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ANKYLOSING SPONDYLITIS
 (Marie-Strümpell disease/
   Bechterew's disease )
 Dr Dhananjaya Sabat MS, DNB, MNAMS
     Assistant Professor Orthopedics
Maulana Azad Medical College, New Delhi
Inflammatory disorder of unknown cause that
  primarily affects the axial skeleton; peripheral
  joints and extra-articular structures may also
  be involved
• Idiopathic
• Rheumatoid factor absent
• HLA-B27 present in > 90% cases
• Disease usually begins in the second or third
  decade.
• M:F= 3:1
PATHOGENESIS
• Immune mediated. In some cases, the disease
  occurs in these predisposed people after
  exposure to bowel or urinary tract infections.
• ? Autoimmunity to the cartilage proteoglycan
  aggrecan.
PATHOLOGY
• The enthesis, the site of ligamentous
  attachment to bone, is thought to be the
  primary site of pathology
• Enthesitis is associated with prominent
  edema of the adjacent bone marrow and is
  often characterized by erosive lesions that
  eventually undergo ossification.
• Sacroiliitis is usually one of the earliest
  manifestations.
• The early lesions consist of subchondral
  granulation tissue, infiltrates of lymphocytes and
  macrophages in ligamentous and periosteal
  zones, and subchondral bone marrow edema.
• Synovitis follows and may progress to pannus
  formation with islands of new bone formation.
• The eroded joint margins are gradually replaced
  by fibrocartilage regeneration and then by
  ossification. Ultimately, the joint may be totally
  obliterated.
•Axial Arthritis (Eg, Sacroiliitis And Spondylitis)
 •Arthritis Of ‘Girdle Joints’ (Hips And Shoulders)
 •Peripheral Arthritis Uncommon
 •Others:
 Enthesitis
 Osteoporosis
 Vertebral Fractures
 Spondylodiscitis
 Costochondritis



The outer annular fibers are eroded and eventually replaced by bone
→ bony syndesmophytes, which then grows by continued enchondral
ossification, ultimately bridging the adjacent vertebral bodies =
“bamboo spine”.                                                   7
PRESENTATION
• Variable
• From intermittent episodes of back pain that
  occur throughout life to a severe chronic
  disease.
• Attacks the spine, peripheral joints and other
  body organs, resulting in severe joint and back
  stiffness, loss of motion and deformity as life
  progresses.
CLINICAL FEATURES
Initial symptom-
• Insidious onset dull pain in the lower lumbar or gluteal
   region
• Low-back morning stiffness of up to a few hours' duration
   that improves with activity and returns following periods of
   inactivity.
• Pain usually becomes persistent and bilateral. Nocturnal
   exacerbation +.
• Predominant complaint- Back pain or stiffness.
• Bony tenderness may present at- costosternal junctions,
   spinous processes, iliac crests, greater trochanters, ischial
   tuberosities, tibial tubercles, and heels.
• Neck pain and stiffness from involvement of the cervical spine : late
  manifestations
• Arthritis in the hips and shoulders (“root” joints) : in25 to 35% of
  patients.
• Arthritis of other peripheral joints: usually asymmetric.
• Pain tends to be persistent early in the disease and then becomes
  intermittent, with alternating exacerbations and quiescent periods.
• In a typical severe untreated case- the
  patient's posture undergoes
  characteristic changes, with obliterated
  lumbar lordosis, buttock atrophy, and
  accentuated thoracic kyphosis. There
  may be a forward stoop of the neck or
  flexion contractures at the hips,
  compensated by flexion at the knees.
• Complication of the spinal disease is
  spinal fracture, which can occur with
  even minor trauma to the rigid,
  osteoporotic spine; cervical spine is
  most commonly involved.
TEST and MEASUREMENT for AS
  Cervical mobility   Thoracic mobility   Lumber mobility

• Occiput-to-wall     • Chest expansion   Modified schober index
  distance
• Tragus-to-wall                          Finger-to-floor distance
  distance
• Cervical rotation                       Lumber lateral flexion
Occiput To Wall Distance / Flesche Test
The severity of cervical flexion deformity in
ankylosing spondylitis can be assessed by
measuring the occiput to wall distance
(Flesche test).
With the patient standing erect, the heels
and the buttocks are placed against a wall; the
patient is then instructed to extend his or her
neck maximally in an attempt to touch the wall
with the occiput.
The distance between the occiput and the
wall is a measure of the degree of flexion
deformity of the cervical spine.



