Rheumatology Book

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Ankylosing SpondylitisAka: Juvenile Ankylosing Spondylitis

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  1. Epidemiology
    1. Strong Association with HLA-B27
      1. HLA-B27 positive in up to 95% of AS cases
      2. Only 2% of HLA-B27 develop Ankylosing Spondylitis
      3. Populations with higher Incidence HLA-B27 positive
        1. Native Americans
        2. Asian populations (except Japanese)
        3. European and United States Caucasian
    2. Men more often affected by ration of 3:1
    3. Onset between Puberty and age 40 years
    4. Low Prevalence Groups
      1. South American Indians
      2. Japanese
      3. African-Americans
  2. Diagnosis: Criteria
    1. Back pain
      1. Starts with dull low back radiating to gluteal area
      2. Progresses up spine to ultimately involve neck
    2. Onset before age 40 years (may occur as early as 13)
    3. Insidious onset
    4. Duration longer than 3 months
    5. Pain worse in the morning
    6. Morning stiffness lasts longer than 30 minutes
    7. Pain decreases with Exercise or activity
    8. Pain provoked by prolonged inactivity or lying down
    9. Pain accompanied with constitutional Symptoms
      1. Anorexia
      2. Malaise
      3. Low grade fever
  3. Articular Symptoms and Signs
    1. Monoarticular Arthritis or Oligoarticular Arthritis
    2. Asymmetric and nonerosive arthritis
    3. Common joint involvement
      1. Inflammatory low back (esp. Sacroiliitis)
      2. Large joints:
        1. Shoulders
        2. Hips (Hip Flexion contractures with rigid gait)
        3. Peripheral joint involvement more common in women
          1. Women have less axial skeleton involvement
    4. Costosternal Pleuritic Chest Pain
    5. Heel Pain
      1. Achilles tendon insertion at calcaneus
      2. Plantar fascia insertion at calcaneus
  4. Systemic Signs
    1. Acute anterior Uveitis (Nongranulomatous)
      1. Occurs in 20-40% of Ankylosing Spondylitis
    2. Microscopic Colitis (often asymptomatic)
      1. Occurs in 25-40% of Ankylosing Spondylitis
    3. Cardiac involvement rare
      1. Aortic Insufficiency
      2. Aortitis
      3. Conduction defects
      4. Arrhythmias
    4. Pulmonary Involvement
      1. Restrictive Lung Disease
        1. Restricted costovertebral mobility
      2. Apical lobe fibrosis
    5. Neurologic Involvement
      1. Spine Fractures or dislocations
      2. Cauda Equina Syndrome
      3. Atlantoaxial subluxation
  5. Complications of Late Spondyloarthropathy
    1. Spondylodiscitis
    2. Cauda Equina Syndrome
    3. Pseudoarthrosis with spinal cord compression
      1. Resultant neurologic deficits
  6. Exam
    1. Observation of back
      1. Lumbar lordosis flattened
      2. Thoracic kyphosis exaggerated
      3. Cervical spine hyperextended
    2. Tests for Sacroiliac Joint Inflammation
      1. Gaenslen's Test
      2. Patrick's Test
    3. Tests for range of motion loss at Lumbar spine
      1. Schober's Test
      2. Decreased lateral bending and lumbar extension
  7. Radiology
    1. Anteroposterior Pelvis XRay
      1. Usually sufficient as only XRay confirmation
      2. Reveals bilateral and symmetric sacroiliitis
        1. Sclerosis may be present (usually not in children)
        2. Later findings include Erosions or SI joint fusion
    2. Spine XRay other findings
      1. Initial
        1. Bony sclerosis appears as squaring of vertebrae
      2. Next
        1. Osteitis of vertebral margins
      3. Late
        1. Annulus fibrosus ossifies
        2. Syndesmophytes between vertebrae
          1. Classic "Bamboo" spine (<10%) appearance
          2. Progresses up spine
    3. Special XRay views
      1. Ferguson's View (specialized sacroiliac view)
    4. Other studies with limited indications
      1. Bone Scan
      2. CT or MRI spine
  8. Labs
    1. HLA-B27 Assay
      1. Not recommended for routine testing
      2. Nonspecific: Present in up to 10% of Caucasians
    2. Acute phase reactants
      1. General
        1. Increased in up to 70% of ankylosing spondylitis
        2. Not correlated with disease activity or severity
      2. Markers
        1. C-Reactive Protein (CRP)
        2. Erythrocyte Sedimentation Rate (ESR)
  9. Management: Non-pharmacologic
    1. Regular therapeutic Exercise
    2. Erect posture
    3. Firm mattress (without a pillow)
    4. Deep breathing Exercises
      1. Maintain normal chest expansion
    5. Spinal extension Exercises
    6. Range of Motion Exercises
      1. Cervical spine
      2. Shoulders
      3. Hips
      4. Knees
    7. Consider physical therapy
  10. Management: Medications
    1. First Line: NSAIDS
      1. Indomethacin (up to maximum of 50 mg PO tid)
      2. Tolmetin 400 mg PO tid-qid
    2. Second Line: NSAID refractory cases or NSAID Adjuncts
      1. Sulfasalazine 2-4g/day divided doses
        1. Effective peripheral arthritis
        2. Less effective for axial skeleton symptoms
      2. Methotrexate
        1. Effective for peripheral but not axial arthritis
      3. Local Corticosteroids injection
        1. For persistent synovitis and enthesopathy
    3. Other agents potential benefit
      1. Pamidronate (Aredia) IV
      2. Tumor Necrosis Factor alpha agents
        1. Etanercept (Enbrel)
        2. Infliximab (Remicade)
        3. Braun (2003) Ann Rheum Dis 62:817
    4. Medications to avoid
      1. Avoid long term Systemic Corticosteroids
        1. Not generally effective in ankylosing spondylitis
      2. Avoid gold and Penicillamine
  11. References
    1. Inman in Klippel (1997) Primer Rheumatic, p. 189-93
    2. van der Linden in Ruddy (2001) Kelley's Rheum, p. 1039
    3. Dougados (2002) Ann Rheum Dis :
    4. Kataria (2004) Am Fam Physician 69:2853

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