Ankylosing Spondylitis (Bechterew Disease)

0510 Spondylitis ankylosans (morbus bechterew) anagoria[1]

Ankylosing spondylitis (rheumatoid spondylitis, Marie-Strümpell disease, von Bechterew disease) is a chronic, inflammatory disorder primarily involving the sacroiliac joints, the axial skeleton, peripheral joints and entheses. The etiology of Ankylosing spondylitis is unknown, though genetic factors seem to be involved (HLA-B27).

Ankylosing Spondylitis stems from the following process: auto-immune.

Presentation

A typical case of ankylosing spondylitis presents with a gradual onset of lower lumbar pain which is of an inflammatory type. Fatigue may present along with the inflammation [5].

The first symptom of pain may arise from sacroilititis, with pain in the buttocks radiating down the back of both the legs. The pain can lead to stiffness and rigidity. The symptoms of pain and stiffness are worse in the morning or after long periods of inactivity. Heat and warmth generally ameliorate the condition. Once fusion occurs, pain reduces but flexibility and mobility of the spine are severely affected. This occurs due to overgrowth of bones resulting in bony fusion. Chronic spondylitis leads to loss of lumbar lordosis and increased kyphosis which may cause difficulty in breathing which is a serious complication of this disorder.

People with this disorder can also have arthritis of other joints, showing the typical signs of inflammation. If peripheral polyarthritis occurs, usually joints of lower limb are affected. Plantar faciitis may cause heel pain due to inflammation of the tendon or even pain at the back of the ankle leading to Achilles tendon [5].

Other manifestations of ankylosing spondylitis also include iritis and uveitis, which is a serious complication and can severely damage the eye. Kidney and heart can also be affected.

