Juvenile Idiopathic Arthritis (JIA)/Adult-onset Still's Disease

Overview

JIA

In JIA, autoantibodies target healthy joint cells and tissues. Formerly known as juvenile rheumatoid arthritis, JIA is a diagnosis that covers all forms of arthritis in individuals under 16 that last more than 6 weeks and whose origin is unknown. 

 

Adult-onset Still’s Disease

When diagnosed in adults, JIA is more commonly called adult-onset Still’s disease. Some people only experience one episode of Still’s disease, but for others, it can cause chronic progressive illness. The cause of Still’s disease is unknown, though several different treatments are available to help manage symptoms. As symptoms, progression, and severity differ widely amongst individuals with Still’s, the disease can be challenging to diagnose and treat.

JIA can arise in 6 different subtypes:

Oligoarthritis
The most common subtype which affects four or fewer joints, typically the large ones (knees, ankles, elbows).
Polyarthritis
Affects five or more joints, often on both sides of the body (both knees, both wrists, etc.). May affect large and small joints.
Psoriatic arthritis
Joint symptoms and a scaly rash behind the ears and/or on the eyelids, elbows, knees, belly button and scalp. Skin symptoms may occur before or after joint symptoms appear. May affect one or more joints, often the wrists, knees, ankles, fingers or toes.
Psoriatic Arthritis
Enthesitis-related/Spondyloarthritis
Affects where the muscles, ligaments or tendons attach to the bone (entheses). Commonly affects the hips, knees and feet, but may also affect the fingers, elbows, pelvis, chest, digestive tract (Crohn’s disease or ulcerative colitis) and lower back (ankylosing spondylitis). More common in boys; typically appears in children between the ages of eight and 15.
Undifferentiated
Symptoms don’t match up perfectly with any of the subtypes, but inflammation is present in one or more joints.
Systemic
Affects the entire body (joints, skin and internal organs). Symptoms may include a high spiking fever (103°F or higher) that lasts at least two weeks and rash.

Common Symptoms

JIA

Joint pain, swelling, stiffness, fevers, swollen lymph nodes, and rashes (particularly on the torso of the body).

 

Adult-onset Still’s Disease

Joint pain, recurrent high fevers, skin rash, muscle pain, and sore throat.

Coexisting Diseases and Conditions

JIA

Eye inflammation (uveitis) can arise if left untreated, such as cataracts, glaucoma, and blindness, growth and bone development can be affected both by the condition itself and certain medications (mainly corticosteroids) used to treat the condition, psoriasis, Hashimoto’s thyroiditis, Crohn’s disease, ulcerative colitis, diabetes mellitus.

 

Adult-onset Still’s Disease

Chronic organ and joint inflammation leads to most complications including joint destruction, heart inflammation, excess fluid around the lungs, and macrophage activation syndrome.

Risk Factors and Prevalence

JIA

Girls tend to be more than twice as likely as boys to develop JIA. Further studies have shown that children aged 2-4 and 6-12 are at the highest risk of developing JIA. Studies have found that exposure to cigarette smoke puts children at further risk of developing JIA.

 

Adult-onset Still’s Disease

There are two peaks of age ranges when developing adult Still’s disease is most common, 15-25 years and 36-46 years.

Sources

  1. Article Sources

    1. Ellis, J. A., Munro, J. E., & Ponsonby, A.-L. (2010). Possible environmental determinants of juvenile idiopathic arthritis. Rheumatology, 49(3), 411–425. https://doi.org/10.1093/rheumatology/kep383.

    2. França, C. M. P., Sallum, A. M. E., Braga, A. L. F., Strufaldi, F. L., Silva, C. A. A., & Farhat, S. C. L. (2018). Risk Factors Associated with Juvenile Idiopathic Arthritis: Exposure to Cigarette Smoke and Air Pollution from Pregnancy to Disease Diagnosis. The Journal of Rheumatology, 45(2), 248–256. https://doi.org/10.3899/jrheum.161500.

    3. Juvenile Arthritis | Boston Children’s Hospital. (n.d.). Https://Www.Childrenshospital.Org/Conditions-and-Treatments/Conditions/j/Juvenile-Arthritis. Retrieved July 9, 2021, from https://www.childrenshospital.org/conditions-and-treatments/conditions/j/juvenile-arthritis. 

    4. Juvenile idiopathic arthritis. (n.d.). Ada. Retrieved July 9, 2021, from https://ada.com/conditions/juvenile-idiopathic-arthritis/.

    5. Juvenile Idiopathic Arthritis (JIA) | Arthritis Foundation. (n.d.). Retrieved July 9, 2021, from https://www.arthritis.org/diseases/juvenile-idiopathic-arthritis.

    6. Juvenile idiopathic arthritis—Symptoms and causes. (n.d.). Mayo Clinic. Retrieved July 9, 2021, from https://www.mayoclinic.org/diseases-conditions/juvenile-idiopathic-arthritis/symptoms-causes/syc-20374082.

    7. Simon, T. A., Harikrishnan, G. P., Kawabata, H., Singhal, S., Brunner, H. I., & Lovell, D. J. (2020). Prevalence of co-existing autoimmune disease in juvenile idiopathic arthritis: A cross-sectional study. Pediatric Rheumatology, 18(1), 43. https://doi.org/10.1186/s12969-020-00426-9.

    8. Rose, N. R., & Mackay, I. R. (2020). The Autoimmune Diseases (6th ed., pp. 676-687). Academic Press.

    9. Liu Z, et al. Clinical features and prognosis of adult-onset Still’s disease: 75 cases from China. International Journal of Clinical and Experimental Medicine. 2015;8:16634