Rheumatic Fever

Title

Other Names:

Acute Rheumatic Fever; Inflammatory Rheumatism

Categories:

Bacterial infections

Summary Summary

Rheumatic fever is an inflammatory condition that may develop after infection with group A Streptococcus bacteria, such as strep throat or scarlet fever. It is primarily diagnosed in children between the ages of 6 and 16 and can affect the heart, joints, nervous system and/or skin. Early signs and symptoms include sore throat; swollen red tonsils; fever; headache; and/or muscle and joint aches. Some affected people develop rheumatic heart disease, which can lead to serious inflammation and scarring of the heart valves.^[1]^[2]^[3] It is not clear why some people who are infected with group A Streptococcus bacteria go on to develop rheumatic fever, while others do not; however, it appears that some families may have a genetic susceptibility to develop the condition.^[4]^[5] Treatment usually includes antibiotics and/or anti-inflammatory medications.^[2]^[6]

Last updated: 1/11/2015

Symptoms Symptoms

Rheumatic fever is primarily diagnosed in children between the ages of 6 and 16 and can affect many different systems of the body, including the heart, joints, nervous system and/or skin. The condition usually develops approximately 14-28 days after infection with group A Streptococcus bacteria, such as strep throat or scarlet fever. Early signs and symptoms may include sore throat; swollen red tonsils; fever; headache; and/or muscle aches. Affected people may also experience:^[1]^[2]^[3]

Abdominal pain
Rheumatic heart disease
Joint pain and/or swelling
Nosebleeds
Skin nodules (painless, firm, round lumps underneath the skin)
Skin rash
Sydenham chorea (abrupt, non-repetitive limb movements and grimaces)

People with a history of rheumatic fever have a high risk of developing recurrent episodes of the condition. This can cause progressive (worsening over time) heart damage.^[1]^[2]

Last updated: 1/11/2015

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Abnormality of the immune system	-
Fever	-

Last updated: 9/1/2017

Cause Cause

Rheumatic fever is an inflammatory condition that may develop approximately 14-28 days after infection with group A Streptococcus bacteria, such as strep throat or scarlet fever.^[3] About 5% of those with untreated strep infection will develop rheumatic fever. Although group A Streptococcus bacterial infections are highly contagious, rheumatic fever is not spread from person to person.^[1] The exact underlying cause of the condition is not well understood and it is unclear why some people with strep infections go on to develop rheumatic fever, while others do not. However, some scientists suspect that an exaggerated immune response in genetically susceptible people may play a role in the development of the condition.^[2]^[7]

Last updated: 1/11/2015

Inheritance Inheritance

Rheumatic fever is likely inherited in a multifactorial manner, which means it is caused by multiple genes interacting with each other and with environmental factors. The condition is thought to occur in genetically susceptible children who are infected with group A Streptococcus bacteria and live in poor social conditions.^[4] Some studies suggest that differences in the expression of various genes involved in the immune response may contribute to rheumatic fever susceptibility.^[5]

Last updated: 1/12/2015

Diagnosis Diagnosis

A diagnosis of rheumatic fever is usually based on the following:^[2]^[8]

Characteristic signs and symptoms identified by physical examination and/or specialized testing such as a blood test, chest X-ray and echocardiogram
Confirmation of group A Streptococcus bacterial infection with a throat culture or blood tests

The diagnosis can also be supported by blood tests that confirm the presence of certain proteins that increase in response to inflammation (called acute-phase reactants) and tend to be elevated in rheumatic fever. Additional tests may be recommended to rule out other conditions that cause similar features.^[8]^[1]^[2]^[3]

Last updated: 1/12/2015

Treatment Treatment

Treatment of rheumatic fever usually consists of antibiotics to treat the underlying group A Streptococcus bacterial infection and anti-inflammatory medications such as aspirin or corticosteroids. Because people with a history of rheumatic fever have a high risk of developing recurrent episodes of the condition, low dose antibiotics are often continued over a long period of time to prevent recurrence.^[1]^[2]^[3]^[6]

Last updated: 1/12/2015

Prognosis Prognosis

The long-term outlook (prognosis) for people with rheumatic fever depends on the severity of the heart involvement at the initial diagnosis. Severe carditis (inflammation of the heart) is associated with a poor prognosis and generally leads to rheumatic heart disease. People with a history of rheumatic fever have a high risk of developing recurrent episodes of the condition, which can cause progressive (worsening over time) heart damage. Prophylactic low dose antibiotics can significantly improve prognosis in many cases by preventing these recurrences.^[2]^[3]

Last updated: 1/12/2015

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are studying or have studied Rheumatic Fever. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

World Heart Federation
32, rue de Malatrex
1201 Geneva
Switzerland
Telephone: (+41 22) 807 03 20
E-mail: info@worldheart.org
Website: http://www.world-heart-federation.org/
Information about rheumatic heart disease: http://www.world-heart-federation.org/what-we-do/rheumatic-heart-disease-rhd/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? Send us your suggestions.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Genetics Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by GeneTests, the American College of Medical Genetics, and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manual provides information on this condition for patients and caregivers.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Merck Manual for health care professionals provides information on Rheumatic Fever.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
PubMed is a searchable database of medical literature and lists journal articles that discuss Rheumatic Fever. Click on the link to view a sample search on this topic.

News & Events News & Events

News

Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017

NCATS Co-Sponsored Conferences

Workshop on Stem Cell Therapy for Rheumatic and Autoimmune Disease Friday, September 11, 1998
Location: NIH Campus, Bethesda, MD
Contact: Ms. Joanne Odenkirchen(301) 594-5055

Co-funding Institute(s): National Institute of Arthritis and Musculoskeletal and Skin Diseases

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References References

Rheumatic Fever. NORD. April 2009; http://rarediseases.org/rare-disease-information/rare-diseases/byID/469/viewAbstract.
Mark R Wallace, MD, FACP, FIDSA. Rheumatic Fever. Medscape Reference. March 2014; http://emedicine.medscape.com/article/236582-overview.
Rheumatic fever. MedlinePlus. May 2012; http://www.nlm.nih.gov/medlineplus/ency/article/003940.htm.
Engel ME, Stander R, Vogel J, Adeyemo AA, Mayosi BM. Genetic susceptibility to acute rheumatic fever: a systematic review and meta-analysis of twin studies. PLoS One. 2011; 6(9):1-6.
Bryant PA, Smyth GK, Gooding T, Oshlack A, Harrington Z, Currie B, Carapetis JR, Robins-Browne R, Curtis N. Susceptibility to acute rheumatic fever based on differential expression of genes involved in cytotoxicity, chemotaxis, and apoptosis. Infect Immun. February 2014; 82(2):753-761.
Allan Gibofsky, MD, JD, FACP, FCLM; John B Zabriskie, MD. Treatment and prevention of acute rheumatic fever. UpToDate. October 2013; Accessed 1/11/2015.
RHEUMATIC FEVER-RELATED ANTIGEN. OMIM. June 2009; http://www.omim.org/entry/268240.
Allan Gibofsky, MD, JD, FACP, FCLM; John B Zabriskie, MD. Clinical manifestations and diagnosis of acute rheumatic fever. UpToDate. October 2013; Accessed 1/12/2015.