'Ankylosing spondylitis' ('AS'; also known as 'Bechterew's disease'; 'Bechterew syndrome'; 'Marie Strümpell disease' / 'Marie Struempell disease' / 'Spondyloarthritis') is a chronic, painful,
degenerative inflammatory
arthritis primarily affecting
spine and
sacroiliac joints, causing eventual fusion of the spine; it is a member of the group of the
autoimmune spondyloarthropathies with a probable genetic
predisposition. Complete fusion results in a complete rigidity of the spine, a condition known as 'bamboo spine'.
[1]
Signs and symptoms
The typical patient is young, of 15 to 30 years of age with
chronic pain and stiffness in the lower part of the spine. Men are affected more than 2:1 over women.
[2] In 40% of cases, ankylosing spondylitis is associated with
iridocyclitis (anterior
uveitis, also known as iritis) causing eye pain and
photophobia (increased sensitivity to light). Other common symptoms are recurring
mouth ulcers (aphthae) and
fatigue. Pain fluctuation from one side to the other.
Typical
prodromes (early symptoms) may occur at a very young age (e.g. 3 years old), where the patient may experience recurring painful joints (e.g. knees, elbows), commonly misinterpreted as simple rheumatism.
AS is also associated with
ulcerative colitis,
Crohn's disease,
psoriasis, and
Reiter's disease.
Diagnosis
The ankylosis process.
There is no direct test to diagnose AS. A clinical examination and
X-ray studies of the spine, which show characteristic spinal changes and
sacroiliitis, are the major diagnostic tools. A drawback of X-ray diagnosis is that signs and symptoms of AS have usually been established as long as 8-10 years prior to X-ray evident changes occurring on a plain film X-ray, which means a delay of as long as 10 years before adequate therapies can be introduced. An option for more accurate (and much earlier) diagnosis are
tomography and
magnetic resonance imaging of the sacroiliac joints. The
Schober's test is a useful clinical measure of flexion of the lumbar spine performed during examination.
[3]
During acute inflammatory periods, AS patients will usually show an increase in the blood concentration of
C-reactive protein (CRP) and an increase in the
erythrocyte sedimentation rate (ESR).
Variations of the HLA-B gene increase the risk of developing ankylosing spondylitis, although it is not a diagnostic test. Those with the
HLA-B27 variant are at highest risk of developing the disorder. HLA-B27, demonstrated in a
blood test, is occasionally used as a diagnostic, but does not distinguish AS from other diseases and is therefore not of real diagnostic value. Over 95% of people with AS are HLA-B27 positive, although this ratio varies from population to population (only 50% of African American patients with AS possess HLA-B27, and it is close to 80% among AS patients from Mediterranean countries).
The Bath Ankylosing Spondylitis Disease Activity Index (
BASDAI), developed in
Bath (UK), is an index designed to detect the inflammatory burden of active disease. The BASDA can help to establish a diagnosis of AS in the presence of other factors such as HLA-B27 positivity, persistent buttock pain which resolves with exercise, and X-ray or MRI evident involvement of the sacroiliac joints. (See: "Diagnostic Tools", below)
[4]
It can be easily calculated and accurately assesses a patient's need for additional therapy; a score of 4 out of a possible 10 points while on adequate NSAID therapy is usually considered a good candidate for biologic therapy.
The Bath Ankylosing Spondylitis Functional Index (BASFI) is a functional index which can accurately assess a patient's functional impairment due to the disease, as well as improvements following therapy. (See: "Diagnostic Tools", below)
[5]
The BASFI is not usually used as a diagnostic tool, but as a tool to establish a patient's current baseline and subsequent response to therapy.
Pathophysiology
AS is a systemic
rheumatic disease, and is one of the
seronegative spondyloarthropathies. About 90% of the patients express the
HLA-B27 genotype.
Tumor necrosis factor-alpha (TNF α) and
IL-1 are also implicated in ankylosing spondylitis. Although specific autoantibodies cannot be detected, its response to immunosuppresive medication has prompted its classification as an autoimmune disease.
