Acute Rheumatic Fever 

Updated: Nov 04, 2018
Author: Robert J Meador, Jr, MD; Chief Editor: Herbert S Diamond, MD 

Overview

Practice Essentials

Acute rheumatic fever (ARF) is a sequela of streptococcal infection—typically following 2 to 3 weeks after group A streptococcal pharyngitis—that occurs most commonly in children and has rheumatologic, cardiac, and neurologic manifestations.[1, 2] The incidence of ARF has declined in most developed countries, and many physicians have little or no practical experience with the diagnosis and management of this condition. Occasional outbreaks in the United States make complacency a threat to public health.

Diagnosis rests on a combination of clinical manifestations that can develop in relation to group A streptococcal pharyngitis.[3] These include chorea, carditis, subcutaneous nodules, erythema marginatum, and migratory polyarthritis. (See the image below.) Because the inciting infection is completely treatable (see Treatment), attention has been refocused on prevention.

Clinical manifestations and time course of acute r Clinical manifestations and time course of acute rheumatic fever.

Pathophysiology

Although the inciting bacterial agent is well known, susceptibility factors remain unclear. The location of the streptococcal infection seems to play an important role. The clinical syndrome typically follows a streptococcal pharyngitis, but streptococcal cellulitis has never been implicated.

The earliest and most common feature is a painful migratory arthritis, which is present in approximately 80% of patients. Large joints such as knees, ankles, elbows, or shoulders are typically affected. Sydenham chorea was once a common late-onset clinical manifestation but is now rare.[4] Carditis (with progressive congestive heart failure, a new murmur, or pericarditis) may be the presenting sign of unrecognized past episodes and is the most lethal manifestation.

Genetics may contribute, as evidenced by an increase in family incidence. No significant association with class-I human leukocyte antigens (HLAs) has been found, but an increase in class-II HLA antigens DR2 and DR4 has been found in black and white patients, respectively.[5] Evidence suggests that elevated immune-complex levels in blood samples from patients with ARF are associated with HLA-B5.[6]

In a study of 15 patients with rheumatic heart disease and a control group of 10 patients who had been exposed to group A streptococci but did not develop either acute rheumatic fever or rheumatic heart disease, 13 genes were differentially expressed in the same direction (predominantly decreased) between the two groups. Seven of those were immune response genes involved in cytotoxicity, chemotaxis, and apoptosis. The researchers concluded that the high proportion of differentially expressed apoptotic and immune response genes supports a model of autoimmune and cytokine dysregulation in ARF.[7]

 

Etiology

Although the mechanism by which streptococcal organisms cause disease is not entirely clear, overwhelming epidemiologic evidence suggests that ARF is caused by streptococcal infection, and recurrences can be prevented with prophylaxis.

Strains of group A streptococci that are heavily encapsulated and rich in M protein (signifying virulence in streptococcal strains) seem to be most likely to result in infection.

Group A Streptococcus (Streptococcus pyogenes) is thought to cause the myriad of clinical diseases in which the host's immunologic response to bacterial antigens cross-react with various target organs in the body, resulting in molecular mimicry. In fact, autoantibodies reactive against the heart have been found in patients with rheumatic carditis. The antibody can cross-react with brain and cardiac antigens, and immune complexes are present in the serum. The problem has been the uncertainty of whether these antibodies are the cause or result of myocardial tissue injury.

Epidemiology

United States

The incidence of an acute rheumatic episode following streptococcal pharyngitis is 0.5-3%. The peak age is 6-20 years. Although the incidence of ARF has steadily declined, the mortality rate has declined even more steeply. Credit can be attributed to improved sanitation and antibiotic therapy. Several sporadic outbreaks in the United States could not be blamed directly on poor living conditions. New virulent strains are the best explanation.

In Hawaii, the incidence of ARF has remained several times higher than in the continental United States, particularly among ethnic Polynesians. Unusual group A streptococci emm types that are uncommon in the continental United States appear to play a significant role in the epidemiology of ARF in Hawaii.[8]

International

Worldwide, as many as 20 million new cases of ARF occur each year. The introduction of antibiotics has been associated with a rapid worldwide decline in the incidence of ARF. Currently, the incidence is 0.23-1.88 patients per 100,000 population. From 1862-1962, the incidence per 100,000 population declined from 250 patients to 100 patients, primarily in teenagers.

