Background/Aims: Data on the frequency of acute rheumatic fever in Turkey is limited. The study aims to evaluate patients' demographic and clinical characteristics retrospectively followed up with a diagnosis of acute rheumatic fever in the Eastern Black Sea Region. Materials and Methods: Demographic, clinical and laboratory characteristics of patients diagnosed with acute rheumatic fever in our clinic between January 2005 and December 2017 were evaluated from archival records. Results: Included in the study were 175 patients with a mean age of 11±3.52 years (26 months–17 years), with a female/male ratio of 1.38. Patients are most frequently presented in February, May and August. Carditis was the most common (80%) major finding, while arthritis was seen in 51.4% and chorea in 16%. Erythema marginatum was present in only one patient. While isolated mitral valve involvement (54.3%) was most common in the patients with carditis, simultaneous mitral and aortic valve involvement was found in 30.7% of the patients, and isolated aortic valve involvement in 10%. Tricuspid regurgitation was seen in three (2.1%) patients with mitral involvement. Recurrence was detected in four (57.1%) of the seven patients who were incompatible with secondary prophylaxis. During clinical follow-up, two patients underwent mitral and aortic valve replacement. No mortality was observed in the patients. Conclusion: Despite improving socio-economic conditions in Turkiye in recent years, acute rheumatic fever remains a significant health problem for the Eastern Black Sea Region.

The issue of reducing the incidence of acute rheumatic fever (ARF) has mainly been addressed, particularly in developed countries, due to a better understanding of the disease's etiology and the introduction of penicillin. However, recent outbreaks in various countries have reignited interest in this problem. In response to statistical data, the World Health Organization has adopted a resolution to enhance global and national efforts to prevent and combat ARF and rheumatic heart disease. ARF is a systemic inflammatory disease that often results from pharyngitis caused by group A streptococci. It remains one of the leading causes of heart disease in developing nations. A significant factor in the disease's development is the interplay between the source of infection, the type of microbe, and the individual's susceptibility to it. Key clinical manifestations include carditis, migratory polyarthritis, chorea, erythema marginatum, and subcutaneous nodules. Diagnosis of rheumatic fever relies on the Jones criteria, which were first developed in 1944 and later revised twice by the American Heart Association (AHA) in 1992 and 2015. This article examines aspects of ARF diagnostics, specifically evaluating the updated Jones criteria proposed by the AHA in May 2015. Emphasis is placed on disease prevention, particularly secondary prevention, which aims to prevent recurrent episodes. An analysis of materials published from 2014 to 2024 is included, highlighting significant advancements in scientific research. This analysis utilized reputable resources, including PubMed and ResearchGate, which are extensive knowledge bases in the fields of medicine and science. Keywords: Rheumatic fever, Jones criteria, diagnostics, prevention, children.

To describe the clinical profile of acute rheumatic fever (ARF) presentations to paediatric cardiology tertiary services in Western Australia (WA). A retrospective clinical audit of individuals with confirmed ARF referred to the only paediatric tertiary cardiac service in WA (1 January 1987 to 31 December 2020). Comparisons between inpatient, outpatient, remote and non-remote groups were assessed. Four hundred seventy-one episodes of ARF in 457 individuals (235 male; median age = 8 years) met clinical criteria. The majority were Aboriginal and Torres Strait Islander children (91.2%), with 62.1% living in remote areas. The number of ARF and rheumatic heart disease (RHD) diagnoses per year increased from 1987 to 2017 with notable peaks in 2013 and 2017. The average annual incidence of tertiary-referred ARF in WA of 4-15-year-olds from 1987 to 2020 was 4.96 per 100 000. ARF features included carditis (59.9%), chorea (31%), polyarthritis (30%) and polyarthralgia (24.2%). RHD was evident in 61.8% of cases and predominantly manifested as mitral regurgitation (55.7%). Thirty-four children (7.4%) with severe RHD underwent valvular surgery. 12% had at least one recurrent ARF episode. Remote individuals had more than double the rate of recurrence compared to non-remote individuals (P = 0.0058). Compared to non-remote episodes, remote presentations had less polyarthritis (P = 0.0022) but greater proportions of raised ESR (P = 0.01), ASOT titres (P = 0.0073), erythema marginatum (P = 0.0218) and severe RHD (P = 0.0133). The high proportion of Aboriginal and Torres Strait Islander Australians affected by ARF/RHD in WA reflects the significant burden of disease within this population. Children from remote communities were more likely to present with concurrent severe RHD. Our study reinforces the persisting need to improve primary and secondary ARF initiatives in rural and remote communities.

