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Acute rheumatic fever: Clinical manifestations and diagnosis

Authors
Andrew Steer, MBBS, PhD, FRACP
Allan Gibofsky, MD, JD, FACP, FCLM
Section Editors
Robert Sundel, MD
Daniel J Sexton, MD
Deputy Editor
Elizabeth TePas, MD, MS

INTRODUCTION

Acute rheumatic fever (ARF) is a nonsuppurative sequela that occurs two to four weeks following group A Streptococcus (GAS) pharyngitis and may consist of arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules. Damage to cardiac valves may be chronic and progressive, resulting in cardiac decompensation.

The clinical manifestations and diagnosis of ARF are reviewed here. The epidemiology, pathogenesis, treatment, and prevention of this disorder are presented separately. (See "Acute rheumatic fever: Epidemiology and pathogenesis" and "Acute rheumatic fever: Treatment and prevention".)

CLINICAL MANIFESTATIONS

Acute illness — ARF can present with several different clinical findings within weeks of a group A streptococcal (GAS) tonsillopharyngitis (or streptococcal pyoderma in patients from tropical regions) [1-3]. The possible major and minor manifestations are reviewed here. These manifestations are used for diagnosis (Revised Jones Criteria) [4,5]. The diagnostic criteria are reviewed below. (See 'Diagnosis' below.)

The five major manifestations (and percent of patients with each) are [4]:

Carditis and valvulitis (eg, pancarditis) that is clinical or subclinical – 50 to 70 percent

                        

