Juvenile Idiopathic Arthritis: Difference between revisions

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== Introduction ==
== Introduction ==
[[File:JIA-swollenjoint.jpg|alt=|right|frameless]]
[[File:Juvenile arthritis.jpg|alt=|thumb|419x419px|'''Figure.1''' Swollen joints in Patient with JIA]]
'''Image 1:  Swollen right knee in JIA patient'''
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>  
Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children and teens under the age of 16, 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 can appear in many different ways and can range in severity. It mostly affects the joints and the surrounding tissues, although it can affect other organs like the eyes.
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>


Symptoms of JIA include joint pain, swelling and stiffness.<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 ==
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" />


== Etiology ==
* Genetic associations, genetic factors classified into: HLA genes and non-HLA-related genes.
The cause and trigger of chronic arthritis in JIA remain unclear. 
** 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>   
# Abnormal [[Immune System|immune]] responses triggered by the interactions between environmental factors in a [[Genetic Conditions and Inheritance|genetically]] susceptible individual is speculative.  
* Environmental factors (e.g. infectious agents, antibiotics, stress etc.) may be risk factors
# JIA is not hereditary – it is not passed on from parent to child. It is rare for two children in the same family to have JIA, although this can happen<ref name=":0">Arthritis org. JIA Available:https://www.arthritisact.org.au/juvenile-arthritis/ (accessed 18.10.2021)</ref>.  
** 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.
# Some environmental factors such as [[Antibiotics|antibiotic]] exposure and [[Cesarean Section|C-section deliveries]] are potential risks; however, breastfeeding and household siblings are possible protectives.<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>
* 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" />  


== Pathology ==
== Characteristics and Clinical Presentation ==
[[Image:JIA-prevalence.jpg|border|399x399px|Prevalence of subtypes of JIA|right|alt=|frameless]]There are several subtypes of JIA:
[[File:Anterior-uveitis.jpg|thumb|231x231px|Anterior-uveitis]]
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" />


1.Oligoarticular JIA (Pauciarticular)
* Synovitis
* Joint effusion
* Swelling of soft tissues
* Osteopenia
* Bone oedema, erosions


* affected ≤4 joints in the first six months of illness
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" />
* peak age: 1-6 years
* mainly affects medium and large joints


2. Polyarticular JIA (pJIA)
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>
* Pain and stiffness, including morning stiffness
* Fatigue


* ≥5 joints are affected
*[[Fever]]
* peak age: 1-4 years; 7-10 years
* Skin rash
* mainly affects small and medium joints
* Generally feeling unwell (malaise)
* Uveitis (see image)
* Decreased range of motion and soft tissue contractures
* Muscle atrophy
* Weakness, or reduced muscular endurance
* Gait deviations


3. Systemic onset JIA (Still's disease, not to be confused with adult onset Still's disease)
== 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" />
* 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>
* Medical history
 
* Physical examination
4. Enthesitis-related JIA
*[[Blood Tests|Laboratory tests]] - typical inflammatory markers are said to be common, particularly in oligoarthritis<ref name=":1" />
 
* Imaging
5. Psoriatic JIA
** 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
6. Undifferentiated JIA<ref name=":2" />.<sup></sup>'''Image 2: Prevalence of major subtypes of JIA'''<ref name="Pathology" />  
** MRI (the gold standard)
== Characteristics/Clinical Presentation  ==
* Eye examination
[[Image:Uveitis.jpg|alt=|right]]
== Pathology ==
The symptoms the child experiences will depend on the type of arthritis they have. Common symptoms include:
[[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" />
 
* 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.
* Less common symptoms include:
* fever
* rash
* feeling generally unwell
* eye inflammation (uveitis)<ref name=":2" />,see '''image 3.'''
 
