Rheumatism

Rheumatism is a systemic inflammatory disease of connective tissue with a predominant heart attack. Children and young people are mostly ill: women are 3 times more likely than men.

Etiology, pathogenesis. The main etiological factor in acute forms of the disease is beta-hemolytic group A streptococcus. In patients with prolonged and continuously recurring forms of rheumatic heart disease, the association of the disease with streptococcus often fails to be established. In such cases, heart damage, which completely corresponds to all the main criteria of rheumatism a, apparently has a different nature - allergic (out of communication with streptococcus or in general infectious antigens), infectious-toxic, viral.

In the development of rheumatism, essential importance is attached to immune disorders, although specific stages of pathogenesis have not been elucidated. It is suggested that sensitizers (streptococcus, virus, nonspecific antigens, etc.) can lead in the first stages to immune inflammation in the heart, and then to a change in the antigenic properties of its components, converting them into autoantigens and the development of an autoimmune process. An important role in the development of rheumatism is played by genetic predisposition. Systemic inflammatory process in rheumatism is morphologically manifested by characteristic phase changes in connective tissue (mucoid swelling - fibrinoid alteration - fibrinoid necrosis) and cellular reactions (infiltration by lymphocytes and plasmocytes, formation of Ashot-Talalaev granulomas). Since the stage of fibrinoid changes, complete tissue repair is impossible, and the process is completed by sclerosing.

Clinical picture. In typical cases, the disease develops 1 to 3 weeks after the angina or less often another infection. With repeated attacks, this period may be less. In some patients, even primary rheumatism occurs 1 to 2 days after cooling without any connection with the infection. Relapses of the disease often develop after any intercurrent diseases, surgical interventions, physical overload.

The most characteristic manifestation of rheumatism, its "basic syndrome" is the combination of acute migratory and fully reversible polyarthritis of large joints with a mild carditis. Usually the onset of the disease is acute, violent, less often subacute. Rapidly developing polyarthritis, accompanied by remitting fever to 38 - 40 'C with diurnal fluctuations 1 - 2' C and strong sweat, but usually without chills. The first symptom of rheumatic polyarthritis is the growing acute pain in the joints, which increases with the slightest passive and active movements and reaches a high degree in untreated patients. To the pain quickly swollen soft tissue in the joints, almost simultaneously there is an effusion in the joint cavity. The skin over the affected joints is hot, their palpation is sharply painful, the volume of movements due to pain is extremely limited. Characteristic symmetrical lesion of large joints - usually knee, wrist, ankle, elbow. Typical is the "volatility" of inflammatory changes, manifested in the rapid (within a few days) reverse development of arthritic phenomena in some joints and their rapid growth in others. All joint manifestations disappear without a trace; Even without treatment, they last no more than 2 - 4 weeks. With modern therapy, the clinical symptoms of rheumatic polyarthritis can be eliminated within the first 24 hours. It is believed that with acute rheumatism, polyarthritis develops in 60-70% of cases.

As the joint phenomena subsided (less often from the very beginning of the disease), usually not so vivid symptoms of heart damage, which are considered to be the most frequent, and in many patients the only organ manifestation of rheumatism, appear. At the same time, contrary to previous ideas, the disease is not so rare without apparent cardiac changes.

Rheumatic myocarditis in the absence of concomitant heart disease in adults occurs, as a rule, not very serious. Complaints of weak pains or vague discomfort in the heart, mild dyspnoea with exertion, less often - palpitations or irregularities.

When examining the area of ​​the heart, pathology is usually not noted. According to percussion and roentgenoscopy, the heart is of normal size or moderately increased. Usually there is an increase in the left, less often a diffuse expansion. For auscultation and FHG data, satisfactory sonority of tones or slight muffling of I tone are characteristic, sometimes occurrence of III, less often IV tone, mild systolic murmur at the apex of the heart and projection of the mitral valve.

Blood pressure is normal or moderately reduced. Sometimes there is a moderate tachycardia, somewhat higher than the level corresponding to the temperature. On the ECG - flattening, pacification and serration of the P wave and the QRS complex, less frequently the PQ interval elongation is more than 0.2 s. In a number of patients, a small shift of the S-T interval is recorded downstream from the iso-electric line and a change in the T wave (low, negative, rarely biphasic primarily in the leads V1-V3). Seldom are extrasystoles, atrioventricular blockade of the I or III degree, intraventricular block, nodal rhythm.