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Literature review current through: Jun 2017. | This topic last updated: Jan 06, 2017.
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References
Top
  1. Parks T, Smeesters PR, Steer AC. Streptococcal skin infection and rheumatic heart disease. Curr Opin Infect Dis 2012; 25:145.
  2. Noonan S, Zurynski YA, Currie BJ, et al. A national prospective surveillance study of acute rheumatic fever in Australian children. Pediatr Infect Dis J 2013; 32:e26.
  3. Whitehall J, Kuzulugil D, Sheldrick K, Wood A. Burden of paediatric pyoderma and scabies in North West Queensland. J Paediatr Child Health 2013; 49:141.
  4. Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation 2015; 131:1806.
  5. Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography: A Scientific Statement From the American Heart Association. See Table 7 for the revised Jones criteria. http://circ.ahajournals.org/content/early/2015/04/23/CIR.0000000000000205.
  6. Wallace MR, Garst PD, Papadimos TJ, Oldfield EC 3rd. The return of acute rheumatic fever in young adults. JAMA 1989; 262:2557.
  7. FEINSTEIN AR, SPAGNUOLO M. The clinical patterns of acute rheumatic fever: a reapraisal. Medicine (Baltimore) 1962; 41:279.
  8. Carapetis JR, Currie BJ. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Arch Dis Child 2001; 85:223.
  9. SACKS L, FEINSTEIN AR, TARANTA A. A controlled psychologic study of Sydenham's chorea. J Pediatr 1962; 61:714.
  10. Eshel G, Lahat E, Azizi E, et al. Chorea as a manifestation of rheumatic fever--a 30-year survey (1960-1990). Eur J Pediatr 1993; 152:645.
  11. BURKE JB. Erythema marginatum. Arch Dis Child 1955; 30:359.
  12. Perry CB. Erythema marginatum (rheumaticum). Arch Dis Child 1937; 12:233.
  13. BALDWIN JS, KERR JM, KUTTNER AG, DOYLE EF. Observations on rheumatic nodules over a 30-year period. J Pediatr 1960; 56:465.
  14. Harris TN. The erythrocyte sedimentation rate in rheumatic fever: Its significance in adolescent and overweight children. Am J Med Sci 1945; 210:173.
  15. Balli S, Oflaz MB, Kibar AE, Ece I. Rhythm and conduction analysis of patients with acute rheumatic fever. Pediatr Cardiol 2013; 34:383.
  16. Karacan M, Işıkay S, Olgun H, Ceviz N. Asymptomatic rhythm and conduction abnormalities in children with acute rheumatic fever: 24-hour electrocardiography study. Cardiol Young 2010; 20:620.
  17. Clarke M, Keith JD. Atrioventricular conduction in acute rheumatic fever. Br Heart J 1972; 34:472.
  18. Reeves BM. Complete heart block complicating Acute Rheumatic Fever. J Paediatr Child Health 2011; 47:844.
  19. Marcus RH, Sareli P, Pocock WA, Barlow JB. The spectrum of severe rheumatic mitral valve disease in a developing country. Correlations among clinical presentation, surgical pathologic findings, and hemodynamic sequelae. Ann Intern Med 1994; 120:177.
  20. Horstkotte D, Niehues R, Strauer BE. Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis. Eur Heart J 1991; 12 Suppl B:55.
  21. Albert DA, Harel L, Karrison T. The treatment of rheumatic carditis: a review and meta-analysis. Medicine (Baltimore) 1995; 74:1.
  22. 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 adolescents. Heart 2005; 91:1019.
  23. Chaurasia AS, Nawale JM, Patil SN, et al. Jaccoud's arthropathy. Lancet 2013; 381:2108.
  24. Jones TD. The diagnosis of rheumatic fever. JAMA 1944; 126:481.
  25. Jones criteria (revised) for guidance in the diagnosis of rheumatic fever. Circulation 1965; 32:664.
  26. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. JAMA 1992; 268:2069.
  27. Ferrieri P, Jones Criteria Working Group. Proceedings of the Jones Criteria workshop. Circulation 2002; 106:2521.
  28. Veasy LG, Wiedmeier SE, Orsmond GS, et al. Resurgence of acute rheumatic fever in the intermountain area of the United States. N Engl J Med 1987; 316:421.
  29. 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; 4:240.
  30. McDonald MI, Towers RJ, Andrews RM, et al. Low rates of streptococcal pharyngitis and high rates of pyoderma in Australian aboriginal communities where acute rheumatic fever is hyperendemic. Clin Infect Dis 2006; 43:683.
  31. Steer AC, Smeesters PR, Curtis N. Streptococcal Serology: Secrets for the Specialist. Pediatr Infect Dis J 2015; 34:1250.
  32. RANTZ LA, RANDALL E, RANTZ HH. Antistreptolysin O; a study of this antibody in health and in hemolytic streptococcus respiratory disease in man. Am J Med 1948; 5:3.
  33. Kaplan EL, Rothermel CD, Johnson DR. Antistreptolysin O and anti-deoxyribonuclease B titers: normal values for children ages 2 to 12 in the United States. Pediatrics 1998; 101:86.
  34. Steer AC, Vidmar S, Ritika R, et al. Normal ranges of streptococcal antibody titers are similar whether streptococci are endemic to the setting or not. Clin Vaccine Immunol 2009; 16:172.
  35. Sethi S, Kaushik K, Mohandas K, et al. Anti-streptolysin O titers in normal healthy children of 5-15 years. Indian Pediatr 2003; 40:1068.
  36. Johnson DR, Kurlan R, Leckman J, Kaplan EL. The human immune response to streptococcal extracellular antigens: clinical, diagnostic, and potential pathogenetic implications. Clin Infect Dis 2010; 50:481.
  37. Kaplan EL, Ferrieri P, Wannamaker LW. Comparison of the antibody response to streptococcal cellular and extracellular antigens in acute pharyngitis. J Pediatr 1974; 84:21.
  38. Ayoub EM, Wannamaker LW. Evaluation of the streptococcal desoxyribonuclease B and diphosphopyridine nucleotidase antibody tests in acute rheumatic fever and acute glomerulonephritis. Pediatrics 1962; 29:527.
  39. Blyth CC, Robertson PW. Anti-streptococcal antibodies in the diagnosis of acute and post-streptococcal disease: streptokinase versus streptolysin O and deoxyribonuclease B. Pathology 2006; 38:152.
  40. Godown J, Lu JC, Beaton A, et al. Handheld echocardiography versus auscultation for detection of rheumatic heart disease. Pediatrics 2015; 135:e939.
  41. Watson G, Jallow B, Le Doare K, et al. Acute rheumatic fever and rheumatic heart disease in resource-limited settings. Arch Dis Child 2015; 100:370.
  42. Schaffer FM, Agarwal R, Helm J, et al. Poststreptococcal reactive arthritis and silent carditis: a case report and review of the literature. Pediatrics 1994; 93:837.
  43. Arnold MH, Tyndall A. Poststreptococcal reactive arthritis. Ann Rheum Dis 1989; 48:686.
  44. Aviles RJ, Ramakrishna G, Mohr DN, Michet CJ Jr. Poststreptococcal reactive arthritis in adults: a case series. Mayo Clin Proc 2000; 75:144.
  45. Jansen TL, Janssen M, de Jong AJ, Jeurissen ME. Post-streptococcal reactive arthritis: a clinical and serological description, revealing its distinction from acute rheumatic fever. J Intern Med 1999; 245:261.
  46. Mackie SL, Keat A. Poststreptococcal reactive arthritis: what is it and how do we know? Rheumatology (Oxford) 2004; 43:949.
  47. Moorthy LN, Gaur S, Peterson MG, et al. Poststreptococcal reactive arthritis in children: a retrospective study. Clin Pediatr (Phila) 2009; 48:174.
  48. Barash J, Mashiach E, Navon-Elkan P, et al. Differentiation of post-streptococcal reactive arthritis from acute rheumatic fever. J Pediatr 2008; 153:696.
  49. van Bemmel JM, Delgado V, Holman ER, et al. No increased risk of valvular heart disease in adult poststreptococcal reactive arthritis. Arthritis Rheum 2009; 60:987.
  50. De Cunto CL, Giannini EH, Fink CW, et al. Prognosis of children with poststreptococcal reactive arthritis. Pediatr Infect Dis J 1988; 7:683.