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Juvenile Rheumatoid Arthritis (Juvenile Idiopathic Arthritis, Juvenile virtualchildshealth.com | Juvenile Idiopathic Arthritis (JIA) - Condition Listings - HSS hss.edu | Unraveling the mechanisms of Juvenile Idiopathic Arthritis arthritis.arizona.edu | Overview | Juvenile Idiopathic Arthritis | Seattle Cancer Care Alliance seattlecca.org |
Juvenile idiopathic arthritis (JIA) is the most common form of persistent arthritis in children. (Juvenile in this context refers to an onset before age 16,[1] idiopathic refers to a condition with no defined cause, and arthritis is the inflammation of the synovium of a joint.) JIA is a subset of arthritis seen in childhood, which may be transient and self-limited or chronic. It differs significantly from arthritis commonly seen in adults (osteoarthritis, rheumatoid arthritis), and other types of arthritis that can present in childhood which are chronic conditions (e.g. psoriatic arthritis and ankylosing spondylitis).
[edit] TerminologyThe Terminology used is evolving, and each term has some limitations. According to some sources, JIA replaces the term juvenile rheumatoid arthritis (JRA).[2] Other sources still use the latter term.[3] JIA is sometimes referred to as juvenile chronic arthritis (JCA),[4] a term that is not precise as JIA does not encompass all forms of chronic childhood arthritis. A majority of cases is rheumatoid factor negative, which leads some to consider the "chronic" or "idiopathic" labels more appropriate.[5] However, if a cause was determined, then "idiopathic" may no longer be appropriate (making JIA a diagnosis of exclusion or wastebasket diagnosis), and if the course was self-limited, then "chronic" may no longer be appropriate. Adding to the confusion, the term rheumatoid itself lacks a consistent, unambiguous definition. MeSH uses "Juvenile Rheumatoid Arthritis" as the primary entry, and uses "chronic" and "idiopathic" in alternate entries.[6] [edit] SymptomsSymptoms of JIA are often non-specific initially, and include lethargy, reduced physical activity, and poor appetite (often due to medication).[citation needed] The first manifestation, particularly in young children, may be limping. Children may also become quite ill, presenting with flu-like symptoms that persist. The cardinal clinical feature is persistent swelling of the affected joint(s), which commonly include the knee, ankle, wrist and small joints of the hands and feet. Swelling may be difficult to detect clinically, especially for joints such as those of the spine, sacroiliac joints, shoulder, hip and jaw, where imaging techniques such as ultrasound or MRI are very useful. Pain is an important feature of JIA, but young children may have difficulty in communicating this symptom. Late effects of arthritis include joint contracture (stiff, bent joint) and joint damage. Children with JIA vary in the degree to which they are affected by particular symptoms. [edit] EtiologyThe cause of JIA, as the word idiopathic suggests, is unknown and currently an area of active research.[7] Current understanding of JIA suggests that it arises in a genetically susceptible individual due to environmental factors.[8] [edit] Types of JIAThe 3 major types of JIA are oligoarticular JIA, polyarticular JIA and systemic JIA.[9][10]
[edit] Oligoarticular (pauciarticular) JIAOligoarticular (or pauciarticular) JIA affects 4 or fewer joints in the first 6 months of illness. Oligo means few. Oligoarticular is used with JIA terminology, and pauciarticular is used with JRA terminology.[11] Patients with oligoarticular JIA are more often ANA positive, when compared to other types of JIA.[12] Accounts for about 50% of JIA cases. Usually involves large joints such as the hips, ankles, and knees, but smaller joints such as the fingers and toes may also be affected. It is usually not symmetrical, meaning the affected joints are on one side of the body rather than on both sides simultaneously. Children with pauciarticular JRA are at risk for developing chronic iridocyclitis or uveitis, which is inflammation of the eye. This condition is often gone unnoticed; therefore your child should be closely monitored by an ophthalmologist.[13] [edit] Polyarticular JIAAffecting 5 or more joints in the first 6 months of disease. This subtype can include the neck and jaw as well as the small joints usually affected. This type of JIA is more common in small girls to that of boys. Usually the smaller joints are affected in polyarticular JIA, such as the fingers and hands, although weight-bearing joints such as the knees, hips, and ankles may also be affected. The joints affected are usually symmetrical, meaning that it affects both joints on both sides of the body (such as both wrists.) Children with polyarticular JRA are also at risk for developing chronic iridocyclitis or uveitis (inflammation of the eye) and should also be monitored by an ophthalmologist. [13] Rheumatoid factor is positive in children with polyarticular JRA and is rarely positive in children with systemic JRA. [edit] Systemic JIAIs characterized by arthritis, fever and a salmon pink rash. Systemic JIA can be challenging to diagnose because the fever and rash come and go. It affects males and females equally, unlike the other two subtypes of JIA. Systemic JIA may have internal organ involvement and lead to serositis (e.g. pericarditis). It is also known as "systemic onset juvenile rheumatoid arthritis".[14] A polymorphism in macrophage migration inhibitory factor has been associated with this condition.[15] It is sometimes called "adolescent-onset Still's disease", to distinguish it from adult-onset Still's disease. However, there is some evidence that the two conditions are closely related.[16] [edit] OnsetJIA occurs in both sexes, but, like other rheumatological diseases, is more common in females. Symptoms onset is frequently dependent on the subtype of JIA (see Types of JIA) and is from the pre-school years to the early teenage years. [edit] PrevalenceJuvenile idiopathic arthritis affects an estimated 300,000 children in the United States.[citation needed] Of these children, 50 percent have pauciarticular JIA, 40 percent have polyarticular JIA and 10 percent have systemic JIA. [edit] Extra-articular manifestations
1. Eye disease: JIA is associated with inflammation in the front of the eye (chronic anterior uveitis). This complication may not have any symptoms and can be detected by an experienced optometrist or ophthalmologist using a slit lamp. Most children with JIA are enrolled in a regular slit lamp screening program, as poorly controlled chronic anterior uveitis may result in permanent eye damage, including blindness. 2. Growth disturbance: Children with JIA may have reduced overall rate of growth, especially if the disease involves many joints or other body systems. Paradoxically, individually affected large joints (such as the knee) may grow faster, due to inflammation - induced, increased blood supply to the bone growth plates situated near the joints [edit] Treatment
The treatment of JIA is best undertaken by an experienced team of health professionals, including pediatric rheumatologists, nurse specialists, physiotherapists, and occupational therapists. Many others in the wider health and school communities also have valuable roles to play, such as ophthalmologists, dentists, orthopaedic surgeons, school nurses and teachers, careers advisors and, of course local general practitioners, paediatricians and rheumatologists. It is essential that every effort is made to involve the affected child and their family in disease education and balanced treatment decisions. There have been very beneficial advances in drug treatment over the last 20 years. Most children are treated with non-steroidal anti-inflammatory drugs and intra-articular corticosteroid injections. Methotrexate is a powerful drug which helps suppress joint inflammation in the majority of JIA patients with polyarthritis[citation needed] (though less useful in systemic arthritis).[17] Newer drugs have been developed recently, such as TNF alpha blockers, such as etanercept.[18] There is no controlled evidence to support the use of alternative remedies such as specific dietary exclusions, homeopathic treatment or acupuncture. Celecoxib has been found effective in one study.[19] [edit] See also
[edit] References
[edit] External links
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