ankylosing spondylitis

definition

·      chronic systemic inflammatory disease

·      mainly affects the cartilaginous joints of the axial skeleton

·      SI joints (hallmark) and spine

·      is a seronegative spondyloarthropathy along with

·      Reiter’s syndrome

·      psoriatic arthritis

·      arthritis associated with inflammatory bowel disease

Diagnostic criteria (1966 New York)

·      positive x-rays of sacroiliitis PLUS

·      one or more criteria:

·      history of pain in lumbar spine

·      decreased lumbar spine movement

·      limitation of chest expansion to 1” at 4th intercostal space

epidemiology

·      incidence is 0.1% in Caucasians

·      geographic variation

·      varies with prevalence of HLA-B27

·      genetic predisposition

·      more common in family members

·      male preponderance M:F = 3:1

·      women have

·      less progressive spinal disease

·      more peripheral disease

·      average age of onset is 25 yrs

aetiology
HLA-B27

·      associated with HLA-B27 in 95% of cases

·      considerable geographic variation in prevalence of B27 in general population

·      50% of Canadian Indians and absent in Black Africans

·      thought that HLA-B27 is disease susceptibility factor

·      trigger may be infection with GIT or GU organism

pathology

·      much greater tendancy than rheumatoid arthritis to affect cartilagenous joints

·      two basic lesions

·      enthesitis

·      synovitis of diathrodial synovial joints

Enthesitis

·      enthesis is site of insertion of tendon, ligament or capsular attachment into bone

·      affects

1.   ligamentous structures of cartilagenous joints

·      intervertebral discs

·      manubriosternal joints

·      symphysis pubis

2.   capsular attachments of synovial joints

·      hip

·      shoulder

3.   ligamentous attachments

·      spinous processes of vertebrae

·      iliac crests

·      trochanters

·      initial inflammatory erosive process of enthesis

·      followed by healing with new bone formation

·      bone forms in fibrous tissue and fills defect

·      forms new enthesis above original level of cortical surface

·      result is irregular bony prominence with sclerosis of adjacent cancellous bone

Synovitis

·      similar changes to rheumatoid arthritis

·      villous proliferation of synovial tissue with pannus formation

·      articular cartilage destroyed by pannus

·      joint surfaces ankylosed by bone and/or fibrous tissue

·      areas of articular cartilage may be preserved

Axial skeleton

Intervertebral discs

·      initial feature is erosive lesions of ligamentous insertions into bone

·      of anterolateral part of outer annulus just below annular flange

·      spondylodiscitis (Anderson lesion)

·      lesions heal by formation of new bone

·      Romanos lesion

·      new bone on outer annulus forms syndesmophyte

·      thin, vertical projections from end-plate

·      syndesmophytes fuse to form osseous casing for disc (bamboo spine)

·      later in disease see destructive end-plate changes

·      due to replacement of bone by granulation tissue

Manubriosternal and pubic symphysis

·      initial inflammation

·      followed by destruction of cartilage

·      then replacement of joint by fibrous tissue

·      finally ossification and obliteration of joint

Sacro-iliac joints, apophyseal joints and costovertebral joints

·      typical changes with

·      erosive synovitis

·      capsular enthesopathy and ossification

Large synovial joints

·      synovitis with pannus formation and ankylosis

·      may be inflammatory enthesopathy of capsular insertion with resulting ossification of joint capsule

·      especially involves hip and shoulder

clinical features
Spine

Symptoms

·      back complaints are first features in 75%

·      insidious onset of low back pain

·      usually dull and poorly localised

·      back stiffness

·      worse in morning and after inactivity

·      improved by warming up

·      chest pain and decreased expansion

·      neck pain and stiffness

·      inflammatory pain suggested by

·      age < 40

·      gradual onset

·      persistence > 3 months

·      morning stiffness

·      improvement with exercise

Signs

·      altered posture

·      loss of lumbar lordosis,  increased thoracic kyphosis and decrease in cervical lordosis

·      inability to perform occiput-to-wall test (occiput, shoulders, buttocks and heels to wall)

