Distinguishing Acute Rheumatic Fever From Post-streptococcal Reactive Arthritis.

We report an initial episode of post-streptococcal reactive arthritis (PRSA) in a 61-year-old male with group A streptococcal (GAS) bacteremia. The disease is commonly reported in young children and young adults. Additionally, this patient exemplifies the nonlinear boundaries of acute rheumatic fever (ARF) and PRSA, bringing into question whether they are truly distinct disease entities. These two conditions oftentimes present in similar fashions, making it difficult for clinicians to determine a specific diagnosis. We highlight the importance of recognizing ARF versus PRSA as an incorrect diagnosis can lead to the development of harmful complications including rheumatic heart disease (RHD).

Outcome following acute and recurrent rheumatic fever.

BACKGROUND: Rheumatic carditis is the leading cause of permanent disability caused by damage of the cardiac valve. This study aimed to determine the outcome and predictors of valve surgery in patients with acute rheumatic fever (ARF) and recurrent rheumatic fever (RRF). METHODS: This was a retrospective study of patients diagnosed with ARF and RRF between 2006 and 2021. The predictors of valve surgery were analysed using multivariable Cox proportional regression. RESULTS: The median age of patients with ARF and RRF (n=92) was 11 years (range 5-18). Seventeen patients (18%) were diagnosed with RRF. The most common presenting symptoms included clinical carditis (87%), heart failure (HF) (63%), fever (49%) and polyarthralgia (24%). Patients with moderate-to-severe rheumatic carditis (88%) were given prednisolone. After treatment, the severity of valvular regurgitation was reduced in 52 patients (59%). Twenty-three patients (25%) underwent valve surgery. The incidence of HF, RRF, severe mitral regurgitation on presentation, left ventricular enlargement and pulmonary hypertension was greater in the surgical group than in the non-surgical group. Recurrent rheumatic fever (hazard ratio 7.9, 95% CI 1.9-33.1), tricuspid regurgitation (TR) gradient ≥ 42 mmHg (HR 6.3, 95%CI 1.1-38.7) and left ventricular end-diastolic dimension (LVEDD) ≥6 cm (HR 8.7, 95% CI 2.1-35.9) were predictors of valve surgery (multivariable Cox proportional regression analysis). CONCLUSION: Clinical carditis was the most common presenting symptom in patients with ARF and RRF. The majority of patients responded positively to prednisolone. These findings highlight the predictors of valve surgery following ARF, including RRF, TR gradient ≥ 42 mmHg and LVEDD ≥ 6 cm.Abbreviations: ARF: acute rheumatic fever; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GAS: group A beta-haemolytic Streptococcus; HF: heart failure; HR: hazard ratio; LVEDD: left ventricular end-diastolic dimension; MR: mitral regurgitation; RHD: rheumatic heart disease; RRF: recurrent rheumatic fever; TR: tricuspid regurgitation.

A Case Report: Acute Rheumatic Fever or Something More?

Acute rheumatic fever (ARF) is an autoimmune response that may occur after a group A Streptococcus (GAS) infection. Subcutaneous nodules are considered a rare manifestation of acute rheumatic fever with an incidence of 0%-10%. We present a case study of a 13-year-old girl who presented to us with subcutaneous nodules and articular involvement described as a non-migratory polyarticular joint pain involving the small joints of the hands, wrist, elbows, knees, and ankles for three months with poor response to the non-steroidal anti-inflammatory drug (NSAID) Ibuprofen. Accompanied with the presence of carditis, the patient fulfilled three major and two minor criteria of the revised Jones criteria 2015. Therefore, a diagnosis of acute rheumatic fever was made. The child was asymptomatic on subsequent visits, and although the subcutaneous nodules subsided, she will continue to receive penicillin every month for five years. We describe the successful diagnosis and management of a patient with ARF.

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

Acute rheumatic fever (ARF) is a non-septic complication of group A ß-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.

Toward the Knowledge of the Epidemiological Impact of Acute Rheumatic Fever in Italy.

