2. • Spondyloarthropathy (SpA) is a frequent,
chronic, inflammatory condition with
potential disabling outcomes.
• A recent epidemiologic study performed in
Brittany (France) evaluated the disease
prevalence as 0.49% compared with that of
rheumatoid arthritis (RA) as 0.64%
3. • Many SpA patients have mild disease with a
good clinical response to nonsteroidal
antiinflammatory drugs (NSAIDs).
4. • Nevertheless, some patients have clinical,
biological, or radiological elements of poor
prognosis or are refractory to NSAIDs with
persistent signs of active disease.
• In this set of patients, the initiation of slow-acting
drugs (sulphasalazine or methotrexate)
might at best induce symptomatic
improvement.
5. • The recent development of biotherapies such
as tumor necrosis factor (TNF)-a blockers and
their use in SpA have demonstrated promising
results.
7. Classification criteria
• SpA consists of several disorders, with
ankylosing spondylitis (AS) as the prototype of
this group of inflammatory conditions:
reactive arthritis,
psoriatic arthritis,
arthritis related to inflammatory bowel
disease (Crohn’s disease and ulcerative
colitis), and the
undifferentiated forms of the disease.
8. • ankylosing spondylitis (AS)
• reactive arthritis,
• psoriatic arthritis,
• arthritis related to inflammatory bowel disease
• undifferentiated forms
These subgroups are characterized by:
• axial or peripheral articular involvement
• enthesitis,
• potential extra-articular manifestations, and
• high association to a genetic factor of
predisposition, the tissue antigen HLA-B27.
9. • axial or peripheral articular involvement
• enthesitis,
• potential extra-articular manifestations
• A common familial aggregation of SpA cases is
another characteristic of this group of
diseases.
• These disorders are difficult to differentiate
because they can occur simultaneously or
sequentially.
14. • New York diagnostic criteria have been proposed, but they
imply radiographic evidence of sacroiliitis for AS diagnosis.
• An incremental frequency of radiographic sacroiliitis over
time has been demonstrated in SpA (40% prevalence after 10
years of disease duration, 86% prevalence after 20 years of
disease duration)
• Thus, because of a frequent absence of radiographic
sacroiliitis at the beginning of the disease, this radiologic item
appears to be an inappropriate request in early diagnosis.
29. The following localizations of the disease are
considered as axial involvement:
• sacroiliac joints,
• spine,
• anterior chest wall, and
• root joints (hip and shoulder).
30. Sacroiliitis
• Is responsible for pain and functional
impairment caused by inflammation with a
long-term evolution often resulting in
ankylosis of the sacroiliac joints.
• In some cases, a persistent inflammation
without ankylosis leads to chronic pain and
functional disability.
31. • Anteroposterior Ferguson view of the sacroiliac joints in a patient with AS. The area of the
sacroiliac joint inferior to the arrows is imaged by the Ferguson view and not by an
anteroposterior view. Note the bilateral, symmetric involvement with erosions and
eburnation
35. • MR image of the sacroiliac joints in a patient with undifferentiated spondyloarthropathy.
Axial T1-weighted image with fat suppression after intravenous administration of Gd-DTPA
demonstrates increased signal intensity in the subchondral bone marrow within the iliac and
sacral sides of the right joint (arrows) and early erosions of the left joint (arrowhead).
36. Spinal involvement
• Can be related to vertebral ligament inflammation or
interapophyseal joint inflammation. Both afflictions
can be responsible for night pain, morning stiffness,
and reduction in spinal mobility.
37. • Vista lateral de la columna lumbosacra que
muestra esquina brillante (puntas de flecha),
la cuadratura de los cuerpos vertebrales, y la
formación temprana sindesmofito (flechas)
en un paciente con EA
38. • Lateral view of the
lumbosacral spine
showing
syndesmophytes of AS,
giving it a bamboo
appearance
39. • Lateral view of the
cervical spine in a
patient with PsA. The
posterior spinous
processes show erosive
changes at ligamentous
attachments
(arrowheads).
40. MR (sagittal STIR) images of the lumbar spine of a patient with AS. (a) Note the areas of marrow edema within the
anteroinferior corners of the third and fourth lumbar vertebrae, respectively (arrows). (b) Four weeks after the start of
therapy with anti–tumor necrosis factor the areas of edema had resolved and so had the patient's symptoms (arrows).