Hypotheses on its pathogenesis include a cross-reaction with antigens of the
Klebsiella bacterial strain (Tiwana et al. 2001).
[6]
Particular authorities argue that elimination of the prime nutrients of Klebsiella (starches) would decrease antigenemia and improve the musculoskeletal symptoms. On the other hand, Khan (2002) argues that the evidence for a correlation between Klebsiella and AS is circumstantial so far, and that the efficacy of low-starch diets has not yet been scientifically evaluated.
[7]
Similarly, Toivanen (1999) found no support for the role of klebsiella in the etiology of primary AS.
[8]
Epidemiology
The sex ratio is 3:1 for men:women. In the
USA, the
prevalence is 0.25%, but as it is a chronic condition the
incidence (number of new cases) is fairly low.
History
Leonard Trask the Wonderful Invalid.
AS was probably first recognized as a disease which was different from
Rheumatoid Arthritis by
Galen as early as the second century AD
[9]; however, skeletal evidence of the disease (ossification of joints and entheses primarily of the
axial skeleton, known as "bamboo spine") were first discovered in an archaeological dig that unearthed the skeletal remains of a 5000 year–old Egyptian mummy with evidence of "bamboo spine".
[10]
The anatomist and surgeon
Realdo Colombo described what could have been the disease in 1559,
[11]
and the first account of pathologic changes to the
skeleton possibly associated with AS was published in 1691 by
Bernard Connor.
[12]
In 1818,
Benjamin Brodie became the first physician to document that
iritis accompanied what is believed to have been a patient with active AS.
[13]
In 1858,
David Tucker published a small booklet which clearly described a patient by the name of
Leonard Trask who suffered from severe spinal deformity subsequent to AS.
[14][15]
In 1833 Trask fell from a horse, exacerbating the condition and resulting in severe deformity. Tucker reported that
evidence of inflammatory disease characteristics of AS, and the hallmark of deforming injury in AS. This account became the first documented case of AS in the United States.
It was not until the late nineteenth century (1893-1898), however, when the
neurophysiologist Vladimir Bekhterev of Russia in 1893,
[16]
Adolph Strümpell of Germany in 1897,
[17]
and
Pierre Marie of France in 1898,
[18]
were the first to give adequate descriptions which permitted an accurate diagnosis of AS prior to severe spinal deformity. For this reason, AS is also known as Bechterew Disease or Marie–Strümpel Disease.
Prognosis
AS can range from mild to progressively debilitating, and from medically controlled to refractive.
Unattended cases of AS normally lead to knee pain, and may be accompanied by
dactylitis or
enthesitis, which may result in a misdiagnosis of normal rheumatism. In a long-term undiagnosed period,
osteopenia or
osteoporosis of AP spine may occur, causing eventual compression fractures and a back "hump" if untreated. Typical signs of progressed AS are the visible formation of
syndesmophytes on X-rays, an abnormal bone outgrowth similar to
osteophytes, affecting the spine. Due to the fusion of the vertrbrae
paresthesia is a complication due to the inflammation of the tissue surrounding nerves.
Organs affected by AS, other than the axial
spine and other joints, are commonly the
heart,
lungs,
colon, and
kidney. Other complications are
Aortic regurgitation,
Achilles tendinitis,
AV node block and
Amyloidosis. Due to lung fibrosis,
chest X-rays may show apical fibrosis while
pulmonary function testing may reveal a restrictive lung defect.
Very rare complications involve
neurologic conditions such as the
cauda equina syndrome [19].
Therapy
No cure is known for AS, although treatments and medications are available to reduce symptoms and pain.
Physical therapy and exercise, along with medication, are at the heart of therapy for ankylosing spondylitis. Physiotherapy and physical exercises are clearly preceded by medical treatment in order to reduce the inflammation and pain, and commonly followed by a physician. This way the movements will help in diminishing pain and stiffness, while exercises in an active inflammatory state will just make the pain worse.
Patients who are able to do so, lie flat on their face or back on the floor for a prescribed cumulative period of time each week, to prevent the chronic stooping which may otherwise result.
[20]
Medication
There are three major types of medications used to treat ankylosing spondylitis.
