Juvenile Idiopathic Arthritis: Difference between revisions

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== Definition/Description  ==
== Introduction ==
[[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>


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:  
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>


*Pauciarticular/Oligoarthritis JIA
* Oligoarticular
*Polyarthritis JIA (positive RF)
* Polyarticular
*Polyarthritis JIA (negative RF)
* Systemic
*Systemic onset JIA
* Psoriatic
*Psoriatic JIA
* Enthesitis-related
*Enthesitis-related arthritis
* Undifferentiated
*Undifferentiated arthritis
== 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" /> 


JIA is an autoimmune disorder that is classified based on the number of joints involved/affected and the presence of other signs and symptoms. <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>
* 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).


== Prevalence ==
== 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" />


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


*Girls with an oligoarticular onset outnumber boys by a ratio of 3:1.
== Characteristics and Clinical Presentation ==
*In children with uveitis, the ratio of girls to boys is 5-6.6:1.  
[[File:Anterior-uveitis.jpg|thumb|231x231px|Anterior-uveitis]]
*Among children with polyarticular onset, girls outnumber boys by 2.8:1.  
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" />
*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" />
* Synovitis
* Joint effusion
* Swelling of soft tissues
* Osteopenia
* Bone oedema, erosions


<sup></sup>  
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" />  


[[Image:JIA-prevalence.jpg|border|300x300px|Prevalence of subtypes of JIA|right]
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


<ref name="Pathology" />Prevalence of major subtypes of JIA
*[[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


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>  
== 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" />


== Characteristics/Clinical Presentation  ==
# 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


[[Image:JIA-rash2.jpg|frame|right|http://search.creativecommons.org/?q=juvenile%20rheumatoid%20arthritis]
== 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>   
Symptoms common among most subtypes of JIA<ref name="NIAMS" /><ref name="eMedicine" />:
*[http://www.physio-pedia.com/index.php5?title=Lyme_Disease Lyme Disease]
* Patient's may experience periods of remission (no symptoms) and flare ups (worsening symptoms)
*Rheumatic fever
* Joint pain, stiffness and swelling
*[http://www.physio-pedia.com/index.php5?title=Osteomyelitis Osteomyelitis]
** More severe in the morning or following naps
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001450/ Psoriatic arthritis]
**Commonly in, but not limited to knees, hands and feet                       
*Inflammatory bowel disease
*Loss of joint function (reduced range of motion/ROM)<ref name="Pathology" />                       
*Leukemia
*Limp, due to pain in lower extremities                       
*[http://emedicine.medscape.com/article/406697-overview#a01 Caffey Disease]
*Joint deformities, abnormal growth (i.e. leg length discrepancies)                       
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001133/ Chronic Eosinophilic Granuloma]
*Eye irritations/inflammation ([http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002000/ uveitis])                       
*[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001400/ Osteoporosis]
*Recurrent fevers (during flare ups)                       
*Skin rash                       
*Myalgia (muscle aches)                       
*Weight loss                       
*Growth problems (slow or fast growth, overall or in affected limb/joint)                       
[[Image:JIA-swollenjoint.jpg|200x300px|http://search.creativecommons.org/?q=juvenile%20rheumatoid%20arthritis]]  Swollen right knee in JIA patient            [[Image:Uveitis.jpg|center]]                                                            Uveitis - eye irritation related to JIA
 
=== Subcategories of JIA ===
 
==== Pauciarticular JIA (PaJIA, Oligoarthritis) meaning "few joints"  ====
 
*Most common type of JIA (50% of all JIA cases)<ref name="Pathology" />
*Most common in girls younger than 8 years old<ref name="eMedicine" />
*Affects less than 4 joints
*Most often affects large joints (knees, elbows, wrists, ankles, shoulders, hips)<ref name="Pathology" />
*Commonly asymmetrical pattern<ref name="Pathology" />
*Child might exhibit swollen joints and/or limb/gait abnormalities, most common upon waking<ref name="Pathology" />
*Leg length discrepancy common
*Those diagnosed are 20-30% more likely to develop inflammatory eye problems<ref name="eMedicine" />
Three subtypes:<ref name="Pathology" />
#ANA (antinuclear antibodies) present - there is an increased risk of developing uveitis
#Juvenile AS (ankylosing spondylitis) - characterized by spinal involvement and commonly a positive HLA-B27 gene
#Exclusively joint involvement
 
