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PROF. DR. MD. SHAH
ALAM
FCPS, MS, FRCS
ANKYLOSING
SPONDYLITIS
Definition
It is a chronic systemic inflammatory
disorder involving axial skeleton with
variable involvement of peripheral
joints & non-articular structures
Synonym: Marie-Strümpell
disease, Bechterew's disease
 Generalized chronic inflammatory disease
 Effects are seen mainly in the spine and
sacroiliac joints.
 There is a strong tendency to familial
aggregation and association with the genetic
marker HLA-B27 & ERAP1(Endoplasmic
reticulum aminopeptidase 1)
Etiology: Unknown
 Genetic Predisposition: HLA-B27 is present
in over 95 per cent of Caucasian patients and
in half of their first-degree relatives (as
compared with 8 per cent of the general
population).
 Bacteria: The ‘triggering factor’ that initiates
the abnormal immune response may be a
bacterial antigen which closely resembles
HLA-B27.
Klebsiella pneumonie & some other
PATHOLOGY
 There are two basic lesions:
 Synovitis of diarthrodial joints and
 Inflammation at the fibro-osseous junctions of
syndesmotic joints and tendons.
affects intervertebral discs,
Sacroiliac ligaments,
Symphysis pubis,
Manubrium sterni and
The bony insertions of large tendons(Enthesitis).
PATHOLOGY: 3 STAGES
Ossification of the fibrous tissue
Ankylosis of the joint
Replacement of the granulation tissue
by fibrous tissue
Inflammatory reaction with cell
infiltration
Granulation tissue
formation
Erosion of adjacent bone
Pathogenesis
 There are 2 theories:
 Receptors theory— HLA B27 is a receptor for
etiologic factor (bacteria, virus, etc.). The
resulting complex provokes production of
cytotoxic T-cells which cause damage to cells
with I-ILA B27 molecule.
So, urinary or bowel infection can be a trigger
for AS.
Pathogenesis
 Molecular mimicry theory — bacterial
antigen (or other damaging factor) in complex
with other HLA molecule gets similar to HLA
B27 properties and is been recognized by
cytotoxic T-cells as HLA B27 or decreases the
immune reaction at pathologic peptide
(immunological tolerance).
Clinical features
 pain and stiffness of the
back
 variable involvement of the
hips and shoulders
 the peripheral joints (more
rarely)
 M: F= 2-10:1
 The usual age at onset is
between 15 and 25 years.
Clinical features
 Insidious onset
 Teenager or young adult complains of
backache and stiffness recurring at intervals
over a number of years.
 The symptoms are worse in the early morning
and after inactivity.
 Gradually pain and stiffness become
continuous
Clinical features
Other symptoms
 General fatigue,
 Pain and swelling of joints,
 Tenderness at the insertion of the Achilles
tendon, ‘foot strain’, or
 Intercostal pain and tenderness.
Clinical features
 Loss of extension is always the earliest and the
most severe disability.
 Marked loss of cervical extension may restrict the
line of vision to a few paces.
 Chest expansion, which should be at least 7 cm in
young men, is often markedly decreased.
 Peripheral joints (usually shoulders, hips and
knees) are involved in over a third of the patients
In the most
advanced
stage the spine
may be
completely
ankylosed from
occiput to
sacrum –
grotesque
deformity(ugly
)
In established cases the posture is
typical
Loss of the normal lumbar lordosis,
Increased thoracic kyphosis and
Forward thrust of the neck
(Question mark posture)
upright posture and balance are
maintained by standing with the hips
and knees slightly flexed, and in late
cases these may become fixed
deformities.
Extraskeletal
manifestations
 General fatigue and
loss of weight are
common.
 Acute anterior uveitis
(25%) - if neglected -
---Glucoma.
 Aortic valve disease
 carditis
 pulmonary fibrosis
Pulmonary fibrosis Uveitis
Costochondral articulation
Progressive spinal
flexion may lead to
“chin chest
deformity”
Tests
Cervical
mobility
•Occiput-to-
wall distance
•Tragus-to-
wall distance
•Cervical
rotation
Thoracic mobility Lumbar mobility
Chest expansion Modified
schober index
Finger-to-floor
distance
Lumbar lateral
flexion
Wall test/ Flesche Test
 The patient is asked to stand
with his back to the wall;
heels, buttocks, scapulae
and occiput should all be
able to touch the wall
simultaneously. If extension
is seriously diminished the
patient will find this
impossible.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 & 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
 Usually <15 cm
Cervical rotation
 Patient supine, head in neutral
position, forehead horizontal
 Gravity goniometer / bubble
inclinometer placed centrally on
the forehead.
