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Ankylosing spondylitis

Ankylosing spondylitis
Classification & external
resources

ICD-10

M08.1, M45.

ICD-9

720.0

OMIM

106300

DiseasesDB

728

MedlinePlus 000420
eMedicine

radio/41

MeSH

D013167

Ankylosing spondylitis (AS; also known as Bechterew's disease; Bechterew


syndrome; Marie Strmpell disease / Marie Struempell disease / Spondyloarthritis)
is a chronic, painful, progressive 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.

Contents
[hide]

1 Signs and symptoms


2 Diagnosis
3 Pathophysiology
4 Epidemiology
5 History
6 Prognosis
7 Therapy
o 7.1 Medication
o 7.2 Surgery
o 7.3 Physical therapy
o 7.4 Alternative medicine
8 Famous patients
9 See also
10 Footnotes
11 External links
o 11.1 National organizations
o 11.2 Diagnostic tools
o 11.3 Support groups
o

11.4 Current research

[edit] Signs and symptoms


The typical patient is a young man, or sometimes a woman, of 15 to 30 years of age with
chronic pain and stiffness in the lower part of the spine. 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.
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.

[edit] 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 test is a useful clinical
measure of flexion of the lumber spine performed during examination.[citation needed]
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) [1] 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) [2] 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.

[edit] 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 ) 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).[3] 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.[4] Similarly, Toivanen (1999)
found no support for the role of klebsiella in the etiology of primary AS.[5]

[edit] 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.

[edit] 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[citation needed]; 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 yearold Egyptian mummy with evidence of "bamboo
spine".[6]
The anatomist and surgeon Realdo Colombo described what could have been the disease
in 1559,[7] and the first account of pathologic changes to the skeleton possibly associated
with AS was published in 1691 by Bernard Connor.[8] In 1818, Benjamin Brodie became
the first physician to document that iritis accompanied what is believed to have been a
patient with active AS.[9] 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.[10][11] In 1833 Trask fell from a horse, exacerbating the
condition and resulting in severe deformity. Tucker reported that

It was not until he [Trask] had exercised for some time that he could perform
any labor [..., and that] his neck and back have continued to curve drawing his
head downward on his breast.

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,[12] Adolph Strmpell of
Germany in 1897,[13] and Pierre Marie of France in 1898,[14] 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 MarieStrmpel
Disease.

[edit] 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[citation needed],
Achilles tendonitis, AV node block[citation needed] and Amyloidosis[citation needed]. Due to lung
fibrosis[citation needed], 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 [15].

[edit] 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.[16]

[edit] 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 antiinflammatory 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;
TNF 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;

TNF 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- 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.

[edit] 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.

[edit] 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.

[edit] 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[17] Patients can find relief
under acupuncture treatments and herbal medicine. It can be treated by the use of various
points directly on the spine where the pain is located. Herbal medicine, when correctly
formulated by a doctor, may also treat by relieving pain, reducing inflammation, and
providing natural supplements to strengthen the body's bones, muscles, and tendons.[citation
needed]

[edit] Famous patients


Well known sufferers of AS include:

Mtley Cre 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;[18]
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 apek[19].

[edit] See also

NASC, the AS patients' federation


NIAMS, the National Institute of Arthritis and Musculoskeletal and Skin Diseases

[edit] Footnotes
1. ^ Garrett S, Jenkinson T, Kennedy L, Whitelock H, Gaisford P, Calin A (1994). "A new
approach to defining disease status in ankylosing spondylitis: the Bath Ankylosing
Spondylitis Disease Activity Index.". J Rheumatol 21 (12): 2286-91. PMID 7699630.
2. ^ Calin A, Garrett S, Whitelock H, Kennedy L, O'Hea J, Mallorie P, Jenkinson T (1994).
"A new approach to defining functional ability in ankylosing spondylitis: the
development of the Bath Ankylosing Spondylitis Functional Index.". J Rheumatol 21
(12): 2281-5. PMID 7699629.
3. ^ Tiwana H, Natt R, Benitez-Brito R, Shah S, Wilson C, Bridger S, Harbord M, Sarner
M, Ebringer A (2001). "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.". Rheumatology (Oxford) 40 (1): 15-23. PMID 11157137.
4. ^ Khan MA. (2002). Ankylosing spondylitis: The facts. Oxford University Press. ISBN 019-263282-5.
5. ^ Toivanen P, Hansen D, Mestre F, Lehtonen L, Vaahtovuo J, Vehma M, Mttnen T,
Saario R, Luukkainen R, Nissil M (1999). "Somatic serogroups, capsular types, and
species of fecal Klebsiella in patients with ankylosing spondylitis.". J Clin Microbiol 37
(9): 2808-12. PMID 10449457.
6. ^ Calin A. (1985). "Ankylosing spondilitis.". Clinics in Rheumatic Diseases 11: 4160.
7. ^ Pierre Marie (1995). "Benoist M. - Historical Perspective". Spine 20: 849852.
8. ^ Blumberg BS (1958). "?". Arch Rheum 1: 553.
9. ^ Leden I (1994). "Did Bechterew describe the disease which is named after him? A
question raised due to the centennial of his primary report.". Scand J Rheumatol 23 (1):
42-5. PMID 8108667.
10. ^ Life and sufferings of Leonard Trask (PDF for registered members)). Ankylosing
Spondylitis Information Matrix.
11. ^ Life and sufferings of Leonard Trask.
12. ^ Bechterew W. (1893). "Steifigkeit der Wirbelsaule und ihre Verkrummung als
besondere Erkrankungsform.". Neurol Centralbl 12: 426434.
13. ^ Strumpell A. (1897). "Bemerkung uber die chronische ankylosirende Entzundung der
Wirbelsaule und der Huftgelenke.". Dtsch Z Nervenheilkd 11: 338342.
14. ^ Marie P. (1898). "Sur la spondylose rhizomelique.". Rev Med 18: 285315.
15. ^ Nicholas U. Ahn, Uri M. Ahn, Elizabeth S. Garrett et al. (2001). "Cauda Equina
Syndrome in AS (The CES-AS Syndrome): Meta-analysis of outcomes after medical and
surgical treatments.". J of Spinal Disorders 14 (5): 427-433. PMID 11586143.
16. ^ Remicade.com. Living with Ankylosing Spondylitis. Retrieved on 2007-01-11.
17. ^ Ebringer A, Wilson C (Jan 15 1996). "The use of a low starch diet in the treatment of
patients suffering from ankylosing spondylitis.". Clin Rheumatol 15 Suppl 1: 62-66.
PMID 8835506.
18. ^ 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.
19. ^ Trnavsky K., Sabova L. (1992). "Karel Capek-Czech writer, sufferer from ankylosing
spondylitis.". Clin Rheumatol. 11 (3): 337-40. PMID 1458780.

[edit] External links

[edit] National organizations

The Ankylosing Spondylitis group (Australia)


Vlaamse Vereniging voor Bechterew-Patinten vzw (Belgium)
Associazione Italiana per la Lotta alla Spondilite Anchilsante (Italy)
National Ankylosing Spondylitis Society (UK)
Spondylitis Association of America (USA)

[edit] Diagnostic tools

Bath Ankylosing Spondylitis Disease Activity Index Calculator (BASDAI)


Bath Ankylosing Spondylitis Functional Index Calculator (BASFI)

[edit] Support groups

KickAS.org (online community; support and information)

[edit] Current research

Ankylosing Spondylitis Research Recent primary literature


Ankylosing Spondylitis Information Matrix Muhammad A. Khan, MD

Retrieved from "http://en.wikipedia.org/wiki/Ankylosing_spondylitis"


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