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.
Entire Body System
- Fatigue
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]
[…] of Caucasians with the disease have the human leucocyte antigen HLA-B27, compared with around 10% of the general population of North and Central Europe the disease is highly debilitating and can reduce quality of life as a result of stiffness, pain, fatigue [gpnotebook.co.uk]
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]
Periodic rest periods with avoidance of fatigue. [physio-pedia.com]
- Reiter Syndrome
The term Reiter's Syndrome is an older term that most rheumatologists have now replaced with Reactive Arthritis. [my.clevelandclinic.org]
Search for Reiter's syndrome after an outbreak of Shigella sonnei dysentery. J Rheumatol. 1979 Sep-Oct; 6 (5):562–566. [ PubMed ] [ Google Scholar ] Keat A. Reiter's syndrome and reactive arthritis in perspective. [ncbi.nlm.nih.gov]
The Seronegative Spondyloarthropathies • Ankylosing spondylitis • Reactive arthritis (formerly Reiter syndrome) [The name of this syndrome was changed because Hans Reiter, after whom the syndrome was named, was a convicted Nazi war criminal, and use of [medlink.com]
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. The fused spine is more likely to break ( fracture ) if injured, especially the neck (cervical spine). [uofmhealth.org]
- 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]
- Nocturnal Awakening
Figures and Tables - Analysis 1.8 Comparison 1 Sulfasalazine versus placebo, Outcome 8 Frequency of nocturnal awakening (change from baseline). [doi.org]
Respiratoric
- 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]
Anterior Chest Wall Pain in Recent Inflammatory Back Pain Suggestive of Spondyloarthritis. Data From the DESIR Cohort. Journal of Rheumatology. July 2013. Deminger A, Klingberg E, et al. [everydayhealth.com]
- Recurrent Chest Pain
We present a rare case of cardiac manifestations of AS in a 25-year-old man with recurrent chest pain and pericardial effusions. [ncbi.nlm.nih.gov]
Cardiovascular
- Chest Pain
He initially presented with pleuritic chest pain, was diagnosed with cardiac tamponade and required emergent pericardiocentesis. The patient returned again with chest pain and was found to have reaccumulation of pericardial effusion. [ncbi.nlm.nih.gov]
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]
For example, you may have: Red, irritated eyes and sometimes trouble seeing (uveitis) Heart problems causing chest pain, trouble breathing, or an abnormal heart rhythm Lung problems causing cough and trouble breathing Most people with ankylosing spondylitis [msdmanuals.com]
Eyes
- Anterior Uveitis
Extra-articular manifestations [ 5 ] Eye involvement Acute anterior uveitis occurs in 20-30% of patients. Of all patients presenting with acute anterior uveitis, a third to a half have or will go on to develop AS. [patient.info]
These include severe episodes of acute anterior uveitis, osteoporosis with fragility fractures, and also post-traumatic spinal fractures on three different occasions. [ncbi.nlm.nih.gov]
- Blurred Vision
See an eye specialist immediately if you develop a painful red eye, severe light sensitivity or blurred vision. Causes Ankylosing spondylitis has no known specific cause, though genetic factors seem to be involved. [mayoclinic.org]
Signs of iritis or uveitis are: Eye(s) becoming painful, watery, and red, blurred vision, and sensitivity to bright light. More information on complications of AS, including iritis/uveitis Learn More [spondylitis.org]
Eye pain and redness, significant sensitivity to light, or blurred vision—in addition to or instead of back pain. [arthritis-health.com]
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]
- 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]
Musculoskeletal
- Arthritis
What causes enteropathic arthritis? The cause is unknown. What are the signs and symptoms of enteropathic arthritis? The arthritis typically occurs after the bowel disease is well established. Rarely, the arthritis can start before IBD is diagnosed. [my.clevelandclinic.org]
Ankylosing spondylitis shares many features with several other arthritis conditions, such as psoriatic arthritis, reactive arthritis (formerly called Reiter's disease), and arthritis associated with Crohn's disease and ulcerative colitis. [medicinenet.com]
Ankylosing Spondylitis is one of a group of arthritis conditions known as spondoarthritides, including psoriatic, colitic and reactive arthritis. [sportsinjuryclinic.net]
Arthritis in the hips and shoulders may also occur. [en.wikipedia.org]
- Back Pain
The most common symptom is Back pain Back pain varies in intensity from one episode to another and from one person to another. Pain is often worse during the night and in the morning. [merckmanuals.com]
People with this disorder experience lower back pain, morning stiffness, and limited motion. These symptoms commonly improve after exercise and worsen after sleep or prolonged rest. [hss.edu]
back pain care, and cost. [ncbi.nlm.nih.gov]
Inflammatory back pain will tend to improve with stretching and physical activity and worsen with prolonged inactivity. [hopkinsarthritis.org]
[…] chronic back pain and stiffness. [hlab27.com]
- Osteoporosis
