| disease | Rheumatic Fever |
| alias | Bi Disease, Rheumatic Fever |
Rheumatic fever is a common recurrent acute or chronic systemic connective tissue inflammation, primarily affecting the heart, joints, central nervous system, skin, and subcutaneous tissues. The main clinical manifestations are carditis and arthritis, which may be accompanied by fever, toxemia, rash, subcutaneous nodules, and chorea. During acute episodes, arthritis is usually more prominent, but at this stage, rheumatic carditis can lead to patient death. After acute episodes, varying degrees of cardiac damage often remain, with valvular lesions being the most significant, leading to chronic rheumatic heart disease or rheumatic valvular disease.
bubble_chart Epidemiology
Acute wind-dampness can occur at any age but is extremely rare in infants under 3 years old. It is most common in children and adolescents aged 5–15, with roughly equal incidence between males and females. Recurrence mostly happens within 3–5 years after the initial onset, with a recurrence rate as high as 5–50%, particularly among those with cardiac involvement. Epidemiological studies indicate that, on average, about 3% of patients develop acute wind-dampness fever following streptococcal pharyngitis. The susceptible age, regional distribution, incidence, and severity of acute wind-dampness fever reflect the rate and severity of streptococcal infections. After streptococcal infection, the incidence of acute wind-dampness is directly correlated with the intensity of the immune response triggered by Group A streptococcus. Various environmental factors (geography, humidity, season, etc.), economic status, and age can influence the incidence of wind-dampness fever.
The prevalence of wind-dampness fever and rheumatic heart disease has significantly declined over the past 30 years, closely linked to improvements in socioeconomic conditions (housing and economic status) and the widespread adoption of preventive measures. In China, the incidence is higher in the Northeast and North China regions, followed by East, Central, Southwest, and Northwest China, with the lowest rates in South China. Outbreaks are more frequent in cold winter and early spring, with cold and dampness being major precipitating factors. The proportion of acute wind-dampness fever among internal medicine inpatients has decreased from 2.49% in 1958 to 0.86% in recent years. At Zhongshan Hospital and Huashan Hospital affiliated with Shanghai Medical University, analyses of hospitalized cardiac patients in 1948–1957, 1958–1968, and 1969–1979 showed that this disease accounted for 50.3%, 40.8%, and 29.95% of cases, respectively. This indicates that wind-dampness fever remains a disease requiring active prevention and treatment in China today.The etiological relationship between Group A streptococcus and wind-dampness heat or rheumatic heart disease has been supported by some indirect evidence from clinical, epidemiological, and immunological studies. Numerous clinical and epidemiological studies have shown a close association between Group A streptococcal infection and wind-dampness heat. Immunological research has also confirmed that a history of prior streptococcal infection exists before the acute onset of wind-dampness heat. Prospective long-term follow-up studies indicate that antibacterial treatment and prevention of streptococcal infection can predict the initial onset and recurrence of Saposhnikovia Root dampness-heat. Additionally, the route of infection is crucial, as streptococcal pharyngeal infection is a necessary condition for the onset of wind-dampness heat.
Nevertheless, the mechanism by which Group A streptococcus causes wind-dampness heat remains unclear to this day. Wind-dampness heat is not directly caused by streptococcal infection, as its onset does not occur during the acute phase of streptococcal infection but rather 2–3 weeks afterward. In wind-dampness Rebing individuals, Group A streptococcus has never been isolated from blood cultures or cardiac tissues. Moreover, only 1–3% of patients develop wind-dampness heat after streptococcal pharyngitis.In recent years, it has been discovered that the cell wall of Group A streptococcus contains a layer of proteins composed of M, T, and R proteins, with the M protein being the most significant. It not only impedes phagocytosis but also serves as the basis for bacterial typing and is referred to as the "cross-reactive antigen." Additionally, within the polysaccharide components of the streptococcal cell wall, there is a specific antigen known as "C substance." Following streptococcal infection, some individuals may produce corresponding antibodies that act not only on the streptococcus itself but also on the heart valve membrane, leading to valvular lesions. The mucopolysaccharide composition of the heart valve membrane varies with age, which may explain the differing incidence of valvular lesions between adolescents and adults. Immunological studies suggest that acute wind-dampness heat involves defects in immune regulation, characterized by an increase in B-cell and helper T-cell counts and a relative decrease in suppressor T-cells, resulting in enhanced humoral and cellular immunity. Although chronic rheumatic heart disease shows no active wind-dampness heat, persistent elevation in B-cell counts indicates that the immune-inflammatory process is still ongoing. Whether wind-dampness heat develops after streptococcal infection is also related to the individual's reactivity. This reactivity correlates, on one hand, with the quantity of antibodies produced against streptococcal antigens—higher antibody levels increase the likelihood of hypersensitivity reactions—and on the other hand, with changes in the functional state of the nervous system.
