Juvenile Idiopathic Arthritis: Difference between revisions

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<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div> <div class="editorbox">
'''Original Editors '''- Emily Betz [[Pathophysiology of Complex Patient Problems|from Bellarmine University's Pathophysiology of Complex Patient Problems project.]]  
'''Original Editors '''- Emily Betz&nbsp;[[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  


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== Definition/Description<br>  ==
== Introduction ==
 
[[File:Juvenile arthritis.jpg|alt=|thumb|419x419px|'''Figure.1''' Swollen joints in Patient with JIA]]
Juvenile Idiopathic Arthritis (JIA), formerly Juvenile Rheumatoid Arthritis (JRA) is a chronic inflammatory disorder that occurs before the age 16 and can occur in all races. The heterogeneous group of diseases that JIA refers to all share synovitis as a common symptom. The subcategories are:
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>  
 
*Oligoearthritis JIA  
*Polyarthritis JIA (positive RF)
*Polyarthritis JIA (negative RF)  
*Systemic onset JIA
*Psoriatic JIA
*Enthesitis-related arthritis
*Other arthritis
 
JIA is an autoimmune disorder that is classified based on the number of joints involved/affected and the presence of other signs and symptoms.&nbsp;<ref name="Differential Diagnosis">Goodman, Snyder.  Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed.  St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2007.</ref><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>
 
== Prevalence  ==
 
JIA affects 30,000 to 50,000 children in the United States.<ref name="Pathology" /> 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> JIA occurs in children under the age of 16 and affects more than twice as many girls as their male counterparts. <br>
 
*Girls with an oligoarticular onset outnumber boys by a ratio of 3:1.<br>
*In children with uveitis, the ratio of girls to boys is 5-6.6:1.
*Among children with polyarticular onset, girls outnumber boys by 2.8:1.
*In striking contrast, systemic onset occurs with equal frequency in boys and girls.<ref name="eMedicine" />
 
The mean age of onset is 1-3 years old.<ref name="eMedicine" />
 
<sup></sup>
 
[insert figure 27-16 Patho]
 
<br>
 
According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) and the National Arthritis Data Workgroup, in 2008 the US had roughly 294,000 children that had arthritis or other rheumatic conditions.<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>
 
== Characteristics/Clinical Presentation  ==
 
add text here <br>
 
== Associated Co-morbidities  ==
 
add text here <br>  


== Medications  ==
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>


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. Disease-modifying antirheumatic drugs or DMARDs [Methotrexate], immunosuppressants, tumor necrosis factor (TNF) inhibitors (biologic agent [Enbrel]), infliximab [Remicadel], NSAIDs and corticosteroids.<ref name="Pathology" /><br>
* 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" />


== Diagnostic Tests/Lab Tests/Lab Values ==
* 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).


JIA is diagnoses according to patient and family medical history, symptoms, number of joints involved, lab tests and sometimes imaging.<br>  
== Epidemiology ==
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" />


Lab Tests:<br>  
* Females are at higher risk of JIA than males<ref name=":4" />
* The oligoarticular subtype is more common<ref name=":4" />


*Erythrocyte Sedimentation Rate (ESR or sed rate): A nonspecific marker for JIA, it is used more to rule out other conditions and to aid in determining the classification of JIA subtype. An elevated rate can indicate inflammation. In systemic JIA the sed rate will be elevated, polyarticular JIA usually results in an elevated sed rate, and the sed rate is often normal in pauciarticular JIA.<ref name="eMedicine" /><br>
== Characteristics and Clinical Presentation ==
*Anti-Nuclear Antibody (ANA): Detects the anti-nuclear bodies that are commonly present in those with an autoimmune diease. In pauciarticular JIA the ANA usually tests positive. In those with systemic JIA the ANA usually tests negative. ANA is more likely to test positive in diseases that could be a differential diagnosis for JIA (SLE or scleroderma) than it would in JIA. A positive ANA that is extremely elevated could indicate an increased risk of eye involvement and may indicate that JIA could progress to an adult-type SLE.<ref name="eMedicine" /><ref name="NIAMS" /><br>
[[File:Anterior-uveitis.jpg|thumb|231x231px|Anterior-uveitis]]
*Complete Blood Count (CBC): A nonspecific test for JIA. Anemia is common in children with JIA, therefore a low level of hemoglobin could be detected. White blood cells and platelets are often normal in those with JIA.<ref name="eMedicine" /><br>
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" />
*Rheumatoid Factor (RF): Though more commonly found in adults with RA, a positive RF could be found in those with polyarticular JIA. RF is usually used to aid in classification of the JIA subtype. A positive RF could indicate the JIA will progress to adult RA.<ref name="eMedicine" /><br>
*Anti-Cyclic Citrullinated Peptide (anti-CCP) Antibodies: Can be detected in healthy individuals before the onset of RA, and can predict the development of undifferentiated arthritis into rheumatoid arthritis.<ref name="NIAMS" /><br>


