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The signs and symptoms of ankylosing spondylitis often appear gradually, with peak onset being between 20 and 30 years of age. Initial symptoms are usually a chronic dull pain in the lower back or gluteal region combined with stiffness of the lower back. Individuals often experience pain and stiffness that awakens them in the early morning hours.
As the disease progresses, loss of spinal mobility and chest expansion, with a limitation of anterior flexion, lateral flexion, and extension of the lumbar spine, are seen. Systemic features are common, with weight loss, fever, or fatigue often present. Pain is often severe at rest but may improve with physical activity, but inflammation and pain to varying degrees may recur regardless of rest and movement.
AS can occur in any part of the spine or the entire spine, often with pain referred to one or the other buttock or the back of the thigh from the sacroiliac joint.
Arthritis in the hips and shoulders may also occur. When the condition presents before the age of 18, it is more likely to cause pain and swelling of large lower limb joints, such as the knees. In prepubescent cases, pain and swelling may also manifest in the ankles and feet where heel pain and enthesopathy commonly develop. Less commonly ectasia of the sacral nerve root sheaths may occur.
About 40% of people with AS will also experience inflammation of the anterior chamber of the eye, causing eye pain, redness, floaters and sensitivity to light. This is thought to be due to the association that both AS and uveitis have with the inheritance of the HLA-B27 antigen.
Inflammation of the prostate occurs with increased frequency in men.
Cardiovascular involvement may include inflammation of the aorta, aortic valve insufficiency or disturbances of the heart's electrical conduction system. Lung involvement is characterized by progressive fibrosis of the upper portion of the lung.
Spondyloarthropathy or spondyloarthrosis refers to any joint disease of the vertebral column. As such, it is a class or category of diseases rather than a single, specific entity. It differs from spondylopathy, which is a disease of the vertebra itself. However, many conditions involve both spondylopathy and spondyloarthropathy.
Spondyloarthropathy with inflammation is called axial spondyloarthritis. In the broadest sense, the term spondyloarthropathy includes joint involvement of vertebral column from any type of joint disease, including rheumatoid arthritis and osteoarthritis, but the term is often used for a specific group of disorders with certain common features, the group often being termed specifically seronegative spondylarthropathies. They have an increased incidence of HLA-B27, as well as negative rheumatoid factor and ANA. Enthesopathy is also sometimes present in association with seronegative.
Non-vertebral signs and symptoms of degenerative or other not-directly-infected inflammation, in the manner of spondyloarthropathies, include asymmetric peripheral arthritis (which is distinct from rheumatoid arthritis), arthritis of the toe interphalangeal joints, sausage digits, Achilles tendinitis, plantar fasciitis, costochondritis, iritis, and mucocutaneous lesions. However, lower back pain is the most common clinical presentation of the causes of spondyloarthropoathies; this back pain is unique because it decreases with activity.
Ankylosing spondylitis (AS) is a type of arthritis in which there is long term inflammation of the joints of the spine. Typically the joints where the spine joins the pelvis are also affected. Occasionally other joints such as the shoulders or hips are involved. Eye and bowel problems may also occur. Back pain is a characteristic symptom of AS, and it often comes and goes. Stiffness of the affected joints generally worsens over time.
Although the cause of ankylosing spondylitis is unknown, it is believed to involve a combination of genetic and environmental factors. More than 90% of those affected have a specific human leukocyte antigen known as the HLA-B27 antigen. The underlying mechanism is believed to be autoimmune or autoinflammatory. Diagnosis is typically based on the symptoms with support from medical imaging and blood tests. AS is a type of seronegative spondyloarthropathy, meaning that tests show no presence of rheumatoid factor (RF) antibodies. It is also within a broader category known as axial spondyloarthritis.
There is no cure for ankylosing spondylitis. Treatments may improve symptoms and prevent worsening. This may include medication, exercise, and surgery. Medications used include NSAIDs, steroids, DMARDs such as sulfasalazine, and biologic agents such as infliximab.
