Acute Rheumatic Fever: Where Do We Stand? An Epidemiological Study in Northern Italy.

Achille Marino,Anna Cogliardi,Patrizia Calzi,Andrea Biondi,Massimo Andreotti,Rolando Cimaz,Marco Sala,Francesco Morandi,Roberto Bellù,Tiziana Varisco,Giulia Tattesi,Maria Antonietta Pelagatti,Laura Menni,Claudia Addis,Francesca Cortinovis

doi:10.3389/fmed.2021.621668

Abstract

Acute rheumatic fever (ARF) is a non-septic complication of group A β-hemolytic streptococcal (GAS) throat infection. Since 1944, ARF diagnosis relies on the Jones criteria, which were periodically revised. The 2015 revision of Jones criteria underlines the importance of knowing the epidemiological status of its own region with updated data. This study aims to describe ARF features in a retrospective cohort retrieved over a 10-year timespan (2009–2018) and to report the annual incidence of ARF among children in the Province of Monza-Brianza, Lombardy, Italy during the same period. This is a multicentric cross-sectional/retrospective study; 70 patients (39 boys) were diagnosed with ARF. The median age at diagnosis was 8.5 years (range, 4–14.2 years). Overall, carditis represented the most reported major Jones criteria followed by arthritis and chorea (40, 27, and 20 cases, respectively). In order to calculate the annual incidence of ARF, only children resident in the Province of Monza-Brianza were included in this part of the analysis. Therefore, 47 patients aged between 5 and 14 years were identified. The median incidence during the study time was 5.7/100,000 (range, 2.8–8.3/100,000). In the Province of Monza-Brianza, we found an incidence rate of ARF among children aged 5–14 years constantly above the threshold of low-risk area as defined in the 2015 revision of Jones criteria. Therefore, the diagnosis of ARF should be based on the moderate–high-risk set of Jones criteria. However, given the burden of secondary prophylaxis, expert opinion is advisable when the diagnosis of ARF is uncertain.

Highlights

Acute rheumatic fever (ARF) is a non-septic complication of group A β-hemolytic streptococcal (GAS) throat infection
Carditis represented the most reported major Jones criteria followed by arthritis and chorea (40, 27, and 20 cases, respectively)
The improvements in living conditions and hygiene along with the increased accessibility to the healthcare system were the main reasons for the decline of ARF incidence worldwide during the nineteenth century, together with the increased use of antibiotics, especially in industrialized countries

Summary

Introduction

Acute rheumatic fever (ARF) is a non-septic complication of group A β-hemolytic streptococcal (GAS) throat infection. ARF is a multisystem autoimmune disease causing inflammation in different sites (joint, heart, basal ganglia, skin); it affects typically school-aged children ( between 5 and 14 years) without sex differences [1]. Several factors influence the likelihood of ARF developing such as genetic predisposition and the virulence of the infecting GAS strain. The immunological pathogenesis is supported by the typical latency of about 3 weeks between GAS pharyngitis and ARF onset [3, 4]. The genetic predisposition for ARF might be acquired (44% of concordance among monozygotic twins), this inheritance is polygenic with variable penetrance [5, 6]. Heart involvement may result in permanent damage of valves resulting in rheumatic heart disease (RHD) and represents the major cause of acquired heart disease in the developing world [8, 9].

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