bubble_chart Pathological ChangesRheumatic fever is a systemic inflammatory disease of connective tissue, with joint and cardiac involvement being most common in the early stages, and cardiac damage becoming the most significant later. According to the progression of the disease, it can be divided into the following late stage [third stage].
(1) Degenerative exudative phase: Collagen fibers in the connective tissue split and swell, forming hyaline and fibrinous degeneration. Around the lesions, inflammatory cells such as lymphocytes, plasma cells, eosinophils, and neutrophils infiltrate. This phase can last 1–2 months, followed by recovery or progression to the second or late stage [third stage].
(2) Proliferative phase: The hallmark of this phase is the appearance of rheumatic granulomas or Aschoff bodies on the basis of the aforementioned lesions. These are characteristic pathological changes of rheumatic fever and serve as the basis for pathological diagnosis and indicators of rheumatic activity. The center of the Aschoff body contains fibrinous necrosis, surrounded by lymphocytes, plasma cells, and rheumatic cells. Rheumatic cells are round, oval, or polygonal, with abundant basophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Occasionally, binucleated or multinucleated giant cells form, leading to the sclerotic phase. This phase lasts about 3–4 months.
(3) Sclerotic phase: The degenerative necrotic material in the center of the Aschoff body is gradually absorbed, inflammatory cell infiltration decreases, and fibrous tissue proliferates, forming scar tissue at the granuloma site.
Due to the recurrent nature of the disease, the progression of the aforementioned late stage [third stage] may overlap, lasting approximately 4–6 months. The initial stage [first stage] and intermediate stage [second stage] are often accompanied by serous exudation and inflammatory cell infiltration, which largely determine the clinical symptoms. In joints and pericardium, the pathological changes are primarily exudative, while scar formation is mainly confined to the endocardium and myocardium, particularly the valve membranes.
The inflammatory lesions of rheumatic fever affect the collagen fibers of connective tissue throughout the body, initially involving joints and the heart, with cardiac damage becoming predominant later. The pathological changes vary by organ: joints and pericardium are mainly exudative, leading to arthritis and pericarditis. Later, the exudate may be completely absorbed, though incomplete absorption in the pericardium can lead to adhesions. In the myocardium and endocardium, proliferative changes dominate, eventually forming fibrous scars. Proliferative changes and adhesions in the valve membranes often result in chronic rheumatic valvular disease.
The pathological changes in various organs and tissues are described as follows:
1. **Heart**: Almost every patient with rheumatic fever recurrence has cardiac involvement. Grade I cardiac damage may not lead to chronic rheumatic heart disease. Acute rheumatic carditis can affect the endocardium, myocardium, and pericardium, resulting in pancarditis, with myocarditis and endocarditis being the most significant. Typical Aschoff bodies are widely distributed in the myocardium, primarily in the perivascular connective tissue. Endocarditis mainly affects the valve membranes, causing congestion, swelling, and thickening, with small vegetations forming on the surface, leading to valvular insufficiency. Fibrin deposition at the valve coaptation sites can cause adhesions, and combined with shortening of chordae tendineae and papillary muscles, this leads to valvular deformity, eventually resulting in stenosis. The pericardial cavity may develop fibrinous or serofibrinous exudates.
After the active phase, milder cases may fully recover, but in most patients, valvular deformity and myocardial or pericardial scarring result in chronic inactive heart disease, with valvular lesions being the most prominent. Early valvular defects primarily cause regurgitation, while mitral stenosis typically takes over two years to develop, and aortic stenosis requires an even longer period.
2. Arthritis The synovial membrane of the joint and surrounding tissues show edema, with mucoid degeneration, fibrinoid necrosis, and inflammatory cell infiltration in the sub-synovial connective tissue. Sometimes, atypical wind-dampness bodies are present. Since the exudate usually contains little fibrin, it is easily absorbed and generally does not cause adhesions. After the active phase, sequelae such as joint stiffness or deformity do not occur.
