Fibrous ankylosis (also known as false ankylosis [1] ) is a condition that affects fibrous connective tissue causing a limited range of movement. [1]
Most causes occurs due to physical trauma. Other cases can be attributed to the contraction of diseases such as tubercular arthritis, (arthritis developed after contracting tuberculosis), or septic arthritis. Surgery, arthritis, rheumatoid arthritis, immobilization are also cases of this condition. [1] Fibrous ankylosis was thought to be a precursor progress into bony ankylosis, in which osseous bone tissue fuses the affected joint, causing a greater reduction of mobility. [2]
The symptoms of fibrous ankylosis include:
For those who have contracted this condition via a viral disease such as tuberculosis, or a disease such as arthritis, inflammation is also reported. [1]
Fibrous ankylosis can occur in the jawbone, limbs, and any other joints. Depending on which joint is affected, serious complications can occur.
For example, there is ankylosis spondylitis which is ankylosis that affects the spine. It can lead to the development of enthesitis, which is inflammation of the entheses (the site in which tendons/ligaments are inserted into bone). A severe case can lead the patient to become paralyzed. In addition, complications due to the inflammation, can lead to problems with cardiovascular (heart), pulmonary (lung), or even neurological (brain) health. Some of the secondary conditions are reported include aortitis (inflammation of the aortic wall (heart wall)) and breathing dysfunction. [3]
There is also temporomandibular joint (TMJ) ankylosis, a rare condition in which the condyle of the mandible (jawbone) and the maniable fossa of the temporal bone fuses together. This condition can occur bilateral (on both sides of the body) and unilateral (one side of the body). The patient will have reduced ability to eat, speak properly, and maintain proper dental hygiene furthering causing starvation and malnutrition.
One of the most prominent causes of Fibrous anklylosis is physical trauma. [4] After experiencing a severe physical injury, the body will try to recover by activating it's mensenchymal stromal cells (MSCs), a type of stem cells that can differentiate into different cells such as osteoblasts, chondrocytes, and other factors such as bone matrix/growth factors that are used to stimulate growth/self mending. However, this process is altered due to the changes within the MSCs causing improper restoration of the affected part. Due to this, it allowed blood to travel outside from the initial wound to form a hematoma which will then cause the joints to stiffen as a result. [5]
Other causes include systemic diseases such as tubercular arthritis (a form of arthritis that developed due to being infected by tuberculosis), septic arthritis, and non-infective inflammatory arthritis. When a patient contracts a disease such as tuberculosis or a bacterial infection, it causes damage to the blood vessels. The MSCs are also activated to add growth factors such as Fibroblast growth factor (FGF) or VEGF to induce angiogenesis (growth of new blood vessels). During this time, as the vessels are healing, blood will leave from outside the capillaries causing a hematoma. [5]
Hematomas can also form if surgery is done incorrectly or the wound does not properly heal after treatment. [5]
Sometimes even having certain genes or alleles, put a person at risk to have a certain type of ankylosis. For example, for ankylosing spondylitis, the gene HLA allele B27 is an important factor in having this disease. This allele is a human leukocyte antigen whose role is to help with the immune system by helping with identifying foreign (harmful) and native (our body's) cells. Those who have this gene have an increased risk of having ankylosing spondylitis. [6]
Prolong immobilization is also a cause of this condition, since blood is not circulating causing it to collect within the capillaries and move from the vessels due to the pileup causing the formation of hematomas.
