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 all types of arthritis in individuals aged less than 16 years at the time of onset. Symptoms last for more than 6 weeks and there is no known cause.<ref>Rigante D, Bosco A, Esposito S. The Etiology of Juvenile Idiopathic Arthritis. Clin Rev Allergy Immunol. 2015 Oct;49(2):253-61.</ref>
[[File:Juvenile arthritis.jpg|alt=|thumb|419x419px|'''Figure.1''' Swollen joints 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>  


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."<ref>Moore TL. [https://www.frontiersin.org/articles/10.3389/fimmu.2016.00177/full 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)</ref>
JIA ranges in severity. It affects the joints, as well as other structures such as the eyes, skin, and organs,<ref name=":3" /> and it can be divided into the following subtypes:<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>
 
Thisranges in severity. It mostly affects the [[Joint Classification|joints]] and the surrounding tissues, although it can affect other organs like the eyes. Symptoms associated with JIA include:<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>  


* Oligoarticular
* Polyarticular
* Systemic
* Psoriatic
* Enthesitis-related
* Undifferentiated
== 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> There may be hereditary and environmental factors.  
The aetiology of JIA is unclear.<ref name=":1">Thatayatikom A, Modica R, De Leucio A. [https://www.ncbi.nlm.nih.gov/books/NBK554605/ Juvenile Idiopathic Arthritis]. [Updated 2023 Jan 16]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan.</ref> A range of factors have been proposed, including:<ref name=":3" />   


* It is not considered a hereditary condition.<ref name=":0">Arthritis org. JIA Available:https://www.arthritisact.org.au/juvenile-arthritis/ (accessed 18.10.2021)</ref>
* Genetic associations, genetic factors classified into: HLA genes and non-HLA-related genes.
** HLA class II molecules (A2, DRB1, DPB1) are associated with certain non-systemic JIA types, while systemic JIA is not linked to HLA genes.
* Autoimmune factors<ref name=":4">Al-Mayouf SM, Al Mutairi M, Bouayed K, Habjoka S, Hadef D, Lotfy HM, Scott C, Sharif EM, Tahoun N. [https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-021-00650-x Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East]. Pediatr Rheumatol Online J. 2021 Dec 2;19(1):166. </ref> 
* Environmental factors (e.g. infectious agents, antibiotics, stress etc.) may be risk factors
** maternal factors (e.g. smoking<ref name=":3" /><ref>França CMP, Sallum AM, Silva CAA, Aikawa NE, Braga AL, Farhat SC. [https://www.jrheum.org/content/45/2/248 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. </ref>) may be involved, but breastfeeding / having household siblings may reduce risk.
* Infectious viruses (e.g., Epstein-Barr virus, Parvovirus B), bacteria (e.g., Salmonella spp., Shigella spp.) have been linked to the development of JIA, in addition, vaccinations, antibiotics, vitamin D deficiency, stress, and trauma may be causal factors.
* Gastrointestinal infections that result in reduced diversity in the gut microbiome and disturbances in tryptophan metabolism can elevate the susceptibility to developing enthesitis-related arthritis (ERA).


== Epidemiology ==
== Epidemiology ==
JIA is the most common rheumatic disease reported in children of the Western world. The incidence and prevalence are varied among 1.6 to 23 new cases for 100000 children<ref name=":1" />.
JIA is the most common chronic heterogenous rheumatologic condition in children.<ref name=":1" /><ref name=":4" />  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.<ref name=":4" />


* JIA affects 30,000 to 50,000 children in the United States.<ref name="Pathology">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. .  St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2009.</ref>   
* Females are at higher risk of JIA than males<ref name=":4" />
* JIA affects at least one child in every 1,000 in Australia<ref name=":0" />. For unknown reasons, it has been shown to be more prevalent in Norway and Australia.<ref name="eMedicine">eMedicine.  Disease fact sheet: juvenile rheumatoid arthritis. http://emedicine.medscape.com/article/409980-overview (accessed 10 March 2011)</ref>  
* The oligoarticular subtype is more common<ref name=":4" />
* Effects more than twice as many girls as their male counterparts. 
* The mean age of onset is 1-3 years old.<ref name="eMedicine" />  


