Juvenile idiopathic arthritis

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Juvenile idiopathic arthritis
Classification and external resources
ICD-10 M08.0
ICD-9 714.3
OMIM 604302
DiseasesDB 12430
MedlinePlus 000451
eMedicine ped/1749 
MeSH D001171
This article does not deal with the more general topic of childhood arthritis.

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).

Contents

[edit] Terminology

The 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 are 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] Symptoms

Symptoms 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] Etiology

The 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 JIA

The 3 major types of JIA are oligoarticular JIA, polyarticular JIA and systemic JIA.[9][10]

 
 
 
 
 
 
 
 
 JIA
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 Oligoarticular JIA
 
 
 Polyarticular JIA
 
 
 Systemic JIA

[edit] Oligoarticular (pauciarticular) JIA

Oligoarticular (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]

[edit] Polyarticular JIA

Affecting 5 or more joints in the first 6 months of disease. This subtype can include the affect of 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.

[edit] Systemic JIA

Is 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".[13]

A polymorphism in macrophage migration inhibitory factor has been associated with this condition.[14]

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.[15]

[edit] Onset

JIA occurs in both sexes. 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] Prevalence

Juvenile 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

 This article does not cite any references or sources. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (March 2007)

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

 This article does not cite any references or sources. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (March 2007)

The treatment of JIA is best undertaken by an experienced team of health professionals, including paediatric rheumatologists, nurse specialists, physiotherapists, occupational therapists, and psychologists. 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).[16] Newer drugs have been developed recently, such as TNF alpha blockers, such as etanercept.[17] 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.[18]

[edit] References

  1. ^ "Juvenile Idiopathic Arthritis (JIA): Joint Disorders: Merck Manual Professional". http://www.merck.com/mmpe/sec04/ch034/ch034c.html. Retrieved on 2008-12-15. 
  2. ^ Ringold S, Burke A, Glass R (2005). "JAMA patient page. Juvenile idiopathic arthritis". JAMA 294 (13): 1722. doi:10.1001/jama.294.13.1722. PMID 16204672. 
  3. ^ juvenile rheumatoid arthritisr at Dorland's Medical Dictionary
  4. ^ Dana D, Erstad S. Juvenile Idiopathic Arthritis. bchealthguide.org. Available at: http://www.bchealthguide.org/kbase/topic/major/hw104391/descrip.htm. Accessed on: March 11, 2007.
  5. ^ "Case Based Pediatrics Chapter". http://www.hawaii.edu/medicine/pediatrics/pedtext/s16c02.html. Retrieved on 2008-12-15. 
  6. ^ MeSH Juvenile+Rheumatoid+Arthritis
  7. ^ Phelan J, Thompson S (2006). "Genomic progress in pediatric arthritis: recent work and future goals". Curr Opin Rheumatol 18 (5): 482–9. doi:10.1097/01.bor.0000240359.30303.e4. PMID 16896287. 
  8. ^ Førre O, Smerdel A (2002). "Genetic epidemiology of juvenile idiopathic arthritis". Scand J Rheumatol 31 (3): 123–8. doi:10.1080/713798345. PMID 12195624. 
  9. ^ Burnham JM, Shults J, Dubner SE, Sembhi H, Zemel BS, Leonard MB (August 2008). "Bone density, structure, and strength in juvenile idiopathic arthritis: importance of disease severity and muscle deficits". Arthritis Rheum. 58 (8): 2518–27. doi:10.1002/art.23683. PMID 18668565. 
  10. ^ Chen CY, Tsao CH, Ou LS, Yang MH, Kuo ML, Huang JL (February 2002). "Comparison of soluble adhesion molecules in juvenile idiopathic arthritis between the active and remission stages". Ann. Rheum. Dis. 61 (2): 167–70. doi:10.1136/ard.61.2.167. PMID 11796405. 
  11. ^ "www.ped.med.utah.edu". http://www.ped.med.utah.edu/jra/default.htm. Retrieved on 2008-12-15. 
  12. ^ "Uveitis and Anti Nuclear antibody Positivity in Children with Juvenile Idiopathic Arthritis". Indian Pediatr 41 (10): 1035–1039. 2004. PMID 15523130. 
  13. ^ systemic onset juvenile rheumatoid arthritis at Dorland's Medical Dictionary
  14. ^ De Benedetti F, Meazza C, Vivarelli M, et al. (May 2003). "Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis". Arthritis Rheum. 48 (5): 1398–407. doi:10.1002/art.10882. PMID 12746913. 
  15. ^ Luthi F, Zufferey P, Hofer MF, So AK (2002). ""Adolescent-onset Still's disease": characteristics and outcome in comparison with adult-onset Still's disease". Clin. Exp. Rheumatol. 20 (3): 427–30. PMID 12102485. 
  16. ^ Hashkes PJ, Laxer RM (October 2005). "Medical treatment of juvenile idiopathic arthritis". JAMA 294 (13): 1671–84. doi:10.1001/jama.294.13.1671. PMID 16204667. http://jama.ama-assn.org/cgi/pmidlookup?view=long&pmid=16204667. 
  17. ^ Lovell DJ, Reiff A, Ilowite NT, et al. (May 2008). "Safety and efficacy of up to eight years of continuous etanercept therapy in patients with juvenile rheumatoid arthritis". Arthritis Rheum. 58 (5): 1496–504. doi:10.1002/art.23427. PMID 18438876. 
  18. ^ Foeldvari I, Szer IS, Zemel LS, et al. (November 2008). "A Prospective Study Comparing Celecoxib with Naproxen in Children with Juvenile Rheumatoid Arthritis". J. Rheumatol.. doi:10.3899/jrheum.080073. PMID 19012356. http://www.jrheum.com/subscribers/08/13/1128.html. 

[edit] External links

v • d • e
Musculoskeletal disorders: Arthropathies (M00-M19, 711-719)
Arthritis
(monoarthritis/
polyarthritis)
Noninfectious
Noninflammatory
Other
see also congenital
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