
Rheumatic small knots – roundish dense from several millimeters to 1-2 cm painless hypodermic educations. Are localized at places of an attachment of sinews, over bone surfaces and ledges, in knee, elbow, pyastnofalangovy joints, an occipital bone. Meet in 1-3% of cases, usually during the first attack of ORL. The cycle of involution makes 1-2 months, without the residual phenomena.
Defeat of serous covers. At 5-7% of patients in a debut of ORL the abdominal syndrome connected with involvement of a peritoneum is noted. It is shown by abdominal pains which can be various on the expressiveness and localization. Against the background of antirheumatic treatment bystry involution of symptoms is, as a rule, noted.
Intensive implementation of antibiotics, glucocorticosteroids (GKS), change of virulence of a streptococcus, and also prevention of the repeated attacks of ORL promoted that the course of a disease became more favorable; the rheumatic carditis not always comes to the end with formation of heart disease. At the same time influence of the minimum activity of a disease on progressing of heart disease, aggravation of heart failure, increase in quantity of complications, such as arrhythmias, thromboembolisms, acute coronary insufficiency is proved.

Reasons of Acute rheumatic fever:

Chronological connection between quinsy or pharyngitis caused by a β-hemolitic streptococcus of group A, and development of ORL is established.
It is interesting that for the first time the hypothesis of the streptococcal nature of rheumatism in 1935 was made and proved by the outstanding therapist, the academician N. D. Strazhesko: "Rheumatism is sepsis in a giperergezirovanny organism. At the same time the large role is played by increase in an immune responsiveness. If in development of acute rheumatism the major role is played by a streptococcal infection, then at synchronization of rheumatism the main factor is strengthening of antibodyformation concerning heart covers (a myocardium and an endocardium) that not only leads to injury of heart, but also is the reason of development of complications".
However not all β-hemolitic streptococci of group A infecting a nasopharynx can cause ORL but only "revmatogenny" strains (M1, M3, M5, M18, M24) which have the following properties:
• a tropnost to nasopharynx tissue;
• existence of the big hyaluronic capsule;
• ability to induce synthesis of type-specific antibodies;
• high contageousness;
• existence of large molecules of the M-protein on a surface of strains;
• characteristic genetic structure of the M-protein;
• existence in molecules of the M-protein of the epitopes which are cross reacting with various fabrics of a macroorganism of the owner: myosin, synovia, brain, sarcolemma.
The M-protein has properties of the superantigen inducing effect of an autoimmunity. The acquired antiself response can be strengthened, in turn, by the subsequent infection with the revmatogenny strains of a β-hemolitic streptococcus of group A containing cross and reactive epitopes.

Treatment of Acute rheumatic fever:

Hospitalization with obligatory observance of a bed rest during the first 3 weeks of a disease is shown to all patients with ORL if there is no carditis. In case of existence at sick arthritis, a carditis the bed and semi-bed rest is cancelled only after elimination of clinical signs of activity, heart failure, at decrease in SOE it is less than 25 mm/h also of SRB (–) remaining during 2 weeks.
Appoint a diet with restriction of table salt and carbohydrates, rich with protein (not less than 1 g/kg), vitamins and potassium.
Drug treatment of ORL – complex: consists of causal (antimicrobic), pathogenetic (antiinflammatory) and symptomatic treatment.