== Characteristics and Clinical Presentation ==
== Characteristics and Clinical Presentation ==
[[Image:Uveitis.jpg|alt=|thumb|'''Figure.3''' Uveitis]]
[[File:Anterior-uveitis.jpg|thumb|231x231px|Anterior-uveitis]]
The symptoms the child experiences will depend on the type of arthritis they have. Common symptoms include:
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 cause joint damage. But there is a general pattern of inflammatory joint disease in one or more joints:<ref name=":1" /><ref name=":2" />
* pain, swelling and stiffness in one or more joints
*[[skin]] over the affected joints may be warm or red
* mental and physical tiredness, or fatigue.
<br>
Less common symptoms include:
*[[fever]]
* rash
* feeling generally unwell
* eye inflammation (uveitis)<ref name=":2" />


== Diagnostic Tests ==
* Synovitis
JIA is diagnoses according to patient and family medical history, symptoms, number of joints involved, lab tests and sometimes imaging.
* Joint effusion
* Swelling of soft tissues
* Osteopenia
* Bone oedema, erosions


Juvenile idiopathic arthritis is diagnosed using a number of tests including: 
Certain features of JIA will depend on the age of the child affected (e.g. involvement of epiphyseal growth plates, early physeal fusion or the development of discrepancies in limb length).<ref name=":1" />  
* medical history
* 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
* x-rays and scans
* eye examination.<ref name=":2" />


== Pathology ==
Individuals may also experience:<ref name=":2" /><ref>National Institute of Arthritis and Musculoskeletal and Skin Diseases. [https://www.niams.nih.gov/health-topics/juvenile-arthritis/basics/symptoms-causes Juvenile Idiopathic Arthritis (JIA) Basics]. (last accessed 1 May 2023).</ref><ref name=":5">Eskay K. Paediatric Conditions – Autism, Developmental Coordination Disorder, Spina Bifida, Juvenile Idiopathic Arthritis, Haemophilia Course. Plus, 2023.</ref>
[[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:
* Pain and stiffness, including morning stiffness
* Fatigue


# Oligoarticular JIA (Pauciarticular)
*[[Fever]]
#* affected ≤4 joints in the first six months of illness
* Skin rash
#* peak age: 1-6 years
* Generally feeling unwell (malaise)
#* mainly affects medium and large joints
* Uveitis (see image)
# Polyarticular JIA (pJIA)
* Decreased range of motion and soft tissue contractures
#* ≥5 joints are affected
* Muscle atrophy
#* peak age: 1-4 years; 7-10 years
* Weakness, or reduced muscular endurance
#* mainly affects small and medium joints
* Gait deviations
# 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" />
== Diagnostic Tests ==
There is no definitive test to diagnose JIA,<ref name=":1" /> so JIA is a diagnosis based on exclusion. The following evaluations are often included:<ref name=":1" /><ref name=":2" />
* Medical history
* Physical examination
*[[Blood Tests|Laboratory tests]] - typical inflammatory markers are said to be common, particularly in oligoarthritis<ref name=":1" />
* Imaging
** Radiography for symptomatic joints (in early JIA, there are no detectable changes on x-ray, but indirect signs may be present, increased soft tissue density etc<ref name=":1" />)
** Ultrasound
** MRI (the gold standard)
* Eye examination
== 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:<ref name=":1" /><ref name=":2" /><ref name=":0" />


The main treatments for arthritis include:
# Oligoarticular JIA (also known as pauciarticular)
* medications to control the inflammation (including tablets, injections, infusions, eye drops)
#* affects four or fewer joints in the first six months of disease
* exercises to keep the joints moving well and the muscles strong
#* peak age for oligoarticular JIA is 1-6 years
* splints to support the joints
#* more common in females
* joint injections to reduce inflammation in particular joints
#* tends to impact medium and large joints
* pain management strategies to reduce pain and to help your child cope with pain<ref name=":0" />
#* risk of uveitis
# Polyarticular JIA
#* affects more than 5 joints
#* peak ages for polyarticular JIA are 1-4 years and 7-10 years
#* more common in females
#* tends to impact small and medium joints
# Systemic onset JIA
#* also known 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 (these 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


=== '''Pharmacology Management''' ===
== Differential Diagnosis ==
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.
Conditions that might be considered in a differential diagnosis include:<ref name="eMedicine" /><ref name="NCBI">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)</ref> 
*[http://www.physio-pedia.com/index.php5?title=Lyme_Disease Lyme Disease]
*Rheumatic fever
*[http://www.physio-pedia.com/index.php5?title=Osteomyelitis Osteomyelitis]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001450/ Psoriatic arthritis]
*Inflammatory bowel disease
*Leukemia
*[http://emedicine.medscape.com/article/406697-overview#a01 Caffey Disease]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001133/ Chronic Eosinophilic Granuloma]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]
*Involutional Osteoarthritis
{{#ev:youtube|https://www.youtube.com/watch?v=YGO9pAIhoJg&t=72s}}
== Management ==
==='''Pharmacology'''===
The choice of medication depends on type and severity of JIA and if there are other conditions present. Common medications include analgesics, 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 and eye drops.<ref name=":1" /><ref name=":2" />


