ACUTE RHEUMATIC FEVER, A CHANGING DISEASE

Excerpt Although many careful studies have been made of rheumatic heart disease throughout the world, many misconceptions of the etiologic factors of the disease still exist. If one studies the rep...

ABSTRACTObjectiveTo describe the clinical and demographic profile of Australian children first diagnosed with acute rheumatic fever (ARF) or rheumatic heart disease (RHD) before the age of 5 years, with comparison to children aged 5–14 years.MethodsLinked emergency department, hospitalisation, RHD register and death records from the End RHD in Australia: Study of Epidemiology were used to identify first ARF/RHD diagnosis occurring in < 15‐year‐olds. Demographic/clinical profiles and pre‐diagnosis healthcare interactions were analysed with stratification into 5‐year age groups.DesignRetrospective cross‐sectional linked administrative data analysis.SettingNorthern Territory, South Australia, Queensland and Western Australia.ParticipantsChildren aged < 15 years at first hospitalisation or notification for ARF or RHD, 2001–2017.Main Outcome MeasuresDisease stage and severity at diagnosis, register notification status, clinical history prior to ARF or RHD diagnosis.ResultsOf 2382 children diagnosed with ARF/RHD aged < 15 years, 180 (7.6%) were aged under 5 years. Among under 5‐year‐olds with ARF or RHD, 30.6% had not been notified to RHD registers. A total 49 under 5‐year‐olds were diagnosed with RHD; with 22 (44.9%) classified as having mild disease, 16 (32.7%) moderate and 6 (12.2%) severe. High hospitalisation rates for injury in the first year of life were observed for the < 5‐year‐old cohort with ARF/RHD.ConclusionsWe present the first comprehensive Australian evidence that ARF and RHD diagnoses are occurring in Australian children aged under 5 years. Greater awareness among clinicians is needed regarding ARF/RHD as a potential diagnosis in this young, high risk age group.

To the Editor. As we move into the third millennium, acute rheumatic fever (ARF) appears to be a vanishing disease in the United States. However, conflicting reports regarding the incidence of ARF have been published during the past decade.1–3 Veasy et al1continue to see 20 or more new patients/year with ARF. We would like to support Veasy's observations, and note that 30 to 50 new ARF patients/year have been seen at the Children's Hospital of Denver, Colorado, during the past decade. While this is a striking reduction from the 450 cases/year reported in Colorado in 1970, it remains an important public health issue. It is highly probable that ARF persists at a reduced but significant level in other areas of the United States, as evidenced by the spate of reports4–10 following Veasy's original report in 1987.11 It is also highly probable that ARF is being underreferred by primary care physicians for many reasons including: 1) lower incidence of ARF during the last 30 years, resulting in lack of awareness of the disease in younger physicians; 2) ARF is now largely managed as an outpatient disease, further diluting the training experience of young pediatric health professionals; 3) alteration of the physical features of ARF a) common coexistence of chorea and carditis (historically felt to be rare); b) increasingly atypical migratory polyarthritis (the response to aspirin was a previously useful feature).ARF is not gone, but appears to be forgotten by many contemporary pediatric health care providers. Our experience is similar to Veasy et al, and we urge that medical school and postgraduate education continue to include ARF. The fact that ARF is neither an extinct nor a benign disease is supported by the need to replace mitral or aortic valves in 5 patients at the Children's Hospital during the past 10 years in our region. In addition, two teen-aged females from Colorado have presented with critical mitral stenosis, requiring balloon dilation in one case and emergency valve replacement in another.

