individual

Information for Doctors

Sports-Medicine Kalyan-Dombivali

Ankylosing spondylitis (Marie - strumpell's or Von Bechterew's disease)

Introduction
Etiopathogenesis 
Clinical features
Articular manifestations
Symptoms
Enthesopathy
Chest pain in ankylosing spondylitis
Frequency of HLA-B27 among seronegative spondyloarthropathies
Extraskeletal manisfestations of AS
Proposed clinical criteria for AS
Clinical examination
Laboratory studies
Radiographic findings
Radiographic view recommended for sacroilities
Difference between osteophyte and syndesmophyte
D/D of sacroilial changes seen in X-rays
Differential diagnosis of AS
Differential diagnosis of ankylosing spondylitis with other causes of Backache
Subsets of ankylosing spondylitis
Prognosis
Treatment
  A) Physiotherapy
  B) Medication
Newer therapies
Complications

Introduction:

It is a chronic systemic inflammatory disease mainly affecting the axial skeleton (with sacroilitis as its hallmark) and sometimes peripheral joint [ankylos (greek) means "bent" (ankylosis means joint fusion), and spondylosis meaning spinal vertebra]

Etiopathogenesis:

Probably hypothesis is that of interaction between the genetic factor. HLA B27 and the environmental factor the bacterial infection leads to immune response resulting In ankylosing spondylitis.

Clinical features:

Age: Commonly below 40 years of age.

Sex: Males are commonly affected,

Articular manifestations:

Joints involved are

  1. Axial skeleton: Sacroilliac joint, intervertebral disc spaces, apophyseal, costovertebral joints
  2. Anterior central joints: Manubriosternal and sternoclavicular joints, symphysis pubis
  3. Large peripheral joints: Hips, shoulders, knees
  4. Rarely small peripheral joints

Symptoms:

Patient complain of back pain with prolonged morning and often nocturnal stiffness. The stiffness improves with movement and exercise.

Enthesopathy:

The initial inflammatory process of the disease invloves the enthesis (site of bony attachment of ligament, tendon or capsule) resulting in new bone formation or fibrosis.

Sites for enthesopathy are:

  1. Sacroilliac joints
  2. Ligament structure of intervertebral discs.
  3. Manubriosternal joints and symphysis pubis
  4. Ligamentous attachments in the spinous processes, the illac crest, trochanters, calcanei.
  5. Capsules and intracapsular ligaments of large synovial joints.

Chest pain in ankylosing spondylitis:

It occurs due to involvement of thoracic spines, costovertebral joints and also due to enthesopathy at costosternal and manubriosternal joints. This results in reduction of chest expansion.

Frequency of HLA-B27 among seronegative spondyloarthropathies

Click here for the table

Extraskeletal manisfestations of AS:

  1. Aortic insufficiency, ascending aortitis and rare other cardiac manifestations.
  2. Neurologic: Atlantoaxial subluxations and cauda equina syndrome
  3. Renal: Secondary amyloidosis.
  4. Pulmonary: Upper lobe fibrosis, restrictive change
  5. Ocular: Anterior aveitis (25 -30% of patients)
  6. Nephropathy

Proposed clinical criteria for AS:

Diagnosis

Clinical criteria:

  1. Low back pain and stiffness for more than 3 months improved by exercise unrelieved by rest.

  2. Limitation of lumbar spine motion in both sagittal and frontal planes

  3. Limitation of chest expansion (corrected for age and sex)

Radiologic criterion:

Sacroilitis grade? 2 bilaterally or grade 3-4 unilaterally

 

Grading:

Define any losing spondylitis if radiologic criteria is associated with at least one clinical criterion.

 

From Vander Linden etal. Arthritis rheum 1984, 27 366

Clinical examination:

  1. Sacroilloac joint

                                I.     Direct pressure over sacroilliac (S1) joint elicits pain

                             II.       Pelvic compression: Lateral compression of pelvis elicits pain

                           III.      Gaenslen test: With the patient supine, a leg is allowed to drop over the slide of examination table while the patient draws the other leg towards the chest. This test should elicit sacroiliac joint pain on the side of the dropped leg.

                          IV.      Patrick's test: With the patients heel placed on the opposite knee, downward pressure on flexed knee with the hip now in flexion, abduction and external rotation (FABER) should elicit contralateral SI joint tenderness.

Spine: Schober test:

It detects limitation of forward flexion of the lumbar spine. Two marks at a distance of 10 cms are placed, one at the level of posterior superior illiac spine and another above in the midline. With maximal forward spinal flexion with locked knees, the measured distance should increase from 10 cm to atleast 15 cm.

Cervical spine:

Occiput -to -wall test assesses loss of cervical range of motion.

Chest Expansion:

Measure at the fourth intercostal space, normal chest expansion is approximately 5 cm.

