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20-September-2008 10:21:21 - Ankylosing spondylitis Ankylosing spondylitis Classification and 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 Strümpell disease and 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 Contents 1 Signs and symptoms 2 Diagnosis 3 Pathophysiology 4 Epidemiology 5 History 6 Prognosis 7 Therapy 7.1 Medication 7.2 Surgery 7.3 Physical therapy 7.4 Starch-free diet 8 Notable patients 9 Additional images 10 See also 11 References 12 External links 12.1 Diagnostic tools 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 women by a ratio in excess of 2:1.2 In 40% of cases, ankylosing spondylitis is associated with iridocyclitis causing eye pain and photophobia increased sensitivity to light. Other common symptoms are recurring mouth ulcers aphthae and fatigue. Pain can fluctuate from one side to the other. Typical prodromes early symptoms may occur at a very young age e.g., 3 years old, when 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 Lateral Lumbar Spine X-Ray demonstrating Ankylosing spondylitis Lateral Lumbar Spine X-Ray demonstrating Ankylosing spondylitis The ankylosis process. 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. Options 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 a higher risk than the general population 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 that have been diagnosed 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 Merranean countries. In early onset disease HLA-B7/B2705 heterozygotes exhibited the highest risk for disease.4 In 2007, a collaborative effort by an international team of researchers in the U.K., Australia and the United States led to the discovery of two genes, ARTS1 and IL23R, that also cause AS. The findings were published in the November 2007 ion of Nature Genetics, a journal that emphasizes research on the genetic basis for common and complex diseases.5 Together with HLA-B27, these two genes account for roughly 70 percent of the overall incidence of the disease. 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, below6 It can be easily calculated and accurately assesses a patient's need for additional therapy; a patient with 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, below7 The BASFI is not usually used as a diagnostic tool but rather 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. 2001.8 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.9 Similarly, Toivanen 1999 found no support for the role of klebsiella in the etiology of primary AS.10 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. Many rheumatologists believe the number of women with AS is underdiagnosed, as women tend to manifest symptoms in their lower spinal area lumbar, sacrum, hips, pelvis instead of their shoulders/upper chest/neck where the first symptoms appear in male patients11. History Leonard Trask, the Wonderful Invalid. Leonard Trask, the Wonderful Invalid. It has been suggest that AS was first recognized as a disease which was different from rheumatoid arthritis by Galen as early as the second century A.D.12; however, skeletal evidence of the disease ossification of joints and entheses primarily of the axial skeleton, known as bamboo spine was first discovered in an archaeological dig that unearthed the skeletal remains of a 5000-year-old Egyptian mummy with evidence of bamboo spine.13 The anatomist and surgeon Realdo Colombo described what could have been the disease in 1559,14 and the first account of pathologic changes to the skeleton possibly associated with AS was published in 1691 by Bernard Connor.15 In 1818, Benjamin Brodie became the first physician to document that a patient believed to have active AS had accompanying iritis.16 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.1718 In 1833 Trask fell from a horse, exacerbating the condition and resulting in severe deformity. Tucker reported: 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. This account became the first documented case of AS in the United States, since its indisputable description of inflammatory disease characteristics of AS, and the hallmark of deforming injury in AS. It was not until the late nineteenth century 1893-1898, however, when the neurophysiologist Vladimir Bekhterev of Russia in 1893,19 Adolph Strümpell of Germany in 1897,20 and Pierre Marie of France in 1898,21 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 the 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 and abnormal bone outgrowths similar to osteophytes affecting the spine. The fusion of the vertebrae paresthesia is a complication due to the inflammation of the tissue surrounding nerves. Organs commonly affected by AS, other than the axial spine and other joints, are the heart, lungs, colon, and kidney. Other complications are aortic regurgitation, Achilles tendinitis, AV node block and amyloidosis.22 Owing 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.2322 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 to be preceded by medical treatment in order to reduce the inflammation and pain and are commonly followed by a physician. This way the movements will help in diminishing pain and stiffness, while exercise in an active inflammatory state will just make the pain worse. Medical professionals and experts in AS have widely speculated that maintaining good posture can reduce the likelihood of a fused or curved spine which occurs in a significant percentage of diagnosed persons. 