Hereditary angioedema with normal C1-inhibitor: Clinical and genetic characterization of 15 Portuguese unrelated families

Background: Acute rheumatic fever is an autoimmune disorder that develops commonly few weeks following a group A beta hemolytic Streptococcal tonsillopharyngitis. The autoimmune response can affect joints, skin, heart, and the brain. One of the important serious consequences of acute rheumatic fever that have to be prevented is the the long-term heart disease resulting from valves damage which may occur after one severe illness or following multiple recurrence of the illness. The diagnosis of acute rheumatic fever is generally based on the presence of either two Major diagnostic criteria (multiple joint arthritis, carditis, erythema marginatum, chorea, and subcutaneous nodules) or one Major diagnostic criteria and two Minor criteria (Fever, arthralgia, first degree heart block, elevated inflammatory markers [ESR and C-reactive protein]) plus appropriate evidence of preceding streptococcal infection. The association of acute rheumatic fever with vitamin B12 deficiency-induced neutropenia has not been reported previously in the medical literature. Patients and methods:The case of a ten-year old girl who developed acute febrile illness associated with arthralgia, arthritis, skin rash and neutropenia is described. Results: The patient developed fever and arthritis of one elbow (Painful and swollen) followed shortly by the development of macular rash consisting of pink rings mostly on the extensor surfaces and arthritis of the second elbow. The patient had a previous febrile illness associated with sore throat which occurred during the previous 2 to 3 weeks. ASO titer was 150 iu/ml and CRP was positive 300 mg/dL. The patient had a high ESR, leukopenia, and blood film showed severe neutropenia, poikilocytosis and, frequent eliptocytosis. Serum vitamin B12 level was low, 152 pg/mL. Echocardiography showed low mitral valve prolapse. Conclusion: The novel association of acute rheumatic fever with vitamin B12 deficiency-induced neutropenia is reported.

IntroductionSydenham’s chorea (SC) is the most common form of acquired chorea in childhood, it is considered a neurological complication of streptococcal pharyngitis. In this study, we aimed to determine the clinical pattern, association of Sydenham’s chorea with other manifestations of acute rheumatic fever, and the laboratory findings of Sydenham’s chorea among Sudanese patients.MethodsA prospective cross-sectional study involving fifty patients of various ages diagnosed with Sydenham’s chorea and followed up at The National Center for Neurological Sciences from January 2017 to November 2019. Data were obtained after patients’ consent through personal interviews or personal review of patients’ records via a structured questionnaire composed of demographic data, symptoms, co-morbid illness, risk factors, physical examination, and related investigations.ResultsAbout 50 patient was enrolled in this study with a median age of 13.7 years. Females were (n=35) (70%) and (30%) (n=15) s were males. Generalized chorea was seen in 33 (66%) and hemichorea in 17 (34%) patients. Weakness (38%) and hypotonia (46%) were common, such as behavior change (44%), dysarthria (70%), gait change (18%), and deterioration of handwriting (12%). Arthritis occurred in (36%), carditis in 30 (60%), both arthritis and carditis in 18 (36%), and pure chorea in 14 (28%). Erythema marginatum and subcutaneous nodules were not observed in our patients. Only 13 patients (26%) gave a history of pharyngitis.ConclusionSydenham chorea is more common in young female Sudanese, with a familial predominance and a tendency towards mitral valve disease. All pediatric Patients with chorea should be screened for Sydenham’s chorea.

Figurate annulare erythemas.

Liver manifestations of tick-borne diseases.

