Wednesday, July 17, 2024

What Is Juvenile Arthritis Definition

How Is It Treated

Juvenile Rheumatoid Arthritis for USMLE

Some kids who have JIA might take medicine like ibuprofen to help control pain and inflammation. If the arthritis is more severe, they may need to take other medicines to help lower the pain and inflammation. Some of these medicines are pills, but others are shots.

It’s important that kids with JIA keep their joints moving. Often a kid will see a physical therapist or occupational therapist. In addition to working with children to move their joints and strengthen their muscles, these therapists can help create special exercise programs for home or school that can help a kid stay active.

In addition to joint problems, JIA may cause uveitis , an inflammation of the eye that can lead to problems with vision if it’s not treated. All kids diagnosed with JIA should get their eyes checked by an ophthalmologist, a doctor who specializes in diagnosing and treating eye problems. If the eyes are affected, they may be treated with eye drops.

Besides taking medicines, a kid can do a few things to help with the symptoms of JIA:

  • Keep joints warm and stay active.
  • Take warm baths, which can make a kid’s joints hurt less.
  • Use an electric blanket on a timer that turns on 1 hour before a kid wakes up, which can help warm the joints and help a kid move better.
  • Some kids dip their hands in a special warm wax called paraffin that helps their joints ache less.

Points To Remember About Juvenile Idiopathic Arthritis

  • JIA is a type of arthritis that affects children. It causes joint pain, swelling, warmth, stiffness, and loss of motion.
  • JIA begins when the immune system, which normally helps to fight off infections and heal cuts and wounds, becomes overactive and creates inflammation.
  • Treatment depends on the type of JIA and how bad the symptoms are, but usually includes a mix of medications, physical therapy, and regular doctor visits. Treatments can help children with the disease participate in activities.
  • There are many things you can do at home to help your child live with JIA, such as taking care of painful joints, balancing rest and exercise, working with their school, and coping with stress.

What Is The Treatment For Juvenile Arthritis

Treatment for juvenile arthritis generally includes both exercise and medications. The treatment plans are also based on the type of juvenile arthritis. For instance, children who have polyarticular juvenile arthritis and who have a positive result on the rheumatoid factor test have the potential for more joint damage and may need more aggressive treatment.

In general, though, treatment for juvenile arthritis has several main goals:

  • To relieve pain
  • To increase joint mobility and strength
  • To prevent joint damage and complications

The following types of drugs may be used to treat juvenile arthritis:

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Cause Of Juvenile Idiopathic Arthritis

In JIA, the bodys immune systemwhich normally helps to fight off infections and heal cuts and woundsmistakenly attacks some of its own healthy cells and tissues. The result is inflammation, marked by pain, swelling, warmth, and stiffness. Inflammation from JIA can damage the joints, eyes, or other affected organs.

Scientists do not know why the immune system attacks healthy tissues in children with JIA, but they believe that a complex mix of genes and environmental factors are involved.

While the origin of the immune systems overreaction in JIA is unknown, scientists have identified some of the molecules that contribute to inflammation in some forms of the disease. We now know that three moleculesTNF-alpha, IL-6, and IL-1are involved in creating inflammation in the joints of many children with JIA. This has led to new therapies that specifically target these molecules.

Treatment Of Juvenile Arthritis: Systemic Onset Disease

Juvenile rheumatoid arthritis

The polyarthritis associated with systemic onset JRA may have a different pathogenesis than other forms of juvenile inflammatory arthritis68 and may require a different therapeutic approach. SoJRA is more likely to be in the spectrum of autoinflammatory diseases, such as familial Mediterranean fever or other cryopyrin-associated periodic syndromes, a theory supported by the finding of an increased frequency of MEFV alleles in a small study of patients with SoJRA.69 The spectrum of SoJRA can vary from mild disease that responds to NSAIDs to life-threatening illness with macrophage activation syndrome. Mortality among children with JRA is largely limited to this subtype, so patients with moderate to severe SoJRA should be referred to a pediatric rheumatologist with experience in managing these difficult patients.

