How accurate is the diagnosis of rheumatic fever in Egypt? Data from the national rheumatic heart disease prevention and control program (2006-2018).

Alaa Ghamrawy,Ekram W Abd El-Wahab,Nermeen N Ibrahim,Joseph M Vinetz

doi:10.1371/journal.pntd.0008558

Abstract

Rheumatic heart disease (RHD) as a chronic sequela of repeated episodes of acute rheumatic fever (ARF), remains a cause of cardiac morbidity in Egypt although it is given full attention through a national RHD prevention and control program. The present report reviews our experience with subjects presenting with ARF or its sequelae in a single RHD centre and describes the disease pattern over the last decade. A cross-sectional study was conducted in El-Mahalla RHD centre between 2006 and 2018. A total of 17014 individual were enrolled and evaluated. Diagnosis ARF was based on the 2015 revised Jones criteria and RHD was ruled in by echocardiography. The majority of the screened subjects were female (63.2%), in the age group 5–15 years (64.6%), rural residents (61.2%), had primary education (43.0%), and of low socioeconomic standard (50.2%). The total percentage of cases presenting with ARF sequelae was 29.3% [carditis/RHD (10.8%), rheumatic arthritis (Rh.A) (14.9%), and Sydenham’s chorea (0.05%)]. Noticeably, 72% were free of any cardiac insult, of which 37.7% were victims of misdiagnoses made elsewhere by untrained practitioners who prescribed for them long term injectable long-acting penicillin [Benzathine Penicillin G (BPG)] without need. About 54% of the study cohort reported the occurrence of recurrent attacks of tonsillitis of which 65.2% underwent tonsillectomy. Among those who experienced tonsillectomy and/or received BPG in the past, 14.5% and 22.3% respectively had eventually developed RHD. Screening of family members of some RHD cases who needed cardiac surgery revealed 20.7% with undiagnosed ARF sequalae [RHD (56.0%) and Rh.A (52.2%)]. Upon the follow-up of RHD cases, 1.2% had improved, 98.4% were stable and 0.4% had their heart condition deteriorated. Misdiagnosis of ARF or its sequelae and poor compliance with BPG use may affect efforts being exerted to curtail the disease. Updating national guidelines, capacity building, and reliance on appropriate investigations should be emphasized. Since the genetic basis of RHD is literally confirmed, a family history of RHD warrants screening of all family members for early detection of the disease.

Highlights

Acute rheumatic fever (ARF) is a multisystemic sequela of a post infection autoimmune reaction due to untreated group A β haemolytic Streptococcus (GABHS) pharyngitis and possibly pyoderma [1,2]
This represented 46.1% of all cardiac operative procedures performed in El-Mahalla cardiac centre alone, and placed a total cost of approximately 358 million Egyptian pounds (EGP) (Fig 2)
The number of cases diagnosed with Rh.A was consistently higher compared to the number of confirmed Rheumatic heart disease (RHD) cases, the latter showed an unusual peak in 2018 (S1 Fig)

Summary

Introduction

Acute rheumatic fever (ARF) is a multisystemic sequela of a post infection autoimmune reaction due to untreated group A β haemolytic Streptococcus (GABHS) pharyngitis and possibly pyoderma [1,2]. Recurrent episodes of ARF and its associated cardiac inflammation lead to permanent valvular damage and rheumatic heart disease (RHD) [2]. Except for a lingering burden in elderly people, ARF and RHD are virtually disappearing in high-income countries due to improved living conditions and medical care. These conditions are not receiving enough attention and are inadequately controlled in most of the low resource countries [3,7]. 30 million people are currently affected by RHD, with 233,000 directly attributable deaths This represents 25–40% of all cardiovascular disease and accounts for 25–50% of all cardiac admissions [9]

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Discussion

Conclusion