Juvenile Idiopathic Arthritis: Difference between revisions
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== Characteristics/Clinical Presentation == | == Characteristics/Clinical Presentation == | ||
Symptoms common among most subtypes of JIA<ref name="NIAMS" /><ref name="eMedicine" />: | |||
*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) | |||
*Limp, due to pain in lower extremities | |||
*Joint deformities, abnormal growth (i.e. leg length discrepancies) | |||
*Eye irritations/inflammation | |||
*Reccurrent fevers (during flare ups) | |||
*Skin rash | |||
*Myalgia (muscle aches) | |||
*Weight loss | |||
*Growth problems (slow or fast growth, overall or in affected limb/joint) | |||
Pauciarticular JIA (PaJIA, Oligoarthritis) meaning "few joints"<br> | Pauciarticular JIA (PaJIA, Oligoarthritis) meaning "few joints"<br> | ||
*Most common type of JIA (50% of all JIA cases)<ref name="Pathology" /> | *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" /> | *Most common in girls younger than 8 years old<ref name="eMedicine" /> | ||
*Affects less than 4 joints | *Affects less than 4 joints | ||
*Most often affects large joints (knees, elbows, wrists, ankles, shoulders, hips)<ref name="Pathology" /> | *Most often affects large joints (knees, elbows, wrists, ankles, shoulders, hips)<ref name="Pathology" /> | ||
*Commonly asymetrical pattern<ref name="Pathology" /> | *Commonly asymetrical pattern<ref name="Pathology" /> | ||
*Child might exhibit swollen joints and/or limb/gait abnormalities, most common upon waking<ref name="Pathology" /> | *Child might exhibit swollen joints and/or limb/gait abnormalities, most common upon waking<ref name="Pathology" /> | ||
*Leg length discrepancy common | *Leg length discrepancy common | ||
*Those diagnosed are 20-30% more likely to develop inflammatory eye problems<ref name="eMedicine" /> | *Those diagnosed are 20-30% more likely to develop inflammatory eye problems<ref name="eMedicine" /> | ||
*Three subtypes<ref name="Pathology" /> | *Three subtypes<ref name="Pathology" /> | ||
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Polyarticular JIA (PoJIA) | Polyarticular JIA (PoJIA) | ||
*Makes up 30-40% of all JIA cases<ref name="Pathology" /> | *Makes up 30-40% of all JIA cases<ref name="Pathology" /> | ||
*More common in girls at any age | *More common in girls at any age | ||
*Affects 5 or more joints | *Affects 5 or more joints | ||
*Both large and small joints, but more commonly affects small joints<ref name="eMedicine" /><ref name="Pathology" /> | *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" /> | *Commonly affects joint in a symmetrical pattern<ref name="Pathology" /> | ||
*PoJIA is most like adult RA | *PoJIA is most like adult RA | ||
*Two subtypes<ref name="Pathology" /><ref name="NIAMS" /> | *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 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" /> | #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" /> | ||
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*Makes up 10-20% of all JIA cases<ref name="Pathology" /> | *Makes up 10-20% of all JIA cases<ref name="Pathology" /> | ||
*Affects both genders equally<ref name="Pathology" /> | *Affects both genders equally<ref name="Pathology" /> | ||
*Onset is most often in early childhood<ref name="eMedicine" /> | *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 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" /> | *Symptoms of Still's disease include<ref name="Pathology" /> | ||
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Psoriatic JIA | 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 tow (dactylitis), nail abnormalities, and a family history of psoriasis.<ref name="Pathology" /> | *Characterized by a diagnosis of arthritis and psoriasis and at least two of the following: inflammation and swelling of an entire finger or tow (dactylitis), nail abnormalities, and a family history of psoriasis.<ref name="Pathology" /> | ||
*Uveitis can be present as well | *Uveitis can be present as well | ||
*Treated by aggressive immunosuppressives | *Treated by aggressive immunosuppressives | ||
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- Positive HLA-B27 | - 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" /> | - 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 | - Acute uveitis | ||
- Polyarticular or pauciarticular arthritis in boys older than 6 years<ref name="eMedicine" /> | - Polyarticular or pauciarticular arthritis in boys older than 6 years<ref name="eMedicine" /> | ||
Undifferentiated arthritis<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 do not fulfill the criteria for any of the other six subcategories | ||
Revision as of 00:41, 4 April 2011
Original Editors - Emily Betz from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Lead Editors - Your name will be added here if you are a lead editor on this page. Read more.
Definition/Description
[edit | edit source]
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:
- 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. [1][2]
Prevalence[edit | edit source]
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]
[insert figure 27-16 Patho]
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[edit | edit source]
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)
- Limp, due to pain in lower extremities
- Joint deformities, abnormal growth (i.e. leg length discrepancies)
- Eye irritations/inflammation
- Reccurrent fevers (during flare ups)
- Skin rash
- Myalgia (muscle aches)
- Weight loss
- Growth problems (slow or fast growth, overall or in affected limb/joint)
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 asymetrical 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]
- 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[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]
- 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]
- 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 tow (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[edit | edit source]
See each subtype of JIA above.
Medications[edit | edit source]
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.[2]
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
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
[edit | edit source]
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[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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Alternative/Holistic Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
Some signs and symptoms of JIA are shared with those of the following diseases[3][6]:
- Lyme Disease
- Rheumatic fever
- Osteomyelitis
- Psoriatic arthritis
- Inflammatory bowel disease
- Leukemia
- Caffey Disease
- Chronic Eosinophilic Granuloma
- Osteoporosis
- Involutional Osteoarthritis
Case Reports/ Case Studies[edit | edit source]
add links to case studies here (case studies should be added on new pages using the case study template)
Resources
[edit | edit source]
National Center for Biotechnology Information, U.S. National Library of Medicine www.ncbi.nlm.nih.gov/pubmedhealth
eMedicine from WebMD emedicine.medscape.com/
Arthritis Foundation www.arthritis.org/
Arthritis Today www.arthritistoday.org/
Kids Health kidshealth.org/
Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
see adding references tutorial.
- ↑ Goodman, Snyder. Differential Diagnosis for Physical Therapists: Screening for Referral. 4th ed. St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2007.
- ↑ 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 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. . St. Louis, MO: Saunders, an imprint of Elsevier Inc, 2009.
- ↑ 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 eMedicine. Disease fact sheet: juvenile rheumatoid arthritis. http://emedicine.medscape.com/article/409980-overview (accessed 10 March 2011)
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 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)
- ↑ Mayo Clinic. Disease fact sheet: juvenile rheumatoid arthritis. http://www.mayoclinic.com/health/juvenile-rheumatoid-arthritis/DS00018 (accessed 10 March 2011)
- ↑ 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)