== Medications  ==
 
An aggressive course of medications is now accepted as the standard of care for JIA. A larger factor in the symptoms management with medications is dealing with the adverse side-effects. The main goals of treatment with medications are pain control, joint preservation (mobility and function), assist in the normal growth of the child, decrease and control other systemic symptoms of JIA and in some cases the disease is prevented from progressing.<ref name="Pathology" />  


The following categories of medications are currently used in the treatment of JIA<ref name="Mayo Clinic">Mayo Clinic. Disease fact sheet: juvenile rheumatoid arthritis.  http://www.mayoclinic.com/health/juvenile-rheumatoid-arthritis/DS00018 (accessed 10 March 2011)</ref>:
# 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
# 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


*Disease-modifying antirheumatic drugs or DMARDs - [Methotrexate:Rheumatrex; Sulfasalazine: Azulfidine] used in combination with NSAIDs, slow the progression of JIA {side effects: nausea and liver problems}
== Differential Diagnosis ==
*Immunosuppressants - slow inflammation reactions {side effects: increased risk of infection and other immune threats}
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>   
*Tumor necrosis factor (TNF) inhibitors - [Etanercept:Enbrel; Infliximab: Remicade] biologic agents that help reduce pain, joint swelling and morning stiffness. {side effects: increased risk of infection and cancer}
*[http://www.physio-pedia.com/index.php5?title=Lyme_Disease Lyme Disease]
*NSAIDs - [ibprofen:Advil, Motrin; naproxen:Aleve] reduce pain and swelling {side effects: bleeding, liver and stomach problems}
*Rheumatic fever
*Corticosteroids - [Prednisone] for those with severe JIA, used to control symptoms until DMARDs take effect or to prevent complications of JIA. {side effects: interfere with growth hormone, increase risk of infection} 
*[http://www.physio-pedia.com/index.php5?title=Osteomyelitis Osteomyelitis]
*Analgesics - [Acetominophen, Tramadol, Codeine, Opiates] used to reduce pain when a child cannot take NSAIDs due to hypersensitivity, ulcers, liver or stomach irritations, or interactions with other drugs, the downside with analgesics they have no effect of inflammation, swelling or joint destruction. {side effects: liver problems}<ref name="eMedicine" />
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001450/ Psoriatic arthritis]
 
*Inflammatory bowel disease
[[Image:JIA1.jpg|3x2px]]
*Leukemia
 
*[http://emedicine.medscape.com/article/406697-overview#a01 Caffey Disease]
[[Image:JIA1.jpg|border|center|400x300px]]                                                                                                                                            <ref name="eMedicine" /> 
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001133/ Chronic Eosinophilic Granuloma]
 
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]
== Diagnostic Tests/Lab Tests/Lab Values  ==
 
JIA is diagnoses according to patient and family medical history, symptoms, number of joints involved, lab tests and sometimes imaging. 
 
Juvenile idiopathic arthritis is diagnosed using a number of tests including: 
 
* medical history
* physical examination
* 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" />
 
== Systemic Involvement  ==
 
See each subtype of JIA [http://www.physio-pedia.com/index.php5?title=Juvenile_Rheumatoid_Arthritis#Characteristics.2FClinical_Presentation above]. 
 
== Medical Management ==
 
The goal of treatment of JIA is to stop or slow the progression of inflammation, relieving symptoms (swelling, pain, stiffness), improving function (maintain full/functional ROM), and prevent further joint damage.<ref name="eMedicine" /> These goals are met by finding the correct combination of medications that best benefit the child. 
 
The most common types of medications used for JIA are:
 
* Analgesics – for temporary pain relief
* 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 – used to quickly control or reduce 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) – 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<ref name=":2" />.  
 
== Physical Therapy Management  ==
 
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<ref name="eMedicine" />:
 
*Muscle tone
*Strengthening
*Range of motion
*Stretching
*Education on joint protection
*Home exercise plan
*Education on pain reducing techniques
*Muscle relaxation techniques
*Splints or 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>
 
Some modalities that can be used to help reduce symptoms such as pain are:  
 
*Ultrasound
*Paraffin wax dips (hands and feet primarily)
*Moist compress (hot pack)
*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
*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" />


