Juvenile idiopathic arthritis ( JIA ), also known as juvenile rheumatoid arthritis , is the most common form of arthritis in children and adolescents. ("Youth" in this context refers to onset before age 16, "idiopathic" refers to a condition without a clear cause, and "arthritis" is inflammation of the synovium joint.)
JIA is an autoimmune, non-infectious, inflammatory joint disease, with a duration of more than 6 weeks in children younger than 16 years. This disease generally occurs in children from ages 1 to 6 years, but can develop until the age of 15 years. This is part of the arthritis seen in childhood, which may be temporary and self-limited or chronic. It differs significantly from the arthritis commonly seen in adults (osteoarthritis, rheumatoid arthritis), and the type of arthritis that can present in childhood which is a chronic condition (eg psoriatic arthritis and ankylosing spondylitis). Aetiopathology is similar to rheumatoid arthritis, but with less marked cartilage erosion, and joint instability and no rheumatoid factor.
JIA affects about one in 1,000 children each year, with about one in 10,000 having a more severe form.
Video Juvenile idiopathic arthritis
Signs and symptoms
Symptoms of JIA are often not specific at first, and include lethargy, reduced physical activity, and poor appetite. The first manifestations, especially in young children, may stagger. Children can also become very sick, with flu-like symptoms persisting. The cardinal clinical feature is the persistent swelling of the affected joint (s), which usually includes the knees, ankles, wrists, and small joints of the hands and feet. Swelling may be difficult to detect clinically, especially for joints such as the spine, sacroiliac joints, shoulders, hips, and jaws, where imaging techniques such as ultrasound or MRI are very useful.
Pain is an important symptom. Improved morning stiffness later in life is a common feature (this suggests an inflammatory joint type pain rather than a mechanical type of joint pain). The final effects of arthritis include joint contractures (stiff, bent joints due to fibrosis) and joint damage. Children with JIA vary in the rate at which they are affected by certain symptoms. Symptoms may also differ between the sexes, affecting girls and boys differently between different geographic locations. This is predicted to be due to biological differences in different geographic areas. Children may also have swollen joints (inflammatory swelling, or in chronic arthritis due to proliferation of synovial membranes and thickening, and periarticular soft tissue swelling).
Extra-articular
Eye disease: JIA is associated with inflammation in the front of the eye (especially iridocyclitis, a form of chronic anterior uveitis), affecting about one in five children who have JIA, most often girls. These complications are usually asymptomatic and can be detected by an ophthalmologist or an experienced eye doctor using a gap lamp. Then features a gap lamp including synechiae. Most children with JIA are enrolled in regular slitlight screening programs, because uncontrolled uncontrolled chronic uveitis can cause permanent eye damage, including blindness.
Growth disorders: Children with JIA may have reduced overall growth rates, especially if the disease involves many joints or other body systems. Paradoxically, large individual-affected joints (such as knees) can grow faster, as inflammatory-induced blood supply increases to the bone growth plate located near the joint. This can cause different leg lengths, as well as deformities such as genu valgum. Asymmetric growth can also affect other bones, eg. difference in digit length. The marked differences in bone age (skeletal maturation) can be seen.
Complications
JIA is a chronic disorder, which, if ignored, can cause serious complications. Proper follow-up with healthcare professionals can significantly reduce the likelihood of developing complications.
A form of inflammation of the eye called uveitis is common with several types of JIA. Inflamed eyes, if left untreated, can cause glaucoma, scars, cataracts, and even blindness. Often, inflammation of the eyes occurs without symptoms, or while JIA is otherwise in remission, so it is important for all children to get regular eye examinations from an ophthalmologist. The presence of ANA is a predictor of eye involvement (common in oligoarthritis-type JIA).
Retardation of growth is common in children with JIA. In addition, drugs (corticosteroids) used to treat JIA have powerful side effects that can limit growth. Other musculoskeletal problems may include joint contracture, muscle weakness or muscle loss, and osteoporosis.
Children who postpone treatment or do not participate in physical therapy, or children with severe illness, can often develop joint deformities of the hands and fingers. Over time, the hand function disappears and is almost impossible to recover.
Maps Juvenile idiopathic arthritis
Cause
The cause of JIA remains a mystery. However, the disorder is autoimmun - which means that the immune system itself begins to attack and destroy cells and tissues (especially in joints) for no apparent reason. The immune system is thought to be provoked by environmental changes, in combination with mutations in many related genes and/or other causes of different gene expression. Experimental studies have shown that certain mutated viruses may be able to trigger JIA. The disease appears to be more common in girls, and it is most common in Caucasians.