• The occiput to wall distance should be zero
Tragus-to-wall distance
• Maintain starting position i.e.
  ensure head in neutral
  position (anatomical
  alignment), chin drawn in as
  far as possible. Measure
  distance between tragus of
  the ear and wall on both sides,
  using a rigid ruler. Ensure no
  cervical extension, rotation,
  flexion or side flexion occurs.

                                      13
Cervical rotation
• Patient supine, head in neutral
  position, forehead horizontal (if
  necessary head on pillow or
  foam block to allow this, must
  be documented for future
  reassessments).
• Gravity goniometer / bubble
  inclinometer placed centrally
  on the forehead. Patient rotates
  head as far as possible, keeping
  shoulders still, ensure no neck     Normal ROM: 70-900
  flexion or side flexion occurs.
Chest expansion
• Measured as the difference between maximal
  inspiration and maximal forced expiration in
  the fourth intercostal space in males or just
  below the breasts in females. Normal chest
  expansion is ≥5 cm.
Lumbar flexion (modified Schober)
   • With the patient standing
     upright, place a mark at the
     lumbosacral junction (at
     the level of the dimples of
     Venus on both sides).
     Further marks are placed 5
     cm below and 10 cm
     above. Measure the
     distraction of these two
     marks when the patient
     bends forward as far as
     possible, keeping the knees
     straight

The distance less than 5 cm is abnormal



                                          16
Finger to floor distance
• Expression of spinal
  column mobility when
  bending over forward;
  the dimension that is
  measured is the
  distance between the
  tips of the fingers and
  the floor when the
  patient is bent over
  forward with knees
  and arms fully
  extended.
Lateral spinal flexion
Patient standing with heels and buttocks touching the
wall, knees straight, shoulders
back, outer edges of feet 30 cm apart, feet parallel.
Measure minimal fingertip-to-floor distance in full lateral
flexion without flexion, extension or rotation of the trunk
or bending the knees.
                                                      Greater than 10cm is normal.




                       >>>>                              >>>>




                                                                                     18
Range of motion
Cervical Spine
•   Forward flexion: 0 to 45 degrees
•   Extension: 0 to 45 degrees
•   Left Lateral Flexion: 0 to 45
•   Right Lateral Flexion: 0 to 45
•   Left Lateral Rotation: 0 to 80
•   Right Lateral Rotation: 0 to 80

           Thoracolumbar spine
           •    Forward flexion: 0 to 90 degrees
           •   Extension: 0 to 30 degrees
           •    Left Lateral Flexion: 0 to 30
           •   Right Lateral Flexion: 0 to 30
           •   Left Lateral Rotation: 0 to 30
           •   Right Lateral Rotation: 0 to 30
TESTS FOR SACROILITIS
  •   Pelvic compression test
  •   Faber test
  •   Gaenslen Test
  •   Pump Handle test