urogenital
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  • Eyes
    Blurred Vision
    • See an eye specialist (ophthalmologist) immediately if you develop a painful red eye, severe light sensitivity or blurred vision.[mayoclinic.org]
    • Symptoms include pain , lacrimation, photophobia, and blurred vision.[emedicine.medscape.com]
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  • cardiovascular
    Chest Pain
    • Common sites for enthesitis are: at the top of the shin bone behind the heel (Achilles tendon) under the heel where the ribs join the breast bone If your ribs are affected, you may experience chest pain and find it difficult to expand your chest when[nhs.uk]
    • Chest wall cartilage inflammation can cause chest pain and tenderness.[emedicinehealth.com]
    • You may experience chest pain and stiffness with ankylosing spondylitis.[uofmhealth.org]
    • Costochondritis, with chest pain is common.[arthritis.co.za]
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  • musculoskeletal
    Arthritis
    • Synonym(s): acute epidemic leukoencephalitis ; spondylitis deformans Strümpell-Marie disease - arthritis of the spine, resembling rheumatoid arthritis, that may progress to bony ankylosis.[medical-dictionary.thefreedictionary.com]
    • Other forms of arthritis may mimic AS.[healthcentral.com]
    • Enteropathic Arthritis - Arthritis Associated with Inflammatory Bowel Disease.[arthritis.co.za]
    • […] spine , arthritis; spine or vertebra, Marie-Strümpell , arthritis; spine or vertebra, ankylosing , Marie-Strümpell; spondylitis , Marie-Strümpell , Strümpell-Marie , Von Bechterew , Bechterew , rheumatoid; arthritis, spine , rheumatoid; spondylitis ,[fpnotebook.com]
    Back Pain
    Low Back Pain
    • There is often severe low back pain, along with buttock, hip, and thigh pain on one or both sides.[depuysynthes.com]
    • At the onset of AS, there is often severe low back pain, buttock, hip, and thigh pain on one or both sides.[umm.edu]
    • The most common first symptom is chronic low back pain and stiffness of gradual (insidious) onset.[hlab27.com]
    • These symptoms may include: Intermittent low back pain, usually most severe in the morning or after a period of inactivity Stiffness and limited motion of the lumbar spine Pain and limited expansion of the chest due to involvement of the costovertebral[healthcentral.com]
    Buttock Pain
    • This inflammation can cause buttock pain that radiates into the thigh and/or diffuse lower back pain.[arthritis-health.com]
    • […] and ligaments attach to bones (entheses), mainly in your spine, but sometimes along the back of your heel The cartilage between your breastbone and ribs Your hip and shoulder joints When to see a doctor Seek medical attention if you have low back or buttock[mayoclinic.org]
    • The spinal disease starts in the sacroiliac joints (bilateral lumbosacral region) and may be felt as diffuse nonspecific buttock pain.[patient.info]
    • pain ( Box 1 ).[clevelandclinicmeded.com]
    • […] more Insidious onset Not relieved with rest Improvement with exercise Pain at night Heel pain due to Achilles tendinitis or plantar fasciitis (enthesitis) Dactylitis (sausage digit; painful digit inflammation) Uveitis Family history of AS Alternate buttock[clinicaladvisor.com]
    Neck Pain
    • Spondylitis : - disorder has a tendency to progress slowly, w/ diminished mobility of the spine and sacroiliac joints; - early diagnosis can often be made with a CT scan of the sacroiliac joints; - cervical spine in ankylosing spondylitis : - acute neck[wheelessonline.com]
    • On arrival to the emergency room he complained of neck pain, but his neurologic exam is normal.[orthobullets.com]
    • Neck pain and fatigue also are common.[mayoclinic.org]
    • Neck pain and stiffness are usually late manifestations.[clinicaladvisor.com]
    Hip Pain
    • Total hip replacement is very useful for those with hip pain and disability due to joint destruction from cartilage loss.[rheumatology.org]
    • Hip pain is quite common with ankylosing spondylitis and may be associated with pain in the groin or buttocks, as well as difficulty walking.[arthritis.about.com]
    • Symptoms of AS include: low back and hip pain and stiffness duration of symptoms longer than three months difficulty expanding the chest early morning stiffness improved by a warm shower or light exercise pain in the neck, shoulders, knees, and ankles[encyclopedia.com]
    • Hip pain – Arthritis of the hips is relatively common in AS and can cause pain in the groin or buttocks or difficulty walking.[uptodate.com]
    • Average 3.0 of 30 Ratings Question COMMENTS (12) (OBQ06.154) A 32-year-old man presents with low back and hip pain that has been gradually worsening over the past year.[orthobullets.com]