The most common types of medications used for JIA are:
=== Physiotherapy Management ===
* pain-relievers (analgesics) – for temporary pain relief
Physiotherapy interventions focus on maintaining joint function and strength and teaching joint preservation strategies. Exercise programmes should incorporate low-impact, weight-bearing exercises (e.g. aquatic or bike programmes, gentle resistance exercises).<ref name=":5" />   
* 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 ==
Rehabilitation might include:<ref name=":5" /><ref name="eMedicine">eMedicine.  Disease fact sheet: juvenile rheumatoid arthritis. http://emedicine.medscape.com/article/409980-overview (accessed 10 March 2011)</ref><ref>Bosques G, Singh MP. Juvenile Idiopathic Arthritis [Internet]. 2011, updated 2020 (cited 1 May 2023). Available from: https://now.aapmr.org/juvenile-idiopathic-arthritis/#rehabilitation-management-and-treatments</ref>  
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. 
*[[Strength Training|strengthening]]
 
*[[Range of Motion|range of motion]] exercises and [[stretching]]
Aspects that should be focused on during a physical therapy session are as follows<ref name="eMedicine" />:
*education on joint protection and pain reducing techniques
*Muscle tone
*[[Adherence to Home Exercise Programs|home exercise programme]]
*[[Strength Training|Strengthening]]
*muscle relaxation techniques
*[[Range of Motion|Range of motion]]
*splints / orthotics to help maintain bone and joint alignment / prevent deformities<ref name="NIAMS">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)</ref>
*[[Stretching]]
*adaptive equipment
*Education on joint protection  
Some modalities that might be used to help symptoms such as pain include:  
*[[Adherence to Home Exercise Programs|Home exercise plan]]
*[[Ultrasound therapy|ultrasound]]
*Education on pain reducing techniques
*paraffin wax dips (primarily for hands and feet)
*Muscle relaxation techniques  
*moist compress ([[Thermotherapy|hot packs]])
*Splints or [[Introduction to Orthotics]] maybe be beneficial to help maintain normal bone and joint growth/prevent deformities during growth<ref name="NIAMS">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)</ref>
*[[Aquatherapy|hydrotherapy]]
Some modalities that can be used to help reduce symptoms such as pain are:  
*cold packs
*[[Ultrasound therapy|Ultrasound]]
*Paraffin wax dips (hands and feet primarily)  
*Moist compress ([[Thermotherapy|hot pack]])
*[[Aquatherapy|Hydrotherapy]] (warm)
*Cold packs  
 
== Differential Diagnosis ==
Some signs and symptoms of JIA are shared with those of the following diseases<ref name="eMedicine" /><ref name="NCBI">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)</ref>: 
*[http://www.physio-pedia.com/index.php5?title=Lyme_Disease Lyme Disease]
*Rheumatic fever
*[http://www.physio-pedia.com/index.php5?title=Osteomyelitis Osteomyelitis]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001450/ Psoriatic arthritis]
*Inflammatory bowel disease
*Leukemia
*[http://emedicine.medscape.com/article/406697-overview#a01 Caffey Disease]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001133/ Chronic Eosinophilic Granuloma]
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]
*Involutional Osteoarthritis


== Case Reports/ Case Studies  ==
== Other Professionals involved in Managment of Juvenile Arthritis ==
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/
JIA is a chronic inflammatory disease that affects children and adolescents. It is important to note that prompt diagnosis and proper treatment are crucial in preventing joint damage and promoting healthy growth and development<ref name=":6">Zaripova, L.N., Midgley, A., Christmas, S.E. ''et al.'' [https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-021-00629-8 Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches]. ''Pediatr Rheumatol'' 19, 135 (2021).</ref><ref>Upadhyay J, Lemme J, Cay M, Van Der Heijden H, Sibai D, Goodlett B, Lo J, Hoyt K, Taylor M, Hazen MM, Halyabar O, Meidan E, Schreiber R, Chang MH, Nigrovic PA, Jaimes C, Henderson LA, Ecklund K, Sundel RP. [https://pubmed.ncbi.nlm.nih.gov/34139523/ A multidisciplinary assessment of pain in juvenile idiopathic arthritis.] Semin Arthritis Rheum. 2021 Aug;51(4):700-711. </ref>.


