Clinical Case: JUVENILE IDIOPATHIC - JIA Juvenile Idiopathic Arthritis

Case Study Kara is a 3 year-old female who presents to your office with her father for the second time this month complaining of persistent and swelling in the left knee beginning three days ago. Her father notes she has had persistent in the past recorded as high as 103.2°F, but this is the first time he has noticed any swelling in her knee. Kara’s father thinks she is looking a bit pale and indicates she has lost weight in recent weeks. He states she sometimes limps in the morning but appears fine later in the day. Her father notes she has not had any rash or recent injury to her knee. Kara’s past medical history is unremarkable.

Kara at a Glance Vitals upon exam: »» Temp: 101.8°F »» BP: 107/68 »» HR: 96 bpm »» Resp: 22

Is It Juvenile Idiopathic Arthritis? Based on Kara’s history of prior persistent fevers and recent swelling in her knee, you suspect she may be exhibiting symptoms of juvenile idiopathic arthritis. Juvenile Idiopathic Arthritis By The Numbers Juvenile idiopathic arthritis (JIA), also called juvenile rheumatoid arthritis, is the most common type of arthritis • Nearly 300,000 children under age 18 are in children under the age of 16.1 It may affect children at any affected by childhood arthritis3 age, though rarely in the first six months of life.2 JIA causes • Childhood arthritis accounts for more than persistent joint pain, swelling and stiffness. Although some 827,000 health care visits per year3 children may experience symptoms for a few months, others have symptoms for the rest of their lives.1

JIA is caused by a malfunctioning of a child’s immune system leading to of the synovial membrane. If this inflammation persists, joint damage may occur (see figure at left).2 There are several types of arthritis (see list below), all involving chronic joint inflammation, falling under the JIA heading.2 Inflammation is considered chronic when it has lasted at least 3 months.

Kara’s father states she has been limping in the morning since the swelling began, but seems fine throughout the rest of the day. With report of high, persistent fevers and swelling localized in one joint, Kara might be showing signs of systemic onset JIA or oligoarticular JIA. synovial membrane There is no known cause for the underlying immune system bone malfunction in JIA. These conditions are not considered hereditary; though cartilage erosion some research suggests some individuals may have a genetic tendency to develop the disease.2 JIA is also more common among females.1

Children with JIA may face serious complications as a result of their disease. JIA may interfere with a child’s growth and bone development. Some used to treat JIA can inhibit growth.1 Other problems include synovial fluid iritis or uveitis, which if left untreated, could cause cataracts, glaucoma or even blindness.1 Eye inflammation may persist independently of the arthritis. A child may not display symptoms so it is crucial that children capsule synovitis with JIA get regular exams by an ophthalmologist. JIA IS CAUSED BY A MALFUNCTIONING OF A CHILD’S IMMUNE SYSTEM LEADING TO INFLAMMATION OF THE SYNOVIAL MEMBRANE.

The table below outlines different types of JIA.2

Type of JIA Description

• Affects about 10 percent of children with arthritis. • Begins with repeating fevers that can be 103°F or higher, often accompanied by a salmon- colored rash that comes and goes. Systemic Onset • May cause inflammation of the internal organs as well as the joints, though joint swelling may JIA not appear until months or even years after the fevers begin. • Anemia and elevated white blood cell counts are also typical findings in blood tests ordered to evaluate the fevers and ongoing symptoms. • Arthritis may persist even after the fevers and other symptoms have disappeared.

• Involves fewer than five joints in its first stages. • Affects about half of all children with arthritis. • Older children with oligoarticular JIA may develop “extended” arthritis that involves multiple Oligoarticular JIA joints and lasts into adulthood. • Children who develop the oligoarticular form of JIA when they are younger than seven years old have the best chance of having their joint disease subside with time. • At increased risk of developing an inflammatory eye problem (iritis or uveitis).

• Affects five or more joints and can begin at any age. Polyarticular JIA • Children diagnosed in their teens may actually have the adult form of rheumatoid arthritis at an earlier-than-usual age.

• Children have both arthritis and psoriasis or a family history of psoriasis in a parent or sibling. Psoriatic • Typical signs of psoriatic arthritis include nail changes and widespread swelling of a toe or Arthritis finger called dactylitis.