    Spine Pain
    • These symptoms may include: Intermittent low back pain, usually most severe in the morning or after a period of inactivity Stiffness and limited motion of the lumbar spine Pain and limited expansion of the chest due to involvement of the costovertebral[healthcentral.com]
    • Other symptoms depend on the disease stage, and may include: ·Stiffness and limited motion of the lumbar spine ·Pain and limited chest expansion caused by involvement of the costovertebral joints · Arthritis involving shoulders, hips and knees ·Kyphosis[healthcentral.com]
    • When AS progresses, the spine pain may disappear.[umm.edu]
    • ., The clinical anatomy and management of back pain series, Volume 2 Clincal Anatomy and Management of Thoracic Spine Pain, Butterworth Heinemann, 2000: 61-66.[physio-pedia.com]
    Shoulder Pain
    • You may also gradually experience hip and shoulder pain.[moveforwardpt.com]
    • Shoulder pain – Inflammation of the tendon and bone may cause shoulder pain and limited mobility of the affected shoulder(s).[uptodate.com]
    • Physical therapy [ edit ] Though physical therapy remedies have been scarcely documented, some therapeutic exercises are used to help manage lower back, neck, knee, and shoulder pain.[en.wikipedia.org]
    Asymmetrical Arthritis
    • Not relieved with rest Improvement with exercise Pain at night Heel pain due to Achilles tendinitis or plantar fasciitis (enthesitis) Dactylitis (sausage digit; painful digit inflammation) Uveitis Family history of AS Alternate buttock pain Psoriasis Asymmetric[clinicaladvisor.com]
    • The hip joints are most common, but one can also see an asymmetric arthritis, which is more common in the lower extremities.[clevelandclinicmeded.com]
    Joint Swelling
    • For joint swelling that is localized (not widespread), injections, or shots, of corticosteroid medications into joints or tendon sheaths (the membrane around a tendon) can be effective quickly.[rheumatology.org]
    Arthralgia
    • Adverse events were frequent, consisting mainly of transient arthralgias or myalgias after the first intravenous infusion (in 68.3% and 46.5% of patients in the 60- and 10-mg groups, respectively).[arthritis-research.biomedcentral.com]
    Sacroiliac Pain
    • In some cases, a glucocorticoid injection into the sacroiliac joint may help provide relief in patients who have sacroiliac pain that has not responded to other therapies.[uptodate.com]
    Loss of Lumbar Lordosis
    • In the advanced stages, patients develop loss of lumbar lordosis, buttock atrophy, and an exaggerated thoracic kyphosis with a stooped forward neck sometimes referred to as a 'question mark posture'.[patient.info]
    Spine Stiffness
    • X-rays usually, but not always, show a wearing away (erosion) of the joint between the spine and the hip bone (sacroiliac joint) and the formation of bony bridges between the vertebrae, making the spine stiff (sacroiliitis).[merckmanuals.com]
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  • respiratoric
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  • neurologic
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  • Entire body system
    Fatigue
    • Fatigue Fatigue is a common symptom of untreated AS.[nhs.uk]
    • Periodic rest periods with avoidance of fatigue.[physio-pedia.com]
    • Early symptoms of ankylosing spondylitis (AS) often include stiffness and pain in the lower back and pelvis, but may be accompanied by other symptoms including fatigue.[arthritisresearchuk.org]
    • AS can cause other symptoms such as appetite loss, weight loss, fatigue, fever, anemia, eye inflammation, and GI illness.[myhealth.umassmemorial.org]
    • Other common symptoms are fever, generalized fatigue, and loss of appetite.[moveforwardpt.com]
    Reiter's Syndrome
    • The term Reiter's Syndrome is an older term that most rheumatologists have now replaced with Reactive Arthritis.[my.clevelandclinic.org]
    • Reiter Syndrome : Therapy.[arthritis.co.za]
    • Other syndromes in this group include psoriatic arthritis, reactive arthritis/Reiter's syndrome, enteropathic arthritis, and enthesitis-related idiopathic juvenile arthritis.[weillcornellbrainandspine.org]
    • […] in the morning and in the middle of the night; - note: that in women, the symptoms of ankylosing spondylitis often first present during pregnancy ; - labs: HLA-B27 (may be present in up to 8% of unaffected white population); - may also be present in Reiter's[wheelessonline.com]
    Unable to Bend
    • As the bones fuse , back pain will gradually go away, but the spine will remain very stiff and unable to bend.[uofmhealth.org]
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  • Workup