Background: To estimate the incidence of Acute Rheumatic Fever (ARF) in Tuscany, a region of Central Italy, evaluating the epidemiological impact of the new diagnostic guidelines, and to analyse our outcomes in the context of the Italian overview. Methods: A multicenter and retrospective study was conducted involving children <18 years old living in Tuscany and diagnosed in the period between 2010 and 2019. Two groups were established based on the new diagnostic criteria: High-Risk (HR) group patients, n = 29 and Low-Risk group patients, n = 96. Results: ARF annual incidence ranged from 0.91 to 7.33 out of 100,000 children in the analyzed period, with peak of incidence registered in 2019. The application of HR criteria led to an increase of ARF diagnosis of 30%. Among the overall cohort joint involvement was the most represented criteria (68%), followed by carditis (58%). High prevalence of subclinical carditis was observed (59%). Conclusions: Tuscany should be considered an HR geographic area and HR criteria should be used for ARF diagnosis in this region.

Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

Rheumatic heart disease: current status of diagnosis and therapy.

Rheumatic heart disease (RHD) is the only preventable cardiovascular disease which causes significant morbidity and mortality particularly in low- and middle-income countries. Early clinical diagnosis is key, the updated Jones criteria increases the likelihood of diagnosis in endemic settings, including the echo diagnosis of sub-clinical carditis, polyarthralgia and monoarthritis as well as amended thresholds of minor criteria. The mainstay of rheumatic heart valve disease (RHVD) is a thorough clinical and echocardiographic investigation while severe disease is managed with medical, interventional and surgical treatment. In this report we detail some of the more recent epidemiological findings and focus on the diagnostic and interventional elements of the specific valve lesions. Finally, we discuss some of the recent efforts to improve medical and surgical management for this disease. As we are already more than a year from the historic 2018 World Heart Organization Resolution against Rheumatic Fever and Rheumatic Heart Disease, we advocate strongly for renewed efforts to prioritize this disease across the endemic regions of the world.

Changes in valvular regurgitation in mid-term follow-up of children with first attack acute rheumatic fever: first evaluation after the updated Jones criteria.

AIM: In present study, we aimed to evaluate the changes in valvular regurgitations in mid-term follow-up of children with first attack acute rheumatic fever diagnosed after updated Jones criteria. MATERIALS AND METHODS: The medical records of the children diagnosed with acute rheumatic fever between June 2015 and November 2018 were evaluated retrospectively. When compared to the findings during diagnosis, the changes in the degree of valvular regurgitation in the last visit were coded as same, regressed, or disappeared. RESULTS: A total of 50 children were diagnosed with the first attack of acute rheumatic fever between the noted dates. Nine patients (18%) could be diagnosed depending on the new criteria. Eight patients did not have carditis, and 35 patients (49 valves) could be followed for a median follow-up period of 11.7 ± 3.3 months. In our study, the valvar lesions continued in 82% of patients with clinical carditis at the end of the first year and the degree of valvular regurgitation decreased in 39% of them. Despite this, in a significantly higher (p = 0.031) ratio of patients with silent carditis (41%), valvar lesions disappeared in the same follow-up period. In 18.4% of the involved valves, regurgitation regressed to physiological level. CONCLUSION: Updated Jones criteria make it possible to diagnose a significant number of patients, and the ratio of complete recovery among patients with silent carditis is significantly higher. Also, it can be speculated that the normal children in whom a physiological mitral regurgitation is detected should be followed in terms of rheumatic heart disease.

Rheumatic Fever and Rheumatic Heart Disease in Children.

Acute rheumatic fever (ARF) and its sequelae, Rheumatic heart disease (RHD), contribute significantly to the cardiovascular morbidity and mortality in developing countries. Generally considered a disease of poverty and poor socio-economic conditions, RHD affects the population at the most productive phase of their life. The diagnostic criteria for ARF have been constantly updated to improve the sensitivity. The diagnosis of ARF was entirely clinical however, recently echocardiographic evidence has been added as a major criterion. The disease seems to be on the decline in India, but recent studies using echocardiography have shown high prevalence of RHD among school children. The focus of management has been on prompt recognition and treatment of streptococcal pharyngitis and preventing recurrences of ARF with long-term antibiotic prophylaxis. However, emphasis should be placed on the appropriate management of patients with established RHD, in order to limit the RHD related mortality.