                                20
GAENSLEN TEST


Gaenslen test stresses
the sacroiliac joints,
Increased pain during
this test could be
indicative of joint
disease.
PELVIC COMPRESSION TEST
• Test irritability by compressing the pelvis with
  the patient prone. Sacroiliac pain will be
  lateralised to the inflamed joint.
Patrick's test or FABER test
• The test is
  performed by
  having the tested
  leg flexed,
  abducted and
  externally rotated.
  If pain results, this
  is considered
  a positive Patrick's
  test.
EXTRASKELETAL MANIFESTATION
• Most common is acute anterior uveitis- typically unilateral,
  causing pain, photophobia, and increased lacrimation.
• Cataracts and secondary glaucoma are not uncommon
  sequelae.
• Inflammation in the colon or ileum.
• Aortic insufficiency.
• Third-degree heart block.
• Subclinical pulmonary lesions.
• Slowly progressive upper pulmonary lobe fibrosis.
• Retroperitoneal fibrosis.
• Prostatitis.
LAB. TESTS
•   HLA B27: present in ≈ 90% of patients.
•   ESR and CRP – often elevated.
•   Mild anemia.
•   Elevated serum IgA levels.
•   ALP & CPK raised.
X-RAY
Sacroiliitis-
• Early: blurring of the cortical margins of
   the subchondral bone
• Followed by erosions and sclerosis.
• Progression of the erosions leads to
   “pseudo widening” of the joint space
• As fibrous and then bony ankylosis
   supervene, the joints may become
   obliterated.
• The changes and progression of the
   lesions are usually symmetric.
• Seen in Ferguson's View (specialized
   sacroiliac view).
• Dynamic MRI is the procedure of choice
   for establishing a diagnosis of sacroiliitis.
Lumbar spine:
• Loss of lordosis/ straightening
• Diffuse osteoporosis
• Reactive sclerosis- caused by
  osteitis of the anterior corners of
  the vertebral bodies with
  subsequent erosion (Romanus
  lesion), leading to “squaring” of
  the vertebral bodies.
• Ossification os supraspinous &
  interspinous ligaments “ dagger
  Sign”.
• Formation of marginal
  syndesmophytes,
• Later Bamboo spine appearance
  when ankylosis of spine occurs.

• Odontoid erosion.
0 normal

0 normal
1 erosion

1 sclerosis
1 squaring

2 syndesmophyte
2 syndesmophyte

3 complete bridging
3 complete bridging
DIAGNOSIS
• Modified Newyork Criteria (1984) – 4 + any of 1/2/3

   1. Inflammatory low back pain > 3 months
   (Age of onset < 40, Insidious onset, Duration longer than 3 months, Pain
   worse in the morning, Morning stiffness lasts longer than 30 minutes, Pain
   decreases with Exercise, Pain provoked by prolonged inactivity or lying
   down, Pain accompanied with constitutional Symptoms- Anorexia,
   Malaise, Low grade fever)

• 2. Limited motion of lumbar spine in sagittal & frontal planes

• 3. Limited chest expansion (<2.5cm at 4th ICS)

• 4. Definite radiologic sacroiliitis
Disease Specific Instruments For The Measurement In
                     Ankylosing Spondylitis

                        Instrument                                   Measures
Bath ankylosing spondylitis disease activity index (BASDAI)    Disease activity

Bath ankylosing spondylitis functional index (BASFI)           Function
Dougados functional index (DFI)                                Function
Bath ankylosing spondylitis metrology index (BASMI)            Function
Modified stoke ankylosing spondylitis spinal score (m-sasss)   Structural damage
TREATMENT
1. Regular physical therapy
2. NSAIDS Indomethacin (up to maximum of 50 mg PO tid)
        COX-2 inhibitors
3. Sulfasalazine, in doses of 2 to 3 g/d- Effective for axial and peripheral
    arthritis
4. Methotrexate, in doses of 10 to 25 mg/wk- primarily for peripheral
    arthritis
5. Local Corticosteroids injection- for persistent synovitis and
    enthesopathy 6. Medications to avoid- Long term Systemic
    Corticosteroids, gold and Penicillamine
7. Anti-TNF-α therapy - heralded a revolution in the management of AS.
    Infliximab (chimeric human/mouse anti-TNF-α monoclonal antibody)
    Etanercept (soluble p75 TNF-α receptor–IgG fusion protein)
       have shown rapid, profound, and sustained reductions in all clinical
    and laboratory measures of disease activity.
8. Pamidronate, thalidomide, α-emitting isotope 224Ra
9. Most common indication for surgery - severe hip joint arthritis, total hip
    arthroplasty.

