ALG

BASIC INFORMATION
DEFINITION
Ankylosing spondylitis is a type of inflammatory arthritis involving the sacroiliac joints and
axial skeleton characterized by ankylosis and
enthesitis (inflammation at tendon insertions).
It is one of a family of overlapping syndromes
called seronegative spondyloarthropathies that
includes reactive arthritis (Reiter syndrome),
psoriatic spondylitis, and enteropathic arthritis.

ICD-9CM CODES
720.0 Ankylosing spondylitis
ICD-10CM CODES
M45.9 Ankylosing spondylitis of unspecified
sites in spine

EPIDEMIOLOGY &
DEMOGRAPHICS
PREVALENCE: Between 0.1% and 1% of the
population
PREDOMINANT AGE AT ONSET: 15 to 35 yr
PREDOMINANT SEX: Male/female ratio 2 to
3:1

LABORATORY TESTS
Elevated sedimentation rate, C-reactive protein
Mild hyperchromic anemia
Demonstration of inflammatory sacroiliitis by
radiography or MRI is essential for diagnosis
HLA/B27 antigen is not useful in the evaluation of noninflammatory back pain because it
is present in up to 8% to 10% of the normal
population.

In advanced cases the typical posture consists of compensatory hyperextension of

neck, fixed flexion of hips, and compensatory
flexion of knees (Fig. A1-88)
Extraskeletal manifestations may affect the
cardiovascular system (aortic insufficiency),
lungs (pulmonary fibrosis), and eye (uveitis),
but are not usually severe.

ETIOLOGY
Genetic factors, particularly HLA-B27, play an
important role in susceptibility to the spondyloarthropathies. Infectious triggers have been
implicated in some cases. Tumor necrosis factor
is important in the inflammatory response.

IMAGING STUDIES
Classic features are those of bilateral sacroiliitis on radiographs of the pelvis
Vertebral bodies lose anterior concave shape
and become square
With progression, calcification of the annulus fibrosus and paravertebral ligaments
develop, giving rise to the so-called bamboo spine and a trolley track appearance
(Fig. A1-89).
MRI (Fig A1-90) may be useful in detecting
early inflammatory lesions and is especially
helpful when the history is suggestive but
radiographs are equivocal.

DIAGNOSIS
DIFFERENTIAL DIAGNOSIS


Diffuse idiopathic skeletal hyperostosis
(Forestier disease)
Noninflammatory back pain (A clinical algorithm for the evaluation of back pain is
described in Section III.)
Table A1-55 compares ankylosing spondylitis
and related disorders.

PHYSICAL FINDINGS & CLINICAL

PRESENTATION
Prolonged morning back stiffness of insidious
onset lasting more than 3 mo
Bilateral sacroiliac tenderness (sacroiliitis)
Limited lumbar spine motion (Fig. A1-87)
Tenderness at tendon insertion sites, especially the Achilles tendons and plantar fascia
Loss of chest expansion reflecting rib cage
involvement
Occasionally, peripheral joint arthritis, usually involving the large joints of the lower
extremities

Compensatory
hyperextension
of neck

Exaggerated
thoracic
kyphosis
Loss of lumbar
lordosis

Vertebrae fused
together
Fixed flexion
of hips

Compensatory
flexion of knees

Normal posture

Posture in patient with

advanced spondylitis

FIGURE A1-88 Ankylosing spondylitis. Typical posture in advanced cases compared with normal posture.
(From Ballinger A: Kumar & Clarks essentials of clinical medicine, ed 6, Edinburgh, 2012, Saunders.)

TABLE A1-55 Comparison of Ankylosing Spondylitis and Related Disorders

FIGURE A1-87 Loss of lumbodorsal spine mobility in a boy with ankylosing spondylitis.The
lower spine remains straight when the patient bends
forward. (From Behrman RE: Nelson textbook of pediatrics, ed 17, Philadelphia, 2005, Saunders.)

Feature

Ankylosing
Spondylitis

Psoriatic
Arthritis

Reactive
Arthritis

Enteropathic
Arthropathy

Gender (M:F)
Age at onset
Sacroiliitis or spondylitis
Symmetry of sacroiliitis
Peripheral arthritis
Distribution
HLA-B27
Uveitis

2-3:1
<40
100%
Symmetric
25%
Axial and lower limbs
85%-95%
25%-40%

1:1
35-55
20%
Asymmetric
95%
Any joint
25%
25%

8:1 (GU) [1:1 (GI)]

20-40
40%
Asymmetric
90%
Lower limbs
30%-80%
25%

1:1
Young adult
<20%
Symmetric
15%-20%
Variable
7%
10%-36%

From Hochberg MC etal: Rheumatology, ed 5, St Louis, 2011, Mosby.

121

Diseases
and Disorders

SYNONYMS
Marie-Strmpell disease

Ankylosing Spondylitis

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Ankylosing Spondylitis

ALG
are efficacious and may be better tolerated.
One study suggested that continuous NSAID
therapy may retard the radiographic progression of ankylosing spondylitis.