Traditional Jones Criteria: Limitation in the Diagnosis of Rheumatic Fever in Patients with Mitral Valve Repair

@#The present study aims to determine the limitations of traditional Jones criteria during the first episode of acute rheumatic fever (ARF) at the initial referral hospital, in a cohort of patients below 18 years old who had undergone mitral valve repair in National Heart Institute (IJN) from 2011 to 2016. Carditis followed by fever and joint involvement were the most frequent manifestations at first diagnosis. Of the 50 patients, only seven (14%) fulfilled the traditional Jones criteria for the diagnosis of the first episode of ARF. When compulsory evidence of a previous group A Beta hemolytic streptococcus (GABHS) was disregarded, this figure rose to 54%. Therefore, strict adherence to Jones criteria with absolute documentation of GABHS will lead to underdiagnoses of ARF. The application of echocardiographic diagnostic criteria of rheumatic heart disease (RHD) needs to be emphasized to allow early diagnosis and administration of secondary prophylaxis to prevent progression to severe valvular disease.

Comparative Performance of Modified Kenneth Jones Criteria Scoring, World Health Organization Criteria, and Antibodies in Lymphocyte Supernatant for Diagnosing Tuberculosis in Severely Malnourished Children Presenting With Pneumonia.

Background: The diagnosis of childhood tuberculosis (TB) can be difficult in severely malnourished children. This is mainly due to the fact of our perceived notion that clinical signs of TB are often subtle in severely malnourished children and we may rely on laboratory investigation for the diagnosis. However, comparative data on the performance of clinical and laboratory diagnostics of TB in such population are also very limited. Objectives: To compare the performance of composite clinical criteria and a technique that measures antibodies in lymphocyte supernatant (ALS) for the diagnosis of TB in severely malnourished children with pneumonia. Methods: Severely malnourished children under five with radiological pneumonia admitted to the Dhaka Hospital of International Centre for Diarrhoeal Disease Research, Bangladesh were enrolled consecutively following informed consent. We collected venous blood for ALS, gastric lavage fluid and induced sputum for microscopy, mycobacterial culture, and real-time PCR by Xpert MTB/RIF. We compared the sensitivity, specificity, positive, and negative predictive values, and accuracy of modified Kenneth Jones criteria (MKJC) score, World Health Organization (WHO) criteria, and ALS in diagnosing TB in severely malnourished children with pneumonia for "Confirmed TB" and "All TB" ("Confirmed TB" plus "Probable TB") vs. "Not TB." Results: Compared to culture confirmed TB, the sensitivity, and specificity (95% CI) for MKJC were 60 (27-86) and 84 (79-87)% and for WHO criteria were 40 (14-73) and 84 (80-87)%, respectively. Compared to culture and/or Xpert MTB/RIF positive TB, the sensitivity and specificity (95% CI) for the criteria were 37 (20-58) and 84 (79-87)%; and 22 (9-43) and 83 (79-87)%, respectively. For both these comparisons, the sensitivity and specificity of ALS were 50 (14-86) and 60 (53-67)%, respectively. Conclusion: Our data suggest that WHO criteria and MKJC scoring mainly based on clinical criteria are more useful than ALS in diagnosing TB in young severely malnourished children with pneumonia. The results underscore the importance of using clinical criteria for the diagnosis of TB in severely malnourished children that may help to minimize the chance of over treatment with anti-TB in such population, especially in resource limited TB endemic settings.

Comparison of antistreptolysin O and anti-deoxyribonucleic B titers in healthy children to those with acute pharyngitis, acute rheumatic fever, and rheumatic heart disease aged 5-15 years.

BACKGROUND: Acute rheumatic fever (ARF) affects millions of children in the third world countries like India. The diagnosis of rheumatic fever is based on the Jones criteria with serological titers, antistreptolysin O titer (ASO), and anti-deoxyribonucleic B (ADB), taken as evidence of recent streptococci infection. There is a lack of recent data available on ASO and ADB titers in children from the Delhi/NCR and thus adequate geographical area-specific cutoffs for the region are not available. AIMS AND OBJECTIVES: The aim of this study is to determine and compare the ASO and ADB antibody titers in children with acute pharyngitis, ARF, rheumatic heart disease (RHD), and in healthy children of the Delhi/NCR region. MATERIALS AND METHODS: Twenty-six cases of ARF, 51 cases of RHD, 50 cases of acute pharyngitis, and 84 healthy normal children were included in the study. A single ASO and ADB titer measurement was done in these children. RESULTS: The ASO titers was raised in acute pharyngitis - 303 IU/ml (interquartile range [IQR], 142-520 IU/ml) and ARF - 347.5 IU/ml (IQR, 125-686 IU/ml) children in comparison to healthy controls - 163.5 IU/ml (IQR, 133-246.5 IU/ml) and RHD patients - 163 IU/ml (IQR, 98.250-324.500). The ADB titers were highest in ARF patients - 570.5 IU/ml (IQR, 276-922 IU/ml) followed with RHD - 205 IU/ml (IQR, 113.6-456.5), healthy controls - 78.25 IU/ml (IQR, 53.39-128.15 IU/ml), and acute pharyngitis - 75.12 IU/ml (IQR, 64.5-136 IU/ml). The upper limit of normal (ULN) values of ASO and ADB computed from normal healthy children were 262.4 IU/ml and 134.44 IU/ml, respectively, and these can be used as cutoff values for recent streptococcal infection in this geographical area. CONCLUSIONS: The median ASO titers in acute pharyngitis group and ARF were significantly raised compared to that of the control group. The ADB titers were raised in ARF and RHD patients albeit the levels were higher in ARF patients. The derived ULN values can be used as cutoff reference.