Insufficiency of blood circulation in connection with primary rheumatic myocarditis develops in rare cases.

In some patients, the so-called diffuse rheumatic myocarditis is more common in childhood. It manifests itself as a violent inflammation of the myocardium with its pronounced edema and impaired function. From the very beginning of the disease, the expressed dyspnea is disturbed, forcing to assume the position of orthopnosis, constant pain in the region of the heart, palpitations. Characteristic "pale cyanosis, swelling of the cervical veins. The heart is considerably and diffusely enlarged, the apical impulse is weak. Tones abruptly muffled, often heard a clear And! Tone (proto-diastolic gallop rhythm) and a distinct but mild systolic noise. Pulse is frequent, weak filling. Arterial pressure is significantly lowered, a collapoid state can develop. Venous pressure rises rapidly, but with the addition of collapse also falls. On the ECG there is a decrease in the voltage of all teeth, flattening of the tooth 1, change of the interval S - T, atrioventricular block. It is very characteristic for diffuse myocarditis development of circulatory insufficiency both in the left ventricular and right ventricular types. This version of rheumatic myocarditis among adults is almost never found.

The outcome of rheumatic myocarditis in the absence of active treatment may be myocarditic cardiosclerosis, the severity of which often reflects the prevalence of myocarditis. Focalized cardiosclerosis does not impair myocardial function. Diffuse myocarditis cardiosclerosis is characterized by signs of a decrease in the contractile function of the myocardium: weakening of the apical impulse, muffling of tones (especially 1), systolic murmur. After physical exertion, there are moderate signs of decompensation: the pastosity of the shins and a slight enlargement of the liver. Often in a combination of myocardial cardiosclerosis with heart defects, it exacerbates its negative effect on hemodynamics.

Rheumatic endocarditis, which is the cause of rheumatic heart disease, is very poor in symptoms. Its significant manifestation is a clear systolic noise with sufficient sonority of tones and the absence of signs of marked myocardial damage. Unlike the noise associated with myocarditis, zondokarditichesky noise is more coarse, and sometimes has a musical tint. Its sonority increases with a change in the position of the ballroom or after a load. A very reliable sign of endocarditis is the variability of already existing noises and especially the appearance of new ones with unchanged (especially at normal) heart boundaries. Light and quickly disappearing diastolic murmurs, sometimes heard at the beginning of a rheumatic attack on the projection of the mitral valve or vessels, may also partly be associated with cardiac stones. Deep endacarditis (valvulitis) of the valves of the mitral or aortic valves in a number of patients is reflected in the zhokardiogram: thickening of the valves, their "lohmatost", multiple echoes from them.

Patients who have cardiac is the only or at least the main localization of rheumatism, for a long time, maintain a good general state of health and ability to work, forming a group of patients with the so-called outpatient course of rheumatism. Only hemodynamic disorders due to the imperceptibly formed heart disease cause such patients to first consult a doctor.

Pericarditis in the clinic of modern rheumatism is rare. Dry pericarditis is manifested by constant pain in the heart area and pericardial friction noise, which is heard more often in the distance of the left edge of the sternum. The intensity of noise is different, usually it is determined in both phases of the cardiac cycle. The ECG is characterized by a shift of the S-T interval in all leads at the onset of the disease. In the future, these intervals gradually return to the iso-electric line, at the same time biphasic or negative teeth are formed. 1. Sometimes ECG data are not indicative. Dry pericarditis by itself does not cause augmentation of the heart.

Exudative pericarditis is essentially a further stage in the development of dry pericarditis. Often the first sign of the appearance of effusion is the disappearance of pain in connection with the separation of inflamed papillary sheets with accumulating exudate. Appears dyspnea, worse in prone position. The heart area with a large amount of exudate somewhat bulges, the intercostal space is smoothened, the apical impulse is not palpable. The heart greatly increases and takes the characteristic shape of a trapezoid or round decanter. Pulsation of the contours with fluoroscopy is small. Tones and noises are very deaf (due to effusion). Pulse is frequent, small filling; Blood pressure is reduced. Venous pressure is almost always increased, swelling of cervical and even peripheral veins is noted. The electrocardiogram is basically the same as with dry pericarditis; An additional symptom is only a noticeable decrease in the voltage of the QRS complex. Essential diagnostic significance is echocardiography, which undoubtedly establishes the presence of fluid in the heart bag.