★
NSAIDs such as
aspirin,
ibuprofen,
indometacin,
naproxen and
COX-2 inhibitors, which reduce inflammation and pain. These drugs tend to have a personal response to the pain and inflammation, although commonly used anti-inflammatory drugs like
nimesulide are less effective than others;
★
DMARDs such as
cyclosporin,
methotrexate,
sulfasalazine, and
corticosteroids, used to reduce the immune system response through
immunosuppression;
★
TNFα blockers (
antagonists) such as
etanercept,
infliximab and
adalimumab (also known as biologics), are indicated for the treatment of and are effective
immunosuppressants in AS as in other autoimmune diseases;
TNFα blockers have been shown to be the best promising treatment, slowing the progress of AS in the majority of clinical cases. They have also been shown to be highly effective in treating not only the arthritis of the joints but the spinal arthritis associated with AS. A drawback is the fact that these drugs increase the risk of infections. For this reason, the protocol for any of the TNF-α blockers include a test for
tubercolosis (like
Mantoux or
Heaf) before starting treatment. In case of recurrent infections, like even recurrent
sore throats, the therapy may be suspended due to the involved
immunosuppression.
Surgery
In severe cases of AS,
surgery can be an option in the form of joint replacements, particularly in the knees and hips. Surgical correction is also possible for those with severe flexion deformities (severe downward curvature) of the spine, particularly in the neck, although this procedure is considered risky.
In addition, AS can have some manifestations which make anaesthesia more complex.
Changes in the upper airway can lead to difficulties in intubating the airway, spinal and epidural anaesthesia may be difficult due to calicification of ligaments, and a small number have aortoc insufficiency. The stiffness of the thoracic ribs results in ventilation being mainly diaphragm-driven, so there may be a decrease in pulmonary function.
Physical therapy
All physical therapies must be approved in advance by a rheumatologist, since movements that normally have great benefits on one's health, may harm a patient with AS: massages and physical manipulations should be practiced by therapists familiar with this disease.
★
Physical therapy has been shown to be of great benefit to AS patients;
★
Swimming is one of the preferred exercises since it involves all muscles and joints in a low gravity environment;
★ Slow movements exercises like
stretching,
yoga,
tai chi;
★ Any physical movement like,
jogging,
Pilates method, etc.
Alternative medicine
Although the effectiveness of alternative medicines has not been proved by any
clinical trial, some patients find some relief in adding these alternative treatments to the medicaments and physical exercises, like a
starch free
diet[21] (also known as the No Starch Diet or NSD
[22] ). Some patients may find relief under acupuncture treatments. This is administered at various points directly on the spine where the pain is located, although efficacy is uncertain. Herbal medicine, may also relieve pain, partly due to placebo effect.
[23]
Famous patients
Well known sufferers of AS include:
★
Mötley Crüe guitarist
Mick Mars;
★ Former England
cricket captain
Mike Atherton;
★ Former Australian cricketer
Michael Slater;
★ British comedian
Lee Hurst;
★ Canadian radio personality Mike Stafford;
★ Norwegian Prime Minister
Jens Stoltenberg;
★ World Chess Champion
Vladimir Kramnik;
★ Former author and "Saturday Review" editor
Norman Cousins;
[24]
★ Scottish former snooker player
Chris Small;
★ Former US Major League baseball player
Rico Brogna;
★
Ed Sullivan, the Ed Sullivan Show, US;
★ Taiwanese musician
Jay Chou;
★ Czech writer
Karel Čapek[25].