Osteoporosis : bisphosphonates are often used to treat osteoporosis and reduce the risk of fracture in AS [ 17 ]. [patient.info]
However, in patients with severe osteoporosis and prevalent fractures, available guidelines in osteoporotic participants and male osteoporosis must be applied. [doi.org]
After AS diagnosis, a higher proportion of patients developed newly diagnosed cases of cardiovascular diseases, depression, osteoporosis, spinal fracture, IBD, psoriasis, and uveitis than matched controls. [ncbi.nlm.nih.gov]
- 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]
[…] a sample with no recalled history of ever having had low back pain. [doi.org]
AS starts with low back pain that comes and goes. Low back pain becomes present most of the time as the condition progresses. Pain and stiffness are worse at night, in the morning, or when you are less active. The discomfort may wake you from sleep. [nlm.nih.gov]
- Buttock Pain
A 33-year-old woman was diagnosed with AS due to chronic buttock pain and progressive sacroiliitis on plain X-ray and magnetic resonance imaging (MRI). Her buttock and low back pain gradually worsened despite proper treatment for AS. [ncbi.nlm.nih.gov]
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]
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]
Neurologic
- Sleep Disturbance
Compared with healthy controls, AS patients had more severe psychological disorders, sleep disturbance, and stressful life events (P [ncbi.nlm.nih.gov]
Figures and Tables - Analysis 1.7 Comparison 1 Sulfasalazine versus placebo, Outcome 7 Score of sleep disturbance (end point) (0 to 4, 0 = no disturbance, 4 = severe disturbance). [doi.org]
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]
- Sciatica
Neurologic signs occasionally result from compression radiculitis or sciatica, vertebral fracture or subluxation, or cauda equina syndrome. [merckmanuals.com]
Symptoms that can be associated with low back pain include dull ache, numbness, tingling, sharp pain, pulsating pain, pain with movement of the spine, pins and needles sensation, muscle spasm, tenderness, sciatica with shooting pain down one or both lower [medicinenet.com]
Torticollis Spondylopathy inflammatory: Ankylosing spondylitis • Sacroiliitis • Discitis • Pott disease noninflammatory: Spondylosis • Spondylolysis • Spondylolisthesis • Spinal stenosis Back pain Neck pain • Upper back pain • Low back pain ( Coccydynia, Sciatica [marefa.org]
- Burning Sensation
"Pain" is generally described as a sharp ache, or a burning sensation in the associate muscles and tendons. [roshehomeo.com]
Symptoms include scratchy, gritty, irritated, dry eyes, dry mouth, nose, throat, larynx, bronchi, skin; burning sensation in mouth or throat, hoarse or weak voice, difficulty swallowing. Salivary glands may be enlarged and painful. [margarethillsclinic.com]
The pain may be characterized as a dull ache, shooting or piercing pain, or a burning sensation. Discomfort can radiate into the arms and hands as well as the legs or feet,[3] and may include numbness,[1] or weakness in the legs and arms. [en.wikipedia.org]
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
- 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]
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. Other blood tests may provide evidence of inflammation in the body. [medicinenet.com]
- Nephrolithiasis
[…] regulation 7.5% Abnormality of the aortic valve 7.5% Abnormality of the pericardium 7.5% Abnormality of the pleura 7.5% Anorexia 7.5% Arrhythmia 7.5% Autoimmunity 7.5% Cartilage destruction 7.5% Hematuria 7.5% Hemiplegia/hemiparesis 7.5% Hyperkeratosis 7.5% Nephrolithiasis [web.archive.org]
Serum
- Normocytic Anemia
Hematology tests showed a mild normocytic anemia, and a normal white count. Erythrocyte sedimentation rate and alkaline and creatinine phosphatase were elevated. There was no rheumatoid factor present. [mhmedical.com]
Anemia Patients may have a mild normochromic normocytic anemia, another nonspecific finding. [rheumatologyadvisor.com]
- Hypercapnia
PaO 2 values at rest generally were within normal limts and no hypercapnia was seen any of the patients. In 40% of all cases D(A-a)O 2 was > 90 mmHg. TLC was normal in all 40 patients in whom it was measured. [archbronconeumol.org]
Pulmonary Function Test
- 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 Type
- HLA-B27
[…] spondylitis in HLA-B27 (+) individuals. [ncbi.nlm.nih.gov]
Those with the HLA-B27 variant are at a higher risk than the general population of developing the disorder. [en.wikipedia.org]
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.
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
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
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- 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.
- 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.
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- Benoist M. Pierre Marie. Pioneer investigator in ankylosing spondylitis. Spine (Phila Pa 1976). 1995 Apr1;. 20 (7): 849–52.
- Calin A. Ankylosing spondylitis. Clin Rheum Dis. 1985 Apr; 11 (1): 41–60.
- 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.
- 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.