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Ankylosing spondylitis UG lecture

  • 1. ANKYLOSING SPONDYLITIS (Marie-Strümpell disease/ Bechterew's disease ) Dr Dhananjaya Sabat MS, DNB, MNAMS Assistant Professor Orthopedics Maulana Azad Medical College, New Delhi
  • 2. Inflammatory disorder of unknown cause that primarily affects the axial skeleton; peripheral joints and extra-articular structures may also be involved
  • 3. • Idiopathic • Rheumatoid factor absent • HLA-B27 present in > 90% cases • Disease usually begins in the second or third decade. • M:F= 3:1
  • 4. PATHOGENESIS • Immune mediated. In some cases, the disease occurs in these predisposed people after exposure to bowel or urinary tract infections. • ? Autoimmunity to the cartilage proteoglycan aggrecan.
  • 5. PATHOLOGY • The enthesis, the site of ligamentous attachment to bone, is thought to be the primary site of pathology • Enthesitis is associated with prominent edema of the adjacent bone marrow and is often characterized by erosive lesions that eventually undergo ossification. • Sacroiliitis is usually one of the earliest manifestations.
  • 6. • The early lesions consist of subchondral granulation tissue, infiltrates of lymphocytes and macrophages in ligamentous and periosteal zones, and subchondral bone marrow edema. • Synovitis follows and may progress to pannus formation with islands of new bone formation. • The eroded joint margins are gradually replaced by fibrocartilage regeneration and then by ossification. Ultimately, the joint may be totally obliterated.
  • 7. •Axial Arthritis (Eg, Sacroiliitis And Spondylitis) •Arthritis Of ‘Girdle Joints’ (Hips And Shoulders) •Peripheral Arthritis Uncommon •Others: Enthesitis Osteoporosis Vertebral Fractures Spondylodiscitis Costochondritis The outer annular fibers are eroded and eventually replaced by bone → bony syndesmophytes, which then grows by continued enchondral ossification, ultimately bridging the adjacent vertebral bodies = “bamboo spine”. 7
  • 8. PRESENTATION • Variable • From intermittent episodes of back pain that occur throughout life to a severe chronic disease. • Attacks the spine, peripheral joints and other body organs, resulting in severe joint and back stiffness, loss of motion and deformity as life progresses.
  • 9. CLINICAL FEATURES Initial symptom- • Insidious onset dull pain in the lower lumbar or gluteal region • Low-back morning stiffness of up to a few hours' duration that improves with activity and returns following periods of inactivity. • Pain usually becomes persistent and bilateral. Nocturnal exacerbation +. • Predominant complaint- Back pain or stiffness. • Bony tenderness may present at- costosternal junctions, spinous processes, iliac crests, greater trochanters, ischial tuberosities, tibial tubercles, and heels.
  • 10. • Neck pain and stiffness from involvement of the cervical spine : late manifestations • Arthritis in the hips and shoulders (“root” joints) : in25 to 35% of patients. • Arthritis of other peripheral joints: usually asymmetric. • Pain tends to be persistent early in the disease and then becomes intermittent, with alternating exacerbations and quiescent periods. • In a typical severe untreated case- the patient's posture undergoes characteristic changes, with obliterated lumbar lordosis, buttock atrophy, and accentuated thoracic kyphosis. There may be a forward stoop of the neck or flexion contractures at the hips, compensated by flexion at the knees. • Complication of the spinal disease is spinal fracture, which can occur with even minor trauma to the rigid, osteoporotic spine; cervical spine is most commonly involved.
  • 11. TEST and MEASUREMENT for AS Cervical mobility Thoracic mobility Lumber mobility • Occiput-to-wall • Chest expansion Modified schober index distance • Tragus-to-wall Finger-to-floor distance distance • Cervical rotation Lumber lateral flexion
