Ankylosing spondylitis

BASICS
DESCRIPTION:
A chronic, usually progressive, condition in which inflammatory changes and new bone formation occurs at
the attachment of tendons and ligaments to bone (enthesopathy)
Sacroiliac joint involvement is the hallmark of ankylosing spondylitis with variable degrees of spinal
involvement. However, 20-30% of patients also have larger peripheral joint involvement
System(s) affected: Musculoskeletal
Genetics: Familial clustering and higher than expected frequency of HLA-B27 tissue antigen
Incidence/Prevalence in USA:
0.5-5 per 1000 in white males
Less common in women and Blacks
Predominant age:
Usually symptoms begin in early twenties
Onset of symptoms - rarely occurs after age 40
Predominant sex: Male > Female

SIGNS AND SYMPTOMS:

Subgluteal or low back pain and/or stiffness
Insidious onset
Onset usually in 3rd decade
Duration greater than 3 months
Morning stiffness
Frequently awaken at night to "walk off" stiffness
Improvement in stiffness with activity
Increased symptoms with rest
Pleuritic chest pain is often an early feature
Thoracic and cervical spine complaints in advanced disease
Hip, shoulder, or knee complaints
Diminished range of motion in the lumbar spine in all three planes of motion
Loss of lumbar lordosis
Thoracocervical kyphosis (rarely occurs before ten years of symptoms)
Aortic root dilatation (20%)
Aortic regurgitation murmur (2%)
Acute anterior uveitis (20-30%)
Osteoporosis

CAUSES:
Unknown

RISK FACTORS:
HLA-B27
Positive family history
10% risk of developing AS (Ankylosing Spondylitis) for HLA-B27 positive child of spondylitic parent

DIAGNOSIS
DIFFERENTIAL DIAGNOSIS:
Reiter's syndrome
Psoriatic arthritis
Diffuse idiopathic skeletal hypertrophy (DISH)
Spondylitis associated with inflammatory bowel disease
Rheumatoid arthritis

LABORATORY:
HLA-B27 tissue antigen is present in 90% of patients compared to 5-8% incidence in general population
Erythrocyte sedimentation rate (ESR) is elevated in 80% of cases, but correlates poorly with disease
activity and prognosis
Absent rheumatoid factor
Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS:
Erosive changes coupled with new bone formation at attachment of tendons and ligaments to bone
resulting in ossification of periarticular soft-tissues
Synovial changes are indistinguishable from rheumatoid arthritis. Erosion of articular cartilage is less
severe than in rheumatoid arthritis.

SPECIAL TESTS:
Synovial fluid - mild leukocytosis, decreased viscosity
Cerebrospinal fluid - increased protein
EKG - conduction defects
Measurement of respiratory excursion of chest wall - less than 5 cm maximal respiratory excursion of
chest wall measured at fourth intercostal space. Less than 2.5 cm is virtually diagnostic of ankylosing
spondylitis.
Wright-Schober test for lumbar spine flexion is abnormal

IMAGING:
Sacroiliac joint early - sclerosis on both sides of joint not extending more than 1 cm from articular
surface
Sacroiliac joint late - ankylosis of sacroiliac joint
Spine - "squaring" of vertebral bodies and ossification of annulus fibrosis giving appearance of "bamboo
spine". Ankylosis of facet joints.
Peripheral joint - symmetric erosive changes in larger joints. Pericapsular ossification, sclerosis, loss of
joint space.

DIAGNOSTIC PROCEDURES:
Physical examination
Radiographs - sacroiliac joint films, lumbar spine series

TREATMENT
APPROPRIATE HEALTH CARE:
Outpatient

GENERAL MEASURES:
Posture training and range of motion exercises for spine are essential
Firm bed
Sleep in prone position or supine without a pillow
Breathing exercises 2-3 times/day
Swimming
Physical therapy
Stop smoking, if a smoker

SURGICAL MEASURES:
N/A

ACTIVITY:
Encourage active lifestyle

DIET:
No special diet

PATIENT EDUCATION:
For a listing of sources for patient education materials favorably reviewed on this topic, physicians may
contact: American Academy of Family Physicians Foundation, P.O. Box 8418, Kansas City, MO 64114,
(800)274-2237, ext. 4400

MEDICATIONS
DRUG(S) OF CHOICE:
Nonsteroidal anti-inflammatory drugs provide symptomatic relief
Selection is empiric, but traditionally indomethacin, 50 mg tid or qid has been used
Steroids and cytotoxic agents are not effective
Contraindications: See Precautions
Precautions:
All patients on long term NSAIDs should have renal function monitored
NSAIDs may aggravate peptic ulcer disease or cause gastritis
Don't use NSAIDs for patients with a bleeding diathesis or patients requiring anticoagulants
Significant possible interactions: Refer to manufacturer's profile of each drug

ALTERNATIVE DRUGS:
Other NSAIDs, such as sulindac, naproxen

FOLLOW UP
PATIENT MONITORING:
Visits every six to twelve months to monitor posture and range of motion

PREVENTION/AVOIDANCE:
N/A

POSSIBLE COMPLICATIONS:
Spine: Pseudarthrosis, cervical spine fracture (high mortality rate), C1-C2 subluxation, spondylodiscitis,
cauda equina syndrome (rare)
Peripheral joint ankylosis
Pulmonary: Restrictive lung disease, diaphragmatic breathing, upper lobe fibrosis (rare)
Cardiac: Conduction defects (20%), aortic insufficiency (2%)
Uveitis

EXPECTED COURSE AND PROGNOSIS:

Unpredictable course
Prognosis good if mobility and upright posture maintained. Usually progressive disability.

MISCELLANEOUS
ASSOCIATED CONDITIONS:
Inflammatory bowel disease
Uveitis
Iritis

AGE-RELATED FACTORS:
Pediatric: N/A
Geriatric: N/A
Others: N/A

PREGNANCY:
N/A

SYNONYMS:
Rheumatoid spondylitis
Marie-Strumpell disease

ICD-9-CM:

720.0 ankylosing spondylitis

SEE ALSO:
Reiter's syndrome
Crohn's disease
Ulcerative colitis
Arthritis, psoriatic
Arthritis, rheumatoid (RA)
OTHER NOTES:
Flexion contractures of the hip and ankylosis may be major contributors to poor posture. Hip arthroplasty
should be strongly considered as it may restore upright posture. Heterotopic ossification may occur post
operatively and appropriate prophylaxis should be considered.

ABBREVIATIONS:
N/A

REFERENCES
Calin A, ed: Spondyloarthropathies. New York, Grune & Stratton, 1983
Calin A, Fries J: Ankylosing Spondylitis Discussions in Patient Management. Garden City, New York,
Medical Examination Publishing Company, 1978

Web citations:
N/A

Author(s):
Mark R. Dambro, MD, FAAFP, FABHPM

IMAGES
Illustrations:
N/A