==== Polyarticular JIA (PoJIA)  ====
 
*Makes up 30-40% of all JIA cases<ref name="Pathology" />
*More common in girls at any age
*Affects 5 or more joints
*Both large and small joints, but more commonly affects small joints<ref name="eMedicine" /><ref name="Pathology" />
*Commonly affects joint in a symmetrical pattern<ref name="Pathology" />
*PoJIA is most like adult RA
Two subtypes<ref name="Pathology" /><ref name="NIAMS" />
# Rheumatoid factor positive (RF+) - patient tests positive on at least two tests at least three months apart<ref name="NIAMS" />. Subcutaneous nodules, cervical spine fusion, chronic uveitis, and destructive hip disease occur RF+ PoJIA.<ref name="Pathology" />
# Rheumatoid factor negative (RF-) - patient tests negative on all tests for the RF.<ref name="NIAMS" /> Morning stiffness, fatigue and possibly a low grade fever, along with joint involvement are typical.<ref name="Pathology" />
 
==== Systemic-onset JIA (SoJIA)  ====
 
*Also called Still's disease
*Makes up 10-20% of all JIA cases<ref name="Pathology" />
*Affects both genders equally<ref name="Pathology" />
*Onset is most often in early childhood<ref name="eMedicine" />
*Symptoms can be present in any number of joints and is the most severe type of JIA in that it has many extraarticular manifestations<ref name="Pathology" />
*Symptoms of Still's disease include<ref name="Pathology" />
**High-spiking fever
**Rash on thighs and chest (often dissipates within a few hours)
**Lymphadenopathy (enlargement of lymph nodes)
**Hepatosplenomegaly (enlargement of the spleen)
**Polyarthritis
**Pericarditis (inflammation of the pericardium/covering of the heart)
**Pleuritis (inflammation of lung tissues/pleura)
**Peptic ulcer disease
**Hepatitis
**Anemia
**Anorexia
**Weight loss
**Myalgia, myositis
**Tenosynovitis
**Skeletal growth disturbances
*Approximately half of children with SoJIA almost entirely recover while one third of children remain ill<ref name="Pathology" />
 
==== Psoriatic JIA  ====
 
*Characterized by a  diagnosis of arthritis and psoriasis and at least two of the following: inflammation and swelling of an entire finger or toe (dactylitis), nail abnormalities, and a family history of psoriasis.<ref name="Pathology" />
*Uveitis can be present as well
*Treated by aggressive immunosuppressives
 
==== Enthesitis-related JIA  ====
 
*Characterized by inflammation of the enthesis (attachment of tendon, ligament, joint capsule to bone)<ref name="eMedicine" />
*Enthesitis most common at Achilles tendon, spine and knee<ref name="Pathology" />
*Pt diagnosed will have both arthritis and enthesitis OR have either arthritis or enthesitis with at least two of the following:<ref name="Pathology" />
**Sacroiliac inflammation or pain
**Spinal inflammation or pain
**Positive HLA-B27
**Positive family history of either ankylosing spondylitis, enthesitis- related arthritis, inflammation of SI , inflammatory bowel disease, Reiter's syndrome, or acute uveitis <ref name="eMedicine" />
**Acute uveitis
**Polyarticular or pauciarticular arthritis in boys older than 6 years<ref name="eMedicine" />
 
==== Undifferentiated arthritis<ref name="eMedicine" />  ====
 
*If a patient's symptoms do not fulfill the criteria for any of the other six subcategories
*If a patient's symptoms fulfill the criteria for more than one subcategory
 
== Associated Co-morbidities  ==
 
See each subtype of JIA [http://www.physio-pedia.com/index.php5?title=Juvenile_Rheumatoid_Arthritis#Characteristics.2FClinical_Presentation above]. 
 
== 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" />:
 
*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}
*Immunosuppressants - slow inflammation reactions {side effects: increased risk of infection and other immune threats}
*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}
*NSAIDs - [ibprofen:Advil, Motrin; naproxen:Aleve] reduce pain and swelling {side effects: bleeding, liver and stomach problems}
*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} 
*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" />
 
[[Image:JIA1.jpg|3x2px]]
 
[[Image:JIA1.jpg|border|center|400x300px]]                                                                                                                                            <ref name="eMedicine" /> 
 
== 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. 
 
