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CHAPTER SUMMARY AND CENTRAL ILLUSTRATION

Chapter Summary

This chapter discusses the pathogenesis, clinical presentation, diagnosis, and management of acute rheumatic fever (ARF) and rheumatic heart disease (RHD). Group A Streptococcal disease can infrequently result in ARF when it goes untreated or unrecognized. Postinfective immunologic phenomena may cause multisystem involvement. All three layers of the heart may be affected as fibrinous pericarditis, interstitial myocarditis, and verrucous endocarditis or valvulitis. The endocardial affliction could evolve into severe, even life-threatening heart failure in the acute stages and may progress to permanent heart valve damage. Over 40 million people, largely living in low- and middle-income countries, live with and are affected by this disease. The 2015 revised Jones criteria have helped improve the diagnostic accuracy of ARF in moderate- to high-risk populations, while increased awareness and global advocacy will improve case detection, foster research, and determine the global burden of both ARF and RHD. The pathogenesis of ARF development remains poorly understood, and disease awareness and prevention remain the cornerstones for the eradication of this disease, which primarily afflicts the productive years of life.

eFig 27-01 Chapter 27: Acute Rheumatic Fever

INTRODUCTION

Acute rheumatic fever (ARF) is a multisystem autoimmune response to untreated or partially treated group A Streptococcus (GAS) pharyngitis. A single severe episode of ARF or recurrent episodes of ARF can result in permanent heart valve damage known as rheumatic heart disease (RHD). Despite a marked decline in ARF and RHD in high-income regions of the world, ARF and RHD persist as major public health problems in low- and middle-income regions of the world, indicative of inadequate access to health care, poorly functioning health systems, and continued social inequality.

EPIDEMIOLOGY OF ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE

The incidence of ARF began to decline in developed countries toward the end of the 19th century, and by the second half of the 20th century, ARF had become rare in most affluent populations. This decline is attributed to more hygienic and less crowded living conditions, better nutrition, improved access to medical care, and, to a lesser extent, the advent of antibiotics in the 1950s. The decline in prevalence of RHD in wealthy countries has followed a similar pattern, albeit with a delay compared to ARF incidence, which is explained by the chronic nature of RHD. However, these diseases continue largely unabated in resource-poor countries and in some populations living in relative poverty in industrialized countries.1

It was previously estimated that approximately 470,000 individuals acquire ARF each year, of whom 97% are in developing countries, where the incidence of ARF exceeds 50 per 100,000 children per year.2 Estimates from the 2015 Global Burden of Disease study put the RHD burden at 33 million prevalent cases, causing 275,000 deaths and more than 9 ...

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