Guideline sources The following summarized guidelines for the evaluation and management of rheumatic fever (ARF) are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2021), the American Heart Association (AHA 2015), the Infectious Diseases Society of America (IDSA 2012), the European Society for Microbiology and Infectious Diseases (ESCMID 2012), and the American Heart Association (AHA/AAP 2009). 1 2 3 4 5 6 6 7 8 9 9 Definition ARF is a multiorgan autoimmune disease caused by group A -hemolytic streptococcal infection in people with genetic predisposition. 6 Epidemiology ARF is predominantly caused by Streptococcus pyogenes or Group A -hemolytic Streptococcus. 7 Pathophysiology The incidence of ARF varies from <0.5/100,000 person-years to >100/100,000 person-years in highly developed and poor countries, respectively. 6 Disease course https://web.pathway.md/diseases/recGsmfUVYozAyVRR 1/5 6/24/23, 2:57 PM Rheumatic fever Pathway Group A streptococcus infection in susceptible individuals produces antigens that activate specific B and T cells through molecular mimicry triggering autoimmune reactions against host tissues (heart, brain, joints, skin) resulting in rheumatic carditis, rheumatic heart disease and its complications (AF, endocarditis, embolic stroke, HF), chorea arthritis, and erythema marginatum and subcutaneous nodules. 8 Prognosis and risk of recurrence The cumulative incidence of progression to rheumatic heart disease at 1-year, 5-years, and 10- years is 27.1%, 44.0%, and 51.9%, respectively. The cumulative incidence of ARF recurrence at 10 years is 19.8%. 9 9 Calculator Revised Jones criteria for acute Guidelines 1. Screening and diagnosis Diagnostic criteria: consider making a presumptive diagnosis of acute ARF based on 2 major or 1 major and 2 minor or 3 minor manifestations in patients with a reliable past history of acute ARF or established rheumatic heart disease and in the face of documented group A streptococcal infection. C Show 3 more 2. Classification and risk stratification Risk stratification: As per AHA 2015 guidelines, consider stratifying persons being at low risk for acute ARF if they come from a setting or population known to experience low rates of acute ARF or rheumatic heart disease. C Show 2 more As per AAP 2009 guidelines, recognize that adult patients with acute pharyngitis have a much lower incidence of group A Streptococcus infections, and the risk of an initial attack of acute ARF is extremely low even in patients with an undiagnosed and untreated episode of group A Streptococcus pharyngitis. B 3. Diagnostic investigations https://web.pathway.md/diseases/recGsmfUVYozAyVRR 2/5 6/24/23, 2:57 PM Rheumatic fever Pathway Laboratory tests: View any of the following as evidence of preceding infection: increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNAse B) positive throat culture for group A -hemolytic streptococci positive rapid group A streptococcal carbohydrate antigen test in a pediatric patient with clinical presentation suggesting a high pretest probability of streptococcal pharyngitis. B Echocardiography: obtain echocardiography with Doppler in all patients with suspected or confirmed acute ARF. B Show 3 more 4. Specific circumstances Patients with post-streptococcal reactive arthritis: recognize that no more than half of patients with post-streptococcal reactive arthritis patients who have a throat culture performed have group A Streptococcus infection isolated, even though all patients have serological evidence of recent group A Streptococcus infection. B Show 2 more 5. Preventative measures Primary prevention, testing: As per IDSA 2012 guidelines, obtain a rapid antigen detection test for GAS pharyngitis in most patients with symptoms of acute pharyngitis because the clinical features alone do not reliably discriminate between group A streptococcal and viral pharyngitis, except when overt viral features such as rhinorrhea, cough, oral ulcers, and/or hoarseness are present. A Show 9 more As per AAP/AHA 2009 guidelines, obtain post-treatment throat cultures 2-7 days after completion of therapy only