 Patient rotates head as far as
possible, keeping shoulders still,
ensure no neck flexion or side
flexion occurs
Normal ROM: 70-900
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 ≥7
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
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.
>>>> >>>>
Finger to floor distance
 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.
Range of
motion
Cervical Spine
 Forward flexion: 0 to 450
 Extension: 0 to 450
 Left Lateral Flexion: 0 to 450
 Right Lateral Flexion: 0 to 450
 Left Lateral Rotation: 0 to 800
 Right Lateral Rotation: 0 to 800
Thoracolumbar spine
• Forward flexion: 0 to 900
• Extension: 0 to 300
• Left Lateral Flexion: 0 to 300
• Right Lateral Flexion: 0 to 300
• Left Lateral Rotation: 0 to 300
• Right Lateral Rotation: 0 to 300
Tests for sacroilitis
 Pelvic compression test
 Faber test
 Gaenslen Test
 Pump Handle test
GAENSLEN TEST
Gaenslen test stresses the
sacroiliac joints,
Increased pain during this
test could be indicative of
joint disease.
 Test irritability by compressing the pelvis
with the patient prone. Sacroiliac pain will
be lateralised to the inflamed joint.
PELVIC COMPRESSION
TEST
Faber test/Patrick’s 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.
Imaging
X-rays
 The cardinal sign – and often
the earliest – is erosion and
fuzziness of the sacroiliac
joints.
 The earliest vertebral change is
flattening of the normal anterior
concavity of the vertebral body
(‘squaring’).
 Osteoporosis is common in
longstanding cases ---
hyperkyphosis of the thoracic
spine.
 Ossification across the
disc gives rise to small
bony bridges or
syndesmophytes linking
adjacent vertebral
bodies. If many
vertebrae are involved
the spine may become
absolutely rigid
(bamboo spine)
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.
Grading sacroiliitis
 Grading of radiographs
Normal 0
Suspicious 1
Minimal sacroiliitis 2
Moderate sacroiliitis 3
Ankylosis 4
Ossification os
supraspinous &
interspinous ligaments
“dagger Sign”
 MRI
MRI allows detailed investigation of sacroiliac joints
and may show typical erosions and features of
inflammation such as bone oedema.
 Special investigations
The ESR and CRP are usually elevated during
active phases of the disease.
HLA-B27 is present in 95 per of cases.
Serological tests for rheumatoid factor usually
negative.
Diagnosis
 Diagnosis is easy in spinal rigidity and
typical deformities, but missed in early
disease or unusual presentation.
 In >10 % with an asymmetrical
inflammatory arthritis, it may took several
years to appear back pain.
 Atypical onset is more common in women
 A history of AS in a close relative is
strongly suggestive.
Diagnosis: Modified Newyork
Criteria (1984) – 4 + any of 1/2/3
1. Inflammatory low back pain > 3 months
2. Limited motion of lumbar spine in sagittal &
frontal planes
3. Limited chest expansion (<2.5cm at 4th ICS)
4. Definite radiologic sacroiliitis
Differntials
1. Mechanical disorders
Low back pain in young adults due to muscular
strain, facet joint dysfunction or spondylolisthesis.
These conditions differ from AS in several
ways:
The pain is related to specific physical activities,
Stiffness is less pronounced and
Symptoms are eased rather than aggravated by
inactivity.
Tenderness is also more localized and the
peripheral joints are normal.
Differntials
2. Diffuse idiopathic hyperostosis
(Forestier’s disease)/DISH
Common disorder, mainly older
men, characterized by widespread
ossification of ligaments and
tendon insertions.
Non inflammatory disease
Spinal pain and stiffness are
seldom severe,
The SI joints are not eroded and
the ESR is normal.
Differntials
3. Other seronegative spondyloarthropathies
 Reiter’s disease (genitourinary and ocular
inflammation)
 Psoriatic arthritis(the rash or nail changes),
 Ulcerative colitis + Crohn’s disease (intestinal
ulceration)
 Whipple’s disease
 Behçet’s syndrome. (buccal and genital
ulceration)
 All show some familial aggregation and all are
associated HLAB27.