Additional images
See also
★
NASC, North American AS federation
★
NIAMS, the National Institute of Arthritis and Musculoskeletal and Skin Diseases
References
1. Ankylosing spondylitis: clinical course in women and men., Jiménez-Balderas FJ, Mintz G., , , J Rheumatol, 1993
2. http://www.spondylitis.org/patient_resources/women.aspx
3. Association between measures of spinal mobility and low back pain. An analysis of new attenders in primary care., Thomas E, Silman AJ, Papageorgiou AC, Macfarlane GJ, Croft PR., , , Spine, 1998
4. A new approach to defining disease status in ankylosing spondylitis: the Bath Ankylosing Spondylitis Disease Activity Index., Garrett S, Jenkinson T, Kennedy L, Whitelock H, Gaisford P, Calin A, , , J Rheumatol, 1994
5. A new approach to defining functional ability in ankylosing spondylitis: the development of the Bath Ankylosing Spondylitis Functional Index., Calin A, Garrett S, Whitelock H, Kennedy L, O'Hea J, Mallorie P, Jenkinson T, , , J Rheumatol, 1994
6. Correlation between the immune responses to collagens type I, III, IV and V and Klebsiella pneumoniae in patients with Crohn's disease and ankylosing spondylitis., Tiwana H, Natt R, Benitez-Brito R, Shah S, Wilson C, Bridger S, Harbord M, Sarner M, Ebringer A, , , Rheumatology (Oxford), 2001
7. Ankylosing spondylitis: The facts, Khan MA., , , Oxford University Press, 2002, ISBN 0-19-263282-5
8. Somatic serogroups, capsular types, and species of fecal Klebsiella in patients with ankylosing spondylitis., Toivanen P, Hansen D, Mestre F, Lehtonen L, Vaahtovuo J, Vehma M, Möttönen T, Saario R, Luukkainen R, Nissilä M, , , J Clin Microbiol, 1999
9. Did Galen describe rheumatoid arthritis?, Dieppe P, , , Annals of the Rheumatic Diseases, 1988
10. Ankylosing spondilitis., Calin A., , , Clinics in Rheumatic Diseases, 1985
11. Benoist M. - Historical Perspective, Pierre Marie, , , Spine, 1995
12. ?, Blumberg BS, , , Arch Rheum, 1958
13. Did Bechterew describe the disease which is named after him? A question raised due to the centennial of his primary report., Leden I, , , Scand J Rheumatol, 1994
14. Life and sufferings of Leonard Trask
15. Life and sufferings of Leonard Trask
16. Steifigkeit der Wirbelsaule und ihre Verkrummung als besondere Erkrankungsform., Bechterew W., , , Neurol Centralbl, 1893
17. Bemerkung uber die chronische ankylosirende Entzundung der Wirbelsaule und der Huftgelenke., Strumpell A., , , Dtsch Z Nervenheilkd, 1897
18. Sur la spondylose rhizomelique., Marie P., , , Rev Med, 1898
19. Cauda Equina Syndrome in AS (The CES-AS Syndrome): Meta-analysis of outcomes after medical and surgical treatments., Nicholas U. Ahn, Uri M. Ahn, Elizabeth S. Garrett et al., , , J of Spinal Disorders, 2001
20. Remicade.com
21. The use of a low starch diet in the treatment of patients suffering from ankylosing spondylitis., Ebringer A, Wilson C, , , Clin Rheumatol, 1996
22. http://www.kickas.org/ubbthreads/postlist.php?Board=starch 'No Starch Diet'
23. http://www.nlm.nih.gov/medlineplus/news/fullstory_51426.html National Institute of Health
24. Cousins wrote a bestselling book about his illness,''Anatomy of an Illness''. But since he was "cured" of the disease, which is incurable, he may have been misdiagnosed.
25. Karel Capek-Czech writer, sufferer from ankylosing spondylitis., Trnavsky K., Sabova L., , , Clin Rheumatol., 1992
External links
National organizations
★
The Ankylosing Spondylitis group (Australia)
★
Vlaamse Vereniging voor Bechterew-Patiënten vzw (Belgium)
★
Associazione Italiana per la Lotta alla Spondilite Anchilosante (Italy)
★
National Ankylosing Spondylitis Society (UK)
★
Spondylitis Association of America (USA)
Diagnostic tools
★
Bath Ankylosing Spondylitis Disease Activity Index Calculator (BASDAI)
★
Bath Ankylosing Spondylitis Functional Index Calculator (BASFI)
Support groups
★
KickAS (online community; support and information)
Current research
★
Ankylosing Spondylitis Research (Recent primary literature)
★
Ankylosing Spondylitis Information Matrix (Muhammad A. Khan, MD)
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