What is the natural history of Rheumatic Fever (RF)?

From the Guidelines

Rheumatic fever is a serious inflammatory disease that can lead to permanent heart valve damage and rheumatic heart disease, with a significant risk of morbidity and mortality if left untreated or inadequately treated, as evident from the high global burden of 15.6-19.6 million existing cases and 282,000 new cases per year 1. The natural history of rheumatic fever begins with a group A β-haemolytic streptococcal (GAS) infection of the throat, followed by a latent period of 2-3 weeks, and then the autoimmune response begins, causing various manifestations including carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules.

• The acute phase of rheumatic fever typically lasts 6-12 weeks, and without treatment, the acute symptoms gradually subside, but permanent heart valve damage may occur, particularly to the mitral and aortic valves.
• This valvular damage can lead to rheumatic heart disease, which may remain clinically silent for years or decades before manifesting as heart failure, atrial fibrillation, or other cardiac complications, with an estimated 233,000-468,164 individuals dying from RHD each year 1.
• Recurrent streptococcal infections can trigger additional episodes of rheumatic fever, causing cumulative cardiac damage, highlighting the importance of prevention through prompt antibiotic treatment of streptococcal infections and prophylactic antibiotics for those with a history of rheumatic fever, as recommended by the American Heart Association 1.
• The duration of secondary rheumatic fever prophylaxis varies depending on the category of rheumatic fever, with patients with carditis and residual heart disease requiring 10 years or until 40 years of age of prophylaxis, and those without carditis requiring 5 years or until 21 years of age of prophylaxis 1.
• Recent studies have highlighted the ongoing impact of rheumatic fever and rheumatic heart disease, with an estimated 30 million people affected globally, and 305,000 deaths and 11.5 million disability-adjusted life years lost in 2015, emphasizing the need for continued efforts to prevent and control these diseases 1.

From the Research

Natural History of Rheumatic Fever (RF)

The natural history of Rheumatic Fever (RF) is characterized by:

• High risk of recurrent attacks and development of rheumatic heart disease following a streptococcal throat infection 2, 3
• Risk of recurrence and progression to rheumatic heart disease can be reduced with antibiotic prophylaxis, particularly with intramuscular penicillin 2, 3, 4
• Intramuscular penicillin is more effective than oral penicillin in preventing RF recurrence and streptococcal throat infections 2, 4

Recurrence and Progression

• Recurrence of RF can occur at 2 to 4 weeks after a streptococcal infection 3
• Progression to rheumatic heart disease can occur later in life, carrying significant risk of morbidity and mortality 3
• Antibiotic prophylaxis can reduce the risk of recurrence and progression, but the evidence is based on poor-quality trials and outdated formulations of oral penicillin 2, 3, 4

Prevention and Treatment

• Secondary prophylaxis with antibiotics is recommended for people after their initial episode of RF to prevent recurrent group A streptococcal infections, recurrences of RF, and progression to rheumatic heart disease 5
• Benzathine penicillin G is the first-line antibiotic for secondary prophylaxis, delivered intramuscularly every 2 to 4 weeks 5
• Two-weekly or 3-weekly injections of intramuscular penicillin appear to be more effective than 4-weekly injections in preventing RF recurrence and streptococcal throat infections 2, 4, 6