1. Spondylo-arthritis

2. Objectives
• Gain a basic understanding of the
Spondylo-Arthritis
• Learn specific Characteristics from SpA
• Differentiate SpA from other chronic
Arthritis ( RA, OA )
• Become familiar with joints, and extra-
articular manifestations of SpA
• Understand the current treatment
paradigm and medications used

3. Introduction
• Spondyloarthropathies =
• Seronegative Spondyloarthritis
– Absence of Rheumatoid Factor or other
autoantibody serologic abnormalities:
• Psoriatic Arthritis-PsA
• Ankylosing Spondylitis-AS (Prototype)
• Reactive Arthritis-ReA
• Enteropathic Arthritis-IBD
• Undifferentiated Spondyloarthropathy-SpA

4. Criteria for classification of spondyloarthropathy
• Inflammatory spinal pain or synovitis (asymmetric or
predominantly in lower limbs) plus more than 1 of the
following:
– Positive family history
– Psoriasis
– Inflammatory bowel disease
– Urethritis, cervicitis, or acute diarrhea < 1 mo. before arthritis
– Buttock pain alternating between right and left gluteal areas
– Enthesopathy
– Sacroiliitis
• Sensitivity 78.4% and specificity 89.6%

5. Spondyloarthropathies
• Are a diverse group of chronic inflammatory
conditions linked by distinctive clinical,
radiographic, and genetic features
• Refers to inflammatory changes involving the
spine and the spinal joints.
• subtypes often overlap
• may be considered one heterogeneous and
phenotypically diverse disease that has the
potential to evolve into AS.

6. Introduction
Confusion
Can often see peripheral joint symptoms in
the absence of spinal symptoms

7. Spondyloarthropaties
• The pathology of spondyloarthropathies is
very different from that of RA. In RA, it is
the synovitis that plays the major role, and
the synovitis leads to bony erosions that
don't heal.
• In spondyloarthritis there is some
synovitis, but it's the enthesitis that is the
major problem, especially in the axial
disease.

8. Spondyloarthropaties
• Not only is there enthesitis but also the
osteitis that follows, which is different
because there is reactive bone sclerosis
and bone absorption, but then more bone
remodeling sets in and it goes on to result
in new bone formation that can result in
ankylosis

9. Spondyloarthropaties
• In RA, the cytokines lead to excessive
osteoclastic activity resulting in bone
erosions,
• In spondyloarthritis, the cytokines that are
playing a major role result in osteoblastic
activity
• Gradual bony bridging follows after being
initiated by the inflammation

10. Spondyloarthropaties

11. Spondyloarthropaties

12. Spondyloarthropathies
• Hallmarks of this family of disorders
• Inflammatory back pain (IBP)
• and Enthesitis: (inflammation at sites
where tendons, ligaments, and joint
capsule fibers attach to bone, with a
strong tendency to produce fibrosis and
calcifications).

13. Spondyloarthropathies
• IBP: inflammatory Back Pain
• worse in the late night and early morning
• pain interferes with sleep to the point that the
patient gets up to walk in the middle of the night,
• the discomfort can be characterized by
alternating buttock pain.
• prolonged morning stiffness of greater than 30
minutes.

14. Spondyloarthropathies
• Exercise alleviates the pain of IBP while
rest makes it worse.
• IBP affects younger patients,
• peaking during the mid-20s
• onset before the age of 40,
• at the height of their productivity.

15. Spondyloarthropathies
• IBP persists for at least 3 months.
• detrimental effects on quality of life
• increased disability, and morbidity --
equal, and in some cases exceeding, that
seen in RA

16. Spondyloarthropathies
• Although new bone formation is central to the
pathogenesis of AS, is also associated with
osteoporosis and low bone mineral density
• ectopic bone formation occurs within the
inflamed vertebral enthesis
• bone resorption, (increased osteoclast activity),
occurs at an unregulated rate within the vertebra
and promotes weakening of the spinal column.

