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Ankylosing Spondylitis

Bechterew Disease

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).


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.

Chest Wall Pain
  • Enthesitis or inflammation at insertions of tendons, ligaments and joint capsules into bone can present as Achilles tendinitis, plantar fasciitis and intercostal enthesitis, which causes chest wall pain.[racgp.org.au]
Fatigue
  • LLoA;ULoA [bias] for fatigue was - 37.4;36.2 [- 0.6], for pain - 34.1;32.5 [- 0.8], for PaGl - 35.7;32.9 [- 1.4] and for BASFI - 23.2;22.6 [- 0.3].[ncbi.nlm.nih.gov]
  • Measures of disease activity, mobility, function, mood, fatigue, quality of life, work productivity, night-time pain and general health were gathered.[ncbi.nlm.nih.gov]
  • GETTY Ankylosing spondylitis: Symptoms include fatigue Ankylosing spondylitis causes back pain, stiffness and fatigue A total of five factors were identified as independent predictors of a poor quality of life among patients: moderate to severe fatigue[express.co.uk]
  • Fatigue Fatigue is a common symptom of untreated AS. It can make you feel tired and lacking in energy. Page last reviewed: 08/07/2016 Next review due: 08/07/2019[nhs.uk]
  • Back pain and stiffness, joint pain, swelling, fatigue, and a hunched posture are common symptoms. Treatment involves medications and exercises to relieve symptoms and keep the back as strong and flexible as possible.[kidshealth.org]
Reiter's Syndrome
  • Other syndromes in this group include psoriatic arthritis, reactive arthritis/Reiter's syndrome, enteropathic arthritis, and enthesitis-related idiopathic juvenile arthritis.[weillcornellbrainandspine.org]
  • syndrome (unilateral asymmetric SI joint involvement, paravertebral ossifications) Psoriatic arthritis (unilateral asymmetric SI joint involvement, paravertebral ossifications) Inflammatory bowel disease Associated with: Ulcerative colitis Regional enteritis[learningradiology.com]
  • […] 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]
  • Reiter Syndrome. - reactive arthritis Reiter Syndrome was first described in 1916 by H. Reiter and is the prime model of reactive arthritis.[arthritis.co.za]
  • The other disorders are psoriatic arthritis, inflammatory bowel disease, and Reiter's syndrome.[stanfordchildrens.org]
Unable to Bend
  • As the bones fuse , back pain will gradually go away, but the spine will remain very stiff and unable to bend. The fused spine is more likely to break ( fracture ) if injured, especially the neck (cervical spine).[healthlinkbc.ca]
Chronic Fatigue Syndrome
  • fatigue syndrome (M.E) Chronic inflammatory demyelinating polyneuropathy (CIPD) Chronic recurrent multifocal ostomyelitis (CRMO) Cogan’s syndrome Cold agglutinin disease Congenital heart block Coxsackie viral myocarditis CREST syndrome (limited systemic[autoimmunecoach.co.uk]
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]
  • Tell your doctor if you have any symptoms of an allergic reaction while taking SIMPONI such as hives, swollen face, breathing trouble, or chest pain. Some reactions can be serious and life-threatening.[simponi.com]
  • pain, which may suggest heart, heart valve, or lung problems Eye pain, visual changes, increased tearing Less common symptoms may include: Fatigue Loss of appetite or weight loss Fever Numbness (if arthritic spurs compress the spinal nerves) Blood in[cancercarewny.com]
  • AS can affect any part of the spine and may progress to the ribcage, causing chest pain that may be confused with angina or pleurisy. Sometimes joints such as the knee, and tendons (especially around the ankle), can be affected.[southerncross.co.nz]
  • Chest wall cartilage inflammation can cause chest pain and tenderness.[emedicinehealth.com]
Blurred Vision
  • One of the most common complications of ankylosing spondylitis, uveitis can cause rapid-onset eye pain, sensitivity to light and blurred vision. See your doctor right away if you develop these symptoms. Compression fractures.[mayoclinic.org]
  • This may include pain in the eye or brow region, pain associated with exposure to light, blurred vision or a reddened eye. The lining of the bowel may also be affected, causing symptoms of inflammatory bowel disease such as diarrhoea and bloating.[physioworks.com.au]
  • Symptoms are floaters and, sometimes, blurred vision, and there may be anterior chamber cells and flare. Ophthalmoscopic examination may show vitreous condensation and gelatinous exudates (' cotton balls' or ' snowballs' ).[medical-dictionary.thefreedictionary.com]
  • Extra-articular features Uniocular anterior uveitis occurs in about 40% of patients and typically presents as acute painful red eye, with blurred vision and photophobia.[racgp.org.au]
  • vision), vomit that looks like coffee grounds, black/bloody stools, severe stomach/abdominal pain, mental/mood changes (such as depression, mood swings, agitation), slow wound healing, thinning skin, bone pain, menstrual period changes, puffy face, seizures[espanol.kaiserpermanente.org]
Ciliary Injection
  • Acute anterior uveitis is accompanied by pain, photophobia and lacrimation and some loss of vision because of exudation of cells (aqueous flare), protein-rich fluid and fibrin into either the anterior chamber or vitreous body, as well as ciliary injection[medical-dictionary.thefreedictionary.com]
Arthritis
  • These disorders include ankylosing spondylitis (AS), reactive arthritis, psoriatic arthritis and spondylitis, enteropathic arthritis and spondylitis, juvenile-onset spondyloarthritis, and undifferentiated spondyloarthritis.[ncbi.nlm.nih.gov]