Introduction: Congestive cardiac failure is the failure of the heart to pump blood at a rate commensurate with the requirements of the metabolizing tissue and or its ability to do so requires an abnormally elevated pressure. CCF once sets in, pressure with malignant progression with one year mortality of 38% and 15% and six year mortality of 80% and 65% in men and women respectively. Acute rheumatic fever(ARF) and its sequel Rheumatic Heart Disease(RHD) may impair myocardial function to precipitate failure. Aim: To study clinical features of Congestive Cardiac Failure in Rheumatic Heart Disease. Methods: A prospective study with 50 patients admitted between August 2006 and September 2008 in Shree Birendra Hospital formed the subject of this study.Patients of rheumatic valvular heart disease of both sexes in congestive cardiac failure at the time of admission or who developed failure during hospitalization were taken into the study. Detailed history was taken from all patients. Detailed examination was done in all patients with emphasis in cardiovascular and relevant system. Modified Framingham heart study criteria was used for the diagnosis of CCF. Results: A total of 50 patients with RHD in CCF were the subject of study. The average age was 33.78 years. The presenting clinical features of the patients were breathlessness on exertion and it was the commonest presenting complaint and was present in all patients. Palpitation was present 37 of 50 patients(74%). Basal crepitation was the commonest clinical sign present and 29 of 50 patients(58%) had the raised jugular venous pressure. Mitral valve was the commonest valve involved (with/without) other valves) and was seen in 94% (47) patients. Key words: Congestive Cardiac Failure; Rheumatic Heart Disease DOI: http://dx.doi.org/10.3126/mjsbh.v9i2.5019 Medical Journal of Shree Birendra Hospital Vol.9(2) 2010: 8-12

By the late 1980s, acute rheumatic fever (ARF) had become a rare disease in Taiwan. The low prevalence rate in this area is attributed to a better economic status, which has led to improved public health and adequate medical services. The increasing number of patients with adult-onset ARF in the United States described in the literature prompted us to evaluate the cases diagnosed in our medical center. A retrospective chart review was performed for patients with arthritis from July 1988 to October 2004. To be included, patients had to meet revised Jones criteria. Three adult patients with ARF have been diagnosed since June 2001, with no childhood ARF being diagnosed. All cases presented with migratory polyarthritis, whereas 1 had erythema marginatum and transient carditis. These patients responded well to treatment with antibiotics and nonsteroidal antiinflammatory drugs. Clinicians must provide careful assessment and treatment to patients presenting with acute pharyngitis. A possible resurgence of ARF can be eradicated by primary prevention of streptococcal pharyngitis.

Rheumatic fever is a poststreptococcal disease that is receiving renewed attention by the medical community. We describe a recent increase in the number of observed cases of acute rheumatic fever (ARF) in West Virginia. This is the fifth report of a recent increase in the incidence of ARF in the Ohio Valley area in the last 4 years. In contrast to the other reports, nearly two thirds of our cases of ARF were in adults, more than half of whom had suffered previous bouts of ARF. In these adults with recurrences, none was taking prophylactic penicillin at the time of presentation. Carditis was present in seven adults, two without a history of carditis. Arthritis was present in all adult patients. These data indicate a possible geographic phenomenon related to the increased number of observed cases of ARF and document that ARF is not simply a disease of childhood. Furthermore, our findings highlight the need for extended penicillin prophylaxis for secondary prevention of ARF, especially for those with an increased risk of acquiring a streptococcal upper respiratory tract infection.