17. Spondyloarthropathies
• The spine in the patient with AS fuses
through:
– ligamentous ossification and
– syndesmophytosis,
• Rigid hyperkyphotic deformity develops.
• Biomechanically, the fused spine acts as a
long bone incapable of appropriately
dissipating the energy of a traumatic
event.

18. Spondyloarthropathies
• Altered spinal biomechanics
• Combined with the brittle quality of the
osteoporotic bone
• Increase susceptibility to vertebral column
factures,and Spinal Cord injury, even after
minor, often trivial, trauma
• Significantly impaired mobility and peripheral
joint arthritis:
– exacerbate gait unsteadiness
– increase susceptibility to falls

19. Sagittal reformatted CTscan showing a highly displaced
thoracic fracture. Asterisk indicates apposition of the caudal
fracture fragment on the thoracic aorta

20. Spondyloarthropathies
• Extra-articular manifestations including:
– psoriasis,
– anterior uveitis,
– IBD,
– as well as rarer cardiac, renal, and pulmonary
manifestations
• a wide range of clinical manifestations.

21. Extra-articular manifestations
• Ocular Manifestations
• Uveitis is one of the most common extra-
articular manifestations of the SpA,
• occurring in 25% to 40% of patients.
• there appears to be no correlation
between the course of inflammatory eye
disease and that of the arthritis.

22. Extra-articular manifestations
• The uveitis typically presents as acute
unilateral pain and photophobia; blurring
of vision may also occur.
• Recurrence despite successful treatment
is common. Cataracts and glaucoma with
associated increased intraocular pressure
and posterior synechiae can also be
observed

23. Extra-articular manifestations
• In addition, conjunctivitis is commonly observed
in SpA (up to 30% to 60% of ReA), typically in a
more transient and less relapsing course than
uveitis
• patients who present with visual complaints in
the SpA setting should be referred promptly to
an ophthalmologist for thorough evaluation
including slit lamp examination.

24. Cutaneous
Manifestations
• Plaque psoriasis, characterized by scaly,
erythematous, hyperkeratotic lesions, is
the most common form of psoriasis and is
an important component of diagnosing
PsA.
• Careful examination of the skin should be
performed in SpA evaluation,

25. Cutaneous Manifestations
• assessment of less conspicuous areas
including the gluteal cleft, scalp and scalp
line, groin, and posterior auricular regions
should be performed.

26. Psoriasis

27. Psoriasis

28. Psoriasis

29. Psoriasis

30. Psoriasis

31. Cutaneous Manifestations
• no strict correlation between the extent of
psoriasis skin disease and the extent of
joint disease

32. Nail Changes
• Diffuse and numerous nail pitting (plate
depressions), onicholysis (separation of
the nail from underlying nail bed), and
crumbling of the nail plate can be
observed in both psoriasis and PsA.
• The extent of nail involvement in PsA
parallels both skin and joint disease

33. Nail pitting/onicholysis

34. Nail Pitting

35. Onicholysis toe nails

36. Crumbling nail/DIP joint involvement

37. Pencil in Cup deformities

38. Other Extra- articular Manifestations
• Aortic regurgitation is perhaps the most
recognized cardiac manifestation, with
scar tissue in the aorta and aortic valve
cusps leading to incompetent valves.
• Conduction abnormalities and heart block
have also been described, characterized
by extension of the fibrosis process into
the AV bundle

39. Other Extra- articular Manifestations
• Apical pulmonary fibrosis, mostly
asymptomatic and typically observed in
patients with substantial disease duration,
has been noted in a small percentage of
AS patients

40. Other Extra- articular Manifestations
• Renal disease beyond analgesic and
nonsteroidal anti-inflammatory drug
adverse effects has appeared in 2 forms
in AS, both of which are rarely observed:
IgA nephropathy that can manifest as
proteinuria and hematuria, and secondary
amyloidosis manifested by nephrotic
syndrome and, if associated with renal
failure, generally a poor prognosis.