Treatment
Treatment consists of:
(1) General measures to maintain satisfactory
posture and preserve movement;
(2) Anti-inflammatory drugs to counteract pain
and stiffness;
(3) The use of TNF inhibitors for severe disease;
(4) Operations to correct deformity or restore
mobility
General measures
 Patients are encouraged to remain active and
taught how to maintain satisfactory posture and
urged to perform spinal extension exercises
every day.
 Swimming, dancing and gymnastics are ideal
forms of recreation.
 Rest and immobilization contraindicated
because they tend to increase the general
feeling of stiffness.
Non-steroidal anti-inflammatory
drugs
 They do not prevent or retard the progress to
ankylosis, but
 They control pain and counteract soft-tissue
stiffness, thus making it possible to benefit
from exercise and activity.
 They may have to be continued for many
years.
 DMRD’S: Extremely effective in case of
peripheral form of AS.
 Steroids: Used when NSAlDs noneffective.
 Local Corticosteroids injection- for
persistent synovitis and enthesopathy
TNF inhibitors
 They are pharmaceutical drugs that
suppresses the physiologic response to tumor
necrosis factor (TNF), which is part of the
inflammatory response.
 Advantages: high specificity, selectivity;
decreased risk of immunosuppression.
 Disadvantages: high price, increased
oncological risk.
TNF inhibitors
 TNF inhibitors is used to treat the underlying
inflammatory processes active in AS.
 These therapies are generally reserved for
individuals who have failed to be controlled by
NSAIDs
Infliximab
Etanercept
Have shown rapid, profound, and sustained reductions
in all clinical and laboratory measures of disease
activity.
Operation
 Significantly damaged
hips can be treated by
joint replacement.
 Deformity of spine may
need correction. But
both procedure
associated with
infection & longer
rehabilitation time.
Surgery
 Spine osteotomy is a surgical procedure in
which a section of the spinal bone is cut and
removed to allow for correction of spinal
alignment.

Usually needed for correction of severe
deformed, rigid and fixed spinal deformity.
 The three main types of osteotomy are:
 Smith-Petersen Osteotomy (SPO)
 Pedicle Subtraction Osteotomy (PSO)
 Vertebral Column Resection Osteotomy
(VCR)
As

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As

  • 1. PROF. DR. MD. SHAH ALAM FCPS, MS, FRCS ANKYLOSING SPONDYLITIS
  • 2. Definition It is a chronic systemic inflammatory disorder involving axial skeleton with variable involvement of peripheral joints & non-articular structures
  • 3. Synonym: Marie-Strümpell disease, Bechterew's disease  Generalized chronic inflammatory disease  Effects are seen mainly in the spine and sacroiliac joints.  There is a strong tendency to familial aggregation and association with the genetic marker HLA-B27 & ERAP1(Endoplasmic reticulum aminopeptidase 1)
  • 4. Etiology: Unknown  Genetic Predisposition: HLA-B27 is present in over 95 per cent of Caucasian patients and in half of their first-degree relatives (as compared with 8 per cent of the general population).  Bacteria: The ‘triggering factor’ that initiates the abnormal immune response may be a bacterial antigen which closely resembles HLA-B27. Klebsiella pneumonie & some other
  • 5. PATHOLOGY  There are two basic lesions:  Synovitis of diarthrodial joints and  Inflammation at the fibro-osseous junctions of syndesmotic joints and tendons. affects intervertebral discs, Sacroiliac ligaments, Symphysis pubis, Manubrium sterni and The bony insertions of large tendons(Enthesitis).
  • 6. PATHOLOGY: 3 STAGES Ossification of the fibrous tissue Ankylosis of the joint Replacement of the granulation tissue by fibrous tissue Inflammatory reaction with cell infiltration Granulation tissue formation Erosion of adjacent bone
  • 7. Pathogenesis  There are 2 theories:  Receptors theory— HLA B27 is a receptor for etiologic factor (bacteria, virus, etc.). The resulting complex provokes production of cytotoxic T-cells which cause damage to cells with I-ILA B27 molecule. So, urinary or bowel infection can be a trigger for AS.
  • 8. Pathogenesis  Molecular mimicry theory — bacterial antigen (or other damaging factor) in complex with other HLA molecule gets similar to HLA B27 properties and is been recognized by cytotoxic T-cells as HLA B27 or decreases the immune reaction at pathologic peptide (immunological tolerance).