The formation of the disease begins after a person would develop an intra-articular hematoma due to either physical trauma or by infection. An intra-articular hematoma is a type of hematoma that is fixed within a joint. The formation of an intra-articular hematoma is a gradual process that can occurs within months or years. The creation of a hematoma begins when the body begins to recover damage done to the blood vessels after a serious injury or a viral infection. The mesenchymal stromal cells (MSCs) will begin to differentiate to change into different forms to heal the body. For an injury, it could be an osteoblast, to promote bone health by adding new bone matrix. For an infection, it could be trying to repair the blood capillaries by inducing growth factors. At the site of either infection or injury, the MSCs will incorrectly target the area due to a wrong signal or another issue. The area will be treated, but it will redirect blood outside of the capillaries to form a hematoma due to incorrectly targeting the site or not healing properly. Generally, the hematoma will be gone after exercise or other methods, but it persists then fibrous anklyosis can form. [5]
The direct cause of the MSCs's improper function is still unknown. However, with recent studies with animal models (sheep), it is believed that other factors such as growth factors and mechanics pay a role in developing hematomas though their effect on gene expression. [5] For example, there is hypoxia which is a state of reduced oxygen. It is caused by a gene called hypoxia inducing factor 1α or HIF-1α. Low expression of this gene increases the severity of the hematomas. It also has many other effects such the increased promotion of osteogenesis (the creation of Osteoblasts), but the suppression of adipogenesis of MSCs, (important for differation of fat cells into other cells such as osteoblasts, myocytes, and chondrocytes). Growth factors are used to regulate cellular function and help with the differentiation of mesenchymal stromal cells through regulation. It was thought that the increased presence of some growth factors such as vascular endothelial growth factor (helps with bone regeneration and promote MSCs differentiation) or bone morphogenetic protein 7 (helps with bone fracture healing, enhancing differentiations) helps to differentiate cells. The type of ankylosis that is developed is influenced by the regulation of the MSCs. Depending on what type is sent as well as the amount makes a different in repairing injuries points. Mechanical forces also played a part inducing the type of differentiation of the MSCs, but studies on this subject isn't very clear. If the fibrous ankylosis's damage on affected joint continued, then the disease would progress to bony ankylosis, where the affected joint becomes ossified or fibrotic. The joint will then become fused and the patient will experience limited movement within it. [5]
The patient's history, physical examinations, and other types of tests are used to figure out their condition. Imaging techniques are used to locate and track inflammation and misaligned bones.
One such type of imaging techinque used are projectional radiography otherwise known as x ray exams. Using x rays, they would take 2d images of the person's structure to figure out if any of the joints has. It is good to use initially to see what is wrong with the joints. However, x rays are limited, and cannot see inflammation or soft tissue. [7]
Another type test is used is CT scanning. It is used to monitor the condition of the joints, and pinpoint important information. CT Scans are used to capture the state of the affected joints, to check whether or not they are fused. It will also provide an image of where inflammation is in the joints. However, it cannot provide information on inflammation of the etheses, and it has a harder time detecting inflammation in the soft tissues. [7]
An MRI may also be used to determine results. In comparison to the CT scan, it is more powerful as it has a greater scope of range. It can detect the soft tissue, and inflammation in the spine, joints, and other locations in great depth. [7]
After collecting this information, the doctors will then plan their methods on how to treat their patients.
Early detection is necessary to avoid the major complications of this disease. Those with forms of inflammatory and autoimmune arthritis can get treatment such as NSAIDS (Aspirin, ibuprofen, Lodine, or Naproxen) to reduce inflammation and pain. [8] Another treatment that is used are disease-modifying Antirheumatic drugs to slow down the further progression of the arthritis before it becomes ankylosis. Drugs such as Methotrexate, Hydroxychloroquine or Sulfasalazine are recommended to treat rheumatoid arthritis. [8] If symptoms manage to process to bony ankylosis, then non-surgical treatment is the most optimal choice for treating this disease. Non surgical treatments involve taking medicine, exercise to strengthen joints, and other methods. [8]
For more serious conditions like temporomandibular anklyosis, surgery is recommended. Condylectomy, is a type of surgery that involves removing the condyle, and readjusting the jawbone/upper month with pins/wires to straighten out the structure. [9] In addition, another type of Condylectomy surgery, called high condylectomy surgery can be performed. Instead of removing the condyle, it is reshaped for better balance within the mouth. Arthroplasty, (surgery to replace the remove/replace bones to improve structure) is also suggested when the ankylosis has become advanced and more repair is needed for the damaged part. [10] There was also brisement force surgery. It involves forcing the mouth open using a mouth gag to mobilize the jaw. Then, the patient is given instructions to exercise their jaw in order to keep with its consistency. It is recommended to use this as soon as the condition (TMJ ankylosis) is recognized. [11]
Fibrous ankylosis's prognosis differs depending on the joint affected.
Tempormandiubular joint ankylosis (TMJ ankylosis) is a rare condition affects the jawbone causing reduced movement in that area. Children around the age demographics of 4 -14 years of age catch this condition. [12] However, it has a high mortality rate of 90% among children. This is due to the patients suffering from secondary conditions such as malnutrition, periodontal disease, and muscle atrophy due to this condition. [13] It must be treated with surgery as soon as possible. Even with successful surgery, it has a 50% chance of reoccurring.
