Ankylosing Spondylitis and Other Spondyloarthritides

Published on 27/03/2015 by admin

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Chapter 150 Ankylosing Spondylitis and Other Spondyloarthritides

The diseases collectively referred to as spondyloarthritides include ankylosing spondylitis (AS), arthritis associated with inflammatory bowel disease (IBD) and psoriasis, and reactive arthritis following gastrointestinal or genitourinary infections (see Table 150-1 on the Nelson Textbook of Pediatrics websiteimage at www.expertconsult.com). Pediatric rheumatologists have adopted the International League of Associations for Rheumatology (ILAR) classification scheme for juvenile idiopathic arthritis (JIA) and use the term enthesitis-related arthritis (ERA) to encompass most forms of spondyloarthritis in children, except those with co-existing psoriasis.

Clinical Manifestations

Axial and peripheral joint inflammation and enthesitis cause pain and swelling, localized tenderness, stiffness, and loss of range of motion. Common extra-articular manifestations include gastrointestinal inflammation even in the absence of overt IBD and ocular inflammation, which causes pain, erythema, and photophobia.

Enthesitis-Related Arthritis

Children are classified as having ERA if they have either arthritis and enthesitis or arthritis or enthesitis, with two of the following additional characteristics: (1) sacroiliac joint tenderness or inflammatory lumbosacral pain (see Table 150-1), (2) the presence of HLA-B27, (3) age > 6 yr and male sex, (4) acute anterior uveitis, and (5) a family history of an HLA-B27–associated disease (ERA, sacroiliitis with IBD, reactive arthritis, or acute anterior uveitis) in a first-degree relative. Patients with psoriasis (or a family history of psoriasis in a first-degree relative), a positive rheumatoid factor (RF) test result, or systemic arthritis are excluded from this group. Many children with ERA go on to eventually have AS, but many do not, and it is not currently possible to determine whose ERA will progress.

Juvenile Ankylosing Spondylitis

JAS frequently begins with oligoarthritis and enthesitis. The arthritis occurs predominantly in the lower extremities and often involves the hips, in contrast to oligoarticular JIA. Also unlike in adult-onset AS, in JAS axial involvement is usually absent until later in the disease course (Fig. 150-1). Enthesitis is particularly common, manifesting as localized and often severe tenderness at characteristic tendon (as well as ligament, fascia or capsule) insertions around the plantar surface of the foot, ankle (Achilles), and knee (patella). The disease course is variable and can include periods of low disease activity. Fever and weight loss are uncommon and, if present, raise the possibility of IBD.

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