2425 Medication There are three major types of medications used to treat ankylosing spondylitis. Anti-inflammatory drugs, which include NSAIDs such as aspirin, ibuprofen, indometacin, naproxen and COX-2 inhibitors, which reduce inflammation, and consequently 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. Opioid analgesics have also been proven by clinical evidence to be very effective in alleviating the type of chronic pain commonly experienced by those suffering from AS, especially in low dose time-release formulations. While NSAIDs should generally be tried first, the use of opioid analgesics either apart from or in concert with NSAIDs should not be summarily dismissed for fear of addiction, as studies have shown that patients who properly take opioid analgesics for pain rarely suffer from addiction as a result of using such opioid therapy for pain relief purposes. 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 most 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 also 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 tuberculosis like Mantoux or Heaf before starting treatment. In case of recurrent infections, even recurrent sore throats, the therapy may be suspended because of 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 owing to calicification of ligaments, and a small number have aortic regurgitation. 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 to one's health may harm a patient with AS; massages and physical manipulations should be practiced by therapists familiar with this disease. Some of the therapies that have been shown to benefit AS patients include: Physical therapy/Physiotherapy, shown to be of great benefit to AS patients; Swimming, one of the preferred exercises since it involves all muscles and joints in a low gravity environment; Slow movement muscle extending exercises like stretching, yoga, tai chi, Pilates method, etc. Moderate-to-high impact exercises like jogging are generally not recommended or recommended with restrictions due to the jarring of affected vertabrae that can worsen pain and stiffness in some patients. Starch-free diet The majority of patients with AS exhibit the HLA-B27 antigen and high levels of immunoglobulin A IgA in the blood. The HLA-B27 antigen is also expressed by Klebsiella bacteria, which is found in high levels in the feces of AS patients. A theory suggests that the presence of the bacteria may be a trigger of the disease, and reducing the amount of starch in the diet which the bacteria require to grow may be of benefit to AS patients. A test of this diet resulted in reduced symptoms and inflammation in patients with AS as well as IgA levels in individuals with and without AS.26 Notable patients Taiwanese musician Jay Chou2728 Mötley Crüe's guitarist Mick Mars29 Former England cricket captain Mike Atherton30 British comedian Lee Hurst30 World Chess Champion Vladimir Kramnik31 Czech writer Karel ÄŒapek32; British golfer Ian Woosnam30 Additional images Morbus Bechterew MRI of SI joints See also NASC, North American AS federation NIAMS, the National Institute of Arthritis and Musculoskeletal and Skin Diseases SAA, Spondylitis Association of America AF, Arthritis Foundation References ^ Jiménez-Balderas FJ, Mintz G. 1993. Ankylosing spondylitis: clinical course in women and men. J Rheumatol 20 12: 2069-72. PMID 7516975. ^ Patient Resources - Women's Health ^ Thomas E, Silman AJ, Papageorgiou AC, Macfarlane GJ, Croft PR. 1998. Association between measures of spinal mobility and low back pain. An analysis of new attenders in primary care. Spine 23 2: 343-7. doi:10.1097/00007632-199802010-00011. PMID 9507623. ^ Harjacek M, Margetić T, Kerhin-Brkljacić V, Martinez N, Grubić Z 2008. HLA-B27/HLA-B07 in combination with D6S273-134 allele is associated with increased susceptibility to juvenile spondyloarthropathies. Clin. Exp. Rheumatol. 26 3: 498-504. PMID 18578977. ^ Spondylitis Association of America October 21, 2007, Spondylitis Association of America Participates in Study That Uncovers Two Genes Related to Disabling Form of Arthritis ^ 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. ^ 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. ^ 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. doi:10.1093/rheumatology/40.1.15. PMID 11157137. ^ Khan MA. 2002. Ankylosing spondylitis: The facts. Oxford University Press. ISBN 0-19-263282-5. ^ Toivanen P, Hansen D, Mestre F, Lehtonen L, Vaahtovuo J, Vehma M, Möttönen T, Saario R, Luukkainen R, Nissilä 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. ^ Patient Resources for Women, Spondylitis Association of America; retrieved May 26, 2008. ^ Dieppe P 1988. Did Galen describe rheumatoid arthritis?. Annals of the Rheumatic Diseases 47: 84-87. ^ Calin A. 1985. Ankylosing spondilitis. Clinics in Rheumatic Diseases 11: 41-60. ^ Pierre Marie 1995. Benoist M. - Historical Perspective. Spine 20: 849-852. ^ Blumberg BS 1958. ?. Arch Rheum 1: 553. doi:10.1002/art.1780010609. ^ 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. doi:10.3109/03009749409102134. PMID 8108667. ^ Life and sufferings of Leonard Trask PDF