Laboratory studies:

1)      HLA B27 is positive in almost 95% of cases

2)      Mild normocytic anaemia positive

      WBC count: normal

            ESR: Increased but does not corelate with disease activity

      3)   PFT tests: usual findings are

            ¯ Vital capacity

            ¯ Total lung capacity

            ­ Residual volume

            ­ Functional residual volume

Radiographic findings:

1)      Sacroiliac joint: The SI joint involvement is more noticeable in lower 2/3 of SI joint (as this portion is the synovial joint)

Grading:

Grade 1: Suspicious: mild burning of SI joint

Grade 2: Minimal: pseudowidening due to erosions, subchondral sclerosis

Grade 3: Moderate: narrowing of SI joint

Grade 4: Ankylosis: complete loss of joint space due to bridging, changes first occur on the iliac side of the joint as the cartilage is thinner on the iliac side.

Refer to Diagram : Click here

Spine:

  1. Periostitis of the periphery of the vertebral body leads to SQUARING" of vertebral bodies.
  2. Romanus sign: It is an erosion surrounded by sclerosis at a vertebral body margin.
  3. Gradual ossification of the superficial layers of annulus fibrosus forms Intervertebral body bridges called syndesmophytes.
  4. Fusion of apophyseal joints and calcification of the spinal ligaments along with bilateral syndesmophyte formation can result in complete fusion of the vertebral column known as bamboo spine.
Refer to diagrams :Click  Diagram 1      Diagram 2    

Enthesopathy:

Proliferative bony margin and protruding bony spicules may be seen at the sites of enthesopathy.

Click here for the diagram

Radiographic view recommended for sacroilities:

  1. Anteroposterior view: It is often sufficient
  2. Ferguson view (AP with the tube angled 25 -30 cephalad): It counteracts the overlap of the sacrum with the ilium, enabiling a full view of SI joint.
Difference between osteophyte and syndesmophyte:
Refer to table : Click here

D/D of sacroilial changes seen in X-rays:

1.      Inflammatory: Spondyloarthropathy -infection (bacterial, fungal, myobacterial)

2.      Traumatic: Fracture, osteoarthritis, osteitis condensans illi.

3.      Generalized disease:

·        Gout

·        Hyperparathyroidism

·        Metastasis

·        Pagets disease

Differential diagnosis of AS

I.      Lambrosacral Disc disease

II.   Other seronegative spondyloarthropathies including Reiter's syndrome and psoriatic arthropathy.

III.   Degenerative joint disease

IV.  Diffuse iodiopathic skeletal hyperostosis seen in elderly characterized by new bone formation at anterior longitudinal ligament, skeletal attachments of ligaments and tendons. It can be differentiated from AS by absence of sacroilitis.

Osteitis condens as Ilii: It is seen in multiparous women characterized by triangular area of dense sclerotic region over the iliac bones adjacent to lower half of SI joint. The SI joint by itself is not affected.

Differential diagnosis of ankylosing spondylitis with other causes of Backache:
Refer to table : Click here

Subsets of ankylosing spondylitis:

  1. Juveline ankylosing spondylitis: It is usually presents in older children with asymmetric oligarticular arthritis of lower extremities and heel pain. Later patient develop typical features of AS with HLA B27
  2. positive from the very begining.
  3. Asymptomatic sacroilitis

Ankylosing spondylitis in females: The axial skeleton and hip joint involvement are lesser with more commonly a symmetrical peripheral arthritis as the main feature. The onset is late.

Prognosis:

Most patients have a satisfactory functional outcome.

Poor prognosis factors:

These include:

  1. Cervical spine ankylosis
  2. flip joint involvement
  3. Uveitis
  4. Pulmonary fibrosis

Treatment:

Aim:

I.      Control of pain

II.     To maintain ROM

III.   Prevention of deformity

A) Physiotherapy

It includes

              I.      Maintenance of erect posture during sitting, standing and walking

           II.      A single or no pillow with a firm mattress must be used while sleeping.

         III.      Spinal extension exercises, breathing exercises, walking and swimming is useful in preventing stiffness.

B) Medication:

1.      Indomethacin: The dosage used for this most commonly prescribed drug is 25 to 50 mg three to 4 times daily or one 75 mg slow release capsule daily.

2.      Other NSAIDS: Other useful NSAIDS are

·        Naproxen (500 mg bd)

·        Proxicam (20 mg daily)

3.      Sulfasalazine: If used early in disease relieves spinal symptoms and decreases acute phase reactants. The dosage used is 1.5 gm bd in patients.

4.      Other medication: Gold and antunalarial drugs are not useful immunosuppressive agents e.g. methotrexase, cyclophosphomide may be tried in uncontrolled cases but are of doubtful efiicacy

5.      Intraarticular steroids: These may be useful in acutely inflamed joint

Surgery: In advanced disease with painful deformity or loss of function, joint replacement surgeries may be done. Cervical and lumbar osteotomies may be required to relieve severe spinal kyphosis AAD may require suffiical stabilization.

Newer therapies:

  1. Clinical trials of anti -TNF -alpha and monoclonal antibody infliximab has shown rapid improvement in symptoms and function in AS. .
  2. Pamidronate, a biphosphonate may have useful anti-inflammatory action thereby preventing subchondral bone loss because of its ability to localize to sites of active bone turnover.

Complications:

  1. Patient with ankylosed spines have a tendency towards fracture due to stiffness & disuse osteoporosis .
  2. Cauda efuina syndrome: It may occur due to nerve rest compression by abnormal bony growths.