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Rheumatic Fever in Emergency Medicine

  • Author: Steven J Parrillo, DO, FACOEP, FACEP; Chief Editor: Robert E O'Connor, MD, MPH  more...
 
Updated: Mar 06, 2014
 

Background

Rheumatic fever causes chronic progressive damage to the heart and its valves and is the most common cause of pediatric heart disease in the world. 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.

Research into the subtypes of streptococci has made it clear that differences among those types are 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.

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Pathophysiology

Acute rheumatic fever is a sequela of a previous group A streptococcal infection, usually of the upper respiratory tract. This is an autoimmune response secondary to molecular mimicry following group A streptococcal pharyngitis. 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] Susceptibility studies have focused on human leukocyte antigens, B-cell alloantigens, and cytokine genes. Study results, however, often conflict.[3]

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

Some have questioned the possibility that viruses may also be implicated as a correlating cause; however, most studies have shown no correlation, with the single exception of Epstein-Barr virus. In that setting, one author found DNA positivity in acute cases but not in controls.[4]

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.

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Epidemiology

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.[5] Consistent with earlier reports, most patients were children and most had carditis. Curiously, most studies report that rheumatic fever is very unusual if not rare in developed countries, but remains a major problem in developing nations.[6, 7]

Acute rheumatic fever is common among American Samoans in Hawaii.[8]

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.[6] ARF is clearly still a major problem is less developed areas of the world.[9] 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.[10]

Wang et al reported on a possible acute rheumatic fever resurgence in Taiwan.[11] Authors in India and Turkey make a plea for more liberal application of the Jones criteria in order to avoid misdiagnosis.[12, 13, 14] Similarly, Steer et al found a significant pocket of cases on the island of Fiji when clinicians liberally applied diagnostic criteria and followed with echocardiography.[15] Meira et al report on the high incidence in Brazil.[16] 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.[17] Other authors continue to report the problem of ARF in underdeveloped countries and in the indigenous populations of developed countries.[9]

Pastore et al studied cases in Trieste, Italy and report that acute rheumatic fever still occurs in industrialized countries.[18]

Marijon et al believe that the 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.[19]

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. ARF causes inflammation of valvular endocardium. One or more valves (most commonly the mitral valve) may be permanently deformed. Those valves are then dysfunctional and may lead to problems including left ventricular dilation and congestive heart failure, sometimes decades later. Vegetations may develop on damaged valves and become infected leading to endocarditis. Myocarditis is present but is not the direct 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. [20] 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. [21]

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. Paulo et al report that acute rheumatic fever can be found in children younger than 5 years with no significant difference in the frequency and severity of clinical signs.[22]

Yee lists rheumatic pericarditis and myocarditis as cardiac emergencies in the first year of life.[23]

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Contributor Information and Disclosures
Author

Steven J Parrillo, DO, FACOEP, FACEP Clinical Adjunct Professor, Medical Director and Faculty, Disaster Medicine and Management Masters Program, Philadelphia University College of Health Sciences; Associate Professor, Clinical and Educational Scholarship Track, Jefferson Medical College of Thomas Jefferson University; Director, Division of EMS and Disaster Medicine, Albert Einstein Healthcare Network

Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, World Association for Disaster and Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Anne Klimke, MD, MS Assistant Professor of Emergency Medicine, Jefferson Medical College of Thomas Jefferson University; Associate Director of EMS Fellowship, Attending Physician, Department of Emergency Medicine, Albert Einstein Medical Center; Attending Physician, Department of Emergency Medicine, Montgomery Hospital Medical Center

Anne Klimke, MD, MS is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Gino A Farina, MD, FACEP, FAAEM Professor of Emergency Medicine, Hofstra North Shore-LIJ School of Medicine at Hofstra University; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Robert E O'Connor, MD, MPH Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Association for Physician Leadership, American Heart Association, Medical Society of Delaware, Society for Academic Emergency Medicine, Wilderness Medical Society, American Medical Association, National Association of EMS Physicians

Disclosure: Nothing to disclose.

Additional Contributors

Assaad J Sayah, MD, FACEP Chief, Department of Emergency Medicine; Senior Vice President, Primary and Emergency Care, Cambridge Health Alliance

Assaad J Sayah, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians, Massachusetts Medical Society, National Association of EMS Physicians

Disclosure: Nothing to disclose.