Since the liquid in the pericardial cavity restricts the diastolic expansion of the heart, then with a significant accumulation of effusion, there may be insufficient filling of the heart cavities during the diastole - the so-called hypodiastole. The latter prevents the influx to the heart, which leads to stagnation in the small and especially in the great circle of blood circulation. A peculiar feature of hypodiastolic circulatory failure is the development of decompensation even without significant damage to the myocardium.

The outcome of rheumatic pericarditis usually involves small adhesions between both leaves or adhesions of the outer sheet with surrounding tissues, which is only recognized with careful roentgenoscopy (deformation of the pericardial contour).

The presence of pericarditis in patients with rheumatism often means the defeat of all layers of the heart - rheumatic pancarditis.

Among the lesions of the skin is almost pathognomonic annular erythema, which is pink ring-shaped elements, never itchy, located mainly on the skin of the inner surface of the hands and feet, abdomen, neck and trunk. It is found only in 1 - 2% of patients. "Rheumatic nodules," described in the old manuals, are practically not found at present. Utic erythema, hemorrhages, hives are not at all characteristic.

Lesions of the lungs. Rheumatic pneumonia is extremely rare and usually occurs against the background of an already developed disease. Their symptoms are the same as those of banal pneumonia; Distinctive signs are the resistance to antibiotic treatment and the good effect of antirheumatic drugs (without antibacterial). Only with this condition the diagnosis can be considered justified. Many modern authors doubt the very existence of rheumatic pneumonia. Rheumatic pleurisy is also nonspecific in its manifestations. Its diagnosis is facilitated when combined with other signs of rheumatism. He thickets are two-sided and characterized by good reversibility. Exudate with rheumatic pleurisy serous-fibrinous and always sterile. Rivalta's test is positive. At the beginning of the disease, neutrophils predominate in the exudate, later - lymphocytes; It is also possible the admixture of erythrocytes, endothelial cells and eosinophils. The first, and even more so, the only sign of rheumatism pleurisy does not happen. It should be borne in mind that if there is a lack of blood circulation in patients with heart defects, congestive changes in the lungs and hydrodrugs can easily simulate "rheumatic" pneumonia and pleurisy.

Renal lesions. In the acute phase of the disease, as a rule, slightly pronounced proteinuria and hematuria are found (consequence of generalized vasculitis and lesion of renal glomeruli and tubules).

Disorders of the digestive system are rare . Gastritis and especially ulceration of the stomach and intestines are usually the result of prolonged use of drugs, especially steroid hormones. In children with rheumatism, there is sometimes severe abdominal pain associated with a rapidly swollen allergic peritonitis. The pain can be very sharp and, combined with a positive symptom of Shchetkin-Blumberg, makes you think about an acute surgical disease of the abdominal cavity. Distinctive features are the diffuse nature of pain, its combination with other signs of rheumatism (or indication of this ailment in an anamnesis), a very rapid effect of antirheumatic therapy; Often the pain disappears on its own in a short time.

Changes in the nervous system and sensory organs. Malignant chorea, a typical "nervous form" of rheumatism, is found mainly in children, especially girls. It is manifested by a combination of emotional lability with muscle hypotension and violent pretentious movements of the trunk, limbs and facial muscles. Small chorea can occur with relapses, but by 17 - 18 years it almost always ends. Features of this form are relatively small heart damage and slightly expressed laboratory indices of rheumatic activity (including often normal ESR).

Acute rheumatic lesions of the central nervous system, proceeding according to the type of encephalitis or meningitis, are extremely rare. They are only a manifestation of generalized rheumatic vasculitis, which is almost not present in our days, and always combined with other signs of rheumatism. Numerous reports of neuropathologists about isolated "rheumatic cerebrovascular disorders" are unreliable. "Rheumatic psychosis" does not exist (contrary to frequent descriptions in psychiatric literature). The existence of rheumatic plexitis, radiculitis and neuritis proper has not been reliably proven.