    There is always a time lag between the onset of the disease and the diagnosis as the disease has an insidious onset [6].

    An accurate diagnosis by the physician is mainly done based on these main criteria:

    • Symptoms of the patient
    • A thorough physical examination
    • Various imaging techniques mainly radiographs
    • Laboratory blood tests

    Physical examination reveals signs of inflammation and decreased range of movements mainly seen in the spine. There may be tenderness on sacroiliac joints and postural abnormalities.

    Radiographs of the spine reveal irregular margins with erosion of sacroiliac joints which are not seen in early stages. There may be fusion of joints along with demonstration of syndesmophytes between the vertebrae.

    Blood tests to detect the HLA –B27 [7] are not always useful as one should bear in mind that many people can carry this gene. Other blood tests can include an ESR to support inflammation.

    Laboratory

    Cerebrospinal Fluid
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  • Serum
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  • HLA Type
    HLA-B27
    • "The ramifications of HLA-B27".[en.wikipedia.org]
    • It is important to note however, that you do not need to have to be HLA-B27 positive to have AS and also a lot of HLA-B27 positive people never contract AS.[uab.edu]
    • About 90% of people with AS have the HLA B27 gene.[my.clevelandclinic.org]
    • In a study of German and Austrian patients with AS, the age of onset of disease symptoms was 25 years in HLA-B27 –positive and 28 years in HLA-B27 –negative patients, with a delay in diagnosis of 8.5 years in HLA-B27 –positive and 11.4 years in HLA-B27[emedicine.medscape.com]
    • The major gene that causes this disease is HLA-B27.[rheumatology.org]
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  • Imaging

    X-ray
    X-Ray Abnormal
    • Further clues to the diagnosis are suggested by X-ray abnormalities of the spine and the presence of the genetic marker HLA-B27 identified by a blood test.[medicinenet.com]
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  • ECG

    PR Interval
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  • Test Results

    Pulmonary Function Test
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  • Treatment

    Ankylosing spondylitis cannot be cured and treatment mainly focuses on relieving pain. Physical therapy and exercise enable the patient to carry out daily activities.

    • Physical and occupational therapy are recommended to reduce physical deformity and maintain function of the affected joints. Exercises should be done regularly twice a day.
    • Medications are mainly given to reduce inflammation and pain, and are usually aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs). In addition, medicines like sulfasalazine are given to suppress the immune system to patients who do not respond to NSAIDs [8].
    • Surgery is an option when there are severe deformities especially hip and knee replacements. Surgery can also be done for spine deformities, but is considered a risky option [9].

    Prognosis

    Most of the cases of ankylosing spondylitis do well, and death is rarely associated with this condition [4]. Despite restriction of spinal movement, patients are able to lead a normal life with a correct exercise regime.

    In women, the disease is milder with little restriction and no physical deformity. Ankylosing spondylitis improves with age but does not resolve completely.

