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group_2_presentation_2_-_juvenile_idiopathic_arthritis [2017/03/09 21:31]
gillr23
group_2_presentation_2_-_juvenile_idiopathic_arthritis [2018/01/25 15:18] (current)
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 Juvenile idiopathic arthritis (JIA), also known as juvenile rheumatoid arthritis (JRA) is a chronic form of arthritis that can be seen in children between the ages of 1-16 (Shiel, n.a). This disease refers to a group of conditions that pertain to joint inflammation (Figure 1). According to The Genetics Home Reference, “It is classified as an autoimmune disorder which means that the immune system malfunctions and attacks the body’s organs and tissues, in this case joints.” Through much research, it has been determined that there are seven types of JIA which are classified in accordance to their signs and symptoms, number of affected joints, results from medical tests and familial history. All seven types of JIA are chronic, thus individuals must develop coping methods that are long lasting and effective. The prevalence of the seven types of JIA are shown in Figure 2  (Genetics Home Reference, 2015).  ​ Juvenile idiopathic arthritis (JIA), also known as juvenile rheumatoid arthritis (JRA) is a chronic form of arthritis that can be seen in children between the ages of 1-16 (Shiel, n.a). This disease refers to a group of conditions that pertain to joint inflammation (Figure 1). According to The Genetics Home Reference, “It is classified as an autoimmune disorder which means that the immune system malfunctions and attacks the body’s organs and tissues, in this case joints.” Through much research, it has been determined that there are seven types of JIA which are classified in accordance to their signs and symptoms, number of affected joints, results from medical tests and familial history. All seven types of JIA are chronic, thus individuals must develop coping methods that are long lasting and effective. The prevalence of the seven types of JIA are shown in Figure 2  (Genetics Home Reference, 2015).  ​
  
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 **Figure 1:** This image illustrates a comparison between a normal joint, osteoarthritis and rheumatoid arthritis. Retrieved from: http://​www.onhealth.com/​content/​1/​rheumatoid_arthritis_ra **Figure 1:** This image illustrates a comparison between a normal joint, osteoarthritis and rheumatoid arthritis. Retrieved from: http://​www.onhealth.com/​content/​1/​rheumatoid_arthritis_ra
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 Undifferentiated arthritis is given to patients who have symptoms that are not explained by the descriptions of any of the above forms of JIA or who have fit the criteria for more than one of the given descriptions of JIA (Genetics Home Reference, 2015). ​ Undifferentiated arthritis is given to patients who have symptoms that are not explained by the descriptions of any of the above forms of JIA or who have fit the criteria for more than one of the given descriptions of JIA (Genetics Home Reference, 2015). ​
  
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 **Figure 2:** This graph shows the percentage at which each subtype of JIA is prevalent. Retrieved from: https://​warmsocks.files.wordpress.com/​2012/​08/​jiabreakdown.png **Figure 2:** This graph shows the percentage at which each subtype of JIA is prevalent. Retrieved from: https://​warmsocks.files.wordpress.com/​2012/​08/​jiabreakdown.png
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 Flares are known as a classification of symptoms referring to joint pain and inflammation,​ which are more prevalent in areas of the body highlighted in figure 3. Flares can last from time periods ranging from weeks to months. JIA patients tend to have periods of remission where these symptoms aren’t as prevalent as they would have been in the past. Following this grace period where pain is minimal, these symptoms can reappear, and this is known as a relapse. This notion of relapsing and remission varies from patient to patient and can also be a known trend with other symptoms as well (Shiel, n.a).  Flares are known as a classification of symptoms referring to joint pain and inflammation,​ which are more prevalent in areas of the body highlighted in figure 3. Flares can last from time periods ranging from weeks to months. JIA patients tend to have periods of remission where these symptoms aren’t as prevalent as they would have been in the past. Following this grace period where pain is minimal, these symptoms can reappear, and this is known as a relapse. This notion of relapsing and remission varies from patient to patient and can also be a known trend with other symptoms as well (Shiel, n.a). 
  
