Rheumatic Fever, Acute

DRG Category:	315
Mean LOS:	4 days
Description:	MEDICAL: Other Circulatory System Diagnosis With CC
DRG Category:	546
Mean LOS:	5.2 days
Description:	MEDICAL: Connective Tissue Disorders With CC

Acute rheumatic fever is an autoimmune disorder that follows an upper respiratory infection with group A beta-hemolytic streptococci. Rheumatic fever affects the heart, central nervous system, skin, and musculoskeletal system. In addition to an initial insult, recurrences are common. Only 3% of people who have a pharyngeal streptococcal infection actually develop rheumatic fever.

Acute rheumatic fever is most destructive to the heart. Rheumatic heart disease (RHD) occurs in up to 60% of patients with acute rheumatic fever and may affect any of the layers of the heart during the acute phase. Endocarditis leads to leaflet swelling of the valves, leaflet erosion, and deposits of blood and fibrin on the valves; these deposits are called vegetation. Myocarditis causes cellular swelling, damage to collagen, and formation of fibrosis and scarring. Pericarditis can occur as well, which can lead to pericardial effusion. In addition to valvular disease, acute rheumatic fever can lead to severe carditis and life-threatening heart failure. Complications lead to a 20% death rate within the first 10 years after the initial illness.

Causes

Acute rheumatic fever is caused by a prior streptococcal infection and is often associated with nasopharyngitis or upper respiratory infections. The group A beta-hemolytic streptococcus infection, which may have been mild and even unnoticed and untreated, usually occurs 2 to 6 weeks before the development of symptoms of acute rheumatic fever. Experts suspect that rheumatic fever is an autoimmune response triggered by antibodies that are produced in response to the streptococcal infection. The antibodies react with the body’s cells and produce characteristic lesions in the target organs.

Genetic considerations

Increased susceptibility to rheumatic fever is known to occur in families. The human leukocyte antigen type DR7DR53 has been associated with the development of valve lesions in severe RHD patients. A novel B-cell alloantigen marker, 883, was identified as a marker, and while genetic studies have observed an autosomal recessive pattern of inheritance, causative mutations remain to be found.

Gender, ethnic/racial, and life span considerations

Although acute rheumatic fever primarily targets the school-age population, it also occurs in adults. It is particularly common in children ages 5 to 20 from disadvantaged backgrounds. It rarely occurs in people over age 30. Valvular heart disease is most likely to damage the mitral valve in females and the aortic valve in males. In both genders, tricuspid and pulmonic valve damage occurs rarely. No specific racial or ethnic considerations exist.

Global health considerations

Around the world, 95% of cases occur in tropical countries, those with limited resources, and those with underrepresented indigenous groups. Particular regions of risk for children are Africa, the Middle East, South America, and India. The World Health Organization reports that approximately 20 million new cases occur each year. In developing nations, acute rheumatic fever is the most common acquired heart disease from childhood to young adulthood and is the cause of up to half of all intensive care admissions.

Assessment

History

Usually, the patient has a sore throat and a fever of at least 100.4°F for a few days to several weeks before the onset. This latent period between the acute infection of streptococcal pharyngitis and acute rheumatic fever averages 18 days. The patient either may have been treated with antibiotics or may not have completed a full course of treatment. Determine if the patient has experienced migratory joint tenderness (polyarthritis), chest pain, fever, and fatigue. Some patients describe unexplained nosebleeds as well. Patients with pericarditis may describe sharp pain over the shoulder that radiates to the neck, back, and arms. The pain may increase with inspiration and decrease when the patient leans forward from a sitting position. Patients with heart failure may describe shortness of breath, cough, and right upper quadrant abdominal pain. In addition, the patient may describe fatigue or activity intolerance, along with periorbital, abdominal, or pedal edema.

