Introduction
Juvenile idiopathic arthritis (JIA) or juvenile rheumatoid arthritis (JRA) is a type of arthritis affecting children. Juvenile idiopathic arthritis is an autoimmune disease affecting one or more joints of children aged 16 or below. An autoimmune disorder is a medical condition in which the immune system attacks and kills the body's normal cells by mistake. Rheumatoid arthritis is a chronic inflammatory and autoimmune disorder that causes pain and swelling in the lining of the joints. It commonly affects the joints of fingers, knees, wrists, ankles, elbows, shoulders, and hips. In adults, rheumatoid arthritis is chronic and lifelong, but children outgrow juvenile rheumatoid arthritis. Sometimes juvenile idiopathic arthritis affects bone development in growing children.
What Are the Different Types of Juvenile Idiopathic Arthritis?
Types of juvenile idiopathic arthritis are:
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Oligoarthritis- Oligoarthritis commonly affects one to four joints in the first six months of the onset. If no more joints are involved further, it is known as the persistent type; if more joints are involved after six months, it is known as the extended type.
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Polyarticular- Polyarticular juvenile idiopathic arthritis (JIA) type affects five or more joints in the first six months of the onset. It is the second most common type of JIA. It causes fever and painful swelling in the wrists, ankles, toes, fingers, knees, hips, jaw, and neck. A blood test for RF (rheumatoid factor) can determine whether it is RF positive or RF negative.
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Systemic- Systemic arthritis is the least common type of arthritis that affects one or more joints and also causes skin rashes and high spiking fever that lasts at least two weeks. It also affects internal organs such as the liver, heart, spleen, and lymph nodes. It occurs in one in ten children with JIA.
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Psoriatic Arthritis- In psoriatic arthritis, children might have joint pain along with reddish, scaly skin rashes on the scalp, elbow, behind the ears, and knee. It commonly causes inflammation of the toes and fingers and pits in the fingernails.
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Enthesitis Related- In this type, children have arthritis along with enthesis. Enthesis affects areas where the tendon or ligament joins the bone, most common in the knees, feet, and hips.
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Undifferentiated- Undifferentiated arthritis has symptoms of two or more types of juvenile idiopathic arthritis, or sometimes it does not match with any of the JIA types.
What Are the Signs and Symptoms of Juvenile Idiopathic Arthritis?
The symptoms of juvenile idiopathic arthritis vary in children. They might appear only during flare-ups or sometimes chronic. They are:
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Joint pain.
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Swollen joints.
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Fever.
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Rashes.
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Swollen lymph nodes.
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Fatigue (tiredness).
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Redness and warmth in joints.
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Decreased appetite.
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Weight loss.
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Decreased ability to use one or more joints.
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Swollen and dry eyes.
What Are the Most Commonly Involved Joint Structures in Juvenile Idiopathic Arthritis?
The most commonly involved joint structures in juvenile idiopathic arthritis are:
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Knee - The knee is the most commonly affected joint in juvenile idiopathic arthritis. Knee joint involvement is characterized by painful, swollen, warm joints with decreased function. The common imaging features of JIA in the knee joint are joint effusion (fluid collection in the joint), synovial thickening, and bone marrow edema. Sometimes bone erosions, cartilage loss, tendinopathy, and bone cysts are also observed.
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Hip - Juvenile idiopathic arthritis affects the hip joint in about 20 to 50 percent of children, and it becomes irreversible destructive damage within five years. The hip joint shows tendinitis, bursitis (swelling of the small, fluid-filled sacs [bursae]), and synovitis (swelling of the synovium).
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Spine - Cervical spine is most commonly involved in JIA. Contrast-enhanced MRI shows joint effusion, cervical spine arthritis, bone marrow edema, and synovial thickening. In the later stage, it shows subluxation (incomplete dislocation of the joint), erosions, joint ankylosis (stiffness of the joints due to fusion), and nerve compression.
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Sacroiliac Joint - Sacroiliac joints are involved in about 30 percent of children in JIA, which are not affected in the early stage. Magnetic resonance imaging (MRI) is an excellent imaging tool for detecting the inflammation of sacroiliac joints. In addition, MRI shows bone marrow edema, fatty deposition, and synovitis.
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Temporomandibular Joint - Contrast-enhanced MRI is used to detect TMJ involvement in JIA. MRI shows erosions, bone marrow edema, synovial thickening, and joint effusion. MRI is taken in both open and closed-mouth sequences to detect JIA.
What Are the Imaging Techniques Used in the Diagnosis of Juvenile Idiopathic Arthritis?
The imaging techniques used in the diagnosis of juvenile idiopathic arthritis are:
Plain Radiograph:
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In plain radiograph, JIA appears as bony erosions, soft tissue swelling, periostitis, growth disturbances, and joint space narrowing.
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Osteopenia, osteoporosis, epiphyseal compression fracture, joint subluxation, intra-articular bony ankylosis, and synovial cysts (fluid-filled sacs) are also seen.
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The major drawback of plain radiography in juvenile idiopathic arthritis is that it does not allow direct examination of synovium, articular cartilage, and non-calcified structures in the joints.
Magnetic Resonance Imaging (MRI):
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Magnetic resonance imaging is the standard imaging tool used in diagnosing juvenile idiopathic arthritis.
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It is effective in distinguishing bony erosions from changes in articular cartilage.
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Bony erosions appear as a loss of low signal intensity of cortical bone and loss of high signal intensity of trabecular bone in T1 weighted images.
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On T2 weighted images, subchondral cysts appear as hyperintense lesions, and bone erosions appear as hypointense lesions.
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The MRI imaging protocol for diagnosing juvenile idiopathic arthritis should include a T1 spin-echo sequence, T1 fat-suppressed sequence precontrast, and post-contrast fat-suppressed sequence. The post-contrast fat-suppressed sequence includes:
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Classic T2 fat-sat.
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Short-tau inversion recovery (STIR).
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DIXON fat-suppression sequence.
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MRI is a highly sensitive radiologic indicator of disease activity. It can show soft tissue swelling, synovial hypertrophy, and the status of the articular cartilage and joint integrity.
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MRI can detect both bone marrow and soft tissue changes.
Ultrasonography:
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Ultrasonography is more sensitive than plain radiography in diagnosing cartilage effusions and erosions, but its sensitivity is based on the operator's efficiency.
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Synovial inflammation appears as a mixed echogenic area lining the articular cartilage.
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The effusion volumes and synovial thickness are measured to evaluate the disease progression.
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Doppler ultrasound is helpful in detecting the blood supply of the synovium.
Conclusion
Plain radiography is the primary imaging modality for the diagnosis and follow-up of juvenile idiopathic arthritis (JIA). Contrast-enhanced MRI sequences are used to improve the visualization of cartilaginous erosions and synovial hypertrophy in inflammatory arthritis. Gadolinium-enhanced T1 weighted (T1W) MRI images are used to distinguish synovitis from joint effusion as both of them are hyperintense on short-tau inversion recovery images (STIR) and T2 weighted (T2W) images. Ultrasonography plays an important role in pediatric rheumatology by monitoring the treatment and guiding joint injections.