Between 0.1% and 1.8% of people are affected. Onset is typically in young adults. Males are more often affected than females. The condition was first fully described in the late 1600s by Bernard Connor, but skeletons with ankylosing spondylitis are found in Egyptian mummies. The word is from Greek "ankylos" meaning stiffening, "spondylos" meaning vertebra, and "-itis" meaning inflammation.
The following conditions are typically included within the group of "seronegative spondylarthropathies":
Some sources also include Behcet's disease and Whipple's disease.
People suffering from sacroiliitis can often experience symptoms in a number of different ways, however it is commonly related to the amount of pressure that is put onto the sacroiliac joint. Sacroiliitis pain is typically axial, meaning that the location of the condition is also where the pain is occurring. Symptoms commonly include prolonged, inflammatory pain in the lower back region, hips or buttocks.
However, in more severe cases, pain can become more radicular and manifest itself in seemingly unrelated areas of the body including the legs, groin and feet.
Symptoms are typically aggravated by:
- Transitioning from sitting to standing
- Walking or standing for extended periods of time
- Running
- Climbing stairs
- Taking long strides
- rolling over in bed
Axial spondyloarthritis (also often referred to as axSpA) is a chronic, autoinflammatory disease predominantly affecting the axial skeleton (sacroiliac joints and spine). The most known member of the axial spondyloarthritis disease family is ankylosing spondylitis. Axial spondyloarthritis is an umbrella term that has been introduced in the year 2009 to characterize a diverse disease family that share clinical and genetic features, such as the involvement of the axial skeleton. The expression was introduced in order to unify (1) less severe forms of spondylitis, (2) the early phase of ankylosing spondylitis as well as (3) ankylosing spondylitis itself into one term.
Sacroiliitis is a condition caused by inflammation within the sacroiliac joint. This joint is located where the base of the spine, known as the sacrum, and the pelvis, known as the ilium, intersect. "Itis" is a latin term denoting inflammation.
Since sacroiliitis can describe any type of inflammation found within the sacroiliac joint, there can be a number of issues that cause it. These include:
- Degenerative arthritis, or osteoarthritis of the spine, can cause degeneration within the sacroiliac joints and lead to inflammation and joint pain.
- Any form of spondyloarthropathies, which includes ankylosing spondylitis, psoriatic arthritis, reactive arthritis or arthritis related to inflammatory bowel diseases, including ulcerative colitis or Crohn's disease.
- Pregnancy can cause inflammation as a result of the widening and stretching of the sacroiliac joints to prepare for childbirth. Additionally, the added weight carried during childbearing can put an extra amount of stress on the SI joints, leading to abnormal wear.
- Traumatic injury such as a fall or car crash that affects the lower back, hips, buttocks or legs.
- Though rare, infection within the sacroiliac joints or another part of the body, such as a urinary tract infection, can cause inflammation.
In 1984, a joint effort led to the definition of specific classification criteria for ankylosing spondylitis, called the “Modified New York Criteria”. One of the central New York criteria was the existence of radiographically visible changes in the sacroiliac joints and/or spine, which have formed due to bone fusion, erosion and/or formation caused by the disease. Even though these criteria helped to improve uniformly define ankylosing spondylitis, such radiologic changes often only manifested several years after the first disease symptoms appeared. In order to be able to study also patients with early and less typical forms, new criteria were needed that could identify the disease already at an early stage. In 2009 the Modified New York criteria were extended by a broad set of new classification criteria that aimed to classify patients based on the presence of typical spondyloarthritis disease features. These included inflammatory back pain, family history for axial spondyloarthritis, response to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs), past history of or current inflammation in the joints (arthritis), tendon-bone attachment of the heel (enthesitis), or eyes (uveitis), bowel (inflammatory bowel disease), skin (psoriasis) or signs of elevated inflammation (C-reactive protein and erythrocyte sedimentation rate. Important parts of the ASAS axSpA criteria is the biomarker HLA-B27 and magnetic resonance imaging (MRI). The criteria can only be applied in people that have chronic back pain (at least 3 months duration) started before the age of 45 years and only in those patients that already have a diagnosis of axial SpA. Since the disease ankylosing spondylitis was still defined by the Modified New York criteria of 1984, there was the need to find a new disease term that would also include the less severe forms or early onset of ankylosing spondylitis. This expression was found in the umbrella term axial spondyloarthritis. The 2009 classification criteria are called the ASAS (Assessment of SpondyloArthritis international Society) axial spondayloarthritis criteria.