On the other hand, those who suffer from Ankylosis spondylitis have not perished due to this condition, but due to secondary complications of it. As a result, conditions of the heart, lung, or brain, it increases the mortality rate for those patients. In addition, smoking and other bad habits increase the mortality rate as well. [14]
The epidemiology, (incidence rate) changes depending on the joints affected.
Tempormandiubular joint ankylosis (TMJ ankylosis) which is ankylosis of the mandible, affects children around the ages of 4-14. It also has a tendency to affect women more than men. For children, the most common cause of this condition is due to physical trauma and infection. [12]
Case of Ankylosis spondylitis tend to develop between the ages of 30-45. It has a tendency to affect men more than women. It also seems to affect those of a low socioeconomic status. [15] It also has a tendency to appear during youth affecting 5 % of children. The direct cause of ankylosis spondylitis is unknown, but those who have the allele HLA-B27, have a greater chance in getting this condition. [15]
Further research in this area of study focus on developing new methods for better treatment for those who are suffering from different forms of ankylosis. There are new studies featuring methods to improve grafts for TMJ ankylosis by studying current models. [16] Fat grafting is also being studied for their use, and how it used as a definitive treatment for treating adults with TMJ. [17] There is also research via animal studies to preserve the foundation of the mandibular condyle to reduce risk of ankylosis. [18]
There is also research focusing on isolating and identifying certain genes that cause the condition to form. However, it is a more complex matter since more factors are involved in the process. A recent study identified more than 100 loci to inherit ankylosis spondylitis, but it explains less than 30% of heritability. More factors including presentation and antigen processing need to be studied to understand more. [19]
Temporomandibular joint dysfunction is an umbrella term covering pain and dysfunction of the muscles of mastication and the temporomandibular joints. The most important feature is pain, followed by restricted mandibular movement, and noises from the temporomandibular joints (TMJ) during jaw movement. Although TMD is not life-threatening, it can be detrimental to quality of life; this is because the symptoms can become chronic and difficult to manage.
In anatomy, the temporomandibular joints (TMJ) are the two joints connecting the jawbone to the skull. It is a bilateral synovial articulation between the temporal bone of the skull above and the condylar process of mandible below; it is from these bones that its name is derived. The joints are unique in their bilateral function, being connected via the mandible.
Rheumatology is a branch of medicine devoted to the diagnosis and management of disorders whose common feature is inflammation in the bones, muscles, joints, and internal organs. Rheumatology covers more than 100 different complex diseases, collectively known as rheumatic diseases, which includes many forms of arthritis as well as lupus and Sjögren's syndrome. Doctors who have undergone formal training in rheumatology are called rheumatologists.
Trismus is a condition of restricted opening of the mouth. The term was initially used in the setting of tetanus. Trismus may be caused by spasm of the muscles of mastication or a variety of other causes. Temporary trismus occurs much more frequently than permanent trismus. It is known to interfere with eating, speaking, and maintaining proper oral hygiene. This interference, specifically with an inability to swallow properly, results in an increased risk of aspiration. In some instances, trismus presents with altered facial appearance. The condition may be distressing and painful. Examination and treatments requiring access to the oral cavity can be limited, or in some cases impossible, due to the nature of the condition itself.
Ankylosing spondylitis (AS) is a type of arthritis from the disease spectrum of axial spondyloarthritis. It is characterized by long-term inflammation of the joints of the spine, typically where the spine joins the pelvis. With AS, eye and bowel problems—as well as back pain—may occur. Joint mobility in the affected areas sometimes worsens over time. Ankylosing spondylitis is believed to involve a combination of genetic and environmental factors. More than 90% of people affected in the UK have a specific human leukocyte antigen known as the HLA-B27 antigen. The underlying mechanism is believed to be autoimmune or autoinflammatory. Diagnosis is based on 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.
Spondyloarthritis (SpA), also known as spondyloarthropathy, is a collection of clinical syndromes that are connected by genetic predisposition and clinical manifestations. The best-known clinical subtypes are enteropathic arthritis (EA), psoriatic arthritis (PsA), ankylosing spondylitis (AS), and reactive arthritis (ReA). Spondyloarthritis typically presents with inflammatory back pain and asymmetrical arthritis, primarily affecting the lower limbs, and enthesitis, inflammation at bone-adhering ligaments, tendons, or joint capsules.