SoJRA is defined as arthritis in children younger than 16 years of age accompanied by daily fevers of at least 2 weeks duration and one or more of the following: evanescent erythematous rash, generalized lymphadenopathy, or hepatosplenomegaly. Commonly encountered laboratory findings include leukocytosis, anemia, and elevated inflammatory markers. SoJRA affects boys and girls equally and may have its onset in children of any age overall, it accounts for no more than 10% of cases of JRA. Other more common entities such as infection or malignancy must be excluded before confirming the diagnosis and initiating therapy.

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Living With Juvenile Arthritis

There are many treatment options for juvenile arthritis. The primary goal of all treatment options is to bring about remission of the arthritis. Remission means the child should not have any swelling or inflammation detected by exam or imaging.

Treatment also focuses on preserving children’s quality of life by making it possible for them to participate in play, sports, school, and social activities.

In addition to treatment options, school administrators, social workers, and teachers can be important resources. They may be able to develop helpful lesson plans that teach classmates about juvenile arthritis.

With treatment, children should be expected to be able to attend school and have exellent long-term outcomes. Although pain sometimes limits sports and physical activity, children with juvenile arthritis can often fully participate when symptoms are under control.

How Is Juvenile Rheumatoid Arthritis Diagnosed

Your childs doctor will ask about your childs symptoms and do a physical exam. It can be hard to diagnose. You doctor may do an X-ray or blood test to rule out other illnesses. X-rays also can show more severe damage or deformities. Your childs doctor may want to take a sample of fluid from an actively inflamed joint or spinal fluid. It may take a few months before your doctor makes a diagnosis. This is so he or she can watch your childs symptoms over time.

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Specific Secondary Or Associated Conditions And Complications

  • Anorexia, weight loss, growth failure
  • Cardiopulmonary involvement
  • Hepatomegaly, splenomegaly, lymphadenopathy
  • Rare pulmonary manifestations may include interstitial lung disease or pulmonary hypertension16
  • Macrophage Activation Syndrome resulting from uncontrolled activation and proliferation of T-lymphocytes and macrophages is a complication with systemic JIA. It is a life-threatening condition resulting in persistent fever, pancytopenia, hepatosplenomegaly, and coagulopathy.
  • Pain is an incredibly common symptom of JIA but not one that is explained by disease activity alone. Recent studies have shown that older age of onset, poor function or increased disability, and longer disease duration were all associated with consistently higher pain levels. Patients at risk of chronic pain should be identified early and targeted with multidisciplinary pain management interventions.17

How Is Juvenile Idiopathic Arthritis Diagnosed

Juvenile Idiopathic Arthritis: Clinical Guideline for Diagnosis and Management

Diagnosing JIA may be difficult. There is no single test to confirm the disease. Your childs healthcare provider will take your childs health history and do a physical exam. Your child’s provider will ask about your child’s symptoms, and any recent illness. JIA is based on symptoms of inflammation that have occurred for 6 weeks or more.

Tests may also be done. These include blood tests such as:

Your child may also have imaging tests. These can show how much damage the bones have. The tests may include:

  • X-rays. This test uses a small amount of radiation to make images of organs, bones, and other tissues.
  • CT scan. This uses a series of X-rays and a computer to make detailed images of bones, muscles, fat, and organs. CT scans are more detailed than regular X-rays.
  • MRI. This test uses large magnets and a computer to make detailed pictures of organs and structures in the body.
  • Bone scan. This uses a small amount of radiation to highlight the bones in a scanner.

Other tests may include:

  • Urine tests. These look for blood or protein in the urine. This can mean the kidneys are not working normally.
  • Joint aspiration . A small sample of the synovial fluid is taken from a joint. It’s tested to see if crystals, bacteria, or viruses are present.
  • Full eye exam done by an ophthalmologist

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How Will Jia Affect Me

Many children who have JIA won’t have any symptoms when they’re adults, but its not possible to accurately predict this. In most cases, childhood arthritis has a good outcome. You should look forward to a future thats no different from those of your friends and classmates.