Acute Rheumatic fever (ARF) is the most common cause of acquired heart disease in the developing world. It is an immunological reaction to group A beta-hemolytic streptococcus Pyogenes. It occurs 2-4 weeks after group A streptococcal pharyngitis due to the development of antistreptococcal antibodies that cross-react with antigens in various tissues of the body, especially cardiac and basal ganglia neuronal antigens. Clinical features of acute rheumatic fever include fever, joint pain, myocarditis, nodules in subcutaneous tissue, Erythema Marginatum, and chorea. Patients with immunodeficiency like mucocutaneous candidiasis are especially prone to recurrent mucocutaneous infections (tonsillopharyngitis, thrush, skin abscess), which can predispose to acute rheumatic fever and subsequent development of rheumatic heart disease over the years. Keywords: Acute rheumatic fever, chronic mucocutaneous syndrome, skin rash,

Introduction: The incidence of rheumatic fever is decreasing day by day in Bangladesh. Recent advances in the diagnosis and management of Rheumatic fever are demanding a review of the cause of Rheumatic fever. The illness may present itself in several ways, such as carditis, arthritis, chorea, subcutaneous nodules, and the recognizable erythema marginatum rash [2]. Aim of the Study: The aim of the study was to observe the rate of sore throat in patients with rheumatic fever. Methods: This retrospective study was carried out at the National Centre for control of Rheumatic fever and heart disease, Sher-c-Bangla Nagar, Dhaka. The study period was 11 months, starting from January 2019 to November 2019. The study was performed on recorded data of 300 patients with Rheumatic fever. Result: The present study showed that 30 (10%) patients out of 300 came with an acute sore throat, 216 (72%) patients with a history of sore throat, and 54 (18%) patients with no history of sore throat either acute or past. Rheumatic fever is undoubtedly a morbid one but can be prevented at its beginning by primary prophylactic measures. Conclusion: Rheumatic fever is a disease, which usually follows streptococcal infection of the throat. The study's underlying presumptions appear to overstate rather than understate the dangers of non-antibiotic sore throat treatment. Low socio-economic status, poverty, illiteracy; overcrowding, low levels of nutrition and non-immunization status were the predisposing factors in the causation of rheumatic fever.

Hereditary angioedema is associated with an increased risk of venous thromboembolism

Acute rheumatic fever (ARF) is considered as a disorder of children, and attacks in adults are usually a recurrence of disease acquired in the child’s life. Although the incidence of ARF in children has a decreasing trend in developed countries, resurgent and sporadic epidemics still occur in adults. The first attacks of ARF in adult patients without a childhood history can lead to a diagnostic dilemma.A medical record review in adults at least 18 years of age with an arthralgia complaint fulfilling 2015 revised Jones criteria was performed from January 1, 2000 to December 31, 2019.Eleven ARF patients were identified, including 8 with initial attacks (6 females aged 26–42 years, 33.9 ± 5.3) and 3 pre-existing valvular heart disease with recurrent attacks (2 females aged 38–52 years, 45.0 ± 7.0). In addition to febrile pharyngitis and migratory polyarthritis in initial attacks, pericarditis was encountered in 1, valvulitis in 2, prolong PR interval in 3 and skin involvement in 2 patients with erythema marginatum and IgA vasculitis. All responded to antibiotics and nonsteroidal anti-inflammatory drugs therapy with normalized clinical and laboratory abnormalities, no new-onset carditis, and no recurrent disease during a long-term follow-up (3.8–19.8 years, 12.7 ± 5.4).A sporadic occurrence of adult ARF is observed in southern Taiwan. This disease should be considered by physicians for the differential diagnosis of febrile pharyngitis with arthritis and/or carditis in adults, even in areas with a low incidence of ARF.

