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Rheumatic fever (RF) and rheumatic heart disease (RHD) continue to be a major health hazard in most developing countries as well as sporadically in developed economies. Despite reservations about the utility, echocardiographic and Doppler (E&D) studies have identified a massive burden of RHD suggesting the inadequacy of the Jones′ criteria updated by the American Heart Association in 1992. Subclinical carditis has been recognized by E&D in patients with acute RF without clinical carditis as well as by follow up of RHD patients presenting as isolated chorea or those without clinical evidence of carditis. Over the years, the medical management of RF has not changed. Paediatric and juvenile mitral stenosis (MS), upto the age of 12 and 20 yr respectively, severe enough to require operative treatement was documented. These negate the belief that patients of RHD become symptomatic ≥20 years after RF as well as the fact that congestive cardiac failure in childhood indicates active carditis and RF. Non-surgical balloon mitral valvotomy for MS has been initiated. Mitral and/or aortic valve replacement during active RF in patients not responding to medical treatment has been found to be life saving as well as confirming that congestive heart failure in acute RF is due to an acute haemodynamic overload. Pathogenesis as well as susceptibility to RF continue to be elusive. Prevention of RF morbidity depends on secondary prophylaxis which cannot reduce the burden of diseases. Primary prophylaxis is not feasible in the absence of a suitable vaccine. Attempts to design an antistreptococcal vaccine utilizing the M-protein has not succeeded in the last 40 years. Besides pathogenesis many other questions remain unanswered.

Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus . The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances — including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life — give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty. Acute rheumatic fever (ARF) is caused by an autoimmune response to group A streptococcal infection. Severe and/or repeated episodes of ARF can lead to rheumatic heart disease (RHD). Both conditions predominantly affect those experiencing economic disadvantage.

For more than a century, certain bacterial infections that can breach the skin and mucosal barriers have been implicated as common triggers of autoimmune syndromes, especially post-infection autoimmune diseases that include rheumatic fever and post-streptococcal glomerulonephritis. However, only in the past few years has the importance of imbalances within our own commensal microbiota communities, and within the gut, in the absence of infection, in promoting autoimmune pathogenesis become fully appreciated. A diversity of species and mechanisms have been implicated, including disruption of the gut barrier. Emerging data suggest that expansions (or blooms) of pathobiont species are involved in autoimmune pathogenesis and stimulate clonal expansion of T cells and B cells that recognize microbial antigens. This Review discusses the relationship between the gut microbiome and the immune system, and the potential consequence of disrupting the community balance in terms of autoimmune development, focusing on systemic lupus erythematosus. Notably, inter-relationships between expansions of certain members within gut microbiota communities and concurrent autoimmune responses bear features reminiscent of classical post-infection autoimmune disease. From such insights, new therapeutic opportunities are being considered to restore the balance within microbiota communities or re-establishing the gut-barrier integrity to reinforce immune homeostasis in the host.This Review discusses the interplay of the gut microbiome and the immune system in the context of systemic lupus erythematosus. Dysbiosis and gut-barrier dysfunction are implicated in promoting disease and are potential therapeutic targets.

Background Acute rheumatic fever (ARF) is caused by an autoimmune response to throat infection with group A Streptococcus. ARF and its sequel, chronic rheumatic heart disease, remain significant public health problems in many low-income countries. Rapid diagnosis and timely treatment of ARF are essential to avoiding serious adverse outcomes. Case presentation A 16-year-old adolescent with hyperthyroidism and treatment noncompliance presented with symptoms such as fever, difficulty lying flat at night, cough with expectoration, palpitations, and tachypnea. On the basis of the patient’s symptoms, physical signs, and laboratory test results, a preliminary diagnosis of thyroid storm (TS) was made, and hydrocortisone, antithyroid drugs, and anti-infective agents were promptly administered. However, subsequent examinations revealed hemorrhagic pericardial and pleural effusions, indicating that hyperthyroidism complicated by other conditions (including tuberculosis, malignancies, and certain autoimmune diseases) should be considered. Ultimately, the diagnosis of ARF was confirmed on the basis of his dramatic clinical response to hydrocortisone, cardiac inflammatory manifestations, and significantly elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and anti-streptolysin O (ASO) levels. Follow-up revealed that the patient’s rheumatic fever was well controlled and alleviated by treatment. Conclusions ARF may have multiple clinical manifestations, but hemorrhagic pericardial and/or pleural effusions are extremely rare. In children and adolescents from developing countries, even in the absence of pharyngitis or tonsillitis, ARF should always be considered when symptoms such as unexplained fever, diaphoresis, palpitations, emaciation and epistaxis are present.