Most major outbreaks occur under conditions of impoverished overcrowding where access to antibiotics is limited. Rheumatic heart disease accounts for 25-50% of all cardiac admissions internationally. Rates of rheumatic heart disease and releated deaths are particularly high in Oceania, South Asia, and central sub-Saharan Africa.[9] Some areas of South America are also strongly affected.[10]

Rates of ARF are exceptionally high in natives of Polynesian ancestry in Hawaiian and Maori populations. For example, in a study from a New Zealand district, the ethnicity of ARF patients was 85% Maori and 10% Pacific. Although the annual incidence of ARF was 3.1 per 100,000 population, in Maori children aged 5-14 years the incidence was 46.1 per 100,000 population. Almost three-quarters of all patients lived in severely socioeconomically deprived areas.[11]

In the last decade, an increase in the incidence of ARF was observed in Slovenia, in south-central Europe. From 2008 through 2014, the estimated annual incidence of ARF was 1.25 cases per 100,000 children.[12]

A study of pediatric patients (age 0-17 years) in Lombardy, Italy who were hospitalized with the diagnosis of ARF from 2014 to 2016 found that the annual hospitalization rate was 4.24 cases per 100,000 children. A seasonal trend was evident, with fewer cases in the autumn and a peak in the spring.[13]

Mortality/Morbidity

Mortality rates are steadily improving because of better sanitation and health care. The current pattern of morbidity is difficult to measure because the first attack of rheumatic fever follows an unpredictable course. As many as 90% of episodes are clinically contained within 3 months.

Carditis causes the most severe clinical manifestation because heart valves can be permanently damaged. The disorder also can involve the pericardium, myocardium, and the free borders of valve cusps. Death or total disability may occur years after the initial presentation of carditis. Worldwide, the prevalence of rheumatic heart disease may range from 33 to 78 million cases, and deaths from rheumatic heart disease may range from 275,000 to 1.4 million deaths each year.[14]

Racial, Sexual, and Age-related Demographics

An association between certain class-II HLA antigens (DR2 in blacks and DR4 in whites) and ARF has been reported.[5]

No general clear-cut sex predilection for ARF has been reported, but certain of its manifestations seem to be sex variable. For example, chorea and tight mitral stenosis occur predominantly in females, while aortic stenosis develops more often in males.

The initial attack of ARF occurs most frequently in persons aged 5-15 years and rarely occurs in persons older than 30 years.[15] The disease may cluster in families. In some countries, a shift into older groups may be a trend.

 

Presentation

History

Usually, a latent period of approximately 18 days occurs between the onset of streptococcal pharyngitis and the onset of acute rheumatic fever (ARF). This latent period is rarely shorter than 1 week or longer than 5 weeks.

Approximately 70% of older children and young adults recollect the pharyngitis. However, only approximately 20% of young children recollect pharyngitis. Therefore, younger children who present with signs or symptoms consistent with ARF merit a higher index of suspicion.[16]

Typically, the first manifestation of ARF is a very painful migratory polyarthritis. Large joints such as knees, ankles, elbows, or shoulders are typically affected. Often, associated fever and constitutional toxicity develop. Sydenham chorea (ie, rapid, irregular, aimless involuntary movements of the arms and legs, trunk, and facial muscles[17] ) was once a common late-onset clinical manifestation but is now rare.[4] Acute attacks usually resolve within 12 weeks.

Physical Examination

Physical findings can be nonspecific and misleading. Therefore, a high index of suspicion is required for diagnosis.

Suspicious signs for carditis include new or changing valvular murmurs, cardiomegaly, congestive heart failure, and/or pericarditis. Nearly 60% of patients with carditis develop isolated mitral valve involvement, followed in prevalence by combined mitral and aortic valve involvement.

When present, Sydenham chorea is seldom evident at the time of initial presentation. Erythema marginatum and subcutaneous nodules are rare (< 10% of patients). See the image below.

Erythema marginatum, the characteristic rash of ac Erythema marginatum, the characteristic rash of acute rheumatic fever.

Arthritis, which occurs in 80% of patients, usually involves multiple large joints, particularly the knees, ankles, elbows, and wrists. Hips and smaller joints of hands and feet are less commonly involved.

Migratory polyarthritis is usually associated with a febrile illness. It involves a series of painful joints, followed by another series of painful joints. This form of arthritis rarely causes permanent joint deformity.