41. Spondyloarthropathies-Gene association
• Up to 70% of individuals suffering from
SpA carry the HLA-B27 gene
• strength of the association between HLA-
B27 and disease susceptibility varies
among SpA subtypes and ethnic groups
• the B27 gene accounts for only 35% of the
SpA genetic load.[1]

42. Associations with HLA-B27
Degree of
associations
Rheumatic diseases
Ankylosing spondylitis >90%
Reiter’s syndrome/reactive >80%
arthritis
~75%
IBD related arthritis
~50%
Psoriatic arthritis
Normal Associations
Native Americans 13%
Caucasians 8%
Blacks 4%

43. Ankylosing Spondylitis
• An inflammatory disease of the spine and axial
joints
• Typically affects young men ages 15-30
• Women can be affected but much less than men
(3:1 men:women)
• Affects about 1 in 1000
• women with AS may present differently from
men, complaining of neck and breast pain
without the typical inflammatory low back pain

44. Ankylosing Spondylitis
New York criteria for diagnosis of ankylosing
spondylitis
• Definite ankylosing spondylitis If:
Criterion 4 or 5 plus 1, 2, or 3.
– 1. Limited lumbar motion
– 2. Low back pain for 3 months improved with exercise
not relieved by rest
– 3. Reduced chest expansion
– 4. Bilateral, grade 2 to 4, sacroiliitis on X-ray
– 5. Unilateral, grade 3 to 4, sacroiliitis on X-ray

45. Ankylosing Spondylitis

46. Previous Picture Hx
• A photographic series of a patient with
ankylosing spondylitis was taken over a period of
26 years. By 1957 a thoracic kyphosis has
become apparent, and loss of the normal lumbar
lordosis has occurred. There are early flexion
contractures of the hips and knees. In the next
two sequences, increasing flexion contractures of
the hips and knees have occurred along with
progressive ankylosis of the spine. In 1973,
bilateral total hip arthroplasties were performed,
which improved the patient's posture.

50. Ankylosing spondylitis: lumbar vertebrae, bamboo
spine (radiograph)

51. • This anteroposterior projection of the lumbar
spine shows the characteristic changes of
syndesmophyte formation or "bambooing."
Ossification is present deep to the lateral
collateral ligaments, and it follows the contour of
the intervertebral disc spaces. This ossification
involves the outer layers of the intervertebral
disc (annulus fibrosus). In contrast, degenerative
spurs will extend almost at right angles to the
vertebral bodies, rather than snugly following the
contour of the disc as in ankylosing spondylitis.

52. Sacroiliitis grading
0-normal
1-possible
2-minimal
3-moderate
4-ankylosing

53. Ankylosing Spondylitis
• Begins in the Sacroiliac Joints and progresses
upwards and can involve the entire spine
• Beyond the spine, axial joints including the
shoulders and hips can be involved with varying
degrees of severity particularly in AS.
• Shoulder involvement can be detected
radiographically with characteristic erosive
changes at the rotator cuff insertion.[
• Hip involvement is considered a poor prognostic
factor in AS

57. Ankylosing Spondylitis
• Inflammatory Stages
– Can be extremely painful (flares)
– Prolonged morning stiffness (hours)
– Fatigue (pain & lack of sleep)
• Ankylosis
– Stiffness increases
– Significantly reduced ROM
– Abnormal posture

59. Enthesitis
• Inflammation at the insertion of tendon,
ligament, or articular capsule into bone
• Lower limb entheses are more commonly
involved than the upper limb
• Patients with AS get “funny” pains

60. Achilles Enthesopathy

61. Enthesitis

63. Enthesitis
• Common sites for Enthesitis
– Heel – Most Common (Achilles)
– Patella
– Tibial Tubercle
– Base of the 5th metatarsal
– Plantar Fascia
– Other sites include:
• Anserine Bursa
• Greater Trochanter
• Iliac Crest
• Rotator Cuff (Common in Ankylosing Spondylitis)
• Costochondral

