Rheumatic Fever-Related Antigen

Alternative Names

  • Rheumatic Fever, Acute, Susceptibility to
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WHO-ICD-10 version:2010

Diseases of the circulatory system

OMIM Number

268240

Mode of Inheritance

? Autosomal recessive

Description

Rheumatic fever (RF) is an inflammatory disease that affects many systems in the body (multisystem disease). It is a delayed outcome of untreated group A streptococcal pharyngitis. The major organs that can be involved in RF are the heart (the connective tissues of the heart), joints, skin, and vessels. RF occurs most frequently in the age between five to 20 years. In developing countries, rheumatic fever is endemic and remains one the major causes of acquired cardiovascular disease because of lacking the suitable antibiotics for group A step. pharyngitis. Heart disease associated with rheumatic fever accounts for 25-50% of all cardiac admissions worldwide. Also, RF is a major cause of mortality among individuals under 50 years of age. Arthritis is present in about 75% of patients. RF has higher prevalence in developing countries. The annual incidence rate of RF can reach up to 30 million new cases. The incidence rate in developing countries is estimated to be 100-200 times greater than that observed in industrialized countries. Treatment depends on the symptoms associated with RF. Aspirin is the choice to treat fever and joint pain/swelling. Antibiotics should be given to the children without carditis at least up to adulthood, whereas patients with carditis must complete taking the antibiotics forever. Also, prophylactic antibiotics must be given to prevent further infections.

Molecular Genetics

To date, the pathogenic mechanisms of developing RF remain unclear. However, it is thought that an abnormal humoral and cellular immune response occurs in persons with genetic predisposition to RF. The abnormal immune response occurs because of the antigenic mimicry between streptococcal antigens and human tissues, such as heart valves, and cartilage. Several populations show the association of RF with class-II human leukocyte antigen (HLA). For instance, black patients reveal the association with class-II HLA DR2, whereas DR4 is found in white patients. It is also suggested that elevated immune-complex levels in blood samples of patients with acute RF are associated with HLA-B5. In addition, studying the genetic control and mode of inheritance of RF are established by genetic markers such as HLA, blood groups, and immunoglobulin allotypes. Genetic predisposition to RF was proved in the mid 1990s to be dependent upon the presence of B-cell alloantigen (D8/17) in 90-100% patients.

Epidemiology in the Arab World

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Other Reports

Egypt

Majeed et al. (1981) described a four year prospective clinical study of the initial attack of acute rheumatic fever in 210 children. Pain in the joints was found to be the major characteristic that was caused in 79% of patients by arthritis and 15% by arthralgia. The mortality rate was found to be 0.5%. Through this study, Majeed et al. (1981) presented the mild nature of acute rheumatic fever in Kuwait compared to the aggressive nature of the disorder portrayed in the neighboring and developing countries. Later, Majeed et al. (1984) reported a prospective study for a period of over seven years, which included the clinical profile of acute rheumatic fever for 53 children below the age of five years. Pain in the joints was found to be the main characteristic revealing arthritis in 81% and arthralgia in 15%. Patients demonstrated congestive heart failure (4%) and carditis (42%). Pericarditis and congestive heart failure were found in high incidence rates, 6% and 15%, respectively, and a mortality rate of 2%. Furthermore, the incidence of erythema marginatum was found to be 2%, whereas no evidence of chorea and subcutaneous nodules was noticed. Majeed et al. (1984) compared the results of this study with previous studies revealing analogous incidence of arthritis and carditis along with a somewhat more aggressive nature of carditis in patients under the age of five years, while controverting with the results of previous clinical profiles of acute rheumatic fever among the same age group. Nine years later, Majeed et al. (1993) studied the incidence of acute rheumatic fever in children aged 5-14 years for the period between 1984 to 1988 and established a mean annual incidence during the study period of 2.9/100,000 children. A fall in the incidence was noticed right through 1984 to 1988 from 3.7/100,000 to 2.5/100,000, respectively.

Kuwait

Majeed et al. (1981) described a four year prospective clinical study of the initial attack of acute rheumatic fever in 210 children. Pain in the joints was found to be the major characteristic that was caused in 79% of patients by arthritis and 15% by arthralgia. Furthermore, patients suffered from only chorea in 5%, only congestive heart failure in 1%, carditis in 46.2%, cardiomegaly in 10%, pericarditis in 1.4%, and congestive heart failure in 4.8%. The incidence was found to be 7.6% for chorea, 1% for erthema marginatum, and 0.5% of subcutaneous nodules. Meanwhile, the mortality rate was found to be 0.5%. Through this study, Majeed et al. (1981) presented the mild nature of acute rheumatic fever in Kuwait compared to the aggressive nature of the disorder portrayed in the neighboring and developing countries. Later, Majeed et al. (1984) reported a prospective study for a period of over seven years, which included the clinical profile of acute rheumatic fever for 53 children below the age of five years. Pain in the joints was found to be the main characteristic revealing arthritis in 81% and arthralgia in 15%. Patients demonstrated congestive heart failure (4%) and carditis (42%). Pericarditis and congestive heart failure were found in high incidence rates, 6% and 15%, respectively, and a mortality rate of 2%. Furthermore, the incidence of erythema marginatum was found to be 2%, whereas no evidence of chorea and subcutaneous nodules was noticed. Majeed et al. (1984) compared the results of this study with previous studies revealing analogous incidence of arthritis and carditis along with a somewhat more aggressive nature of carditis in patients under the age of five years, while controverting with the results of previous clinical profiles of acute rheumatic fever among the same age group. Nine years later, Majeed et al. (1993) studied the incidence of acute rheumatic fever in children aged 5-14 years for the period between 1984 to 1988 and established a mean annual incidence during the study period of 2.9/100,000 children. A fall in the incidence was noticed right through 1984 to 1988 from 3.7/100,000 to 2.5/100,000, respectively. During 1985, 27% of children with acute rheumatic fever were portrayed as recurrences and that percentage was declined to 11% in 1988.

Saudi Arabia

Abdallah et al., (2016) enrolled 124 Saudi patients with rheumatic heart disease (RHD) in a pilot study at pediatric cardiology clinics in Al Madina between 2013 and 2014.  The control consisted of 202 healthy Saudi participants.  The diagnosis was achieved in line with the modified Jones criteria at initial diagnosis and confirmed by echocardiography.  All samples were genotyped for MIF-173 (rs755622) and MIF-794 (rs5844572) polymorphisms.  The authors found that the age of onset was significantly higher for MIF-173C allele carriers than non-MIF-173C allele carriers in the group of RHD patients.  The mean ages of onset of carriers of C-allele and non-carriers were 9.2 ± 1.6 and 7.5 ± 2.4, respectively.  No significant association between the tetranucleotide variation and disease age of onset was found.  The authors concluded that variations in the MIF influenced disease severity and progression and recommended further studies in order to address and analyze the disease stage and patient age.

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