  • 12. Occiput To Wall Distance / Flesche Test The severity of cervical flexion deformity in ankylosing spondylitis can be assessed by measuring the occiput to wall distance (Flesche test). With the patient standing erect, the heels and the buttocks are placed against a wall; the patient is then instructed to extend his or her neck maximally in an attempt to touch the wall with the occiput. The distance between the occiput and the wall is a measure of the degree of flexion deformity of the cervical spine. • The occiput to wall distance should be zero
  • 13. Tragus-to-wall distance • Maintain starting position i.e. ensure head in neutral position (anatomical alignment), chin drawn in as far as possible. Measure distance between tragus of the ear and wall on both sides, using a rigid ruler. Ensure no cervical extension, rotation, flexion or side flexion occurs. 13
  • 14. Cervical rotation • Patient supine, head in neutral position, forehead horizontal (if necessary head on pillow or foam block to allow this, must be documented for future reassessments). • Gravity goniometer / bubble inclinometer placed centrally on the forehead. Patient rotates head as far as possible, keeping shoulders still, ensure no neck Normal ROM: 70-900 flexion or side flexion occurs.
  • 15. Chest expansion • Measured as the difference between maximal inspiration and maximal forced expiration in the fourth intercostal space in males or just below the breasts in females. Normal chest expansion is ≥5 cm.
  • 16. Lumbar flexion (modified Schober) • With the patient standing upright, place a mark at the lumbosacral junction (at the level of the dimples of Venus on both sides). Further marks are placed 5 cm below and 10 cm above. Measure the distraction of these two marks when the patient bends forward as far as possible, keeping the knees straight The distance less than 5 cm is abnormal 16
  • 17. Finger to floor distance • Expression of spinal column mobility when bending over forward; the dimension that is measured is the distance between the tips of the fingers and the floor when the patient is bent over forward with knees and arms fully extended.
  • 18. Lateral spinal flexion Patient standing with heels and buttocks touching the wall, knees straight, shoulders back, outer edges of feet 30 cm apart, feet parallel. Measure minimal fingertip-to-floor distance in full lateral flexion without flexion, extension or rotation of the trunk or bending the knees. Greater than 10cm is normal. >>>> >>>> 18
  • 19. Range of motion Cervical Spine • Forward flexion: 0 to 45 degrees • Extension: 0 to 45 degrees • Left Lateral Flexion: 0 to 45 • Right Lateral Flexion: 0 to 45 • Left Lateral Rotation: 0 to 80 • Right Lateral Rotation: 0 to 80 Thoracolumbar spine • Forward flexion: 0 to 90 degrees • Extension: 0 to 30 degrees • Left Lateral Flexion: 0 to 30 • Right Lateral Flexion: 0 to 30 • Left Lateral Rotation: 0 to 30 • Right Lateral Rotation: 0 to 30
  • 20. TESTS FOR SACROILITIS • Pelvic compression test • Faber test • Gaenslen Test • Pump Handle test 20
  • 21. GAENSLEN TEST Gaenslen test stresses the sacroiliac joints, Increased pain during this test could be indicative of joint disease.
  • 22. PELVIC COMPRESSION TEST • Test irritability by compressing the pelvis with the patient prone. Sacroiliac pain will be lateralised to the inflamed joint.
  • 23. Patrick's test or FABER test • The test is performed by having the tested leg flexed, abducted and externally rotated. If pain results, this is considered a positive Patrick's test.
  • 24. EXTRASKELETAL MANIFESTATION • Most common is acute anterior uveitis- typically unilateral, causing pain, photophobia, and increased lacrimation. • Cataracts and secondary glaucoma are not uncommon sequelae. • Inflammation in the colon or ileum. • Aortic insufficiency. • Third-degree heart block. • Subclinical pulmonary lesions. • Slowly progressive upper pulmonary lobe fibrosis. • Retroperitoneal fibrosis. • Prostatitis.
