Rheumatic fever - symptoms and treatment

Rheumatic feverRheumatic fever

 

 

Rheumatic fever- it is a non-purulent acute inflammatory complication of diseases caused by group A streptococci, leading to the development of arthritis, carditis, subcutaneous nodules, anular erythema and chorea in various combinations. Diagnosis is based on the identification of Jones criteria in the history of anamnesis, physical and laboratory examination. Treatment is carried out with aspirin or other NSAIDs, gluco-corticoids in severe carditis and antibacterial drugs for the eradication of residual streptococcal infection and the prevention of reinfection.

 

The first episode of acute rheumatic fever most often develops between 5 and 15 years and rarely occurs before the age of 3 and after 21 years. Therefore, examination to detect diseases caused by group A streptococcus for the primary prevention of rheumatic fever is usually not necessary in patients under 3 years of age with pharyngitis.In the United States, the incidence is less than 1 per 100,000. The incidence of rheumatic fever with pharyngitis of MUH-etiology without treatment ranges from 0.4 to 3.0%. A higher incidence of rheumatic fever in the case of pharyngitis of the MUH-etiology without treatment is noted when certain serotypes of M-protein streptococcus are detected, as well as with a more pronounced immune response of the organism. In patients with a previous episode of ORL, the incidence of rheumatic fever attack in pharyngitis of MUH-etiology reaches 50% without treatment, emphasizing the importance of long-term anti-streptococcal prophylaxis. The incidence has decreased in most developed countries, but remains high in developing countries. At the same time, recent local outbreaks of ORL may suggest that more virulent streptococcal strains could return to the US. The prevalence of chronic rheumatic heart disease is not precisely known, since the criteria for its diagnosis are not standardized, and an autopsy is not carried out by all.

 

Rheumatic fever - etiology and pathophysiology

 

 

MHA infection is the etiological predecessor of ARF, but factors from the macroorganism and the environment are also important.MUH M-protein has epitopes common with proteins that are found in synovial membranes, myocardium and heart valves, which may suggest that molecular mimicry is important for the development of arthritis, carditis and valvular lesions. Genetic risk factors include the D8 / 17 antigen of B lymphocytes, as well as some histocompatibility class II antigens. Posters of antibacterial drugs; about 13% of cases a year disappear. Although it is usually believed that anti-relapse therapy reduces the frequency of relapses and prevents damage to the kidneys, the results of long-term studies are few. Drugs used for anti-relapse therapy include nitrofurantoin 2 mg / kg orally once a day or TMP-SMK 2 mg / kg orally once a day, usually overnight. In case of MRV IV — V degrees or the detection of major malformations of the organs of the urinary system, anti-recurrent therapy is used; surgical intervention is often necessary, since spontaneous disappearance is observed in less than 5% of cases a year, reduced nutrition, large crowds and low socioeconomic status predispose to streptococcal infections and subsequent episodes of rheumatic fever.

 

The most commonly affected joints, heart, skin and central nervous system.Pathological anatomy varies depending on the location of the lesion.

 

Joints.Joint damage in synovial biopsy manifests as nonspecific inflammation, sometimes with the formation of small areas resembling Aschoff granulomas.

 

A heart.Heart disease is manifested as carditis, in which myocardium, endocardium and pericardium can be affected, and sometimes accompanied by the development of chronic rheumatic heart disease years later. Rheumatic granulomas are often formed in the myocardium and other membranes of the heart. Nonspecific fibrinous pericarditis, sometimes with effusion into the pericardial cavity, develops only in patients with endocarditis and, as a rule, subsides without causing irreversible changes. Characteristic and potentially dangerous valve damage may develop. Acute interstitial valvulitis may cause valve edema. If left untreated, this can lead to valve thickening, development of adhesions, sclerosis, or destruction of the leaflets, with the result that valve stenosis or insufficiency develops. Similarly, chords can be shortened, thickened or soldered to surrounding tissues, contributing to regurgitation on the affected valve or leading to its development on an unchanged valve.Expansion of the valve tendon ring may also cause regurgitation. Mitral, aortic, tricuspid, or pulmonary artery valves are listed, in order of decreasing frequency of injury. Regurgitation and stenosis, as a rule, develop with the defeat of the mitral and tricuspid valves; with the defeat of the aortic valve, regurgitation first appears, and the stenosis develops much later.