Acute rheumatic fever (ARF) is a sequela of streptococcal infection—typically following 2 to 3 weeks after group A streptococcal pharyngitis—that occurs most commonly in children and has rheumatologic, cardiac, and neurologic manifestations. Rheumatic heart disease (RHD) refers to the long-term cardiac damage caused by either a single severe episode or multiple recurrent episodes of ARF. Secondary prophylaxis with regular intramuscular injections of benzathine penicillin G (BPG) is a key component to prevent ARF and RHD recurrent. This study aimed to evaluate the secondary prophylaxis effectiveness based on the profile of clinical manifestations and blood laboratory findings. The data were collected by interviews, complete blood counts, the anti-streptolysin titer O (ASTO) analysis, the C-reactive protein Assay, electrocardiography, and echocardiography. Data were analyzed using SPSS 18, and the results were presented as the percentage frequency. The samples were 63 patients with ARF and RHD who get secondary prophylaxis, consisting of 32 males (50.8%) and 31 females (49.9%). The echocardiography examination showed that valve regurgitation was more common than valve stenosis, indicating longer P-R interval in 49 subjects (77.7%). Sixty subjects (92.2%) received secondary prophylaxis with BPG injection, and three subjects (4.8%) got Erythromycin oral. The main factor of secondary prophylaxis adherence is pain and injection disliking. How to Cite this Article Pubmed Style DIMIATI H, SOFIA S, GANI BA. for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. SRP. 2020; 11(9): 452-457. doi:10.31838/srp.2020.9.63 Web Style DIMIATI H, SOFIA S, GANI BA. for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. http://www.sysrevpharm.org/?mno=11016 [Access: March 29, 2021]. doi:10.31838/srp.2020.9.63 AMA (American Medical Association) Style DIMIATI H, SOFIA S, GANI BA. for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. SRP. 2020; 11(9): 452-457. doi:10.31838/srp.2020.9.63 Vancouver/ICMJE Style DIMIATI H, SOFIA S, GANI BA. for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. SRP. (2020), [cited March 29, 2021]; 11(9): 452-457. doi:10.31838/srp.2020.9.63 Harvard Style DIMIATI, H., SOFIA, . S. & GANI, . B. A. (2020) for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. SRP, 11 (9), 452-457. doi:10.31838/srp.2020.9.63 Turabian Style DIMIATI, HERLINA, SOFIA SOFIA, and BASRI A. GANI. 2020. for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Reviews in Pharmacy, 11 (9), 452-457. doi:10.31838/srp.2020.9.63 Chicago Style DIMIATI, HERLINA, SOFIA SOFIA, and BASRI A. GANI. Penicillin for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. Systematic Reviews in Pharmacy 11 (2020), 452-457. doi:10.31838/srp.2020.9.63 MLA (The Modern Language Association) Style DIMIATI, HERLINA, SOFIA SOFIA, and BASRI A. GANI. Penicillin for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Children. Systematic Reviews in Pharmacy 11.9 (2020), 452-457. Print. doi:10.31838/srp.2020.9.63 APA (American Psychological Association) Style DIMIATI, H., SOFIA, . S. & GANI, . B. A. (2020) for Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease in Acehnese Reviews in Pharmacy, 11 (9), 452-457. doi:10.31838/srp.2020.9.63

Introduction: Rheumatic heart disease (RHD) remains a leading cause of cardiovascular mortality in sub-Saharan Africa. Identifying geographic “hot spots” and high risk populations are crucial for the development of RHD prevention and screening strategies in high burden areas. Objectives: We aimed to identify demographic patterns and geographic variation in RHD prevalence in western Kenya. Methods: We conducted a retrospective chart review of all patients &lt;50 years old (n=937) attending adult cardiology clinic at a national referral hospital in western Kenya. Demographic information, residential location and cardiac history were collected. We mapped the spatial distribution of cardiac disease rates and analyzed the effect of distance from the hospital on RHD status using multivariable logistic regression. Results: Two-thirds (64%) of all patients &lt;50 years old had RHD. RHD patients were younger (26 vs. 33 years, p&lt;0.001) and more often female (69% vs. 59%, p=0.002) than non-RHD patients. Patients of Nilote ethnicity had over twice the odds of RHD than non-Nilotes (OR 2.5, 95%CI 1.7-3.7). Global clustering of disease rates exists within 200km of the hospital with significant clustering of the RHD and non-RHD rate difference seen surrounding the hospital in the (Moran’s I: 0.3, p=0.001) (Figure). Clustering was attenuated after controlling for age, gender, ethnicity and distance from the hospital. There was an interaction between ethnicity and distance to the hospital such that the odds of RHD increased with further distance for Nilotes, but the odds of RHD in non-Nilotes decreased with further distance. Conclusion: Most adult cardiology patients treated at a national referral hospital in western Kenya have RHD. Young females of Nilote ethnicity are commonly affected. Ethnicity and distance to the hospital interdependently affect the odds of RHD, and geographic clustering of disease rates may reflect this relationship. Future studies in this area should consider the impact of ethnic predisposition to RHD.