Acknowledgements

Catherine V Parrillo, DO, FACOP, FAAP Retired Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine

Catherine V Parrillo, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

References
  1. Bryant PA, Robins-Browne R, Carapetis JR, Curtis N. Some of the people, some of the time: susceptibility to acute rheumatic fever. Circulation. 2009 Feb 10. 119(5):742-53. [Medline].

  2. Guilherme L, Kalil J. Rheumatic Fever and Rheumatic Heart Disease: Cellular Mechanisms Leading Autoimmune Reactivity and Disease. J Clin Immunol. 2009 Oct 3. [Medline].

  3. Bryant PA, Robins-Browne R, Carapetis JR, Curtis N. Some of the people, some of the time: susceptibility to acute rheumatic fever. Circulation. 2009 Feb 10. 119(5):742-53. [Medline].

  4. Olgunturk R, Okur I, Cirak MY, Oguz AD, Akalin N, Turet S, et al. The role of viral agents in aetiopathogenesis of acute rheumatic fever. Clin Rheumatol. 2011 Jan. 30(1):15-20. [Medline].

  5. Martin JM, Barbadora KA. Continued high caseload of rheumatic fever in western Pennsylvania: Possible rheumatogenic emm types of streptococcus pyogenes. J Pediatr. 2006 Jul. 149(1):58-63. [Medline].

  6. Shulman ST, Tanz RR. Group A streptococcal pharyngitis and immune-mediated complications: from diagnosis to management. Expert Rev Anti Infect Ther. 2010 Feb. 8(2):137-50. [Medline].

  7. Steer AC, Carapetis JR. Acute rheumatic fever and rheumatic heart disease in indigenous populations. Pediatr Clin North Am. 2009 Dec. 56(6):1401-19. [Medline].

  8. Erdem G, Dodd A, Tuua A, Sinclair S, I'atala TF, Marrone JR, et al. Acute rheumatic fever in the American Samoa. Pediatric Inf Dis J. Dec 2007. 26(12):1158-9. [Medline].

  9. Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Acute rheumatic fever and its consequences: a persistent threat to developing nations in the 21st century. Autoimmun Rev. 2009 Dec. 9(2):117-23. [Medline].

  10. McDonald M, Currie BJ, Carapetis JR. Acute rheumatic fever: a chink in the chain that links the heart to the throat?. Lancet Infect Dis. 2004 Apr. 4(4):240-5. [Medline].

  11. Wang CR, Liu CC, Li YH, Liu MF. Adult-onset acute rheumatic fever: possible resurgence in southern Taiwan. J Clin Radiology. June 2005. 11(3):146-149. [Medline].

  12. Olgunturk R, Canter B, Tunaoglu FS, Kula S. Review of 609 patients with acute rheumatic fever in terms of revised and updated Jones criteria. Int J Cardiol. Sep 10 2006. 112(1):91-8. Epub 2005 Dec 20. [Medline].

  13. Vijayalakshmi IB, Mithravinda J, Deva AN. The role of echocardiography in diagnosing carditis in the setting of acute rheumatic fever. Cardiol Young. Dec 2005. 15(6):583-8. [Medline].

  14. Tavli V, Canbal A, Saylan B, Saritas T, Mese T, Atlihan F. Assessment of myocardial involvement using troponin-I and echocardiography in rheumatic carditis in Izmir, Turkey. Pediatr Int. Feb 2008. 50(1):62-4. [Medline].

  15. Steer AC, Kado J, Wilson N, Tuiketei T, Batzloff M, Waqatakirewa L, et al. High prevalence of rheumatic heart disease by clinical and echocardiographic screening among children in Fiji. J Heart Valve Dis. 2009 May. 18(3):327-35; discussion 336. [Medline].

  16. Meira ZM, Goulart EM, Colosimo EA, Mota CC. Long term follow up of rheumatic fever and predictors of severe rheumatic valvar disease in Brazilian children and adolscents. Heart. Aug 2005. 91(8):1019-22. [Medline].

  17. Parks T, Kado J, Colquhoun S, Carapetis J, Steer A. Underdiagnosis of acute rheumatic fever in primary care settings in a developing country. Trop Med Int Health. 2009 Nov. 14(11):1407-13. [Medline].

  18. Pastore S, De Cunto A, Benettoni A, Berton E, Taddio A, Lepore L. The resurgence of rheumatic fever in a developed country area: the role of echocardiography. Rheumatology (Oxford). 2011 Feb. 50(2):396-400. [Medline].

  19. Marijon E, Celermajer DS, Tafflet M, El-Haou S, Jani DN, Ferreira B, et al. Rheumatic heart disease screening by echocardiography: the inadequacy of World Health Organization criteria for optimizing the diagnosis of subclinical disease. Circulation. 2009 Aug 25. 120(8):663-8. [Medline].