3. Subcutaneous nodules Degeneration and necrosis of subcutaneous connective tissue, splitting of collagen fibers, infiltration of basal cells and lymphocytes, forming granulomas that fuse into nodules. This is an important sign indicating wind-dampness activity, but is only seen in 10% of cases.
4. Arterial lesions Can affect all layers of the arterial wall, leading to thickening of the arterial wall and predisposing to thrombosis. Commonly seen in coronary arteries as well as arteries in the kidneys, pancreas, mesentery, lungs, and brain.
5. Pulmonary lesions May present as irregular grade I consolidation in the lungs, with inflammatory cell exudation in the pulmonary interstitium and alveoli of the consolidated areas. Lesions are mostly distributed around small blood vessels.
6. Brain lesions Congestion of small blood vessels in the brain parenchyma, with infiltration of lymphocytes, plasma cells, and a tendency to form small nodules around small blood vessels. These nodules are distributed in the striatum, substantia nigra, and cerebral cortex. When lesions in the striatum are prominent, clinical manifestations of chorea often occur.
Other conditions, such as rheumatic pleuritis or peritonitis, may also occasionally occur.
bubble_chart Clinical Manifestations
Most patients have a history of upper respiratory tract infections such as pharyngitis or tonsillitis 1 to 5 weeks before the onset of the disease. At the onset, there is general fatigue, loss of appetite, and dysphoria. The main clinical manifestations include: fever, arthritis, carditis, subcutaneous nodules, erythema marginatum, and chorea.
(1) Fever Most patients have irregular grade I or grade II fever, but some may present with remittent fever or persistent low-grade fever. The pulse rate increases, and there is profuse sweating, often disproportionate to the body temperature.
(2) Arthritis The typical manifestation is migratory polyarthritis, often symmetrically involving large joints such as the knees, ankles, shoulders, wrists, elbows, and hips. Local inflammation presents as redness, swelling, heat, and pain, but without suppuration. Some patients may experience simultaneous onset in several joints, and small joints of the hands and feet or spinal joints may also be affected. It usually occurs within one month after streptococcal infection, and the titer of anti-streptococcal antibodies is often elevated. After the acute inflammation subsides, joint function fully recovers without leaving stiffness or deformity, but recurrence is common. Typical cases have become rare in recent years. The degree of local joint inflammation is not significantly related to the presence of carditis or valvular lesions.
(3) Carditis This is the most clinically significant manifestation, with 65–80% of affected children having cardiac involvement. Acute rheumatic carditis is the most common cause of congestive heart failure in childhood.
1. Myocarditis The earliest clinical manifestation of acute rheumatic myocarditis is murmurs of the mitral and aortic valves, caused by valvular regurgitation. These murmurs may occur alone or simultaneously, with mitral valve murmurs being the most common. Mild localized myocarditis may not present obvious clinical symptoms. Diffuse myocarditis may present with clinical symptoms of pericarditis and congestive heart failure, such as precordial discomfort or pain, palpitations, dyspnea, and edema. Common signs include:
(1) Tachycardia: The heart rate is often 100–140 beats per minute, disproportionate to the elevated body temperature. Salicylates may reduce fever but may not normalize the heart rate.
(2) Cardiomegaly: The apical impulse is diffuse and weak, and the cardiac dullness border is enlarged.
(3) Changes in heart sounds: Gallop rhythm is often heard, and the first heart sound is weakened, resembling fetal heart sounds.
(4) Heart murmurs: A blowing systolic murmur may be heard at the apex or aortic valve area. Sometimes, a mild rumbling diastolic murmur may be present at the apex, mainly caused by relative mitral stenosis due to cardiac enlargement. After the acute inflammation subsides, these murmurs may lessen or disappear.
(5) Arrhythmias and ECG abnormalities: Premature beats, tachycardia, varying degrees of atrioventricular block, and paroxysmal atrial fibrillation may occur. Prolongation of the PR interval is the most common ECG finding. Additionally, there may be ST-T wave changes, prolonged QT interval, and intraventricular conduction block.
(6) Heart failure: Heart failure caused by acute rheumatic fever is often due to acute rheumatic myocarditis, especially in younger patients, where the condition is severe and manifests as dyspnea, pale complexion, hepatosplenomegaly, and edema. In adults, heart failure often occurs on the basis of chronic valvular disease.
