Juvenile Rheumatoid Arthritis

Definition/Description

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:

  • Pauciarticular/Oligoarthritis JIA
  • Polyarthritis JIA (positive RF)
  • Polyarthritis JIA (negative RF)
  • Systemic onset JIA
  • Psoriatic JIA
  • Enthesitis-related arthritis
  • Undifferentiated 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. [1][2]

Prevalence

JIA affects 30,000 to 50,000 children in the United States.[2] For unknown reasons, it has been shown to be more prevalent in Norway and Australia.[3] JIA occurs in children under the age of 16 and affects more than twice as many girls as their male counterparts.

  • Girls with an oligoarticular onset outnumber boys by a ratio of 3:1.
  • 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.[3]

The mean age of onset is 1-3 years old.[3]

Prevalence of subtypes of JIA

[2]Prevalence of major subtypes of JIA

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.[4]

Characteristics/Clinical Presentation

[[Image:JIA-rash2.jpg|frame|right|http://search.creativecommons.org/?q=juvenile%20rheumatoid%20arthritis]

Symptoms common among most subtypes of JIA[4][3]:

  • Patient's may experience periods of remission (no symptoms) and flare ups (worsening symptoms)
  • Joint pain, stiffness and swelling
    • More severe in the morning or following naps
    • Commonly in, but not limited to knees, hands and feet
  • Loss of joint function (reduced range of motion/ROM)[2]
  • Limp, due to pain in lower extremities
  • Joint deformities, abnormal growth (i.e. leg length discrepancies)
  • Eye irritations/inflammation (uveitis)
  • Recurrent fevers (during flare ups)
  • Skin rash
  • Myalgia (muscle aches)
  • Weight loss
  • Growth problems (slow or fast growth, overall or in affected limb/joint)
http://search.creativecommons.org/?q=juvenile%20rheumatoid%20arthritis Swollen right knee in JIA patient
Uveitis.jpg
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)[2]
  • Most common in girls younger than 8 years old[3]
  • Affects less than 4 joints
  • Most often affects large joints (knees, elbows, wrists, ankles, shoulders, hips)[2]
  • Commonly asymmetrical pattern[2]
  • Child might exhibit swollen joints and/or limb/gait abnormalities, most common upon waking[2]
  • Leg length discrepancy common
  • Those diagnosed are 20-30% more likely to develop inflammatory eye problems[3]
  • Three subtypes[2]
  1. ANA (antinuclear antibodies) present - there is an increased risk of developing uveitis
  2. Juvenile AS (ankylosing spondylitis) - characterized by spinal involvement and commonly a positive HLA-B27 gene
  3. Exclusively joint involvement
Polyarticular JIA (PoJIA)
  • Makes up 30-40% of all JIA cases[2]
  • More common in girls at any age
  • Affects 5 or more joints
  • Both large and small joints, but more commonly affects small joints[3][2]
  • Commonly affects joint in a symmetrical pattern[2]
  • PoJIA is most like adult RA
  • Two subtypes[2][4]
  1. Rheumatoid factor positive (RF+) - patient tests positive on at least two tests at least three months apart[4]. Subcutaneous nodules, cervical spine fusion, chronic uveitis, and destructive hip disease occur RF+ PoJIA.[2]
  2. Rheumatoid factor negative (RF-) - patient tests negative on all tests for the RF.[4] Morning stiffness, fatigue and possibly a low grade fever, along with joint involvement are typical.[2]
Systemic-onset JIA (SoJIA)
  • Also called Still's disease
  • Makes up 10-20% of all JIA cases[2]
  • Affects both genders equally[2]
  • Onset is most often in early childhood[3]
  • Symptoms can be present in any number of joints and is the most severe type of JIA in that it has many extraarticular manifestations[2]
  • Symptoms of Still's disease include[2]
    • 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[2]
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.[2]
  • 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)[3]
  • Enthesitis most common at Achilles tendon, spine and knee[2]
  • Pt diagnosed will have both arthritis and enthesitis OR have either arthritis or enthesitis with at least two of the following:[2]
    • 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 [3]
    • Acute uveitis
    • Polyarticular or pauciarticular arthritis in boys older than 6 years[3]
Undifferentiated arthritis[3]
  • 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 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.[2]

The following categories of medications are currently used in the treatment of JIA[5]:

  • 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}[3]

JIA1.jpg

JIA1.jpg
[3]

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.[3]
  • 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.[3][4]
  • 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.[3]
  • 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.[3]
  • 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.[4]

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. [3][5]
  • 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.[3]


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.[2]

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.[3]

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.[4]

Systemic Involvement

See each subtype of JIA 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.[3] 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:[4]

  • 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[3]

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[3]:

  • 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[4]

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[2]:

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[3][6]:

Case Reports/ Case Studies

Resources

Clinical Trials:

References

see adding references tutorial.

  1. Goodman, Snyder. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2007.
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 2.17 2.18 2.19 2.20 2.21 2.22 2.23 2.24 2.25 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. . St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2009.
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 3.17 3.18 3.19 3.20 3.21 3.22 3.23 3.24 eMedicine. Disease fact sheet: juvenile rheumatoid arthritis. http://emedicine.medscape.com/article/409980-overview (accessed 10 March 2011)
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 4.9 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)
  5. 5.0 5.1 Mayo Clinic. Disease fact sheet: juvenile rheumatoid arthritis. http://www.mayoclinic.com/health/juvenile-rheumatoid-arthritis/DS00018 (accessed 10 March 2011)
  6. 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)
[[Category:Paediatrics]