Associated factors that may worsen or be associated with rheumatoid arthritis include:
- genetic predisposition; When one family member has been diagnosed with rheumatoid arthritis or other autoimmune disorders, the odds are higher that other family members or siblings may also develop arthritis.
- Women are more likely to develop rheumatoid arthritis than men of all ages.
- Strong beliefs state that psychological stress may aggravate the symptoms of rheumatoid arthritis. However, when emotional stress is under control, the symptoms of arthritis do not always disappear, suggesting that the relationship is not direct.
- Although no different immune factors have been isolated as a cause of arthritis, some experts believe that the trigger factor may be like a virus that then disappears from the body after permanent damage is done.
- Since rheumatoid arthritis is more common in women, it may be that sex hormones may play a role in causing or modulating arthritis. Unfortunately, both the lack of sex hormones and replacement have been shown to improve or aggravate arthritis.
The cause of JIA, as the word "idiopathic" suggests, is unknown and the area of ​​study is active. The current understanding of JIA suggests that it appears in genetically susceptible individuals because of environmental factors.
Diagnosis
JIA diagnosis is difficult because joint pain in children can come from many other causes. There is no single test that can confirm the diagnosis, and most doctors use a combination of blood tests, X-rays, and clinical presentations to make an initial diagnosis of JIA. Blood tests measure antibodies and rheumatoid factors. Unfortunately, the rheumatoid factor is absent in all children with JIA. In addition, in some cases, blood work is rather normal. X-rays are obtained to ensure that joint pain is not from fractures, cancer, infections, or congenital abnormalities.
In most cases, the fluid from the joint is sucked and analyzed. This test often helps in making a diagnosis of JIA by excluding other causes of joint pain.
Differential diagnosis
One possible differential diagnosis for JIA is Farber's disease, which is a rare, fatal, and genetic lysosomal abnormality caused by a deficiency of seramidase acid enzymes. It has symptoms similar to JIA including swelling, stiffness, and pain in the joints. This joint disorder is progressive and develops early in life in patients with Farber's disease. Patients with Farber's disease usually have subcutaneous nodules and hoarseness or weakness due to the growth of nodules in the larynx. The diagnosis of Farber's disease can be confirmed by gene sequencing.
There are several disorders and other diseases that appear with symptoms similar to JIA. One possibility can be acute pediatric lymphoblastic leukemia in which children will exhibit joint pain and arthritis due to expansion in lymphoblasts in bone metaphysis. Thrombocytopenia and lymphocytosis are rare in children and should be seen when identifying the cause of joint pain as both are seen in children with leukemia. Behcet Syndrome is a multisystem disease also with symptoms similar to JIA. Aetiology is unknown and causes multisystem inflammation. It can develop into aphthous ulcers, genital ulceration and blindness of uvitis. Additional symptoms and age onset, because Behcet's syndrome is rarely diagnosed before school age, can help distinguish. Lyme disease can also be often confused with JIA due to arthritis that develops if not treated or undiagnosed. Systemic arthritis that develops affects several joints and can mimic arthritis caused by JIA. To rule out Lyme disease, ELISA blood tests can be performed to identify antibodies to bacteria that cause lyme disease.
Classification
The three main types of JIA are oligoarticular, polyarticular, and systemic.
Oligoarticular
Oligoarticular (or pauciarticular) JIA affects four or fewer joints in the first six months of the disease. The "oligo-" and "pauci-" prefixes mean "little".
Oligoarticular is used with JIA terminology, and pauciarticular is used with JRA terminology.
Patients with an oligoarticular JIA are more likely to be ANA positive, when compared with other JIA types.
This form accounts for about 50% of JIA cases, and usually involves large joints such as knees, ankles, and elbows, but smaller joints (such as fingers and toes) may also be affected. Hips are not affected like polyarticular JIA. Usually not symmetrical, which means the affected joints are on one side of the body rather than on both sides simultaneously. Long differences and muscle atrophy are common, leading to asymmetric growth and flexural contracture risk. Early-onset patients are at risk of chronic iridocyclitis or anterior uveitis, which is an inflammation of the eye. This condition is often overlooked; therefore, these children should be closely monitored by an ophthalmologist. If ANA, patients need routine eye examinations every 3 months. If ANA- and more than 7 years, they can perform eye examinations every 6 months. Children with onset of onset oligoarticular onset are at risk of sacroiliitis and spondyloarthropathy; the onset of an oligoarticular JIA slowly attacks boys more often than girls and is associated with HLA-B27 .
Polyarticular
Polyarticular JIA affects five or more joints in the first 6 months of the disease. This subtype may include the neck and jaw, as well as the small joints that are normally exposed. This type of JIA is more common in girls than boys. This accounts for about 40% of JIA cases.