Clinical characteristics of pediatric patients with first-attack acute rheumatic fever following the updated guideline.

AIM: To evaluate the clinical features of children diagnosed as having acute rheumatic fever between June 2015 and November 2018, and the changes observed in patient groups in comparison with data obtained in previous years. The diagnosis of acute rheumatic fever was made using the updated Jones criteria. MATERIAL AND METHODS: The medical records of pediatric patients who were diagnosed as having acute rheumatic fever between June 2015 and November 2018 using the updated criteria, were examined retrospectively. The data of a previous study that used the old criteria were reorganized and the two groups were compared. RESULTS: A total of consecutive 50 patients [22 males (44%)] who presented in the study period and were diagnosed as having first-attack acute rheumatic fever, were included in our study. Carditis was found in 42 (84%) patients. Manifest carditis was found in 24 patients and silent carditis was found in 18 patients. Joint involvement was present in 34 (68%) patients. Accompanying carditis was present in all 14 patients (28%) who were found to have chorea. Erythema marginatum and subcutaneous nodules were not found in our patients. When evaluated in terms of the updated criteria, a diagnosis of rheumatic fever was made with silent carditis+polyarthralgia in two patients, with silent carditis+monoarthritis in two patients, with polyarthralgia in four patients, and with monoarhtritis in one patient in our study. A diagnosis could be made by means of the updated criteria in a total of 9 (18%) patients. When compared with the previous study, an increase in the rate of silent carditis (from 21.8% to 36%) and a reduction in the rate of total carditis (from 92% to 84%) were found. CONCLUSION: Our results show that the updated Jones criteria prevent under diagnosis of acute rheumatic fever in an important number of patients.

Rheumatic fever - new diagnostic criteria.

Rheumatic fever (RF) is an autoimmune disease associated with group A ß-hemolytic streptococcal infection, in the course of which the patient develops carditis, arthritis, chorea, subcutaneous nodules and erythema marginatum. Rheumatic fever diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015. The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with echocardiographic examination, the introduction of a concept of subclinical carditis and the isolation of low, medium and high risk populations among the patients. AHA recommends that all the patients with suspected RF undergo Doppler echocardiographic examination after the Jones criteria have been verified, even if no clinical signs of carditis are present.

Epidemiologic Impact of the New Guidelines for the Diagnosis of Acute Rheumatic Fever.

OBJECTIVES: To estimate the incidence of acute rheumatic fever (ARF) in a metropolitan area of Northern Italy and study how the introduction of the 2015 revised Jones criteria affects the epidemiology in a region with moderate to high incidence of ARF. STUDY DESIGN: The incidence of ARF in children 5-14 years old living in the Province of Turin was estimated using low-risk criteria in a 10-year period (group A patients). The proportion of patients fulfilling only high-risk (HR) criteria (group B patients) was also calculated both prospectively (from July 2015 through December 2016) and retrospectively (from January 2007 through June 2015). RESULTS: One hundred thirty-five group A patients were identified for an annual incidence of 3.2-9.6 out of 100 000 children. The use of HR criteria identified an additional 28 patients (group B), resulting in a 20.7% increase in the incidence of ARF. Age, sex annual incidence, and seasonal distribution pattern were comparable between group A and group B patients. CONCLUSIONS: HR criteria should be used for the diagnosis ARF in our region. The application of these criteria led to a 20% increase in patients with the diagnosis of ARF. The characteristics of patients fulfilling only HR criteria are similar to the remaining patients, suggesting that these criteria are sensitive and specific.

Atypical Articular Presentations in Indian Children With Rheumatic Fever.

