Juvenile idiopathic arthritis
Juvenile rheumatoid arthritis (JRA)Juvenile idiopathic arthritis (JIA) is a term used to describe a group of disorders in children that includes arthritis. They are long-term (chronic) diseases that cause joint pain and swelling. The names describing this group of conditions has changed over the past several decades as more is learned about the condition.
Causes
The cause of JIA is not known. It is thought to be an autoimmune illness. This means the body attacks and destroys healthy body tissue by mistake.
Autoimmune illness
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...
Read Article Now Book Mark ArticleJIA most often develops before age 16. Symptoms may start as early as 6 months old.
The International League of Associations for Rheumatology (ILAR) has proposed the following way of grouping this type of childhood arthritis:
- Systemic-onset JIA. Involves joint swelling or pain, fevers, and rash. It is the least common type but it can be the most severe. It appears to be different than the other types of JIA and is similar to Adult Onset Stills Disease.
- Polyarthritis. Involves many joints. This form of JIA may turn into rheumatoid arthritis. It may involve 5 or more large and small joints of the legs and arms, as well as the jaw and neck. Rheumatoid factor may be present.
- Oligoarthritis (persistent and extended). Involves 1 to 4 joints, most often the wrists, or knees. It also affects the eyes.
- Enthesitis-related arthritis. Resembles spondyloarthritis in adults and often involves the sacroiliac joint.
- Psoriatic arthritis. Diagnosed in children who have arthritis and psoriasis or nail disease, or have a close family member with psoriasis.
Symptoms
Symptoms of JIA may include:
- Swollen, red, or warm joint
- Limping or problems using a limb
- Sudden high fever, which may come back
- Rash (on trunk and extremities) that comes and goes with fever
- Stiffness, pain, and limited movement of a joint
Limited movement
Limited range of motion is a term meaning that a joint or body part cannot move through its normal range of motion.
Read Article Now Book Mark Article - Low back pain that does not go away
- Bodywide symptoms such as pale skin, swollen lymph gland, and a sick appearance
JIA can also cause eye problems called uveitis, iridocyclitis, or iritis. There may be no eye symptoms. When eye symptoms occur, they can include:
Uveitis
Uveitis is swelling and inflammation of the uvea. The uvea is the middle layer of the wall of the eye. The uvea supplies blood for the iris at the ...
Read Article Now Book Mark Article- Red eyes
Red eyes
Eye redness is most often due to swollen or dilated blood vessels. This makes the surface of the eye look red or bloodshot.
Read Article Now Book Mark Article - Eye pain, which may get worse when looking at light (photophobia)
Eye pain
Pain in the eye may be described as a burning, throbbing, aching, or stabbing sensation in or around the eye. It may also feel like you have a forei...
Read Article Now Book Mark Article - Vision changes
Vision changes
There are many types of eye problems and vision disturbances, such as: Halos Blurred vision (the loss of sharpness of vision and the inability to see...
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Exams and Tests
The physical exam may show swollen, warm, and tender joints that hurt to move. The child may have a rash. Other signs include:
- Swollen liver
Swollen liver
Enlarged liver refers to swelling of the liver beyond its normal size. Hepatomegaly is another word to describe this problem. If both the liver and ...
Read Article Now Book Mark Article - Swollen spleen
Swollen spleen
Splenomegaly is a larger-than-normal spleen. The spleen is an organ in the upper left part of the belly.
Read Article Now Book Mark Article - Swollen lymph nodes
Swollen lymph nodes
Lymph nodes are present throughout your body. They are an important part of your immune system. Lymph nodes help your body recognize and fight germ...
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Blood tests may include:
- Rheumatoid factor
Rheumatoid factor
Rheumatoid factor (RF) is a blood test that measures the amount of the RF antibody in the blood.
Read Article Now Book Mark Article - Anti-cyclic citrullinated peptide (CCP) antibody
- Erythrocyte sedimentation rate (ESR)
ESR
ESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures the level of certain protei...
Read Article Now Book Mark Article - Antinuclear antibody (ANA)
ANA
The antinuclear antibody panel is a blood test that looks at antinuclear antibodies (ANA). ANA are antibodies produced by the immune system that bind...
Read Article Now Book Mark Article - Complete blood count (CBC)
CBC
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
Read Article Now Book Mark Article - HLA-B27
Any or all of these blood tests may be normal in children with JIA.
The health care provider may place a small needle into a swollen joint to remove fluid. This can help to find the cause of the arthritis. It can also help relieve pain. The provider may inject steroids into the joint to help reduce swelling.
Other tests that may be done include:
- X-ray of a joint
X-ray
X-rays are a type of electromagnetic radiation, just like visible light. An x-ray machine sends individual x-ray waves through the body. The images...
Read Article Now Book Mark Article - Bone scan
Bone scan
A bone scan is an imaging test used to diagnose bone diseases and find out how severe they are.