64. Physical Examination
• Peripheral Entheseal Involvement
• Spinal Involvement

65. Spinal Involvement
• Measure the Occiput to Wall distance
• Chest Expansion
– Place the tape measure at the xiphisternum
– Deep inhalation, exhalation, then inhalation
– Measurement is age and sex dependant
– Useful for following patients over time
• Modified Schober Test
– Potentially useful diagnostically
– Unsure of benefit over time (insensitive)

66. Modified Schober Test

67. Occiput to Wall Test

68. Spinal Involvement
• Finger Tip to Floor Distance
– Feet at specified distance apart
– Measure fingertips to floor
– Useful for following patients over time
• Lateral Flexion
– Stand with back against the wall and feet at specified
distance apart
– Make a mark where the finger tips touch the thigh
– Ask the patient to flex laterally and place a second
mark at the maximal extent of the fingertips
– Measure the distance between the two marks
– Useful for following patients over time

69. Finger Tip to Floor Distance

70. Spinal Involvement
• SI Compression Testing
• Cervical Spine
– Examine!
– Can have Cervical Spine involvement alone
– Can result in Atlanto-Axial Instability

71. Laboratory Investigations
• Evidence of Inflammation
– CBC Normochromic normocytic anemia
– Elevated ESR/CRP
– Reactive thrombocytosis
• HLA-B27 found in 90-95% of patients with
Ank Spond vs 6% of general population

72. Juvenile Ankylosing Spondylitis
Onset 8 to 14
Sex Ratio M:F 7 to 1
HLA-B27 91 %
Systemic symptoms rare
Iritis 10 to 27 %
Polyarticular 97%
Prognosis Probably good

73. PSORIATIC ARTHRITIS

74. Case #1
• 50 y.o. man presents to the office with
painful, swollen fingers
• Intermittent flares over the last year with
limited morning stiffness and slight loss of
energy
• Presents with the following findings:

75. Case #1

76. Case #1

77. Case #2
• 48 year old woman originally from Russia,
moved to Canada 1 year ago.
• Has complained of persistent arthritis and
deformities in hands for years.
• Presents with the following findings:

78. Case #2

79. Case #2

80. Case #3
• 51 year old man has had pain, stiffness,
and progressive deformity in the hands for
many years
• Presents with the following findings:

81. Case #3

82. Case #4
• 28 year old man presents with pain and
stiffness in both wrists and the left knee
• Also has intermittent swelling of the toes
• Has the following findings:

83. Case #4

84. Case #4

85. Case #5
• 35 year old man presents with low back
pain and stiffness which improves with
exertion
• He has the following findings:

86. Case #5

87. What is the Diagnosis?
• In all of these cases the diagnosis is
Psoriatic Arthritis!
• 5 Presentations
– DIP Involvement (Case #1)
– Arthritis Mutilans (Case #2)
– Symmetric Polyarthritis (Case #3)
– Oligoarthritis (Case #4)
– Spondylitis (Case #5)

88. What is the Diagnosis?
Confusion
Psoriatic Arthritis is a heterogenous disease
which can present in a multitude of ways

89. Psoriasis/Nail changes/arthritis
DIPs and Sausage deformity

90. Psoriatic Arthritis PsA
• Subchondral bone resorption of the distal
interphalangeal joint of the thumb and
middle fingers has resulted in the "pencil-
in-cup" appearance. A flexion deformity of
the distal interphalangeal joint of the small
finger is present, and the corresponding
joint of the ring finger has fused.
• (Next slide)

91. Fusion
Pencil in Cup
deformity

92. Who Gets Psoriatic Arthritis?
• Most common age of onset is 30-50 years
old
• 1-3% of the population has psoriasis
• 6-42% of people with psoriasis get
psoriatic arthritis
• Therefore, it is rare (about 1 in 1000)

