National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Enthesitis-related juvenile idiopathic arthritis



Other Names:
Juvenile enthesitis-related arthritis; Enthesitis related arthritis, juvenile; Enthesitis-related arthritis; Juvenile enthesitis-related arthritis; Enthesitis related arthritis, juvenile; Enthesitis-related arthritis; ERA; Juvenile spondylarthropathy; Enthesitis-related JIA See More
Categories:

Enthesitis-related juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis that is characterized by both arthritis and inflammation of an enthesitis site (the point at which a ligament, tendon, or joint capsule attaches to the bone). Signs and symptoms generally develop in late childhood or early adolescence and include pain, tenderness, and swelling in joints and at the enthesis. The knee and the back of the ankle (at the Achilles tendon) are the most commonly affected parts of the body. The underlying cause of enthesitis-related juvenile idiopathic arthritis is currently unknown (idiopathic). It is very rare for more than one member of a family to have juvenile arthritis; however, research suggests that having a family member with juvenile arthritis or any autoimmune disease may increase the risk of having juvenile arthritis, in general. Treatment usually involves different types of medications to help manage symptoms and/or physical therapy.[1][2]
Last updated: 11/23/2015

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Anterior uveitis 0012122
Elevated C-reactive protein level 0011227
Elevated erythrocyte sedimentation rate
High ESR
0003565
Joint swelling 0001386
Juvenile rheumatoid arthritis 0005681
Sacroiliac arthritis 0012317
30%-79% of people have these symptoms
Cartilage destruction 0100773
Dental malocclusion
Bad bite
Malalignment of upper and lower dental arches
Misalignment of upper and lower dental arches
[ more ]
0000689
Enthesitis 0100686
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Last updated: 7/1/2020

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

  • Methotrexate oral solution (Brand name: Xatmep) - Manufactured by Silvergate Pharmaeuticals, Inc.
    FDA-approved indication: April 2017, methotrexate oral solution (Xatmep) was approved for the treatment of oligoarticular juvenile idiopathic arthritis (persistent oligoarthritis, psoriatic juvenile idiopathic arthritis, enthesitis-related arthritis, or undifferentiated arthritis) and polyarticular juvenile idiopathic arthritis in children 0 through 16 years of age.
    National Library of Medicine Drug Information Portal

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The differential diagnosis should include infectious arthritis and other inflammatory diseases, as well as haematooncologic diseases that may lead to arthritis (in particular, connective tissue diseases and acute leukaemia).
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Enthesitis-related juvenile idiopathic arthritis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Enthesitis-related juvenile idiopathic arthritis by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease


Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

  • The Assistance Fund provides various services, including education and financial aid, to help patients with a chronic or serious illness cover the cost of FDA-approved medications. Patients must be U.S citizens or permanent residents.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The Cincinnati Children's Hospital has an information page on Enthesitis-related juvenile idiopathic arthritis. Click on the link to view this information page.
  • Genetics Home Reference (GHR) contains information on Enthesitis-related juvenile idiopathic arthritis. This website is maintained by the National Library of Medicine.
  • The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Enthesitis-related juvenile idiopathic arthritis. Click on the link to view a sample search on this topic.

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  1. David D Sherry, MD. Juvenile Idiopathic Arthritis. Medscape Reference. September 2015; http://emedicine.medscape.com/article/1007276-overview.
  2. Juvenile Arthritis. National Institute of Arthritis and Musculoskeletal and Skin Diseases. June 2015; http://www.niams.nih.gov/Health_Info/Juv_Arthritis/default.asp#2.