Typically, smaller joints are affected by polyarthicular JIA, such as fingers and hands, although burden-bearing joints such as knees, hips, and ankles may also be affected. The affected joints are usually symmetrical, which means affecting both joints on both sides of the body (such as both wrists.) Children with polyarthenic JIA are also at risk for chronic iridocyclitis or uveitis and should also be monitored by ophthalmologists or ophthalmologists.. The rheumatoid factor may be positive, ie seropositive, in children with polyarticular JIA occurring between 9 and 16 years and associated with HLA DR4 and HLA DW4 . This group has a worse prognosis with about 50% progressing to severe disabiling arthritis, persisting into adulthood. In general seronegative in JIA occurs under the age of 10 with a mild disease process and responds better to treatment. Seropositive is rare in children with systemic JIA. As more and more joints are affected by polyarticular JIA, and the tendency to worsen over time, JIA polybicular should be treated aggressively.
Systemic
Systemic JIA is characterized by arthritis, fever, which is usually higher than the mild fever associated with polyarthicular, and salmon pink rash. It accounts for 10-20% of JIA and affects men and women alike, unlike the other two subtypes of JIA, and affects adolescents. Usually involving large and small joints. Systemic JIA can be challenging to be diagnosed because of fever and rash coming and going. Fever can occur at the same time every day or twice daily (often in the afternoon or evening) quickly returning to the spontaneous baseline (vs septic arthritis of ongoing fever). This rash is a pink macula of different sizes. It migrates to different locations on the skin, rarely surviving in one location for more than an hour. This rash is often seen in the body and limbs proximal to or above the pressure area.
Systemic JIA may have internal organ involvement - hepatosplenomegaly, lymphadenopathy, serositis, hepatitis, tenosynovitis, etc.
It is also known as "rheumatic arthritis systemic rheumatic".
A polymorphism in the inhibitory factor of macrophage migration has been linked to this condition.
Sometimes called the "Still Adolescent onset disease", to distinguish it from adult Still onset disease. However, some evidence suggests these two conditions are closely related.
Rheumatoid and ANA factors are generally negative in systemic JIA.
Other types
Some doctors include two other less common forms: arthritis-related enthesitis and psoriasis JIA. Enthesitis is an inflammation of the tendon insertion point. This form is most common in boys older than 8 years, large joints of lower extremities are usually exposed; in nature, causes back pain, and is associated with ankylosing spondylitis and inflammatory bowel disease. Psoriatic JIA is most common in girls, along with psoriasis or two other features - namely dactylitite and pitting nails, although joint problems may precede several years of skin manifestation.
Treatment
JIA is best handled by multidisciplinary teams. The main emphasis of treatment for JIA is to help children regain normal levels of physical and social activity. This is achieved by the use of physical therapy, pain management strategies, and social support. Another emphasis of care is to control inflammation and extra-articular symptoms quickly. Doing so will help reduce joint damage and other symptoms, which will help reduce the level of permanent damage that causes disability.
Advantageous advances in drug treatment have been conducted over the past 20 years. Most children are treated with nonsteroidal anti-inflammatory drugs and intra-articular corticosteroid injections. Methotrexate, a disease-modifying antirheumatic drug (DMARD) is a powerful drug that helps suppress joint inflammation in the majority of JIA patients with polyarthritis (although less useful in systemic arthritis). New drugs have been developed recently, such as TNF alpha blockers, such as etanercept. No controlled evidence supports the use of alternative treatments such as special dietary exclusions, homeopathic remedies, or acupuncture. However, increased consumption of omega-3 fatty acids proved useful in two small studies.
Celecoxib has been found to be effective in one study.
Other aspects of managing JIA include physical and occupational therapy. The therapist can recommend the best exercises and also make the protective equipment. In addition, children may require the use of special support, ambulatory devices, or splints to help them move and function normally.
Surgery is only used to treat the most severe cases of JIA. In all cases, surgery is used to remove scars and improve joint function.
Home remedies that can help JIA include getting regular exercise to improve muscle strength and flexibility of joints. Swimming is probably the best activity for all children with JIA. Stiffness and swelling can also be reduced by cold pack applications, but a warm bath or shower can also increase joint mobility.
In the future, genetic testing may be available enabling early detection of JIA. Early detection will help determine the severity of the disease in each child and help identify which treatments will be the most effective and beneficial treatment option.
Therapy
A common approach to treating a child with JIA usually involves a team of medical professionals including rheumatologist, occupational therapist (OT), physical therapist (PT), nurse and social worker.
