RHEUMATISM (ACUTE RHEUMATIC FEVER)

Rheumatism (Sokolsky-Bouillaud's disease) - systemic inflammatory disease of

connective tissue with a primary lesion of the cardiovascular system which develops
in predisposed him at a young age (7-15 years) after infection by the ji-haemolytic
streptococcus group A. In the English literature rheumatism often referred to as acute
rheumatic fever.

The incidence of rheumatism in the average is 5 per 100 OOO population, but in
developing countries, according to WHO, it is significantly higher (2.2 per 1000
children). In Russia the incidence of rheumatic fever in the average 0.3 per 1000 child
population. Primary morbidity of children in Moscow is quite low (0.03 in 1000).

Etiology

Acute rheumatic fever develops within 2-4 weeks after previous infection caused by
the p-haemolytic streptococcus group A. We have identified a number of
streptococcus Ar and toxins, in particular the M-cell wall proteins (virulence factors),
streptolysin S and O, streptokinase and gialuro-nidazu. Confirmation of exposure to
the streptococcus organism of rheumatic patients is the detection in most patients
protivostreptokokkovyh AT - antistreptolizina-O antistreptogialuronidazy,
antistreptokinazy, antidezoksiribonukleazy in capable of damaging various tissues and
cells of the body.

In the occurrence of rheumatism important role played by genetic predisposition of

the patient. Rheumatism ill only 0,3-1% of children who have had a streptococcal
infection. At the "family" nature of rheumatism in the early XX century Russia
entered a well-known pediatrician, AA Kissel. The value of the role of genetic factors
in the development of rheumatic fever is confirmed by more frequent its development
in siblings and a higher incidence among monozygotic than among heterozygous
twins. Ar D-8, D-17 B-lymphocytes detected in 98% of patients with carditis and
arthritis, and 75% of patients with rheumatoid trochee genesis.

Pathogenesis

The development of rheumatic fever is determined by several mechanisms. A role can

play a direct toxic damage of components of myocardium cardiotropic enzymes (i-
haemolytic streptococcus group A. However, a leading value add features of cellular
and humoral immune responses to various Ag streptococcus, leading to the synthesis
of protivostreptokokkovyh AT, cross-reacts with Ag infarction (the phenomenon of
molecular mimicry) as well as its cytoplasmic Ag-corona tissue localized in the
subthalamic area and the basal ganglia of the brain. In addition, M protein has the
properties of "superantigens", that is able to induce activation of T-and B-
lymphocytes, without prior processing of Ar - presenting cells and interaction with
molecules of class II major histocompatibility complex.

Pathomorphology

For rheumatism is characterized by preferential damage of connective tissue. Classically divided

into four stages of pathological process in rheumatic disease: Muko-idnoe swelling, fibrinoidnye
changes, proliferative reaction and sclerosis. In the stage mucoid swelling may reverse the
development process. Proliferative stage is characterized by the formation of rheumatic
granuloma composed of large basophilic cells gistiotsitarnogo origin of giant polynuclear cells,
as well as from lymphoid, plasma and mast cells. Typical rheumatic granulomas reveal only in
the heart (now quite rare). In the pathological process involved also microvasculature vessels,
serous membranes, joints and nervous system. The basis of the defeat of the nervous system is
you-rheumatic Kulit, while chorea - the defeat of cells of the subcortical nuclei. Changes in the
skin and subcutaneous tissue are also due to vasculitis and focal inflammatory infiltration.

Clinical picture

Rheumatic fever usually occurs in children of school age and much less frequently in
preschool children. The disease is characterized by polymorphism of symptoms, the
most important of which are polyarthritis, carditis and the defeat of the subcortical
nuclei of the brain. Lesions of the internal organs in the form of rheumatic pneumonia,
nephritis, abdominal syndrome (rheumatic peritonitis) is now practically not observed.

Rheumatoid polyarthritis. Rheumatoid polyarthritis occurs, according to different

authors, in 40-60% of cases. It is characterized by acute onset on the background of a
low rise in body temperature, pain and swelling mainly of large, sometimes medium-
sized joints, volatility and the rapid regression of the process. Rheumatoid arthritis can
be attributed to the group of reactive arthritis that have arisen as a result of infection.

Rheumatic carditis. The defeat of the heart (rheumatic heart disease) is a leader in
the clinical picture of disease and determine its course and prognosis. In 70-85% of
cases the disease occurs primary rheumatic heart disease. In Rheumatic heart disease
can attack all the shell of the heart - myocardium, endocardium and pericardium. The
most widely considered to be the defeat of the myocardium - diffuse
myocarditis. However, in the early stages of the disease to distinguish myocarditis and
endocarditis clinically is often very difficult, it requires a comprehensive clinical and
instrumental examination.