Clinical Trials: Rheumatology Network  https://www.rheumatologynetwork.com/clinical/juvenile-arthritis
Due to its complex and diverse nature, a team of healthcare experts from various disciplines should regularly evaluate and manage JIA. While there is no cure for JIA, achieving better clinical outcomes is possible. New biological disease-modifying antirheumatic drugs have effectively treated severe cases but come with an increased risk of infection <ref name=":6" />. The British Society for Paediatric and Adolescent Rheumatology has published care standards for children and young people with JIA. In managing JIA, it is crucial to involve multiple healthcare professionals, including physicians and allied health professionals (AHP)<ref name=":6" /> <ref name=":7">Fingerhutova, S., Saifridova, M., Vranova, M. ''et al.'' [https://ped-rheum.biomedcentral.com/articles/10.1186/1546-0096-12-S1-P177 Is there an evidence for the role of multidisciplinary team in the management of active juvenile idiopathic arthritis?.] ''Pediatr Rheumatol'' 12 (Suppl 1), P177 (2014).</ref>. A team of multidisciplinary healthcare experts, such as family doctors, rheumatologists, nurses, physiotherapists, occupational therapists, podiatrists, and social workers, should provide comprehensive JIA management, including drug treatment, physiotherapy/occupational therapy, education, and counselling <ref name=":7" />.


== References ==
== References ==
<references />  
<references />  


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[[Category:Paediatrics - Conditions]]
[[Category:Paediatrics - Conditions]]
[[Category:Autoimmune Disorders]]
[[Category:Autoimmune Disorders]]
[[Category:Rheumatology]]

Latest revision as of 13:45, 27 September 2023

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

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

Introduction[edit | edit source]

Figure.1 Swollen joints 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]

JIA ranges in severity. It affects the joints, as well as other structures such as the eyes, skin, and organs,[2] and it can be divided into the following subtypes:[2]

  • Oligoarticular
  • Polyarticular
  • Systemic
  • Psoriatic
  • Enthesitis-related
  • Undifferentiated

Aetiology[edit | edit source]

The aetiology of JIA is unclear.[3] A range of factors have been proposed, including:[2]

  • Genetic associations, genetic factors classified into: HLA genes and non-HLA-related genes.
    • HLA class II molecules (A2, DRB1, DPB1) are associated with certain non-systemic JIA types, while systemic JIA is not linked to HLA genes.
  • Autoimmune factors[4]
  • Environmental factors (e.g. infectious agents, antibiotics, stress etc.) may be risk factors
    • maternal factors (e.g. smoking[2][5]) may be involved, but breastfeeding / having household siblings may reduce risk.
  • Infectious viruses (e.g., Epstein-Barr virus, Parvovirus B), bacteria (e.g., Salmonella spp., Shigella spp.) have been linked to the development of JIA, in addition, vaccinations, antibiotics, vitamin D deficiency, stress, and trauma may be causal factors.
  • Gastrointestinal infections that result in reduced diversity in the gut microbiome and disturbances in tryptophan metabolism can elevate the susceptibility to developing enthesitis-related arthritis (ERA).

Epidemiology[edit | edit source]

JIA is the most common chronic heterogenous rheumatologic condition in children.[3][4] 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.[4]

  • Females are at higher risk of JIA than males[4]
  • The oligoarticular subtype is more common[4]

Characteristics and Clinical Presentation[edit | edit source]

Anterior-uveitis

Symptoms of JIA vary depending on the type of arthritis.[6] It has an unpredictable course - it can be self-limiting or cause joint damage. But there is a general pattern of inflammatory joint disease in one or more joints:[3][6]

  • Synovitis
  • Joint effusion
  • Swelling of soft tissues
  • Osteopenia
  • Bone oedema, erosions

Certain features of JIA will depend on the age of the child affected (e.g. involvement of epiphyseal growth plates, early physeal fusion or the development of discrepancies in limb length).[3]

Individuals may also experience:[6][7][8]

  • Pain and stiffness, including morning stiffness
  • Fatigue
  • Fever
  • Skin rash
  • Generally feeling unwell (malaise)
  • Uveitis (see image)
  • Decreased range of motion and soft tissue contractures
  • Muscle atrophy
  • Weakness, or reduced muscular endurance
  • Gait deviations

Diagnostic Tests[edit | edit source]

There is no definitive test to diagnose JIA,[3] so JIA is a diagnosis based on exclusion. The following evaluations are often included:[3][6]