In at least 30% of cases, however, arthritis can remain active into adult life. Some young adults with JIA have joint damage that limits their daily activities to some extent and a few may need joint replacements. Other problems can sometimes occur. Some people are physically smaller than average or have osteoporosis as a result of their arthritis and/or treatment with steroids.

A successful outcome in JIA requires many things, including:

  • a positive approach
  • an experienced team working alongside your GP
  • a caring, helpful environment with support from family, friends and teachers.

You also need to know how to get help that you can understand, know who to approach and be confident enough to ask for support and advice.

You can read more about JIA and how it will affect you in our Young people section of the website.

Symptoms Of Juvenile Idiopathic Arthritis

Symptoms of JIA vary depending on the type, but all forms share persistent joint pain, swelling, warmth, and stiffness that are typically worse in the morning and after a nap or prolonged sitting. The pain may limit movement of the affected joint, although many children, especially younger ones, will not complain of pain. One of the earliest signs may be limping in the morning due to disease in one or both legs.

The symptoms of JIA may go through cycles, flaring for a few weeks or months followed by periods when they go into remission. Some children have just one or two flares and never have symptoms again, while others have many flares or symptoms that never fully go away.

Besides joint problems, the inflammation associated with JIA can cause other symptoms, such as:

If left untreated, uveitis can lead to eye problems such as cataracts, glaucoma, and vision loss, so it is important for children with JIA to have frequent eye exams.

  • Skin changes. Depending on the type of JIA a child has, he or she may develop skin changes. Children with:
  • Systemic JIA who have fevers can get a light red or pink rash that comes and goes.
  • Psoriatic JIA can develop scaly red patches of skin. Psoriatic JIA can also cause pitted nails and dactylitis .
  • Polyarticular JIA with rheumatoid factor can get small bumps or nodules on parts of the body that receive pressure, such as from sitting.

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Systemic Juvenile Idiopathic Arthritis

It is the most common type of JA. Systemic juvenile idiopathic arthritis affects boys and girls equally, and it is predicted that it affects 10-20% of all children who have JA.

Systemic juvenile idiopathic arthritis is a particularly troublesome form of JA because it can affect other parts of the body apart from the joints.

For example, systemic juvenile idiopathic arthritis can cause adverse effects on the liver, lungs, and the heart.

These types of juvenile arthritis differentiate itself from other forms of JA by being an auto-inflammatory rather than an auto-immune disease. Systemic juvenile idiopathic arthritis usually strikes at the age of two and can be difficult to treat.

Sadly, there is limited information on what causes systemic juvenile idiopathic arthritis, but genetic factors have been identified.

Living With Juvenile Rheumatoid Arthritis

Juvenile idiopathic arthritis

Whether your childs symptoms come and go or are long-lasting some things can help. This includes:

  • Take a hot shower. Use a hot or cold pack or sleep in a warm bed to relieve stiffness.
  • Stretching and do range-of-motion exercises. This reduces joint stiffness and improves flexibility. Exercise at the same time every day. Make it easy. Do it while watching TV or with family members.
  • Take medicine at the right time and consistently. Have your child take his or her medicine at the same time as another activity. This makes it easy to remember.

Being active is important in managing the disease. Children who have the disease may need emotional support. This is important in managing the anger and sadness of having the disease.

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What Are The Complications Of Juvenile Idiopathic Arthritis

Nearly half of all children with JIA recover fully. Others may have symptoms for years. Some will have rashes and fever. Others may have arthritis that gets worse. Problems may include slow growth and thinning bones . In rare cases, there may be problems with the kidneys, heart, or endocrine system.

