Rheumatic fever (RF) is a systemic autoimmune disorder related to prior streptococcal infection and is the leading cause of heart disease in those under the age of 40 years living in developing nations.

A. The incidence of RF and prevalence of rheumatic heart disease vary substantially among countries. In many developing countries, the incidence of acute RF approaches or exceeds 200 per 100,000, whereas in the Unites States, it is estimated to be less than 1 per 100,000. Since the first half of this century, there has been a
gradual decline in the incidence of RF in the United States, Japan, and most European countries. This is due to improved public health and living conditions, the development of modern antibiotics, as well as a shift in the endemic strains of group A streptococcus (GAS). Localized outbreaks of RF have occurred in the United States as recently as the mid-1980s.

B. RF is more common among populations at high risk for streptococcal pharyngitis, such as military recruits, those in close contact with school-aged children, and persons of low socioeconomic status. It most commonly occurs between the ages of 5 and 18 years. RF affects both sexes equally, except for Sydenham’s chorea, which is more prevalent in females after puberty.

The clinical manifestations of RF develop 3 weeks after a GAS tonsillopharyngitis. It is important to note that one-third of patients with RF do not remember having had a sore throat. Patients with RF present initially with a sudden onset of constitutional symptoms, including fever (101° to 104°), malaise, weight loss, and pallor. An exudative and proliferative inflammatory process involving collagen fibrils characterizes the acute phase of RF. Multiple organ systems, such as the dermis, central nervous system, synovium, and heart, may be involved. In addition, manifestations may include serositis and involvement of the lungs, kidneys, and central nervous system.

1. The Jones criteria are designed to aid in the diagnosis of the first episode of RF. It can be diagnosed when a previous upper airway infection with GAS is detected in conjunction either with two major manifestations or with one major and two minor manifestations. Major manifestations include arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules. Minor manifestations include fever, arthralgias, high C-reactive protein (CRP) level or high erythrocyte sedimentation rate (ESR), and a prolonged PR interval on electrocardiogram (ECG) (Table 20.1).

2. In some circumstances, the diagnosis of RF can be made without strict adherence to Jones criteria, as in cases of indolent or recurrent carditis or isolated cases of chorea when other causes have been excluded.

B. Major manifestations (Table 20.1)

1. Carditis. This is the most serious and is often regarded as the most specific manifestation of RF, affecting 41% to 83% of patients. It may manifest
as pancarditis affecting the endocardium, myocardium, and pericardium simultaneously.

a. Cardiac involvement ranges from an asymptomatic presentation to progressive congestive heart failure and death.

b. The most typical manifestations include increased heart rate, rhythm disturbances, new murmurs or pericardial friction rub, cardiomegaly, and heart failure.

c. Heart failure is rare in the acute phase; if present, it is usually the result of myocarditis.

d. The most characteristic component of rheumatic carditis is a valvulitis (endocarditis) involving the mitral and aortic valves.

(1) Mitral insufficiency is the hallmark of rheumatic carditis. Aortic insufficiency is less common and is almost always associated with mitral insufficiency. As a rule of thumb, patients under the age of 30 years tend to present with isolated mitral regurgitation, whereas patients develop mitral stenosis during the third decade, with mixed mitral valve disease predominating thereafter.

(2) Acute mitral valve regurgitation produces an apical systolic murmur that may be accompanied by a mid-diastolic Carey Coombs murmur of relative mitral stenosis (a high-pitched early diastolic murmur that varies from day to day). Right-sided valves are rarely involved.

(3) Those valvular lesions that are diagnosed by echocardiogram but are clinically silent usually heal without scarring and have a good prognosis. Controversy exists whether echocardiographic findings of mitral regurgitation or aortic insufficiency constitute subclinical rheumatic carditis sufficient to meet the Jones criteria.

e. Pericarditis may cause chest pain, friction rubs, and distant heart sounds but is often clinically silent.

2. Arthritis. This is the most common manifestation of RF but is the least specific. It occurs in 80% of patients and is described as painful, asymmetric, migratory, and transient. It involves large joints, such as the knees, ankles, elbows, wrists, and shoulders. It is more common in older patients and improves markedly with the use of salicylates within 48 hours of treatment. Monoarthritis, oligoarthritis, and involvement of small joints of the extremities are less common. However, arthritis of the first metatarsophalangeal joint, enthesopathy, and axial involvement, especially of the cervical spine, have also been reported. Arthritis of RF is benign and self-limiting (lasting 2 to 3 weeks) and does not result in permanent sequelae. Inflammatory changes without signs of infection are seen in the joint fluid.

3. Sydenham’s chorea. Also known as Saint Vitus’ dance or chorea minor, this extrapyramidal disorder is characterized by purposeless and involuntary movements of face and limbs, muscular hypotonia, and emotional lability.

a. Initial manifestations include difficulty in writing, talking, or walking. Handwriting may deteriorate; speech may change to an explosive and halting tone; and the patient may become uncoordinated and easily frustrated.

b. Symptoms tend to be more evident when the patient is under stress or awake and usually disappear during sleep.

c. Sydenham’s chorea is a delayed manifestation of RF, usually appearing 3 months or more after an upper airway infection; it is often the sole manifestation of acute RF. Chorea has been reported in up to 30% of the patients. Most cases tend to follow a benign course, with complete resolution of symptoms in 2 to 3 months, although cases in which symptoms persisted for > 2 years have been reported.

d. It is important to differentiate the symptoms of Sydenham’s chorea from tics, athetosis, conversion reactions, hyperkinesia, and behavioral abnormalities.

4. Subcutaneous nodules. These usually measure 0.5 to 2 cm and are firm, painless, and freely mobile nodules that can be isolated or found in clusters over the extensor surfaces of joints (knees, elbows, and wrists), bony prominences, tendons, dorsum of foot, occipital region, and cervical processes. They are seen in up to 20% of patients with RF and last for a few days. The skin overlying the nodules is freely mobile and shows no signs of discoloration or inflammation.

5. Erythema marginatum. This is an evanescent erythematous macular rash with a pale center of irregular shape. It is usually nonpruritic and tends to disappear after a few days. It is highly specific, occurring in < 5% of patients, and is obvious only in fair-skinned individuals. The lesions vary in size and affect mainly the trunk, abdomen, and inner aspect of arms and thighs, but not the face. The rash may be induced by application of heat. Its presence is suggestive of coexisting carditis.

C. Minor manifestations. Fever and arthralgias are common, but nonspecific findings of RF that can be used to support the diagnosis of RF when only a single major manifestation is present (Table 20.1).

1. Fever is encountered during the acute phase of the disease and does not follow a specific pattern.

2. Arthralgia is defined as pain in one or more large joints without objective findings of inflammation on physical examination.

3. Other clinical manifestations of RF include abdominal pain, epistaxis, acute glomerulonephritis, rheumatic pneumonitis, hematuria, and encephalitis. These are not included as diagnostic criteria for the diagnosis of RF.

A. The association between tonsillopharyngitis-scarlet fever epidemics and acute RF in the 1930s, the findings of high levels of antistreptolysin O (ASO) in sera of patients with RF, and the confirmation of antibiotics as an efficient mode of prophylaxis of RF provide strong evidence that GAS is the agent causing initial and recurrent attacks of RF.

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