Rheumatic Fever
B. Denise Hemby and Theresa M. Campo
Definition
A.Rheumatic fever (RF) is an autoimmune inflammatory process that occurs as sequelae of a group A beta-hemolytic streptococcal (GAS) tonsillopharyngitis. RF is a preventable disease through the detection and adequate treatment of streptococcal pharyngitis. It is characterized by nonsuppurative inflammatory lesions of heart, joints, central nervous system (CNS) and subcutaneous tissue. RF is a clinical syndrome with no specific diagnostic test and varying manifestations making a definitive diagnosis difficult. It consists of exudate and proliferative inflammatory lesions found in connective tissue involving the joints, blood vessels, heart, and subcutaneous tissue:
1.Revised Jones Criteria, low-risk populations:
a.Major criteria: Carditis (clinical and/or subclinical), arthritis (polyarthritis), chorea, erythema marginatum, and subcutaneous nodules.
b.Minor criteria: Polyarthralgia, fever (≥101.3 F), sedimentation rate ≥60 mm and/or C-reactive protein (CRP) ≥3.0 mg/dL, and prolonged release (PR) interval (unless carditis is a major criterion).
2.Revised Jones Criteria, moderate-and high-risk populations:
a.Major criteria: Carditis (clinical and/or subclinical), arthritis (monopolyarthritis or polyarthritis, or polyarthralgia), chorea, erythema marginatum, and subcutaneous nodules.
b.Minor criteria: Fever (≥101.3 F), sedimentation rate ≥30 mm and/or CRP ≥3.0 mg/dL, and prolonged PR interval (unless carditis is a major criterion).
3.Acute rheumatic fever (ARF) diagnosis (initial episode):
a.The diagnosis of an initial episode of ARF requires two major criteria, or one major plus two minor criteria.
4.ARF diagnosis (subsequent episode): Patients with a history of ARF or rheumatic heart disease (RHD) are at high risk for recurrent attacks if reinfected with group A streptococci:
a.With reliable past history of ARF or established RHD, and in the face of documented group A streptococcal infection, two major, one major and two minor, or three minor manifestations may be sufficient for presumptive diagnosis.
b.When minor manifestations alone are present, the exclusion of other more likely causes of the clinical presentation is recommended before a diagnosis of an ARF recurrence is made.
The most significant complication of ARF is RHD, which occurs after repeated bouts of acute illness.
B.RF is the predominant cause of mitral stenosis.
Incidence
A.RF has markedly decreased in the past 50 years and remains rare in the United States. However, higher number of cases in Hawaii and American Samoa are seen. In developing countries RF is enormous. It has been suggested that 15.6 million people worldwide with 470,000 new cases occur annually:
1.About 20% diagnosed with RF have a positive history of pharyngitis or recall any upper respiratory symptoms within the preceding 3 months.
2.Ages: Most common age is 5 to 15 years, rarely in infants, preschoolers, and after the age of 35 years.
3.RF does not have a definite sexual predication but certain manifestations are more common in females during puberty.
B.Cardiac involvement is the most serious complication and causes significant morbidity due to valvular fibrosis leading to stenosis and/or insufficiency. Of the 470,000 patients diagnosed with RF, 60% eventually will develop carditis. Morbidity due to congestive heart failure (CHF), strokes, and endocarditis is common among individuals with RHD, and about 1.5% of persons with rheumatic carditis die of the disease annually. Mitral stenosis and Sydenham chorea are more common in females who have gone through puberty. People who have had a previous attack of RF are at high risk for a recurrent attack, which worsens the damage to the heart.
Pathogenesis
A.RF is caused by a preceding infection with GAS and Streptococcus pyogenes. Nonsuppurative inflammatory lesions of the joints, heart, subcutaneous tissue, and CNS characterize ARF. The incubation period is between 1 and 5 weeks and 6 months after a group A strep pharyngitis.
Predisposing Factors
A.Group A pharyngitis, untreated or inadequately treated.
B.Most common in developing countries, crowding and poverty.
C.Gender: More common in females—5 to 15 years.
Common Complaints
A.Sore throat.
B.Polyarthritis—involves large joints such as knees, ankles, elbows, and wrists
C.Fever.
D.Abdominal pain.
Other Signs and Symptoms
A.Fatigue.
B.Appetite loss.
C.Sydenham chorea (more common in women).
D.Erythema marginatum.
E.Enlarged lymph nodes.
F.Carditis: Development of new heart murmurs, cardiomegaly, and CHF.
G.Pericarditis, pericardial friction rub, and/or pericardial effusion.
Subjective Data
A.Review a recent history:
1.Sore throat and the onset, duration, severity, and treatment of symptoms.
B.Complete a drug history.
C.Prescribed antibiotics patient take aspirin? Use of aspirin can mask signs of inflammation and tends to prolong the course of the disease.
D.Assess the patient for signs and symptoms of rheumatic and scarlet fever.
E.Discuss the patient’s history of heart problems, chest pain, or shortness of breath.
F.Evaluate the onset and complaints of chorea: Fidgety, clumsy, uncoordinated erratic facial movements, including grimaces, grins, and frowns. Tongue movements. Ask if the movements and other symptoms disappear with sleep.
G.Review symptoms of joint pain.
Physical Examination
A.Check temperature, pulse, respirations, and blood pressure.
B.Inspect:
1.Inspect joints for swelling and warmth.
2.Observe for signs of chorea (symptoms noted earlier).
3.Conduct a dermal exam, especially the trunk and proximal aspects of the extremities. Individual lesions of erythema marginatum are evanescent, moving over the skin in wavy patterns or with indented margins. The lesions may be macular and can develop and disappear in minutes, appearing to change shape while being examined.
4.Complete an ear, nose, oral, and throat exam. Evaluate tonsillopharyngeal erythema with or without exudates. Observe for beefy, red, swollen uvula.
C.Auscultate:
1.Auscultate heart. Note heart murmur, pericardial friction rub, or effusion. Characteristic
murmurs of acute carditis include the high-pitched, blowing, holosystolic, apical murmur of mitral regurgitation and a high-pitched, decrescendo, diastolic murmur of aortic regurgitation heard in the aortic area. The features of CHF include tachycardia, a third heart sound, rales, and edema.
2.Auscultate all lung fields. Note shortness of breath.
D.Palpate:
1.Palpate the neck lymph nodes.
2.Palpate the extremities: Apical and radial pulses.
3.Palpate the abdomen.
E.Neuromuscular examination:
1.Have the patient stick out his or her tongue for observation of a bag of worms
when protruded.
2.Have the patient grip your hand—with chorea, he or she will be unable to maintain a grip; rhythmical squeezing results.
3.Observe for the spooning sign—a flexion at the wrist with finger extension when the hand is extended.
4.Observe for the pronator sign—the palms turn outward when held above the head.