Laboratory data. In acute rheumatism neutrophilic leukocytosis (up to 12 - 20 'B0 10е ~ l) occurs with a shift of the leukogram to the left, thrombocytosis, an increase in ESR to 40-60 mm / h. In chronic course, these parameters have not been changed so much, anemia of type or normochromic develops occasionally). Characteristic growth of anti-streptococcal antibody titres: anti-streptogialuronidase and antistreptokinase more than 1: 300, anti-streptolysin more than 1: 250. The height of the anti-streptococcal antibody titers and their dynamics do not reflect the degree of activity of rheumatism. In addition, many patients with chronic forms of rheumatic fever have no signs of involvement of streptococcal infection.

All known biochemical indices of activity of the rheumatic process are nonspecific and unsuitable for nosological diagnosis. Only in those cases when the diagnosis of rheumatism is justified by clinicoinstrumental data, the complex of these indicators becomes very useful for judging the degree of activity of the disease (but not about the presence of rheumatism). These indicators include increased plasma fibrinogen levels above 4 g / l, alpha globulins above 10%, gamma globulins above 20%, hexoses above 1.25 gm, ceruloplasmin above 9.25 g, seromucoid above 0.16 g, appearance In the blood of the C-reactive protein. In most cases, biochemical activity indicators are parallel to ESR values.

Classification of rheumatism and features and its course. In accordance with the current classification it is necessary to isolate the previously inactive or active phase of the disease. Activity can be minimal (I degree), medium (II degree) and maximum (grade III). To judge about it, both the severity of clinical manifestations and changes in laboratory parameters are used. Classification is also carried out on the localization of the active rheumatic process (carditis, arthritis, chorea, etc.), the nature of residual phenomena (myocardiosclerosis, etc.), the state of circulation and the course of the disease. There is an acute course of rheumatism, subacute, prolonged, continuously recurrent and latent (clinically asymptomatic). Isolation of the "latent flow" is justified only for retrospective characterization of rheumatism: latent formation of heart disease, etc.

Diagnosis. Specific methods for diagnosing rheumatism do not exist. At the same time, with a detailed picture of the disease, the diagnosis is relatively simple. Difficulties arise, as a rule, first in those patients in whom the disease manifests itself by any one bright, clinical symptom (carditis or polyarthritis). The most accepted international system for diagnosing rheumatism is Jones' criteria. High diagnostic criteria for rheumatism are carditis, polyarthritis, chorea, ring erythema, rheumatic nodules and small fever (at least 38'C), arthralgia, past rheumatism or rheumatic heart disease, increased ESR or a positive reaction to C-reactive Protein, extended interval P - Q on the ECG. The diagnosis is considered reliable if the patient has two large criteria and one small or one large and two small, but only if one of the following evidence of a prior streptococcal infection concurrently exists: recent scarlet fever (which is an undeniable streptococcal disease); Sowing group A streptococcus from the pharyngeal mucosa; Increases titer of antistreptolysin O or other streptococcal antibodies. These criteria appear to be very stringent, but they serve as a serious guarantee of an objectively justified diagnosis and a necessary obstacle to the extremely widespread overdiagnosis of rheumatism.

Among the large criteria, the diagnosis of carditis as such, in turn, requires the presence of objective signs. These include shortness of breath, widening of the heart boundaries, the appearance of a clear soft systolic murmur at the tip or projection of the mitral valve, gentle mesodiastolic noise in the same area, or proto diastolic noise on the aorta, pericardial friction noise, dynamic and usually mild changes in WBC, in particular atrioventricular blockade! Degree. Significant muffling of heart sounds, pronounced cardialgia and rhythm disturbances (in particular, paroxysmal tachycardia) are not characteristic. Subjective disorders and anamnestic information, not documented, can not serve as a basis for the diagnosis of rheumatism. The combination of elevated temperature with normal ESR virtually excludes the diagnosis of active rheumatism. It should also be borne in mind that sick rheumatism is not at all characteristic of "withdrawal to the disease," neuroticism and the desire to describe in detail and colorfully their sensations, palpitations from allergic (infectious-allergic) myocarditis, the feature of which is frequent dissociation between distinct cardiac changes and low Often normal) laboratory signs (ESR, globulins, fibrinogen, etc.), heart defects are never formed .. Patients with functional cardiopathy are characterized by emotional coloration of complaints, their diversity and inadequacy to the absence of objective cardiac pathology.The most frequent complaints of permanent pain in the heart, completely Not characteristic for rheumatism, there are never any reliable signs of organic damage to the heart, laboratory indicators are normal.

