What is rheumatic fever

Rheumatic fever is a autoimmune inflammatory disease that affects many of the body’s connective tissues especially those of the heart, joints, brain, blood vessels, and skin. Rheumatic fever is a very rare complication of untreated Streptococcus pyogenes throat infection (pharyngitis) or scarlet fever infection (also called group A Streptococcus or group A strep). Before antibiotic medicines became widely used, rheumatic fever was the single biggest cause of heart valve disease. Children aged 5 to 15 years old are most at risk, especially children living in rural or remote areas or in difficult conditions. Rheumatic fever is very rare in the U.S. and can be prevented by the prompt diagnosis and treatment (with antibiotics) of strep throat. Worldwide, however, rheumatic heart disease remains a significant cause of cardiovascular disease.

Global estimates of the burden of rheumatic fever disease:

• 470,000 new cases of acute rheumatic fever each year ¹
• 282,000 new cases of rheumatic heart disease each year ¹
• Approximately 30 million people are living with rheumatic heart disease ²
• 305,000 people die each year from rheumatic heart disease or its complications ²

The annual national incidence of acute rheumatic fever in the United States is largely unknown as it is no longer a nationally notifiable disease; however, there is likely significant regional variation. For instance, Hawaii and American Samoa have higher annual incidence rates than the continental United States, and may be as high as 1.1 to 1.5 cases per 1,000 population ³. The rate in Tennessee was estimated to be 0.6 cases per 100,000 population in 1977–1981 ⁴. The rate in Utah, a state with a history of resurgences of acute rheumatic fever in the 1980s, has been estimated to be 3.7 cases per 100,000 population ⁵. Hospitalization data suggests that Asian/Pacific Islanders, Hispanics, blacks, and Native Americans were more likely to be hospitalized for acute rheumatic fever compared to white persons ⁶.

Rheumatic fever can damage body tissues by causing them to swell, but its greatest danger lies in the damage it can do to your heart. More than half of the time, rheumatic fever leads to scarring of the heart’s valves. This scarring can narrow the valve and make it harder for the valve to open or close properly, causing your heart to work harder pumping blood to the rest of your body. This valve damage can lead to a condition called rheumatic heart disease, which, in time, can lead to congestive heart failure.

Symptoms and signs of rheumatic fever may include:

• sore throat, usually starting 2-6 weeks earlier
• fever
• feeling tired
• muscle aches
• joint aches and pain
• a rash on the trunk or limbs (although this is uncommon)

Rheumatic fever is a serious condition and rheumatic fever must be treated right away. Early treatment with antibiotics can prevent long-term, serious complications of rheumatic heart disease. Most people make a full recovery, but it can come back.

If you have a sore throat that lasts longer than 3 days, or if you have a fever and headache along with your sore throat, you should see your doctor for a throat culture. Even if you do not have a sore throat but have a fever and a skin rash, this could also mean a strep infection, and you should get tested. Remember rheumatic fever can result from an untreated strep infection, so it is very important to treat the infection before it leads to a worse condition.

If you or your child are diagnosed with rheumatic fever, you’ll have treatment to relieve the symptoms and control inflammation.

You may need:

• antibiotics
• painkillers – given as tablets, capsules or a liquid you drink
• steroid injections – if your pain is severe
• medicines – if you’re having jerky, uncontrollable movements

You should also get plenty of bed rest to help with your recovery.

Most people usually make a full recovery after about a month. But it can sometimes take longer to get better.

Ongoing treatment for rheumatic fever

If you have had rheumatic fever once, it makes it more likely that it could come back, so make sure to get sore throats treated early. You may also be advised to take antibiotics for several years to try to stop it returning.

It’s less likely that it’ll come back if it’s been 5 years since you last had an episode and if you’re older than 25.

But rheumatic fever can cause permanent damage to your heart’s valves (rheumatic heart disease). This can take years to show up, so you may need regular check-ups and further treatment when you’re older.

Always ask a doctor what ongoing treatment you may need.

Is rheumatic fever contagious?

No. Streptococcus bacteria is contagious, but rheumatic fever isn’t.

Who is at risk for rheumatic fever?

Fewer than 0.3% of people who have strep throat also get rheumatic fever. Rheumatic fever is most common among children aged 5 to 15, but adults may have the condition as well. Doctors think that a weakened immune system may make some people more likely to get rheumatic fever. And, although antibiotic medicines have reduced the number of cases of rheumatic fever in developed countries, there are still thousands of reported cases.