Angioedema Due to Acquired Deficiency of C1-Inhibitor: A Cohort Study in Spain and a Comparison With Other Series

BackgroundErythema marginatum is an uncommon presentation in children with acute rheumatic fever and it is one of the major criteria needed to make a diagnosis. It is seen in less than 10% of cases. It is also reported to be difficult to detect in black-skinned children. This is the first and only patient to present with the above since the inception of the unit about 14 years ago and also the first to be reported in Nigeria as far as the authors are aware, after a careful literature search; hence, we report this case based on the rarity of this symptom of acute rheumatic fever.Case presentationThis is a case report of O.E, a 12-year-old Nigerian girl who presented with features of acute rheumatic fever, and these features included the rare manifestation of erythema marginatum. She presented with generalized skin eruptions on the trunk and extremities, sparing the face, migratory polyarthritis, features of congestive heart failure and high grade continuous fever. The skin lesions consisted of papules, patches, plaques and polycycles with a reticular pattern having serpiginous and raised borders. Diagnostic investigations revealed elevated erythrocyte sedimentation rate of 83mm/h, anti-streptolysin O titer of 2020IU/L and echocardiography which showed thickened mitral valves with grade II mitral regurgitation and a mild pulmonary artery hypertension. The patient was treated with anti-inflammatory and anti-failure drugs and commenced secondary prophylaxis with benzathine penicillin. Skin eruptions resolved within 3 weeks of management and are currently on follow up.ConclusionsWe present the above to increase awareness on the possibility of acute rheumatic fever presenting with erythema marginatum in our region, to encourage early diagnosis of acute rheumatic fever to reduce morbidity and mortality from its sequel, rheumatic heart disease.

Rheumatic fever in the children's hospital of Rabat, about 116 cases (2014–2016)

Figurate erythemas (FE) represent an etiopathophysiologically heterogeneous group of diseases defined by their characteristic annular erythematous skin lesions. Diagnosis is made primarily by clinical examination together with histological findings; often it is a diagnosis made by exclusion. While some authors discuss FE as clinical reaction pattern rather than distinct clinical entities, others identify four classic FE: erythema annulare centrifugum, erythema gyratum repens, erythema migrans and erythema marginatum. The differential diagnoses of FE are numerous and often challenging. We therefore present a potential diagnostic algorithm for FE that discriminates the differentials according to their temporal evolution and the clinical/histological phenotype of the various subtypes. Since some FE may present with an underlying malignancy, diligent clinicians are needed when dealing with those entities.

BackgroundDespite a notable decrease in acute rheumatic fever (ARF) incidence in the past few decades, there are still cases in our setting. Sydenham chorea (SC) may be the initial manifestation for this condition in childhood in a significant proportion of children. We report two cases of choreoathetosis in children as the first manifestation of ARF.Case presentationA previously healthy 8-year-old boy presented with right hemichorea with a predominance in the brachial region, orofacial dyskinesias and speech difficulties for the past 2 weeks. The only medical history of interest was a common catarrhal illness 3 weeks before and nonspecific bilateral tenosynovitis in both feet since a year prior. A brain computerized tomography was normal and the echocardiogram showed mild mitral and aortic regurgitation, meeting ARF criteria. He demonstrated clinical improvement with treatment based on prednisone and carbamazepine.The second patient was a 10-year-old girl with choreic movements of the right half of the body and repetitive right eye closure of 1 week duration. She had symptoms of fever and rash the previous week and pharyngitis that resolved without antibiotic 2 months before. Blood tests revealed elevated C reactive protein (12 mg/dl) and erythrocyte sedimentation rate (96 mm/h). Brain magnetic resonance was normal and echocardiogram showed left ventricle dilation and mild mitral regurgitation, leading to the diagnosis of ARF. Due to neurological involvement, she received corticosteroids and intravenous immunoglobulin treatment, with worsening of neurological symptoms that required valproic acid with remission of the hemichorea. In addition skin lessions compatible with erythema marginatum appeared on the upper limbs.ConclusionsSC should be the main diagnostic consideration in cases of hemichorea with normal neuroimaging in children. The cases reported highlight the need to maintain a high index of suspicion even in settings where incidende of ARF is low and the need to perform cardiological investigations in all patients with suspected SC, due to the possibility of subclinical valve lesions. Good adherence to secondary prophylaxis is crucial to avoid chorea relapses and worsening valve disease.