It is noteworthy that most patients with rheumatic myocarditis do not exhibit obvious cardiac symptoms. When chronic valvular lesions appear, there is no clear history of rheumatic fever. Rebing
2. Endocarditis is extremely common pathologically. It often involves the endocardium of the left atrium, left ventricle, and valve membranes, with the mitral valve membrane being the most frequently affected, followed by the aortic valve, while the tricuspid and pulmonary valves are rarely involved. Almost all cases of myocarditis exhibit manifestations of endocardial involvement. The symptoms appear later than those of myocarditis. Clinically, a grade I systolic murmur at the cardiac apex is mostly functional and may be secondary to myocarditis, fever, anemia, or other factors. The murmur diminishes or disappears after the rheumatic fever activity is controlled. In cases of organic mitral regurgitation, a rougher systolic murmur of grade II or higher is heard at the cardiac apex, with a higher pitch that radiates to the axilla, accompanied by a weakened first heart sound. A soft, short, low-pitched mid-diastolic murmur (Carey Coombs murmur) may be present at the cardiac apex, caused by left ventricular dilation, relative mitral stenosis, valve leaflet edema, or rapid blood flow through the mitral orifice. In aortic regurgitation, a blowing diastolic murmur is heard at the left sternal border in the 3rd to 4th intercostal spaces, radiating to the cardiac apex, along with water-hammer pulse and other peripheral vascular signs. Diastolic murmurs at the aortic valve area are less common and often do not disappear after rheumatic fever episodes.
3. Pericarditis occurs during the active phase of wind-dampness heat and coexists with myocarditis, representing a severe manifestation of carditis. Clinical features include precordial pain and audible pericardial friction rub, lasting from a few days to 2–3 weeks. When pericardial effusion occurs, the fluid volume is generally small. X-ray reveals an enlarged, flask-shaped cardiac shadow. Electrocardiography shows ST-segment elevation in precordial leads. Echocardiography demonstrates a hypoechoic area behind the epicardium of the left ventricular posterior wall. After absorption of the exudate, the serous membrane may exhibit adhesions and thickening, but cardiac function remains unaffected. Clinically, no significant sequelae are observed, and progression to constrictive pericarditis is exceedingly rare.
(IV) Cutaneous Manifestations
1. Exudative Type: May present as urticaria, maculopapular rash, erythema multiforme, erythema nodosum, or annular erythema, with annular erythema being the most common and diagnostically significant. It typically appears on the inner limbs and trunk as faint red circular halos, initially small but rapidly expanding outward. The edges are grade I elevated, while the skin within the ring remains normal in color. Sometimes, the lesions merge to form garland-like patterns. The erythema may come and go, is non-pruritic and non-indurated, blanches on pressure, and can persist for months.
2. Proliferative Type: Subcutaneous nodules. These nodules are pea-sized, vary in number, are firm and non-tender, and commonly located over bony prominences or tendon attachments such as the elbows, knees, wrists, ankles, extensor surfaces of finger (toe) joints, occiput, forehead, and spinous processes. They are not adherent to the skin. Often, multiple nodules cluster symmetrically, usually resolving spontaneously within 2–4 weeks but may persist for months or recur intermittently. Subcutaneous nodules are associated with severe carditis and indicate active wind-dampness.
(V) Chorea Occurs predominantly in children aged 5–12, more frequently in females than males. It typically develops 2–6 months after streptococcal infection, resulting from wind-dampness heat inflammation affecting the central nervous system, including the basal ganglia, cerebral cortex, cerebellum, and striatum. Onset is insidious. Clinical manifestations include:
1. Psychiatric Abnormalities: Initial symptoms often include emotional instability, irritability, and impaired comprehension and memory.
2. Involuntary Movements: Facial manifestations include grimacing, head-shaking, neck-twisting, and tongue-protrusion; limb movements involve arrhythmic alternating actions such as extension and flexion, adduction and abduction, pronation and supination, more pronounced in the upper limbs than the lower. Symptoms worsen with stress or fatigue and disappear during sleep.
3. Muscle Weakness and Ataxia: Reduced muscle tone, diminished or absent tendon reflexes in the limbs. Severe cases may exhibit postural instability, unsteady gait, difficulty swallowing and chewing, and inability to perform daily activities. Chorea may occur alone or accompany other manifestations of wind-dampness heat, such as carditis, but does not coincide with arthritis. Other laboratory findings may be normal.
(VI) Other Manifestations In addition to the classic presentations, wind-dampness heat may occasionally involve other sites, leading to wind-dampness-related pleuritis, peritonitis, or vasculitis, which warrant attention.
bubble_chart Auxiliary Examination
There is no specific laboratory test for wind-dampness heat. Currently, diagnosis is mainly assisted from two aspects: ① confirming previous streptococcal infection; ② clarifying the existence and persistence of wind-dampness activity.