41. • Secondary vicious attitudes can be explained by the
analgesic position (fetal position) unconsciously
taken by the patient during the second part of the
night during inflammatory phases of the disease.
42. Anterior chest wall pain
• Is observed in about 15% of patients who
have SpA and can be related to
sternoclavicular, manubriosternal, or
sternocostal arthritis.
43. • Anterior chest wall involvement together with
costovertebral joint involvement can lead to
decreased chest expansion and impaired respiratory
function.
44. Shoulder and hip involvement
• Can be responsible for severe disability.
• Moreover, hip arthritis (defined as the
presence of pain localized in the groin
associated with lameness) has been
considered as a criterion of poor outcome
prognosis.
46. • In patients suffering from isolated axial
involvement, an increase in C-reactive protein
(CRP) is not a rare phenomenon; it is observed
in about 40% of patients.
47. An increase in
CRP might be
correlated with
the clinical
severity of the
disease and
might be
considered to
be a potential
predisposing
factor of
structural
progression.
48. • In clinical trial, NSAIDs had little effect on
erythrocyte sedimentation rate (ESR) or CRP.
• At variance, recent data on TNF-a blocking
agents demonstrate a huge effect on both
markers of inflammation.
49. Definition of active axial disease
• Pain and disability can reflect the
inflammatory process and the structural
damage caused by the disease.
• level of night disturbance, duration and
intensity of the morning stiffness, level of
pain, and functional disability.
50. • The remaining question when considering
second-line drugs is whether or not an
objective demonstration of inflammation is
necessary (eg, CRP elevation, inflammatory
signals assessed by MRI, or abnormal
vascularization of the entheses using
ultrasonography).
51. • Musculoskeletal
ultrasound features in
psoriatic arthritis:
• A, Right plantar fascia
thickening compared
with the left.
• C, Power Doppler
ultrasound through
left third finger at the
metacarpophalangeal
joint confirming
increased vascularity
(synovitis).
52. • Musculoskeletal
ultrasound features in
psoriatic arthritis:
• B, Transverse section
through left third
finger at the
metacarpophalangeal
joint showing right
tenosynovitis.
• D, Right Achilles
tendinitis with
calcaneal erosion.
53. Definition of severe axial disease
• The severity of axial disease is defined by
radiologic damages such as bamboo spine.
• An active disease despite optimal NSAID
treatment is usually considered to be a
predictive factor of subsequent severity
defined by radiological progression.
57. • Dactylitis and oligoarthritis of the lower limbs,
predominantly involving knees and ankles, are
characteristic of the peripheral involvement in
SpA and substantially differ from RA
(polyarticular, bilateral, symmetrical arthritis
involving small joints).
58. • Sausage digits in a 14-year-old HLA-B27+ boy with onset of
spondyloarthropathy at the age of 12.
59. Definition of active peripheral
disease
• Activity of peripheral articular manifestations
has a definition similar to the one that
describes activity in RA: number of swollen
and tender joints together with biological
signs of inflammation.
61. • The enthesitis is the anatomical site of insertion of a
tendon, ligament, or articular capsule into bone.
Local or regional pain occurring in an SpA patient
usually reflects enthesitis, which is responsible for
local inflammation and local bone remodeling.
62. • Radiographic image of entheseal calcification and surface irregularity at
calcaneal insertion and Achilles tendon insertion of a patient with SpA
63. Extra-articular manifestations
• Various extra-articular manifestations can be
observed in SpA patients:
psoriasis,
gut inflammation with chronic diarrhea,
uveitis,
cardiac manifestations,
66. ETIOLOGY
• Animal and laboratory studies suggest that
the HLAB27 molecule itself plays a key role,
and that involvement of class I major
histocompatibility complex (MHC) antigens in
the presentation of microbial peptides is
central to the pathogenic mechanism
(Klebsiella aeruginosa)
67. CLINICAL FEATURES
• The principal musculoskeletal lesions
associated with AS are enthesitis and
synovitis, with sacroiliitis also involving
adjacent bone.
• Inflammatory eye lesions, myocardial
changes, gut mucosal lesions, and skin lesions
are inconsistent but characteristic features of
AS.
68. • Spinal features of AS seldom appear before
the age of 16 to 18 years.
• Before this age, children and teenagers may
develop oligoarthritis—typically a swollen
knee or metatarsophalangeal (MTP) joint—
sometimes associated with iritis and/or
enthesitis.
• Juvenile AS is remarkable because it does not
involve the spine.
