Paget’s disease is a disease of the skeleton, also known as osteodystrophia deformans. In Paget’s disease, bone metabolism is disrupted, resulting in thickening of the bones. Those affected by Paget’s disease have a high susceptibility to fractures and deformities.
What is Paget’s disease?
Paget’s disease is also known as osteodystrophia deformans and is a disease of the bony musculoskeletal system. Due to hyperactive bone metabolism, the bones thicken over time. See growtheology for Comprehensive Guide to Chronic Venous Insufficiency.
The spine, the pelvic bones and the bones of the lower and upper extremities are particularly affected. Osteodystrophia deformans usually occurs after the age of 40. Since Paget’s disease often progresses without symptoms, it is either not discovered at all or only discovered by chance, e.g. B. X-rays are made due to other complaints.
The bones do not consist of any fixed, static structures, but are constantly being remodeled. Two different cell types in particular are involved here, the osteoclasts and the osteoblasts. The osteoblasts are used to build up bone substance, the osteoclasts break it down. Normally, build-up and breakdown are in balance, but in Paget’s disease this process is uncoordinated.
Causes
The causes of Paget’s disease are largely unknown. It is assumed that a genetic defect is responsible for the abnormal bone metabolism.
It is also currently being discussed whether an infection with a certain type of virus is a possible trigger, but no reliable results have been presented to date.
Symptoms, Ailments & Signs
90 percent of all those who become ill have no symptoms or symptoms whatsoever. In these cases, a diagnosis is usually not made at all. In ten percent of those affected by Paget’s disease, however, different problems appear. Cracks and fractures in the bones often lead to severe bone pain.
This can result in malpositions and incorrect loads, which in turn result in pain in the joints and muscles. The pain is often described as a diffuse, pulling pain in the affected areas and occurs primarily at night. In the course of the disease, deformations occur in the bones, which are also visible from the outside.
Typical are, among other things, a shortening of the shinbone, a curvature of the spine or an enlargement of the head circumference. Another possible symptom of Paget’s disease is overheating of the affected parts of the body. Due to increased blood flow, new blood vessels develop, which can dilate and become inflamed due to the high local blood pressure.
Compression of nerve tissue can lead to hearing loss, blindness, pain and functional impairments of the central nervous system. In rare cases, malignant tumors can develop, especially on the bones in the pelvis, thighs and upper arms. The first signs of this are a significant worsening of the existing symptoms and increasing deformation of the affected bones.
Diagnosis & History
Since Paget’s disease does not always show symptoms, it is difficult to diagnose. It is usually found as an incidental finding during another investigation.
If there is a suspicion of Paget’s disease, the following examinations are carried out. The enzyme alkaline phosphatase (AP) is determined with the help of a blood test, because the AP allows conclusions to be drawn about the activity of the osteoblasts. The osteoclasts are determined using a urine sample. Here, the value of the amino acid hydroxyproline is determined.
In addition, imaging methods such as B. X- ray, CT, and MRI are used to determine which bones of the skeleton are affected. Areas with increased bone turnover can be identified particularly well using bone scintigraphy.
The course of Paget’s disease depends on the stage at which the disease is and how severe it is. Since Paget’s disease does not always show symptoms, it can go undetected and the affected person can lead a completely normal life.
In other cases, bone remodeling occurs very quickly, which can result in secondary diseases. Depending on which area is affected, there may be restricted movement and pain. Thickening of the spine can squeeze the exiting nerve tracts, so that sensory disturbances and paralysis can occur. If the skull is affected in Paget’s disease, this can lead to hearing loss and blindness over time. In addition to kidney disease and heart failure, Paget’s disease can, in rare cases, result in a bone tumor.
Complications
Patients with Paget’s disease suffer from severe bone pain. Tension also occurs, which significantly reduces the quality of life of those affected. In most cases, those affected also suffer from cramps in the muscles and can no longer easily carry out usual activities or sports activities. Furthermore, the shins of those affected are significantly shortened and the patients suffer from warm extremities.
As the disease progresses, Paget’s disease also leads to hearing loss. Especially in children and young people, hearing loss can lead to severe psychological problems or depression. As a rule, treatment of Paget’s disease occurs late, since it is diagnosed late and only by chance.