Read Article Now Book Mark Article - X-ray of the chest
X-ray of the chest
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
Read Article Now Book Mark Article - ECG
ECG
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
Read Article Now Book Mark Article - Regular eye exam by an ophthalmologist -- This should be done even if there are no eye symptoms.
Treatment
Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen may be enough to control symptoms when only a small number of joints are involved.
Corticosteroids may be used for more severe flare-ups to help control symptoms. Because of their toxicity, long-term use of these medicines should be avoided in children.
Children who have arthritis in many joints, or who have fever, rash, and swollen glands may need other medicines. These are called disease-modifying antirheumatic drugs (DMARDs). They can help reduce swelling in the joints or body. DMARDs include:
- Methotrexate
- Biologic DMARDs such as anti-tumor necrosis factor (TNF) agents, IL-1 inhibitors and IL-6 inhibitors.
Children with JIA need to stay active.
Exercise will help keep their muscles and joints strong and mobile.
- Walking, bicycling, and swimming may be good activities.
- Children should learn to warm up before exercising.
- Talk to the doctor or physical therapist about exercises to do when your child is having pain.
Children who have sadness or anger about their arthritis may need extra support.
Support
The following organizations provide more information on arthritis:American Academy of Orthopaedic Surgeons -- orthoinfo. aaos. org/en/diseases--condi...
Read Article Now Book Mark ArticleSome children with JIA may need surgery, including joint replacement.
Outlook (Prognosis)
Children with only a few affected joints may have no symptoms for a long period.
In many children, the disease will become inactive and cause very little joint damage.
The severity of the disease depends on the number of affected joints. It is less likely that symptoms will go away in these cases. These children more often have long-term (chronic) pain, disability, and problems at school. Some children may continue to have arthritis as adults.
Possible Complications
Complications may include:
- Wearing away or destruction of joints (can occur in people with more severe JIA)
- Slow rate of growth
- Uneven growth of an arm or leg
- Loss of vision or decreased vision from chronic uveitis (this problem may be severe, even when the arthritis is not very severe)
Loss of vision
Blindness is a lack of vision. It may also refer to a loss of vision that cannot be corrected with glasses or contact lenses. Partial blindness mean...
Read Article Now Book Mark ArticleDecreased vision
There are many types of eye problems and vision disturbances, such as: Halos Blurred vision (the loss of sharpness of vision and the inability to see...
Read Article Now Book Mark Article - Anemia
Anemia
Anemia is a condition in which the body does not have enough healthy red blood cells. Red blood cells provide oxygen to body tissues. Different type...
Read Article Now Book Mark Article - Swelling around the heart (pericarditis)
Pericarditis
Pericarditis is a condition in which the sac-like covering around the heart (pericardium) becomes inflamed.
Read Article Now Book Mark Article - Long-term (chronic) pain, poor school attendance
- Macrophage activation syndrome, a severe illness that may develop with systemic JIA
When to Contact a Medical Professional
Contact your provider if:
- You, or your child, notice symptoms of JIA
- Symptoms get worse or do not improve with treatment
- New symptoms develop
Prevention
There is no known prevention for JIA.
References
Beukelman T, Nigrovic PA. Juvenile idiopathic arthritis: an idea whose time has gone? J Rheumatol. 2019;46(2):124-126. PMID: 30710000 pubmed.ncbi.nlm.nih.gov/30710000/.
Nordal EB, Rygg M, Fasth A. Clinical features of juvenile idiopathic arthritis. In: Hochberg MC, Gravallese EM, Smolen JS, van der Heijde D, Weinblatt ME, Weisman MH, eds. Rheumatology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 105.
Ombrello MJ, Arthur VL, Remmers EF, et al. Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications. Ann Rheum Dis. 2017;76(5):906-913. PMID: 27927641 pubmed.ncbi.nlm.nih.gov/27927641/.
Onel KB, Horton DB, Lovell DJ, et al. 2021 American College of Rheumatology Guideline for the treatment of juvenile idiopathic arthritis: therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2022;74(4):521-537. PMID: 35233986 pubmed.ncbi.nlm.nih.gov/35233986/.
Schulert GS, Minoia F, Bohnsack J, et al. Effect of biologic therapy on clinical and laboratory features of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2018;70(3):409-419. PMID: 28499329 pubmed.ncbi.nlm.nih.gov/28499329/.
Ter Haar NM, van Dijkhuizen EHP, Swart JF, et al. Treatment to target using recombinant interleukin-1 receptor antagonist as first-line monotherapy in new-onset systemic juvenile idiopathic arthritis: results from a five year follow-up study. Arthritis Rheumatol. 2019;71(7):1163-1173. PMID: 30848528 pubmed.ncbi.nlm.nih.gov/30848528/.
Wu EY, Rabinovich CE. Juvenile idiopathic arthritis. In: Kliegman RM, St. Geme JW, Schor NF, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 180.
Review Date: 4/30/2023
Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, New York, NY, and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.