  • Furthermore; inflammatory disorders such as rheumatoid arthritis (RA), psoriasis, psoriatic arthritis (PSA), and ankylosing spondylitis (AS) have been associated with IL-17, and therapeutically targeting this inflammatory pathway could improve patients[ncbi.nlm.nih.gov]
  • Ankylosing spondylitis (AS) is an extreme form of inflammatory arthritis which always leads to bony fusion of vertebral and chronic pain of back.[ncbi.nlm.nih.gov]
  • Enteropathic arthritis is spinal arthritis that also involves inflammation of the intestinal wall. Symptoms can come and go. And when the abdominal pain is flaring, this arthritis may also flare.[northshore.org]
  • What are the different types of arthritis? Tue, February 21, 2017 Arthritis: From osteoarthritis to rheumatoid, here are different types of the painful condition that affects around 10 million people in the UK.[express.co.uk]
Back Pain
  • Studies examining geographic variation in care for low back pain often focus on process and outcome measures conditional on patient diagnosis but generally do not take into account a physician's ability to diagnose the root cause of low back pain.[ncbi.nlm.nih.gov]
  • The Bath ankylosing spondylitis disease activity index (BASDAI), nocturnal back pain (NBP), total back pain (TBP), patient global disease activity (PGDA), the Bath ankylosing spondylitis functional index (BASFI), and the Bath ankylosing spondylitis metrology[ncbi.nlm.nih.gov]
  • This quiz is not meant to diagnose patients with inflammatory back pain or diseases associated with inflammatory back pain such as AS. If you are experiencing back pain, you should talk with your doctor.[faceyourbackpain.com]
  • But you should be aware that back pain can also signal a more serious condition called ankylosing spondylitis. Unlike ordinary back pain, ankylosing spondylitis (AS) is not caused by physical trauma to the spine.[healthline.com]
Low Back Pain
  • Studies examining geographic variation in care for low back pain often focus on process and outcome measures conditional on patient diagnosis but generally do not take into account a physician's ability to diagnose the root cause of low back pain.[ncbi.nlm.nih.gov]
  • This case highlights how a differential diagnosis of low back pain with neurological symptoms can be particularly troublesome in ankylosing spondylitis and that continuous vigilance is warranted in patients treated with long-term immunosuppressive therapies[ncbi.nlm.nih.gov]
  • Ankylosing spondylitis most commonly presents in young males (15-30 years old) as persistent low back pain and stiffness that is worse in the morning and at night and improves with activity.[ncbi.nlm.nih.gov]
  • Spondyloarthropathies often cause: Low back pain that may spread into the buttock. Morning stiffness, especially in the back or neck, that gets better during the day and after exercise. Fatigue.[northshore.org]
  • HLA-B27 There is no single laboratory test which is diagnostic of AS, although the presence of the genetic marker HLA-B27 is supportive in a patient who has inflammatory low back pain.[rheumatologyadvisor.com]
Neck Pain
  • If neck pain involves compression of your nerves, you may feel numbness, tingling, or weakness in your arm or hand. Causes A common cause of neck pain is muscle strain or tension. Most often, everyday activities are to blame.[mountsinai.org]
  • The condition affects the joints in the spine primarily and can cause abnormal bone growth, ligament calcification, and fusion of the spine, with ramifications for mobility with back and neck pain .[painneck.com]
  • pain in pt w/ AS is usually caused by frx thru ankylosed spine; - frx involves all columns & may be assoc w/ dramatic change in neck alignment; - ref: Uncommon Complication After Revision Hip Surgery - Peripheral Arthritis: - may involve hips, knees,[wheelessonline.com]
Buttock Pain
  • pain radiating to the posterior thigh, associated with numbness in the leg, gait disturbance and low-grade fever.[ncbi.nlm.nih.gov]
  • The more common symptoms include: back pain or buttock pain, that may be worse in bed at night stiffness in the morning, or after long periods of rest other joint pain pain in tendons and ligaments, such as in the chest wall, soles of the feet, or heels[healthdirect.gov.au]
  • This inflammation can cause buttock pain that radiates into the thigh and/or diffuse lower back pain. The sacroiliac joints can eventually fuse together, limiting mobility. The same process of inflammation and calcification can spread up the spine.[arthritis-health.com]
  • pain that came on slowly, is worse in the morning or awakens you from your sleep in the second half of the night — particularly if this pain improves with exercise and worsens with rest.[mayoclinic.org]
Sleep Disturbance
  • AS patients had more anxiety, depression, stressful life events, and sleep disturbance than healthy controls. Pain, functional limitation, sleep disturbance, and education were major contributors to psychological disorders.[ncbi.nlm.nih.gov]
  • This study also highlights the need to standardise the measurement of sleep disturbance in axSpA to facilitate comparisons between patient groups and interventions.[ncbi.nlm.nih.gov]
  • Symptoms tend to worsen in the early morning hours, causing sleep disturbance in many patients. Inflammatory back pain will tend to improve with stretching and physical activity and worsen with prolonged inactivity.[hopkinsarthritis.org]
  • Sleep disturbance, particularly in the second half of the night. Persistence of the above symptoms for more than 3 months. Pain relieved for a time after a shower, bath or heat treatments. Inflammation of the iris, within the eye.[physioworks.com.au]