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 **Figure 3:** This shows the location of different body parts with high prevalence of body inflammation Retrieved from: http://​www.onhealth.com/​content/​1/​rheumatoid_arthritis_ra **Figure 3:** This shows the location of different body parts with high prevalence of body inflammation Retrieved from: http://​www.onhealth.com/​content/​1/​rheumatoid_arthritis_ra
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 This symptom is extremely helpful when diagnosing JIA, especially in the acute onset of disease. According to Miller, “the characteristic rheumatoid rash is an erythematous,​ salmon-pink,​ evanescent, usually circumscribed macular (although occasionally maculopapular) eruption involving the trunk, neck, velar aspect to the arms, inner aspect of the thighs, buttocks and face. It may last for only a few minutes, a few hours or several years.” Again, the degree to which the rash may last varies between a few minutes to many hours. It appears strongly during times of high fever or during warm baths. Many times, the rash experienced in patients who have JIA can be mistaken for erythema annulare or marginatum, however erythema annulare or marginatum do not spread to facial regions (Miller, 1994). ​ This symptom is extremely helpful when diagnosing JIA, especially in the acute onset of disease. According to Miller, “the characteristic rheumatoid rash is an erythematous,​ salmon-pink,​ evanescent, usually circumscribed macular (although occasionally maculopapular) eruption involving the trunk, neck, velar aspect to the arms, inner aspect of the thighs, buttocks and face. It may last for only a few minutes, a few hours or several years.” Again, the degree to which the rash may last varies between a few minutes to many hours. It appears strongly during times of high fever or during warm baths. Many times, the rash experienced in patients who have JIA can be mistaken for erythema annulare or marginatum, however erythema annulare or marginatum do not spread to facial regions (Miller, 1994). ​
  
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 **Figure 4:** This image is showing the rheumatoid rash when present on a child’s face.  Retrieved from: http://​www.findarthritistreatment.com/​wp-content/​uploads/​2013/​12/​Skin-rash.jpg **Figure 4:** This image is showing the rheumatoid rash when present on a child’s face.  Retrieved from: http://​www.findarthritistreatment.com/​wp-content/​uploads/​2013/​12/​Skin-rash.jpg
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 Nodules have been observed in approximately 10% of children who have been diagnosed with JIA and can appear subcutaneously,​ as shown in figure 5. Characteristics of nodules include variances in sizes (from a few millimeters to several centimetres in diameter), nontender texture, no attachment to overlying skin and therefore moving freely under the skin . Many times, they are found over the extensor tendon sheath of the hands, specifically over the metacarpophalangeal,​ proximal interphalangeal and distal interphalangeal joints . They are also found near the olecranon process of the elbow, over the anterior tibial surfaces and around the wrists and when fever is prevalent in the patient, nodules are found along the tendons of the erector spinae group and over the aponeurosis of the scalp (Miller, 1994). ​ Nodules have been observed in approximately 10% of children who have been diagnosed with JIA and can appear subcutaneously,​ as shown in figure 5. Characteristics of nodules include variances in sizes (from a few millimeters to several centimetres in diameter), nontender texture, no attachment to overlying skin and therefore moving freely under the skin . Many times, they are found over the extensor tendon sheath of the hands, specifically over the metacarpophalangeal,​ proximal interphalangeal and distal interphalangeal joints . They are also found near the olecranon process of the elbow, over the anterior tibial surfaces and around the wrists and when fever is prevalent in the patient, nodules are found along the tendons of the erector spinae group and over the aponeurosis of the scalp (Miller, 1994). ​
  
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 **Figure 5:** This image illustrates what nodules look like on the hands of a child. Retrieved from: http://​c.ymcdn.com/​sites/​www.aocd.org/​resource/​resmgr/​ddb/​rheumatoid_nodules_1_low.jpg **Figure 5:** This image illustrates what nodules look like on the hands of a child. Retrieved from: http://​c.ymcdn.com/​sites/​www.aocd.org/​resource/​resmgr/​ddb/​rheumatoid_nodules_1_low.jpg
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 Maternal smoking has also been observed in the onset of JIA. A mother who smokes while pregnant can induce some permanent immunological abnormality in the child which can later lead to development of arthritis in the child. ​ Smoking while pregnant may also result in a low birth weight which could in turn lead to an increase in the susceptibility to infection and JIA. Lastly, second-hand smoking leads to a contaminated environment in which the risk of JIA during the early years of that child’s life increases (Symmons, 2005). ​ Maternal smoking has also been observed in the onset of JIA. A mother who smokes while pregnant can induce some permanent immunological abnormality in the child which can later lead to development of arthritis in the child. ​ Smoking while pregnant may also result in a low birth weight which could in turn lead to an increase in the susceptibility to infection and JIA. Lastly, second-hand smoking leads to a contaminated environment in which the risk of JIA during the early years of that child’s life increases (Symmons, 2005). ​
  
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 **Figure 6:** This image illustrates where rheumatoid arthritis is present on a hand. Retrieved from: https://​www.epainassist.com/​images/​Juvenile-Arthritis.jpg **Figure 6:** This image illustrates where rheumatoid arthritis is present on a hand. Retrieved from: https://​www.epainassist.com/​images/​Juvenile-Arthritis.jpg
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 The incidence of JIA in North America and Europe ​ is 4 - 16 affected children out of a sub-population of 10000 children. 1 in 1000, or approximately 294000 children in the United States, are affected by the most common type of JIA in the United States, which is oligoarticular JIA. For reasons that continue to be studied, females seem to be affected with JIA more frequently than males. In the case of enthesitis-related JIA, males are affected more often than females. Furthermore,​ the incidence of JIA varies between different populations and ethnic groups (Genetics Home Reference, 2015). The incidence of JIA in North America and Europe ​ is 4 - 16 affected children out of a sub-population of 10000 children. 1 in 1000, or approximately 294000 children in the United States, are affected by the most common type of JIA in the United States, which is oligoarticular JIA. For reasons that continue to be studied, females seem to be affected with JIA more frequently than males. In the case of enthesitis-related JIA, males are affected more often than females. Furthermore,​ the incidence of JIA varies between different populations and ethnic groups (Genetics Home Reference, 2015).
  
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 **Figure 7:** This image shows the prevalence of the common forms of arthritis in the United States. Retrieved from: https://​rheumatoidarthritis.net/​wp-content/​uploads/​2013/​07/​prevalence_arthritis.png **Figure 7:** This image shows the prevalence of the common forms of arthritis in the United States. Retrieved from: https://​rheumatoidarthritis.net/​wp-content/​uploads/​2013/​07/​prevalence_arthritis.png
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 In addition to associations between numerous HLA variants and JIA, non-HLA polymorphisms have also shown to be linked to JIA. For example, genes such as PTPN22, tumor necrosis factor (TNF) alpha (TNFA), MIF, WISP3 and SLC11A6 have been associated with JIA  (Figure 9) (Prahalad, 2004; Rosen et al., 2003).  ​ In addition to associations between numerous HLA variants and JIA, non-HLA polymorphisms have also shown to be linked to JIA. For example, genes such as PTPN22, tumor necrosis factor (TNF) alpha (TNFA), MIF, WISP3 and SLC11A6 have been associated with JIA  (Figure 9) (Prahalad, 2004; Rosen et al., 2003).  ​
  
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 **Figure 9:**This figure shows non-HLA genetic genes associated with JIA that have been independently confirmed. Retrieved from (Prahalad and Glass, 2008) **Figure 9:**This figure shows non-HLA genetic genes associated with JIA that have been independently confirmed. Retrieved from (Prahalad and Glass, 2008)
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