rheumatism



OVERVIEW

Definition

Rheumatic fever is a systemic inflammatory disease of connective tissue caused by infection of the throat with group A beta-hemolytic streptococcus, which may begin acutely or become chronic with recurrent episodes.

It mainly involves the joints, heart, skin and subcutaneous tissues, and occasionally the central nervous system, blood vessels, plasma membranes, and organs such as the lungs and kidneys.

Epidemiology

The incidence of rheumatic fever varies in different parts of China, with a total prevalence of about 22/100,000 people.

It is most common in children and adolescents between the ages of 4 and 15 years, and is rarely seen in infants and young children under 3 years of age and adolescents after 15 years of age.

The prevalence of rheumatic fever is approximately equal in both sexes.

Rheumatic fever can occur in all seasons of the year, but is more common in winter and spring.

Causes

Causes

Group A hemolytic streptococcus B pharyngeal infection

Rheumatic fever is the main cause of rheumatic fever. Infection with group A beta-hemolytic streptococcus in the skin and other areas of the body does not cause rheumatic fever.

Rheumatic fever occurs in 0.3% to 3% of children with untreated pharyngitis caused by this organism after 1 to 4 weeks.

Group A beta-hemolytic streptococci produce a variety of exotoxins and enzymes that may have toxic effects on human heart muscle and joints.

Genetic factors

It has been reported that HLA-DR and/or DQ alleles, and the presence of the signature D8/17 antigen on the surface of B lymphocytes may be associated with the development of rheumatic fever.

Immunologic factors

The antigens of group A beta-hemolytic streptococcus are complex, and the molecular structures of many antigens are homologous to the organ antigens of the organism, and the anti-streptococcal immune antibodies of the organism can produce immune reactions with human tissues, leading to organ damage.

Predisposing factors

Cold and damp environment.

Overcrowded living room.

Malnutrition.

Symptoms

Rheumatic fever tends to have an acute onset or an insidious process. The main manifestations are roving polyarthritis, carditis, subcutaneous nodules, erythema annulare, and chorea, which may occur singly or in combination.

Prodromal symptoms

A history of post-streptococcal pharyngitis is often present 1 to 6 weeks prior to the onset of typical symptoms, which may be characterized by fever, sore throat, submandibular lymph node enlargement, and cough.

Low-to-moderate fever is common, and high fever may be present.

More than half of the patients have mild or transient prodromal symptoms, which are often overlooked.

Typical symptoms

Arthritis symptoms

Wandering, polyarthritic arthritis is usually present, with the knees, ankles, elbows, wrists and other joints being the predominantly affected.

Mild and atypical patients may have single joint or oligoarticular or oligoarticular involvement, or involvement of some uncommon joints such as hip joints, finger joints, jaw joints, shoulder joints, toe joints and so on.

The manifestations are localized redness, swelling, burning, pain, limitation of movement, and sometimes joint effusion.

Joint pain is usually relieved within 1 week and rarely lasts more than 1 month.

No deformity remains after healing, but it may recur and may be aggravated by low temperature or rainy weather.

Carditis Symptoms

The heart is the only organ sustainably damaged by rheumatic fever. In the first attack of rheumatic fever, symptoms of carditis usually appear within 1 to 2 weeks of onset.

Valvulitis and endocarditis are the most common, and pericarditis can also occur, which is asymptomatic in mild cases, but can lead to heart failure and heart block in severe cases.

It is mostly characterized by palpitations, shortness of breath, pain or discomfort in the precordial area after exercise.

Circumscribed erythema

Pale erythema with obvious circular or semi-circular borders, varying in size and with a pale center, appears on the skin.

It appears on the trunk and proximal extremities, does not involve the face, and is transient, appearing, disappearing, and reoccurring within a few hours, or presenting and delaying, and may last for several weeks.

It often occurs in patients with acute carditis, in combination with other symptoms.

Subcutaneous nodules

Firm, painless nodules palpable under the skin, ranging in size from a few millimeters to 2 centimeters, not adherent to adjacent skin, subsiding within 1 to 2 weeks, rarely lasting more than 1 month.

