What is rheumatic fever?

From the Guidelines

Rheumatic fever is an inflammatory disease that can develop as a complication of inadequately treated strep throat or scarlet fever caused by group A streptococcus bacteria, primarily affecting children between 5 and 15 years old and potentially damaging the heart, joints, skin, and brain. The condition occurs when the body's immune system overreacts to the streptococcal infection, causing inflammation in various tissues 1. Symptoms typically appear 2-3 weeks after a strep infection and may include fever, painful and swollen joints, chest pain, heart murmur, fatigue, and a distinctive rash called erythema marginatum.

Key Aspects of Rheumatic Fever

• It is an autoimmune disease that follows a group A β‑haemolytic streptococcal (GAS) infection of the throat (streptococcal pharyngitis) 1.
• After recovery from the initial episode of RF, 60–65% of patients develop valvular heart disease.
• RF recurrences can lead to progressive valve damage (rheumatic heart disease; RHD), which in turn can cause atrial fibrillation and heart failure.
• The global burden of RHD is significant, with an estimated 15.6–19.6 million existing cases and an approximate global incidence of 282,000 new cases per year 1.

Diagnosis and Treatment

• Diagnosis of GAS pharyngitis is best accomplished by combining clinical judgment with diagnostic test results, the criterion standard of which is the throat culture 1.
• Penicillin (either oral penicillin V or injectable benzathine penicillin) is the treatment of choice for GAS pharyngitis because it is cost-effective, has a narrow spectrum of activity, and has long-standing proven efficacy 1.
• Long-term preventive antibiotic therapy (usually monthly penicillin injections or daily oral penicillin) is often necessary to prevent recurrence, especially for those who develop heart complications 1.
• The recommended duration of prophylaxis depends on the number of previous attacks, the time elapsed since the last attack, the risk of exposure to GAS infections, the age of the patient, and the presence or absence of cardiac involvement 1.

Prevention of Recurrence

• Prevention of recurrent episodes of GAS pharyngitis is the most effective method to prevent the development of severe rheumatic heart disease 1.
• Continuous prophylaxis is recommended for patients with well-documented histories of rheumatic fever and those with definite evidence of rheumatic heart disease 1.
• The duration of rheumatic fever prophylaxis should be at least 10 years or until the patient is 40 years of age (whichever is longer) for patients with documented valvular heart disease 1.

From the Research

Definition and Overview of Rheumatic Fever

• Rheumatic fever is a non-suppurative, inflammatory sequela of group A Streptococcus pharyngitis that can occur at two to four weeks after infection 2.
• It is an immunologically mediated inflammatory disease that occurs as a delayed sequel to group A streptococcal throat infection, in genetically susceptible individuals 3.

Causes and Risk Factors

• Rheumatic fever is caused by a group A Streptococcus (GAS) infection, which can lead to rheumatic heart disease (RHD) later in life 2.
• The risk of developing rheumatic fever is higher in individuals who have had a previous episode of rheumatic fever or RHD 2.

Symptoms and Diagnosis

• The symptoms of rheumatic fever can include fever, joint pain, and inflammation of the heart 4.
• The diagnosis of rheumatic fever is based on the modified Jones criteria (mJC) 2.

Treatment and Prevention

• The goals of acute rheumatic fever therapy are to relieve symptoms, mitigate cardiac valve damage, and eradicate streptococcal infection 4.
• Long-term secondary antibiotic prophylaxis is recommended to prevent future recurrences of rheumatic fever and progression to RHD 4, 2.
• Antibiotics such as benzathine benzylpenicillin G and erythromycin are commonly used for prophylaxis 4, 2.
• Antibiotics have been shown to be effective in reducing the incidence of acute rheumatic fever following an episode of suspected GAS pharyngitis 5.

Complications and Prognosis

• Rheumatic fever can lead to rheumatic heart disease (RHD), which is a significant cause of cardiovascular disease in young people 2.
• RHD can lead to cardiac complications, including heart failure and arrhythmias 4.
• The prognosis for individuals with rheumatic fever is generally good if treated promptly and effectively, but the risk of recurrence and progression to RHD remains a concern 2.