in patients remaining symptomatic, experiencing recurrence of symptoms, or with a history of ARF and therefore being at unusually high risk for recurrence. B Primary prevention, antibiotic therapy: As per ESCMID 2012 guidelines, do not initiate antibiotics to prevent the development of ARF in low-risk patients (patients with no previous history of ARF) with an acute sore throat. D Show 2 more As per AAP/AHA 2009 guidelines, administer either intramuscularly benzathine penicillin G or oral penicillin V (250 mg BID) as antimicrobial agents of choice for the treatment of group A Streptococcus, except in patients with histories of penicillin allergy. B Show 3 more Primary prevention (chronic GAS carriers): Recognize that recurrent episodes of pharyngitis in patients with laboratory evidence of group A streptococcal infection may represent either: multiple episodes of bona fide recurrent streptococcal pharyngitis at close intervals, or https://web.pathway.md/diseases/recGsmfUVYozAyVRR 3/5 6/24/23, 2:57 PM Rheumatic fever Pathway multiple episodes of viral pharyngitis in chronic pharyngeal group A streptococcus carriers. B Do not attempt to identify nor administer antimicrobial therapy in patients with asymptomatic group A streptococcus carriage, as they are unlikely to spread GAS pharyngitis to their close contacts and are at little or no risk for developing suppurative or non-suppurative complications, such as acute ARF. D Secondary prevention: As per ACC 2021 guidelines, initiate secondary prevention of ARF in patients with rheumatic heart disease. B As per AHA 2015 guidelines: Consider offering 12 months of secondary prophylaxis followed by re-evaluation to include a careful history and physical examination in addition to a repeat echocardiogram in patients with genuine uncertainty. C Consider concluding that the recurrent symptoms are not likely related to acute ARF and discontinuing antibiotic prophylaxis in patients with recurrent symptoms (particularly involving the joints) being adherent to prophylaxis recommendations but lacking serological evidence of group A streptococcal infection and lacking echocardiographic evidence of valvulitis. C As per AAP 2009 guidelines, initiate continuous prophylaxis in patients with well-documented histories of ARF (including cases manifested solely by Sydenham chorea) and in patients with definite evidence of rheumatic heart disease. A Show 8 more References 1. Michael A Gerber, Robert S Baltimore, Charles B Eaton 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. Open 2. Michael H Gewitz, Robert S Baltimore, Lloyd Y Tani 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 May 19;131 20 1806 18. Open 3. Shulman ST, Bisno AL, Clegg HW et al. Clinical practice guideline for the diagnosis and management of group A streptococcal pharyngitis: 2012 update by the Infectious Diseases Society of America. Clin Infect Dis. 2012 Nov 15;55 10 1279 82. Open 4. ESCMID Sore Throat Guideline Group, C Pelucchi, L Grigoryan et al. Guideline for the management of acute sore throat. Clin Microbiol Infect. 2012 Apr;18 Suppl 1 1 28. Open 5. Catherine M Otto, Rick A Nishimura, Robert O Bonow et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Thorac Cardiovasc Surg. 2021 Aug;162 2):e183-e353. Open https://web.pathway.md/diseases/recGsmfUVYozAyVRR 4/5 6/24/23, 2:57 PM Rheumatic fever Pathway 6. Szczygielska I, Hernik E, Kolodziejczyk B et al. Rheumatic fever - new diagnostic criteria. Reumatologia. 2018;56 1 37 41. Open 7. Seckeler MD, Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease. Clin Epidemiol. 2011 Feb 22;3 67 84. Open 8. Carapetis JR, Beaton A, Cunningham MW et al. Acute rheumatic fever and rheumatic heart disease. Nat Rev Dis Primers. 2016 Jan 14;2 15084. Open 9. He VY, Condon JR, Ralph AP et al. Long-Term Outcomes From Acute Rheumatic Fever and Rheumatic Heart Disease: A Data-Linkage and Survival Analysis Approach. Circulation. 2016 Jul 19;134 3 222 32. Open 10. No authors listed. 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 Oct 21;268 15 2069 73. Open https://web.pathway.md/diseases/recGsmfUVYozAyVRR 5/5