Pain, swelling, or stiffness in one or more joints is commonly present in psoriatic arthritis. Psoriatic arthritis is inflammatory, and affected joints are generally red or warm to the touch. Asymmetrical oligoarthritis, defined as inflammation affecting one to four joints during the first six months of disease, is present in 70% of cases. However, in 15% of cases, the arthritis is symmetrical. The joints of the hand that is involved in psoriasis are the proximal interphalangeal (PIP), the distal interphalangeal (DIP), the metacarpophalangeal (MCP), and the wrist. Involvement of the distal interphalangeal joints (DIP) is a characteristic feature and is present in 15% of cases.
In addition to affecting the joints of the hands and wrists, psoriatic arthritis may affect the fingers, nails, and skin. Sausage-like swelling in the fingers or toes, known as dactylitis, may occur. Psoriasis can also cause changes to the nails, such as pitting or separation from the nail bed, onycholysis, hyperkeratosis under the nails, and horizontal ridging. Psoriasis classically presents with scaly skin lesions, which are most commonly seen over extensor surfaces such as the scalp, natal cleft and umbilicus.
In psoriatic arthritis, pain can occur in the area of the sacrum (the lower back, above the tailbone), as a result of sacroiliitis or spondylitis, which is present in 40% of cases. Pain can occur in and around the feet and ankles, especially enthesitis in the Achilles tendon (inflammation of the Achilles tendon where it inserts into the bone) or plantar fasciitis in the sole of the foot.
Along with the above-noted pain and inflammation, there is extreme exhaustion that does not go away with adequate rest. The exhaustion may last for days or weeks without abatement. Psoriatic arthritis may remain mild or may progress to more destructive joint disease. Periods of active disease, or flares, will typically alternate with periods of remission. In severe forms, psoriatic arthritis may progress to arthritis mutilans which on X-ray gives a "pencil-in-cup" appearance.
Because prolonged inflammation can lead to joint damage, early diagnosis and treatment to slow or prevent joint damage is recommended.
The exact causes are not yet known, but a number of genetic associations have been identified in a genome-wide association study of psoriasis and psoriatic arthritis including HLA-B27.
Symptoms of inflammatory arthritis include stiffness, pain, and swelling of the joints, restricted motions, and reduced physical strength. Other symptoms may include systemic complaints including fatigue.
Arthritis of joints involves inflammation of the synovial membrane. Joints become swollen, tender and warm, and stiffness limits their movement. With time, multiple joints are affected (polyarthritis). Most commonly involved are the small joints of the hands, feet and cervical spine, but larger joints like the shoulder and knee can also be involved. Synovitis can lead to tethering of tissue with loss of movement and erosion of the joint surface causing deformity and loss of function.
RA typically manifests with signs of inflammation, with the affected joints being swollen, warm, painful and stiff, particularly early in the morning on waking or following prolonged inactivity. Increased stiffness early in the morning is often a prominent feature of the disease and typically lasts for more than an hour. Gentle movements may relieve symptoms in early stages of the disease. These signs help distinguish rheumatoid from non-inflammatory problems of the joints, such as osteoarthritis. In arthritis of non-inflammatory causes, signs of inflammation and early morning stiffness are less prominent with stiffness typically less than one hour, and movements induce pain caused by mechanical arthritis.