Forecast. The immediate threat of life from rheumatism proper arises extremely rarely and almost exclusively in childhood due to diffuse myocarditis. In adults, the predominantly articular and dermal forms proceed most favorably. Chorea is often combined with smaller changes in the heart. In general, the prognosis for rheumatism is determined by the state of the heart (the presence and severity of the defect, the degree of myocardiosclerosis). One of the main prognostic criteria is the degree of reversibility of symptoms of rheumatic heart disease. Continuously recurrent rheumatic heart disease is most unfavorable. With late-onset treatment, the likelihood of the formation of vices increases. In children, rheumatism is more severe and often leads to persistent valve measurements. In the primary disease, over the age of 25, the process flows favorably, and heart disease is very rare. If the primary rheumatic fever occurred without obvious signs of rheumatic heart disease, or the latter turned out to be completely reversible, then it can be assumed that future relapses will not lead to the formation of valvular disease. If more than 3 years have passed since the diagnosis of a specific heart failure, the probability of a new defect is small, despite the continuing activity of rheumatism.

Treatment. In the first 7 to 10 days, the patient with a mild course of the disease should comply with the semi-fast regime, and with severe severity in the first period of treatment - strict bed rest (15-20 days). A criterion for the expansion of motor activity is the rate of onset of clinical improvement and normalization of ESR, as well as other laboratory indicators. At the time of discharge (usually 40 to 50 days after admission), the patient should be transferred to a free regime close to the sanatorium. In the diet, it is recommended to limit table salt.

Until recently, the early combined use of prednisolone (less often triamcinolone) in gradually decreasing doses and acetylsalicylic acid in a constant non-reducing dose of 3 $ g per day was considered the basis of treatment of patients with active rheumatic fever. The initial daily dose of prednisolone was usually 20-25 mg, triamcinolone 16-18 mg, prednisolone about 500-600 mg, triamcinolone 400-500 mg. In recent years, however, facts have been established that call into question the advisability of combining prednisolone with acetylsalicylic acid. Thus, in this case, the negative influence on the gastric mucosa occurs. It was also found that prednisolone significantly reduces the concentration of acetylsalicylic acid in the blood (including below the therapeutic level). With the rapid cancellation of prednisolone, the concentration of acetylsalicylic acid, on the contrary, can rise to toxic. Thus, the combination in question does not seem to be justified, and its effect, apparently, is mainly due to prednisolone. Therefore, with active rheumatism, prednisolone is advisable to be assigned to the quality of a single antirheumatic drug, starting at a daily dose of about 30 mg. This is all the more rational, and because there is objective clinical evidence of any benefits of combination therapy as there is.

The therapeutic effect of glucocorticoids in rheumatism is greater, the higher the activity of the process. Therefore, balloons with particularly high disease activity (pancarditis, polyserositis, etc.), the initial dose is increased to 40 - 50 mg prednisolone or more. The syndrome of cancellation of corticosteroids with rheumatism practically does not happen, therefore, if necessary, even a high dose of them can be drastically reduced or canceled. The best corticosteroid for the treatment of rheumatism is prednisolone.

In recent years, it has been established that the isolated administration of voltaren or indomethacin in full doses (150 mg / day) leads to equally pronounced immediate and long-term results of treatment of acute rheumatism in adults, as well as the use of prednisolone. A rapid positive dynamics in this case, found all the manifestations of the disease, including rheumatic heart disease. At the same time, the tolerability of these drugs (especially voltarenas) was significantly better. However, the question remains of the effectiveness of voltaren and indomethacin in the most severe forms of carditis (with dyspnea at rest, cardiomegaly, exudative pericarditis and circulatory insufficiency), which in adults are practically not found. Therefore, while in such forms of the disease (especially in children), the means of choice are corticosteroids in sufficiently large doses.

