The pathophysiology of mitral stenosis
Neeraj Parakh, Ravi S. Math, Vivek Chaturvedi in Mitral Stenosis, 2018
Rheumatic fever is an inflammatory disease that usually affects children and young adults aged between 1 and 19 years. It is a prototype of a postinfectious autoimmune disease caused by untreated oropharyngeal infection by group A β-hemolytic streptococcus (GAS).27 Only 0.3%–3% of individuals affected by streptococcus develop rheumatic fever. Approximately two-thirds of rheumatic fever patients progress to rheumatic carditis and only a portion of them suffer from severe cardiac manifestations.28,29 The exact mechanism leading to chronic inflammation of the heart valves is still unclear. However, studies have shown evidence of an epitope-spreading phenomenon (molecular mimicry) and the development of a long-lasting cellular immune response that are responsible for the cardiovascular damage.27
Streptococcus and Streptococcal Toxins
Dongyou Liu in Handbook of Foodborne Diseases, 2018
Depending on the species affecting the host, streptococcal infections cause a wide range of clinical symptoms. Acute rheumatic fever (ARF) is an autoimmune illness which when unrecognized or inadequately treated may cause rheumatic heart disease (RHD), a serious and sometimes fatal condition [18]. ARF has a 2–3 week period of latency before symptom onset. Arthritis occurs in about 35%–66% of patients, primarily affects large joints, and is found to be migratory [19]. Carditis arises in 50%–70% patient and is the major reason for mortality [20]. Streptococcal pharyngitis is a common infection, affecting the young age that spreads by respiratory secretions and presents after a 2- to 4-day incubation period with fever, inflamed pharynx, sore throat, tonsillar enlargement, and tender regional lymphadenopathy [21]. Scarlet fever denotes a clinical condition that is characterized by the presence of a rash along with inflammation of pharynx [22].
Fever in Diseases of the Cardiovascular System
Benedict Isaac, Serge Kernbaum, Michael Burke in Unexplained Fever, 2019
The clinical picture includes fever, arthralgia, or polyarthritis, tachycardia, disproportionate to the degree of fever and persisting during sleep, murmurs, conduction disturbances and cardiac failure. Additional “major” manifestations, viz., chorea, erythema marginatum, and subcutaneous nodules, are exceedingly rare. Some patients will present as a FUO,76,77 although most cases will have migratory polyarthritis as well. Any joint may be involved, the larger lower limbs joints being affected most frequently. Arthritis tends to be of longer duration and more severe in the adult.76 Carditis usually occurs within the first 3 weeks of the attack and is virtually always manifested by a regurgitant murmur (mitral and/or aortic) and frequently by an apical middiastolic (Carey—Coombs) murmur. Pericardial friction rub and signs of cardiac failure may also be present. Carditis is most frequent in the younger age group and is less common in adults.
Association of IL17 and IL23R gene polymorphisms with rheumatic heart disease in South Indian population
Published in Immunological Investigations, 2018
Maheshkumar Poomarimuthu, Sivakumar Elango, Pravin Raj Solomon, Sambath Soundrapandian, Jayalakshmi Mariakuttikan
Rheumatic heart disease (RHD) is an autoimmune disease caused by abnormal immune response against group A β-hemolytic streptococci (GAS) infection aggravated by rheumatic fever (RF). The autoimmune reaction in RHD occurs via molecular mimicry between the GAS antigens and human cardiac proteins mediated by both cross-reactive antibodies and T cells (Carapetis et al., 2016; Cunningham, 2014; Mastrandrea, 2015). Global Burden of Disease study estimates that there are 33 million RHD patients in the world and it accounts for 275,000 annual deaths. RHD is known to cause the highest cardiovascular disease-related loss of disability-adjusted-life-years among children and young adults (10–14 years) in low- and middle-income countries (Abubakar et al., 2015). Various studies reported that there are 2–4 million RHD patients in India (Kumar and Tandon, 2013). Rheumatic carditis is an inflammatory condition characterized by cellular infiltration (mainly CD4+ T cells) of endocardium and valve tissues which results in progressive valvular damage that leads to RHD (Guilherme and Kalil, 2010; Guilherme et al., 2001; Roberts et al., 2001). Recent studies indicated the involvement of Th17 cells in the pathogenesis of RHD (Bas et al., 2014; Wen et al., 2015).
DRESS syndrome: a detailed insight
Published in Hospital Practice, 2018
Sapan Kumar Behera, Saibal Das, Alphienes Stanley Xavier, Sandhiya Selvarajan
A mild cough in initial stage can progress to pneumonitis and acute respiratory distress syndrome in the later stage [117]. Two cases of pulmonary involvement in DRESS syndrome has been reported due to antiretroviral therapy [131] and homeopathic medicine [132]. Chylous ascites is also reported in DRESS syndrome [133]. Kumari et al. have reported muscle involvement in this syndrome which may range from mild elevation in creatinine kinase level to myositis in moderate form and rhabdomyolysis in severe cases [117]. DRESS syndrome presenting as leukoencephalopathy have been reported in a child taking phenytoin and phenobarbitone [134]. Cardiac involvement in this syndrome is reported by Thongsri et al. [135], which can present as pericarditis in the mild form, carditis in moderate and congestive heart failure in severe form [117]. There are also reports of myocarditis [136] and fulminant myocarditis [137] in patients with DRESS syndrome. This syndrome can also present as pancreatitis involving elevation of the pancreatic enzyme [138]. A case of encephalopathy with stroke has also been reported by Vidula et al. [139]. Cicatrizing conjunctivitis involving all palpebral conjunctival surfaces with evidence of subepithelial fibrosis in a patient with DRESS syndrome receiving lamotrigine and levetiracetam has been reported by Bohm et al. [140]. Table 4 shows some atypical presentations of DRESS syndrome [78–82,110,116,129,140].
Henoch–Schonlein purpura, post-streptococcal glomerulonephritis and acute rheumatic carditis after Group A β-haemolytic streptococcal infection
Published in Paediatrics and International Child Health, 2018
Seçil Arslansoyu Çamlar, Alper Soylu, İpek Akil, Mehtat Ünlü, Şenol Coşkun, Pelin Ertan, Salih Kavukçu
Cardiac involvement is an infrequent but often life-threatening complication of HSP [4]. Myocarditis, elevated cardiac enzymes and congestive cardiac failure and/or arrhythmias may indicate vasculitic cardiac involvement [4,9]. In one case, cytotoxic immunosuppression in addition to corticosteroid therapy was required to prevent a deleterious outcome [4]. It is difficult but important to distinguish between vasculitic cardiac involvement owing to HSP and rheumatic fever. Rheumatic carditis is characterised by pancarditis, and endocarditis (valvulitis) is a universal finding, while pericarditis and myocarditis are variable [10]. This patient had exudative tonsillitis 3 weeks before presentation and she had a highly elevated ASO titre along with isolation of GABHS from the throat, and had mitral and aortic insufficiency (valvulitis). Although the ECG showed a normal PR interval, echocardiography demonstrated mitral and aortic valve insufficiency.
Related Knowledge Centers
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