Physical examination

The patient may have a distinctive red rash, referred to as erythema marginatum, fever, and joint and muscle pain. This nonpruritic rash appears primarily on the trunk of the body, the buttocks, and the extremities; it appears on the face in only rare instances. In addition, subcutaneous nodules of less than 1 cm in diameter form on the skin. Painless and movable, they usually appear over bony prominences: the hands, wrists, elbows, knuckles, feet, and vertebrae. If the patient has heart failure, there may be peripheral edema.

The patient may also demonstrate chorea (previously referred to as St. Vitus’ dance). Mild chorea produces hyperirritability, problems concentrating, and illegible handwriting. Severe chorea causes purposeless, uncontrollable, jerky movements and muscle spasms, speech disturbances, muscle fatigue, and incoordination. Transient chorea may not appear until several months after the initial streptococcal infection.

When the joints are palpated, the patient may have migratory polyarticular arthritis (more than four joints are progressively involved). The most frequently involved joints include the knees, elbows, hips, shoulders, and wrists. These joints are extremely warm and tender to the touch, and even a light palpation can cause pain. The pain usually subsides after the patient becomes afebrile.

Heart murmurs serve as an indicator that carditis has occurred. The aortic and mitral valves are particularly involved as a result of the Aschoff bodies (small nodules of cells and leukocytes) that form on the tissues of the heart. You are more likely to hear the murmurs at the third intercostal space right of the sternum for the aortic valve and at the apex of the heart if the mitral valve is involved. When you palpate peripheral pulses, you may note a rapid heart rate.

Psychosocial

The disease is likely to occur at an age when children are active and industrious. Those who require extended bedrest may have trouble coping with the limitations placed on them.

Diagnostic highlights

Modified Jones Criteria (American Heart Association Major and Minor Criteria). To make the diagnosis, there needs to be evidence of a previous streptococcal infection and two major Jones criteria or one major plus two minor Jones criteria.

Table l1.
Criteria	Description	Explanation
Major manifestations	Carditis	Cardiomegaly, new murmur, congestive heart failure, pericarditis
	Migratory polyarthritis	Polyarticular, involves the large joints
	Subcutaneous nodules (Aschoff bodies)	Firm, painless fibrous nodules on the extensor surfaces of the wrists, elbows, and knees
	Erythema marginatum	Long-lasting rash
	Chorea (St. Vitus’ dance)	Movement disorder, rapid and purposeful movements of face and arms; generally, movements cease during sleep
Minor manifestations	Clinical findings	Arthralgia, fever
	Laboratory findings	See below

Laboratory Findings
Test	Normal Results	Abnormality With Condition	Explanation
Throat cultures	Negative culture	Positive for group A beta-hemolytic streptococci	Identifies causative organism in the acute phase of pharyngeal infection; positive culture found in 25% of patients
Antistreptolysin O (ASO) titer	< 166 Todd units	< 250 Todd units for an inactive infection; 500–5,000 Todd units for an active infection	Antibody to the streptolysin-O enzyme produced by group A beta-hemolytic streptococci; titers raise about 7 days after infection and gradually return to baseline after 12 mo

Other Tests: Complete blood count, rapid antigen test, C-reactive protein, erythrocyte sedimentation rate, chest x-ray, echocardiogram, chest computed tomography scan, chest magnetic resonance imaging

Primary nursing diagnosis

Diagnosis

Pain (acute) related to tissue swelling

Outcomes

Comfort level; Pain control behavior; Pain: Disruptive effects; Pain level

Interventions

Pain management; Analgesic administration; Positioning; Teaching: Prescribed activity/exercise; Teaching: Procedure/treatment; Teaching: Prescribed medication

Planning and implementation

Collaborative

The goal of management is to end the infection, relieve the symptoms, and prevent recurrence. Complete eradication of the streptococcal infection is necessary so that the heart and kidneys are not damaged. The physician may prescribe antibiotic therapy, intramuscular benzathine penicillin G, if the patient has no known history of allergy to penicillin. Reinforce the need for the patient to complete all medications and to watch for potential side effects, such as rash, hives, wheezing, or anaphylaxis. Activity restrictions are required to ensure full recovery. In patients with active carditis, strict bedrest may be needed for approximately 5 weeks. The physician then prescribes a progressive increase in activity. If valvular dysfunction leads to persistent heart failure, the patient may need surgery to correct the deficit in heart function.