Background In February 2025, pediatricians alerted the Alaska Department of Health to a possible increase in cases of acute rheumatic fever (ARF) and rheumatic heart disease (RHD). ARF/RHD are immune-mediated sequelae of group A Streptococcus (GAS) infections. ARF primarily affects school-aged children and can progress to RHD without appropriate antibiotic treatment and prophylaxis. RHD is characterized by damage to heart valves and can result in death. Early diagnosis, appropriate antibiotic treatment, and prophylaxis for people with ARF/RHD reduce the risk of ARF recurrence and RHD progression.Table 1:Demographic characteristics of patients with ARF and RHD included in medical records review, Alaska, Jan 2022–Feb 2025. (N=52)Table 2.Incidence of ARF/RHD among ages 3-17 years from medical records review, Alaska, Jan 2022–Feb 2025. Methods We calculated ARF/RHD incidence among children aged 3–17 years living in Alaska during January 2022–February 2025 using data reported by healthcare systems in response to a statewide call for cases. We conducted medical chart reviews to collect information on presenting signs and symptoms, prior GAS infections, clinical management, and outcomes. We also conducted a secondary data analysis of the Indian Health Service’s (IHS) National Patient Information Reporting System to calculate incidence of ARF/RHD during 2014–2023 among Alaska Native children aged 3–17 who received care within the Tribal Health System.Figure 1.Indian Health Service National Patient Information Reporting System trends in reported incidence of new ARF and RHD among AI/AN children aged 3–17 years, Alaska, 2014–2023Figure 2.Indian Health Service’s National Patient Information Reporting System reported average ARF incidence (3–17 years) among AI/AN people by region, Alaska, 2014–2023 Results ARF and RHD incidence among children aged 3–17 increased from 3.8 and 1.9 per 100,000 persons per year in 2022 to 5.3 and 5.3 per 100,000 persons per year, respectively, in 2024. Incidence was highest in the northern (ARF: 33.5; RHD: 33.5) and southwestern (ARF: 23.0; RHD: 41.4) regions of Alaska and among children who were Alaska Native (ARF: 11.1; RHD: 11.1) or Native Hawaiian/Pacific Islander (ARF: 27.3; RHD: 16.4). Analysis of IHS data found the average annual incidences of ARF/RHD during 2014–2023 among Alaska Native children aged 3–17 were 6.2 per 100,000 persons and 8.5 per 100,000 persons, respectively. Conclusion ARF/RHD incidence in Alaska is higher than the rest of the United States, particularly among Alaska Native and Native Hawaiian/Pacific Islander people. The observed recent increases may be linked to factors such as a rise in GAS infections. Potential opportunities to improve early diagnosis and clinical management of GAS infections and sequelae can inform public health recommendations and clinical guidance. Disclosures All Authors: No reported disclosures

Rheumatic fever, an immune sequela of untreated streptococcal infections, is an important contributor to global cardiovascular disease. The goal of this study was to describe trends, characteristics, and cost burden of children discharged from hospitals with a diagnosis of RF from 2000 to 2012 within the United States. Using the Kids' Inpatient Database, we examined characteristics of children discharged from hospitals with the diagnosis of rheumatic fever over time including: overall hospitalisation rates, age, gender, race/ethnicity, regional differences, payer type, length of stay, and charges. The estimated national cumulative incidence of rheumatic fever in the United States between 2000 and 2012 was 0.61 cases per 100,000 children. The median age was 10 years, with hospitalisations significantly more common among children aged 6-11 years. Rheumatic fever hospitalisations among Asian/Pacific Islanders were significantly over-represented. The proportion of rheumatic fever hospitalisations was greater in the Northeast and less in the South, although the highest number of rheumatic fever admissions occurred in the South. Expected payer type was more likely to be private insurance, and the median total hospital charges (adjusted for inflation to 2012 dollars) were $16,000 (interquartile range: $8900-31,200). Median length of stay was 3 days, and the case fatality ratio for RF in the United States was 0.4%. Rheumatic fever persists in the United States with an overall downwards trend between 2003 and 2012. Rheumatic fever admissions varied considerably based on age group, region, and origin.

Acute rheumatic fever (ARF) is an autoimmune condition following a preceding beta-haemolytic streptococcal throat infection. It affects children in the age group of 5-15 years predominantly. In the older age group, the incidence decreases, with the first episode being very rare, though recurrence may occur. Further, patients who develop ARF in childhood, unless they quit the penicillin prophylaxis, recurrence or reactivation is also less. This is a retrospective case series of six adult patients aged >30 years who presented to our hospital in the last one year and were diagnosed with either ARF or reactivation as per the 2015 revised Jones criteria.This study was conducted to emphasise that ARF is not an uncommon disease in adults in our country. Though the patient may have subtle symptoms and signs, we need to identify them and evaluate further with the required investigations to prove the diagnosis with the modified Jones criteria.When an adult patient presents with fever, joint pain, and valvular lesions, rheumatic fever is least thought of due to the age of the patient, and multiple other differentials are considered. Patients in our case series had varied presentations and acute rheumatic activity. To conclude, physicians should have a low threshold to consider ARF irrespective of age, and once ARF is diagnosed, patients need to be properly counselled about the strict adherence to penicillin prophylaxis.

