Juvenile Idiopathic Arthritis: Difference between revisions

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== Introduction ==
== Introduction ==
[[File:JIA-swollenjoint.jpg|alt=|thumb|'''Figure.1''' Swollen Right Knee in Patient with JIA]]Juvenile idiopathic arthritis (JIA) is used to describe types of arthritis of unknown cause, where symptoms last more than 6 weeks, in individuals aged under 16 years at the time of onset.<ref>Rigante D, Bosco A, Esposito S. The Etiology of Juvenile Idiopathic Arthritis. Clin Rev Allergy Immunol. 2015 Oct;49(2):253-61.</ref> It is divided into the following types of arthritis:<ref name=":3">Zaripova LN, Midgley A, Christmas SE, Beresford MW, Baildam EM, Oldershaw RA. [https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-021-00629-8 Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches]. Pediatr Rheumatol Online J. 2021 Aug 23;19(1):135. </ref>
[[File:JIA-swollenjoint.jpg|alt=|thumb|'''Figure.1''' Swollen Right Knee in Patient with JIA]]Juvenile idiopathic arthritis (JIA) is a term used to describe types of arthritis of unknown cause, where symptoms last for more than 6 weeks, in individuals aged under 16 years at the time of onset.<ref>Rigante D, Bosco A, Esposito S. The Etiology of Juvenile Idiopathic Arthritis. Clin Rev Allergy Immunol. 2015 Oct;49(2):253-61.</ref> It is divided into the following types of arthritis:<ref name=":3">Zaripova LN, Midgley A, Christmas SE, Beresford MW, Baildam EM, Oldershaw RA. [https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-021-00629-8 Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches]. Pediatr Rheumatol Online J. 2021 Aug 23;19(1):135. </ref>


* oligoarticular
* oligoarticular
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== Aetiology ==
== Aetiology ==
The aetiology of JIA is unclear.<ref name=":1">Thatayatikom A, De Leucio A. [https://www.ncbi.nlm.nih.gov/books/NBK554605/ Juvenile Idiopathic Arthritis]. StatPearls [Internet]. 2020 Aug 10. Available: https://www.ncbi.nlm.nih.gov/books/NBK554605/<nowiki/>(accessed 18.10.2021)</ref> The following factors have been proposed:<ref name=":3" />   
The aetiology of JIA is unclear.<ref name=":1">Thatayatikom A, Modica R, De Leucio A. Juvenile Idiopathic Arthritis. [Updated 2023 Jan 16]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: <nowiki>https://www.ncbi.nlm.nih.gov/books/NBK554605/</nowiki></ref> The following factors have been proposed:<ref name=":3" />   


* genetic associations   
* genetic associations   
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== Characteristics and Clinical Presentation ==
== Characteristics and Clinical Presentation ==
[[Image:Uveitis.jpg|alt=|thumb|'''Figure.3''' Uveitis]]
[[Image:Uveitis.jpg|alt=|thumb|'''Figure.3''' Uveitis]]
Symptoms of JIA vary depending on the type of arthritis.<ref name=":2" /> There is a general pattern of inflammatory joint disease or one or more joints:<ref name=":1" /><ref name=":2" />
Symptoms of JIA vary depending on the type of arthritis.<ref name=":2">better Health [https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/arthritis-juvenile Juvenile Arthritis] Available: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/arthritis-juvenile<nowiki/>(accessed 18.10.2021)</ref> It has an unpredictable course - it can be self-limiting or lead on to joint damage. But there is a general pattern of inflammatory joint disease in one or more joints. Joint signs include:<ref name=":1" /><ref name=":2" />


* synovitis
* synovitis
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* bone oedema, erosions
* bone oedema, erosions


Individuals may also experience:<ref name=":2" />
There can be specific features of JIA which are associated with the child's age, including the involvement of epiphyseal growth plates, early physeal fusion and there may be discrepancies in limb length.<ref name=":1" />
 
Individuals may also experience:<ref name=":2" /><ref>National Institute of Arthritis and Musculoskeletal and Skin Diseases. Juvenile Idiopathic Arthritis (JIA) Basics. Available from: https://www.niams.nih.gov/health-topics/juvenile-arthritis/basics/symptoms-causes (last accessed 1 May 2023).</ref>
* pain
* fatigue
* fatigue