Typically, rheumatic heart disease patients do not complain. Parents have noticed that
after 2-3 weeks recovering from a sore throat in a child persist lethargy, fatigue,
subfebrile. During this period, there are clinical signs of rheumatic heart disease in the
form of tachycardia, less often bradycardia, enlargement of the heart, the muffled
tones of the heart. Auscultation listen systolic sound. When PCG exhibit reduced
amplitude distortion, broadening and depletion of high-frequency oscillations
predominantly tone I. The ECG may identify various arrhythmias, pacemaker
migration, slowing atriovent-rikulyarnoy conductivity (sometimes up to
atrioventricular dissociation).

Considerable difficulties is the clinical diagnosis of valvular lesions in the early stages
of the disease, which is of great prognostic value. Important role in the diagnosis is
echocardiography. Most commonly affects the mitral valve. Thus on
echocardiography reveal thickening and "shaggy" echo of the valve leaflets and
chords, limiting the mobility of the back of his sash. When radiography in children
with a lesion of the mitral valve detected "mitral" configuration of the heart,
enlargement of the left chambers. Any damage to the aortic valve at echocardiography
identify melkoamplitudnoe diastolic flutter of his wings. X-rays visible aortic
configuration of the heart with a predominant increase in the left ventricle.

The outcome of rheumatic heart disease with the duration of the acute period of 1,5 up
to 2 months depending on the formation of heart disease (20-25%). The most common
forms of mitral valve insufficiency, rarely - aortic valve insufficiency, mitral-aortic
defect, mitral stenosis.

Relapsing Rheumatic heart disease usually develops on the background of acquired

heart disease. Clinically, it usually manifests intensification of pre-existing or new
noise, the development of circulatory failure.

Chorea. This form of rheumatic disease occurs in 7-10% of cases, mainly girls of
school age. The main symptoms caused by lesion of the subcortical nuclei of the
brain.Characteristic emotional disorders (tearfulness, irritability, mood instability), are
attached to motor disturbances accompanied by decreasing muscle tone. Hyperkinesis
(promiscuity, nekoordi-niruemye, violent movements of individual muscle groups)
lead to inaudibility speech, change in handwriting slovenly eating, and sometimes the
impossibility of self-service. Hyperkinesis increase with excitement, are more often
bilateral. Calling the knee-jerk reaction, it is possible to identify a symptom Gordon
(tonic reduction quadriceps). Hypotonia of muscles impedes normal life. In these
patients, is a positive symptom "flabby shoulders": when lifting a patient standing for
armpits from behind his head deeply immersed in the shoulders. Can complete patient
immobility ( "soft" chorea). Flow chorea is often protracted and recurrent
nature. Typically, the active phase lasts up to 2 months.
Ring-shaped erythema. Ring-shaped erythema - a rash in the form of pale-pink rings
on the skin of the chest and abdomen. The rash is not accompanied by itching, does
not rise above the surface of the skin, rapidly disappearing without pigmentation and
desquamation.

Rheumatic nodules. Rheumatic nodules - rounded dense formations of up to 0,5-1

cm, defined in the place of attachment of tendons, in the occipital region. Currently,
babies are extremely rare.

Classification

The generally accepted at present is the classification and nomenclature of

rheumatism AI Nesterova (Table 13-1). The classification is based on the phase of
illness, clinical and anatomical organ damage, the nature of the disease and the state
of circulation.

Diagnosis

Criteria for diagnosing rheumatic fever developed AA Kissel (1940), Jones (1944),
complemented the AI Nesterov (1963). The main manifestations

1. Cardia.

2. Polyarthritis.

3. Chorea.

4. Subcutaneous nodules.

5. Ring-shaped erythema.

6. "Rheumatoid" history (contact with the transferred epipharungeal streptococcal

infection, the presence of cases of rheumatism in the family).

7. Proof ex juvantibus - improving the patient's condition after the 2-3-week course of
specific therapy.

Additional manifestations of A. General.

1. Increased body temperature.

2. Adynamia, fatigue, weakness.

3. Pale skin.

4. Sweating.

5. Nosebleeds.

6. Abdominal syndrome.

B. Special (laboratory findings). 1. Leukocytosis (neutrophil).

Table 13-1. Working classification and nomenclature of rheumatism

Phase and the degree of activity of rheumatism

Clinical and anatomic characteristics of lesions

Heart

Other organs and systems

Nature of flow

Circulatory failure

Active

The degree of activity I, II, III

Rheumatic heart disease without primary valvular Rheumatic heart disease with relapsing (how) Rheumatic fever without obvious changes in the heart

Polyarthritis

Serozity (pleurisy, peritonitis, abdominal syndrome)

Chorea, encephalitis, meningo-encephalitis, cerebral vasculitis, neuro-psychiatric disorders