Geographic distribution of rheumatic fever and rheumatic heart disease in the United States

In recent years, the incidence of acute rheumatic fever (ARF) has drastically decreased in the United States while remaining common in developing nations. However, immigration to the United States from developing nations has resulted in the continued prevalence of ARF. Patients with ARF face the potential development of significant valvular insufficiency and rheumatic carditis, which are associated with significant morbidity and mortality. Potentially adverse outcomes may be avoided with advanced interventions such as aortic and/or mitral valve replacement or repair, the use of inotropic agents, and ventricular assist devices for mechanical circulatory support. This report describes a series of three ARF carditis cases. This series serves not only to emphasize proper ARF prevention, but also to demonstrate the need for timely surgical intervention once medical therapy has failed.

Background: Rheumatic heart disease (RHD) combined with atrial fibrillation (AF) significantly contributes to thromboembolic complications, including ischemic stroke. The coexistence of these conditions increases the risk of cardiovascular issues, underscoring the need for targeted management strategies. Objective: This study aims to assess the prevalence and determinants of thromboembolic events among patients with RHD and AF at the Tikur Anbessa Specialized Hospital adult cardiac outpatient clinic in Addis Ababa, Ethiopia. Methods: An institution-based retrospective chart review of adult patients with RHD and AF was conducted. Data were gathered from electronic medical records using structured data extraction check list. Descriptive analysis using frequency with percentage and mean with standard deviation was conducted. Binary logistic regression was employed to analyze the relationship between predictor variables and thromboembolic events. Results: A total of 182 participants were included in this study. The mean age of participants was 40.37 (SD± 13.01) and majority of participants were female participants (70.9%). The overall rate of cardioembolic events was 19.78% (95% CI: 14.3-6.3), with stroke occurring in 18.1% (33) of the participants. Disease duration (AOR: 1.07, 95% CI: 1.03–1.13), left atrial size (AOR: 1.14, 95% CI: 1.07–1.24), presence of left atrial thrombus (AOR: 9.83, 95% CI: 1.53–63.21), mitral stenosis (AOR: 1.88, 95% CI: 1.21–17.14), and Subtheraputic INR levels (&lt;2) (AOR: 4.27, 95% CI: 1.15–15.94) were significantly associated with cardioembolic events. Conclusion and recommendation: This study highlights the high prevalence of cardioembolic events in patients with RHD and AF, identifying both modifiable and non-modifiable factors that contribute to increased risk. Monitoring left atrial size and INR levels could help reduce the risk of thromboembolic complications. Further research is needed to develop preventive strategies and optimize management to improve patient outcomes.

The annual incidence of acute rheumatic fever (ARF) in Hawaii has remained several times higher than that in the continental United States, particularly among ethnic Polynesians. The emm types of Streptococcus pyogenes that are associated with this nonsuppurative complication have, to our knowledge, not been previously reported in Hawaii. Patients with ARF were identified through an active surveillance system at Kapiolani Medical Center (Honolulu, HI), the only pediatric tertiary care referral hospital in Hawaii. Specimens were obtained by throat culture from patients who met the Jones criteria for ARF at the time of presentation (63 patients), prior to penicillin treatment, and from consenting family contacts (10 individuals). Eight patients and 2 close family contacts with positive throat culture results were identified from February 2000 through December 2005. Group A streptococci isolates were characterized by emm sequence typing. Unusual emm types were temporally associated with the onset of ARF. Emm types 65/69 (from 2 patients), 71, 92, 93, 98, 103, and 122 were isolated from the 8 patients with ARF, and emm types 52 and 101 were isolated from the 2 household contacts. So-called rheumatogenic emm types and/or serotypes, which were previously associated with ARF in the continental United States, were not found in this study. Instead, emm types that are not commonly included among group A streptococci isolates in the continental United States and that are seldom, if ever, temporally associated with ARF were identified. These findings suggest that unusual group A streptococci emm types play a significant role in the epidemiology of ARF in Hawaii.