BACKGROUND: The objective of the study was to describe the clinical characteristics of atypical articular presentations during the initial outbreak and recurrence in patients with acute rheumatic fever (ARF) in the paediatric age group. METHODS: This was a retrospective, observational study conducted between January 2012 and December 2014 on all suspected cases of acute rheumatic fever (ARF) fulfilling either WHO 2004 or Australian guidelines with atypical articular manifestations ie, presence of at least one of the following features: duration of symptoms more than 3 weeks; monoarthritis/arthralgia; involvement of small joints of hand and feet and/or cervical spine and/or hip joint; and, not responding to salicylates in 1 week. RESULTS: 'Atypical' pattern was present in 63% (39/62) of patients with articular manifestations, of which arthralgia was a common manifestation (57%). Polyarticular afflictions were predominately non-migratory (additive) in both atypical (74%; 29/39) and typical (82%; 18/23) groups. Monoarticular (33%) affliction of the joints constituted a significant disease manifestation. Time from onset to diagnosis was >3 weeks in 79% of patients while small joints involvement and axial joint involvement occurred in half of the cases (51%). Inadequate response to NSAIDs was found in three (7%) cases. CONCLUSION: Atypical manifestations in ARF may well be mistaken for a connective tissue disorder, post streptococcal reactive arthritis and septic arthritis. Physicians should be made aware of these features to prevent diagnostic dilemma, and to effect institution of appropriate management including penicillin prophylaxis.

An Ineffective Differential Diagnosis of Infective Endocarditis and Rheumatic Heart Disease after Streptococcal Skin and Soft Tissue Infection.

We herein report the case of a 68-year-old woman with a skin and soft tissue infection at her extremities. The blood culture results were positive for Streptococcus pyogenes, and we started treatment using ampicillin and clindamycin, although subsequent auscultation revealed a new-onset heart murmur. We therefore suspected rheumatic heart disease and infective endocarditis. The case met both the Jones criteria and the modified Duke criteria. Transesophageal echocardiography revealed vegetation on the aortic valve, although the pathological findings were also compatible with both rheumatic heart disease and infective endocarditis. The present findings suggest that these two diseases can coexist in some cases.

Rheumatic fever: update on the Jones criteria according to the American Heart Association review - 2015 / Febre reumática: atualização dos critérios de Jones à luz da revisão da American Heart Association - 2015

ABSTRACT Rheumatic fever is still currently a prevalent disease, especially in developing countries. Triggered by a Group A β-hemolytic Streptococcus infection, the disease may affect genetically predisposed patients. Rheumatic carditis is the most important of its clinical manifestations, which can generate incapacitating sequelae of great impact for the individual and for society. Currently, its diagnosis is made based on the Jones criteria, established in 1992 by the American Heart Association. In 2015, the AHA carried out a significant review of these criteria, with new diagnostic parameters and recommendations. In the present study, the authors perform a critical analysis of this new review, emphasizing the most relevant points for clinical practice.

RESUMO A febre reumática ainda é uma doença prevalente nos tempos atuais, sobretudo nos países em desenvolvimento. Deflagrada por uma infecção pelo Streptococcus β-hemolítico do grupo A, pode afetar pacientes geneticamente predispostos. A cardite reumática é a mais importante das manifestações clínicas, pode gerar sequelas incapacitantes e de grande impacto para o indivíduo e para a sociedade. Atualmente, seu diagnóstico é feito baseado nos Critérios de Jones, estabelecidos em 1992 pela American Heart Association (AHA). Em 2015, a AHA procedeu a uma significativa revisão desses critérios, com novos parâmetros e recomendações diagnósticas. No presente estudo, os autores fazem uma análise crítica dessa nova revisão e enfatizam os pontos de maior relevância para a prática clínica.

Rheumatic fever: update on the Jones criteria according to the American Heart Association review - 2015.

Rheumatic fever is still currently a prevalent disease, especially in developing countries. Triggered by a Group A ß-hemolytic Streptococcus infection, the disease may affect genetically predisposed patients. Rheumatic carditis is the most important of its clinical manifestations, which can generate incapacitating sequelae of great impact for the individual and for society. Currently, its diagnosis is made based on the Jones criteria, established in 1992 by the American Heart Association. In 2015, the AHA carried out a significant review of these criteria, with new diagnostic parameters and recommendations. In the present study, the authors perform a critical analysis of this new review, emphasizing the most relevant points for clinical practice.