 

Leather.Subcutaneous nodules can not be distinguished from those of rheumatoid arthritis, but with a biopsy they resemble the granulomas of Aschoff. Anular erythema is histologically distinct from other skin lesions of a similar type, for example, rashes in the systemic version of juvenile rheumatoid arthritis, Shenlaine-Genoh-ha, chronic migratory erythema, polymorphic erythema. Perivascular neutrophilic or mononuclear infiltration may develop.

 

CNS.Chorea Stedgam is a form of chorea that develops in ORL, characterized by hyperperfusion and increased metabolism in the basal ganglia. There are also reports of increased levels of anti-neuronal antibodies.

 

As a rule, the first symptoms appear 2-4 weeks after suffering a streptococcal infection. The five major clinical syndromes of ORL, which develop individually or in various combinations, form numerous variants of clinical manifestations.

 

Joints.Migratory polyarthritis is the most common manifestation of ORL, developing in about 70% of children; often accompanied by fever. Monoarthritis is sometimes noted. Characterized by a pronounced pain syndrome, pain on palpation, the skin over the affected joint can be hyperemic, hot to the touch, the joint area can be edematous. Ankle, knee, elbow and wrist joints are usually affected. Arthritis of the shoulder, hip joints and small joints of the hands and feet can also develop, but almost never in the form of monoarthritis. With the defeat of the joints of the spine should think about other diseases.

 

Rheumatic fever - big criteria

 

 

Arthralgia-like symptoms may be the result of nonspecific myalgia or pain in the attachment of tendons in the periarticular region; in the place of attachment of the muscle candevelop tenosynovitis. Articular syndrome and fever usually subside within 2 weeks and rarely last more than 1 month.

 

A heart.With cardiac lesions, carditis can be observed in isolation or in combination with pericardial friction noise, cardiac murmurs, expansion of the borders of the heart, or heart failure. In the first episode, ORL carditis occurs in approximately 50% of patients. The patient can consult a doctor with complaints of fever, pain in the chest, as well as if due to heart failure symptoms of lung damage, impaired peripheral hemodynamics, manifestations on the part of the abdominal cavity organs have developed.

 

Often during auscultation of the heart, they hear noises appearing in the early stages. Difficulties may be encountered in detecting mild diastolic noise of a blowing tone with aortic regurgitation and presystolic noise with mitral stenosis. Noises are often stored for a long time. If over the next 2-3 weeks there is no deterioration, new manifestations of carditis appear rarely. ORL usually does not have chronic continuously recurrent carditis.Scars left after acute damage to the valves may contract and change, and secondary hemodynamic disturbances may develop in the myocardium without persistent infection of acute inflammation.

 

Acute congestive heart failure due to a combination of carditis and valve dysfunction can lead to shortness of breath without wheezing, nausea and vomiting, pain in the right hypochondrium or epigastrium, and dry, unproductive cough.

 

Leather.Skin lesions and rheumatic nodules are infrequent and almost never appear in isolation, usually developing in patients who already have carditis, arthritis, or chorea. Fever and other systemic manifestations, such as anorexia and weakness, can be markedly expressed, but non-specific. Subcutaneous nodules, which are most often located on the extensor surfaces of large joints, are usually combined with arthritis and carditis. Nodules are noted in about 2% of children with ARF. Usually nodules are painless, they do not last long and respond to anti-inflammatory treatment of joint and heart lesions.

 

Anular erythema- it is flat or slightly raised, leaving no cicatricial changes and painless rash. This rash occurs in approximately 2% of children. Sometimes it lasts less than 1 day.The appearance of anular erythema may be delayed after suffering a streptococcal infection; it may appear with or after other manifestations of rheumatic inflammation.