Vasculitis, nephritis, hepatitis, pneumonia, skin lesions, iritis, iridocyclitis, thyroiditis

Acute
H.
Subacute
H,
Protracted
H 11A
Continuously
H 11E
recurrent
Latent

Inactive

Miokardioskleroz rheumatic heart disease (a)

Implications and residual effects of deferred exocardial lesions

th N

(K

about

about

Table 13-2. Criteria diagnosis rheumatism *

More criterion Minor criteria

Cardio Clinical
Polyarthritis Previous rheumatic fever or rheumatic
Chorea disease
Ring-shaped erythema Arthralgia
Subcutaneous rheumatic nodules Fever
Laboratory indicators of acute phase
ESR
C-reactive protein
Leukocytosis

Daiiye confirming streptococcal infection

Increased titer protivostreptokokkovyh AT, antistreptolizina-O, seeding throat group A

streptococcus, newly transferred angina

* From: Nasonova VA et al. Clinical Rheumatology. - M., 1989

The presence of the patient's two large or one large and two small criteria indicates a
high probability of acute rheumatic fever, especially if confirmed by data on past
infection caused by group A streptococci
Differential Diagnosis

Rheumatoid polyarthritis differentiate from reactive arthritis, the debut of Jura and
juvenile spondylitis, SLE, hemorrhagic Vasco-cast. Rheumatic heart disease should be
distinguished from non-rheumatic carditis, PMK, UPU, infective endocarditis. Horeyu
differentiate the functional tics, hyperkinesis in SLE, thyrotoxicosis, tumors of the
brain.

Treatment

Complex method of treatment of primary rheumatism includes the simultaneous

appointment of small doses (0,5-0,7 mg / kg / day) of glucocorticoids and NSAIDs.

2. Dysproteinemia: increased ESR, fibrinosis, the appearance of C-reactive protein,

increased the concentration of ss 2 and y-globulin, increased concentration of serum
mukoproteinov.

3. Changes serological indicators: the emergence of Ar streptococci in the blood,

increasing titers antistreptolizina-O, antistreptokinazy, Al-tistreptogialuronidazy.

4. Increased permeability of capillaries.

Currently, the most common criteria of WHO (1989), developed by the American
Association of rheumatological (Table 13-2).

prednisolone at a dose of 15-25 mg / day, with 1/2-1/3 of the daily dose prescribed in
the morning. Initial dose is gradually reduced until the complete abolition of an
average of 1.5 months.

Of the NSAIDs most frequently prescribed indomethacin and diclofenac. NSAIDs

combined with glucocorticoids and one of the basic drugs, especially with a protracted
course of disease and the formation of heart disease.

-- Indomethacin: 2-3 mg / kg / day in 2-3 doses for 1 -1, 5 months.

-- Diclofenac: 2-3 mg / kg / day in 2-3 doses for 1-1, 5 months.

As a basic therapy using quinoline derivatives:

-- chloroquine (hingaminom, do Lago) in a dose of 0,06-0,25 g depending on the age

of 1 times per day after dinner, the duration of treatment for several months to several
years;
-- hydroxychloroquine (Plaquenil) in a dose of 0,05-0,2 g depending on the age of 1
times per day after dinner, the duration of treatment for several months to several
years.

Given the nature of streptococcal rheumatism, during the first 10-14 days of therapy
prescribed benzylpenicillin or its analogs on 0,75-1 million U / day. In the complex
therapy also includes the reorganization of foci of chronic infection, particularly
chronic tonsillitis. With decompensated chronic tonsillitis tonsillectomy is needed. In
6-8 months after the acute period recommended spa treatment.

Prevention

Recommendations of the WHO (1989) Prevention of rheumatism and its recurrence

include the following activities.

1. Primary prevention - activities that ensure the proper development of the child:

Quenching with the first months of life;

good nutrition with adequate vitamins;

rational physical education and sport;

control of infection caused by group A streptococcal (sore throat, scarlet fever),

including prescriptions penicillin dose 0,75-1,5 million U / day for 10-14
days.Recommended preparation - dentists (smallpox).

2. Secondary prevention is aimed at preventing relapse and pro-disease

progresses. The most optimal year-round prevention is carried out every month for at
least 5 years.All children who have suffered rheumatism, appoint:

Benzathine benzylpenicillin + benzylpenicillin procaine (bitsillin-5) in a dose of 1.5

million ED 1 time in 4 weeks to school age children;

bitsillin-5 in a dose of 0.75 million ED 1 time in 2 weeks to patients of preschool

age.

Forecast

Weather in recent years has improved thanks to the measures of primary and
secondary prevention. The primary rheumatic heart disease leads to the formation of
heart defects only at 20-25% of patients. Rarely have cases of severe course of
rheumatism. Mortality rate decreased from 11.12 to 0,4-0,1%.