Certain immune-mediated diseases (IMDs), such as rheumatoid arthritis and systemic lupus erythematosus, have been linked to cardiovascular disorders. We examined whether there is an association between 32 different IMDs and risk of subsequent hospitalization for coronary heart disease (CHD) related to coronary atherosclerosis in a nationwide follow up study in Sweden. All individuals in Sweden hospitalized with a main diagnosis of an IMD (n = 336,479) without previous or coexisting CHD, between January 1, 1964 and December 31 2008, were followed for first hospitalization for CHD. The reference population was the total population of Sweden. Standardized incidence ratios (SIRs) for CHD were calculated. Overall risk of CHD during the first year after hospitalization for an IMD was 2.92 (95% CI 2.84-2.99). Twenty-seven of the 32 IMDs studied were associated with an increased risk of CHD during the first year after hospitalization. The overall risk of CHD decreased over time, from 1.75 after 1-5 years (95% CI 1.73-1.78), to 1.43 after 5-10 years (95% CI 1.41-1.46) and 1.28 after 10+ years (95% CI 1.26-1.30). Females generally had higher SIRs than males. The IMDs for which the SIRs of CDH were highest during the first year after hospitalization included chorea minor 6.98 (95% CI 1.32-20.65), systemic lupus erythematosus 4.94 (95% CI 4.15-5.83), rheumatic fever 4.65 (95% CI 3.53-6.01), Hashimoto's thyroiditis 4.30 (95% CI 3.87-4.75), polymyositis/dermatomyositis 3.81 (95% CI 2.62-5.35), polyarteritis nodosa 3.81 (95% CI 2.72-5.19), rheumatoid arthritis 3.72 (95% CI 3.56-3.88), systemic sclerosis 3.44 (95% CI 2.86-4.09), primary biliary cirrhosis 3.32 (95% CI 2.34-4.58), and autoimmune hemolytic anemia 3.17 (95% CI 2.16-4.47). Most IMDs are associated with increased risk of CHD in the first year after hospital admission. Our findings suggest that many hospitalized IMDs are tightly linked to coronary atherosclerosis.

Calcific aortic stenosis (AS) is the most prevalent heart valve disorder in developed countries. It is characterized by progressive fibro-calcific remodelling and thickening of the aortic valve leaflets that, over years, evolve to cause severe obstruction to cardiac outflow. In developed countries, AS is the third-most frequent cardiovascular disease after coronary artery disease and systemic arterial hypertension, with a prevalence of 0.4% in the general population and 1.7% in the population >65 years old. Congenital abnormality (bicuspid valve) and older age are powerful risk factors for calcific AS. Metabolic syndrome and an elevated plasma level of lipoprotein(a) have also been associated with increased risk of calcific AS. The pathobiology of calcific AS is complex and involves genetic factors, lipoprotein deposition and oxidation, chronic inflammation, osteoblastic transition of cardiac valve interstitial cells and active leaflet calcification. Although no pharmacotherapy has proved to be effective in reducing the progression of AS, promising therapeutic targets include lipoprotein(a), the renin–angiotensin system, receptor activator of NF-κB ligand (RANKL; also known as TNFSF11) and ectonucleotidases. Currently, aortic valve replacement (AVR) remains the only effective treatment for severe AS. The diagnosis and staging of AS are based on the assessment of stenosis severity and left ventricular systolic function by Doppler echocardiography, and the presence of symptoms. The introduction of transcatheter AVR in the past decade has been a transformative therapeutic innovation for patients at high or prohibitive risk for surgical valve replacement, and this new technology might extend to lower-risk patients in the near future. Calcific aortic stenosis (AS) involves fibro-calcific remodelling of the aortic valve that causes restriction of blood flow. Pibarot and colleagues discuss the mechanisms, diagnosis and management of AS and highlight how the introduction of transcatheter-based valve replacement has transformed patient outcomes.

