eMedicine Specialties > Emergency Medicine > Rheumatology

Rheumatic Fever

Author: Steven J Parrillo, DO, FACOEP, FACEP, Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Medical Director, Disaster Medicine and Management Masters Program, Philadelphia University
Coauthor(s): Catherine V Parrillo, DO, FACOP, FAAP, Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine
Contributor Information and Disclosures

Updated: Oct 30, 2009

Introduction

Background

Rheumatic fever causes chronic progressive damage to the heart and its valves. Until 1960, it was a leading cause of death in children and a common cause of structural heart disease. The disease has been known for many centuries. Baillou (1538-1616) first distinguished acute arthritis from gout. Sydenham (1624-1668) described chorea but did not associate it with acute rheumatic fever (ARF). In 1812, Charles Wells associated rheumatism with carditis and provided the first description of the subcutaneous nodules. In 1836, Jean-Baptiste Bouillaud and, in 1889, Walter Cheadle published classic works on the subject.

The association between sore throat and rheumatic fever was not made until 1880. The connection with scarlet fever was made in the early 1900s. In 1944, the Jones criteria were formulated to assist disease identification. These criteria, with some modification, remain in use today. The introduction of antibiotics in the late 1940s allowed for the development of treatment and preventive strategies. Dramatic declines in the incidence of rheumatic fever are thought to be largely due to antibiotic treatment of streptococcal infection. However, there are pockets where the incidence is significant, especially in tropic areas.

However, research into the subtypes of streptococci has made it clear that differences among those types is also responsible for both the decline in overall US incidence and isolated outbreaks.

The most recent advance is the recognition that there is genetic predisposition to development of acute rheumatic fever, though the exact reason is still a matter of research.

Pathophysiology

Acute rheumatic fever is a sequela of a previous group A streptococcal infection, usually of the upper respiratory tract. One beta-streptococcal serotype (eg, M types 3, 5, 18, 19, 24) is linked directly to acute rheumatic fever. Two vaccines—one that is 26-valent and likely to proceed into clinical trials—are based on the M protein characteristics of responsible subtypes.

Good evidence suggests that there is genetic susceptibility to development of the disease. Several recent studies have shed light on genetic predisposition.^1,2

Non–group A streptococci has never been shown to cause this disease.

The disease involves the heart, joints, central nervous system (CNS), skin, and subcutaneous tissues. It is characterized by an exudative and proliferative inflammatory lesion of the connective tissue, especially that of the heart, joints, blood vessels, and subcutaneous tissue.

Frequency

United States

Disease prevalence in the United States is a function of socioeconomic status, with higher frequency in areas of crowding. The United States had experienced a resurgence of rheumatic fever in the last 2 decades, with many of the reported cases involving persons in upper socioeconomic groups. The reason for this disparity is unclear but may be caused by the emergence of more virulent strains of group A streptococci. The overall incidence has been declining in developed nations but is still rampant in less developed ones.

The incidence is low in most parts of the country but is variable. In a study published in 2006, Martin and Barbadora showed that the disease remains a problem in western Pennsylvania with 121 new cases from 1994-2003.³ Consistent with earlier reports, most patients were children and most had carditis.

Acute rheumatic fever is common among American Samoans in Hawaii.⁴

Frequency of streptococcal infection, virulence of the bacterial strain, and M protein subtypes determine the incidence of rheumatic fever in the population.

As a sequela of beta-streptococcal exposure, acute rheumatic fever occurs during the school-aged years when streptococcal pharyngitis is most prevalent. Similarly, prevalence is higher in the colder months of the year when streptococcal pharyngitis is most likely to occur.

International

Acute rheumatic fever (ARF) is a major problem in the high-risk areas of the tropics, in countries with limited resources, and in communities with minority indigenous populations. Although older literature estimates that 25-40% of cases worldwide appear in those nations, a recent paper suggests the figure may be closer to 95%.

In those less developed nations, post ARF heart disease is the most commonly acquired heart disease in hospitalized children, adolescents, and young adults. In some areas, the incidence of this entity exceeds that of congenital heart disease. Some studies point out the association with heart failure and death in pregnant women.

McDonald et al have suggested that in Aboriginal communities of central and northern Australia, group A streptococcal pyoderma is much more likely to cause acute rheumatic fever than is streptococcal pharyngitis.⁵

Wang et al reported on a possible acute rheumatic fever resurgence in Taiwan.⁶ Authors in India and Turkey make a plea for more liberal application of the Jones criteria in order to avoid misdiagnosis.^7,8 Meira et al report on the high incidence in Brazil.⁹ Others have reminded the medical community that good reporting of prevalence in underdeveloped nations is lacking.

Parks et al suggest that acute rheumatic fever is underdiagnosed in primary care clinics in the United Kingdom.¹⁰

Marijon et al believe that World Health Organization echocardiographic criteria for making the diagnosis in subclinical cases are inadequate. The group advocates for criteria that include valves with morphological changes consistent with rheumatic disease but without pathological regurgitation.¹¹

Mortality/Morbidity

Morbidity from acute rheumatic fever (ARF) is directly proportional to the rate of streptococcal infections. Infections that are not treated adequately are most likely to cause the major sequelae noted in the list of Jones criteria in Physical. Morbidity also is related to the care that the patient receives.