(1) Evidence of streptococcal infection
1. Throat swab culture: Often yields a positive culture for hemolytic streptococcus. However, a positive culture cannot confirm whether it is from a previous infection or a different strain acquired during the course of the disease. For those who have received antibiotic treatment, the throat swab culture may yield a false negative.
2. Measurement of serum hemolytic streptococcal antibodies: Hemolytic streptococcus can secrete various antigenic substances, prompting the body to produce corresponding antibodies. An increase in these antibodies indicates recent hemolytic streptococcal infection. Typically, antibodies increase significantly 2–3 weeks after streptococcal infection, gradually decline after 2 months, and can persist for about 6 months. Commonly measured antibodies include:
(1) Anti-streptolysin O (ASO): >500 units indicates elevation;
(2) Anti-streptokinase (ASK): >80 units indicates elevation;
(3) Anti-hyaluronidase: >128 units indicates elevation;
(4) Others include anti-deoxyribonuclease B (ADNA-B), anti-streptococcal enzyme, and anti-M protein antibody measurements.
(2) Evidence of wind-dampness inflammatory activity
1. Blood routine: White blood cell count increases to grade I–II, with neutrophilia and a left shift; often accompanied by grade I reduction in red blood cell count and hemoglobin levels, presenting as normocytic, normochromic anemia.
2. Non-specific serum component changes: Certain serum components may change in various inflammatory or other active diseases. During the acute or active phase of wind-dampness heat, these also yield positive results. Commonly measured indicators include:
(1) Erythrocyte sedimentation rate (ESR): ESR accelerates, but may not increase in cases of severe heart failure or after anti-wind-dampness treatment with corticosteroids or salicylates.
(2) C-reactive protein: Serum of wind-dampness heat patients contains proteins reactive to C-substance, present in α-globulin. During active wind-dampness, C-reactive protein increases and returns to normal upon remission.
(3) Mucoprotein: Mucoprotein is a chemical component of collagen tissue matrix. During active wind-dampness, collagen tissue is destroyed, leading to increased serum mucoprotein concentration.
(4) Protein electrophoresis: Albumin decreases, while α2 and γ-globulin often increase.
3. Immunological indicators
(1) Positive detection of circulating immune complexes.
(2) Serum total complement and complement C3: Decreases during active wind-dampness.
(3) Immunoglobulins IgG, IgM, IgA: Increase during the acute phase.
(4) B lymphocytes increase, while total T lymphocytes decrease; T suppressor cells significantly decrease, and the ratio of T helper cells to T suppressor cells markedly increases. The reduction in T suppressor cells weakens the body’s inhibition of antigen stimulation, disrupting the immune system's self-tolerance.
(5) Anti-myocardial antibodies: 80% of patients test positive for anti-myocardial antibodies, which can persist for up to 5 years and increase again during relapse.
The combined application of the above tests holds greater diagnostic significance. If both antibodies and specific serum components are positive, it suggests active Bi disease; if both are negative, active Bi disease can be ruled out. Elevated antibodies with negative non-specific serum components indicate a higher likelihood of convalescence or streptococcal infection; if antibodies are normal but non-specific serum components are positive, other conditions should be considered.
To date, there is no specific diagnostic method for wind-dampness heat. Clinically, the revised Jones diagnostic criteria (Table 22-1) are still used, relying primarily on clinical manifestations supplemented by laboratory tests. If a patient exhibits two major manifestations, or one major manifestation plus two minor manifestations, along with evidence of prior streptococcal infection, a diagnosis of wind-dampness heat can be made.
