From the Guidelines
Rheumatic fever is an inflammatory disease characterized by major manifestations including carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules, as outlined in the revised Jones criteria 1. The diagnosis of acute rheumatic fever (ARF) is based on the revised Jones criteria, which considers major and minor manifestations of the disease along with evidence of a preceding streptococcal infection. The major manifestations of ARF include:
- Carditis
- Polyarthritis
- Chorea
- Erythema marginatum
- Subcutaneous nodules These manifestations can vary in frequency and presentation, with carditis being the most common, followed by polyarthritis, and then chorea, which has a female predominance 1. The revised Jones criteria also take into account the role of noninvasive cardiac imaging, such as echocardiography combined with Doppler flow assessment, in the diagnosis of carditis in ARF 1. It is essential to maintain continuing vigilance in the application of the clinical manifestations for the diagnosis of ARF, as there is always the potential for "diagnosis overlap" in the application of the Jones criteria 1. In specific high-risk populations, such as the indigenous Australian population, variable manifestations of ARF have been described, including presentations with aseptic monoarthritis, polyarthralgia, and low-grade fevers 1. However, the applicability of these variable clinical manifestations in low-risk populations has not been tested and is not recommended 1.
From the Research
Definition of Rheumatic (Acute Rheumatic) Fever
Rheumatic fever, also known as acute rheumatic fever (ARF), is defined as:
- An inflammatory sequela of Group A Streptococcal pharyngitis that affects multiple organ systems 2
- A multisystem inflammatory disease that occurs as a delayed sequelae to group A streptococcal pharyngitis 3
- A non-suppurative complication of pharyngeal infection with group A streptococcus 4
- An autoimmune disease associated with group A β-hemolytic streptococcal infection, characterized by the development of carditis, arthritis, chorea, subcutaneous nodules, and erythema marginatum 5
Key Characteristics
- ARF is a clinical diagnosis, subject to the judgment of the clinician 2
- The disease is characterized by a variable presentation, with major manifestations including carditis, polyarthritis, and Sydenham's chorea 2
- The Jones criteria, first developed in 1944 and revised several times, are used to aid in the diagnosis of ARF 2, 5, 6
- Diagnosis is based on the presence of documented preceding Group A Streptococcal infection, in addition to the presence of two major manifestations or one major and two minor manifestations of the Jones criteria 2