They often appear on the extensor surfaces of elbows, knees, wrists, ankles and other joints, or on the occipital region, forehead scalp, and the protrusions of the thoracic and lumbar spinal processes.

It is often accompanied by chronic active carditis and rarely occurs early in the disease.

Chorea Symptoms

It is characterized by involuntary, uncoordinated rapid movements of the whole body or some of the muscles, such as sticking out the tongue and tilting the mouth, squeezing the eyebrows, shrugging the shoulders and shrinking the neck, speech impediments, writing difficulties, and uncoordinated subtle movements, often accompanied by muscle weakness and emotional instability.

Symptoms are usually seen 6 to 8 weeks after streptococcal infection, worsen with excitement or concentration, and disappear after sleep.

Other symptoms

Patients with rheumatic fever may also present with excessive sweating, nosebleeds, skin petechiae, and abdominal pain.

When kidney damage occurs, hematuria and increased foaminess in the urine may occur.

Complications

Heart failure: Mostly seen in severe carditis, frequent recurrence, improper or untimely treatment, it may manifest as weakness, dizziness, shortness of breath after activity, palpitation, dyspnea, cough, coughing up white foamy sputum, hemoptysis, edema, and urinary loss.

Rheumatic heart valve disease: 75% to 80% of rheumatic fever is complicated by rheumatic heart valve disease, with thickening, adhesion, closure insufficiency, stenosis, and prolapse of heart valves, which is more common in mitral valve, followed by aortic valve.

Consultation

Department of Medicine

Rheumatology

Prompt medical consultation is recommended in case of fever, redness, swelling and pain in joints, subcutaneous nodules, and annular erythema of the skin.

Emergency Department

If you experience panic after activity, dyspnea, or discomfort in the precordial area, it is recommended that you visit the Emergency Department or call the 120 emergency number as soon as possible.

Preparation for medical treatment

Preparation for medical consultation: registration, preparation of documents, common problems

Tips for seeking medical treatment

Avoid self-medication before seeking medical treatment, as this may cover up your condition.

Record the changes and characteristics of your condition so that you can give your doctor more references.

Preparation Checklist

Pay particular attention to the time of onset of symptoms, special manifestations, etc.

Is there any fever? If there is fever, how long has it lasted? What is the highest temperature?

At what time of day was the temperature highest? Is the fever persistent or intermittent?

Are there any symptoms of joint pain, redness, swelling, or limited mobility?

Do you experience shortness of breath after exercise? Is it accompanied by a feeling of panic?

Are there any involuntary trunk or limb movements?

Has there been a rash, such as erythema annulare? Are there any small hard nodules under the skin that can be felt?

Has there been any facial edema, lower extremity edema, low urine output, or deepening of urine color?

Have there been any recent symptoms of sore throat or cough?

Has anyone in the family suffered from rheumatic fever?

Any history of rheumatic fever, scarlet fever or other upper respiratory tract infections?

Test results in the last 6 months to bring to the doctor

Laboratory tests: routine blood tests, routine urine tests, throat swab culture, anti-streptococcal hemolysin O test, immunological tests

Imaging tests: chest X-ray, echocardiogram, myocardial nuclear examination

Medications in the last 3 months, if available, bring the box or package with you to the doctor’s office.

Analgesics: indomethacin, acetaminophen, diclofenac, ibuprofen

Antimicrobials: cephalosporin antibiotics, clindamycin, erythromycin

Diagnosis

Disease Diagnosis

Diagnostic basis

There is no specific diagnostic method for rheumatic fever. The diagnosis of rheumatic fever depends on the history, clinical manifestations, results of auxiliary examinations, and exclusion of other diseases.

Recently suffered from scarlet fever or other upper respiratory tract infections.

History of rheumatic fever.

Relatives of patients with the disease.

Presence of typical symptoms such as fever, wandering polyarthritis, carditis, subcutaneous nodules, erythema annulare, and chorea.