The pain associated with RA is induced at the site of inflammation and classified as nociceptive as opposed to neuropathic. The joints are often affected in a fairly symmetrical fashion, although this is not specific, and the initial presentation may be asymmetrical.
As the pathology progresses the inflammatory activity leads to tendon tethering and erosion and destruction of the joint surface, which impairs range of movement and leads to deformity. The fingers may suffer from almost any deformity depending on which joints are most involved. Specific deformities, which also occur in osteoarthritis, include ulnar deviation, boutonniere deformity (also "buttonhole deformity", flexion of proximal interphalangeal joint and extension of distal interphalangeal joint of the hand), swan neck deformity (hyperextension at proximal interphalangeal joint and flexion at distal interphalangeal joint) and "Z-thumb." "Z-thumb" or "Z-deformity" consists of hyperextension of the interphalangeal joint, fixed flexion and subluxation of the metacarpophalangeal joint and gives a "Z" appearance to the thumb. The hammer toe deformity may be seen. In the worst case, joints are known as arthritis mutilans due to the mutilating nature of the deformities.
RA primarily affects joints, but it also affects other organs in more than 15–25% of individuals.
Treatments for inflammatory arthritis vary by subtype, though they may include drugs like DMARDs (disease-modifying anti-rheumatic drugs) and tumor necrosis factor inhibitors.
If not treated right away, there are many consequences and pains various cervical spine disorders can cause.
- Neck pains Pains in the neck area tend to be tenacious and persistent and most muscles in the cervical spinal region tighten causing for discomfort.
- Headaches Headaches are further triggered through the stiffness of neck muscles, which pull at their attachment to the skull. These headaches are recurrent in nature and start from the base of the skull and emanate upwards; they can be painful or mild.
- Arm pains Muscular spasms within the arm are further common symptoms in which such spasms are seen right above the collarbones and pressure is placed on the Brachial plexus causing arms to feel heavy and ache.
- Difficulty walking Hardships arise with cervical spinal injuries when issues with walking, balancing, and posture are affected all due to the spinal cord being compressed resulting in Myelopathy.
- Those with extremely severe outcomes may result in:Impairment
There are several conditions and syndromes that can affect the cervical spine and they all vary due to the difference in place and type of injury.
- Rheumatoid arthritis Those infected with rheumatoid arthritis in their cervical spine are known to have neurological deficits. It results in occipital pain and myelopathy.
- Occipito-cervical junction This disorder may result from rheumatoid arthritis, causing the hyper-mobility of the connection between the neck and head, resulting in paralysis or pain.
- Cerebrovascular disease Cerebrovascular disease is a type of cervical spine disorder that can cause tetraplegia.
- Subaxial cervical spine
- Atlanto-axial joint
Grisel’s syndrome is a non-traumatic subluxation of the atlanto-axial joint caused by inflammation of the adjacent tissues. This is a rare disease that usually affects children. Progressive throat and neck pain and neck stiffness can be followed by neurologic symptoms such as pain or numbness radiating to arms (radiculopathies). In extreme cases, the condition can lead to quadriplegia and even death from acute respiratory failure. The condition often follows soft tissue inflammation in the neck such as in cases of upper respiratory tract infections, peritonsillar or retropharyngeal abscesses. Post-operative inflammation after certain procedures such as adenoidectomy can also lead to this condition in susceptible individuals such as those with Down's syndrome.
Pathophysiology of this disease consists of relaxation of the transverse ligament of the atlanto-axial joint.
A flexion teardrop fracture is a fracture of the anteroinferior aspect of a cervical vertebral body due to flexion of the spine along with vertical axial compression. A teardrop fracture is usually associated with a spinal cord injury, often a result of displacement of the posterior portion of the vertebral body into the spinal canal.
