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BASIC INFORMATION

DEFINITION

Ankylosing spondylitis is a type of inflamma-tory arthritis involving the sacroiliac joints andaxial skeleton characterized by ankylosis andenthesitis (inflammation at tendon insertions).It is one of a family of overlapping syndromes

called seronegative spondyloarthropathies thatincludes reactive arthritis (Reiter syndrome),psoriatic spondylitis, and enteropathic arthritis.

SYNONYMS

Marie-Strümpell disease

ICD-9CM CODES720.0 Ankylosing spondylitisICD-10CM CODESM45.9 Ankylosing spondylitis of unspecified

sites in spine

EPIDEMIOLOGY &

DEMOGRAPHICS

PREVALENCE: Between 0.1% and 1% of thepopulationPREDOMINANT AGE AT ONSET: 15 to 35 yrPREDOMINANT SEX: Male/female ratio 2 to3:1

PHYSICAL FINDINGS & CLINICAL

PRESENTATION

• Prolonged morning back stiffness of insidiousonset lasting more than 3 mo

• Bilateral sacroiliac tenderness (sacroiliitis) • Limited lumbar spine motion (Fig. A1-87) • Tenderness at tendon insertion sites, espe-

cially the Achilles tendons and plantar fascia • Loss of chest expansion reflecting rib cage

involvement • Occasionally, peripheral joint arthritis, usu-

ally involving the large joints of the lowerextremities

• In advanced cases the typical posture con-sists of compensatory hyperextension ofneck, fixed flexion of hips, and compensatoryflexion of knees (Fig. A1-88)

• Extraskeletal manifestations may affect thecardiovascular system (aortic insufficiency),lungs (pulmonary fibrosis), and eye (uveitis),but are not usually severe.

ETIOLOGY

Genetic factors, particularly HLA-B27 , play animportant role in susceptibility to the spondy-loarthropathies. Infectious triggers have beenimplicated in some cases. Tumor necrosis factoris important in the inflammatory response.

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS

• Diffuse idiopathic skeletal hyperostosis(Forestier disease)

• Noninflammatory back pain (A clinical algo-rithm for the evaluation of back pain is

described in Section III.) • Table A1-55 compares ankylosing spondylitisand related disorders.

LABORATORY TESTS

• Elevated sedimentation rate, C-reactive pro-tein

• Mild hyperchromic anemia • Demonstration of inflammatory sacroiliitis by

radiography or MRI is essential for diagnosis • HLA/B27 antigen is not useful in the evalua-

tion of noninflammatory back pain because itis present in up to 8% to 10% of the normalpopulation.

IMAGING STUDIES

• Classic features are those of bilateral sacroi-liitis on radiographs of the pelvis

• Vertebral bodies lose anterior concave shapeand become square

• With progression, calcification of the annu-lus fibrosus and paravertebral ligamentsdevelop, giving rise to the so-called bam-boo spine and a “trolley track” appearance(Fig. A1-89).

• MRI (Fig A1-90) may be useful in detectingearly inflammatory lesions and is especiallyhelpful when the history is suggestive but

radiographs are equivocal.

ALG Ankylosing Spondylitis

FIGURE A1-87 Loss of lumbodorsal spine mobil-

ity in a boy with ankylosing spondylitis. The

lower spine remains straight when the patient bends

forward. (From Behrman RE: Nelson textbook of pedi-

atrics, ed 17, Philadelphia, 2005, Saunders.)

Compensatoryhyperextensionof neck

Fixed flexionof hips

Compensatoryflexion of knees

Loss of lumbarlordosis

Exaggeratedthoracic

kyphosis

Vertebrae fusedtogether

Normal posture Posture in patient withadvanced spondylitis

FIGURE A1-88 Ankylosing spondylitis. Typical posture in advanced cases compared with normal posture.

(From Ballinger A: Kumar & Clark’s essentials of clinical medicine, ed 6, Edinburgh, 2012, Saunders.)

TABLE A1-55 Comparison of Ankylosing Spondylitis and Related Disorders

FeatureAnkylosingSpondylitis

PsoriaticArthritis

ReactiveArthritis

EnteropathicArthropathy

Gender (M:F) 2-3:1 1:1 8:1 (GU) [1:1 (GI)] 1:1

Age at onset <40 35-55 20-40 Young adult

Sacroiliitis or spondylitis 100% ∼20% ∼40% <20%

Symmetry of sacroiliitis Symmetric Asymmetric Asymmetric Symmetric

Peripheral arthritis ∼25% 95% 90% 15%-20%

Distribution Axial and lower limbs Any joint Lower limbs Variable

HLA-B27 85%-95% 25% 30%-80% 7%

Uveitis 25%-40% 25% 25% 10%-36%

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



Ankylosing Spondylitis ALG 122

TREATMENT

NONPHARMACOLOGIC THERAPY

• Exercises primarily to maintain on flexibilityand aerobic activity are important

• Postural training 1. Patients must be instructed on spinal

extension exercises to avoid fusion in aflexed position

2. Sleeping should be in the supine positionon a firm mattress; pillows should not be

placed under the head or knees.

CHRONIC Rx

• NSAIDs: Patients with ankylosing spondylitisshould be prescribed full-dose continuousNSAID therapy. There is anecdotal evidencesuggesting that indomethacin may be moreeffective than other NSAIDs, but other NSAIDs

are efficacious and may be better tolerated.One study suggested that continuous NSAIDtherapy may retard the radiographic progres-sion of ankylosing spondylitis.

• Sulfasalazine may be efficacious in somepatients, especially for peripheral arthritis

• Tumor necrosis factor (TNF) antagonists suchas etanercept, infliximab, and adalimumabhave been shown to be very effective for

relieving symptoms of spinal inflammatoryarthritis in numerous controlled studies. Anti-TNF therapy should be recommended forpatients whose symptoms are not completelycontrolled with NSAIDs, and it sometimesresults in dramatic improvement in symp-toms, range of motion of the spine, and qual-ity of life for these patients. There is evidencesuggesting that anti-TNF therapy slows theradiographic progression of the disease.

DISPOSITION

Most patients have a normal life span but manysuffer significant disability from loss of spinalmobility.

REFERRAL

All patients with seronegative spondyloarthrop-athy should be referred to a rheumatologist forconsideration of anti-TNF therapy.

PEARLS &CONSIDERATIONS

A family history of seronegative spondyloar-thropathy increases the specificity of testingfor HLA-B27.

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.

A B

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: Kelley’s textbook of rheumatology, ed 7, Philadelphia, 2005, Saunders.)

A B C

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

[MRI]). A 43-year-old man with HLA-B27–positive 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, et al:

Kelley’s textbook of rheumatology , ed 9, Philadelphia, 2013, Saunders.)

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Ankylosing Spondylitis

SUGGESTED READINGS

Brown J: Ankylosing spondylitis, Lancet 369:1379–1390, 2007.

Davis JC Jr et al.: 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 et al.: The impact of tumor necrosis factor inhibitors on radiographic

progression in ankylosing spondylitis, Arthritis Rheum 65:2645, 2013.

Heiberg MS et al.: The comparative one-year performance of anti-tumor necrosis

factor alpha drugs in patients with rheumatoid arthritis, psoriatic arthritis, andankylosing spondylitis: results from a longitudinal, observational, multicenter

study, Arthritis Rheum 59:234, 2008.

Oosttveen J et al.: Early detection of sacroiliitis on magnetic resonance imaging

and subsequent development of sacroiliitis on plain radiography: a prospec-

tive, longitudinal study, J Rheumatol 26:19523–19528, 1999.

Wanders A et al.: 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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