The role of OT/PT is to help children participate as fully and independently as possible in their daily activities or "work", by preventing psychological and physical dependence. The goal is to maximize the quality of life, and minimize disruption to the lives of children and families. OT works with their children, families and schools, to create individual plans based on child's conditions, limitations, strengths and goals. This is done by continuous assessment of a child's social ability and function. The plan may include the use of various tools, such as splints, that help a person perform the task. The plan may also involve changes to homes, encourage the use of uninvolved joints, and provide children and their families with support and education about diseases and strategies to manage them. OT interventions will be changed depending on JIA development and forgiveness, to promote age-appropriate autonomy. Early PL involvement is very important. The interventions taught by the OT can help a child adapt and adapt to JIA challenges for the rest of their lives.
Self-care
OT/PT can provide many strategies to help children in their dress routine. Clothes with easy openings and Velcro, as well as devices, such as buttons and zipper pulls can be used. For children who have difficulty bending, a recommended reacher and long socks are recommended. OT can also show children how to sit during dressing so that fewer stretches are placed on their joints.
OT/PT can help children maintain cleanliness through recommending tools. For children who have difficulty reaching all areas of their bodies, long-handled sponges with soft grips can be given. If the children find it difficult to sit in the tub or stand in the bathroom, an OT can prescribe a bath bench or bath chair to be installed to help the child stay in a pain-free position. If brushing teeth is challenging, a toothbrush with a larger and soft grip or an electric toothbrush may be recommended. For flossing, flossers with adjustable grips can be provided. Long-handled hairbrushes can be used by children who have difficulty reaching the back of their head. A razor can be adjusted for an easier handle, or an electric razor can be used for shaving. OT can also show girls who want to use make-up, how to increase the size of the handle of make-up application tools for easier grip.
For children with pain in the hands and wrists, lightweight devices and devices with large handles and other devices (such as beveled knives, fastening tools, bottle jars and bottles, back handles, doorknobs, etc.) may be provided to make tasks easier, less painful and more fun. Oblique glass can be used for children who have neck stiffness. Education can be given about good eating habits that help control bone loss due to inactivity and drug side effects. Occupational therapists provide a myriad of strategies to help children with JIA perform self-care tasks.
Spare time
One of the best ways occupational or physical therapists (OT or PT) can help children with JIA participate in activities with their friends is by helping them create home exercise programs in play. Exercise prescribed by both PT and OT to increase the reach of a child can move joints and strengthen joints to reduce pain and stiffness and prevent further limitations in their joint motion. OT can provide children with age-appropriate games and activities to enable children to practice their exercises while playing and socializing with friends. Examples are craft, swimming, and noncompetitive sports.
OTs often prescribe custom-made orthotics to support and improve the position and function of the body. Orthotics maintain alignment to reduce discomfort in the legs and back when children participate in physical activities such as sports. Splints can be used to support joints during activities, to reduce children's pain and increase participation in recreational activities they like. Resting splints may be prescribed for children to wear at night to reduce swelling and joint stiffness, allowing them to have less pain and stiffness when participating in play activities. Furthermore, splints work to be used to support joints and relieve pain while working by hand like during craftsmen. A series of casts can be used to gradually lengthen shortened muscles, allowing increased participation in recreational activities.
OT can help children learn how to interact with their classmates and friends with brainstorming, role playing, and modeling strategies. The OT also helps children see what activities they are mastering and which gives them difficulties. Furthermore, OT can help children learn to communicate their pain to others. The benefits of OT treatment include: increased social interaction, increased self-confidence, and a positive self-image. OT can help children make friendships with other children with similar illnesses to help them feel less alone or less different from others. Many PLs run summer camps for children with similar diseases so that children can get to know others with their illness. Education sessions on JIA and recreation, and activities such as swimming, canoeing, and nature trails are common.
For children who find the weather cool or humid makes it difficult to play with friends outside, OT can provide ideas for clothing that keeps the child warm and dry without restricting movement. An example is cycling gloves, which allow children to move their fingers while still keeping their hands warm, compared to the large winter gloves that limit the functioning of the hands. Warm pajamas and electric blankets can alleviate pain and improve sleep.
Productivity
Children with JIA often require modification of school activity because of symptoms of the disease. OT can work with families and schools to improve school attendance. Therapists help children to succeed by providing ways for full participation in school by working with staff, taking part in planning activities and assessing accommodation and adaptation needs.