93. History – Initial Presentation
• Typical presentation is a peripheral
inflammatory joint disease – usually a
mono or oligo arthritis
– Knees
– Wrists
• May occasionally present with polyarthritis
• Initial presentation of inflammatory spinal
disease is rare

94. History - Progression
• Oligoarticular in 40-50%
• Polyarticular in 30-50%
– Like Rheumatoid Arthritis
• Predominant Spinal Disease in 5%
– Spinal symptoms usually occur after many
years of peripheral arthritis
• DIP involvement in 5%
• Arthritis Mutilans in 5%

95. PSORIATIC ARTHRITIS
Possible Indicators of Bad Prognosis
Younger age at onset, including children
Presence of certain HLA antigens:
HLA-B27 correlates with spondylitic involvement
HLA-DR3, DR4 correlates with erosive disease
Extensive skin involvement
Polyarticular involvement
Lack of clinical response to NSAIDs
Association with HIV infection

96. History - Progression
• Sacroiliac Involvement
– Sacroiliitis in 1/3 of patients
– Usually asymmetric (unilateral)
– May be asymptomatic
• Spinal Involvement
– May affect any part of the spine in a random
fashion
– Different from ankylosing spondylitis

97. History – Rheumatologic Review of
Systems
• Mucocutaneous Involvement
– Psoriatic skin lesions
– Psoriatic Nail lesions
• Entheseal Involvement
• Dactylitis
• Ocular Involvement

98. History - Psoriasis
• Do you have Psoriasis?
– Psoriasis present before the onset of joint disease
(70%)
– Psoriasis comes with the arthritis (15%)
– Psoriasis comes after the arthritis (15%)

99. Umbilical Psoriasis

100. Psoriasis Behind the Ear

101. Psoriasis in the Crease

102. Psoriasis in the Nails

103. History - Dactylitis
• Have you ever had a finger or toe swell up
on you like a sausage?
• Entire digit is involved compared to
“fusiform” swelling around a joint
• Dactylitis – represents inflammation of the
flexor tenosynovium – “flexor
tenosynovitis”

104. History - Dactylitis

105. History – Family History
• Family studies suggest a 50-fold increase
in the risk of psoriatic arthritis in 1st degree
relatives
• Fathers are twice as likely to transmit the
disease

106. Physical Examination
• Skin and Nail Involvement
• Peripheral Joint Involvement
• Peripheral Entheseal Involvement
• Spinal Involvement
– Modified Schober Test
– Occiput to Wall Distance
– Cervical Spine ROM
– Finger tip to floor distance
– Lateral flexion

107. Peripheral Joint Involvement
• Inflammatory Joint Count
– Number of Joints Involved
• Prognostic Importance
• Therapeutic Importance
– Pattern of Joints Involved
• Diagnostic Importance
– Evidence of Damage
– Dactylitis

108. How to Tell the Difference

109. Red Flags – When to Call the
Rheumatologist
• Cervical Spine Instability
– Acute onset neck pain or headache
– Weakness or sensory change in extremities
– Hyper-reflexia
• Flare of disease activity

110. Radiology

111. Reiter’s Syndrome: Diagnostic Criteria
Seronegative asymmetric arthropathy
And at least 1 of the following:
Urethritis or cervicitis
Diarrhea
Inflammatory eye disease
Mucocutaneous disease
Balanitis, oral ulceration, or keratoderma
Other spondyloarthropathies and rheumatic
disease excluded

112. BACTERIA MOST COMMONLY FOUND
TRIGGERING REACTIVE ARTHRITIS
Borrelia burgdorferi
Campylobacter jejuni
Chlamydia trachomatis
Neisseria gonorrhoeae
Salmonella species
Shigella flexneri
Streptococcus pyogenes
Yersinia enterocolitica
Yersinia pseudotuberculosis

113. Reiter’s Syndrome
Frequency Difficult to
define
Male – Female ratio 4 to 1
HLA-B27 90 %
Onset Simultaneous
or
over 3 – 4
weeks
Course Self limited
Relapsing