Presence of heart murmur due to stenosis or insufficient closure of heart valves.

Presence of cardiac insufficiency: weakness, dizziness, shortness of breath after activity, palpitations, dyspnea, and other symptoms.

Includes routine blood tests, routine urinalysis, pharyngeal swab culture, anti-streptococcal antibody measurement, erythrocyte sedimentation rate, C-reactive protein, and immunologic tests. Helps to determine the cause of the disease.

Routine blood tests: peripheral blood leukocyte count and neutrophils may be elevated during the active phase of the disease.

Urine routine examination: when there is renal damage, abnormal red blood cells, urinary occult blood, urinary protein can appear in urine.

Pharyngeal swab culture: Group A hemolytic streptococcus B can be found in 20%～25% of patients.

Anti-streptococcal hemolysin O test: serum anti-streptococcal hemolysin O is elevated in 50% to 80% of patients with rheumatic fever. However, since higher titers of antibodies are prevalent in the population, the results should be interpreted with great caution and the test should be repeated in 2 weeks to determine the presence of a recent infection based on the dynamics of the antistreptococcal antibodies.

Erythrocyte sedimentation rate increases and C-reactive protein is elevated during active disease.

Immunological examination: there may be increased immunoglobulins (e.g. IgM, IgG), positive detection of circulating immune complexes, positive anti-cardiac antibodies, etc., which have some value in the diagnosis of rheumatic fever.

Chest X-ray: It helps to know the size of the heart and changes in the lungs. If accompanied by myocarditis, heart enlargement can be observed; if accompanied by pericarditis, heart shadow can be seen enlarged to both sides in the shape of flask.

Echocardiography: It can detect early and mild carditis as well as subclinical carditis, and is more sensitive to small amounts of pericardial effusion.

Myocardial nuclear examination: a test to determine heart disease by means of radionuclide imaging, which helps to detect mild and subclinical myocarditis.

Sinus tachycardia and prolonged P-R interval are the most common ECG manifestations of carditis. Pericarditis can present with widespread ST-segment elevation.

Diagnostic criteria

There is no specific diagnostic method for rheumatic fever, and the Jones diagnostic criteria, revised by the American Heart Association in 1992, are used clinically. The diagnosis is made with two major manifestations or one major manifestation with two minor manifestations, provided that evidence of streptococcal infection is established.

Carditis.

Polyarthritis.

Chorea.

Erythema annulare.

Subcutaneous nodules.

Arthralgia.

Fever.

Past history of rheumatic fever.

Increased erythrocyte sedimentation rate, increased C-reactive protein, leukocytosis, anemia.

ECG suggests prolonged P-R interval and prolonged Q-T interval.

Recent history of scarlet fever.

Pharyngeal culture was positive for hemolytic streptococci.

Serum anti-streptococcal hemolysin O or rheumatic fever antibodies to streptococci are elevated.

Differential Diagnosis

Juvenile idiopathic arthritis

Similarities: fever, joint swelling, pain, etc.

Differences: Juvenile idiopathic arthritis more often involves the small joints of the fingers (toes), and may also involve the knee and ankle joints. Repeated episodes of the disease may lead to joint deformity, and X-ray bone and joint radiographs may show destruction of the joint surfaces, narrowing of the joint space and osteoporosis of the adjacent bones.

Septic arthritis

Similarity: fever, redness, swelling and pain in the joints.

Difference: septic arthritis has a rapid onset, often accompanied by fever, body temperature can be more than 39 ℃, superficial joints, such as knee, elbow joints, localized redness, swelling, heat, pain is obvious, and joint pain and rapid joint dysfunction can occur.

Acute leukemia

Similarity: fever, joint pain, etc.

Difference: acute leukemia is mostly accompanied by anemia, bleeding tendency, hepatomegaly, splenomegaly and lymph node enlargement, peripheral blood film can see primitive and naive cells, bone marrow examination can be distinguished.

Infective endocarditis

Similarities: fever, joint pain, arrhythmia, heart failure, etc.