Ankylosis is a stiffness of a joint due to abnormal adhesion and rigidity of the bones of the joint, which may be the result of injury or disease. The rigidity may be complete or partial and may be due to inflammation of the tendinous or muscular structures outside the joint or of the tissues of the joint itself.
Avascular necrosis (AVN), also called osteonecrosis or bone infarction, is death of bone tissue due to interruption of the blood supply. Early on, there may be no symptoms. Gradually joint pain may develop, which may limit the person's ability to move. Complications may include collapse of the bone or nearby joint surface.
Hemifacial microsomia (HFM) is a congenital disorder that affects the development of the lower half of the face, most commonly the ears, the mouth and the mandible. It usually occurs on one side of the face, but both sides are sometimes affected. If severe, it may result in difficulties in breathing due to obstruction of the trachea—sometimes even requiring a tracheotomy. With an incidence in the range of 1:3500 to 1:4500, it is the second most common birth defect of the face, after cleft lip and cleft palate. HFM shares many similarities with Treacher Collins syndrome.
Spondylitis is an inflammation of the vertebrae. It is a form of spondylopathy. In many cases, spondylitis involves one or more vertebral joints, as well, which itself is called spondylarthritis.
Cherubism is a rare genetic disorder that causes prominence in the lower portion in the face. The name is derived from the temporary chubby-cheeked resemblance to putti, the chubby-faced infants featured in Renaissance paintings, which were often mistakenly described as cherubs.
Sacroiliitis is inflammation within the sacroiliac joint. It is a feature of spondyloarthropathies, such as axial spondyloarthritis, psoriatic arthritis, reactive arthritis or arthritis related to inflammatory bowel diseases, including ulcerative colitis or Crohn's disease. It is also the most common presentation of arthritis from brucellosis.
Enthesitis is inflammation of the entheses, the sites where tendons, ligaments and joint capsules attach to bones.
Childhood arthritis is an umbrella term used to describe any rheumatic disease or chronic arthritis-related condition which affects individuals under the age of 16. There are several subtypes that differentiate themselves via prognosis, complications, and treatments. Most types are autoimmune disorders, where an individual's immune system may attack its own healthy tissues and cells.
Arthritis mutilans is a rare medical condition involving severe inflammation damaging the joints of the hands and feet, and resulting in deformation and problems with moving the affected areas; it can also affect the spine. As an uncommon arthropathy, arthritis mutilans was originally described as affecting the hands, feet, fingers, and/or toes, but can refer in general to severe derangement of any joint damaged by arthropathy. First described in modern medical literature by Marie and Leri in 1913, in the hands, arthritis mutilans is also known as opera glass hand, or chronic absorptive arthritis. Sometimes there is foot involvement in which toes shorten and on which painful calluses develop in a condition known as opera glass foot, or pied en lorgnette.
Enteropathic arthropathy commonly referred to as enteropathic arthritis, is a type of arthritis linked to Crohn's disease, ulcerative colitis, and chronic inflammatory bowel diseases.
Condylar resorption, also called idiopathic condylar resorption, ICR, and condylysis, is a temporomandibular joint disorder in which one or both of the mandibular condyles are broken down in a bone resorption process. This disorder is nine times more likely to be present in females than males, and is more common among teenagers.
Tooth ankylosis refers to a fusion between a tooth and underlying bony support tissues. In some species, this is a normal process that occurs during the formation or maintenance of the dentition. By contrast, in humans tooth ankylosis is pathological, whereby a fusion between alveolar bone and the cementum of a tooth occurs.
Jaccoud arthropathy (JA), is a chronic non-erosive reversible joint disorder that may occur after repeated bouts of arthritis. It is caused by inflammation of the joint capsule and subsequent fibrotic retraction, causing ulnar deviation of the fingers, through metacarpophalangeal joint (MCP) subluxation, primarily of the ring and little-finger. Joints in the feet, knees and shoulders may also get affected. It is commonly associated with systemic lupus erythematosus (SLE), and occurs in roughly 5% of all cases.
Axial spondyloarthritis is a chronic, immune-mediated disease predominantly affecting the axial skeleton. The term itself is an umbrella term characterizing a diverse disease family united by shared clinical and genetic features, such as the involvement of the axial skeleton. The 2009 introduced term axial spondyloarthritis is a preferred term nowadays and substitutes the old term ankylosing spondylitis.
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