  • 9. Clinical features  pain and stiffness of the back  variable involvement of the hips and shoulders  the peripheral joints (more rarely)  M: F= 2-10:1  The usual age at onset is between 15 and 25 years.
  • 10. Clinical features  Insidious onset  Teenager or young adult complains of backache and stiffness recurring at intervals over a number of years.  The symptoms are worse in the early morning and after inactivity.  Gradually pain and stiffness become continuous
  • 11. Clinical features Other symptoms  General fatigue,  Pain and swelling of joints,  Tenderness at the insertion of the Achilles tendon, ‘foot strain’, or  Intercostal pain and tenderness.
  • 12. Clinical features  Loss of extension is always the earliest and the most severe disability.  Marked loss of cervical extension may restrict the line of vision to a few paces.  Chest expansion, which should be at least 7 cm in young men, is often markedly decreased.  Peripheral joints (usually shoulders, hips and knees) are involved in over a third of the patients
  • 13. In the most advanced stage the spine may be completely ankylosed from occiput to sacrum – grotesque deformity(ugly )
  • 14. In established cases the posture is typical Loss of the normal lumbar lordosis, Increased thoracic kyphosis and Forward thrust of the neck (Question mark posture) upright posture and balance are maintained by standing with the hips and knees slightly flexed, and in late cases these may become fixed deformities.
  • 15. Extraskeletal manifestations  General fatigue and loss of weight are common.  Acute anterior uveitis (25%) - if neglected - ---Glucoma.  Aortic valve disease  carditis  pulmonary fibrosis Pulmonary fibrosis Uveitis Costochondral articulation
  • 16. Progressive spinal flexion may lead to “chin chest deformity”
  • 17. Tests Cervical mobility •Occiput-to- wall distance •Tragus-to- wall distance •Cervical rotation Thoracic mobility Lumbar mobility Chest expansion Modified schober index Finger-to-floor distance Lumbar lateral flexion
  • 18. Wall test/ Flesche Test  The patient is asked to stand with his back to the wall; heels, buttocks, scapulae and occiput should all be able to touch the wall simultaneously. If extension is seriously diminished the patient will find this impossible.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.
  • 19. Tragus-to-wall distance  Maintain & 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  Usually <15 cm
  • 20. Cervical rotation  Patient supine, head in neutral position, forehead horizontal  Gravity goniometer / bubble inclinometer placed centrally on the forehead.  Patient rotates head as far as possible, keeping shoulders still, ensure no neck flexion or side flexion occurs Normal ROM: 70-900
  • 21. 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 ≥7 cm.
  • 22. 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
  • 23. 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. >>>> >>>>
  • 24. Finger to floor distance  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.
  • 25. Range of motion Cervical Spine  Forward flexion: 0 to 450  Extension: 0 to 450  Left Lateral Flexion: 0 to 450  Right Lateral Flexion: 0 to 450  Left Lateral Rotation: 0 to 800  Right Lateral Rotation: 0 to 800 Thoracolumbar spine • Forward flexion: 0 to 900 • Extension: 0 to 300 • Left Lateral Flexion: 0 to 300 • Right Lateral Flexion: 0 to 300 • Left Lateral Rotation: 0 to 300 • Right Lateral Rotation: 0 to 300
  • 26. Tests for sacroilitis  Pelvic compression test  Faber test  Gaenslen Test  Pump Handle test
  • 27. GAENSLEN TEST Gaenslen test stresses the sacroiliac joints, Increased pain during this test could be indicative of joint disease.
  • 28.  Test irritability by compressing the pelvis with the patient prone. Sacroiliac pain will be lateralised to the inflamed joint. PELVIC COMPRESSION TEST
  • 29. Faber test/Patrick’s 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.
  • 30. Imaging X-rays  The cardinal sign – and often the earliest – is erosion and fuzziness of the sacroiliac joints.  The earliest vertebral change is flattening of the normal anterior concavity of the vertebral body (‘squaring’).  Osteoporosis is common in longstanding cases --- hyperkyphosis of the thoracic spine.
  • 31.  Ossification across the disc gives rise to small bony bridges or syndesmophytes linking adjacent vertebral bodies. If many vertebrae are involved the spine may become absolutely rigid (bamboo spine)
  • 32.
  • 33. 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.
