Diseases

Paget's disease

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Paget's disease (Osteitis deformans, Osteodystrophia deformans) is a disease of the bones: it forms in places thickened and deformed bones. The cause of Paget's disease is unknown; genetic factors and a viral infection could play a role. Since many sufferers show no symptoms, the disease is usually discovered late or not at all. Bisphosphonates are suitable drugs for therapy. Find out more about Paget's disease here.

ICD codes for this disease: ICD codes are internationally valid medical diagnosis codes. They are found e.g. in medical reports or on incapacity certificates. M88article overviewMorbus Paget

  • description
  • symptoms
  • Causes and risk factors
  • Examinations and diagnosis
  • treatment
  • Disease course and prognosis

Paget's disease: description

The disease Paget's disease is named after the British surgeon Sir James Paget (1814 - 1899). The term is synonymous with two clinical pictures. On the one hand, "Paget's disease" signifies a bone disease and, on the other hand, a disease of the breast. In the following, however, only the Paget's disease of the bones will be treated.

Paget's disease usually begins after the age of 40 and is the second most common bone disease after osteoporosis. About one to two percent of over-40s in Western Europe are affected by the disease. It is most common in England but is very rare among Asians and Africans. It affects more men than women.

Only about 30 percent of Paget's cases are diagnosed while alive - so there are a significant number of undiagnosed patients. Only one in 25,000 inhabitants needs a therapy.

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Paget's disease: symptoms

Only ten percent of Paget's sufferers show symptoms such as local pain, while 90 percent are symptom free. The disease is therefore not diagnosed in most cases.

Paget's disease: bone, joint and muscle pain

The reduced bone stability and deformation in Paget's disease lead to painful tears and fractures in the bones. The patients complain of bone pain. In addition, the altered bone statics can cause stress levels with muscle tension and stubborn muscle pain.

As Paget's disease progresses, malocclusions develop as a result of bone fractures. Often, extreme deformations of the affected bones, which are also visible from the outside, can occur. For example, curved and thereby shortened shins ("sheath-shin") or an enlarged head circumference are typical. The affected woman often no longer fits her hat.

The adjacent joints can form so-called secondary arthrosis due to the deformities: The deformed bone overloads the joints excessively, causing them to wear out.

Paget's disease: overheating

Paget's disease stimulates bone metabolism. This creates new blood vessels that lead to increased blood flow. The vessels expand and ignite. If the bone lies directly under the skin, for example on the tibia, the increased blood flow can be felt as overheating.

Paget's disease: nerve compression

Uncontrolled bone formation in Paget's disease can also damage nerves. For example, as the size of the skull increases, nerves or even brain tissue can be compressed.

Paget's disease: deafness

If nerves are affected in the area of ​​the skull, deafness occurs in 30 to 50 percent of cases. