Juvenile Rheumatoid Arthritis - Research Paper Example

Juvenile Rheumatoid Arthritis Introduction Juvenile rheumatoid arthritis (JRA), also referred to as juvenile idiopathic arthritis, is a terms that describes several forms of chronic arthritis that are common in children aged 16 years and below (Espinosa &Gottlieb, 303). JIA is a long term disease that is characterized by joint pain and swelling. The cause of JRA is not well known, but it is believed to be an autoimmune disease where the body accidently attacks and destroys body tissues. However, in recent times, there are several genetic and environmental factors that have been found to contribute or cause the disease (Huang, 1). To date, there is no single causative factor that has been determined. In addition, the genetic component of this disease is complex, and this makes it difficult for the various subtypes of JRA to be differentiated. Although there is no cure for the disease, there are treatments used to reduce pain and stop function loss. Classification and Clinical Features There are different types of JRA, and the classification depends on factors such as the number of joints affected and the manifestations of the disease during the first six months. The different categories of JRA are useful because they help physicians worldwide to communicate, guide research and facilitate the understanding of treatment (Espinosa &Gottlieb, 303). The main subsets include systemic onset, oligoarthritis onset, Polyarticular RF positive arthritis, polyarticular RF-negative arthritis, psoriatic arthritis, enthesitis related arthritis and undifferentiated arthritis. All these subtypes have certain symptoms in common, and these include: stiffness, swollen joints, pain and limited activity. The first subtype is the systemic-onset JRA which is characterized by high-spiking fever daily that persist for at least two weeks. In this category, arthritis may not be present during diagnosis, and this makes the diagnosis of this type difficult. This subtype comprises of only 10% of the JRA cases. Secondly, the oligoatricular JRA accounts for close to 60% of the patients, making it the most common category of this disease (Kahn, 197). It mainly affects girls aged between one and three years. The patient has arthritis of no more than four joints during the opening six months of this disease. This category is characterized by severe pain and erythema. Thirdly, there is the polyarticular JRA (polyarthritis onset), and this is characterized by symmetric arthritis of at least five joints within the first six months of the disease. It accounts for 25% to 40% of all the JRA cases (Kahn, 198). This is subdivided into two other categories: the rheumatoid factor (RF) positive and the rheumatoid factor negative patients. Common features include weight loss, anorexia and fatigue. The rheumatoid factor positive accounts for not more than 10% of the JRA, and it is actually the childhood onset of the rheumatoid arthritis that affects adults (Kahn, 198). Forth, there is the psoriatic arthritis, which is characterized by arthritis and psoriasis. Juvenile psoriatic arthritis is manifested as an asymmetric arthritis that affects both the large and small joints. It mainly occurs at mid childhood, and this condition is defined by the occurrence of arthritis and psoriatic rash. If the rash is absent, any two of these may be present: nail pitting, family history of psoriasis in the first degree relative and dactylitis (Espinosa &Gottlieb, 309). The fifth category involves the enthesitis related arthritis in which patients have arthritis and enthesitis. It also involves the combination of arthritis or enthesitis with sacroiliac joint tenderness. Enthesitis related arthritis mainly affects boys under the age of eight years. This subtype is characterized by the presence of enthesitis or inflammation occurring at the regions where tendon inserts into the bone. Patients mainly complain of pain, loss of mobility affecting the lower back and stiffness (Espinosa &Gottlieb, 309). The final category is the undifferentiated arthritis which either fulfils none of the other subsets or fulfils more than one of these subsets. Etiology and Pathogenesis Although the etiology and pathogenesis of this disease are not well known, it is believed that genetic susceptibility plays a critical role. This is a complex disease in which different genes play a role in the onset and the manifestation. Studies on tins and families suggest that there is a link between JRA and genes (Espinosa &Gottlieb, 304). For instance, studies show that siblings of persons affected with the disease have a 15 to 30 fold chance higher of getting the disease compared to the general public (Espinosa &Gottlieb, 304). Research has also revealed that HLA class I and II alleles are linked to certain subtypes of JRA. For instance, HLA-B27 is associated with the occurrence of axial skeleton inflammation with hip involvement (Espinosa &Gottlieb, 304). Cell-mediated and humoral immunity have been found to play a role in the pathogenesis of JRA. Proinflammatory cytokines are released by T cells, and these cytokines are found in patients suffering from the polyarticular JRA. Other studies have attempted to show that the systemic onset of JRA is as a result of auto-inflammatory disorders such as the familial Mediterranean fever (FMF). Other factors that have been attributed to the pathogenesis of this disease are hormonal abnormalities, trauma and infectious jiggers (Espinosa &Gottlieb, 305). Epidemiology According to an article appearing in Medscape by David Sherry (2014), close to 300000 children in the United States are suffering from some type of arthritis. It is estimated that 4-14 children in every 100000 children suffer from JRA annually. The numbers are wide ranging because there are differing definitions and classifications of the disease, as well as the population differences. There is also a general lack of data relating to this condition. At the international level, the prevalence of JRA is estimated to be 1.6 to 86 cases for every 100000 children. There are also notable differences in the frequency of the disease when it comes to gender. For instance, oligoatricular JRA affects girls and boys in the ratio of 3:1 respectively. On the other hand, when it comes to enthesitis-related arthritis, the boys outnumber the girls. In terms of age, the disease is common in children between the age of one and three years. However, children under the age of 16 are generally at risk. Finally, the disease affects European children more, and occurs less in Filipino and Japanese children (Espinosa &Gottlieb, 304). Prognosis It is estimated that nearly 50% of children affected with this disease carry into into their adulthood. As a result, these patients may suffer problems such as disability, visual problems, growth abnormalities and functional limitations (Espinosa &Gottlieb, 312). Complications associated with JRA A deadly complication associated with the disease is iridocyclitis, which is a form of chronic anterior uveitis, which can cause permanent blindness (Espinosa &Gottlieb, 310). It occurs in 15% to 20% of patients suffering from JRA. This disease could also lead to growth disturbances. For example, if the disease is affecting one knee, it could lead to accelerated growth of that leg. Finally, other conditions such as osteoporosis and osteopenia are associated with JRA. Treatment The treatment of JRA is aimed at removing active disease, ensure joint functionality, and maintain normal growth, prevent disability and long-term damage to the joints. First, nonsteroidal anti-inflammatory drugs (NSAIDs) are the most commonly used drugs in the treatment of JRA. Some of the common NSAIDs used in children include naproxen, ibuprofen and indomethacin. Second, intra-articular injection of corticosteroids may be used during the early stages of the disease (Huang, 7). For instance, triamcinolone hexacetonide (TH) can be used in treating patients. Third, biologic agents are used to target specific mediators which help in minimizing the effects of cytokines. These biologic agents are mainly genetically engineered. Thirdly, TNF-α Antagonists are used to target TNF-α which is a potent proinflammatory cytokine (Huang, 8). Forth, Etanercept is a biologic therapy that helps in inactivating TNF-α. Other biologic agents that work in the same way as Etanercept are infliximab and adalimumab. Firth, there are also disease-modifying antirheumatic drugs which are agents that help in slowing the radiologic progression of this disease. Examples of such agents include azathioprine, sulfasalazine and cyclosporin (Espinosa &Gottlieb, 310). In many cases, the effects of such agents are witnessed within six to twelve weeks. Autologous Stem Cell Transplantation may also be used as a treatment method. This method of treatment involves the use of immunosuppression in removing autoreactive lymphocytes. This is then followed by the stem cell transplantation. Other treatment options that can be explored include physical therapy in order to improve mobility and the monitoring of both the psychical and psychological functioning of the affected persons (Espinosa &Gottlieb, 311). Works Cited Espinosa, Maria, and Beth Gottlieb. 'Juvenile Idiopathic Arthritis'. Pediatrics in Review 33.7 (2012): 303-313. Print. Huang, Jing-Long. 'New Advances In Juvenile Idiopathic Arthritis'. Biomedical Journal 35.1 (2012): 1-14. Web. Kahn, Philip. ‘Juvenile Idiopathic Arthritis: What the Clinician Needs to Know.’ Bulletin of the Hospital for Joint Diseases, 71.3 (2013): 194-199 Sherry, David. ‘Juvenile Idiopathic Arthritis.’ Medscape. Web Read More