Sulfasalazine may be efficacious in some
patients, especially for peripheral arthritis
Tumor necrosis factor (TNF) antagonists such
as etanercept, infliximab, and adalimumab
have been shown to be very effective for
relieving symptoms of spinal inflammatory
arthritis in numerous controlled studies. AntiTNF therapy should be recommended for
patients whose symptoms are not completely
controlled with NSAIDs, and it sometimes
results in dramatic improvement in symptoms, range of motion of the spine, and quality of life for these patients. There is evidence
suggesting that anti-TNF therapy slows the
radiographic progression of the disease.

FIGURE A1-89 Ankylosing spondylitis. A, Fusion of the facet joints and ossification of the adjacent soft
tissue have produced a trolley track appearance (arrows). The sacroiliac joints are fused. Syndesmophytes
are present. B, In another patient, there is a prominent fusion of the interspinous ligaments producing a saber
sheath appearance. (From Harris ED: Kelleys textbook of rheumatology, ed 7, Philadelphia, 2005, Saunders.)

DISPOSITION
Most patients have a normal life span but many
suffer significant disability from loss of spinal
mobility.
REFERRAL
All patients with seronegative spondyloarthropathy should be referred to a rheumatologist for
consideration of anti-TNF therapy.

PEARLS &
CONSIDERATIONS
A family history of seronegative spondyloarthropathy increases the specificity of testing
for HLA-B27.

FIGURE A1-90 Spine inflammation in ankylosing spondylitis (magnetic resonance imaging

[MRI]).A 43-year-old man with HLA-B27positive ankylosing spondylitis with deteriorating symptoms,
including inflammatory back pain, had an MRI scan before starting biologic therapy. Baseline sagittal short
tau inversion recovery (STIR) MRI (A) shows diffuse increased signal (edema) in the T2 vertebral body and
multiple foci of corner inflammation anteriorly at T5 and T6, and posteriorly at T7, T8, T9, and T10 (arrows).
Other images confirmed extensive active inflammation in the spine. The patient responded very well, and
after 6 months of therapy, a repeat STIR MRI (B) showed complete resolution of bone marrow inflammation.
Subsequently, the patient experienced recurrence of symptoms, and a third MRI (C) was performed (2 months
after anti-TNF therapy was stopped). This MRI shows no edema at T5-T6, a conspicuous new lesion anteriorly
at T7, and recurrent inflammation posteriorly in the lower thoracic spine (arrows). (From Firestein GS, etal:
Kelleys textbook of rheumatology, ed 9, Philadelphia, 2013, Saunders.)

TREATMENT
NONPHARMACOLOGIC THERAPY
Exercises primarily to maintain on flexibility
and aerobic activity are important
Postural training
1. 
Patients must be instructed on spinal
extension exercises to avoid fusion in a
flexed position

2. Sleeping should be in the supine position

on a firm mattress; pillows should not be
placed under the head or knees.

CHRONIC Rx
NSAIDs: Patients with ankylosing spondylitis
should be prescribed full-dose continuous
NSAID therapy. There is anecdotal evidence
suggesting that indomethacin may be more
effective than other NSAIDs, but other NSAIDs

SUGGESTED READINGS
Available at www.expertconsult.com
RELATED CONTENT
Fig. 3-194 Spondyloarthropathy, diagnosis
(Algorithm)
Fig. 3-195 Spondyloarthropathy, treatment
(Algorithm)
Ankylosing Spondylitis (Patient Information)
AUTHOR: BERNARD ZIMMERMANN, M.D.

Ankylosing Spondylitis
SUGGESTED READINGS
Brown J: Ankylosing spondylitis, Lancet 369:13791390, 2007.
Davis JC Jr etal.: Health-related quality of life outcomes in patients with active
ankylosing spondylitis treated with adalimumab: results from a randomized
controlled study, Arthritis Rheum 57(6):1050, 2007.
Haroon N etal.: The impact of tumor necrosis factor inhibitors on radiographic
progression in ankylosing spondylitis, Arthritis Rheum 65:2645, 2013.
Heiberg MS etal.: The comparative one-year performance of anti-tumor necrosis
factor alpha drugs in patients with rheumatoid arthritis, psoriatic arthritis, and
ankylosing spondylitis: results from a longitudinal, observational, multicenter
study, Arthritis Rheum 59:234, 2008.
Oosttveen J etal.: Early detection of sacroiliitis on magnetic resonance imaging
and subsequent development of sacroiliitis on plain radiography: a prospective, longitudinal study, J Rheumatol 26:1952319528, 1999.
Wanders A etal.: Nonsteroidal antiinflammatory drugs reduce radiographic
progression in patients with ankylosing spondylitis: a randomized clinical trial,
Arthritis Rheum 52(6):1756, 2005.

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