*[[fever]]
*[[fever]]
* rash
* rash
* feeling generally unwell
* generally feeling unwell (malaise)
* eye inflammation (uveitis)<ref name=":2">better Health [https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/arthritis-juvenile Juvenile Arthritis] Available: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/arthritis-juvenile<nowiki/>(accessed 18.10.2021)</ref>
* uveitis (see image)
* decreased range of motion


== Diagnostic Tests ==
== Diagnostic Tests ==
JIA is diagnoses according to patient and family medical history, symptoms, number of joints involved, lab tests and sometimes imaging.
There is no specific test to diagnose JIA,<ref name=":1" /> but the following may be included:<ref name=":1" /><ref name=":2" />
 
Juvenile idiopathic arthritis is diagnosed using a number of tests including:
* medical history
* medical history
* physical examination
* physical examination
*[[Blood Tests|blood tests]] – however, most children diagnosed with juvenile idiopathic arthritis do not have rheumatoid factor in their blood, so blood tests do not eliminate juvenile idiopathic arthritis
*[[Blood Tests|laboratory tests]] - typical inflammatory markers are said to be common, particularly in oligoarthritis<ref name=":1" />
* x-rays and scans
* imaging
* eye examination.<ref name=":2" />
** radiography for symptomatic joints (but in early JIA, there are no detectable changes on x-ray, but indirect signs may be present, including swelling of soft tissues and dislocation of fat folds<ref name=":1" />)
** ultrasound
** MRI (the gold standard)
* eye examination


== Pathology ==
== Pathology ==
[[Image:JIA-prevalence.jpg|399x399px|'''Figure.2''' Prevalence of Major Subtypes of JIA<ref>Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. .  St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2009.</ref>|alt=|thumb]]There are several subtypes of JIA:
[[Image:JIA-prevalence.jpg|399x399px|'''Figure.2''' Prevalence of Major Subtypes of JIA<ref>Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. .  St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2009.</ref>|alt=|thumb]]There are several subtypes of JIA:<ref name=":1" /><ref name=":2" /><ref name=":0" />
 
# Oligoarticular JIA (Pauciarticular)
#* affected ≤4 joints in the first six months of illness
#* peak age: 1-6 years
#* mainly affects medium and large joints
# Polyarticular JIA (pJIA)
#* ≥5 joints are affected
#* peak age: 1-4 years; 7-10 years
#* mainly affects small and medium joints
# Systemic onset JIA (Still's disease, not to be confused with adult onset Still's disease)
#* arthritis may present weeks to months after the onset of systemic symptoms<ref>Radiopedia [https://radiopaedia.org/articles/juvenile-idiopathic-arthritis JIA] Available: https://radiopaedia.org/articles/juvenile-idiopathic-arthritis (accessed 18.10.2021)</ref>
# Enthesitis-related JIA
# [[Psoriatic Arthritis|Psoriatic]] JIA
# Undifferentiated JIA<ref name=":2" />
== Management and Interventions ==
Treatment of JIA requires anti-inflammatory and immuno-modulatory drugs and physical therapy, and eventually, surgery, nutritional support, and psychosocial support may be needed.
 
The choice of pharmacological treatment depends on the disease subtypes, disease severity and damage, associated disease, and family acceptance. Non-steroidal anti-inflammatory drugs ([[NSAIDs in the Management of Rheumatoid Arthritis|NSAIDs]]) are the mainstay of initial symptomatic treatment for all subtypes. The NSAID use in JIA has decreased over time with modern aggressive treatment, including methotrexate and [[BDMARDs in the Management of Rheumatoid Arthritis|biologics]].<ref name=":1" />
 
The main treatments for arthritis include:
* medications to control the inflammation (including tablets, injections, infusions, eye drops)
* exercises to keep the joints moving well and the muscles strong
* splints to support the joints
* joint injections to reduce inflammation in particular joints
* pain management strategies to reduce pain and to help your child cope with pain<ref name=":0">Arthritis org. JIA Available:https://www.arthritisact.org.au/juvenile-arthritis/ (accessed 18.10.2021)</ref>