  • 25. LAB. TESTS • HLA B27: present in ≈ 90% of patients. • ESR and CRP – often elevated. • Mild anemia. • Elevated serum IgA levels. • ALP & CPK raised.
  • 26. X-RAY Sacroiliitis- • Early: blurring of the cortical margins of the subchondral bone • Followed by erosions and sclerosis. • Progression of the erosions leads to “pseudo widening” of the joint space • As fibrous and then bony ankylosis supervene, the joints may become obliterated. • The changes and progression of the lesions are usually symmetric. • Seen in Ferguson's View (specialized sacroiliac view). • Dynamic MRI is the procedure of choice for establishing a diagnosis of sacroiliitis.
  • 27. Lumbar spine: • Loss of lordosis/ straightening • Diffuse osteoporosis • Reactive sclerosis- caused by osteitis of the anterior corners of the vertebral bodies with subsequent erosion (Romanus lesion), leading to “squaring” of the vertebral bodies. • Ossification os supraspinous & interspinous ligaments “ dagger Sign”. • Formation of marginal syndesmophytes, • Later Bamboo spine appearance when ankylosis of spine occurs. • Odontoid erosion.
  • 28. 0 normal 0 normal 1 erosion 1 sclerosis 1 squaring 2 syndesmophyte 2 syndesmophyte 3 complete bridging 3 complete bridging
  • 29. DIAGNOSIS • Modified Newyork Criteria (1984) – 4 + any of 1/2/3 1. Inflammatory low back pain > 3 months (Age of onset < 40, Insidious onset, Duration longer than 3 months, Pain worse in the morning, Morning stiffness lasts longer than 30 minutes, Pain decreases with Exercise, Pain provoked by prolonged inactivity or lying down, Pain accompanied with constitutional Symptoms- Anorexia, Malaise, Low grade fever) • 2. Limited motion of lumbar spine in sagittal & frontal planes • 3. Limited chest expansion (<2.5cm at 4th ICS) • 4. Definite radiologic sacroiliitis
  • 30. Disease Specific Instruments For The Measurement In Ankylosing Spondylitis Instrument Measures Bath ankylosing spondylitis disease activity index (BASDAI) Disease activity Bath ankylosing spondylitis functional index (BASFI) Function Dougados functional index (DFI) Function Bath ankylosing spondylitis metrology index (BASMI) Function Modified stoke ankylosing spondylitis spinal score (m-sasss) Structural damage
  • 31. TREATMENT 1. Regular physical therapy 2. NSAIDS Indomethacin (up to maximum of 50 mg PO tid) COX-2 inhibitors 3. Sulfasalazine, in doses of 2 to 3 g/d- Effective for axial and peripheral arthritis 4. Methotrexate, in doses of 10 to 25 mg/wk- primarily for peripheral arthritis 5. Local Corticosteroids injection- for persistent synovitis and enthesopathy 6. Medications to avoid- Long term Systemic Corticosteroids, gold and Penicillamine 7. Anti-TNF-α therapy - heralded a revolution in the management of AS. Infliximab (chimeric human/mouse anti-TNF-α monoclonal antibody) Etanercept (soluble p75 TNF-α receptor–IgG fusion protein) have shown rapid, profound, and sustained reductions in all clinical and laboratory measures of disease activity. 8. Pamidronate, thalidomide, α-emitting isotope 224Ra 9. Most common indication for surgery - severe hip joint arthritis, total hip arthroplasty.

Editor's Notes

  1. sclerosis  The hardening of a tissue or part due to chronic inflammation. A thickening or hardening of a body part or systemespecially from excessive formation of fibrous interstitial or glial tissue.erosion   Superficial destruction of a surface by friction, pressure,ulceration, or trauma.A syndesmophyte is a bony growth originating inside a ligament, commonly seen in the ligaments of the spine, specifically the ligaments in the intervertebral joints leading to fusion of vertebrae. Syndesmophytes are pathologically similar to osteophytes.Ankylosingspondylitis patients are particularly prone to developing syndesmophytes. They are also commonly seen in patients who have had back surgery or other chronic stresses on the ligaments of their spine.