69. • The average age at onset is 26 years. Although
the disease rarely begins after the age of 40
years, it is not uncommon for the diagnosis to
be made only years later, well after that age.
• Earlier symptoms often are mild, ignored, or
not recognized as being part of AS.
70. • The usual presenting symptom is
inflammatory back pain that is insidious in
onset, persistent for more than 3 months,
worsened by rest and improved by exercise.
• Night pain is a frequent symptom.
71. • Sacroiliitis, the most common initial feature,
causes pain in the buttocks, typically
alternating between right and left in severity.
• This pain sometimes radiates down the thighs
but never below the knee.
72. • A minority of patients present with
oligoarthritis or enthesitis that particularly
affects the heel, or hip pain due to aggressive
synovitis.
• Fatigue, a common and troublesome
symptom, may be caused in large part by
impaired sleep caused by pain and stiffness.
73. • Other constitutional features may include
fever and weight loss. Overt or subclinical
depression, accompanied by a loss of libido
and reduced capacity for work, also may
contribute to lack of well-being.
74. • Spinal discomfort and
stiffness typically ascend
the spine over a period of
years, producing
progressive spinal pain
and restriction.
• One of the first clinical
signs is the disappearance
of the lumbar lordosis.
76. • Bamboo spine’ in a
49-year-old man
with a 14-year
history of
ankylosing
spondylitis.
• This classic but
uncommon
manifestation
occurs at a late
stage following
inflammatory
disease.
• Note the
calcification of the
anterior fibres of
the annulus
(arrow).
77. • This progression affects the costovertebral
joints, reducing respiratory excursion, and the
cervical spine, limiting neck movement.
• Thoracic spine involvement may be associated
with anterior chest pain and sternal/costal
cartilage tenderness, which can be particularly
distressing for patients.
79. • Osteoporosis (which may be prevented by
appropriate therapy) may lead to vertebral
and other fractures later in life.
• Spinal fractures are more common in patients
who have severe involvement with rigidity.
• Aseptic spondylodiscitis may occur in patients
with AS, especially in the thoracic spine
82. Enthesitis
• The central feature of AS is inflammation at
entheses, the sites where tendons and
ligaments attach to bone.
83. • These inflammatory lesions initially lead to
radiographic appearances of osteopenia or
lytic lesions, but subsequently reactive bone
forms a new, more superficial enthesis, which
develops into a radiologically detectable bony
overgrowth or spur.
85. • In the spine, enthesitis occurs at capsular and
ligamentous attachments and discovertebral,
costovertebral, and costotransverse joints, with
involvement also at bony attachments of interspinous
and paravertebral ligaments.
89. • Enthesitis accounts for much of the pain,
stiffness, and restriction at sacroiliac and other
spinal joints.
• The phenomenon also occurs at extraspinal
sites, producing potentially troublesome
symptoms.
90. • Such lesions most commonly affect the
plantar fascia and Achilles tendon insertions
to the calcaneus, leading to disabling heel
pain.
• Plantar fasciitis typically leads to the
formation of fluffy calcaneal spurs visible on
heel radiographs after 6 to 12 months.
91. • Swelling of the right Achilles tendon in a 26-year-old man with
spondyloarthritis
92. • A, Left Achilles tendon (AT), which was normal on both clinical and US examination.
The short white arrows point to the edges of the tendon, and the white dotted line
(with arrows) indicates the depth of the tendon. B, Abnormal right side, with
thickening and hypoechogenicity of the Achilles tendon extending to the insertion.
There is also a distended retrocalcaneal bursa. KFP, Kager's fat pad; ∗, fluid; ∗∗, fat
pad or synovium.
93. Fat-suppressed
magnetic resonance
image of the foot of
a young patient with
ankylosing
spondylitis. The high
signal is consistent
with marrow edema,
reflecting acute
plantar fasciitis
(white arrow). In
addition, there is
retrocalcaneal
bursitis (asterisk).
94. • Similar lesions may occur around the pelvis,
costochondral junctions, tibial tubercles, and
elsewhere, causing marked local tenderness
96. • Sternal and costochondral pain also reflect a
combination of local enthesitis and referred
pain from the thoracic spine.
• This development frequently produces chest
pain that must be distinguished from
myocardial ischemia.
97. Sacroiliitis
• Inflammation of the sacroiliac joints develops
most frequently in the late teens or in the
third decade of life, producing bilateral or
occasionally, unilateral buttock pain, usually
worse after inactivity and sometimes
aggravated by weight bearing.