OT works with children, families and schools to develop strategies to help children manage pain, stiffness and fatigue, which can sometimes limit their ability to participate in school-related activities. A balanced plan will allow children to get enough activity so that they do not become stiff, but also enough rest so they are not tired. For example, a plan may be done with a teacher so that a child will be allowed to stand up and stretch during a long sit, perform a modified sporting activity or take a rest during the gym class. Other general management strategies taught by OT include getting up before school and a warm bath and then doing exercises to reduce stiffness and pain throughout the day. Using proper body movements when doing activities helps reduce tension in the joints and thereby reduces pain and stiffness. OT can also teach children how to relax their muscles.
OT can prescribe special equipment for children at school to make them more comfortable. Tables and chairs with the right height for children are important. The table may have the ability to tilt into a comfortable position for writing. Pencils and pens with bigger and softer grips can be used to make writing easier and less painful. Special keyboards can be prescribed to keep the child's arm in a position that will reduce the strain on the joint when using the computer. OT can work with teachers to educate them about the child's condition, limitations and ways they can help make the school a positive experience for the child. Recommendations may include two sets of textbooks, one for home one for school, to prevent carrying many books. Additional recommendations may involve reduced writing and typing, sitting in chairs instead of floors, additional time for switching between classes, elevator locks for elevated schools but restricting them to students with health problems, providing student-taker notes, and additional time to complete the task.
As adolescents become adults, OT can begin working with them on their future educational and work plans. OT can help teens find ways to inform their employers about their disease in a positive way. OT can also help teenagers understand their rights as employees with disabilities. Assistance to obtain funding for post-secondary education may be provided. OT can help teens to volunteer in the community, to gain experience and confidence in their abilities. It is important that adolescents with JIA understand how to take care of themselves and manage their illness while working full-time or attending universities. OT can help teens develop strategies that will enable them to function at their greatest ability by maintaining their health.
Prognosis
In 1999: With early and aggressive therapy, some children improve over time and live a normal life. However, severe JIA cases that are not treated promptly can lead to poor growth and worsening joint function. Since around 1980, significant improvements have been made in the treatment of JIA and most children can live a decent standard of living. To improve the prognosis of JIA, patients should schedule echocardiographic routine checks to assess cardiac function. New research shows that identifying what type of JIA a child can help target treatment and lead to more positive outcomes. Identifying specific biomarkers associated with each type of JIA may help to establish a more personalized care plan and reduce remission rates.
Children with JIA are more susceptible to cardiovascular disease, depression, sleep disorders, anxiety and fatigue than healthy people. There is also limited information showing that children with JIA are at increased risk for malignancy when treated with TNF blockers. The prognosis is more positive when gene testing is performed to identify which subtypes of JIA are present in the child. Standard care protocols in a special place for each JIA subtype. Treatment is more successful when targeted to JIA specific subtypes.
Epidemiology
Onset
JIA occurs in both sexes, but like other rheumatological diseases, it is more common in women. Symptoms of onset often depend on the subtype of JIA and come from the preschool years to early adolescence.
Prevalence
Adolescent idiopathic arthritis affects between eight and 150 out of every 100,000 children, depending on the analysis. Of these children, 50% have JIA pauciarticular, 40% have polyarticular JIA, and 10% have systemic JIA. In the previously selected group (children under 16 years with orthodontic treatment needs), prevalence increased to one in 100 (0.88% of 1024 children).
Terminology
The terminology used grows, and each term has some limitations.
According to some sources, JIA replaces the term rheumatoid arthritis (JRA). Other sources still use the last term.
JIA is sometimes referred to as juvenile chronic arthritis (JCA), an incorrect term because JIA does not cover all forms of chronic childhood arthritis.
Most cases are a negative rheumatoid factor, which causes some people to consider the label "chronic" or "idiopathic" more appropriate. However, if the cause is determined, the "idiopathic" may no longer be appropriate (making the diagnosis of JIA an exception), and if the course is limited, then "chronic" may no longer be appropriate.
Adding to the confusion, the term rheumatoid itself does not have a consistent and unambiguous definition.
MeSH uses "juvenile rheumatoid arthritis" as the primary entry, and uses "chronic" and "idiopathic" in alternative entries.
Famous patient
- Rosemary Sutcliff, author
- Clark Middleton, actor
References
Further reading
- Weber, Joachim; Weber D., Tzaribachev N. "HIGH TEMPOROMANDIBULER FREQUENCY TOGETHER-ISOLATION JUVENILE IDIOPATHIC ARTHRITIS IN CHILDREN WITH ORTHODONTIC ORIGINAL NEEDS". OP46 @ EOS 2011, Istanbul, Turkey . European Orthodontic Society.
External links
Direktori
- JIA - NIH Medline Plus.
Source of the article : Wikipedia
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