    Complications

    Arthritis
    • Synonym(s): acute epidemic leukoencephalitis ; spondylitis deformans Strümpell-Marie disease - arthritis of the spine, resembling rheumatoid arthritis, that may progress to bony ankylosis.[medical-dictionary.thefreedictionary.com]
    • Other forms of arthritis may mimic AS.[healthcentral.com]
    • Enteropathic Arthritis - Arthritis Associated with Inflammatory Bowel Disease.[arthritis.co.za]
    • […] spine , arthritis; spine or vertebra, Marie-Strümpell , arthritis; spine or vertebra, ankylosing , Marie-Strümpell; spondylitis , Marie-Strümpell , Strümpell-Marie , Von Bechterew , Bechterew , rheumatoid; arthritis, spine , rheumatoid; spondylitis ,[fpnotebook.com]
    Diffuse Idiopathic Skeletal Hyperostosis
    • idiopathic skeletal hyperostosis differential diagnosis of erosive arthritis polyarticular arthropathy[radiopaedia.org]
    • idiopathic skeletal hyperostosis (DISH) • Rheumatoid arthritis [7] • Psoriatic arthritis [7] • Reiter syndrome (milder with asymmetric sacroiliitis) [7] Diagnostic Procedures The diagnosis of AS is commonly made through a combination of thorough subjective[physio-pedia.com]
    Dactylitis
    • Dactylitis (sausage digits), which is painful swelling of a finger or toe, can also develop.[merckmanuals.com]
    • **SpA features include inflammatory back pain, arthritis, enthesitis, uveitis, dactylitis, psoriasis, Crohn’s disease/ulcerative colitis, good response to NSAIDs, family history of SpA, HLA-B27, and elevated CRP.[hopkinsarthritis.org]
    • Features of SpA (spondyloarthritis) Inflammatory back pain 3 months or more Insidious onset Not relieved with rest Improvement with exercise Pain at night Heel pain due to Achilles tendinitis or plantar fasciitis (enthesitis) Dactylitis (sausage digit[clinicaladvisor.com]
    • Examples of extraspinal enthesisits include dactylitis (sausage digit), Achilles tendinitis, and plantar fasciitis.[clevelandclinicmeded.com]
    • GI) disease Metabolic bone disease Undifferentiated spondyloarthropathy Clinical manifestations of undifferentiated spondyloarthropathy include the following: Inflammatory back pain - 90% Buttock pain - 80% Enthesitis - 85% Peripheral arthritis - 35% Dactylitis[emedicine.medscape.com]
    Pleurisy
    • For more information on heart and lung problems, see the topics Heart Attack and Unstable Angina and Pleurisy .[uofmhealth.org]
    Cauda Equina Syndrome
    • equina syndrome (rare) The axial skeleton is predominantly affected, although in 20% of cases the peripheral joints are also involved.[radiopaedia.org]
    • equina syndrome 16, 17 general spine: enteropathic arthritis cervical spine: juvenile rheumatoid arthritis (JRA) diffuse idiopathic skeletal hyperostosis differential diagnosis of erosive arthritis polyarticular arthropathy[radiopaedia.org]
    • Aortic regurgitation, Achilles tendinitis, atrioventricular node block, amyloidosis, pulmonary fibrosis, and even cauda equina syndrome all occur in higher frequency in persons with AS. 3 Clearly, AS is a major cause for reduced quality of life, and when[consultant360.com]
    • Neurologically, one can see cord compression and, rarely, cauda equina syndrome. 12 Although there is syndesmophyte formation, the bone itself becomes osteoporotic.[clevelandclinicmeded.com]
    Iritis
    • Recurrent attacks of iritis can affect either eye.[medicinenet.com]
    • You may have redness and pain in the colored part of your eye ( iritis ).[uofmhealth.org]
    • […] to involvement of the costovertebral joints Peripheral arthritis involving shoulders, hips and knees Hip deformity and associated limited range of motion Tenderness over sites of inflammation Mild fatigue, fever, anorexia, or weight loss Occasional iritis[healthcentral.com]
    • […] middle-aged men; - disease is often more severe when it occurs early in life; - disease characterized by insidious onset of pain and stiffness in lower back associated w/ gradual loss of spinal mobility; - illness may cause inflammation in uveal tract (iritis[wheelessonline.com]
    Osteopenia and Osteoporosis
    • Osteopenia and osteoporosis of the spine are more commonly found in persons with AS.[consultant360.com]
    • Metabolic bone disease Osteopenia and osteoporosis may occur in patients with long-standing spondylitis, further increasing risk of fracture.[patient.info]
    • Over a long-term period, osteopenia or osteoporosis of the AP spine may occur, causing eventual compression fractures and a back "hump". [35] Hyperkyphosis from ankylosing spondylitis can also lead to impairment in mobility and balance, as well as impaired[en.wikipedia.org]
    Arthritis of the Knee
    • Those with arthritis in the knees, ankles, elbows, wrists, hands and feet should try DMARD therapy before anti-TNF treatment.[rheumatology.org]
    Psoriatic Arthritis
    • Psoriatic Arthritis ENBREL is indicated for reducing signs and symptoms, keeping joint damage from getting worse, and improving physical function in patients with psoriatic arthritis.[enbrel.com]
    • Other syndromes in this group include psoriatic arthritis, reactive arthritis/Reiter's syndrome, enteropathic arthritis, and enthesitis-related idiopathic juvenile arthritis.[weillcornellbrainandspine.org]
    • If you have a relative with either AS, psoriatic arthritis, or arthritis related to inflammatory bowel disease, you may have inherited genes that put you at greater risk for AS.[healthline.com]
    • The spondyloarthropathies include: Ankylosing spondylitis Psoriatic arthritis Reactive arthritis/Reiter's syndrome Enteropathic arthritis Undifferentiated: Patients with features of more than one disease who do not fit in the defined categories above[my.clevelandclinic.org]