Differences: Infective endocarditis is a microbial infection of the endocardial surface of the heart, accompanied by the formation of organisms. Symptoms such as anemia, splenomegaly, and ecchymosis of the skin can help in differential diagnosis; echocardiography can show the presence of organisms in the heart valves or endocardium, and blood cultures can assist in the detection of the causative pathogens.

Viral myocarditis

Similarities: fever, malaise, shortness of breath, chest discomfort, arrhythmia, heart failure.

Differences: Viral myocarditis has a less pronounced murmur, less endocarditis, more arrhythmias such as preterm contractions (premature beats), and evidence of viral infection can be found on laboratory tests.

Treatment

Treatment goals: to clear streptococcal infection and remove the cause of rheumatic fever; to control and alleviate acute symptoms; to manage complications, to improve the patient’s physical fitness and quality of life, and to prolong life expectancy.

Treatment: mainly general treatment and drug treatment.

General treatment

If there are no obvious clinical symptoms and complications, no treatment can be carried out for the time being, and regular rechecks can be carried out according to the doctor’s advice.

It is recommended that people with fever, joint swelling and pain should rest in bed until the acute symptoms disappear. Those without carditis are recommended to rest for about 1 month, while those with carditis need to rest for at least 2 to 3 months.

Those with carditis complicated by heart failure should be absolutely bedridden for at least 6 months and then gradually return to normal activities.

Patients with chorea should avoid bright light and noise stimulation, and provide adequate psychological and social support.

Pay attention to keep warm and increase clothing appropriately in cold weather.

Dehumidifier can be applied at home to avoid humid environment.

Eat a light diet, avoid spicy and irritating foods, and eat small and frequent meals.

Monitor the condition and go to the hospital if symptoms worsen or recur.

Medication

During the medication period, you should adhere to the doctor’s instructions, do not change the dose of medication without authorization or stop the medication suddenly, to ensure that the treatment plan is implemented. Do not believe in unrecognized treatments such as local remedies, secret remedies, and biased remedies.

Anti-infective drugs

Used to clear streptococcal infections.

Individualized treatment plan is selected based on the specific condition, drug effectiveness, drug sensitivity test and other comprehensive considerations.

Penicillin is usually chosen, and those who are allergic to penicillin can be switched to other effective antibiotics, such as cephalosporin antibiotics, clindamycin, erythromycin and so on.

Non-steroidal anti-inflammatory drugs (NSAIDs)

NSAIDs are preferred for simple joint involvement, which can relieve the symptoms of redness, swelling, heat and pain in the joints during the acute active phase.

Aspirin is often used, but drugs such as ibuprofen, indomethacin, and loxoprofen are also available.

Be aware of the risk of gastrointestinal ulceration, bleeding, and perforation.

Avoid taking two or more NSAIDs at the same time.

It only relieves the symptoms of joint pain, redness and swelling, and does not fundamentally control or prevent exacerbation of the disease.

Glucocorticoids

Suitable for patients who have developed carditis with heart failure and patients with severe chorea who have not responded to other treatments, and should be applied strictly under the supervision of a doctor.

Prednisone, methylprednisolone, dexamethasone, hydrocortisone, etc. can be used.

If the clinical symptoms are relieved and stabilized, the dosage can be gradually reduced or stopped according to the doctor’s advice.

Long-term use of glucocorticosteroids should be aware of the adverse effects of infection, osteoporosis, elevated blood glucose, elevated blood pressure and hypokalemia.

Other treatments

Treatment of chorea: Valproic acid is preferred on the basis of the above treatment. For patients in whom this drug is ineffective or who have severe chorea (e.g., paralysis), treatment with risperidone may be used. Patients with moderate to severe chorea should be referred to a pediatric neurologist or movement disorder specialist for treatment guidance.

Treatment of congestive heart failure: low-salt diet, restriction of water intake, and administration of oxygen inhalation, diuretics, and vasodilators as needed.

