1. ANKYLOSING SOPNDYLITIS 僵直性脊椎炎

2. Definition AS is an inflammatory disorder of unknown etiology that primarily affects the spine, axial skeleton, and large proximal joints of the body.

3. Prevalence and genetic aspects Prevalence: 1.29/1000 persons in U.S. Genetic aspects: 90% AS patients: HLA- B27: (+), but 8% of normal population also have positive HLA-B27.

4. Family history and sex distribution A positive family history of AS may be found in 15% to 20% of cases. Male: Female: 3:1 Age: typical: second through fourth decades

5. Pathology The axial skeleton: sacroiliac (S-I) joints and intervertebral disk spaces The large joints: hips, knees, shoulders Extraskeletal sites: inflammatory fibrous tissue include the uveal tract, aortic root wall, and heart valves

6. Pathology Fibrocartilage is the primary site of inflammation, ligamentous-bony junctions (enthesitis), periositis, and synovitis Initial inflammation, then fibrosis and often ossification, which lead to bony ankylosis

7. Clinical presentation Classic: intermittent or persistent low back pain and stiffness that is worse in the morning and after prolonged rest, and the pain is relieved by physical activity The pain centered in the lumbarsacral spine, also in the buttocks and hips

8. Clinical presentation Chest pain: thoracic spine neck or shoulder pain and stiffness Peripheral arthritis: Hip: major disability source of AS; Heel: local enthesopathy of the calcaneus, Achilles tentinitis is common

9. Extraskeletal manifestations Aortic valve regurgitation: in 5% of patients, complete heart block may develop Pulmonary: Restriction of the thoracic cage can reduced lung volumes Acute iritis: unilateral, secondary glaucoma

11. Clinical criteria for AS Low back pain and stiffness for more than 3 months, improved by exercise, unrelieved by rest Limitation of lumbar spine motion in both sagittal and frontal planes Limitation of chest expansion

12. Radiologic criterion Sacroiliac joints: the earlist radiographic changes of AS, grade: 1. Punched- out erosions 2. Pseudo-widening of the joint 3. Adjacent sclerosis 4.Bony bridging of the joint with complete loss of joint space

15. Diagnosis Define AS: sacroiliitis grade 2 or more bilaterally or grade 3-4 unilaterally associated with at least one clinical criterion

16. Physical examination Sacroiliac (SI) joints: Lateral compression of the pelvis: elicit pain in the involved joints Costovertebral involvement: decreased chest expansion,< 5 cm during inspiration in the adult Extraaxial joint involvement: proximal and asymmetric

17. Physical examination Spine: loss of spinal (lateral, flexion, and extension) motion occurs early: Finger to floor; occipital to wall Progression: loss of lordosis, kyphosis of the thoracic spine, fixed flexion of the neck

21. Physical examination Schober test: make marks between the lumbosacral junction and a point 10 cm above, then the patient makes maximum forward flexion: less than 5 cm of distraction is abnormal

22. Laboratory studies HLA-B27: not a routine examination for AS patients ( not diagnostic criteria) ESR: elevated but not correlate well with disease activity Spine: chondritis then ossification lead to bony bridging of adjacent vertebrae (syndesmophytes)

24. Laboratory studies Advanced ossification of AS is named “ bamboo spine” Periostitis of the vertebral body leads to early “squaring”

26. Treatment Physical therapy: maintenance of erect posture, sleeping on a firm mattress with a small pillow Walking and swimming are excellent ways to maintain joint mobility

27. Treatment Drugs: 1. Indomethacin: is the most commonly used drug 2. Other NSAIDs: naproxen (Naposin), sulindac, etc. 1,2: for pain relief 3. Sulfasalazine: relieves spinal symptoms and decreases acute-phase reactants. Side effect: 1% neutropenia

28. Treatment Systemic steroids: used with caution Intraarticular corticosteroids: be useful for acutely inflamed joints Surgery: total hip replacement, cervical and lumbar osteotomies to relieve severe kyphosis

29. Prognosis Most patients: maintain exercise and take medicine: relatively normal lives < 10% patients: relentless developing Survival curves of AS: similar to general population

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