JUVENILE ANKYLOSING SPONDYLITIS
 
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    JUVENILE ANKYLOSING SPONDYLITIS

     

    DEFINITION:

    A seronegative spondyloarthropathy involving entheses with joint and systemic (Ophthalmologic) manifestations.

    EPIDEMIOLOGY:

    • prevelance: 11-86/100,000
    • age of onset:
      • usually after 7 years with peak at 11 years
    • risk factors:
      • HLA-B27 (90%), -A2
      • M > F (7:1)
      • family history of other spondyloarthropathies in 65%
    • target joints:
      • major: peripheral - lower distal extremities
      • minor: axial - sacroiliitis, spondylitis

    PATHOGENESIS:

    1. Etiology

    • unknown
    • hypotheses (2):
    • 1. Genetic
      • association with HLA-B27
    • 2. Infectious
      • molecular mimicry - an epitope is shared by some HLA-B27 molecules and some bacterial species

    CLINICAL FEATURES:

    1. Arthritis

    1. Axial Joints

  • 1. Sacroiliitis
  • 2. Spondylitis
    • back limitation not detected until 11-33 years after onset
    • less common - only 24% complain of back/hip pain at onset
    • usually follows peripheral joint disease by years

    • 2. Peripheral Joints

  • 1. Lower Distal Joints
    • distal > proximal; lower > upper
    • feet, ankles, knees, hips most often affected
    • 54% have >4 joints affected; not more than 7
    • asymmetrical
    • more common - 79% have symptoms at onset

    • 2. Entheses

    1. Enthesitis

    • inflammation produces severe discrete pain over the calcaneous, tibial tuberosity, patellae, metatarsal heads

    3. Systemic Manifestations

    1. Ophthalmologic

  • 1. Anterior Uveitis
    • in 10-35% of JAS cases
    • red, painful, photophobic
    • rarely precedes joint complaints

    • 2. Others (rare)

    • severe cervico-occipital pain due to sublexation of atlantoaxial joint
    • decreased chest expansion & vital capacity
    • severe aortic valve insufficiency
    • renal papillary necrosis due to NSAID's
    • amyloidosis

    INVESTIGATIONS:

    1. Serum

    • seronegative - RF and ANA
    • HLA-B27 positive
    • elevated indexes of inflammation
    • anemia of chronic inflammation
    • normal or increased WBC with normal differential
    • increased platelets, ESR, and Ig

    2. Imaging Studies

    1. Skeletal X-Rays

    • sacroiliitis, periostitis,
    • peripheral joint effusions
    • enthesitis

    MANAGEMENT:

    1. Supportive

    • team approach with long-term follow-up
    • physiotheraphy to increase range of motion of affected joints
    • enthesitis
      • custom-made insoles
      • ultrasound or transcutaneous nerve stimulation

    2. Medications

    • anti-inflammatory drugs
      • ASA
      • NSAID

     

     

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