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==Risk Factors==
==Risk Factors==
Common risk factors in the development of rheumatic fever include low socioeconomic status, inadequate healthcare, and poor sanitation.<ref name=WHO> Rheumatic Fever and Rheumatic Heart Disease. World Health Organization (2004). http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf Accessed on October 12, 2015. </ref><ref name="pmid1783067">{{cite journal| author=Vlajinac H, Adanja B, Marinković J, Jarebinski M| title=Influence of socio-economic and other factors on rheumatic fever occurrence. | journal=Eur J Epidemiol | year= 1991 | volume= 7 | issue= 6 | pages= 702-4 | pmid=1783067 | doi= | pmc= | url= }} </ref>


==Screening==
Screening of rheumatic fever is important as many cases of rheumatic heart disease are subclinical. [[Echocardiography]] among inhabitants of high risk regions is recommended. If any abnormality is detected on echocardiography, further cardiac evaluation is performed followed by antimicrobial therapy.<ref name="pmid18297157">{{cite journal| author=Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Sidi D et al.| title=Echocardiographic screening for rheumatic heart disease. | journal=Bull World Health Organ | year= 2008 | volume= 86 |issue= 2 | pages= 84 | pmid=18297157 | doi= | pmc=PMC2647380 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18297157  }} </ref>
==Natural History, Complications, and Prognosis==


==Screening==
Screening of rheumatic fever and carditis is important as many cases of RHD are subclinical. [[Echocardiography]] in inhabitants of high risk regions is recommended. If any abnormality is detected on echocardiography, further cardiac evaluation is done followed by prophylactic treatment<ref name="pmid18297157">{{cite journal| author=Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Sidi D et al.| title=Echocardiographic screening for rheumatic heart disease. | journal=Bull World Health Organ | year= 2008 | volume= 86|issue= 2 | pages= 84 | pmid=18297157 | doi= | pmc=PMC2647380 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18297157  }} </ref>.


==Diagnosis==
==Diagnosis==

Revision as of 18:32, 26 October 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Varun Kumar, M.B.B.S. [2]; Anthony Gallo, B.S. [3]

Overview

Rheumatic fever is a systemic immune disease that may develop after an infection with Streptococcus bacteria, such as strep throat and scarlet fever. It usually affects the heart, joints, blood vessels, and brain. Based on the duration of symptoms, rheumatic fever may be classified into either acute or chronic.[1] The most common cause of rheumatic fever is Group A beta-hemolytic streptococci infection.[2][3] If left untreated, rheumatic fever may cause valvular diseases including stenosis, regurgitation of mitral/aortic valves and myocarditis. This may lead to decreased cardiac output, pulmonary edema and ultimately cardiac failure. For cases without carditis, the prognosis is excellent, demonstrating no residual heart disease. In cases with preexisting heart disease, the prognosis is poor, leading to mortality.[4] Common complications of rheumatic fever include arrhythmias, systemic emboli, and stroke.[5][6][7] Common physical examination findings include fever, cardiac murmurs, and erythema marginatum.[8][9] Echocardiogram and radiograph may be diagnostic of rheumatic fever. The Jones criteria is used to establish the diagnosis of rheumatic fever. Rheumatic fever is usually treated using antibiotics to control Streptococcus infection and medications such as aspirin and corticosteroids to decrease inflammation. A long-lasting injection of penicillin is important and effective to prevent further complications and recurrence.

Historical Perspective

Rheumatic fever was first described by Hippocrates, a Greek physician, between 400-370 B.C.[10] The term was first used post-Renaissance by Guillaume de Baillou, a French physician, in the early 1600s. T. Duckett Jones, MD was the first to publish a set of diagnostic criteria in 1944.[11]

Classification

Based on the duration of symptoms, rheumatic fever may be classified into either acute or chronic.[1]

Pathophysiology

Rheumatic fever is the result of an autoimmunological sequela to a virulent Streptococcus pyogenes infection in a patient who was immunologically sensitized from prior infections. During a streptococcal infection, activated antigen presenting cells, such as macrophages, present the bacterial antigen to helper T cells. Helper T cells subsequently activate B cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies also act against the myocardium and joints, producing the symptoms of rheumatic fever.[12]

Causes

Rheumatic fever is usually caused by an infection with Group A beta-hemolytic Streptococcus pyogenes.


Differential Diagnosis

Rheumatic fever must be differentiated from other diseases that cause fever, skin rash, nausea and fatigue, such as typhoid fever, malaria, lassa fever, ebola, and scarlet fever.[6]

Epidemiology and Demographics

The incidence of rheumatic fever among developed countries such as the USA and Canada was approximately 20-40/100,000 individuals during the period 1970-1990 with rheumatic heart disease occurring sporadically. Over past two decades, the prevalence of rheumatic fever has decreased to 5-20/100,000 individuals in Canada and <5/100,000 individuals in the USA with no new cases of rheumatic heart disease.[13] Rheumatic fever is endemic in many developing countries and is usually observed among children between the ages of 5-15.[14]

Risk Factors

Common risk factors in the development of rheumatic fever include low socioeconomic status, inadequate healthcare, and poor sanitation.[6][15]

Screening

Screening of rheumatic fever is important as many cases of rheumatic heart disease are subclinical. Echocardiography among inhabitants of high risk regions is recommended. If any abnormality is detected on echocardiography, further cardiac evaluation is performed followed by antimicrobial therapy.[16]

Natural History, Complications, and Prognosis

Diagnosis

Physical Examination

  • Fever
  • Cardiac murmurs may be noted on cardiac auscultation if heart valves are involved. Regurgitant murmurs are common
  • Sydenham's chorea
  • Migratory polyarthritis may be noted in 70-75% of patients. Often large joints of lower limbs (knee and ankle joints) and upper limbs (elbow and wrist joints) are involved progressing below-upwards.
  • Subcutaneous nodules over extensor surface of limbs, bony prominences such as elbows, knees, ankles and knuckles[8]
  • Erythema marginatum, also known as erythema annulare are pink-red rash frequently located on trunk, limbs and seldom on face[9].

