What is Rheumatic Fever?

Rheumatic fever is a non-purulent disease of systemic connective tissue related to group A hemolytic streptococcus infection. It was once one of the major diseases that endangered the life and health of school-age children and adolescents, and can affect the heart, joints, and center. Nervous system and subcutaneous tissue, but most obvious in the heart and joints. Clinical manifestations are carditis, ring-shaped erythema, arthritis, sydenham chorea, and subcutaneous nodules. Lesions can be acute or chronic recurrent, and heart valve disease can be left behind to form chronic rheumatic heart valve disease.

Basic Information

English name
rheumatic fever
Visiting department
Division of Rheumatology
Common causes
Related to group A hemolytic streptococcal infection
Common symptoms
Carditis, erythema ring, arthritis, sydenham chorea, subcutaneous nodules
Contagious
no

Causes of rheumatic fever

It is generally accepted that the incidence of this disease is related to group A hemolytic streptococcal infection. The capsular membrane of streptococcus is composed of hyaluronic acid, and there is common antigenicity with the hyaluronic acid protein of human synovium and synovial fluid, which can resist the phagocytosis of leukocytes and play a protective role. Group A streptococcal protein antigens have cross-antigenicity with human heart valves and brain tissues, which can cause cross-immunity. This cross-reaction is very important in the pathogenesis of rheumatic fever valve disease. Streptococci can produce a variety of extracellular toxins and also play an important role in their pathogenicity.
In addition, there is a genetic susceptibility to the onset of rheumatic fever. The incidence of members of the same family is higher than that of families without rheumatic fever, and those with single egg twins who have concurrent rheumatic fever are higher than those with twin eggs.

Clinical manifestations of rheumatic fever

Rheumatic fever lacks typical and specific clinical manifestations, and symptoms vary.
Arthritis
Polyarthritis is a common first symptom, with an incidence of more than 75%, and the affected joints appear red, swollen, burning, pain, and restricted movement during acute attacks. The typical characteristics of arthritis are: migratory, which can migrate from one joint to another in a very short period of hours or days; often multiple, often manifested by the involvement of more than two joints at the same time; Large violations of large joints, such as knees, ankles, elbows, wrists and shoulders, etc .; joint pain is closely related to changes in weather, exacerbated when wet or cold, and symptoms can be relieved naturally as the environment improves; salicylic acid preparations have Significant effect, symptoms can be significantly relieved after 24 to 48 hours of treatment, but arthritis rarely lasts for more than 4 weeks even without treatment; arthritis disappears with rheumatism and joint function recovers, leaving no rigidity or deformity.
2. Carditis
Carditis is the most important manifestation of rheumatic fever in children, accounting for 40% to 80%. It can manifest as myocarditis, endocarditis, pericarditis, or pancarditis. Most of them are affected by both myocardium and endocardium. Simple myocarditis or pericarditis are rare.
3. Circular erythema
In the past, the incidence was 10% to 20%, and the current incidence is less, 2.4%. It usually occurs in the late stage of rheumatic fever, and is often distributed on the trunk and the proximal parts of the limbs, such as the inner thighs, with a ring shape with light red edges. Or semi-circular redness, the ring changes from small to large, the center skin color is normal, the rash can be merged into an irregular shape, it is not painful and itchy, and often disappears quickly within a few hours or 1-2 days, but it can be reproduced in situ after the subsidence, the rash Hidden from time to time, going through months.
4. Subcutaneous nodules
Rarely, ranging from 2% to 10%. It is a hard and painless nodule of 1.5cm to 2.0cm, which can be isolated or gathered together. It is mostly in the bone bulge of the extensor of the joint. It has no adhesion to the skin and no redness and swelling on the surface. It is often accompanied by severe myocarditis. .
5.Sydenham dance disease
Sydenham chorea is caused by extrapyramidal involvement and is a late manifestation of rheumatic fever. It usually occurs 2 months or more after group A hemolytic streptococcal infection. More common in female patients, more children than adults. It is manifested by involuntary movements and emotional instability of facial muscles and limbs, frowning, tongue extension, blinking, shaking head, neck turning; limb straightening and flexion, adduction and abduction, pronation and supination. Alternate action. Aggravates when agitated or excited, and disappears during sleep.
6. Clinical typing
(1) Acute attack type is more common in children, with rapid onset and dangerous illness, manifested as severe carditis, arthritis, rheumatic pneumonia, congestive heart failure, etc., which can cause death if not treated in time. This type is rare in China.
(2) Recurrent type This type of clinical type is the most common. It usually recurs within 5 years after the onset of the disease. It repeats the past clinical characteristics, and the heart valve damage is aggravated every time it recurs.
(3) Chronic type (persistent type) refers to the recurrence of remission, remission, and aggravation of the condition, which persist for more than six months. Carditis is often the main manifestation, and it can also be accompanied by arthritis or joint pain.
(4) Subclinical (concealed rheumatic fever) is concealed, and the clinical manifestations are not typical. There may be non-characteristic manifestations such as sore throat or pharyngeal discomfort, fatigue, limb pain, and pale complexion. A few patients may have low fever. This type of patient can be concealed for many years and gradually develop into chronic rheumatic heart disease. It can also be exacerbated by another streptococcal infection and other symptoms. Typical clinical manifestations appear.

