What is juvenile arthritis (JA)? I’m asking because I’ve heard it’s different from the arthritis older people get, and I’m a bit confused. I’d like to understand the different types of JA, what causes them (if known), what the common symptoms are, how it’s typically diagnosed (like what tests are involved), and what treatment options are available for children who have it. Also, is it something children typically “outgrow,” or is it usually a lifelong condition? And finally, what resources are available for families dealing with a JA diagnosis?

Answer

Juvenile arthritis (JA) is an umbrella term for a group of autoimmune and inflammatory conditions that develop in children aged 16 years or younger. It is characterized by chronic inflammation of the joints, causing pain, swelling, stiffness, and limited range of motion. JA is not a single disease; rather, it encompasses several subtypes, each with distinct features, symptoms, and potential complications.

The exact cause of JA is unknown, but it is believed to be a complex interplay of genetic predisposition and environmental triggers. In individuals with a genetic susceptibility, certain environmental factors, such as infections, may trigger an autoimmune response where the body’s immune system mistakenly attacks its own healthy cells and tissues, specifically targeting the joints.

Subtypes of Juvenile Arthritis:

  • Juvenile Idiopathic Arthritis (JIA): This is the most common type of JA. "Idiopathic" means the cause is unknown. JIA is further classified into several subtypes based on the number of joints involved, the presence of extra-articular manifestations (symptoms outside the joints), and the presence of specific antibodies in the blood.

    • Oligoarticular JIA: Affects four or fewer joints during the first six months of the disease. It is the most common subtype and often affects large joints like the knees and ankles. There is a risk of developing uveitis, an inflammation of the eye.
    • Polyarticular JIA (RF negative): Affects five or more joints in the first six months of the disease, but the child does not have rheumatoid factor (RF) in their blood. RF is an antibody often found in adults with rheumatoid arthritis.
    • Polyarticular JIA (RF positive): Affects five or more joints in the first six months of the disease, and the child has rheumatoid factor (RF) in their blood. This subtype is more similar to adult rheumatoid arthritis.
    • Systemic JIA: Characterized by arthritis along with systemic features such as fever, rash, and inflammation of internal organs, such as the liver, spleen, or heart. It is also known as Still’s disease.
    • Enthesitis-Related Arthritis: Involves inflammation of the entheses, the points where tendons and ligaments attach to bone. It often affects the hips, knees, ankles, and feet. It may be associated with lower back pain and sacroiliitis (inflammation of the sacroiliac joints).
    • Psoriatic Arthritis: Characterized by arthritis in association with psoriasis, a skin condition that causes red, scaly patches. A family history of psoriasis is also considered in the diagnosis.
    • Undifferentiated Arthritis: Arthritis that does not fit into any of the other subtypes or fits into more than one.
  • Other Inflammatory Arthritides: While JIA is the most common, other forms of arthritis can also occur in children, including:
    • Spondyloarthropathies: A group of inflammatory diseases that primarily affect the spine, but can also involve the joints, entheses, and eyes. These include ankylosing spondylitis and reactive arthritis.
    • Lyme Arthritis: Arthritis caused by Lyme disease, a bacterial infection transmitted by ticks.
    • Septic Arthritis: An infection within a joint, often caused by bacteria.
    • Reactive Arthritis: Joint pain and swelling triggered by an infection in another part of the body.
    • Arthritis Associated with Inflammatory Bowel Disease (IBD): Arthritis that occurs in conjunction with Crohn’s disease or ulcerative colitis.

Symptoms:

The symptoms of JA can vary depending on the subtype and severity of the condition. Common symptoms include:

  • Joint pain, swelling, and stiffness: These are the hallmark symptoms of JA. The pain may be worse in the morning or after periods of inactivity.
  • Limited range of motion: Inflamed joints may be difficult to move fully.
  • Limping: Children may limp due to pain and stiffness in their legs or feet.
  • Fatigue: JA can cause persistent tiredness and lack of energy.
  • Eye inflammation (uveitis): This can cause redness, pain, and sensitivity to light. Uveitis can lead to vision loss if left untreated.
  • Skin rash: Some types of JA are associated with a characteristic rash.
  • Fever: Systemic JIA is often accompanied by high fever.
  • Swollen lymph nodes: Enlarged lymph nodes may occur in some cases.
  • Loss of appetite: Inflammation can affect appetite.
  • Growth problems: JA can interfere with normal growth and development.

Diagnosis:

Diagnosing JA can be challenging because there is no single test to confirm the diagnosis. The diagnosis is typically based on a combination of factors, including:

  • Medical history: The doctor will ask about the child’s symptoms, family history, and past medical conditions.
  • Physical examination: The doctor will examine the child’s joints for signs of inflammation, such as swelling, tenderness, and limited range of motion.
  • Blood tests: Blood tests can help to identify markers of inflammation, such as elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Blood tests can also be done to check for specific antibodies, such as rheumatoid factor (RF) and anti-nuclear antibody (ANA).
  • Imaging studies: X-rays, MRI scans, and ultrasound may be used to assess the extent of joint damage.
  • Eye examination: Regular eye exams are important to screen for uveitis, especially in children with oligoarticular JIA.

Treatment:

The goals of JA treatment are to relieve pain and inflammation, control the disease, prevent joint damage, maintain function, and promote normal growth and development. Treatment typically involves a combination of:

  • Medications:
    • Nonsteroidal anti-inflammatory drugs (NSAIDs): These medications help to reduce pain and inflammation.
    • Disease-modifying antirheumatic drugs (DMARDs): These medications can help to slow down the progression of the disease. Examples include methotrexate, sulfasalazine, and leflunomide.
    • Biologic agents: These medications target specific parts of the immune system to reduce inflammation. Examples include etanercept, infliximab, adalimumab, abatacept, tocilizumab, and canakinumab.
    • Corticosteroids: These medications can quickly reduce inflammation, but they have significant side effects and are usually used for short-term treatment.
  • Physical therapy: Physical therapy can help to improve range of motion, strength, and function.
  • Occupational therapy: Occupational therapy can help children learn how to perform daily activities with less pain and fatigue.
  • Eye care: Regular eye exams are essential to detect and treat uveitis. Eye drops or other medications may be needed to control eye inflammation.
  • Surgery: In some cases, surgery may be needed to repair or replace damaged joints.
  • Lifestyle modifications: Rest, exercise, and a healthy diet can help to manage JA symptoms.

Prognosis:

The prognosis for JA varies depending on the subtype, severity, and response to treatment. Some children with JA may experience remission, where the disease becomes inactive. Others may have chronic, ongoing symptoms. Early diagnosis and treatment are important to prevent joint damage and improve long-term outcomes. Many children with JA can lead active and fulfilling lives with proper management.