• The mortality rate has declined steadily over the last 3 decades. A partial explanation for the decrease in mortality rate may be the increase in antibiotic use. In developing nations and lower socioeconomic areas where rheumatic fever is more prevalent, acute rheumatic fever is a major cause of death and disability in children and adolescents.
• Cardiac involvement is the major cause of long-term morbidity. Valvular vegetations (endocarditis) are the cause of mitral valve regurgitation, the end result being left ventricular (LV) dilation and congestive heart failure (CHF). Myocarditis is present but not the cause of heart failure.
• Those with carditis as part of the initial episode are at greater risk of developing recurrences and of sustaining further cardiac injury.
• Carapetis et al estimated that worldwide, approximately 60% of all patients with ARF will develop rheumatic heart disease.¹² Further, they estimate a world burden of 2.4 million children aged 5-14 years affected or a total population of 15-20 million living with rheumatic heart disease.
• Patients without carditis during the initial episode have a relatively low risk of developing carditis during recurrences, although scattered case reports of carditis occurring only during a recurrence exist.
• Migratory polyarthritis occurs early in the disease course and is a common complaint for patients with rheumatic fever. Joint involvement ranges from arthralgia without objective findings to overt arthritis with warmth, swelling, redness, and exquisite tenderness. The larger joints such as the knees, ankles, elbows, and wrists are involved most frequently. An inverse relationship between severity of joint involvement and risk of carditis appears to exist.
• In approximately 75% of cases, the acute attack lasts only 6 weeks.
• Ninety percent of cases resolve in 12 weeks or less.
• Fewer than 5% of patients have symptoms that persist for 6 months or more.
• Literature began to appear in 1998 suggesting that acute rheumatic fever might be another disorder associated with PANDAS — pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. As of 2009, this is considered an unproven hypothesis.¹³

Race

In the United States, the attack rate is more a function of crowding than race, though the socioeconomic realities of those crowded conditions is no doubt a factor.

Sex

No sex predilection exists, except that mitral valve prolapse and Sydenham chorea occur more often in females than in males.

Age

Although individuals of any age group may be affected, most cases are reported in persons aged 5-15 years.

Yee lists rheumatic pericarditis and myocarditis as cardiac emergencies in the first year of life.¹⁴

Clinical

History

• Acute rheumatic fever (ARF) is associated with 2 distinct patterns of presentation.  
  • The first pattern of presentation is sudden onset. It typically begins as polyarthritis 2-6 weeks after streptococcal pharyngitis and is usually characterized by fever and toxicity.
  • If the initial abnormality is mild carditis, ARF may be insidious or subclinical.
• Age at onset influences the order of complications. Younger children tend to develop carditis first, whereas older patients tend to develop arthritis first.

Physical

Diagnosis of acute rheumatic fever (ARF) requires a high index of suspicion.

Guidelines of diagnosis used by the American Heart Association include major and minor criteria (ie, modified Jones criteria). In addition to evidence of a previous streptococcal infection, the diagnosis requires 2 major Jones criteria or 1 major plus 2 minor Jones criteria.

• Major criteria 
  • Carditis: This occurs in as many as 40% of patients and may include cardiomegaly, new murmur, congestive heart failure, and pericarditis, with or without a rub and valvular disease.
  • Migratory polyarthritis: This condition occurs in 75% of patients and is polyarticular, fleeting, and involves the large joints. Note that one group of authors has suggested that atypical cases may only involve small joints.⁷
  • Subcutaneous nodules (ie, Aschoff bodies): These nodules occur in 10% of patients and are edematous, fragmented collagen fibers. They are firm, painless nodules on the extensor surfaces of the wrists, elbows, and knees.
  • Erythema marginatum: This condition occurs in about 5% of patients. The rash is serpiginous and long lasting.
  • Chorea (also known as Sydenham chorea and "St Vitus dance"): This characteristic movement disorder occurs in 5-10% of cases. Sydenham chorea consists of rapid, purposeless movements of the face and upper extremities. Onset may be delayed for several months and may cease when the patient is asleep.
• Minor criteria
  • Clinical findings include arthralgia, fever, and previous history of ARF.
  • Laboratory findings include elevated acute-phase reactants (eg, erythrocyte sedimentation rate, C reactive protein), a prolonged PR interval, and supporting evidence of antecedent group A streptococcal infections (ie, positive throat culture or rapid streptococcal screen and an elevated or rising streptococcal antibody titer).

Note that the current Jones criteria are different than the original. Numerous authors have suggested that more changes may be in order. For example, some have suggested that echocardiography be performed in all suspected cases in order to avoid both underdiagnosis and overdiagnosis. Carapetis and Currie suggest that cases are missed because some patients have only monoarthritis and not polyarthritis.¹⁵ They would like to see monoarthritis become a major criterion. These same authors suggest that the set point of fever at 38 º C might be too high. As mentioned above, at least one author reported on atypical cases in which arthritis involved small joints rather than large joints. Finally, Rayamajhi et al suggest that arthralgia be changed from a minor to a major Jones criterion.¹⁶

As mentioned above, there are authorities who suggest that less stringent echocardiographic criteria for the diagnosis of rheumatic valvular disease will increase the number of cases diagnosed.¹¹

Causes

• Acute rheumatic fever (ARF) has been linked definitively with a preceding streptococcal infection, usually of the upper respiratory tract. Evidence is very strong that the M protein in certain streptococci subtypes is responsible for antigenicity.
• Although streptococcal skin infections also are extremely common, they have not been linked with acute rheumatic fever in the United States. Note the suggestion by McDonald et al that pyoderma may be the cause in Aboriginal populations of Australia.⁵
• See discussion under Pathophysiology for reference to genetic predisposition.

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