Treatment of severe heart valve disease: Valve surgery is required when heart failure due to regurgitation from severe valve disease cannot be managed by medical treatment alone.

Treatment of rash: The rash of acute rheumatic fever is usually temporary and does not require specific management, but if accompanied by itching, antihistamines may be used to reduce itching.

Prognosis

Cure

Most patients recover within 8 to 12 weeks with standardized treatment.

The prognosis is poor for those with significant cardiac involvement at the time of the initial attack, multiple recurrences, or complications of heart failure, which can be life-threatening.

Hazards

Symptoms such as fever, joint swelling and pain, fatigue, and involuntary muscle movement often occur and can seriously affect normal work and life.

Rheumatic fever can lead to varying degrees of heart damage, especially valve lesions are the most significant, easy to recur, further affecting physical health, and bringing a huge psychological and economic burden to themselves and their families.

If glucocorticoids are taken for a long period of time, serious adverse effects such as infection, osteoporosis, hypokalemia, hypertension, hyperglycemia and hyperlipidemia may occur.

Daily life

Daily life

Diet

Eat more fresh vegetables, fruits, lean meat and other high-protein, high-vitamin and easy-to-digest foods.

Avoid spicy and stimulating foods, such as wine, strong tea, coffee, raw garlic, ginger, chili peppers and curry.

Avoid high sugar diet, such as drinks, candies, snacks, etc., to avoid blood sugar fluctuation and inflammation exacerbation.

When combined with hypertension, hyperlipidemia and cardiac insufficiency, low-salt diet should be adopted, avoid eating fried and pan-fried food, eat regularly and avoid overeating.

Lifestyle habits

During the acute active period of the disease, bed rest should be appropriate.

After the symptoms are basically controlled, you should get out of bed as early as possible, and you can choose slow walking, radio gymnastics and other sports.

Exercise should be gradual, avoid strenuous exercise.

Observe the weather changes and increase clothing appropriately in cold weather.

Minimize outdoor activities or work in cold weather.

Wear warm hats, masks, gloves and warm socks to keep warm.

Use warm water to wash your hands and feet.

Keep your skin clean.

Clothing is recommended to choose cotton fabrics and avoid chemical fibers to avoid skin damage.

Avoid going to places where people gather.

Psychological care

Family members should give sufficient care and comfort, and say more active, positive and encouraging words.

Encourage the patient to take care of himself/herself or participate in activities within his/her ability.

Regular follow-up

Follow the doctor’s prescribed time for follow-up consultation.

Seek timely medical attention for any changes in the condition

If symptoms worsen or new symptoms appear, consult the doctor promptly.

Prevention

Rheumatic fever can be prevented by primary and secondary prevention to minimize the occurrence of rheumatic fever and rheumatic heart disease as well as to prevent the progression of the disease.

Primary prevention

Strengthen health care and health education for children and adolescents, and establish a rapid and effective medical security system.

Improve the socio-economic situation.

Improve the living environment and avoid dense population.

Prevent malnutrition, carry out physical exercise, strengthen physical fitness and improve resistance to disease.

Protect against cold and damp, actively prevent upper respiratory tract infections, and thoroughly treat acute and chronic foci of streptococcal infections.

Vaccination with effective anti-streptococcal vaccine is recommended.

Secondary prevention

Prevention of recurrence of rheumatic fever or secondary rheumatic heart disease.

Prophylaxis with long-term antibiotics.

The duration of prophylactic antibiotic use depends on the risk of recurrence of acute rheumatic fever versus disease severity.

In children, patients with a predisposition to recurrent attacks of rheumatic fever or previous carditis with residual valve disease, the duration of prophylaxis should be as long as possible, usually until at least 10 years after the first attack with no recurrences, or until the age of 40 years, or even for the rest of one’s life.

In adults with a history of carditis without residual valve disease, the duration of prophylaxis should be at least 10 years, and in children, prophylaxis should be given until the age of 21 years.

For patients without carditis, the duration of prophylaxis may be slightly shorter, at least 5 years for adults and up to 21 years of age for children.