Enthesitis-related Arthritis

Youn-Soo Hahn

doi:10.4078/jrd.2018.25.4.221

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Hahn: Enthesitis-related Arthritis

Review Article

J Rheum Dis 2018;25(4):221-230.

Published online: 31 October 2018

DOI: https://doi.org/10.4078/jrd.2018.25.4.221

Enthesitis-related Arthritis

Youn-Soo Hahn

Department of Pediatrics, Chungbuk National University College of Medicine, Cheongju, Korea

Corresponding to: Youn-Soo Hahn http://orcid.org/0000-0003-1332-6221 Department of Pediatrics, Chungbuk National University College of Medicine, 1 Sunhwan-ro, Seowon-gu, Cheongju 28644, Korea. E-mail: yshahn@chungbuk.ac.kr

Received 27 June 201811 July 2018 Accepted 22 July 2018

This is a Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Enthesitis-related arthritis (ERA) is a disease predominantly affecting the joints and entheses of the lower extremities and has the potential to eventually affect the sacroiliac joints and spine evolving to juvenile ankylosing spondylitis. ERA is also characterized by rheumatoid factor seronegativity, paucity of antinuclear antibody, and a strong association with the human leukocyte antigen-B27. ERA accounts for a higher proportion of juvenile idiopathic arthritis (JIA) cases in the Asian population compared to other populations. Advances in the understanding of ERA pathogenesis continue to progress and have led to the development of new treatments targeting pro-inflammatory cytokines. In particular, tumor necrosis factor-α inhibitors have become a main-stay of therapy for patients in whom therapy with anti-inflammatory drugs and/or disease-modifying anti-rheumatic drugs are inadequate or contraindicated. Compared to other JIA subtypes, ERA is associated with a poorer quality of life, worse function, and a higher likelihood of ongoing active disease after the initial treatment. Because the current guidelines for the management of ERA is not considered separately from other categories of JIA, there is a need for treatment guidelines specific to ERA to improve the overall disease outcomes.

Keywords: Juvenile arthritis, Enthesitis-related arthritis, Pathogenesis, Therapeutics, Prognosis

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Figure 1.

Modified Schober's test. With patient standing up-right, two points are marked on the back 5 cm below and 10 cm above the horizontal line joining the dimples of Venus which is used as a mark for the lumbosacral junction. On maximal forward flexion, keeping the knees straight, an increase of the distance between the top and bottom points reflects lumbosacral spinal mobility and is considered normal if more than 5 cm.

Figure 2.

Anteroposterior view of the pelvis shows joint space narrowing, bone erosions, and reactive sclerosis in the sacroiliac joints.

Figure 3.

Corner lesion in 15 year old male with en-thesitis-related arthritis. Plain radiograph (A) and T1-weighted magnetic resonance imaging (B) of spine show increased signal at the anterior aspect of the superior endplate of L2 (arrows).

Figure 4.

Achilles tendon enthesitis in a 14 year old boy. (A) Longitudinal grayscale ultrasound image demonstrates increased thickness of Achilles tendon (arrowheads) with hypo-echogenicity and loss of fibrillar pattern in deep layer. (B) Power Doppler images demonstrates increased signals within the tendon consistent with hyperemia.

Figure 5.

Coronal T1-weighted magnetic resonance imaging of pelvis in 15 year old male with enthesitis-related arthritis. There is active sacroiliitis with bone marrow edema (arrows) on both iliac and sacral sides of the sacroiliac joints.

Figure 6.

Flow of treatments for patients with enthesitis-related arthritis. NSAIDs: non-steroidal anti-inflammatory drugs, IA: intraarticular, SSZ: sulfasalazine, MTX: methotrexate, TNF: tumor necrosis factor.

Table 1.

International League of Associations for Rheumatology (ILAR) classification criteria for enthesitis-related arthritis

Arthritis and enthesitis or Arthritis or enthesitis with at least two of the following:

• Sacroiliac joint tenderness and/or inflammatory spinal pain

• Presence of HLA-B27

• Onset of arthritis in a male over 6 years of age

Granulocytosis with flares

• Family history in at least one first-degree relative of ankylosing spondylitis, Enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, reactive arthritis, or acute anterior uveitis

• Acute anterior uveitis Exclusions

• Psoriasis or a history of psoriasis in the patient or a first-degree relative

• Presence of IgM RF on at least two occasions at least 3 months apart

• Systemic JIA in the patient

HLA: human leukocyte antigen, RF: rheumatoid factor, JIA: juvenile idiopathic arthritis.

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