Highlights & Basics
- Acute rheumatic fever (ARF) continues to cause a large burden of mortality and morbidity in low- and middle-income countries. It is less common in high-income countries, but continues to be seen in indigenous communities and during outbreaks.
- Currently, no single test can diagnose ARF. Diagnosis is clinical and relies on the Jones criteria.
- The 5 major manifestations of ARF are carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, of which the most common are carditis and arthritis.
- The Jones criteria were revised in 2015 to include separate criteria for low-risk and moderate- to high-risk populations and introduce the use of Doppler echocardiography to diagnose subclinical carditis.
- While all other manifestations of ARF resolve without sequelae, carditis can lead to chronic rheumatic heart disease (RHD).
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Global prevalence and mortality rates. Source: data derived from Global Burden of Disease data 2010/2013.
ECG showing heart block in acute rheumatic fever
Chest x-ray of biventricular heart failure due to acute carditis in rheumatic fever
Normal values for antistreptolysin O (ASO) and anti-DNase B titers
Typical appearance of erythema marginatum on the back of a child with acute rheumatic fever
Citations
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Carapetis JR, Beaton A, Cunningham MW, et al. Acute rheumatic fever and rheumatic heart disease. Nat Rev Dis Primers. 2016 Jan 14;2:15084.[Abstract][Full Text]
Denny F, Wannamaker LW, Brink WR, et al. Prevention of rheumatic fever; treatment of preceding streptococci infection. J Am Med Assoc. 1950 May 13;143(2):151-3.[Abstract]
Gerber MA, Baltimore RS, Eaton CB, 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.[Abstract][Full Text]
RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3.2 edition, March 2022). 2022 [internet publication].[Full Text]
Heart Foundation of New Zealand. New Zealand guidelines for rheumatic fever: diagnosis, management and secondary prevention of acute rheumatic fever and rheumatic heart disease: 2014 update. 2014 [internet publication].[Full Text]
Manyemba J, Mayosi BM. Penicillin for secondary prevention of rheumatic fever. Cochrane Database Syst Rev. 2002;(3):CD002227.[Abstract][Full Text]
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