=== '''Pharmacology Management''' ===
# Oligoarticular JIA (also known as pauciarticular)
The doctor will always start with the simplest medications at the smallest doses and work their way up to more complex medications and larger doses depending on how the child’s condition responds to the treatments.
#* affected four joints or less in the first six months of disease
#* peak ages are 1-6 years
#* more common in females
#* tends to impact medium and large joints
#* risk of uveitis
# Polyarticular JIA
#* affects more than 5 joints
#* peak ages are 1-4 years and 7-10 years
#* more common in females
#* tends to impact small and medium joints
# Systemic onset JIA
#* known also as Still's disease
#* associated with fever, other signs of systemic inflammation and arthritis<ref>Hedrich CM, Günther C, Aringer M. Morbus Still im Kindes- und Erwachsenenalter [Still's disease in children and adults]. Z Rheumatol. 2017 Sep;76(7):595-608. German. </ref>
#* arthritis may begin weeks / months after the initial symptoms<ref name=":0">Radiopedia [https://radiopaedia.org/articles/juvenile-idiopathic-arthritis JIA] Available: https://radiopaedia.org/articles/juvenile-idiopathic-arthritis (accessed 18.10.2021)</ref>
#* affects females/males equally
#[[Psoriatic Arthritis|Psoriatic]] JIA
#* causes psoriasis and inflammatory arthritis (can develop at different times)
#* more common in females
#* occurs in preschool-aged children and at around 10 years
#* tends to impact fingers, wrists, toes, but not exclusively
# Enthesitis-related JIA
#* inflammation of entheses
#* more common in males
#* tends to occur in older children/adolescents
#* association with acute uveitis
# Undifferentiated JIA
#* JIA that does not fit into another category
== Management ==
Treatment for JIA includes pharmacology, physiotherapy, potentially surgery, and in some cases nutritional and psychosocial support.<ref name=":1" /> 


The most common types of medications used for JIA are:
== '''Pharmacology''' ==
* pain-relievers (analgesics) – for temporary pain relief
'''T'''he choice of medication depends on type and severity of JIA and if there are other conditions. Common medications include analgesics, eye drops, non-steroidal anti-inflammatory drugs ([[NSAIDs in the Management of Rheumatoid Arthritis|NSAIDs]]), corticosteroids, disease modifying anti-rheumatic medications, [[BDMARDs in the Management of Rheumatoid Arthritis|biologics]] and biosimilar medicines.<ref name=":1" /><ref name=":2" />
* creams and ointments – can be rubbed into the skin over a painful joint to provide temporary pain relief
* eye drops – to treat eye inflammation
* non-steroidal anti-inflammatory drugs (NSAIDs) – to control inflammation and provide pain relief
* [[Corticosteroids in the Management of Rheumatoid Arthritis|corticosteroids]] – used to quickly control or reduce [[Inflammation Acute and Chronic|inflammation]]. They can be taken as tablets or given by injection directly into a joint, muscle or other soft tissue
* disease modifying anti-rheumatic medications ([[DMARDs in the Management of Rheumatoid Arthritis|DMARDs]]) – work on controlling the immune system. These medications help relieve pain and inflammation, and can also reduce or prevent joint damage
* biologics and biosimilar medicines (bDMARDs) – are also a type of disease modifying drug. They also work on controlling the immune system. However unlike other disease modifying drugs, biologics target specific cells and proteins that are causing the inflammation and damage, rather than suppressing the entire immune system.  


== Physiotherapy Management ==
== Physiotherapy Management ==
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*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]  
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]  
*Involutional Osteoarthritis
*Involutional Osteoarthritis
== Case Reports/ Case Studies  ==
Juvenile Idiopathic Arthritis Treatment Roundup: What’s Available and What’s Upcoming? https://www.rheumatologyadvisor.com/home/topics/pediatric-rheumatology/juvenile-idiopathic-arthritis-jia-treatment-therapy-strategies-updates-future/
Clinical Trials: Rheumatology Network  https://www.rheumatologynetwork.com/clinical/juvenile-arthritis 
== References  ==
== References  ==
<references />  
<references />  

Revision as of 03:16, 1 May 2023

This article or area is currently under construction and may only be partially complete. Please come back soon to see the finished work! (1/05/2023)