Lab Tests: 
 
*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" /> 
*Anti-Nuclear Antibody (ANA): Detects the anti-nuclear bodies that are commonly present in those with an autoimmune disease. 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" /> 
*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" /> 
*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" /> 
*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" />
 
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.
 
*X-rays 
*Bone scan 
*MRI 
*CT scan 
*Duel-Energy X-Ray Absorptiometry (DEXA)
 
Other Tests:
 
*Arthrocentesis: Often known as joint aspiration. Removing and testing the synovial 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>
*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" />
== Etiology/Causes  ==
 
Etiology is largely unknown. Some evidence supports environmental 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" />
 
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 inflamed 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" />
 
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 activity 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" />
 
== Systemic Involvement  ==
 
See each subtype of JIA [http://www.physio-pedia.com/index.php5?title=Juvenile_Rheumatoid_Arthritis#Characteristics.2FClinical_Presentation above]. 
 
== Medical Management (current best evidence)  ==
 
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 (listed above) that best benefit the child. 
 
JIA should be followed by a rheumatologist (ideally a pediatric rheumatologist) or a family practice physician. A team approach is best when treating children with JIA, a team could include any combination of the following healthcare professionals:<ref name="NIAMS" />
 
*Rheumatologist, pediatric rheumatologist - manage the progression of JIA and develop the correct medical treatment plan specific to the child
*Pediatrician or family practice physician -  monitor the patient's health over all
*Physical therapist - maintain joint function and strength, teach joint protection, energy conservation, minimize muscle pain
*Occupational therapist - help with joint protection, energy conservation, maintain upper extremity (hands) function
*Ophthalmologist - monitor possible eye complications that can accompany JIA and/or are side effects from medications
*Dentist and orthodontist - help to monitor jaw involvement, and aid in oral care due to possible limitations of hand function secondary to JIA
*Counselor or psychologist - aid the child and family in keeping their feelings about the disease and its treatment "sorted out"
*Orthopedic surgeon - in the event that surgery is necessary during the treatment course of JIA
*Dietician - it is important for children with JIA to maintain a healthy weight to ease some of the burden on weight bearing joints. It is also important to ensure the child has a balanced diet with all essential nutrients
*Pharmacist - another level to ensure that the medications prescribed for the treatment of JIA and any other medications the child might be taking do not potentially negatively interact with each other and/or food the child might consume
*Social worker - may help the child and the family to identify helpful resources for the treatment of JIA
*Rheumatology nurse - serve as a way-lay between the doctor and patient in between office visits
*School nurse - help the child manage the disease while at school, if school-age
 
In addition to medications to treat JIA other symptom reducers include:  
 
*Hot or cold packs
*Warm baths or showers
*Gentle ROM
*Staying active to maintain function and ROM, a great form of exercise is swimming because it is low impact and does not require repetitive stress to load bearing joints
*Maintaining a healthy weight is extremely important to reduce the stress of weight bearing joints
*Physical therapy treatments
*Relaxation techniques for releasing muscle tension<ref name="eMedicine" />
 
== Physical Therapy Management (current best evidence)  ==
 
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" />
 
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
 
=== Preferred Practice Patterns<ref name="Pathology" />: ===
 
==== Juvenile Idiopathic (Rheumatoid) Arthritis ====
*4A: Primary Prevention/Risk Reduction for Skeletal Demineralization (low bone density) 
*4E: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Localized Inflammation 
*5B: Impaired Neuromotor Development
 
==== Adult Rheumatoid Arthritis ====
* 4A
* 4B: Impaired Posture (cervical involvement)
*4C: Impaired Muscle Performance 
*4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Dysfunction 
*4H: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Joint Arthroplasty 
*4I: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Bony or Soft Tissue Surgery ( tenosynovectomy, tendon reconstruction) 
*5H: Impaired Motor Function, Peripheral Nerve Integrity, and Sensory Integrity Associate with Nonprogressive Disorders of the Spinal Cord (cervical spine)
*6B: Impaired Aerobic Capacity/Endurance Associated with Deconditioning
 
== 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 />  


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

Latest revision as of 13:45, 27 September 2023

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).