Rheumatic fever check

1. Laboratory examination of rheumatism <br /> Routine blood tests of patients with active stage have elevated white blood cells and neutrophils, and there is a phenomenon of nuclear left shift. Mild anemia can also be seen. ESR increased faster, and CRP increased earlier than the rapid increase in ESR. Serum protein electrophoresis showed decreased albumin, increased a 2 and Y globulin, and increased mucin. Immunoglobulins IgM / IgG, complement, and CIC increase in the acute phase, especially complements C3 and C4 change on the second day of clinical symptoms of rheumatic fever, so it has diagnostic significance for rheumatic activity.
2. Evidence of resistance to streptococci
The most direct evidence of streptococcal infection is the culture of Group A R-hemolytic streptococcus in the pharynx, with a positive rate of only 20% to 25%. The increase in anti-streptococcal antibody titer is also a reliable indicator of recent streptococcal infection. About two weeks after streptococcal infection, the ASO titer of most rheumatic fever patients (75% to 80%) increased by more than 500U, 4-6 It peaks at weeks and gradually returns to normal after 8 to 10 weeks.
3. Chest X-ray and ECG
The chest X-ray may be normal or enlarged. Cardiac involvement can lead to abnormal electrocardiograms, such as sinus tachycardia or bradycardia, premature contraction and other arrhythmias, atrioventricular block, prolonged QT interval, and changes in ST-T.
4. Echocardiography
Patients can find enlarged hearts, edema and thickening of heart valves, insufficiency or stenosis, and pericardial effusion.

Rheumatic fever diagnosis

At present, the Jones standard revised in 1992 is still in clinical use. See the table below. If there is one major indicator and two minor indicators, plus evidence of a precursor streptococcal infection, the diagnosis can be determined.
Diagnostic criteria for onset rheumatic fever
main indicators
Secondary indicator
Evidence of streptococcal infection
Carditis
heat
Increased ASO titer
Multiple arthritis
Arthralgia
Pharyngeal culture positive
Sydengham dance disease
Increased ESR
Erythema ring
CRP rise
Subcutaneous nodule
PR interval extension
At present, it is considered that the Jones standard cannot be mechanically applied when diagnosing rheumatic fever, and comprehensive analysis of clinical data should be performed to make necessary exclusion diagnosis. For example, viral myocarditis or infective endocarditis should be excluded when diagnosing rheumatic carditis; diagnosing rheumatism In arthritis, other arthritis, connective tissue disease, reactive arthritis caused by infection, and tuberculous arthritis should be excluded; when using ring-shaped erythema or subcutaneous nodule as the main diagnostic index, systemic lupus erythematosus and other diseases should also be excluded. Related diseases.