for registered members. Ankylosing Spondylitis Information Matrix. ^ Life and sufferings of Leonard Trask. ^ Bechterew W. 1893. Steifigkeit der Wirbelsaule und ihre Verkrummung als besondere Erkrankungsform. Neurol Centralbl 12: 426-434. ^ Strumpell A. 1897. Bemerkung uber die chronische ankylosirende Entzundung der Wirbelsaule und der Huftgelenke. Dtsch Z Nervenheilkd 11: 338-342. doi:10.1007/BF01674127. ^ Marie P. 1898. Sur la spondylose rhizomelique. Rev Med 18: 285-315. ^ a b Alpert, Joseph S. 2006. The AHA Clinical Cardiac Consult. Lippincott Williams Wilkins. ISBN 0781764904. ^ 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. doi:10.1097/00002517-200110000-00009. PMID 11586143. ^ www.arthritis.org. Anklylosing Spondylitis Understanding its caused, Diagnosis And Treatment. ^ Remicade.com. Living with Ankylosing Spondylitis. Retrieved on 2007-12-30. ^ 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. ^ Chinese Jay Chou discusses the wonder of life in illness 二度造访心情电视 周æ?°ä¼¦è°ˆç—…痛中的精彩人生 News article, sina.com.cn 2003-04-28. Retrieved on 2007-06-02. ^ Chinese Jacky Wu: Jay Chou takes 12 painkillers å?´å®—宪:周æ?°ä¼¦ä¸€åº¦æœ?12颗止痛è?¯ News article, zaobao.com 2006-02-25. Retrieved on 2007-06-02. ^ Rashbaum, A 2004-10-06. Motley Crue Guitarist Undergoes Surgery. mtv.com. Retrieved on 2008-08-07. ^ a b c Probe into arthritis pain, BBC News 2002-08-24. Retrieved on 2008-08-07. ^ ChessBase.com - Chess News - Kramnik drops out of Wijk Super-Tournament ^ Trnavsky K., Sabova L. 1992. Karel Capek-Czech writer, sufferer from ankylosing spondylitis. Clin Rheumatol. 11 3: 337-40. doi:10.1007/BF02207189. PMID 1458780. External links Ankylosing spondylitis at the Open Directory Project Diagnostic tools Bath Ankylosing Spondylitis Disease Activity Index Calculator BASDAI Bath Ankylosing Spondylitis Functional Index Calculator BASFI v d e Diseases of the musculoskeletal system and connective tissue M, 710-739 Arthropathies Arthritis Septic arthritis - Reactive arthritis - Rheumatoid arthritis - Psoriatic arthritis - Felty syndrome - Juvenile idiopathic arthritis - Still's disease - crystal Gout, Chondrocalcinosis - Osteoarthritis Heberden's node, Bouchard's nodes - Monoarthritis/Polyarthritis Specific joints shoulder Winged scapula - elbow Cubitus valgus, Cubitus varus - hand Wrist drop, Boutonniere deformity, Swan neck deformity hip Protrusio acetabuli, Coxa valga, Coxa vara - leg Foot drop, Flat feet, Club foot, Unequal leg length - patella Luxating patella, Chondromalacia patellae - foot Bunion/hallux valgus, Hallux varus, Hallux rigidus, Hammer toe general terms Valgus deformity, Varus deformity Other Hemarthrosis - Arthralgia - Osteophyte - Hypermobility Systemic CT disorders vasculitis: Arteritis Polyarteritis nodosa, Takayasu's arteritis, Temporal arteritis - arterioles/capillaries Wegener's granulomatosis, Kawasaki disease, Churg-Strauss syndrome, Microscopic polyangiitis hypersensitivity/autoimmune: Hypersensitivity vasculitis - Goodpasture's syndrome - Systemic lupus erythematosus Drug-induced - Dermatomyositis Juvenile dermatomyositis - Polymyositis - Scleroderma - Sjögren's syndrome - Behçet's disease - Polymyalgia rheumatica - Eosinophilic fasciitis Dorsopathies spinal curvature Kyphosis, Lordosis, Scoliosis - Scheuermann's disease - Spondylolysis - Torticollis - Spondylolisthesis Spondylopathies Ankylosing spondylitis, Spondylosis, Spinal stenosis - Schmorl's nodes - Degenerative disc disease - Coccydynia - Back pain Radiculopathy, Neck pain, Sciatica, Low back pain Soft tissue disorders Muscle Myositis Pyomyositis - Myositis ossificans Fibrodysplasia ossificans progressiva Synovium and tendon Synovitis/Tenosynovitis Calcific tendinitis, Stenosing tenosynovitis, Trigger finger, DeQuervain's syndrome - Irritable hip - Ganglion cyst Bursa Bursitis Olecranon, Prepatellar, Trochanteric - Baker's cyst Fascia/fibroblastic Fasciitis Plantar fasciitis, Nodular fasciitis, Necrotizing fasciitis - Dupuytren's contracture - Fibromatosis Shoulder lesions Adhesive capsulitis - Rotator cuff tear - Subacromial bursitis Enthesis Enthesopathies Iliotibial band syndrome, Achilles tendinitis, Patellar tendinitis, Golfer's elbow, Tennis elbow, Metatarsalgia, Bone spur, Tendinitis Other, NEC Muscle weakness - Rheumatism - Myalgia - Neuralgia - Neuritis - Panniculitis - Fibromyalgia Osteopathies disorders of bone density and structure: Osteoporosis - Osteomalacia - continuity of bone Pseudarthrosis, Stress fracture - Monostotic fibrous dysplasia - Skeletal fluorosis - Aneurysmal bone cyst - Hyperostosis - Osteosclerosis Osteomyelitis - Avascular necrosis - Paget's disease of bone - Algoneurodystrophy - Osteolysis - Infantile cortical hyperostosis Chondropathies Juvenile osteochondrosis Legg-Calvé-Perthes syndrome, Osgood-Schlatter disease, Köhler disease, Sever's disease - Osteochondritis - Tietze's syndrome - Relapsing polychondritis See also congenital Retrieved from http://en..org/wiki/Ankylosing_spondylitis Categories: Arthritis | Autoimmune diseases Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages ÄŒesky Deutsch Español Français Italiano עברית Nederlands ‪Norsk bokmÃ¥l‬ Polski РуÑ?Ñ?кий СрпÑ?ки / Srpski Suomi Svenska Türkçe 中文 This page was last modified on 13 August 2008, at 16:33

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