Rheumatic fever causes

Rheumatic fever is not an infection itself, but rather the result of an untreated Streptococcus pyogenes throat infection (pharyngitis). Rheumatic fever is a delayed autoimmune reaction to the Streptococcus pyogenes bacteria, which usually occurs only if your child isn’t diagnosed and treated promptly. The exact disease process is not fully known. However, rheumatic fever disease is in part due to an autoimmune response to Streptococcus pyogenes infection involving multiple organ systems. Organ systems involved typically include the heart, joints, and central nervous system. Streptococcal pharyngitis typically precedes the onset of acute rheumatic fever by 1 to 5 weeks ⁷.

Streptococcus pyogenes are gram-positive cocci that grow in chains. They exhibit β-hemolysis (complete hemolysis) when grown on blood agar plates. They belong to group A in the Lancefield classification system for β-hemolytic Streptococcus, and thus are also called group A streptococci.

When your body senses the strep infection, it sends antibodies to fight it. Sometimes, these antibodies attack the tissues of your joints or heart instead. If the antibodies attack your heart, they can cause your heart valves to swell, which can lead to scarring of the valve “doors” (called leaflets or cusps). Symptoms usually start one to five weeks after your child has been infected.

Risk factors for rheumatic fever

Inadequate or lack of antibiotic treatment of streptococcal pharyngitis increases the risk of someone developing acute rheumatic fever. In approximately one-third of patients, acute rheumatic fever follows subclinical streptococcal infections or infections for which medical attention was not sought ⁸.

Individuals with a history of acute rheumatic fever have an increased risk of recurrence with subsequent streptococcal pharyngeal infections ⁸. The risk of recurrence after streptococcal infection is highest within the first few years after the initial attack and then declines.

The incidence of acute rheumatic fever is highest in children between the ages of 5 and 15 years. Acute rheumatic fever is very rare in children 3 years of age and younger in the United States. First-onset acute rheumatic fever is rare in adults, although recurrence may occur through adulthood ⁹.

Crowding, such as found in schools, military barracks, and daycare centers, increases the risk of spreading group A strep and thus increases the risk of developing acute rheumatic fever.

Rheumatic fever prevention

Diagnosis and adequate antibiotic treatment of group A strep pharyngitis is the primary means of preventing acute rheumatic fever. However, in about one-third of patients, acute rheumatic fever follows subclinical streptococcal infections or infections for which medical attention was not sought ⁸.

Secondary prevention of rheumatic fever requires antibiotic prophylaxis to reduce the likelihood of recurrent attacks in persons with a history of acute rheumatic fever. Because acute rheumatic fever frequently recurs with subsequent group A strep pharyngitis infections, long-term prophylaxis duration should be individually tailored but is usually indicated at least until age 21. Prophylaxis typically involves an intramuscular injection of benzathine penicillin every 4 weeks or oral penicillin V twice daily. Sulfadiazine or oral macrolides can be taken daily by individuals who are allergic to penicillin ¹⁰. Current American Heart Association guidelines no longer recommend bacterial endocarditis prophylaxis for patients with rheumatic heart disease, unless the patient has a prosthetic valve ¹¹.

The spread of group A strep infection can be reduced by good hand hygiene, especially after coughing and sneezing and before preparing foods or eating, and respiratory etiquette (e.g., covering your cough or sneeze). Treating an infected person with an antibiotic for 24 hours or longer generally eliminates their ability to transmit the bacteria. Thus, people with group A strep pharyngitis should stay home from work, school, or daycare until afebrile and until at least 24 hours after starting appropriate antibiotic therapy.

Rheumatic fever signs and symptoms

Acute rheumatic fever is usually characterized by fever and affects multiple organ systems. Symptoms of rheumatic fever usually begin 1 to 6 weeks after you have had a strep infection.

Each child may experience symptoms differently, but common symptoms include:

• Joint inflammation (arthritis), including swelling, tenderness, and redness over multiple joints. The joints affected are usually the larger joints in the ankles, knees, wrists or elbows; the inflammation “moves” from one joint to another over several days.
• Small nodules or hard, round bumps under the skin
• A change in your child’s handwriting, or unusual jerky movements
• A raised pink rash or red rash with odd edges on your chest, back, or stomach
• Fever, a high temperature of 100.4 °F (38 °C) or above
• Weight loss
• Fatigue
• Stomach pains or feeling less hungry
• Weakness, shortness of breath, or feeling very tired

Cardiovascular system

Carditis is the major cardiac manifestation of acute rheumatic fever, occurring in 50% to 70% of first episodes, and is associated with valvulitis. Clinical signs of carditis include cardiomegaly, new onset heart murmur (usually with mitral or aortic valvular disease), pericardial friction rub, pericardial effusion, and congestive heart failure. Additionally, a prolonged PR interval can be seen on electrocardiography.