  • Medical history
  • Physical examination
  • Laboratory tests - typical inflammatory markers are said to be common, particularly in oligoarthritis[3]
  • Imaging
    • Radiography for symptomatic joints (in early JIA, there are no detectable changes on x-ray, but indirect signs may be present, increased soft tissue density etc[3])
    • 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:[3][6][10]

  1. Oligoarticular JIA (also known as pauciarticular)
    • affects four or fewer joints in the first six months of disease
    • peak age for oligoarticular JIA is 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 for polyarticular JIA are 1-4 years and 7-10 years
    • more common in females
    • tends to impact small and medium joints
  3. Systemic onset JIA
    • also known 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 (these 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

Differential Diagnosis[edit | edit source]

Conditions that might be considered in a differential diagnosis include:[12][13]

Management[edit | edit source]

Pharmacology[edit | edit source]

The choice of medication depends on type and severity of JIA and if there are other conditions present. Common medications include analgesics, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease modifying anti-rheumatic medications, biologics and biosimilar medicines and eye drops.[3][6]

Physiotherapy Management[edit | edit source]

Physiotherapy interventions focus on maintaining joint function and strength and teaching joint preservation strategies. Exercise programmes should incorporate low-impact, weight-bearing exercises (e.g. aquatic or bike programmes, gentle resistance exercises).[8]

Rehabilitation might include:[8][12][14]

Some modalities that might be used to help symptoms such as pain include:

Other Professionals involved in Managment of Juvenile Arthritis[edit | edit source]

JIA is a chronic inflammatory disease that affects children and adolescents. It is important to note that prompt diagnosis and proper treatment are crucial in preventing joint damage and promoting healthy growth and development[16][17].

Due to its complex and diverse nature, a team of healthcare experts from various disciplines should regularly evaluate and manage JIA. While there is no cure for JIA, achieving better clinical outcomes is possible. New biological disease-modifying antirheumatic drugs have effectively treated severe cases but come with an increased risk of infection [16]. The British Society for Paediatric and Adolescent Rheumatology has published care standards for children and young people with JIA. In managing JIA, it is crucial to involve multiple healthcare professionals, including physicians and allied health professionals (AHP)[16] [18]. A team of multidisciplinary healthcare experts, such as family doctors, rheumatologists, nurses, physiotherapists, occupational therapists, podiatrists, and social workers, should provide comprehensive JIA management, including drug treatment, physiotherapy/occupational therapy, education, and counselling [18].

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. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 3.9 Thatayatikom A, Modica R, De Leucio A. Juvenile Idiopathic Arthritis. [Updated 2023 Jan 16]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 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.
  5. ↑ 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.
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 better Health Juvenile Arthritis Available: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/arthritis-juvenile(accessed 18.10.2021)
  7. ↑ National Institute of Arthritis and Musculoskeletal and Skin Diseases. Juvenile Idiopathic Arthritis (JIA) Basics. (last accessed 1 May 2023).
  8. ↑ 8.0 8.1 8.2 Eskay K. Paediatric Conditions – Autism, Developmental Coordination Disorder, Spina Bifida, Juvenile Idiopathic Arthritis, Haemophilia Course. Plus, 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 Center for Biotechnology Information, U.S. National Library of Medicine. Disease fact sheet: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001487/ (10 March 2011)
  14. ↑ Bosques G, Singh MP. Juvenile Idiopathic Arthritis [Internet]. 2011, updated 2020 (cited 1 May 2023). Available from: https://now.aapmr.org/juvenile-idiopathic-arthritis/#rehabilitation-management-and-treatments
  15. ↑ 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)
  16. ↑ 16.0 16.1 16.2 Zaripova, L.N., Midgley, A., Christmas, S.E. et al. Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches. Pediatr Rheumatol 19, 135 (2021).
  17. ↑ Upadhyay J, Lemme J, Cay M, Van Der Heijden H, Sibai D, Goodlett B, Lo J, Hoyt K, Taylor M, Hazen MM, Halyabar O, Meidan E, Schreiber R, Chang MH, Nigrovic PA, Jaimes C, Henderson LA, Ecklund K, Sundel RP. A multidisciplinary assessment of pain in juvenile idiopathic arthritis. Semin Arthritis Rheum. 2021 Aug;51(4):700-711.
  18. ↑ 18.0 18.1 Fingerhutova, S., Saifridova, M., Vranova, M. et al. Is there an evidence for the role of multidisciplinary team in the management of active juvenile idiopathic arthritis?. Pediatr Rheumatol 12 (Suppl 1), P177 (2014).
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