• Often involves attachments of ligaments as well as the spine. Enthesitis- • Sometimes called a spondyloarthropathy. These children may have joint pain without obvious Related Arthritis swelling and may complain of back pain and stiffness.

Making a Juvenile Idiopathic Arthritis Diagnosis Like many rheumatic diseases, JIA is difficult to diagnose. Full blood work is especially important with children Children may not complain of pain at first, and joint to rule out other conditions such as leukemia. X-rays of swelling may not be obvious.2 Diagnosing JIA depends affected joints may also be used to rule out fractures, on physical findings, medical history and the exclusion tumors, infections or congenital defects.1 of other diagnoses. There is no single blood test to diagnose JIA. Children with JIA typically have a negative Typical symptoms of JIA include: rheumatoid factor blood test, whereas adults typically • limping have a positive rheumatoid factor blood test, further • stiffness when awakening complicating diagnoses.2 Some blood tests that may aid • reluctance to use an arm of leg in diagnosing JIA include antinuclear (ANA), • reduced activity level erythrocyte sedimentation rate or C-reactive protein, but • persistent fever in many children with JIA, no significant abnormality • joint swelling will be found.1 Bear in mind ANA is positive in around • weight loss 50 percent of children with JIA. This test is therefore not • pale or ill appearance the most useful for diagnosing, but is more important for • difficulty with fine motor activities determining uveitis risk. Once diagnosed, the overall treatment goal is to control symptoms, prevent joint damage and maintain function. First-line treatment usually involves NSAIDs and/or pain relievers. NSAIDs treat the inflammation in addition to providing pain relief. If NSAIDs are not effectively managing symptoms, a child may be prescribed DMARDs in combination with NSAID therapies.1 In rare and severe cases, surgery may be indicated to improve the position of a joint.2

The American College of advises each child with JIA be treated by a team of health care providers, including a pediatric rheumatologist, physical therapist and a social worker. This core team is advised to coordinate with the child’s pediatrician and any other physician (such as an ophthalmologist or This irregularly shaped pupil is due orthopedic surgeon). This team of providers not only can manage the disease but to synechiae between the lens and also reach out to schools and community resources as necessary.2 iris.

Referring to a Rheumatologist Juvenile Idiopathic Arthritis Checklist Based on Kara’s swelling and her father’s report of ϽϽ Complete blood count persistent fevers, you share your suspicion of JIA. ϽϽ Comprehensive Metabolic Panel ϽϽ Note of any rash or skin changes Kara’s father is obviously concerned, so you explain you ϽϽ X-ray or any other imaging of affected areas would like to get a few lab tests and X-rays of her knee to ϽϽ Document timing of swelling and fevers, including share with a pediatric rheumatologist who specializes in exact temperatures JIA. You explain there are several types of the disease. It is ϽϽ Document any weight loss important to diagnose the specific type to ensure the best »» Note amount and timeline treatment possible for Kara. ϽϽ Timeline of meds »» What did the patient try at home and how Kara’s father asks about his daughter returning to effective was it? preschool when the fever subsides. You advise to continue »» When did the patient take ? treating the fever as before, with OTC fever-relief medication, and Kara can return so long as she is feeling Reminders better and able to ambulate without any difficulty. You also advise her father to be aware of any inflammation • Discuss the importance of monitoring Kara’s in Kara’s eyes, rashes or swelling in other joints, and to behavior for changes in activity or motion return to your office if there are any concerns. • Be aware of any changes or complaints of pain in Kara’s eyes When you are referring your patient to, or consulting with, a pediatric rheumatologist for the suspicion or treatment of JIA, there are critical steps you can take to provide the best possible patient care, while avoiding the duplication of tests and procedures.

Your role in detecting and managing JIA is crucial to your patients and their families

Citations 1. Mayo Clinic. (2013). Juvenile Rheumatoid Arthritis. [Fact sheet]. Retrieved from 2. American College of Rheumatology (ACR). (2013). Arthritis in Children. [Fact http://www.mayoclinic.org/diseases-conditions/juvenile-rheumatoid-arthritis/ sheet]. Retrieved from http://www.rheumatology.org/practice/clinical/patients/

basics/definition/con-20014378 diseases_and_conditions/juvenilearthritis.asp - JIA 3. Centers for Disease Control and Prevention (CDC). (2013). Childhood Arthritis. [Fact sheet]. Retrieved from http://www.cdc.gov/arthritis/basics/childhood.htm

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