Subclinical carditis may also be present. In these cases, classic murmurs may not be appreciated on physical exam, and valve disease is found solely by echocardiography/Doppler studies ¹². Carditis is the only manifestation that may result in long-term disability or death.

Musculoskeletal system

Polyarthritis is the major musculoskeletal manifestation. The arthritis is typically migratory and involves the following large joints: elbows, wrists, knees, and ankles. Joint involvement may range from general arthralgia to a painful, inflammatory arthritis.

Skin

Subcutaneous nodules and erythema marginatum are the two major skin manifestations. Subcutaneous nodules are firm, painless, variable in size (typically between a few millimeters and 2 centimeters in diameter), and usually found over joint extensor surfaces. Nodules are most commonly present in patients with carditis.

Erythema marginatum is an erythematous, non-pruritic, non-painful macular lesion on the trunk or proximal extremities. Lesions are transient and tend to extend outward with central clearing and are often described as serpiginous.

Figure 1. Rheumatic fever rash

Central Nervous System

Chorea, also called Sydenham’s chorea or St. Vitus dance, is the major central nervous system manifestation. Chorea is a neurological disorder characterized by abrupt, purposeless, non-rhythmic, involuntary movements that is often associated with muscle weakness and emotional lability. Chorea often appears after the other manifestations of acute rheumatic fever. It also can appear as the only manifestation of acute rheumatic fever ⁸.

Rheumatic fever complications

Inflammation caused by rheumatic fever can last a few weeks to several months. In some cases, the inflammation causes long-term complications.

Rheumatic heart disease is the most important long-term complication of acute rheumatic fever due to its ability to cause disability or death ⁸. Rheumatic heart disease is permanent damage to the heart caused by rheumatic fever. It usually occurs 10 to 20 years after the original illness. Problems are most common with the valve between the two left chambers of the heart (mitral valve), but the other valves can be affected. The damage can result in:

• Valve stenosis. This narrowing of the valve decreases blood flow.
• Valve regurgitation. This leak in the valve allows blood to flow in the wrong direction.
• Damage to heart muscle. The inflammation associated with rheumatic fever can weaken the heart muscle, affecting its ability to pump.

Damage to the mitral valve, other heart valves or other heart tissues can cause problems with the heart later in life. Resulting conditions can include:

• An irregular and chaotic beating of the upper chambers of the heart (atrial fibrillation)
• An inability of the heart to pump enough blood to the body (heart failure)

Untreated rheumatic fever increases a person’s risk of recurrent attacks and worsens prognosis. Prognosis is related to the prevention of recurrent attacks, degree of cardiac valvular damage, and degree of overall cardiac involvement. Cardiac complications may vary in severity and include, but are not limited to, pericarditis, endocarditis, arrhythmias, valvular damage, and congestive heart failure.

Rheumatic fever diagnosis

There is no definitive diagnostic test for acute rheumatic fever. A clinical diagnosis of acute rheumatic fever should be made using the Jones Criteria. A 2015 revised version of the Jones Criteria endorsed by the American Heart Association now includes the addition of subclinical carditis as a major criteria and stratification of the major and minor criteria based upon epidemiologic risk (e.g., low, moderate, or high risk populations) ¹².

The presence of 2 major manifestations or 1 major and 2 minor manifestations (see below) indicates a high probability of an initial acute rheumatic fever illness in any risk population. More than one joint and more than one cardiac manifestation can only be classified as either one major or one minor criteria, not both. For example, if there is evidence of carditis (a major criteria), a prolonged PR interval should not also be counted as a minor criteria. Similarly, if there is evidence of arthritis (a major criteria), then arthralgia should not also be counted as a minor criteria.

In most cases, there should also be evidence of preceding group A streptococcal infection.2 Evidence to support an antecedent group A strep infection include:

• Positive throat culture or rapid streptococcal antigen test
• Elevated or rising streptococcal antibody titer

Table 1: Revised Jones Criteria for diagnosing acute rheumatic fever

Footnote: *Low-risk population is defined as an acute rheumatic fever incidence of <2 per 100,000 school-aged children or all age rheumatic heart disease prevalence of ≤1 per 1000 population per year. Those not included in the low-risk population are defined as moderate or high risk depending upon their reference population.