·      tenderness

·      over spinous processes from enthesitis

·      decreased lumbar spine movement

·      decreased forward flexion (Schober’s test < 4cm over 10 cm)

·      decreased lateral flexion

·      decreased hyperextension

·      pain and tenderness in SI joint

·      tenderness to direct palpation of SI joint

·      pain on pressure on ASIS

·      pain on forced flexion of one hip and hyperextension of contralateral hip

·      pain on downward pressure on flexed knee with hip flexed, abducted and externally rotated (figure 4 position)

·      decreased respiratory excursion

·      diaphragmatic breathing

·      limited chest expansion (normal is 2” at 4th intercostal space)

Complications

·      spinal fracture

·      with trauma

·      difficult to diagnose radiologically because of ossified spinal ligaments (CT scan useful)

·      may result in neurological deficit

·      craniocervical instability

·      mechanism similar to RA with erosive synovitis

·      most common basilar invagination

·      pseudarthrosis

·      syn. spondylodiscitis

·      unclear whether due to pathological fracture or erosive process of disease

·      cauda equina syndrome

·      due to spinal stenosis

Extraspinal

·      involvement of hips and shoulders

·      insidious onset of pain and stiffness

·      pain and tenderness at sites of enthesopathy

·      pelvis (iliac crests, greater trochanters, ischial tuberosities, iliac spines, pubic symphsis)

·      thorax (costosternal joints, manubriosternal joint)

·      heels (Achilles tendon, plantar fascia)

Extraskeletal

·      acute anterior uveitis

·      aortitis and aortic valve incompetence

·      pulmonary fibrosis

investigations
Radiology

SI joint

·      initial blurring of subchondral bone

·      followed by bone erosion and sclerosis

·      like postage stamp

·      seen more on iliac side

·      may lead to pseudowidening of joint

·      followed by interosseous bridging and bony ankylosis

Pelvis

·      erosions and whiskerinig at sites of attachment of tendons and ligaments

·      esp. ischial tuberosities and iliac crests

Spine

·      initial erosions on corners of vertebral bodies

·      followed by formation of osteophytes and then syndesmophytes

·      eventual complete fusion with bamboo spine

·      associated osteoporosis and crush fractures

Hip and shoulder

·      concentric joint space narrowing

·      subchondral sclerosis

·      osteophyte formation

·      ultimate bony ankylosis

Laboratory

·      ESR elevated in 75%

·      CRP more accurate marker of disease activity

·      HLA-B27 usually positive

·      rheumatoid factor negative

differential diagnosis

·      seronegative seroarthropathies

·      often indistinguishable

·      diffuse idiopathic skeletal hyperostosis

·      non-inflammatory with no SI joint involvement

·      other causes of back pain

·      mechanical

·      infectious

·      neoplastic

management
Nonoperative

·      simple analgesia

·      NSAIDs

·      problems with associated GIT disease

·      physiotherapy

·      to maintain ROM and posture

Radiotherapy

·      observed to improve pain and decrease progression

·      risk of inducing malignancy decreased use

·      may have isolated indications in peripheral involvement where NSAIDs contraindicated

Spine

Corrective osteotomy

·      indicated for

·      severe deformity leading to difficulty in looking forward

·      respiratory compromise

·      contraindicated in

·      elderly patients

·      aortic calfication

·      poor general health

·      closing wedge osteotomy of posterior elements

·      single stage posterior procedure

·      osteotomies in spinous processes above and below central vertebrae

·      may use posterior instrumentation

·      good correction can be achieved

·      major risk is to aorta

Fracture and pseudarthrosis

·      if stable lesion with no neurological deficit, external bracing may suffice

·      halothoracic brace should be used for cervical lesions

·      surgery indicated for

·      unstable lesion

·      neurological deficit

·      failure of bracing

Hip

Total hip replacement

·      good functional outcome and durability

·      majority of patients have good or excellent pain relief and improvement in mobility

·      no increased loosening seen

·      main problem is heterotopic ossification

·      10-20% develop Brooker III or IV ossification

·      more common with previous surgery or complete ankylosis preoperatively

·      may be reduced with indomethacin or XRT