Describe the echocardiographic evolution of valvular regurgitation in patients with rheumatic carditis (RC) and to establish which features may predict long-term outcome, in the absence of acute rheumatic fever (ARF) relapse. Retrospective cohort study. 123 patients with confirmed RC, diagnosed at Turin Children’s Hospital between 2010 and 2019. We reviewed the echocardiographic images recorded at diagnosis, after 6–8 weeks, after 6 months, then yearly, to assess which predictors at diagnosis are associated with the degree of improvement at 6 months. Secondly, we tested which variables predict the regression of pathological regurgitation of mitral (MV) or aortic valve (AV) during follow-up. At onset, 90.2% patients had MV regurgitation while 42.3% had AV involvement. 115 (93.5%) patients were treated with steroids and 70.8% experienced a downgrading of RC after 6 months. Steroids were associated with better outcomes at six months (p = 0.01). During follow-up (median 56.1 months), MV improved in 58.6% patients, AV in 46.2%. At multivariate analysis, erythrocyte sedimentation rate (ESR) was positively associated with regression of MV regurgitation (OR 1.02, p = 0.02), while higher degree of carditis at onset was negatively associated (OR 0.04, p < 0.01). Conversely, regression of AV regurgitation was more frequent in patients with bi-valvular involvement (OR 20.5, p = 0.03) and in absence of murmur at onset (OR 0.04, p = 0.01). This study indicates that valvular regurgitation improves overtime if there are no ARF recurrences during follow-up, especially when the MV is involved and in patients treated with steroids.

Introduction: Patients with inflammatory rheumatic diseases have an increased risk of developing cardiovascular manifestations. The high risk of cardiovascular pathology in these patients is not only due to traditional cardiovascular risk factors (age, gender, family history, smoking, sedentary lifestyle, cholesterol), but also to chronic inflammation and autoimmunity. Aim: In this review, we present the mechanisms of cardiovascular comorbidities associated with inflammatory rheumatic diseases, as they have recently been reported by different authors, grouped in electrical abnormalities, valvular, myocardial and pericardial modifications and vascular involvement. Methods: We conducted a systematic search of published literature on the following online databases: EBSCO, ScienceDirect, Scopus and PubMed. Searches were limited to full-text English-language journal articles published between 2010 and 2017 using the following key words: heart, systemic inflammation, autoimmunity, rheumatic diseases and disease activity. After the primary analysis we included 50 scientific articles in this review. Results: The results showed that cardiac manifestations of systemic inflammation can occur frequently with different prevalence in rheumatoid arthritis (RA), systemic lupus erythematosus(SLE), systemic sclerosis(SSc) and ankylosing spondylitis(AS). Rheumatologic diseases can affect the myocardium, cardiac valves, pericardium, conduction system and arterial vasculature. Conclusions: Early detection, adequate management and therapy of specific cardiac involvement are essential in rheumatic disease. Electrocardiographic and echocardiographic evaluation should be performed as routine investigations in patients with inflammatory rheumatic diseases.

Acute rheumatic fever (ARF) is the precursor to rheumatic heart disease (RHD) following Group A Streptococcal infection. However, many diagnoses of RHD are made in the absence of ARF history. We compared RHD severity between those with and those without a documented history of ARF. A retrospective audit of echocardiographic images determined RHD stage at diagnosis and at follow-up based on the 2023 WHF guidelines for the diagnosis of RHD.Individuals aged ≤ 20 years from the Top End of the Northern Territory (NT) of Australia with RHD diagnosis between January 2012 and December 2021 were included.Primary outcome was RHD stage at the time of diagnosis. Secondary outcomes were RHD stage progression or regression. Those with ARF and those with no ARF (noARF) were compared. Study population ( ) of 292 individuals with mean age 11.9 ± 3.8 years. At baseline, the ARF group had more Stage A RHD (28.6% versus 12.0%), while the noARF group had more Stage B (50.0% versus 38.0%), = 0.009. There was no difference in advanced RHD (Stage C and D combined) between the groups ( = 0.440). Follow-up (median 46 months, IQR: 27-71 months) sample size was 230. Regression of RHD was greater in the ARF group (46% versus 28%, = 0.014). No difference was found in stage progression (including to surgery), with 21% (32/156) in the ARF group and 15% (11/74) in the noARF group ( = 0.367). Individuals at all stages of RHD severity were detected amongst those with and without an accompanying diagnosis of ARF. Individuals with first RHD diagnosis accompanied by ARF were more likely to regress. These findings support echocardiographic screening in high-risk populations to detect early RHD that can be treated with secondary antibiotic prophylaxis. Further research is required to understand the reason for differences between the ARF and noARF groups.

Mitral stenosis is a common disease that causes substantial morbidity worldwide. The disease is most prevalent in developing countries, but is increasingly being identified in an atypical form in developed countries. All treatments that increase valve area improve morbidity. Mortality improves with surgery; the benefit of percutaneous balloon valvuloplasty to mortality might be similar to that of surgery but needs further study. Percutaneous balloon valvuloplasty is the treatment of choice for patients in whom treatment is indicated, except for those with suboptimum valve morphology, and even these patients are sometimes treated with this procedure if surgery is not feasible or if surgical risk is prohibitive. We review the pathology, diagnosis, and treatment options for patients with mitral stenosis.