Original Editors - Emily Betz from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Emily Betz, Lucinda hampton, Jess Bell, Admin, Ines Musabyemariya, Naomi O'Reilly, Khloud Shreif, Kim Jackson, Elaine Lonnemann, Wendy Walker, George Prudden, WikiSysop, Meaghan Rieke and Mande Jooste

Introduction[edit | edit source]

File:JIA-swollenjoint.jpg
Figure.1 Swollen Right Knee in Patient with JIA

Juvenile idiopathic arthritis (JIA) is a term used to describe types of arthritis of unknown cause, where symptoms last for more than 6 weeks, in individuals aged under 16 years at the time of onset.[1] It is divided into the following types of arthritis:[2]

  • oligoarticular
  • polyarticular
  • systemic
  • psoriatic
  • enthesitis-related
  • undifferentiated

JIA is "characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera."[3]

JIA ranges in severity. It affects the joints, as well as other structures such as the eyes, skin, and organs.[2]

Aetiology[edit | edit source]

The aetiology of JIA is unclear.[4] The following factors have been proposed:[2]

  • genetic associations
  • autoimmune factors[5]
  • environmental factors (e.g. infectious agents, antibiotics, stress etc) may be risk factors
  • infectious viruses and bacteria may be causal factors
  • maternal factors (e.g. smoking[2][6]) may be involved, but breastfeeding / having household siblings may reduce risk

Epidemiology[edit | edit source]

JIA is the most common chronic heterogenous rheumatologic condition in children.[4][5] It is estimated that around 3 million children and young adults have JIA. The global prevalence is believed to range from 3.8 to 400/100,000; the incidence is estimated to be 1.6 to 23/100,000.[5]

  • Females are at higher risk than males of JIA[5]
  • The oligoarticular subtype is more common[5]

Characteristics and Clinical Presentation[edit | edit source]

File:Uveitis.jpg
Figure.3 Uveitis

Symptoms of JIA vary depending on the type of arthritis.[7] It has an unpredictable course - it can be self-limiting or lead on to joint damage. But there is a general pattern of inflammatory joint disease in one or more joints. Joint signs include:[4][7]

  • synovitis
  • joint effusion
  • swelling of soft tissues
  • osteopenia
  • bone oedema, erosions

There can be specific features of JIA which are associated with the child's age, including the involvement of epiphyseal growth plates, early physeal fusion and there may be discrepancies in limb length.[4]

Individuals may also experience:[7][8]

  • pain
  • fatigue
  • fever
  • rash
  • generally feeling unwell (malaise)
  • uveitis (see image)
  • decreased range of motion

Diagnostic Tests[edit | edit source]

There is no specific test to diagnose JIA,[4] but the following may be included:[4][7]

  • medical history
  • physical examination
  • laboratory tests - typical inflammatory markers are said to be common, particularly in oligoarthritis[4]
  • imaging
    • radiography for symptomatic joints (but in early JIA, there are no detectable changes on x-ray, but indirect signs may be present, including swelling of soft tissues and dislocation of fat folds[4])
    • ultrasound
    • MRI (the gold standard)
  • eye examination

Pathology[edit | edit source]

Figure.2 Prevalence of Major Subtypes of JIA[9]

There are several subtypes of JIA:[4][7][10]

  1. Oligoarticular JIA (also known as pauciarticular)
    • affected four joints or less in the first six months of disease
    • peak ages are 1-6 years
    • more common in females
    • tends to impact medium and large joints
    • risk of uveitis
  2. Polyarticular JIA
    • affects more than 5 joints
    • peak ages are 1-4 years and 7-10 years
    • more common in females
    • tends to impact small and medium joints
  3. Systemic onset JIA
    • known also as Still's disease
    • associated with fever, other signs of systemic inflammation and arthritis[11]
    • arthritis may begin weeks / months after the initial symptoms[10]
    • affects females/males equally
  4. Psoriatic JIA
    • causes psoriasis and inflammatory arthritis (can develop at different times)
    • more common in females
    • occurs in preschool-aged children and at around 10 years
    • tends to impact fingers, wrists, toes, but not exclusively
  5. Enthesitis-related JIA
    • inflammation of entheses
    • more common in males
    • tends to occur in older children/adolescents
    • association with acute uveitis
  6. Undifferentiated JIA
    • JIA that does not fit into another category

