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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
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
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)