== Case Reports/ Case Studies  ==
=== Physiotherapy Management ===
 
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" />   
*Condon C, Gormley J, Hussey J. [http://web.ebscohost.com.libproxy.bellarmine.edu/ehost/pdfviewer/pdfviewer?sid=708c0bf9-02f6-4d30-9f19-635e47287b83%40sessionmgr10&vid=15&hid=9 A review of the physical activity levels of children with juvenile arthritis]. Physical Therapy Reviews [serial online]. December 2009;14(6):411-417. Available from: Academic Search Premier, Ipswich, MA. 
*Hoffart C., Sherry D.. [http://proquest.umi.com.libproxy.bellarmine.edu/pqdweb?did=2096479861&sid=1&Fmt=6&clientId=1870&RQT=309&VName=PQD proquest.umi.com.libproxy.bellarmine.edu/pqdweb]. The Journal of Musculoskeletal Medicine [serial online]. 2010;27:106-108. Available from: ProQuest Nursing &amp; Allied Health Source. Document ID: 2096479861. 
*Hutzal C, Wright F, Stephens S, Schneiderman-Walker J, Feldman B. [http://ejournals.ebsco.com/Direct.asp?AccessToken=23S9M998SWHZBYBXYSXWZEZ21SZM813AM3&Show=Object A Qualitative Study of Fitness Instructors' Experiences Leading an Exercise Program for Children with Juvenile Idiopathic Arthritis]. Physical &amp; Occupational Therapy in Pediatrics [serial online]. November 2009;29(4):409-425. Available from: Academic Search Premier, Ipswich, MA.  
*Fragala-Pinkham M, Dumas H, Barlow C, Pasternak A. [http://journals.lww.com/pedpt/Fulltext/2009/02110/An_Aquatic_Physical_Therapy_Program_at_a_Pediatric.12.aspx An aquatic physical therapy program at a pediatric rehabilitation hospital: a case series]. Pediatric Physical Therapy [serial online]. March 2009;21(1):68-78. Available from: CINAHL with Full Text, Ipswich, MA.
 
== Resources    ==
 
*National Center for Biotechnology Information, U.S. National Library of Medicine  [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001487/ www.ncbi.nlm.nih.gov/pubmedhealth] 
*eMedicine from WebMD  [http://emedicine.medscape.com/article/409980-overview emedicine.medscape.com/]
*Arthritis Foundation  [http://www.arthritis.org/ www.arthritis.org/]
*Arthritis Today  [http://www.arthritistoday.org/ www.arthritistoday.org/]
*Kids Health  [http://kidshealth.org/ kidshealth.org/] 
*Rheumatology Image Bank [http://images.rheumatology.org/albums.php?albumId=75693&page=1 images.rheumatology.org/albums.php]
 
Clinical Trials: 


*Juvenile Rheumatoid Arthritis Research Registry (Sponsored by National Institute of Arthritis and Musculoskeletal and Skin Diseases, Cincinnati Children's Hospital Medical Center, Cinncinnati, OH) [http://clinicaltrials.gov/ct/show/NCT00090571 clinicaltrials.gov/ct/show/NCT00090571]
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>
*Current JRA clinical trials: [http://www.clinicaltrials.gov/ct2/results?term=juvenile+arthritis www.clinicaltrials.gov/ct2/results]  
*[[Strength Training|strengthening]]
*[[Range of Motion|range of motion]] exercises and [[stretching]]
*education on joint protection and pain reducing techniques
*[[Adherence to Home Exercise Programs|home exercise programme]]
*muscle relaxation techniques
*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>
*adaptive equipment
Some modalities that might be used to help symptoms such as pain include:
*[[Ultrasound therapy|ultrasound]]
*paraffin wax dips (primarily for hands and feet)
*moist compress ([[Thermotherapy|hot packs]])
*[[Aquatherapy|hydrotherapy]]
*cold packs


== References  ==
== Other Professionals involved in Managment of Juvenile Arthritis ==
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>.


see [[Adding References|adding references tutorial]].  
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 />  


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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, Mande Jooste, Wendy Walker, George Prudden, WikiSysop and Meaghan Rieke

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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