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Rheumatic fever

Rheumatic fever
Other namesAcute rheumatic fever (ARF)
Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium)
SpecialtyCardiology
SymptomsFever, multiple painful joints, involuntary muscle movements, erythema marginatum[1]
ComplicationsRheumatic heart disease, heart failure, atrial fibrillation, infection of the valves[1]
Usual onset2–4 weeks after a streptococcal throat infection, age 5–14 years[2]
CausesAutoimmune disease triggered by pathogenic strains of Streptococcus[1]
Risk factorsGenetic factors, malnutrition and poverty.[1]
Diagnostic methodBased on symptoms and infection history[3]
PreventionPrompt antibiotics for Streptococcus infections, improved sanitation[1][4]
TreatmentProlonged periods of antibiotics, valve replacement surgery, valve repair[1]
Frequency325,000 children a year[1]
Deaths319,400 (2015)[5]

Rheumatic fever (RF) is an inflammatory disease that can involve the heart, joints, skin, and brain.[1] The disease typically develops two to four weeks after a streptococcal throat infection.[2] Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum.[1] The heart is involved in about half of the cases.[1] Damage to the heart valves, known as rheumatic heart disease (RHD), usually occurs after repeated attacks but can sometimes occur after one.[1] The damaged valves may result in heart failure, atrial fibrillation and infection of the valves.[1]

Rheumatic fever may occur following an infection of the throat by the bacterium Streptococcus pyogenes.[1] If the infection is left untreated, rheumatic fever occurs in up to three percent of people.[6] The underlying mechanism is believed to involve the production of antibodies against a person's own tissues.[1] Due to their genetics, some people are more likely to get the disease when exposed to the bacteria than others.[1] Other risk factors include malnutrition and poverty.[1] Diagnosis of RF is often based on the presence of signs and symptoms in combination with evidence of a recent streptococcal infection.[3]

Treating people who have strep throat with antibiotics, such as penicillin, decreases the risk of developing rheumatic fever.[4] In order to avoid antibiotic misuse this often involves testing people with sore throats for the infection; however, testing might not be available in the developing world.[1] Other preventive measures include improved sanitation.[1] In those with rheumatic fever and rheumatic heart disease, prolonged periods of antibiotics are sometimes recommended.[1] Gradual return to normal activities may occur following an attack.[1] Once RHD develops, treatment is more difficult.[1] Occasionally valve replacement surgery or valve repair is required.[1] Otherwise complications are treated as usual.[1]

Rheumatic fever occurs in about 325,000 children each year and about 33.4 million people currently have rheumatic heart disease.[1][7] Those who develop RF are most often between the ages of 5 and 14,[1] with 20% of first-time attacks occurring in adults.[8] The disease is most common in the developing world and among indigenous peoples in the developed world.[1] In 2015 it resulted in 319,400 deaths down from 374,000 deaths in 1990.[5][9] Most deaths occur in the developing world where as many as 12.5% of people affected may die each year.[1] Descriptions of the condition are believed to date back to at least the 5th century BCE in the writings of Hippocrates.[10] The disease is so named because its symptoms are similar to those of some rheumatic disorders.[11]

  1. ^ a b c d e f g h i j k l m n o p q r s t u v w x y z aa Marijon E, Mirabel M, Celermajer DS, Jouven X (10 March 2012). "Rheumatic heart disease". The Lancet. 379 (9819): 953–64. doi:10.1016/S0140-6736(11)61171-9. PMID 22405798. S2CID 20197628.
  2. ^ a b Lee KY, Rhim JW, Kang JH (March 2012). "Kawasaki disease: laboratory findings and an immunopathogenesis on the premise of a "protein homeostasis system"". Yonsei Medical Journal. 53 (2): 262–75. doi:10.3349/ymj.2012.53.2.262. PMC 3282974. PMID 22318812.
  3. ^ a b "Rheumatic Fever 1997 Case Definition". cdc.gov. 3 February 2015. Archived from the original on 19 February 2015. Retrieved 19 February 2015.
  4. ^ a b Spinks A, Glasziou PP, Del Mar CB (9 December 2021). "Antibiotics for treatment of sore throat in children and adults". The Cochrane Database of Systematic Reviews. 2021 (12): CD000023. doi:10.1002/14651858.CD000023.pub5. ISSN 1469-493X. PMC 8655103. PMID 34881426.
  5. ^ a b GBD 2015 Mortality and Causes of Death Collaborators (8 October 2016). "Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980-2015: a systematic analysis for the Global Burden of Disease Study 2015". The Lancet. 388 (10053): 1459–1544. doi:10.1016/S0140-6736(16)31012-1. PMC 5388903. PMID 27733281.
  6. ^ Turkington C, Ashby BL (2007). The Encyclopedia of Infectious Diseases (3rd ed.). New York: Facts On File. p. 292. ISBN 978-0-8160-7507-2. The risk of severe complications is the primary concern with strep throat, and the reason why it is so important to be properly diagnosed and treated. One of the most serious complications is rheumatic fever, a disease that affects up to 3 percent of those with untreated strep infection. Rheumatic fever can lead to rheumatic heart disease.
  7. ^ GBD 2015 Disease and Injury Incidence and Prevalence Collaborators (8 October 2016). "Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990-2015: a systematic analysis for the Global Burden of Disease Study 2015". The Lancet. 388 (10053): 1545–1602. doi:10.1016/S0140-6736(16)31678-6. PMC 5055577. PMID 27733282.
  8. ^ Kumar V, Abbas AK, Fausto N, Mitchell RN (2007). Robbins Basic Pathology (8th ed.). Saunders Elsevier. pp. 403–6. ISBN 978-1-4160-2973-1.
  9. ^ GBD 2013 Mortality and Causes of Death Collaborators (17 December 2014). "Global, regional, and national age-sex specific all-cause and cause-specific mortality for 240 causes of death, 1990–2013: a systematic analysis for the Global Burden of Disease Study 2013". The Lancet. 385 (9963): 117–171. doi:10.1016/S0140-6736(14)61682-2. PMC 4340604. PMID 25530442.
  10. ^ Quinn RW (1991). "Did scarlet fever and rheumatic fever exist in Hippocrates' time?". Reviews of Infectious Diseases. 13 (6): 1243–4. doi:10.1093/clinids/13.6.1243. PMID 1775859.
  11. ^ "rheumatic fever" at Dorland's Medical Dictionary

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