Table 22-1 Revised Jones Diagnostic Criteria (American Heart Association, 1992)
| 1. Carditis | 1. Clinical manifestations | 1. Recent history of scarlet fever |
| (1) Murmur | (1) Previous history of wind-dampness Rebing | 2. Positive throat culture for hemolytic streptococcus |
| (2) Cardiomegaly | (2) Arthralgia | 3. Elevated ASO or other streptococcal antibodies |
| (3) Pericarditis | (3) Fever | |
| (4) Congestive heart failure | ||
| 2. Polyarthritis | 2. Laboratory tests | |
| 3. Chorea | (1) Elevated ESR, positive C-reactive protein, leukocytosis, anemia | |
| 4. Erythema marginatum | (2) Electrocardiogram # : Prolonged PR interval, prolonged QT interval | |
| 5. Subcutaneous nodules |
When applying the above criteria clinically, atypical mild or early cases are prone to fistula disease misdiagnosis. In 1981, the Eighth National Pediatric Congress's Heart Blood Vessel Committee established diagnostic criteria for atypical wind-dampness heat: ① Evidence of streptococcal infection one week before onset: pharyngitis, tonsillitis, or scarlet fever; positive ASO; or positive skin test for hemolytic streptococcal antigen. ② Systemic symptoms: progressive complexion pallor, lack of strength profuse sweating, palpitation, migratory arthralgia, fever lasting more than 2 weeks. ③ Cardiac manifestations: persistent sinus tachycardia without other causes, diminished first heart sound, grade 2 systolic murmur at the apex, or enhanced third heart sound; ECG showing prolonged PR or QT intervals and ST segment changes. ④ Other manifestations: elevated ESR, positive C-reactive protein; effective diagnostic aspirin treatment: aspirin 10mg/kg daily, orally for 3-5 days, resulting in body warm purgation reduction and symptom improvement, with no recurrence of fever during treatment.
In summary, the diagnosis of specific patients must comprehensively consider the condition, analyze it holistically, and perform differential diagnosis carefully, without overemphasizing the above criteria.
bubble_chart Treatment Measures
(1) General Treatment During the active phase of wind-dampness fever, bed rest is essential. If there are obvious signs of cardiac involvement, activity should be controlled until symptoms disappear and the erythrocyte sedimentation rate (ESR) returns to normal. For patients with cardiac enlargement, pericarditis, persistent tachycardia, or significant ECG abnormalities, bed rest should continue for 3–4 weeks after symptoms subside and ESR normalizes. During the convalescence stage, activity should also be appropriately controlled for 3–6 months. Throughout the course of the illness, a diet that is easy to digest and rich in nutrients is recommended.
(2) Anti-wind-dampness Treatment Commonly used medications include salicylates and corticosteroids. Corticosteroids are not necessary for patients without carditis, while salicylates are highly effective for acute arthritis.
1. Salicylates These are the most commonly used drugs for treating acute wind-dampness fever. They are effective in reducing fever, alleviating joint inflammation, and normalizing ESR. Although they significantly suppress inflammation, they do not alter the underlying pathological changes and thus offer no clear preventive effect against cardiac valve damage. Commonly used salicylates include acetylsalicylic acid (aspirin) and sodium salicylate, with aspirin being the most effective. The initial dose of aspirin is: 80–100 mg/kg per day for children and 4–6 g per day for adults, divided into 4–6 oral doses. Sodium salicylate is administered at 6–8 g per day, divided into 4 doses. The dose of salicylates should be gradually increased until satisfactory clinical efficacy is achieved or systemic toxic reactions such as tinnitus, headache, or hyperventilation occur. After symptom control, the dose should be halved and maintained for 6–12 weeks. Salicylates often cause gastric irritation, including nausea, vomiting, and loss of appetite. In such cases, aluminum hydroxide can be used, but sodium bicarbonate should be avoided as it reduces gastrointestinal absorption of salicylates, increases renal excretion, and may trigger or worsen congestive heart failure.
If patients cannot tolerate salicylates, alternatives include: - Chlofenamic acid (anti-wind-dampness ling) 0.2–0.4 g, three times daily; - Benorilate 1.5–4.5 g per day, divided into doses. Benorilate is an ester of aspirin and paracetamol, causing less gastric irritation and slowly releasing salicylic acid into the bloodstream after absorption.
2. Corticosteroids Large clinical studies show no significant difference in efficacy between corticosteroids and aspirin for wind-dampness fever. However, corticosteroids are associated with "rebound" phenomena after discontinuation and more side effects. Therefore, it is generally recommended to start with salicylates for acute wind-dampness fever with cardiac involvement. If the response is inadequate (persistent fever, no improvement in cardiac function), corticosteroids should be added promptly. The initial dose of corticosteroids should be high: prednisone 60–80 mg per day for adults or 2 mg/kg per day for children, divided into 3–4 oral doses, until inflammation is controlled and ESR normalizes. The dose is then gradually reduced, with a maintenance dose of 5–10 mg per day; the total treatment course should last 2–3 months. For severe cases, hydrocortisone 300–500 mg per day or dexamethasone 0.25–0.3 mg/kg per day may be administered intravenously.