Furthermore, this disease leads to disturbances of sensitivity and not infrequently to paralysis in various areas of the body. Those affected can also go blind. A causal treatment of Paget’s disease is usually not possible. Only the discomfort can be limited and reduced in some cases. However, a completely positive course of the disease does not occur. The life expectancy of the affected person is not affected or reduced by the disease.
When should you go to the doctor?
Most sufferers do not notice any symptoms of the associated thickening of the bones. As a result, there are usually no visits to the doctor. In fact, this bone disease is almost as common as osteoporosis. The onset of the disease is around the age of forty. Most sufferers never see a doctor because of the lack of symptoms.
Treatment is very rarely required for Paget’s disease. Only about ten percent of those affected have Paget’s disease with symptoms such as bone pain. The further consequences of this bone disease usually go unnoticed. However, if symptoms such as an increasing head circumference or so-called saber sheath shins appear, a doctor should be consulted.
There is a risk of joint damage and muscle strain. New blood vessels also form in the affected area. This can lead to overheating. Painful nerve compression or the development of osteosarcoma can occur. The contact person for such complaints is a specialist in internal medicine. Family doctors often do not recognize what triggers the symptoms. In most cases, Paget’s disease is discovered incidentally during a routine exam or through an MRI scan for other symptoms.
Treatment & Therapy
Since no exact cause is known for Paget’s disease, only symptomatic treatment can be given. This consists of drug therapy and physiotherapeutic measures.
Drug therapy includes pain-relieving and anti-inflammatory drugs. Furthermore, Paget’s disease is treated with so-called bisphosphonates and the hormone calcitonin.
Bisphosphonates and calcitonin have an inhibitory effect on osteoclasts, which are responsible for breaking down bone. In addition, calcium and vitamin D are administered, which are necessary for healthy bone formation.
In particularly severe cases, surgery may also be necessary. In the case of damage to the hip in particular, it can be replaced with an artificial joint replacement.
Outlook & Forecast
The prognosis for Paget’s disease is generally different. It depends heavily on the severity of the disease. In about a third of those affected, only a few and limited bone areas are affected, so that they have no symptoms. On the other hand, less favorable courses (in about two thirds of those affected) are associated with larger affected areas. The slowly progressing course of the disease eventually leads to physical limitations. But even with lesser or more pronounced physical limitations, life expectancy with Paget’s disease is usually not reduced.
Paget’s disease also increases the risk of bone cancer. For example, in about one percent of those affected, the bone cells degenerate and a so-called Paget’s sarcoma or osteosarcoma develops. This requires early treatment and surgical removal. Therefore, those affected by Paget’s disease are checked by a doctor at regular intervals. The doctors check the concentration of a certain enzyme in the blood – the so-called alkaline phosphatase (AP) – about three months after the start of treatment and then every six months. An increased AP concentration is considered a possible indicator of a bone tumor or bone metastases.
Prevention
Since the cause of Paget’s disease is not known, no preventive measures can be taken. One should attend the annual health and check-ups as a general prophylactic measure. In this way, Paget’s disease can be detected early and thus treated adequately.
You can do that yourself
People suffering from Paget’s disease always require medical treatment. This consists of drug therapy and surgical interventions. The use of medicines can be supported by various natural remedies. Anti-inflammatory agents such as arnica or belladonna, for example, have proven effective. A change in diet ensures that the body receives sufficient vitamin D and calcium, which are essential for healthy bone formation.
In many cases, Paget’s disease must be treated surgically. After such an operation, the patient must first take it easy. After a few weeks you can slowly start exercising again, provided the injury has healed sufficiently. If the patient received an artificial joint replacement as part of the operation, physiotherapeutic measures are recommended. In addition to the physiotherapy prescribed by a doctor, which usually lasts for several weeks to months, the patient can do movement exercises at home to improve handling of the new joint.
Despite everything, the causative disease continues to cause damage in other parts of the body. Therefore, the patient must consult a doctor regularly so that any bone changes can be detected early. The drug therapy must be regularly adapted to the constitution of the patient and the respective symptoms of the disease.