 

CNS- Chorea Sydenham develops in approximately 10% of children. It may appear together with other manifestations, however, it often develops after the other manifestations subside. The beginning of chorea is usually imperceptible; it can be preceded by inappropriate laughter and crying. Chorea consists of fast and irregular muscle twitches, which may begin with distal hyperkinesis, but often become generalized, encompassing the muscles of the feet and face. Movement disorders include loss of control of small movements, weakness and hypotension. Many patients develop obsessional-compulsive disorders.

 

Other manifestations. Drowsiness, weakness and lethargy are common and may be due to heart failure. Other manifestations may include abdominal pain and anorexia, which develop in connection with the hepatic impairment described in the characterization of heart failure or concomitant mesenteritis.Because of the presence of fever, increase in blood leukocytes and muscle tension in the anterior abdominal wall, ORL manifestations may resemble acute appendicitis, especially in the absence of other manifestations characteristic of rheumatism. Nasal bleeding develops in about 4% of children in the first episode and 9% in recurrent rheumatic fever.

 

Prolonged episodes of ORL are observed in about 5% of patients with spontaneous relapses of the inflammatory process, not associated with the attachment of streptococcal infection or the abolition of anti-inflammatory therapy. Relapses are usually similar to the first episode.

 

Rheumatic fever - diagnosis

 

 

The diagnosis of the first episode of ORL is based on modified Jones criteria; requires the presence of 2 main criteria or 1 basic and 2 small criteria in a child with a confirmed previous MHA infection. Jones criteria should not be used to diagnose relapse.

 

A previous streptococcal infection can be assumed with a history of recently transferred pharyngitis and confirmed by a positive smear on the pharyngeal flora, an increase in antistreptolysin O, or a positive rapid test for SGA antigen.Recently transferred scarlet fever allows to assume ORL with a high degree of confidence. Swabs on the pharyngeal flora and a positive rapid test for the MHA antigen are often negative in the debut of ORL, while ASLO and other antibodies titers are usually very high. Only 80% of children with a previous infection have significantly increased ASLO; in such cases, the level of antibodies cDNase B should be determined.

 

Joint puncture may be necessary to rule out other causes, such as infection. Synovial fluid is usually cloudy and yellow, with an increased number of leukocytes, mostly neutrophils; synovial fluid cultures are sterile. The level of complement is usually normal or slightly reduced in comparison with its decrease in other inflammatory arthritis.

 

ECG and echocardiography performed during diagnosis. The normal level of cardiac tro-ponin I in children precludes a pronounced myocardial damage. ECG abnormalities, such as prolongation of PR, do not correlate with other signs of carditis. Only 35% of children with ORL have a prolonged PR interval. Other abnormalities in the ECG may be due to pericarditis, enlarged ventricles or flutter, or arrhythmias. EchoCG can reveal signs of carditis in many patients.Radiography of the chest is not routinely performed, but it can reveal cardiomegaly, frequent manifestation of carditis in ORL. Biopsy of the subcutaneous nodule can help early diagnosis, especially in the absence of other major manifestations. Rheumatic heart disease should be differentiated from congenital heart defects and fibroelastosis; EchoCG or coronary angiography can be used to verify the diagnosis in complex cases of permanent damage to the heart. In patients who have not had a carditis, recurrent episodes of ARF are much less common and almost never develop carditis in the recurrent episode of ARF. Chorea Sydenhama usually lasts several months and disappears completely in most patients, but about /3patients have recurrent episodes. All other manifestations subside without causing irreversible changes.

 

Rheumatic fever - treatment

 

 

Primarily, the goals of treatment include suppression of inflammation and alleviation of acute symptoms, eradication of MHA infection and prevention of future infections to prevent recurrent rheumatic heart disease.

 

Patients usually should be limited to motor activity in cases of arthritis, chorea and heart failure.In the absence of carditis, no restrictions are required after subsiding the initial episode. In patients with carditis without clinical manifestations, strict bed rest does not have a proven value.