After discontinuing corticosteroids, attention should be paid to "rebound" phenomena such as low-grade fever, joint pain, and elevated ESR. To reduce rebound effects, salicylates can be combined before discontinuation, or adrenocorticotropic hormone (ACTH) 12.5–25 mg can be infused once daily for three consecutive days.
(3) Antibiotic Treatment Once wind-dampness fever is diagnosed, a course of penicillin treatment should be administered even if the throat swab culture is negative, to eliminate hemolytic streptococci. Persistent or recurrent hemolytic streptococcal infections can progressively worsen wind-dampness fever. Therefore, eradicating streptococcal infection is an essential measure in treating wind-dampness fever. Typically, procaine penicillin 400,000–800,000 units is administered intramuscularly once daily for 10–14 days, or oxacillin sodium 1.2 million units is given as a single intramuscular injection. For patients allergic to penicillin, oral erythromycin can be prescribed at 0.5g four times daily for 10 days.
(4) Treatment with Chinese Medicine Acute wind-dampness heat mostly belongs to heat arthralgia and should be treated with methods of dispelling wind, clearing heat, and resolving dampness; chronic wind-dampness heat mostly belongs to agonizing arthralgia and should be treated with methods of dispelling wind, dispersing cold, and transforming dampness. Adjunctive treatment with glucocorticoids, salicylate preparations, and Chinese medicine may yield better therapeutic effects. Acupuncture therapy also has a certain effect in alleviating joint symptoms.
(5) Treatment of Chorea Anti-wind-dampness drugs are ineffective for chorea. Patients with chorea should be placed in a quiet environment as much as possible to avoid stimulation. For severe cases, sedatives such as Luminal or diazepam (Valium) can be used, and sleep therapy may also be employed. Chorea is a self-limiting disease, usually without significant neurological sequelae. With patient and meticulous care, appropriate physical activity, and medication, most cases can achieve good outcomes.
In the initial episode of acute wind-dampness heat, 75% of patients recover within 6 weeks, and by 12 weeks, 90% recover. Only 5% of patients have wind-dampness activity persisting beyond 6 months. Patients with prolonged wind-dampness activity often suffer from severe and stubborn carditis or chorea. Recurrences commonly occur after repeated streptococcal infections, with about 20% of patients experiencing a relapse within the first 5 years after the initial onset. The recurrence rate drops to 10% in the second five-year period and further declines to 5% in the third five-year period. The prognosis of acute wind-dampness heat depends on the severity of cardiac involvement, the frequency of recurrences, and the treatment measures. Severe carditis, frequent recurrences, improper or deficient treatment can lead to death due to grade III or refractory heart failure, subacute infective endocarditis, or the development of chronic wind-dampness valvular heart disease.
Rheumatic fever is a preventable disease that is closely related to streptococcal infections. Therefore, preventing streptococcal epidemics is one of the most critical measures in preventing rheumatic fever.
(1) Prevention of initial rheumatic fever: ① Prevent upper respiratory infections by improving living conditions and hygiene, engaging in regular physical exercise, and enhancing overall health. ② For acute streptococcal infections such as scarlet fever, acute tonsillitis, pharyngitis, otitis media, and lymphadenitis, early and thorough antibiotic treatment should be administered, with penicillin as the first choice. Erythromycin can be used for those allergic to penicillin. ③ For chronic tonsillitis with recurrent acute episodes (occurring twice or more per year), surgical removal of the tonsils is recommended. Penicillin should be used to prevent infection from one day before surgery until three days after. Even after tonsillectomy, streptococcal pharyngitis may still occur and should be treated promptly. ④ In closed communities (such as military camps, schools, and kindergartens), measures should be taken to prevent, detect early, and diagnose streptococcal infections promptly. Establishing necessary healthcare systems can help eliminate streptococcal epidemics and significantly reduce the incidence of rheumatic fever.