98. • Changes principally affect the lower anterior
(synovial) portion of the sacroiliac joints and
are associated with juxta-articular osteopenia
and osteitis.
• This condition leads to radiographic
appearances of widening of the sacroiliac
joint.
100. • Endochondral ossification as a consequence of
the osteitis gives the radiographic appearance
of erosion along the lower part of the
sacroiliac joints.
• Osteitis appears as increased water content of
adjacent bone, as seen on magnetic
resonance imaging (MRI).
102. • MRI is a valuable imaging modality for
assessment of inflammation in both the
sacroiliac joints and the spine. This can
frequently be an important aid in establishing
an early diagnosis.
• Capsular enthesopathy also occurs over the
anterior and posterior aspect of the joint
throughout its length, leading to sheets of
ossification that ultimately obscure the joint
completely on standard radiographs, depicted
as ankylosis of the sacroiliac joint.
103. Synovitis
• Synovitis is indistinguishable histologically and
immunohistochemically from typical
rheumatoid disease.
• Peripheral joint synovitis may precede,
accompany, or follow the onset of spinal
symptoms.
104. • Hips, knees, ankles, and MTP joints are
affected most commonly. With the exception
of the shoulders, upper limb joints are almost
never involved in AS.
• In further contrast to rheumatoid arthritis,
peripheral joint synovitis usually is
oligoarticular, often asymmetrical, and
frequently episodic rather than persistent
105. • Joint erosions, especially at the MTP joints,
may lead to subluxation and deformity.
• Temporomandibular joints may be affected,
leading to reduced mouth opening and
discomfort on chewing.
• Dactylitis may lead to pain in one or more toes
that lasts many months.
106. Eye Lesions
• Acute anterior uveitis (iritis) develops at some
time during the course of the disease in
approximately one third of patients with AS,
and may be recurrent.
107. • Acute anterior uveitis in AS, typically unilateral and associated
with redness, pain, and photophobia.
108. • The typical pattern is alternating, unilateral
eye inflammation associated with pain ,
redness, lacrimation, photophobia, and
blurred vision.
• The occurrence of uveitis typically does not
coincide with flares of arthritis.
109. • Untreated or inadequately treated iritis may
lead rapidly to considerable scarring,
irregularity of the pupil, and visual
impairment.
• Red, sore, gritty eyes or blurring of vision in a
patient with AS require urgent ophthalmologic
examination.
110. Inflammatory Bowel Disease
• Sacroiliitis occurs in 6% to 25% of people with
Crohn’s disease or ulcerative colitis.
• Patients with Crohn’s disease or ulcerative
colitis frequently have unilateral sacroiliitis,
and may also suffer from peripheral arthritis
and enthesitis.
111. • Similarly, inflammatory bowel disease may be
present or develop in people with preexisting
AS.
• Indeed, approximately 60% of people with AS
have subclinical changes in the small or large
bowel.
• There is speculation that these changes may
relate to the pathogenesis of AS, but their true
significance is unknown.
112. • Even though some AS lesions closely resemble
those of Crohn’s disease, the great majority of
such lesions never become symptomatic.
• Only about 10% to 15% of the patients with
AS have overt ulcerative colitis or Crohn’s
disease
113. • In a minority of people with colitis and
peripheral arthritis, peripheral joint disease
may diminish substantially after total
colectomy.
114. • Active inflammatory bowel disease increases
the risk and severity of osteoporosis.
• Crohn’s disease with extensive small bowel
involvement also may lead to impaired
vitamin D absorption and osteomalacia,
producing ill-defined musculoskeletal pain and
difficulty with walking.
115. Cardiovascular Involvement
• Cardiac conduction abnormalities and
myocardial dysfunction have been recorded in
a significant minority of people with AS
116. • Aortitis with dilatation of the aortic valve ring
and aortic regurgitation has been
demonstrated in approximately 1% of
patients.
• The risk of occurrence of aortic insufficiency
and cardiac conduction abnormalities increase
with age, disease duration, presence of HLA-B27,
and peripheral joint involvement.
117. Pulmonary Involvement
• Approximately 1% of patients develop
progressive upper lobe fibrosis of the lungs.
• Rigidity of the chest wall results in the inability
to extend the chest fully and to mild
restrictive lung function impairment, but
rarely leads to ventilation insufficiency due to
the compensation by increased diaphragmatic
contribution.
