eMedicine Specialties > Ophthalmology > Connective Tissue Disorders
Ankylosing Spondylitis
Updated: Jan 5, 2008
Introduction
Background
Ankylosing spondylitis (AS) is a chronic, progressive inflammatory disorder primarily affecting the axial skeleton. AS is one of the seronegative spondyloarthropathies, a group of multisystem inflammatory diseases that affects the spine, peripheral joints, adjacent soft tissues, and extra-articular structures. Most of these disorders are associated with the human leukocyte antigen B27 (HLA-B27) antigen.
In addition to AS, the seronegative spondyloarthropathies include the following:
- Reactive arthritis (also referred to as Reiter syndrome)
- Inflammatory bowel disease associated arthritis
- Psoriatic arthropathy
- Juvenile spondyloarthropathy
- Undifferentiated spondyloarthropathies
Several of these disorders share some clinical features, such as sacroiliitis, spondylitis, asymmetric peripheral arthritis, aortitis, clinically evident or occult bowel lesions, and uveitis. On the other hand, some clinical features are more disease specific, such as the urethritis or cervicitis of reactive arthritis or the skin lesions and nail changes in psoriatic arthritis.
Diagnosis of AS is established by clinical and radiographic criteria. The Modified New York Criteria require 1 of 3 clinical features, as well as characteristic radiographic features, to diagnose AS. Clinical criteria for diagnosis of AS include the following: lower back pain greater than 3 months in duration that improves with activity but is not relieved by rest, limited lumbar spine motion in the sagittal and frontal planes, and decreased chest expansion. Characteristic radiographic findings include the following: pseudowidening of the sacroiliac joints, sclerosis, narrowing, and ankylosis.
HLA-B27 typing is not necessary to establish the diagnosis, although it is a useful prognostic indicator in patients with uveitis.
Pathophysiology
Acute anterior uveitis occurs in as many as 30% of patients with AS, particularly in those with the HLA-B27 allele. The exact role of HLA-B27 in the pathology of uveitis is not precisely known, but several theories for its role in disease exist. The oldest theory is that of molecular mimicry, in which an infectious agent triggers an immune response against autoantigens. The HLA-B27 antigen shares some amino acid homology with proteins from several gram-negative bacteria, including Yersinia enterocolitica, Shigella flexneri, and Chlamydia trachomatis, as well as gram-negative bacteria found in the GI tract.
Other theories include the arthritogenic peptide theory, in which the immune response is directed against a peptide presented by HLA-B27; the altered self theory, in which a reactive sulfhydryl group on the HLA-B27 molecule interacts with other self peptides, thereby inducing antigenicity; and, finally, the theory that the HLA-B27 allele confers an altered immune response that directly relates to the disease.
Frequency
United States
Anterior uveitis occurs in 25-30% of patients at some time during the course of the AS.
Mortality/Morbidity
Patients with AS and the HLA-B27 antigen tend to develop uveitis at a younger age, have a longer duration of ocular disease, and have more recurrences. Additionally, patients who are HLA-B27 positive appear to have more severe disease in terms of anterior chamber inflammation and vitritis, resulting in a higher incidence of complications and the requirement for more aggressive therapy.
Age
Uveitis is seen at an earlier age than with idiopathic uveitis.
Clinical
History
A thorough history should be performed, to include the following:
- Chief complaint and history of present illness
- Patients often complain of redness, pain, photophobia, blurry vision, and occasionally tearing. No history of ocular discharge is present.
- Many cases of uveitis are unilateral but may be bilateral.
- Attacks often are relatively acute in onset. Some patients may present with chronic symptoms of blurring and ocular hyperemia.
- Past medical history is important to establish the diagnosis of AS.
- Age at onset - Systemic symptoms often develop during late adolescence through early adulthood.
- Systemic manifestations
- Chronic lower back pain
- Chronic back stiffness
- Hip/shoulder involvement
- Neurologic involvement - Symptoms associated with spinal dislocations, subluxations, fractures, cauda equina syndrome
- Cardiovascular manifestations - Aortitis, aortic/mitral insufficiency, conduction defects
- Current medications for systemic manifestations
- Past ocular history should include the following:
- Previous episodes, duration, and treatment
- Previous complications
- Cataract
- Glaucoma
- Cystoid macular edema (CME)
- Epiretinal membrane
Physical
Patients suspected of having AS should undergo a thorough physical examination, preferably by a rheumatologist.
- A complete ophthalmic examination should be performed with special emphasis on the following:
- Best-corrected distance and near acuity
- Pupil examination
- Slit lamp examination
- Conjunctiva and sclera - Conjunctival injection, watery discharge, no follicles or papillae
- Cornea - Small-to-medium keratic precipitates
- Anterior chamber - Cells and flare, fibrin, and/or hypopyon not uncommon
- Iris - Posterior synechiae, peripheral anterior synechiae, pupillary membrane, no nodules
- Lens - Posterior subcapsular cataract
- Vitreous - Anterior vitreous cells, vitritis
- Intraocular pressure - Often low during acute exacerbations, secondary glaucoma, hypotony, phthisis
- Dilated fundus examination - Vitritis, CME, retinal vasculitis, epiretinal membrane
Causes
The cause of AS is unknown.
More on Ankylosing Spondylitis |
 Overview: Ankylosing Spondylitis |
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| Follow-up: Ankylosing Spondylitis |
| References |
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References
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Further Reading
Keywords
AS, human leukocyte antigen B27, HLA-B27, seronegative spondyloarthropathies, uveitis, anterior uveitis, acute anterior uveitis, AAU, ankylosing spondylitis associated uveitis, AS associated uveitis, HLA-B27 associated uveitis, inflammatory diseases