BackgroundHereditary angioedema (HAE) is a rare but life-threatening condition. HAE types I and II (HAE-1/2) result from C1-inhibitor (C1–INH) deficiency. However, recent genetic analysis has established a new type of HAE with normal C1–INH (HAEnC1-INH). The mutations of factor XII, plasminogen, angiopoietin 1, and kininogen 1 genes may be the cause of HAEnC1-INH. Nevertheless, other causative molecules (HAE-unknown) may be involved. The Japanese therapeutic environment for HAE has been improving owing to the self-subcutaneous injection of icatibant, which was approved for the treatment of acute attack and enables early therapy. Erythema marginatum (EM) is a visible prodromal symptom which occasionally occurs prior to an angioedema attack; hence, recognizing the risk of an acute attack is important for early treatment. However, the detailed characteristics of EM remain unclear. In this study, we first investigated the clinical manifestations of EM in Japanese patients with HAE. MethodsA 20-point survey was developed and distributed to 40 physicians to gather clinical data on EM from patients with HAE. ResultsData on 68 patients with HAE (58 patients with HAE-1/2 and 10 patients with HAE-unknown) were collected. Of the patients with HAE-1/2, 53.4% experienced EM, whereas 43.1% did not. The forearm was the most frequent area of EM (64.5%), followed by the abdomen (29.0%) and upper arm and precordium (19.3%). Of the HAE-1/2 patients with EM, 41.9% always had angioedema following EM, while 29.0% always had colocalization of EM with angioedema. Moreover, 3.2% showed a correlation between the awareness of EM and severity of an angioedema attack. In 60.9% of HAE-1/2 patients with EM, the interval between the awareness of EM and appearance of angioedema was <3 h. Of the patients with HAE-unknown, 30.0% also experienced EM. ConclusionWe confirmed that more than one-half of Japanese patients with HAE-1/2 and one-third of those with HAE-unknown develop EM as the prodromal symptom of an angioedema attack. Physicians should communicate the significance of EM to patients with HAE to prepare them for possible imminent attacks.

Hereditary angioedema (HAE) with C1-inhibitor deficiency belongs to bradykinin-mediated angioedemas. It is characterized by recurrent subcutaneous and/or submucosal swelling episodes (HAE attacks) and erythema marginatum skin rash as a pre-attack (prodromal) phase. HAE attacks were shown to be accompanied by peripheral blood neutrophilia. We aimed to find molecular mechanisms that may explain the distinct role of neutrophil granulocytes in HAE. Plasma levels of blood cells and factors related to neutrophil activation (cytokines, chemokines, chemotactic factors, enzymes, and neutrophil extracellular trap) were measured in plasma samples obtained from patients during symptom-free periods (n = 77), during prodromal phase (n = 8) and attacks (n = 14), during a spontaneously resolved attack (n = 1), and in healthy controls (n = 79). Higher counts of white blood cells, lymphocytes, and neutrophil granulocytes were found in symptom-free patients compared with controls; these cell counts were elevated further during HAE attacks. The level of chemokine (C–C motif) ligand 5, monocyte chemoattractant protein-1, and myeloperoxidase were also higher in the symptom-free patients than in the controls. Levels of monocyte chemoattractant protein-1, leukotriene B4, neutrophil elastase, and myeloperoxidase were elevated during attacks. During erythema marginatum, white blood cells and monocyte count and levels of interleukin 8 were elevated compared with symptom-free period. Similar changes were detected during the attack follow-up. We conclude that the activation of NGs in symptom-free periods and a further increase observed during attacks suggests that NGs may be involved in the pathomechanism of HAE with C1-INH deficiency.

Acute rheumatic fever, Rheumatic carditis, Sydenham’s chorea, Erythema marginatum, Arthritis, Differential diagnosis The revision of the Jones criteria by the American Heart Association allowed the identification and diagnosis of a greater number of cases of Rheumatic Disease (RD); however, the higher incidence of RD is associated with "pathomorphic" phenotypic pictures, making the diagnosis more difficult. Chorea, carditis, arthritis, marginatum erythema are the major criteria for the diagnosis of RD and can represent its clinical onset, but likewise, due to the variety of their associations, they open a range of different differential diagnoses. Through the critical reasoning applied to some clinical cases, these major “criteria” of RD have been discussed to reduce the difficulties of the differential diagnosis.