Unusual presentations, such as indolent carditis and isolated chorea, may also occur. Even rarer manifestations include epistaxis and abdominal pain due to serositis.

 

DDx

Diagnostic Considerations

Other problems to be considered include the following:

  • Bacterial endocarditis

  • Still disease - Systemic-onset juvenile rheumatoid arthritis or adult Still disease

  • Vasculitis

Poststreptococcal reactive arthritis

Barash et al performed a retrospective study to compare clinical and laboratory features of acute rheumatic fever (ARF) versus poststreptococcal reactive arthritis to determine whether the two diseases are separate clinical manifestations of the same disease or are in fact different diseases altogether. Based on a review of 68 patients with ARF and 159 patients with poststreptococcal reactive arthritis, the authors concluded that at least four factors differed significantly enough to show that the two diseases are distinct: erythrocyte sedimentation rate (ESR), C-reactive protein levels, duration of joint symptoms after initiation of anti-inflammatory treatment, and relapse of joint symptoms after treatment cessation. Using the differentiating factors, they were able to determine the correct diagnosis in more than 80% of cases.[18]

Differential Diagnoses

 

Workup

Approach Considerations

Acute rheumatic fever (ARF) is diagnosed on the basis of clinical manifestations supported by laboratory tests.

Jones criteria

Guidelines for diagnosis published in 1944 by T. Duckett Jones[19] have been revised by the American Heart Association (AHA). The most recent update, published in 2015, recognizes the variability in clinical presentation in populations at different levels of risk and includes Doppler echocardiography as a tool to diagnose cardiac involvement; these changes bring the criteria into closer alignment with other international guidelines.[20]

Laboratory evidence of a preceding group A streptococcal infection is needed whenever possible. Without it, the diagnosis of ARF is in doubt, except in patients with chorea, which may be the sole initial manifestation of ARF, and rarely in patients with indolent rheumatic carditis with insidious onset and slow progression.[20]

The AHA suggests that diagnostic criteria may be applied differently, depending on rate of ARF or rheumatic heart disease (RHD) in the population. This can help avoid overdiagnosis in low-incidence populations and underdiagnosis in high-risk ones. The AHA defines low risk as an ARF incidence of < 2 per 100,000 school-aged children (usually 5–14 years old) per year or an all-age prevalence of RHD of ≤1 per 1000 population per year. Children not clearly from a low-risk population are at moderate to high risk depending on their reference population.[20]

Jones criteria for the diagnosis of initial ARF are the presence of two major manifestations or one major and two minor manifestations. For recurrent ARF, the criteria are two major manifestations, one major and two minor manifestations, or three minor manifestations.

Major manifestations comprise the following:

  • Carditis, clinical and/or subclinical (ie, detected by echocardiography)
  • Arthritis
  • Chorea
  • Erythema marginatum
  • Subcutaneous nodules

In patients from low-risk populations, arthritis must be polyarthritis. For patients from moderate- and high-risk populations, either monoarthritis or polyarthritis qualifies; polyarthralgia may qualify if other causes have been excluded.

Minor manifestations in low-risk populations comprise the following:

  • Polyarthralgia
  • Fever ≥38.5°C
  • Acute phase reactions: Erythrocyte sedimentation rate (ESR) ≥60 mm in the first hour and/or C-reactive protein (CRP) level ≥3.0 mg/dL
  • Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)

Minor manifestations in moderate- and high-risk populations comprise the following:

  • Monoarthralgia
  • Fever ≥38°C
  • ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL
  • Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)

Laboratory studies

According to the AHA, any one of the following can serve as evidence of preceding group A streptococcal infection[20] :

  • Elevated or rising  streptococcal antibody titer; a rise in titer is better evidence than a single titer result
  • A positive throat culture for group A β-hemolytic streptococci
  • A positive rapid group A streptococcal carbohydrate antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngitis

Rapid streptococcal tests are principally useful for prevention of ARF. Currently available tests have 90-100% specificity, permitting immediate initiation of antibiotic therapy in patients with positive results. In contrast, the sensitivity of these tests is 70-90%, lessening the value of a negative result.

Antistreptococcal antibodies usually reach a peak titer (in Todd units) at the time of onset of ARF and are more useful for diagnosis. Specific antibodies to streptococcal antigens also indicate true infection rather than mere carriage of the organism. These antibodies target extracellular products produced by streptococci.