118. Neurologic Lesions
• Neurologic deficits are associated most often
with cord or root lesions following spinal
fracture.
• Nerve root pain may arise from the cervical
spine, especially when there is marked flexion
deformity.
119. • Long-tract signs, including quadriplegia, may
follow spinal fracture dislocation after
relatively minor trauma and complicate
spontaneous atlantoaxial subluxation.
• Subluxation also may lead to severe occipital
headache.
120. Skin Involvement
• In various series, between 10% and 25% of the
patients with typical AS have concomitant
psoriasis lesions.
121. Renal Consequences
• Although rarely seen today, secondary
amyloidosis caused by longstanding AS is well
described.
122. IMAGING
• By definition, all patients fulfilling the modified New York
criteria show signs of sacroiliitis on radiographs.
123. • However, about 30% of the patients do not
develop damage of the spine visible on
radiographs.
• If patients show no spinal damage after a
certain disease duration (about 10 years), it is
unlikely that the patient will develop
radiographic abnormalities of the spine at all.
• On the other hand, patients who have spinal
damage are prone to develop more damage.
124. • Characteristic features on radiographs of the
sacroiliac joints are pseudo-widening of the
joint space, sclerosis, erosions, and ankylosis
125. • Anteroposterior radiographs of the pelvis showing complete ankylosis of
both sacroiliac joints and syndesmophyte formation in the lower lumbar
vertebrae.
126. • Many AS-related changes can be seen in the
spine; squaring of the vertebrae, sclerosis,
erosions, syndesmophytes, bony bridging, and
spondylodiscitis are the most relevant.
127. • Radiograph of the lateral cervical spine, demonstrating the
formation of extensive bridging syndesmophytes that involve
almost the entire cervical spine.
128. Radiograph of the lateral
lumbar spine with squaring of
L1 and syndesmophyte
formation from L3 to L5.
129. • Syndesmophytes are characterized by axial
growth that may lead to bridging phenomena.
For making a diagnosis, conventional
radiography is still the preferred option.
• However, if the radiographs are persistently
normal in the setting of high disease suspicion,
MRI of the sacroiliac joints and spine can add
information.
130. • In contrast to conventional radiographs, MRI
has the potential to demonstrate
inflammation, not merely the end results of
inflammation on bone.
131. • Among MRI techniques for delineating
inflammation, the short tau inversion recovery
(STIR) technique is preferred.
• MRI is also useful in visualizing enthesitis, for
example, of the heel or Achilles tendon
insertion.
132. • Short tau inversion recovery (STIR) image of the sacroiliac joints revealing
extensive inflammation (white) involving both the sacral and iliac sides of
the joints bilaterally.
133. • weighted, opposed-phase, gradient echo magnetic resonance image 3 minutes
after the intravenous injection of gadolinium-DTPA in a 23-year-old man with
ankylosing spondylitis and severe inflammatory back pain localized mainly to the
right side, of 3 years' duration. Acute sacroiliitis is demonstrated by the strong
contrast enhancement of the right sacroiliac joint (arrowheads), with impressive
bone marrow edema (white arrow) and erosions (black arrow).
135. • As in many other diseases in which the
etiology is not clearly defined (e.g., by the
isolation of a specific causative pathogen), the
diagnosis of AS must rest on the combination
of clinical features, radiological findings, and
laboratory results.
136. • There are no established diagnostic criteria for
AS.
• On the other hand, classification criteria, used
for the purpose of categorizing patients in
research studies, are available.
• The most widely used classification criteria for
AS are the modified New York criteria.
137. • Although the New York criteria are useful in established disease, their
heavy reliance on the demonstration of radiographic sacroiliitis diminishes
their applicability in patients with early disease.
138. AMOR’S CLASSIFICATION
CRITERIA FOR
SPONDYLOARTHRITIS:
A patient is considered
as suffering from a
spondylarthropathy if
the sum
is ≥6.
139. AMOR’S CLASSIFICATION
CRITERIA FOR
SPONDYLOARTHRITIS:
A patient is considered as
suffering from a
spondylarthropathy if the sum
is ≥6.
143. • Bone marrow oedema (BMO), reflecting active sacroiliitis
144. • Synovitis as an active inflammatory lesion appears as
hyperintense signal on contrast-enhanced
145. • Capsulitis (arrows) is a hyperintense signal of the sacroiliac
(SI) joint capsule. Anteriorly, the joint capsule gradually
continues into the periosteum of the iliac and sacral bones.