Management[edit | edit source]

Treatment for JIA includes pharmacology, physiotherapy, potentially surgery, and in some cases nutritional and psychosocial support.[4]

Pharmacology[edit | edit source]

The choice of medication depends on type and severity of JIA and if there are other conditions. Common medications include analgesics, eye drops, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease modifying anti-rheumatic medications, biologics and biosimilar medicines.[4][7]

Physiotherapy Management[edit | edit source]

Physical therapy is an important part of the treatment of JIA. It is important for the child to remain active and involved in sports and activities with their peers and friends. While pain may limit the amount of activity a child can handle it is important to encourage involvement during periods or remission and allow rest and symptom reducing therapies during periods of flare-ups. Regular activity and general exercise programs help to maintain range of motion in affective joints, build and maintain strength, maintain function and can even help with symptom reduction.

Aspects that should be focused on during a physical therapy session are as follows[12]:

Some modalities that can be used to help reduce symptoms such as pain are:

Differential Diagnosis[edit | edit source]

Some signs and symptoms of JIA are shared with those of the following diseases[12][14]:

References[edit | edit source]

  1. ↑ Rigante D, Bosco A, Esposito S. The Etiology of Juvenile Idiopathic Arthritis. Clin Rev Allergy Immunol. 2015 Oct;49(2):253-61.
  2. ↑ 2.0 2.1 2.2 2.3 Zaripova LN, Midgley A, Christmas SE, Beresford MW, Baildam EM, Oldershaw RA. Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches. Pediatr Rheumatol Online J. 2021 Aug 23;19(1):135.
  3. ↑ Moore TL. Immune complexes in juvenile idiopathic arthritis. Frontiers in immunology. 2016 May 20;7:177.Available:https://www.frontiersin.org/articles/10.3389/fimmu.2016.00177/full (accessed 18.10.2021)
  4. ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 Thatayatikom A, Modica R, De Leucio A. Juvenile Idiopathic Arthritis. [Updated 2023 Jan 16]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK554605/
  5. ↑ 5.0 5.1 5.2 5.3 5.4 Al-Mayouf SM, Al Mutairi M, Bouayed K, Habjoka S, Hadef D, Lotfy HM, Scott C, Sharif EM, Tahoun N. Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East. Pediatr Rheumatol Online J. 2021 Dec 2;19(1):166.
  6. ↑ França CMP, Sallum AM, Silva CAA, Aikawa NE, Braga AL, Farhat SC. Risk factors for juvenile idiopathic arthritis: exposure to tobacco and environmental factors during and before pregnancy. Pediatr Rheumatol Online J. 2014 Sep 17;12(Suppl 1):P27.
  7. ↑ 7.0 7.1 7.2 7.3 7.4 7.5 better Health Juvenile Arthritis Available: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/arthritis-juvenile(accessed 18.10.2021)
  8. ↑ National Institute of Arthritis and Musculoskeletal and Skin Diseases. Juvenile Idiopathic Arthritis (JIA) Basics. Available from: https://www.niams.nih.gov/health-topics/juvenile-arthritis/basics/symptoms-causes (last accessed 1 May 2023).
  9. ↑ Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. . St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2009.
  10. ↑ 10.0 10.1 Radiopedia JIA Available: https://radiopaedia.org/articles/juvenile-idiopathic-arthritis (accessed 18.10.2021)
  11. ↑ Hedrich CM, Günther C, Aringer M. Morbus Still im Kindes- und Erwachsenenalter [Still's disease in children and adults]. Z Rheumatol. 2017 Sep;76(7):595-608. German.
  12. ↑ 12.0 12.1 eMedicine. Disease fact sheet: juvenile rheumatoid arthritis. http://emedicine.medscape.com/article/409980-overview (accessed 10 March 2011)
  13. ↑ National Institute of Arthritis and Musculoskeletal and Skin Diseases. Health information: juvenile arthritis. http://www.niams.nih.gov/Health_Info/Juv_Arthritis/default.asp (accessed 10 March 2011)
  14. ↑ National Center for Biotechnology Information, U.S. National Library of Medicine. Disease fact sheet: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001487/ (10 March 2011)
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