Antistreptococcal antibodies include the following:

  • Antistreptolysin O (ASO)

  • Antideoxyribonuclease B (anti-DNAse B)

  • Antistreptokinase

  • Antihyaluronidase

  • Anti-DNAase (anti-DNPase)

Although age, geographic location, and season affect the titers, an elevated titer of at least one of these antibodies indicates streptococcal infection in 95% of patients. ASO titers are found in 80-85% of patients with ARF.

However, note that an isolated positive ASO titer is not necessarily an indication of ARF, as it may also be found in patients with certain related diseases such as rheumatoid arthritis and Takayasu arteritis. Therefore, rising ASO titers should be combined with a careful clinical evaluation and the discovery of other antistreptococcal antibodies to support the diagnosis of ARF.

The sensitivity of throat culture as evidence of recent streptococcal infection is 25-40%. For comparison, the sensitivity of ASO titer (adults with >240 Todd U and children with >320 Todd U) is 80%. The sensitivity of an elevated ASO titer in addition to anti-DNAse B or antihyaluronidase is 90%.

Acute-phase reactants such as C-reactive protein and ESR are usually elevated and helpful in monitoring disease activity.

Other laboratory tests may be helpful but not for definitive diagnosis. Synovial fluid analysis reveals a sterile inflammatory reaction, usually with fewer than 20,000 cells/μL (mainly polymorphonuclear) without crystals.

Histologic Findings

Synovial biopsy (which is rarely performed) reveals mild inflammatory changes. The synovial membrane may be thickened, erythematous, and covered by a fibrinous exudate.

Endomyocardial biopsies have not contributed significantly to diagnosis. Focal fibrinoid lesions in the heart and histiocytic granulomas called Aschoff nodules may be late findings. Pancarditis develops with involvement of all layers of the heart.

Subcutaneous nodule histopathology reveals edema, fibrinoid necrosis, and mononuclear cell infiltrate.

Chest Radiography

A chest radiograph may reveal cardiomegaly. See the image below.

Chest radiograph showing cardiomegaly due to cardi Chest radiograph showing cardiomegaly due to carditis of acute rheumatic fever.

Echocardiography with Doppler

Echocardiography is the gold standard for diagnosis of rheumatic heart disease (RHD).[14] Current American Heart Association criteria recommendations regarding echocardiography are as follows[20] :

  • Echocardiography with Doppler should be performed in all cases of confirmed and suspected ARF (class I)
  • It is reasonable to consider performing serial echocardiography/Doppler studies in any patient with diagnosed or suspected ARF, even if documented carditis is not present on diagnosis (class IIa)
  • Echocardiography/Doppler testing should be performed to assess whether carditis is present in the absence of auscultatory findings, particularly in moderate- to high-risk populations and when ARF is considered likely (class I)
  • Echocardiography/Doppler findings not consistent with carditis should exclude that diagnosis in patients with a heart murmur otherwise thought to indicate rheumatic carditis (class I)

The World Health Organization has recommended that children in endemic areas undergo echocardiographic screening for RHD, to identify candidates for secondary prophylaxis to prevent ARF recurrences. In 2012, the World Heart Federation published criteria for echocardiographic diagnosis of RHD. However, discriminating mild RHD from normal variants can be very difficult, and false-positive tests expose the patient to inappropriate and lengthy treatment.[14]

Doppler findings in rheumatic valvulitis

To diagnose pathological mitral regurgitation by Doppler, all four of the following criteria must be met:

  • Seen in at least two views
  • Jet length ≥2 cm in at least one view
  • Peak velocity >3 m/sec
  • Pan-systolic jet in at least one envelope

To diagnose pathological aortic regurgitation by Doppler, all four of the following criteria must be met:

  • Seen in at least two views
  • Jet length ≥1 cm in at least one view
  • Peak velocity >3 m/sec
  • Pan-diastolic jet in at least one envelope

Echocardiographic findings in rheumatic valvulitis

Acute mitral valve changes include the following:

  • Annular dilation
  • Chordal elongation
  • Chordal rupture resulting in flail leaflet with severe mitral regurgitation
  • Anterior (less commonly, posterior) leaflet tip prolapse
  • Beading/nodularity of leaflet tips

Chronic mitral valve changes that are not seen in acute carditis include the following:

  • Leaflet thickening
  • Chordal thickening and fusion
  • Restricted leaflet motion
  • Calcification