Abbreviations: ESR = erythrocyte sedimentation rate; CRP = C-reactive protein; mm = millimeters; mg/dl = milligrams per deciliter

In addition, routine echocardiography/Doppler is now recommended for all confirmed or suspected acute rheumatic fever cases regardless of the presence or absence of murmur on physical exam ¹². A heart murmur without echocardiographic evidence of carditis and other clinical signs or symptoms can exclude the presence of rheumatic carditis ¹².

In some instances, a presumptive diagnosis of acute rheumatic fever can be made without fulfilling the Jones Criteria. For example, when clinical evidence is lacking in areas of high acute rheumatic fever incidence, clinical judgment must be used regarding the appropriate diagnosis and use of antibiotic prophylaxis ¹². In addition, acute rheumatic fever can be considered in cases of chorea and indolent, chronic carditis despite the lack of group A streptococcal laboratory confirmation or fulfillment of Jones Criteria ¹².

Diagnosis of recurrent rheumatic fever disease

Individuals with a history of rheumatic heart disease or prior episode of acute rheumatic fever are at increased risk for recurrences of acute rheumatic fever. In these individuals, a presumptive diagnosis of a recurrence can be made in the presence of a documented group A streptococcal infection with: a) 2 major manifestations, b) 1 major and 2 minor manifestations, or c) 3 minor manifestations (see Table 1 above for additional details) ¹². If relying on the presence of 3 minor manifestations, the diagnosis of recurrent acute rheumatic fever should only be made if other more likely causes have been excluded.

Rheumatic fever treatment

Treatments vary based on your child’s age, health, and medical history, the extent of your child’s disease, his tolerance for specific medications, procedures, and therapies, and, of course, your own opinions and preference will be considered. Treatment in most cases combines the following three approaches:

• Antibiotic treatment: Patients should also be started on antibiotics for treatment of group A strep pharyngitis, regardless of the presence or absence of pharyngitis at the time of diagnosis, in order to eradicate any residual group A strep carriage ¹⁰. Your child may continue to receive monthly doses of antibiotics to help prevent further complications.
• Anti-inflammatory medication: Your doctor will prescribe a pain reliever, such as aspirin or naproxen (Naprosyn), to reduce inflammation that occurs in the heart muscle, as well as to relieve joint pain. If symptoms are severe or your child isn’t responding to the anti-inflammatory drugs, your doctor might prescribe a corticosteroid.
• Anticonvulsant medications. For severe involuntary movements caused by Sydenham chorea, your doctor might prescribe an anticonvulsant, such as valproic acid (Depakene) or carbamazepine (Carbatrol, Tegretol, others).
• Management of cardiac failure.
• Bed rest: Often for between two and twelve weeks, depending on the severity of your child’s disease and the involvement of the heart and joints.

If your doctor tells you that you have a strep infection, he or she will prescribe an antibiotic medicine. It is important that you take the antibiotic when and how your doctor tells you. Do not stop taking the antibiotic just because you start to feel better. Many people find that they feel better after a couple of days of therapy, so they stop taking their antibiotic medicine. Even if your sore throat does not come back, without the antibiotics in your bloodstream, the streptococcal bacteria can still multiply and affect your heart and other organs.

If your strep infection leads to rheumatic fever, your doctor may prescribe anti-inflammatory medicines or aspirin to reduce the swelling in your body’s tissues. Sometimes, patients need to take a diuretic to help rid their body of excess water and salt. How long you take them depends on how old you are, how many attacks you have had, and how severe your symptoms are.

After your child has completed the full antibiotic treatment, your doctor will begin another course of antibiotics to prevent recurrence of rheumatic fever. Preventive treatment will likely continue through age 21 or until your child completes a minimum five-year course of treatment, whichever is longer. Close follow-up with your child’s physician is needed. Discuss with your doctor what type of follow-up and long-term care your child will need. Heart damage from rheumatic fever might not show up for years. When your child grows up, he or she needs to include the information in his or her medical history and get regular heart exams.

People who have had heart inflammation during rheumatic fever might be advised to take the preventive antibiotic treatment for 10 years or longer.

In some patients, rheumatic fever damages a heart valve. In these cases, your doctor may recommend surgery to repair or replace the damaged valve.

Lifestyle changes

If rheumatic fever has led to rheumatic heart disease or damage to your heart valves, your doctor may recommend that you take antibiotic medicines continuously for many years. Some patients with rheumatic fever need to take antibiotics for the rest of their lives. In any case, you should always tell your doctor or dentist about your history of rheumatic fever before you have a surgical or dental procedure. Such procedures may cause bacteria to enter the bloodstream and infect your heart valves.

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