  20. Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis. 2005 Nov. 5(11):685-94. [Medline].

  21. [Guideline] Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. 2009 Mar 24. 119(11):1541-51. [Medline].

  22. Paulo LT, Terreri MT, Barbosa CM, Len CA, Hilário MO. [Is rheumatic fever a more severe disease in pre-school children?]. Acta Reumatol Port. 2009 Jan-Mar. 34(1):66-70. [Medline].

  23. Yee L. Cardiac emergencies in the first year of life. Emerg Med Clin North Am. 2007 Nov. 25(4):981-1008. [Medline].

  24. Kiliç A, Unüvar E, Tatli B, Gökçe M, Omeroglu RE, Oguz F, et al. Neurologic and cardiac findings in children with Sydenham chorea. Pediatr Neurol. 2007 Mar. 36(3):159-64. [Medline].

  25. Carapetis JR, Currie BJ. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Arch Dis Child. Sep 2001. 85(3):223-7. [Medline].

  26. Rayamajhi A, Sharma D, Shakya U. Clinical, laboratory and echocardiographic profile of acute rheumatic fever in Nepali children. Ann Trop Paediatr. Sep 2007. 27(3):169-77. [Medline].

  27. Karacan M, Isikay S, Olgun H, Ceviz N. Asymptomatic rhythm and conduction abnormalities in children with acute rheumatic fever: 24-hour electrocardiography study. Cardiol Young. 2010 Dec. 20(6):620-30. [Medline].

  28. Baltimore RS. Re-evaluation of antibiotic treatment of streptococcal pharyngitis. Curr Opin Pediatr. 2010 Feb. 22(1):77-82. [Medline].

  29. Kerdemelidis M, Lennon DR, Arroll B, Peat B, Jarman J. The primary prevention of rheumatic fever. J Paediatr Child Health. 2010 Sep. 46(9):534-48. [Medline].

  30. Lennon D, Kerdemelidis M, Arroll B. Meta-analysis of trials of streptococcal throat treatment programs to prevent rheumatic Fever. Pediatr Infect Dis J. 2009 Jul. 28(7):e259-64. [Medline].

  31. Birdi N, Hosking M, Clulow MK, Duffy CM, Allen U, Petty RE. Acute rheumatic fever and poststreptococcal reactive arthritis: diagnostic and treatment practices of pediatric subspecialists in Canada. J Rheumatol. 2001 Jul. 28(7):1681-8. [Medline].

  32. [Guideline] Bisno AL, Gerber MA, Gwaltney JM Jr, Kaplan EL, Schwartz RH. Practice guidelines for the diagnosis and management of group A streptococcal pharyngitis. Infectious Diseases Society of America. Clin Infect Dis. 2002 Jul 15. 35(2):113-25. [Medline].

  33. Gerber MA. Rheumatic fever. Kliegman RM, Stanton BF, St. Geme JW, Shor N, Behrman RE eds. Nelson: Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier; 2011. 920-5.

  34. Hashkes PJ, Tauber T, Somekh E, Brik R, Barash J, Mukamel M, et al. Naproxen as an alternative to aspirin for the treatment of arthritis of rheumatic fever: a randomized trial. J Pediatr. 2003 Sep. 143(3):399-401. [Medline].

  35. Khriesat I, Najada AH. Acute rheumatic fever without early carditis: an atypical presentation. Eur J Pediatrics. Dec 2003. 162(12):868-71. [Medline].

  36. Lee GM, Wessels MR. Changing epidemiology of acute rheumatic fever in the United States. Clin Infect Dis. 2006 Feb 15. 42(4):448-50. Epub 2006 Jan 17. [Medline].

  37. Ohlsson A, Clark K. Antibiotics for sore throat to prevent rheumatic fever: yes or no? How the Cochrane Library can help. CMAJ. 2004 Sep 28. 171(7):721-3. [Medline].

  38. Padmavati S. Rheumatic heart disease: prevalence and preventive measures in the Indian subcontinent. Keywords: rheumatic heart disease; rheumatic fever. Heart. 2001 Aug. 86(2):127. [Medline].

  39. Steer AC, Carapetis JR, Nolan TM, Shann F. Systematic review of rheumatic heart disease prevalence in children in developing countries: the role of environmental factors. J Paediatr Child Health. 2002 Jun. 38(3):229-34. [Medline].

 
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