114. Reiter’s Syndrome
Clinical Manifestations
Arthritis Oligoarticular, Lower
extremities.
Enthesitis Calcaneous, patella.
Urethritis Dysuria more common
30 % urethritis
Conjuntivitis 2/3 at onset
Mucocutaneous
Manifestations Less frequent

115. Arthritis Conjunctivitis

116. Cervicitis

117. Urethritis

118. Circinate Balanitis. Meatitis, mild balanitis, severe balinitis

119. • Left, previous photograph demonstrates
meatitis and early balanitis. The lesion is
erythematous, moistened by the urethral
discharge, and, like the later stages, painless.
• Center, A discrete, moist, well-circumscribed
lesion is seen on the glans. Such lesions often
begin as small vesicles or pustules.
• Right, More advanced lesions are dry, scaly,
and painless. In circumcised patients, the
lesions on the glans may become crusted and
hyperkeratotic, similar to those seen on the
skin, or they may remain moist and weeping.

120. ReA superficial erosions of the
tongue

121. Superficial oral ulcers can be seen in
ReA and are typically painless

122. • ReA has as part of its constellation of
symptoms characteristic lesions including
keratoderma blennorrhagica characterized
by hyperkeratotic skin lesions on soles
and palms and genital lesions such as
circinate balanitis, an erythematous and
painless lesion of the glans penis.

123. Keratoderma Blenorrhagicum

124. Pustules ReA KB incipient

125. Advanced KB

126. ARTHRITIS AND INFLATORY
BOWEL DISEASE
Crohn’s Disease
Ulcerative Colitis

127. IBD associated arthritis
Arthritis Peripheral Specially lower
extremities
Self limited 4 to 6 weeks
Central SI joints and enthesitis
Frequency 7 to 21 %
No relation between the activity of the
Arthritis and the IBD.

128. In IBD-associated SpA, erythema nodosum can be
observed in Crohn's disease

129. pyoderma gangrenosum can be associated with
ulcerative colitis.

130. SINDROME DE SAPHO
SYNOVITIS
ACNE CONGLOBATA, FULMINANS E
HIDRAENITIS SUPURATIVA
PUSTULOSIS PALMO PLANTAR
HYPEROSTOSIS ESTERNO-CLAVICULAR Y
ESPINAL
OSTEOMIELITIS ESTERIL MULTIFOCAL
RECURRENTE

131. Management - Overview
• Goals of Treatment
– Improve pain
– Improve Function
– Prevent Long-term Damage
– Safely

132. Management
• NSAIDs
– Can be useful in some cases of mono/oligo
arthritis
– Useful for enthesitis
– Useful for spinal disease

133. Management: DMARDs
Ankylosing
Medication Psoriatic
Spondylitis
Hydroxychloroquine Rarely with little
NO
(Plaquenil®) evidence
Methotrexate Rarely with poor
YES efficacy in spinal
disease
Sulfasalazine YES YES
Leflunomide (Arava®)
YES NO
Gold YES NO
Steroids YES YES

134. Management: Biologics
• Biologics Approved for Psoriatic Arthritis
and Ankylosing Spondylitis
– Etanercept (Enbrel®)
– Infliximab (Remicade®)
– Adalimumab (Humira®)
• Biggest advance in the treatment of
spondyloarthropathies in decades!

135. Management: Biologics
• Dramatic clinical efficacy with the greatest
amount of data to date coming from
studies of ankylosing spondylitis and
psoriatic arthritis.
• Substantially improve both peripheral and
axial arthritis, as well as various other
clinical parameters, and has also slowed
the progression of joint damage.

137. SPONDYLOARTHOPATHIES Summary
Absence of rheumatoid factor
Involvement of sacroiliac and
spinal joints
Peripheral arthritis
(predominantly lower limb)
Enthesopathy
Familial clustering
Increased incidence of HLA-B27
Common spectrum of extra-articular features
(predominantly muco-cultaneous)