Lab Tests

Patients with rheumatic fever often have elevated inflammatory markers such as ESR and C-reactive protein which help in monitoring the course of the disease. Presence of streptococcal infection can be established by obtaining throat culture or rapid streptococcal antigen test. Elevated or rising antistreptolysin O antibody titer is often noted. Endomyocardial biopsy demonstrate the presence of Aschoff bodies. However, biopsy is not recommended routinely[17].

Electrocardiogram

Some of the electrocardiographic changes that may be noted in rheumatic heart disease include PR prolongation, conduction abnormalities, arryhthmias or P mitrale depending on the structures involved and the extent of cardiac damage.

Chest X Ray

Cardiomegaly or pulmonary edema secondary to heart failure may be noted on chest x-ray among patients with rheumatic heart disease.

Echocardiography

Echocardiography may be helpful in establishing carditis and in monitoring the progress of valve defect.

Treatment

Primary Prevention

Treatment of streptococcal pharyngitis with appropriate antibiotics (penicillin or cephalosporin) most often prevents development of rheumatic fever.

Secondary Prevention

In order to prevent recurrent development of rheumatic fever, an antibiotic prophylaxis should be initiated immediately after the antibiotic course in treatment of rheumatic fever. Duration of prophylactic treatment varies with degree of cardiac damage secondary to rheumatic fever.

References

  1. 1.0 1.1 Nasonova VA, Kuz'mina NN, Belov BS (2004). "[Present-day classification and nomenclature of rheumatic fever]". Klin Med (Mosk). 82 (8): 61–6. PMID 15468729.
  2. Coburn AF, Pauli RH (1932). "Studies on the relationship of streptococcus hemolyticus to the rheumatic process: III. Observations on the immunological responses of rheumatic subjects to hemolytic streptococcus". J Exp Med. 56 (5): 651–76. PMC 2132197. PMID 19870091.
  3. Martin JM, Barbadora KA (2006). "Continued high caseload of rheumatic fever in western Pennsylvania: Possible rheumatogenic emm types of streptococcus pyogenes". J Pediatr. 149 (1): 58–63. doi:10.1016/j.jpeds.2006.03.006. PMID 16860129.
  4. The Natural History of Rheumatic Fever and Rheumatic Heart Disease, Rheumatic Fever Working Party of the Medical Research Council of Great Britain and the American Heart Association (1965). http://circ.ahajournals.org/content/32/3/457 Accessed on October 12, 2015
  5. Majeed HA, Yousof AM, Khuffash FA, Yusuf AR, Farwana S, Khan N (1986). "The natural history of acute rheumatic fever in Kuwait: a prospective six year follow-up report". J Chronic Dis. 39 (5): 361–9. PMID 3700577.
  6. 6.0 6.1 6.2 Rheumatic Fever and Rheumatic Heart Disease. World Health Organization (2004). http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf Accessed on October 12, 2015.
  7. Garg N, Kandpal B, Garg N, Tewari S, Kapoor A, Goel P; et al. (2005). "Characteristics of infective endocarditis in a developing country-clinical profile and outcome in 192 Indian patients, 1992-2001". Int J Cardiol. 98 (2): 253–60. doi:10.1016/j.ijcard.2003.10.043. PMID 15686775.
  8. 8.0 8.1 Ayoub EM (1992). "Resurgence of rheumatic fever in the United States. The changing picture of a preventable illness". Postgrad Med. 92 (3): 133–6, 139–42. PMID 1518750.
  9. 9.0 9.1 BURKE JB (1955). "Erythema marginatum". Arch Dis Child. 30 (152): 359–65. PMC 2011784. PMID 13249623.
  10. Quinn RW (1991). "Did scarlet fever and rheumatic fever exist in Hippocrates' time?". Rev Infect Dis. 13 (6): 1243–4. PMID 1775859.
  11. THE DIAGNOSIS OF RHEUMATIC FEVER. JAMA (2015). http://jama.jamanetwork.com/article.aspx?articleid=271116 Accessed on October 9, 2015
  12. Rheumatic Fever. Wikipedia (2015). http://www.mayoclinic.org/diseases-conditions/rheumatic-fever/basics/causes/con-20031399 Accessed on October 12, 2015
  13. Seckeler MD, Hoke TR (2011). "The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease". Clin Epidemiol. 3: 67–84. doi:10.2147/CLEP.S12977. PMC 3046187. PMID 21386976.
  14. Grover A, Dhawan A, Iyengar SD, Anand IS, Wahi PL, Ganguly NK (1993). "Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India". Bull World Health Organ. 71 (1): 59–66. PMC 2393425. PMID 8440039.
  15. Vlajinac H, Adanja B, Marinković J, Jarebinski M (1991). "Influence of socio-economic and other factors on rheumatic fever occurrence". Eur J Epidemiol. 7 (6): 702–4. PMID 1783067.
  16. Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Sidi D; et al. (2008). "Echocardiographic screening for rheumatic heart disease". Bull World Health Organ. 86 (2): 84. PMC 2647380. PMID 18297157.
  17. Narula J, Chopra P, Talwar KK, Reddy KS, Vasan RS, Tandon R; et al. (1993). "Does endomyocardial biopsy aid in the diagnosis of active rheumatic carditis?". Circulation. 88 (5 Pt 1): 2198–205. PMID 8222115.

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