Rheumatic fever treatment

There is no specific treatment for this disease. The general treatment principles include: early diagnosis, reasonable treatment, and prevention of irreversible heart disease caused by disease progression.
1. General treatment < br is mainly to take appropriate symptomatic treatment for different symptoms. Patients in the acute phase should stay in bed; if the heart is involved, avoid physical activity or mental stimulation, and continue to stay in bed for 3 to 4 weeks after the body temperature and erythrocyte sedimentation return to normal, tachycardia control or obvious electrocardiogram improvement, and then gradually return to activity For those with enlarged heart and heart failure, it takes about 6 months to gradually return to normal activities. People with congestive heart failure should also limit salt and water appropriately.
2. Application of antibiotics
The purpose of antibiotics is to eliminate residual streptococcal infections. One intramuscular injection of penicillin or 10 days of oral penicillin is recommended. For a few penicillin-resistant strains or allergic to penicillin, erythromycin can be used 4 times a day for 10 days. Resistance to erythromycin can be replaced by other drugs, including ampicillin / clavulanate, neomacrolides, and cephalosporins.
3. Anti-rheumatic treatment
The first choice for rheumatoid arthritis is nonsteroidal anti-inflammatory drugs. Among them, aspirin is still the drug of choice. It is taken orally 3 to 4 times. The general course of treatment is 6 to 8 weeks. Those with mild carditis should use it for 12 weeks. Glucocorticoids are recommended only for severe cardiac inflammation with congestive heart failure. Commonly used drugs are prednisone, which is taken in 3 to 4 times. After the disease is controlled, the dose is gradually reduced. The course of treatment is at least 12 weeks. For patients with pericarditis or myocarditis complicated by acute heart failure, dexamethasone or hydrocortisone can be administered intravenously, and the patient can be switched to oral prednisone after the condition improves. Glucocorticoids can also be used for the treatment of severe arthritis that does not respond to aspirin. The course of treatment is 6-8 weeks.
Severe cardiac inflammation or enlarged heart is prone to heart failure. In addition to glucocorticoid therapy, angiotensin-converting enzyme inhibitors, digitalis, diuretics, and drugs that reduce heart load should be used in combination.
4.Treatment of Sydenham chorea
First of all, patients should be given a quiet environment to avoid strong light and noise stimulation and prevent trauma. When necessary, add sedatives such as diazepam, barbiturates or chlorpromazine on the basis of anti-rheumatic therapy.
5. Treatment of chronic heart valve disease
People with chronic congestive heart failure should be given oral digitalis for a long time, and monitor the blood concentration, and adjust the dose at any time. Severe valve damage can be given surgery for valvuloplasty or replacement.
6. Primary prevention
Because rheumatic fever has multiple families and hereditary tendencies, relatives of patients are high-risk groups of rheumatic fever, and prevention should be focused on. It is currently recommended that patients with group A hemolytic streptococcal pharyngitis, or adolescents over 5 years of age should be treated when they intend to diagnose upper respiratory streptococcal infection. A single dose of long-acting penicillin can be injected intramuscularly, divided into 2 to 4. Then, the medication was continued for 10 days.
7. Secondary prevention
Secondary prevention is mainly aimed at young people, those with high susceptibility factors, multiple recurrences of rheumatic fever, who have had carditis and sequelae of valvular disease, and whose primary purpose is to prevent and reduce heart damage. Long-acting penicillin is administered intramuscularly every 3 to 4 weeks for at least 10 years, or until the age of 40, or even life-long prevention.

Rheumatic fever prognosis

The prognosis of rheumatic fever is mainly determined by the development of rheumatic heart disease. The severity or recurrence of carditis at the first onset is the main factor determining the prognosis of rheumatic heart disease. In the first episode, the heart is obviously affected, multiple relapses or concomitant heart failure have a poor prognosis, and chronic rheumatic heart valve disease often occurs. After acute rheumatic fever, heart valve disease occurs in 10% to 20% of adults and 60% of children. If the disease recurs more than twice, the incidence of heart valve disease is as high as 90%. The prognosis of chorea is good. Most of them can heal naturally after 4 to 10 weeks and rarely recur, but a few patients may have neuropsychiatric symptoms. Multiple arthritis can be cured.

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