 

Aspirin controls fever and pain in arthritis and carditis. The dose is gradually increased to obtain a clinical effect or the appearance of toxic effects. The starting dose in children and adolescents is 15 mg / kg orally 4 times a day. In the absence of the effect the next morning, the dose is increased to 22.5 mg / kg 4 times a day the next day and 30 mg / kg 4 times a day every other day. The toxicity of salicylates is manifested by tinnitus, headache, or hyperplasia, and may not manifest until the treatment lasts more than a week. Salicylates are measured only in order to control their toxic effects. Covered with enteric-soluble shell, with additives that have buffering properties, or complex molecules of salicylates do not represent advantages. Other NSAIDs may also be used. For example, naproxen at 7.5-10 mg / kg orally, 2 times a day, is as effective as aspirin.

 

ESR and C-reactive protein are sensitive, but not specific. ESR is often above 120 mm / h.CRP is often more than 2 mg / dL; as it increases and decreases faster than ESR, the normal level of CRP can confirm the absence of an inflammatory process in a patient with a prolonged increase in ESR after the acute manifestations subside. In the absence of carditis, ESR is usually normalized within 3 months. Signs of acute inflammation, including ESR, usually disappear within 5 months with uncomplicated carditis. Leukocytosis in the blood reaches 12,000–20,000 / μl and can grow even higher with glucocorticoid therapy.

 

Differential diagnosis

 

 

Differential diagnosis is performed with JRA, Lyme disease, reactive arthritis, arthropathy with sickle cell anemia, leukemia or other malignant tumor, SLE, embolic bacterial endocarditis, serum sickness, Kawasaki disease, drug exposure and gonococcal arthritis. Often they are excluded by history and special methods of laboratory diagnosis. The absence of a previous MUH infection, diurnal fluctuations in body temperature, a short rash and a long-term clinically manifested inflammation of the joints usually distinguish systemic forms of JRA from ORL.

 

Forecast

 

 

The forecast mostly depends on the severity of the initial carditis. Patients with severe carditis during the first episode may develop heart disease, which is often worsened by recurrent episodes of rheumatic fever that are characteristic of such patients. Noises eventually disappear around /2patients, in whom the ORL manifested itself with light carditis without significant expansion of the heart or decompensation. The risk of recurrent inflammation is intermediate, between low risk in patients without carditis and a high risk in the presence of severe carditis in the anamnesis.

 

If the therapeutic effect was not achieved after 4 days of treatment, which is sometimes noted in the presence of severe carditis or arthritis, NSAIDs should be discarded and glucocorticoids should be prescribed.

 

Prednisone 0.25-1 mg / kg orally, 2 times a day is recommended up to 60 days. If after 2 days it was not possible to suppress inflammation, pulse-therapy with methylpredni-zolone may be administered with succinate. Orally administered glucocorticoids are taken until the ESR remains normal for a week or more, and then gradually the dose is reduced at a rate of 5 mg every 2 days.To prevent an increase in the activity of inflammation while reducing the dose of glucocorticoids, they simultaneously prescribe NSAIDs so that they continue the treatment for another 2 weeks after the complete abolition of glucocorticoids. Laboratory values ​​are used to monitor disease activity and response to treatment. Markers of inflammation, such as ESR and CRV, are the best indicators of a therapeutic response.

 

Returnable episodes of the inflammatory process in the heart may subside spontaneously, but NSAIDs or glucocorticoids should be resumed for heart failure, which cannot be controlled by cardiotonic drugs. In patients with prolonged recurrent episodes of carditis, immunosuppressive therapy can also be effective. Although NSAIDs and glucocorticoids are effective in acute episodes, they do not prevent or reduce long-term valve damage.

 

Although the post-streptococcal inflammatory process is well developed by the time an ORL is diagnosed, antibiotic therapy is prescribed in order to destroy any remaining microorganisms and prevent reinfection.Relevant treatment regimens for acute infection are described in the chapter “Streptococcal and endococcal infections” on page 1128.