  • 34. Grading sacroiliitis  Grading of radiographs Normal 0 Suspicious 1 Minimal sacroiliitis 2 Moderate sacroiliitis 3 Ankylosis 4
  • 35. Ossification os supraspinous & interspinous ligaments “dagger Sign”
  • 36.  MRI MRI allows detailed investigation of sacroiliac joints and may show typical erosions and features of inflammation such as bone oedema.  Special investigations The ESR and CRP are usually elevated during active phases of the disease. HLA-B27 is present in 95 per of cases. Serological tests for rheumatoid factor usually negative.
  • 37. Diagnosis  Diagnosis is easy in spinal rigidity and typical deformities, but missed in early disease or unusual presentation.  In >10 % with an asymmetrical inflammatory arthritis, it may took several years to appear back pain.  Atypical onset is more common in women  A history of AS in a close relative is strongly suggestive.
  • 38. Diagnosis: Modified Newyork Criteria (1984) – 4 + any of 1/2/3 1. Inflammatory low back pain > 3 months 2. Limited motion of lumbar spine in sagittal & frontal planes 3. Limited chest expansion (<2.5cm at 4th ICS) 4. Definite radiologic sacroiliitis
  • 39. Differntials 1. Mechanical disorders Low back pain in young adults due to muscular strain, facet joint dysfunction or spondylolisthesis. These conditions differ from AS in several ways: The pain is related to specific physical activities, Stiffness is less pronounced and Symptoms are eased rather than aggravated by inactivity. Tenderness is also more localized and the peripheral joints are normal.
  • 40. Differntials 2. Diffuse idiopathic hyperostosis (Forestier’s disease)/DISH Common disorder, mainly older men, characterized by widespread ossification of ligaments and tendon insertions. Non inflammatory disease Spinal pain and stiffness are seldom severe, The SI joints are not eroded and the ESR is normal.
  • 41. Differntials 3. Other seronegative spondyloarthropathies  Reiter’s disease (genitourinary and ocular inflammation)  Psoriatic arthritis(the rash or nail changes),  Ulcerative colitis + Crohn’s disease (intestinal ulceration)  Whipple’s disease  Behçet’s syndrome. (buccal and genital ulceration)  All show some familial aggregation and all are associated HLAB27.
  • 42. Treatment Treatment consists of: (1) General measures to maintain satisfactory posture and preserve movement; (2) Anti-inflammatory drugs to counteract pain and stiffness; (3) The use of TNF inhibitors for severe disease; (4) Operations to correct deformity or restore mobility
  • 43. General measures  Patients are encouraged to remain active and taught how to maintain satisfactory posture and urged to perform spinal extension exercises every day.  Swimming, dancing and gymnastics are ideal forms of recreation.  Rest and immobilization contraindicated because they tend to increase the general feeling of stiffness.
  • 44. Non-steroidal anti-inflammatory drugs  They do not prevent or retard the progress to ankylosis, but  They control pain and counteract soft-tissue stiffness, thus making it possible to benefit from exercise and activity.  They may have to be continued for many years.
  • 45.  DMRD’S: Extremely effective in case of peripheral form of AS.  Steroids: Used when NSAlDs noneffective.  Local Corticosteroids injection- for persistent synovitis and enthesopathy
  • 46. TNF inhibitors  They are pharmaceutical drugs that suppresses the physiologic response to tumor necrosis factor (TNF), which is part of the inflammatory response.  Advantages: high specificity, selectivity; decreased risk of immunosuppression.  Disadvantages: high price, increased oncological risk.
  • 47. TNF inhibitors  TNF inhibitors is used to treat the underlying inflammatory processes active in AS.  These therapies are generally reserved for individuals who have failed to be controlled by NSAIDs Infliximab Etanercept Have shown rapid, profound, and sustained reductions in all clinical and laboratory measures of disease activity.
  • 48. Operation  Significantly damaged hips can be treated by joint replacement.  Deformity of spine may need correction. But both procedure associated with infection & longer rehabilitation time.
  • 49. Surgery  Spine osteotomy is a surgical procedure in which a section of the spinal bone is cut and removed to allow for correction of spinal alignment.  Usually needed for correction of severe deformed, rigid and fixed spinal deformity.  The three main types of osteotomy are:  Smith-Petersen Osteotomy (SPO)  Pedicle Subtraction Osteotomy (PSO)  Vertebral Column Resection Osteotomy (VCR)