Aortic valve changes in either acute or chronic carditis include the following:

  • Irregular or focal leaflet thickening
  • Coaptation defect
  • Restricted leaflet motion
  • Leaflet prolapse
 

Treatment

Medical Care

Treatment strategies for acute rheumatic fever (ARF) can be divided into the following:

  • Management of the acute attack
  • Management of the current infection
  • Prevention of further infection and attacks

The primary goal of treating an ARF attack is to eradicate streptococcal organisms and bacterial antigens from the pharyngeal region. Penicillin is the drug of choice in persons who are not at risk of allergic reaction. A single parenteral injection of benzathine benzylpenicillin can ensure compliance. Oral cephalosporins, rather than erythromycin, are recommended as an alternative in patients who are allergic to penicillin. However, be cautious of the 20% cross-reactivity of the cephalosporins with penicillin.

Prompt treatment of streptococcal pharyngitis in susceptible hosts can prevent repetitive exposure to pathologically reactive antigens.[21, 22] However, management of the current infection will probably not affect the course of the current attack. Antimicrobial therapy does not alter the course, frequency, or severity of cardiac involvement.

Analgesia is optimally achieved with high doses of salicylates, which often induce dramatic clinical improvement. However, a lower dose may be required to avert symptoms of nausea and vomiting. When salicylates are used as therapy, the dosage should be increased until the drug produces either a clinical effect or systemic toxicity characterized by tinnitus, headache, or hyperpnea.

Corticosteroids should be reserved for the treatment of severe carditis. After 2-3 weeks, the dosage may be tapered, reduced by 25% each week. Overlap with high-dose salicylate therapy is recommended as the dosage of the prednisone is tapered over a 2-week period to avoid poststeroid rebound. In extreme cases, intravenous methylprednisolone may be used.

Mild heart failure usually responds to rest and corticosteroid therapy. Digoxin can be useful in patients with severe carditis, but its use should be monitored closely because of the possibility of heart block.

Nocturnal tachycardia may be a sign of cardiac involvement that may be responsive to digoxin. Vasodilators and diuretics also may be used.

Sydenham chorea requires long-term antimicrobial prophylaxis, even if no other manifestations of rheumatic fever evolve. The signs and symptoms of chorea usually do not respond well to treatment with antirheumatic agents. Complete physical and mental rest is essential because the manifestations of chorea may be exaggerated by emotional trauma. Glucocorticoids or salicylates have little or no effect on chorea. Because chorea disappears with sleep, adequate sedation should be provided.

A number of drugs have been used off label for symptomatic treatment of Sydenham chorea, including anticonvulsants (eg, valproate, carbamazepine) and neuroleptics (eg, pimozide, haloperidol, risperidone, olanzapine). Gradual withdrawl of medication is suggested once patients have been symptom free for at least 1 month.[23]

Prevention of ARF has been successful in developed societies. The recommended approach can be divided into primary and secondary prevention. Primary prevention involves eradication of Streptococcus from the pharynx, which generally entails administering a single intramuscular injection of benzathine benzylpenicillin.[24]

The American Heart Association (AHA) Committee on Acute Rheumatic Fever recommends a regimen consisting of benzathine benzylpenicillin at 1.2 million units intramuscularly every 4 weeks. However, in high-risk situations, administration every 3 weeks is justified and advised. High-risk situations include patients with heart disease who are at risk of repetitive exposure.[25]

Oral prophylaxis, which is less reliable, consists of phenoxymethylpenicillin (penicillin V) or sulfadiazine. These can be used in compliant patients.

If penicillin allergy is suspected, oral cephalosporins should be used.

Although no consensus on the required duration of antibacterial prophylaxis has been reached, the AHA recommends that prophylaxis be continued for at least 10 years after the last episode of rheumatic fever or until patients are well into adulthood. For those with heart disease who are at risk of repetitive exposures, prophylaxis should be continued for a longer duration, probably indefinitely. However, discontinuing prophylaxis may be reasonable in patients in their third decade of life in whom more than 5 years have passed since their last attack and who are free from rheumatic heart disease.

The principles of treatment include the following:

  • The risk of rheumatic fever recurrence is greatest during the first 3-5 years following the attack.
  • Prophylaxis must continue indefinitely in patients with established heart disease or in those frequently exposed to streptococci.
  • Treatment for an indefinite period is required for patients with frequent exposure to streptococci or for those who are difficult to monitor.

