The pathophysiology of mitral stenosis
Neeraj Parakh, Ravi S. Math, Vivek Chaturvedi in Mitral Stenosis, 2018
Rheumatic fever is diagnosed by the revised Jones criteria.30 The common clinical findings27,28 include: 1) carditis, the most severe presentation; 2) migratory polyarthritis, seen in almost 100% of individuals, with more severity in younger adults than in teenagers and children. Migratory polyarthritis occurs for between a few days and a few weeks and resolves completely; 3) Sydenham chorea, involuntary, purposeless, jerky movements of the hands and arms, shoulders, feet, legs, face, and trunk, along with hypotonia and weakness, which disappear during sleep; 4) subcutaneous nodules, commonly noticed over the occiput, elbows, knees, ankles, and Achilles tendons. These are firm, painless, and freely mobile with size varying from 0.5–2 cm. On microscopic examination, these nodules show central fibrinoid degeneration of the collagen surrounded by chronic mononuclear inflammatory cell infiltrate; 5) erythema marginatum, less commonly found, these present as a pink, non-pruritic skin rash present over the upper parts of the arms or trunk but not on the face. The rash generally occurs early or late in the course of the disease and extends centrifugally with a clear center.
Autoimmune disorders that can be mistaken for viral illness
Avindra Nath, Joseph R. Berger in Clinical Neurovirology, 2020
Autoimmune encephalitis may rarely occur after viral infections of the CNS. Measles infection, for instance, may trigger acute disseminated encephalomyelitis (ADEM) in 1 per 1000 cases [3], as can numerous other infections [4]. More recently, herpes simplex encephalitis (HSE) has been shown to rarely cause secondary autoimmune encephalitis [5]. These cases may represent defects in self-tolerance triggered by severe CNS viral infection and the associated CNS immune responses needed to control the infections. Additionally, bacterial infection can cause the immune system to attack the nervous system through molecular mimicry. For instance, many cases of Guillain–Barré syndrome (GBS) are post-infectious with Campylobacter jejuni infection being particularly associated with certain forms of GBS, and Sydenham Chorea can occur following streptococcal infection.
The Scientific Revolution
Scott M. Jackson in Skin Disease and the History of Dermatology, 2023
The Sydenham chorea of rheumatic fever is named after him. He described scarlatina (scarlet fever) and how it differed from measles, and he wrote extensively on syphilis and controversially on smallpox. Other noteworthy contributions of Sydenham include his antithetical cooling therapy for smallpox and other fevers, the naming of pertussis for whooping cough, the prescription of Peruvian bark (quinine) for malaria, the distinction between gout and rheumatism, and the promotion of the use of a new preparation of laudanum for a variety of conditions. On the latter, he famously opined, “Among the remedies which it has pleased almighty God to give man to relieve his sufferings, none is so universal and so efficacious as opium.”22
Diagnosing rheumatic heart disease: where are we now and what are the challenges?
Published in Expert Review of Cardiovascular Therapy, 2021
Sarah R. de Loizaga, Andrea Z. Beaton, Bruno R. Nascimento, Frederico Vargas Botinha Macedo, Breno Camargos Mucelli Spolaor, Lucas Bretas de Pádua, Thomas Felipe Silva Ribeiro, Guilherme Catizani Faria Oliveira, Lucas Rocha Oliveira, Luís Felipe Rezende de Almeida, Thomás Diniz Moura, Talisson Taglialegna de Barros, Craig Sable, Maria Carmo Pereira Nunes
The international gold standard for the diagnosis of ARF still remains the Jones criteria. First established in 1944, the Jones criteria have undergone several revisions with the most recent occurring in 2015 [15]. These latest guidelines retain the basic diagnostic tenants, while adapting criteria to make them more globally relevant. Hence, aside from cases of Sydenham chorea and fulminant carditis, documentation of preceding GAS infection must be established either via positive throat culture or confirmatory serology (anti-streptolysin O or anti-streptococcal DNAse B). Of note, within endemic regions in LMICs, obtaining evidence of preceding streptococcal infection may be challenging and less strictly applied, but as to not compromise identification of those at risk for developing RHD [16]. The patient must then exhibit either two major criteria OR one major and a minimum of two minor criteria (Figure 1). The 2015 revision distinguishes certain criteria for low-risk populations (ARF incidence ≤ 2 per 100,000 school-aged children or all-age RHD prevalence of ≤ 1 per 1,000 population year) vs moderate-and high-risk populations, favoring specificity in low risk populations and sensitivity in moderate- and high-risk populations. These distinctions included broadening the joint criteria for moderate- to high-risk populations. Finally, the revised criteria recommend echocardiography for all suspected cases of ARF.
Chorea revealing systemic lupus erythematosus in a 13-year old boy: A case report and short review of the literature
Published in International Reviews of Immunology, 2018
E. Athanasopoulos, I. Kalaitzidou, G. Vlachaki, S. Stefanaki, A. Tzagkaraki, G. Niotakis, I. Tritou, F. Ladomenou
Chorea, our patient's presenting symptom is a well-recognized but rare manifestation of SLE. It is characterized by non patterned, involuntary and hyperkinetic movements with dancelike characteristics, may involve any portion of the body and has a variety in frequency and magnitude.12,17 Chorea can be seen in the context of various diseases and its differential diagnosis includes cerebrovascular accidents, drug intoxication, hyperthyroidism, Huntington's disease, Sydenham's chorea, and collagen vascular diseases like SLE (Table 1).18 The most frequent cause of chorea in the pediatric population is Sydenham chorea, which is a chorea in the frame of streptococcal infection, as the antistreptococcal antibodies cross react with the antigens of basal gangial, but it is rather rare in the developed countries.19 Sydenham chorea often occurs 1 to 2 months after the infection, most frequently is bilateral and coexists with hypotonia, facial dystonia, dysarthria and motor impersistence.17 Sydenham chorea typically improves spontaneously, but in some cases chorea remains. Other entities associated with chorea in children are the pediatric autoimmune neuropsychiatric disorder associated with streptococcus (PANDAS) and pediatric acute-onset neuropsychiatric syndrome (PANS). The latter includes anxiety, emotional lability, irritability, behavioural regression, deterioration in school performance, sensor/motor abnormalities and somatic symptoms, but it remains a controversial for the scientific community and further studies are needed to reveal the underline mechanisms.17–20
Pharmacotherapy for Sydenham’s chorea: where are we and where do we need to be?
Published in Expert Opinion on Pharmacotherapy, 2023
Roberta Bovenzi, Matteo Conti, Tommaso Schirinzi
For this review, an electronic exploration of the PubMed database using the Systematic Review Tool/Rayyan was performed [27]. The final reference list was generated after selecting all the manuscripts with key information regarding the selected topics: ‘Sydenham chorea’ plus ‘Treatment’ or ‘Therapy’ or ‘Pharmacotherapy,’ ‘Chorea gravidarum’ plus ‘Treatment’ or ‘Therapy’ or ‘Pharmacotherapy.’ A search was also conducted on ClinicalTrials.gov and the International Clinical Trials Registry Platform (ICTRP), with no other relevant studies found.
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