Imaging: Though imaging is nonspecific for testing for JIA it can show damage to the joint, fractures, tumors, infection and/or congenital defects all in which would help to narrow down or rule out diseases with similar signs and symptoms.
* Synovitis
* Joint effusion
* Swelling of soft tissues
* Osteopenia
* Bone oedema, erosions


*X-rays<br>
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" />  
*Bone scan<br>
*MRI<br>
*CT scan<br>
*Duel-Energy X-Ray Absorptiometry (DEXA)<br>


Other Tests:  
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


*Arthrocentesis: Often known as joint aspiration. Removing and testing the synocial fluid of affected joints can be used to rule out infection and aid in identifying the cause of the arthritis. <ref name="eMedicine" /><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>
*[[Fever]]
*Synovial Biopsy: Removal of a small portion of the synovial tissue from the affected joint can aid in determining the cause of the inflammation and synovial damage.<ref name="eMedicine" />
* 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


<br>
== 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" />


== Etiology/Causes<br> ==
# 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


Etiology is largely unkown. Some evidence supports enviromental triggers, viral or bacterial infections or genetic predisposition. With further research there are hopes of identifying molecular biomarkers to aid in the diagnosis and treatment of JIA.<ref name="Pathology" />
== Differential Diagnosis ==
 
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>
The disease is accepted to be an autoimmune disease, which means that the body's immune system attacks it's own tissue, instead of foreign tissues. In JIA the body's immune cells and autoanitbodies attack the synovium. As a reaction to the attack the synovium becomes inflammed and irritated. This reaction causes the synovium to thicken and grow abnormally, this abnormal growth eventually will cause damage to the bone and cartilage of the joint and surrounding tissues.<ref name="eMedicine" />
*[http://www.physio-pedia.com/index.php5?title=Lyme_Disease Lyme Disease]
 
*Rheumatic fever
Researchers are beginning to believe that not all forms of JIA are autoimmune. Systemic JIA is now being more accurately defines at an autoinflammatory disease as opposed to autoimmune. This means that while the body's immune system is still over active, like in autoimmune diseases, the inflammation and immune acticity is not caused by autoantibodies but rather by a different part of the immune system that when working properly should cause white blood cells to destroy harmful invaders. When this system is not working properly it causes inflammation, fever and rash for unknown reasons.<ref name="NIAMS" />
*[http://www.physio-pedia.com/index.php5?title=Osteomyelitis Osteomyelitis]
 
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001450/ Psoriatic arthritis]
== Systemic Involvement  ==
*Inflammatory bowel disease
 
*Leukemia
add text here
*[http://emedicine.medscape.com/article/406697-overview#a01 Caffey Disease]
 
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001133/ Chronic Eosinophilic Granuloma]
== Medical Management (current best evidence)  ==
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]
 
add text here
 
== Physical Therapy Management (current best evidence)  ==
 
add text here
 
== Alternative/Holistic Management (current best evidence)  ==
 
add text here
 
== 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>:<br>
 
*Lyme Disease  
*Rheumatic fever  
*Osteomyelitis  
*Psoriatic arthritis  
*Inflammatory bowel disease  
*Leukemia  
*Caffey Disease  
*Chronic Eosinophilic Granuloma  
*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" />


<br>
=== 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" />  


== Case Reports/ Case Studies  ==
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>
 
*[[Strength Training|strengthening]]
add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
*[[Range of Motion|range of motion]] exercises and [[stretching]]
 
*education on joint protection and pain reducing techniques
== Resources <br>  ==
*[[Adherence to Home Exercise Programs|home exercise programme]]
 
*muscle relaxation techniques
National Center for Biotechnology Information, U.S. National Library of Medicine&nbsp;&nbsp; [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001487/ www.ncbi.nlm.nih.gov/pubmedhealth]<br>
*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
eMedicine from WebMD&nbsp; [http://emedicine.medscape.com/article/409980-overview emedicine.medscape.com/]
Some modalities that might be used to help symptoms such as pain include:
 
*[[Ultrasound therapy|ultrasound]]
Arthritis Foundation&nbsp; [http://www.arthritis.org/ www.arthritis.org/]
*paraffin wax dips (primarily for hands and feet)
 
*moist compress ([[Thermotherapy|hot packs]])
Arthritis Today&nbsp;&nbsp; [http://www.arthritistoday.org/ www.arthritistoday.org/]
*[[Aquatherapy|hydrotherapy]]
 
*cold packs
Kids Health&nbsp;&nbsp; [http://kidshealth.org/ kidshealth.org/]<br>
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
 
see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]  
<div class="researchbox">
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1f9BUYPXGXDGlcJqrcoM0CONmQgP38sXnr9HQVTxPXD6gAGs1c|charset=UTF-8|short|max=10</rss>  
</div>


== 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 />  


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

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, Kim Jackson, Khloud Shreif, 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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