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.

X-Ray Abnormal
  • The earliest x-ray abnormalities are pseudowidening caused by subchondral erosions, followed by sclerosis or later narrowing and eventually fusion in the sacroiliac joints. Changes are symmetric.[merckmanuals.com]
Decreased Vital Capacity
  • Pulmonary function tests show decreased vital capacity and total lung capacity, although residual and functional residual lung volumes were increased. Hematology tests showed a mild normocytic anemia, and a normal white count.[mhmedical.com]
HLA-B27
  • In literature, although the evidence for association between AS and periodontitis as well as AS and HLA-B27 are there but the association of aggressive periodontitis in HLA-B27 positive patient with AS are not there.[ncbi.nlm.nih.gov]
  • […] spondylitis in HLA-B27 ( ) individuals.[ncbi.nlm.nih.gov]
  • Several reports have implicated K. pneumoniae as a triggering or perpetuating factor in AS; and the HLA-B27 antigen has also been found in association with AS.[ncbi.nlm.nih.gov]
  • The genetic risk of the HLA-B27 specificity and the CTLA4/CT60 polymorphism were assessed by odds ratios (OR) with 95% confidence intervals (CI).[ncbi.nlm.nih.gov]
  • […] of 215 HLA-B27-positive patients with AS and 216 HLA-B27-positive matched controls were enrolled and genotyped for rs767455, rs2234649, and rs1061622.[ncbi.nlm.nih.gov]

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.

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.

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.

References

Article

  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.
  5. Garrett S, Jenkinson T, Kennedy LG, Whitelock H, Gaisford P. A new approach to defining disease status in ankylosing spondylitis: the Bath Ankylosing Spondylitis Disease Activity Index. J Rheumatol. 1994 Dec;21 (12): 2286–91.
  6. Bond D. Ankylosing spondylitis: diagnosis and management. Nurs Stand. 2013 Dec 18;28 (16-18): 52–9; quiz 60.
  7. Benoist M. Pierre Marie. Pioneer investigator in ankylosing spondylitis. Spine (Phila Pa 1976). 1995 Apr1;. 20 (7): 849–52.
  8. Calin A. Ankylosing spondylitis. Clin Rheum Dis. 1985 Apr; 11 (1): 41–60.
  9. Del Din S, Carraro E, Sawacha Z, Guiotto A, et al. Impaired gait in ankylosing spondylitis. Med Biol Eng Comput. 2011 Jul;49 (7): 801–9.
  10. Ortancil O, Sarikaya S, Sapmaz P, Basaran A, Ozdolap S. The effect(s) of a six-week home-based exercise program on the respiratory muscle and functional status in ankylosing spondylitis. J Clin Rheumatol. 2009 Mar;15 (2): 68–70.

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Last updated: 2018-06-22 01:03