In underdeveloped countries, prophylaxis should be continued as follows:

  • Continue for 5 years after the first attack
  • Continue indefinitely in patients with established heart disease
  • Continue indefinitely in patients who are frequently exposed to streptococci and are difficult to monitor

The decision to withdraw antibacterial treatment should be individualized after carefully assessing the risk of repetitive exposures.

Surgical Care

Valve replacement should be considered in patients with active carditis, especially those with cases that are refractory to medical care or require high doses of vasodilators and diuretics.

Regurgitant lesions respond to valve replacement. Pure stenotic lesions may benefit from more conservative balloon mitral commissurotomy.[26]

Consultations

Primary care physicians should be considered the patient's advocate and guide to medical resources. The role of specialists is as follows:

  • Rheumatologists usually assist in diagnosis in the face of a substantial differential; when the diagnosis is established, they can advise on the therapy plan

  • A cardiologist should be consulted when cardiac involvement is present

  • A neurologist may offer interventions to help manage chorea

Activity

See the list below:

  • All patients should be restricted to bed rest and monitored closely for carditis.

  • Aggressive use of acutely inflamed joints or other exercise may cause permanent joint injury to acutely inflamed joints.

  • When carditis has been documented, a 4-week period of bed rest is recommended. As soon as the signs of acute inflammation subside, patients should resume active ambulation as tolerated.

  • Most patients can be treated safely in an outpatient setting.

 

Medication

Medication Summary

Treatment and prevention of acute rheumatic fever (ARF) may involve multiple specialties, including infectious diseases, cardiology, and neurology. For this reason, several different classes of medications are used. These include antibiotic, cardiac, and neuroleptic medications.

Antibiotics

Class Summary

Antibiotics are the initial pharmacotherapy for prevention and treatment of rheumatic fever.

Penicillin G procaine (Crysticillin)

Long-acting parenteral penicillin indicated in the treatment of moderately severe infections caused by microorganisms sensitive to penicillin G. IM administration only.

Adults: Deep IM injection into the upper outer quadrant of the buttock only.

Infants and small children: IM injection into midlateral aspect of the thigh is suggested.

Some authors prefer 10 d of therapy.

Penicillin G benzathine (Bicillin L-A)

Interferes with synthesis of cell wall mucopeptides during active multiplication, which results in bactericidal activity. Long-acting depot form of penicillin G. Because of its prolonged blood level, several authors believe this to be the DOC. Others prefer daily injections with short-acting penicillin.

Penicillin VK (Beepen-VK, Betapen-VK, Robicillin VK, Veetids)

Inhibits biosynthesis of cell wall mucopeptide and is effective during stage of active multiplication. Inadequate concentrations may produce only bacteriostatic effects. PO alternative.

Erythromycin (EES, E-Mycin, Ery-Tab, Erythrocin)

Alternative for patients allergic to penicillin (although not the DOC).

Drug may inhibit RNA-dependent protein synthesis by stimulating the dissociation of peptidyl t-RNA from ribosomes. Inhibits bacterial growth.

In children, age, weight, and the severity of infection determine proper dosage. When bid dosing is desired, half-total daily dose may be taken every 12 h. For more severe infections, dose may be doubled.

Anti-inflammatory agents

Class Summary

These agents inhibit inflammation to prevent destruction in the joints and heart.

Aspirin (Ascriptin, Bayer Buffered Aspirin, Ecotrin)

For treatment of mild to moderate pain and headache. Considered the first DOC for the treatment of arthritis due to acute rheumatic fever (ARF).

Glucocorticosteroids

Class Summary

These agents demonstrate anti-inflammatory (glucocorticoid) and salt-retaining (mineralocorticoid) properties. Glucocorticoids produce profound and varied metabolic effects. These agents also modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Liquid-Pred, Meticorten, Orasone, Sterapred)

Patients with carditis require prednisone. The goal is to decrease myocardial inflammation. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. After 2-3 wk, dosage may be tapered, reduced 25% each week.

Neuroleptic agents

Class Summary

These agents are used for chorea associated with ARF.

Haloperidol (Haldol)

Dopamine receptor blocker used for irregular spasmodic movements of the limbs or facial muscles.

Positive inotropic agents

Class Summary

Digoxin may be indicated for patients with congestive heart failure.