Pharmacologic highlights

Medication or Drug Class	Dosage	Description	Rationale
Benzathine penicillin G or procaine penicillin G	2.4 million units IM	Antibiotic	Eradicates the infection; injections may be given in the hospital or by a home health nurse; oral penicillin may be given, with erythromycin as an alternative in those allergic to penicillin
Aspirin	650 mg PO as needed	NSAID	Treats the arthralgia (muscle achiness); monitor for side effects such as tinnitus, gastric upset, and petechiae

Other Drugs: Erythromycin; penicillin VK; azithromycin; naproxen; prednisone (to treat the carditis); furosemide (for patients with congestive heart failure). After the acute phase of the disease, the physician usually prescribes monthly injections of benzathine penicillin G or daily oral antibiotics. Preventive treatment with antibiotics may last at least for 5 years.

Independent

Explain to the child and the family the need to take all antibiotics until they are completed. This information needs to be conveyed in such a manner so as to promote compliance, not communicate guilt. Remind the parents that failure to seek treatment for a streptococcal infection is common because the symptoms are so mild. The patient is likely to remain on oral antibiotics indefinitely through his or her life.

Managing activity restrictions is a challenging goal in working with a young person of school age who is on bedrest. As the chorea decreases, the child needs to participate in therapeutic play activities that promote a sense of industry and minimize any feelings of inferiority—activities such as reading, board games, and video game play. Encourage the parents to obtain a tutor so the patient can keep up with schoolwork during her or his convalescence. To protect the patient who develops chorea and has an unsteady gait, make sure that all obstructions are cleared out of the way during ambulation to reduce the risk of injury.

Evidence-Based Practice and Health Policy

Breda, L., Marzetti, V., Gaspari, S., Del Torto, M., Chiarelli, F., & Altobelli, E. (2012). Population-based study of incidence and clinical characteristics of rheumatic fever in Abruzzo, central Italy, 2000–2009. The Journal of Pediatrics, 160(5), 832–836.e1. doi 10.1016/j.jpeds.2011.10.009

In a retrospective population-based study, 88 cases of acute rheumatic fever were identified. The average age at the time of diagnosis was 8.7 years (range, 2.5 to 17 years). Patients presented most often with fever (79.6%), followed by arthritis (59.1%) and carditis (48.9%).
Residual valvular damage occurred in 44.3% of the patients. The mitral valve and the aortic valve were most frequently affected, with reported involvement in 42.1% and 17.1% of all patients, respectively.
Residual symptoms in the postacute phase included mitral regurgitation in 22.7% of patients, aortic regurgitation in 4.5%, mitral stenosis in 2.3%, pulmonic regurgitation in 2.3%, and tricuspid regurgitation in 2.3%.
Recurrence of acute rheumatic fever occurred in 6.8% of the patients.

Documentation guidelines

The extent of the skin lesions for the erythema marginatum rash and subcutaneous nodules
The extent of chest pain and cardiac involvement
The extent of the chorea and joint involvement
Reaction to bedrest and activity restriction

Discharge and home healthcare guidelines

Teach the patient or parents to prevent any further streptococcal infections by good hand washing and avoiding people with sore throats. Encourage the patient or parents to contact the primary healthcare provider if a sore throat occurs. Explain all medications, including dosage, action, route, and side effects. Encourage the patient to resume activity gradually and to use an elevator, if one is available, at school. Teach the patient to return to physical education classes or extracurricular sports gradually, with the guidance of the physician. Encourage the patient to take frequent naps and rest periods.