On manifestations of small chorea, anti-rheumatic drugs do not directly affect. In such cases, it is recommended to attach luminal or psychotropic agents such as aminazine or especially seduxen to the therapy being used. For the management of patients with chorea, the calm environment, the benevolent attitude of others, the suggestion to the patient of the certainty of a full recovery is of particular importance. When necessary, measures must be taken to prevent the self-harm of the patient as a result of violent movements.

In the first or repeated attacks of acute rheumatism, most authors recommend treatment with penicillin within 7 to 10 days (to kill the most likely pathogen - beta-hemolytic streptococcus group A). At the same time, on the actual rheumatic process, penicillin does not have a therapeutic effect. Therefore, the long and not strictly justified use of penicillin or other antibiotics for rheumatism is irrational.

In patients with prolonged and continuously recurring course, the treatment methods considered are, as a rule, much less effective. The best method of therapy in such cases is a long (one year or more) intake of quinoline preparations: chloroquine (delagila) 0.25 g / day or plaquenyl 0.2 g / day under regular medical supervision. The effect of the use of these agents is manifested no earlier than 3 to 6 weeks, reaches a maximum after 6 months of continuous reception. With the help of quinoline preparations, it is possible to eliminate the activity of the rheumatic process in 70-75% of patients with the most torpid and acute forms of the disease. With a particularly long-term prescription of these drugs (more than a year), their dose can be reduced by 50%, and in the summer months, breaks in treatment are possible. Delagil and plakvenil can be prescribed in combination with any antirheumatic drugs.

Insufficiency of blood circulation in rheumatic heart diseases is treated according to general principles (cardiac glycosides, diuretics, etc.). If cardiac decompensation develops in connection with active rheumatic heart disease, antirheumatic drugs should be included in the treatment complex (including steroid hormones that do not cause significant fluid retention - prednisolone or triamcinolone, dexamethasone is not shown). However, in most patients, heart failure is the result of progressive myocardial dystrophy in connection with heart disease; The specific gravity of rheumatic carditis, if its indisputable clinical, instrumental and laboratory features are absent, while insignificant. Therefore, in many patients with heart defects and severe stages of circulatory failure, a completely satisfactory effect can be obtained with only cardiac glycosides and diuretics. The appointment of vigorous antirheumatic therapy (especially corticosteroids) without obvious signs of active rheumatism may in such cases aggravate myocardial dystrophy. To reduce it, we recommend undevit, cocarboxylase, potassium preparations, riboxin, anabolic steroids.

When rheumatism passes to the inactive phase of patients, it is advisable to send them to local sanatoria, however, all methods of physiotherapy are excluded. It is considered possible to resort treatment even patients with minimal activity, but against the backdrop of continuing antirheumatic medicinal treatment and in specialized sanatoria. Patients without heart defect or with insufficiency of the mitral or aortic valve in the absence of decompensation should be directed to Kislovodsk or to the southern coast of Crimea, and patients with circulatory failure of the I degree, including with unruffled mitral stenoses, only in Kislovodsk. Contraindicated spa treatment with pronounced signs of rheumatism (grade II and III), severe combined or combined heart defects, circulatory failure II or III stage.

Prevention of rheumatism includes active sanation of foci of chronic infection and vigorous treatment of acute diseases caused by streptococcus. In particular, treatment of all patients with quinsy with penicillin injections of 500,000 units 4 times a day for 10 days is recommended. These measures are most important in the already developed rheumatism. If the patient had the first signs of a suspected streptococcal infection in the inactive phase of the disease, then in addition to the mandatory 10-day course of penicillin therapy, he must take one of the antirheumatic drugs within the same period: 2 to 3 g of acetylsalicylic acid, 75 mg of indomethacin and Tp

In accordance with the guidelines of the Ministry of Health of the USSR, the patients who underwent primary rheumatic carditis without signs of valvular lesion showed the appointment of bicillin-1 to 1 200 000 ED or bicillin-5 to 1 500 000 units once every 4 weeks for 3 years. After primary rheumatic heart disease with the formation of heart disease and after recurrent rheumatic carditis, bicillin prophylaxis is recommended for up to 5 years.