(2) Prevention of rheumatic fever recurrence: Patients who have previously suffered from rheumatic fever should actively prevent streptococcal reinfection. Generally, benzathine penicillin (long-acting penicillin) at 1.2 million units, administered intramuscularly once a month, is recommended. For those allergic to penicillin, sulfadiazine or sulfisoxazole can be used, with children taking 0.25–0.5 g daily and adults taking 0.5–1.0 g daily, divided into doses. The duration of preventive medication is as follows: - Patients under 18 years of age must continue preventive medication. - Patients over 18 without cardiac involvement should maintain preventive medication for at least five years after the last episode of rheumatic fever. - Patients with cardiac involvement are highly susceptible to rheumatic reactivation and carditis upon reinfection, necessitating strict preventive treatment. Studies show that the level of preventive medication is inversely proportional to the rate of streptococcal infection. The infection rate in those without or with irregular preventive medication is three times higher than in those with complete preventive medication. Notably, the rate of rheumatic reactivation in the non-preventive or irregular preventive group is ten times higher than in the complete preventive group. Even irregular preventive medication provides some benefit.
(1) Other diseases causing arthritis
1. Rheumatoid arthritis: It is a polyarticular symmetrical arthritis affecting small joints such as fingers and palms, as well as spondylitis. Its characteristics include "morning stiffness" and spindle-shaped swelling of fingers. In the late stage [third stage], joint deformities appear. Clinically, cardiac damage is less common, but echocardiography can detect pericardial lesions and valvular damage early. X-rays show destruction of joint surfaces, narrowing of joint spaces, and osteoporosis in adjacent bone tissues. Serum rheumatoid factor is positive, and immunoglobulins IgG, IgM, and IgA are elevated.
2. Migratory arthritis caused by septicemia: Often presents with signs of primary infection. Blood and bone marrow cultures are positive, and joint effusions tend to be purulent, with pathogens detectable.
3. Tuberculous arthritis: Mostly involves a single joint, commonly affecting frequently active, friction-prone, or weight-bearing joints such as the hip, thoracic spine, lumbar spine, or knee. Joint pain occurs without redness or swelling, and there is no cardiac involvement. Other sites often have tuberculous lesions. X-rays show bone destruction, and erythema nodosum may appear. Anti-rheumatic treatment is ineffective.
4. Tuberculous infection-allergic arthritis (Poncet disease): There is a definite tuberculous infection focus in non-joint areas of the body, with recurrent arthritis manifestations but generally good health. X-rays show no bone destruction. Salicylate treatment can alleviate symptoms, but relapses occur. Symptoms resolve after anti-tuberculous treatment.
5. Lymphoma and granuloma: Reports indicate that leukemia can present with fever and acute polyarthritis in 10% of cases, with arthritis symptoms sometimes appearing before changes in peripheral blood counts, leading to misdiagnosis. Similar reports exist for other lymphomas and benign granulomas.
6. Lyme arthritis (Lyme disease): This is an epidemic disease transmitted by ticks. Symptoms usually appear 3–21 days after a tick bite. Clinical manifestations include fever, chronic migratory skin erythema, recurrent asymmetric arthritis (affecting large joints), and possible cardiac damage, often affecting the conduction system. ECG shows varying degrees of atrioventricular block. Neurological symptoms such as chorea, meningoencephalitis, myelitis, and facial nerve paralysis may also occur. Laboratory tests show positive circulating immune complexes and elevated ESR. Serum-specific antibody tests can aid in diagnosis.
(2) Subacute infective endocarditis
More common in individuals with pre-existing valvular disease. Symptoms include progressive anemia, splenomegaly, petechiae, ecchymoses, and clubbing of fingers. Vegetations may be found on valves in the brain, kidneys, or lungs.
(3) Viral myocarditis
Often preceded by or concurrent with respiratory or enteric viral infections. The main affected area is the myocardium, occasionally the pericardium, and rarely the endocardium. Fever duration is short, and arthralgia may occur without arthritis. The first heart sound at the apex is diminished, and a grade II systolic murmur is present. Arrhythmias are common. No annular erythema or subcutaneous nodules are observed. Lab tests show leukopenia or normal counts, with normal ESR, ASO, and C-reactive protein. Complement fixation tests and neutralizing antibodies are positive. Viral isolation is possible via myocardial biopsy.
(4) Post-streptococcal infection state (streptococcal infection syndrome)
Low-grade fever, weakness, joint soreness, elevated ESR, and positive ASO appear during or 2–3 weeks after acute streptococcal infection. ECG may show transient premature beats or grade I ST-T changes, but no cardiac enlargement or significant murmurs. Symptoms resolve quickly after antibiotic treatment and do not recur.
(5) Systemic lupus erythematosus
This disease presents with arthralgia, fever, carditis, and kidney lesions, resembling rheumatic fever. However, symmetrical facial butterfly rash, leukopenia, negative ASO, and the presence of lupus cells in blood or bone marrow smears aid in diagnosis.