    • The other disorders are psoriatic arthritis, inflammatory bowel disease, and Reiter's syndrome.[stanfordchildrens.org]
    Pulmonary Fibrosis
    • Nonskeletal problems associated with AS may include fatigue , inflammation of the iris or uvea (the colored portion of the eye), and less commonly inflammation of the aorta , scarring of the lungs (pulmonary fibrosis), amyloidosis (excess deposition of[emedicinehealth.com]
    • fibrosis (mimicking tuberculosis ) Diagnosis This problem is diagnosed through a combination of clinical history and x-rays.[healthcentral.com]
    • Aortic regurgitation, Achilles tendinitis, atrioventricular node block, amyloidosis, pulmonary fibrosis, and even cauda equina syndrome all occur in higher frequency in persons with AS. 3 Clearly, AS is a major cause for reduced quality of life, and when[consultant360.com]
    • ., uveitis , iridocyclitis), formation of fibrous tissue in the lungs (pulmonary fibrosis), and bowel and bladder dysfunction.[mdguidelines.com]
    • Pulmonary fibrosis of the upper lobes.[patient.info]
    Renal Amyloidosis
    • amyloidosis ascending aortic conditions (aortitis, stenosis, regurgitation) Klebsilella pneumoniae synovitis HLA-B27 individuals are more susceptible to Klebsilella pneumoniae synovitis Orthopaedic manifestations bilateral sacroiliitis progressive spinal[orthobullets.com]
    Polyarthritis
    • , New York Wysteria Publishers 3 , Medical Acronyms, Eponyms, and Abbreviations , 2002 Los Angeles Practice Management Information Corporation 4 , Dictionary of Medical Eponyms , 2001 2nd Lancaster UK Parthenon Publishing Group Ltd. 5 , Seronegative Polyarthritis[academic.oup.com]
    Hip Arthritis
    • Poor prognostic factors include: hip arthritis, swelling of digits, high ESR in blood, weak response to NSAID’s, and diminished motion of the lumbar spine.[healthcentral.com]
    Periostitis
    • Hands Hands are generally involved asymmetrically, with smaller, shallower erosions and marginal periostitis .[radiopaedia.org]
    Reiter's Syndrome
    • The term Reiter's Syndrome is an older term that most rheumatologists have now replaced with Reactive Arthritis.[my.clevelandclinic.org]
    • Reiter Syndrome : Therapy.[arthritis.co.za]
    • Other syndromes in this group include psoriatic arthritis, reactive arthritis/Reiter's syndrome, enteropathic arthritis, and enthesitis-related idiopathic juvenile arthritis.[weillcornellbrainandspine.org]
    • […] in the morning and in the middle of the night; - note: that in women, the symptoms of ankylosing spondylitis often first present during pregnancy ; - labs: HLA-B27 (may be present in up to 8% of unaffected white population); - may also be present in Reiter's[wheelessonline.com]
    Costochondritis
    • Common locations are the costochondral and Achilles regions.[clevelandclinicmeded.com]
    • They have current or previous buttock pain (felt on alternating sides), arthritis, enthesitis, costochondritis, epicondylitis, anterior uveitis, psoriasis or inflammatory bowel disease, or recent infective diarrhoea or sexually transmitted disease.[patient.info]
    • Inflammation can occur in the cartilage around the breastbone ( costochondritis ) as well as in the tendons where the muscles attach to the bone (tendinitis) and in ligament attachments to bone.[medicinenet.com]
    • Costochondritis, with chest pain is common.[arthritis.co.za]
    Uveitis
    Aortic Valve Insufficiency
    • Cardiovascular involvement may include inflammation of the aorta , aortic valve insufficiency or disturbances of the heart's electrical conduction system .[en.wikipedia.org]
    Kyphosis
    • This deformity is called a kyphosis.[umm.edu]
    • The disease can make the mid back round forward, a deformity called kyphosis.[depuysynthes.com]
    • The kyphosis may also cause respiratory problems, by restricting lung expansion.[patient.info]
    • In the thoracic region, the disease can lead to microfractures in the front of the vertebral body, leading to a shortening of the front of the spinal column and resulting in a hunched forward posture, called excessive kyphosis.[arthritis-health.com]
    • Symptoms usually begin between the ages of 20 and 40 years.The spine develops kyphosis (bent forward position) in the cervical, thoracic and lumbar spine and may prevent the patient from being able to look straight ahead. [spinecenter.ucla.edu]
    Spondylarthropathy
    • It is an autoimmune disorder, an inflammatory arthritic disorder, a rheumatic disease, and one of the various spondylarthropathies.[disabilitysecrets.com]
    • Prevalence of spondylarthropathies in HLA B-27 positive and negative blood donors.[consultant360.com]
    • PubMed Google Scholar Amor B, Dougados M, Mijiyawa M: Criteria for the classification of spondylarthropathies.[arthritis-research.biomedcentral.com]
    • The European Spondylarthropathy Study Group preliminary criteria for the classification of spondylarthropathy.[emedicine.medscape.com]
    • AS is the primary disease in an entire group of conditions known as seronegative spondylarthropathies.[encyclopedia.com]
    Enteropathic Arthritis
    • Other syndromes in this group include psoriatic arthritis, reactive arthritis/Reiter's syndrome, enteropathic arthritis, and enthesitis-related idiopathic juvenile arthritis.[weillcornellbrainandspine.org]
    • The cause of enteropathic arthritis is unclear.[rheumatology.org]
    • Andersson lesion: inflammatory spondylodiscitis that occurs in association with ankylosing spondylitis and results in a disc pseudarthrosis rare neurological complications include transverse myelitis and/or cauda equina syndrome 16, 17 general spine: enteropathic[radiopaedia.org]