Digoxin (Lanoxin)

Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

 

Follow-up

Further Outpatient Care

See the list below:

  • Periodic monitoring at 3- to 4-month intervals is critical to evaluate for progress with the resumption of physical activity, resolution of the constitutional symptoms, and freedom from adverse effects from medications.

  • Less frequent visits, perhaps once a year, are appropriate while following a prophylaxis regimen.

Transfer

Transfer to a short-term–care facility should be arranged when patients have active life-threatening sequelae, notably carditis.

Deterrence/Prevention

Patients should be educated to seek medical attention upon the first signs of pharyngitis. Once the disease is established, patients should be educated regarding benefits and risks of compliance with their medical regimen, which may be protracted.

Complications

See the list below:

  • Acute episodes are self-limited, with an average duration of 3 months for untreated attacks. Recurrence tends to occur within the first few years of the attack.

  • The outcome of carditis is likely to be more severe if patients have pre-existing heart disease. Carditis resolves without sequelae in 65-75% of patients.

  • Severe cardiac failure, total disability, and death may occur years after the acute attack.

  • The risk of developing a new episode is highest during the 5 years following an acute attack. This justifies prophylaxis for all patients for at least 5 years or until the patient reaches age 18 years.

Prognosis

The course followed by a patient after a first attack is highly variable and unpredictable. Approximately 90% of episodes last less than 3 months. Only a minority persist longer, in the form of unremitting rheumatic carditis or prolonged chorea.

In an Australian study, recurrence of ARF occurred most often in the first year after initial ARF episode (incidence 3.7 per 100 person-years), but low-level risk persisted for more than 10 years. Risk of progression to rheumatic heart disease was also highest in the first year (incidence 35.9), almost 10 times higher than that of ARF recurrence.[27]

 

Questions & Answers

Overview

What is acute rheumatic fever (ARF)?

What is the pathophysiology of acute rheumatic fever (ARF)?

What causes acute rheumatic fever (ARF)?

What is the US prevalence of acute rheumatic fever (ARF)?

What is the global prevalence of acute rheumatic fever (ARF)?

What is the mortality and morbidity associated with acute rheumatic fever (ARF)?

Which patient groups have the highest prevalence of acute rheumatic fever (ARF)?

Presentation

Which clinical history findings are characteristic of acute rheumatic fever (ARF)?

Which physical findings are characteristic of acute rheumatic fever (ARF)?

DDX

Which conditions are included in the differential diagnoses of acute rheumatic fever (ARF)?

How is poststreptococcal reactive arthritis differentiated from acute rheumatic fever (ARF)?

What are the differential diagnoses for Acute Rheumatic Fever?

Workup

How is acute rheumatic fever (ARF) diagnosed?

What are the Jones diagnostic criteria for acute rheumatic fever (ARF)?

What is the role of lab tests in the workup of acute rheumatic fever (ARF)?

Which histologic findings are characteristic of acute rheumatic fever (ARF)?

What is the role of chest radiography in the workup of acute rheumatic fever (ARF)?

What is the role of echocardiography in the workup of acute rheumatic fever (ARF)?

Treatment

How is acute rheumatic fever (ARF) treated?

What is the role of surgery in the treatment of acute rheumatic fever (ARF)?

Which specialist consultations are beneficial to patients with acute rheumatic fever (ARF)?

Which activity modifications are used in the treatment of acute rheumatic fever (ARF)?

Medications

What is the role of medications in the treatment and prevention of acute rheumatic fever (ARF)?

Which medications in the drug class Positive inotropic agents are used in the treatment of Acute Rheumatic Fever?

Which medications in the drug class Neuroleptic agents are used in the treatment of Acute Rheumatic Fever?

Which medications in the drug class Glucocorticosteroids are used in the treatment of Acute Rheumatic Fever?

Which medications in the drug class Anti-inflammatory agents are used in the treatment of Acute Rheumatic Fever?

Which medications in the drug class Antibiotics are used in the treatment of Acute Rheumatic Fever?

Follow-up

What is included in the long-term monitoring of acute rheumatic fever (ARF)?

When is transfer indicated for the treatment of acute rheumatic fever (ARF)?

What is included in patient education about acute rheumatic fever (ARF)?

What are the possible complications of acute rheumatic fever (ARF)?

What is the prognosis of acute rheumatic fever (ARF)?