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Juvenile idiopathic arthritis

Juvenile idiopathic arthritis is a term used to describe a group of disorders in children that includes arthritis. They are chronic (long-term) diseases that cause joint pain and swelling.

Causes

The cause of juvenile idiopathic arthritis (JIA) is not known. It is thought to be an autoimmune illness. This means the body attacks and destroys healthy body tissue by mistake.

JIA most often develops before age 16. Symptoms may start as early as 6 months old.

There are several types of JIA:

  • Systemic (bodywide) JIA involves joint swelling or pain, fevers, and rash. It is the least common type.
  • Polyarticular JIA involves many joints. This form of JIA may turn into rheumatoid arthritis. It may involve five or more large and small joints of the legs and arms, as well as the jaw and neck.
  • Pauciarticular JIA involves 4 or less joints, most often the wrists, or knees. It also affects the eyes.
  • Spondyloarthritis of children resembles the disorder in adults and often involves the sacroiliac joint.

Symptoms

Symptoms of JIA may include:

  • Swollen, red, or warm joint
  • Limping or problems using a limb
  • Sudden high fever
  • Rash (on trunk and extremities) that comes and goes with fever
  • Stiffness, pain, and limited movement of a joint
  • 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 symptoms. When eye symptoms occur, they can include:

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:

Blood tests may include:

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:

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.

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 drugs, such as etanercept, infliximab, and related drugs

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.

Some 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 chronic (long-term) pain, disability, and problems at school.

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)
  • Anemia
  • Swelling around the heart (pericarditis)
  • Chronic pain, poor school attendance

When to Contact a Medical Professional

Call 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.

Alternative Names

Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis

References

Beukelman T, Patkar NM, Saag KG, et al. 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: initiation and safety monitoring of therapeutic agents for the treatment of arthritis and systemic features. Arthritis Care Res (Hoboken). 2011;63(4):465-82. PMID: 21452260 www.ncbi.nlm.nih.gov/pubmed/21452260.

Colbert RA. Classification of juvenile spondyloarthritis: Enthesitis-related arthritis and beyond. Nat Rev Rheumatol. 2010;6(8):477-85. PMID: 20606622 www.ncbi.nlm.nih.gov/pubmed/20606622.

Long AR, Rouster-Stevens KA. The role of exercise therapy in the management of juvenile idiopathic arthritis. Curr Opin Rheumatol. 2010;22(2):213-7. PMID: 20010296 www.ncbi.nlm.nih.gov/pubmed/20010296.

Nordal E, Rygg M, Fasth A. Clinical features of juvenile idiopathic arthritis. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatolgy. 6th ed. Philadelphia, PA: Elsevier Mosby; 2015:chap 101.

Prince FH, Otten MH, van Suijlekom-Smit LW. Diagnosis and management of juvenile idiopathic arthritis. BMJ. 2010;341:c6434. PMID: 21131338 www.ncbi.nlm.nih.gov/pubmed/21131338.

Rabinovich CE. Evaluation of suspected rheumatic disease. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 147.

Update Date 4/28/2015

Updated by: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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