    Etiology

    Ankylosing spondylitis has no definite cause, but is said to be a genetically determined disease, susceptibility to which is related to the presence of HLA-B27 antigen [2]. In general, people who have this gene are at a higher risk of developing spondylitis.

    Since HLA-B27 antigen is found equally in men and women, the incidence of the disease should also be equal, but it affects men more than women. White people with ankylosing spondylitis almost always carry a copy of the HLA-B gene. Family members are at a higher risk due to the chances of inheriting the carrier gene.

    Causes

    Epidemiology

    The disorder is more common in men than women, and in women the severity of the spondylitis is much milder [1]. Population surveys using radiological findings as a criterion have shown a male preponderance. Onset of symptoms is usually between 15 to 35 years of age and very rarely occurs in elderly people.

    Sex distribution
    Age distribution

    Pathophysiology

    The exact aetiology of this disorder is not clearly established, however, pathogenesis of this condition has clear association with HLA-B27 gene [2]. The primary pathology is enthesitis, which is considered a hallmark of ankylosing spondylitis. Tumour necrosis factor- alpha (TNFα) and Interleukin-1 are also associated with Ankylosing spondylitis. There is a supposed interaction between the HLA-B27 gene and the CD8+ T cell which triggers the immune system to attack the cartilage [3].
    In Ankylosing spondylitis, the rheumatoid factor tests are negative and the typical histological finding is an inflammation of the enthesis which is the insertion of tendon, ligament, capsule or surrounding fascia into bone [3].

    In ankylosing spondylitis, the enthesis is inflamed at the vertebrae, showing that the entheseal fibrocartilage is a major target of the immune system. Early leisons usually occur in the sacroiliac joints which include subchondral granulation that erodes the joint and is slowly replaced by fibrocartilage and ultimately ossification. In the spine, it is seen at the interface of the vertebra and annulus fibrosus of the intervertebral discs. These discs ultimately undergo ossification which results in syndesmophytes. This leads to ‘bamboo spine’ appearance and fusion of the vertebrae.

