JIA
Juvenile Arthritis accounts for a comparatively small number of patients suffering from juvenile arthritis (JA). This paper discusses the clinical manifestations that are considered unique among the subsections of juvenile arthritis, and explores those studies of cytokine profiles that suggest differences between the fundamental mechanisms of the different forms of these diseases. Systemic-onset juvenile arthritis may, in reality, be better classified as differentiated from other subtypes of juvenile arthritis. However, new biologic agents such as the cytokines interleukin-1 and interleukin-6, are presently being licensed or tested, and these look to be promising in treating systemic-onset juvenile arthritis. By comparison, anti-tumor necrosis-factor therapy has proven to be not very effective, if effective at all, in treating this subgroup of JA.
Introduction
Juvenile idiopathic arthritis (JIA) is described by The Merck Manual as "a group of rheumatic diseases that begins at or before age 16." (2014, p. 1) Juvenile idiopathic arthritis is reported to be more than a single disease and is a term applicable to various and chronic arthritides occurring in children although they do have some similar features. (The Merck Manual, 2014, paraphrased) Some of the categories of JIA stated in The Merck Manual include the following categories: (1) Oligoarticular JIA (persistent or extended); (2) Polyarticular JIA (rheumatoid factor [RF] negative or positive); (3) Enthesitis-related arthritis; (4) Psoriatic JIA; (5) Undifferentiated JIA; and (5) Systemic JIA (The Merck Manual, 2014, p. 1) Oligoarticular JIA is reported as the form that is most common generally impact girls who are young and stated to be characterized by more than four joints during the first six months of the disease occurring. There are two types of Oligoarticular JIA including persistent and extended. Polyarticular JIA is reported as the second most commonly occurring form of JIA affecting more than five joints and categorized as RF Negative and RF Positive. (The Merck Manual, 2014, paraphrased) The third type of JIA is Enthesitis-related arthritis and occurs more often among boys who are older with symptoms of inflammation that is painful at the site of tendons and ligaments insertion. This may result in the development of "one of the spondyloarthropathies such as ankylosing spolndylitis or reactive arthritis." (The Merck Manual, 2014, p.1 ) This type of JIA most often occurs in the lower extremities. The fourth type of JIA is Psoriatic JIA and is reported to "typically occurs in young girls and is associated with psoriasis, dactylitis (swollen digits), nail pits, or a family history of psoriasis in a 1st-degree relative. Arthritis is frequently oligoarticular." (The Merck Manual, 2014, p. 1) Undifferentiated JIA is reported to be the diagnosis given when the criteria is not met for any of the aforementioned categories. Finally, Systemic JIA is reported as the form of JIA that is leased common and is such that involves both "fever and systemic manifestations." (The Merck Manual, 2014, p. 1)
The Merck Manual reports that manifestations of JIA include joint involvement and often involvement of the eyes or skin. JIA is such that is reported to potentially impact multiple organs. (2014, paraphrased) Symptoms in children include "joint stiffness, swelling, effusion, pain and tenderness" although it is reported that there is no pain experienced by some children. The manifestations in the joints are reported to be either symmetric or asymmetric and to involve small or large joints. JIA may interfere with the child's development and growth with the reported most common ocular abnormality being "iridocyclitis (inflammation of the anterior chamber and anterior vitreous." (The Merck Manual, 2014, p.1 ) This is reported to be "typically asymptomatic but sometimes causes blurring of vision and miosis. Rarely, in enthesitis-related arthritis, there is conjunctival injection, pain, and photophobia. Iridocyclitis can result in scarring (synechia), glaucoma, or band keratopathy. Iridocyclitis is most common in oligoarticular JIA, developing in nearly 20% of patients, especially if patients are positive for antinuclear antibodies (ANA). It may occur in the other forms but is rare in polyarticular RF-positive JIA and systemic JIA." (The Merck Manual, 2014, p. 1) It is reported that psoriatic JIA is characterized by abnormalities in the skin such as psoriatic skin lesions and systematic JIA is characterized often by a "typical transient rash" reported to appear often with a fever. Systemic JIA is characterized by "high fever, rash, splenomegaly, generalized adenopathy (especially of the axillary nodes), and serositis with pericarditis or pleuritis. These symptoms may precede the development of arthritis. Fever occurs daily (quotidian) and is often highest in the afternoon or evening and may recur for weeks." (The Merck Manual, 2014, p.1) Diagnosis is accomplished through: (1) Clinical criteria; and (2) RF, ANA, and HLA-B27 testing. (The Merck Manual, 2014, p.1 )
Prognosis
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