Symptoms of Bennett fracture are instability of the CMC joint of the thumb, accompanied by pain and weakness of the pinch grasp. Characteristic signs include pain, swelling, and ecchymosis around the base of the thumb and thenar eminence, and especially over the CMC joint of the thumb. Physical examination demonstrates instability of the CMC joint of the thumb. The patient will often manifest a weakened ability to grasp objects or perform such tasks as tying shoes and tearing a piece of paper. Other complaints include intense pain experienced upon catching the thumb on an object, such as when reaching into a pants pocket.
Individuals with Jefferson fractures usually experience pain in the upper neck but no neurological signs. The fracture may also cause damage to the arteries in the neck, resulting in lateral medullary syndrome, Horner's syndrome, ataxia, and the inability to sense pain or temperature.
In rare cases, congenital abnormality may cause the same symptoms as a Jefferson fracture.
The most common symptom is pain over the heel area, especially when the heel is palpated or squeezed. Patients usually have a history of recent trauma to the area or fall from a height. Other symptoms include: inability to bear weight over the involved foot, limited mobility of the foot, and limping. Upon inspection, the examiner may notice swelling, redness, and hematomas. A hematoma extending to the sole of the foot is called "Mondor Sign", and is pathognomonic for calcaneal fracture. The heel may also become widened with associated edema due to displacement of lateral calcaneal border. Involvement of soft tissue (tendons, skin, etc.,) should be evaluated because soft tissue injury has been associated to serious complications (see below).
Tibial plateau fractures typically presents with knee effusion, swelling of the knee soft tissues and inability to bear weight. The knee may be deformed due to displacement and/or fragmentation of the tibia which leads to loss of its normal structural appearance. Blood in the soft tissues and knee joint (hemarthrosis) may lead to bruising and a doughy feel of the knee joint. Due to the tibial plateau's proximity to important vascular (i.e. arteries, veins) and neurological (i.e. nerves such as peroneal and tibial) structures, injuries to these may occur upon fracture. A careful examination of the neurovascular systems is imperative. A serious complication of tibial plateau fractures is compartment syndrome in which swelling causes compression of the nerves and blood vessels inside the leg and may ultimately lead to necrosis or cell death of the leg tissues.
A calcaneal fracture is a break of the calcaneus (heel bone). Symptoms may include pain, bruising, trouble walking, and deformity of the heel. It may be associated with breaks of the hip or back.
It usually occurs when a person lands on their feet following a fall from a height or during a motor vehicle collision. Diagnosis is suspected based on symptoms and confirmed by X-rays or CT scaning.
If the bones remain normally aligned treatment may be by casting without weight bearing for around eights weeks. If the bones are not properly aligned surgery is generally required. Returning the bones to their normal position results in better outcomes. Surgery may be delayed a few days as long as the skin remained intact.
About 2% of all fractures are calcaneal fractures, however, they make up 60% of fractures of the mid foot bones. Undisplaced fractures may heal in around three months while more significant fractures can take two years. Difficulties such as arthritis and decreased range of motion of the foot may remain.
The primary symptom of camptocormia is abnormal forward bending of the torso. This bending becomes worse while walking but does not appear when the affected individual is lying down in a horizontal position. This alleviation of the condition indicates that it is a manifestation of another disease or ailment and is not due to a spine that is actually bent. This is somewhat ironic, since the medically accepted name for the condition is bent spine syndrome.
In an afflicted individual, the abnormal bending consists of an anterior flexion greater than 45 degrees. Because of this bending and the physical limitations caused by the conditions associated with the disease, it is usually impossible for an afflicted person to achieve a fully erect position. In addition, patients suffering from camptocormia often experience low back pain as a result of the condition. BSS often appears in individuals afflicted with Parkinson’s disease, muscular dystrophies, endocrine disorders, inflammatory conditions (myositis), or mitochondrial myopathies. As previously mentioned, the disease is more common in older individuals.