    In addition, there may be mild inflammation of the synovium which is present as a cushion around the joints.

    With progression of the disease there is destruction of nearby articular and joint tissues as well, resulting in complete fusion leading to immobility.

    Prevention

    There is no prevention for this inherited condition. Prevention is aimed at preventing complications and minimising pain. Physiotherapy [10] and exercise programmes are helpful for keeping the spinal cord flexible, thus enabling easy
    movements. Quitting smoking is advised for long term benefits.

    Summary

    Ankylosing spondylitis is a type of arthritis causing chronic multisystem inflammation mainly of the spine with differential involvement of various peripheral joints like the interphalangeal joints and other non-articular structures. It primarily affects the spine, sacroiliac joints in the pelvis and hip joints. Ankylosing spondylitis is a form of inflammatory arthritis. Enthesitis is a major feature of this disorder which involves inflammation of the place where ligaments and muscles are attached to the bones and accounts for much of the pain and rigidity. This can finally lead to severe bony fusion of the joints resulting in decreased flexibility and mobility.

    Ankylosing spondylitis is a systemic, rheumatic disorder that can affect and involve multiple organs such as the eye, heart, lungs, skin as well as the gastrointestinal tract.

    Ankylosing spondylitis is the most important cause for inflammatory back pain in young adults [1]. Other important associations of this disorder include peripheral arthritis and non-articular features such as iritis and uveitis.

    Patient Information

    Ankylosing spondylitis is a disorder that causes chronic inflammation of the joints in your spine, resulting in severe pain, stiffness and immobility. It can also affect other joints of the body as well as multiple organs of the body like eyes, kidney, heart, lungs and the skin. The exact cause is unknown due to which there is no known prevention or cure. It is predominantly seen in men than women.

    The most common complaint is lower back pain which radiates till the hip. The onset of the pain is slow and gradual which can increase to stiffness and restriction of movement. Ankylosing spondylitis can also affect other organs mainly the eye, which should be treated immediately as it can lead to severe complications.

    It is better to consult a medical care provider for correct diagnosis. A simple physical examination with a few tests is sufficient to confirm the condition.

    The treatment is aimed at reducing pain and suppressing inflammation. There is no single treatment. Treatment consists of exercise and physical therapy to ease movements and prevent any physical deformity, which once occurs, is more difficult to treat. Exercise should be done regularly. Medications given are non-steroidal anti-inflammatory drugs to reduce inflammation.
    Artificial joint replacement surgery is commonly performed for advanced cases affecting hip or knee.

    The condition responds well to physical therapy, exercise and medications and does not frequently need surgery. Swimming is a good exercise for ankylosing spondylitis. Patients are advised not to use pillows to prop up their head or legs as it may aggravate fusion of the bones in the joints.

    Ankylosing spondylitis does not get cured completely and the patient may show periods of relapse and remission. The main objective of the treatment is to reduce pain, maintain a good posture, facilitate easy movements and prevent deformity thus enabling patients to lead fairly normal lives.

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    References

    1. Jiménez-Balderas FJ, Mintz G. Ankylosing spondylitis: clinical course in women and men". J Rheumatol. 1993 Dec; 20(12): 2069–72.
    2. Feldtkeller E, Kha MA, van der Heijde D, van der Linden S, Braun J. Age at disease onset and diagnosis delay in HLA-B27 negative vs. Positive patients with ankylosing spondylitis. Rheumatol Int. 2003 Mar;23 (2): 61–6.
    3. Tiwana H, Natt RS, Benitez-Brito R, Shah S, et al. 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). 2001 Jan; 40(1): 15–23.
    4. Bakland G, Gran JT, Nossent JC. Increased mortality in ankylosing spondylitis is related to disease activity. Ann Rheum. Dis. 2011 Nov;70(11): 1921–5.
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