 

Anti-streptococcal prophylaxis should be carried out continuously after the initial episode of ORL to prevent recurrent episodes. Antibiotics taken orally are as effective as with parenteral administration, and oral forms of antibiotics are usually recommended because injections are painful and require clinic visits and follow-up visits to detect post-injection reactions. The optimal duration of anti-streptococcal prophylaxis is unknown. Children without carditis should receive prophylaxis for 5 years or until the age of 21. Despite the fact that the clinical experience of secondary prevention of heart damage is very contradictory, it seems reasonable to prescribe an anti-streptococcal prophylaxis for 1 year and then repeat echocardiography. If the heart damage is detected on EchoCG, long-term prophylaxis is indicated.

 

The American Academy of Pediatrics recommends that children with carditis without signs of residual heart disease be prevented for 10 years.Children with carditis and signs of residual heart disease should receive prophylaxis for more than 10 years; Many experts recommend that such patients continue prophylaxis for the rest of their lives. Some experts believe that prevention should be lifelong in all patients with chorea and should continue in all patients who have close contact with young children due to their high MHA burden.

 

In patients with known or suspected rheumatic valve disease who have stopped taking prophylactic antibacterial drugs, short-term prophylaxis of bacterial endocarditis should be undertaken in dental or other surgical procedures in the oral cavity that can cause gingival bleeding, upper respiratory tract operations and operations or instrumental examination of the genitourinary tract system or gastrointestinal production of thyroid hormones), transplacental tupleniya antibodies from the mother or transplacental Incoming goitrogenic substances. Some causes are hereditary.

 

Dyshormonogenesis

 

 

Genetic defects in thyroid hormone production lead to an increase in thyroid-hormone levels, which in turn can lead to the development of congenital goiter. There are 4 main types of dyshormonogenesis.

 

Type 1caused by a defect in iodide transport as a result of impaired protein synthesis on the cell surface, which is necessary for transportation.

 

Type 2caused by one of several defects in the mechanisms of iodization in the thyroid gland. The peroxidase enzyme necessary for the conversion of iodine may be absent or its function may be impaired. With another defect, hydrogen peroxide formation may be impaired. In children with Pendred syndrome, moderate hypothyroidism or euthyroidism, goiter and sensorineural hearing loss are observed due to an abnormality of the transport protein that is involved in iodine transport, as well as in the cochlear function.

 

Type 3caused by a complete or partial defect in the deiodination of monoyod-tyrosine and diiodotyrosine in thyroglobulin.

 

Type 4caused by one of several thyroglobulin synthesis defects, usually inherited in an X-linked type. In this condition, clinical hypothyroidism does not develop,however, it is characterized by very low total thyroxine in serum with normal TSH and free testosterone.

 

Transplacental intake of maternal antibodies

 

 

In women with autoimmune thyroid lesions observed the formation of antibodies that can pass through the placenta. Depending on the disease, these antibodies can either block the TSH receptors, leading to the development of hypothyroidism, or stimulate them, leading to the development of hyperthyroidism. As a rule, in a child these disorders spontaneously disappear within 3-6 months.

 

Transplacental intake of goitrogenic substances. Goitrogenic substances, such as amiodarone or thyroid-medication, can pass through the placenta, sometimes leading to the development of hypothyroidism and rarely goiter.

 

Symptoms and signs

 

 

The most common symptom is an enlarged thyroid gland which palpation has a dense texture, painless. The increase is often diffuse, but may be nodal. It may be noticeable from birth or show up later. In some patients, goiter is not directly detected,however, with the continued increase in the size of the thyroid gland, a deviation or compression of the trachea may develop, and breathing or passage of food along the esophagus is impaired. Many children with goiter have euthyroidism, but some may develop hypothyroidism or hyperthyroidism.

 

Diagnosis and treatment

 

 

If a congenital goiter is suspected, it is necessary to estimate the size of the thyroid gland using ultrasound. It is necessary to determine the level of TSH in the blood.

 

Hypothyroidism treatment includes thyroid hormone replacement therapy. A child with a breathing disorder and passage of food through the esophagus may need surgical intervention.



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