Translation Into Practice: Practice Pearls/performance Improvement In Practice /changes In Clinical Practice Behaviors And Skills

  • Leg length discrepancy related to joint damage due to chronic synovitis of involved lower extremity joint may require orthotic adjustments, including shoe lifts
  • Symptom management with intra-articular steroids can decrease inflammation and help prevent synovial overgrowth
  • Exercise should be promoted, even in times of disease flare. Regular low to high intensity exercise, including aquatic therapy, has been shown to decrease pain, improve clinical symptoms, and improve quality of life.
  • Patients with JIA are at high risk for chronic pain and early exposure to opioids. Older age of onset, increased disability or decrease function, and longer disease duration have all been associated with higher pain levels. Patients at risk of chronic pain should be identified early and targeted with multidisciplinary pain management interventions in order to improve long term pain outcomes and decrease opioid dependence.17

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What Is Juvenile Rheumatoid Arthritis

Juvenile rheumatoid arthritis , often referred to by doctors today as juvenile idiopathic arthritis , is a type of arthritis that causes joint inflammation and stiffness for more than six weeks in a child aged 16 or younger. It affects approximately 50,000 children in the United States. Inflammation causes redness, swelling, warmth, and soreness in the joints, although many children with JRA do not complain of joint pain. Any joint can be affected, and inflammation may limit the mobility of affected joints.

JRA is an autoimmune disorder, which means that the body mistakenly identifies some of its own cells and tissues as foreign. The immune system, which normally helps to fight off harmful, foreign substances such as bacteria or viruses, begins to attack healthy cells and tissues. The result is inflammation — marked by redness, heat, pain, and swelling.

Researchers still don’t know exactly why the immune system goes awry in children who develop JRA, although they suspect that it’s a two-step process. First, something in a child’s genetic makeup gives them a tendency to develop JRA. Then an environmental factor, such as a virus, triggers the development of JRA.

Doctors classify three kinds of JRA, based on the number of joints involved, the symptoms, and the presence of certain antibodies in the blood. These classifications help describe how the disease will progress.

Outlook And Outstanding Future Work

Juvenile Idiopathic Arthritis

Figure 1 reports the following steps necessary for the evidence-based final validation of the consensus criteria.

In Step 3, it is foreseen that a prospective cohort of 1000 patients with JIA at onset will be collected, with a longitudinal followup for 15 years.

In Step 4, once the prospective data collection is finished and analyzed, a final consensus conference with NGT will be organized to discuss the results and to finalize the new, validated PRINTO JIA classification criteria. The full methodological details for Steps 3 and 4 are reported in Supplementary Data 2 .

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Sexual Differences In Frequency

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, and in children with polyarticular onset, girls outnumber boys by 2.8:1. In striking contrast, systemic-onset occurs with equal frequency in boys and girls. Boys outnumber girls with enthesitis-related arthritis.

Physical Findings & Clinical Presentation

JIA is subdivided into seven categories based on the International League of Associations for Rheumatology classification criteria . Characteristics of the various categories of JIA are summarized inTable 2.

Systemic onset JIA


Arthritis in 1 joint with or preceded by fever of at least 2-wk duration that is quotidian for at least 3 days and associated with at least one of the following: evanescent erythematous rash generalized lymphadenopathy hepatomegaly, splenomegaly, or both and serositis

Oligoarticular JIA


Arthritis in < 4 joints in the first 6 mo of disease. There are two subtypes:


Persistent: 4 joints throughout the disease course


Extended: 4 joints during the first 6 mo extending to > 4 joints after 6 mo

Polyarthritis, rheumatoid factor negative


Arthritis in > 5 joints during first 6 mo of the disease with negative RF

Polyarthritis, RF positive


Arthritis involves 5 joints during first 6 mo of the disease with positive RF on > 2 tests run 3 mo apart


Anticyclic citrullinated antibodies may also be present


Most similar to adult rheumatoid arthritis most likely to progress

Psoriatic arthritis


Psoriasis and arthritis or psoriasis and 2 of the following:


Dactylitis, nail pitting, onycholysis, and psoriasis in a first-degree relative

Undifferentiated arthritis


M.L. Stoll, R.Q. Cron, in, 2014

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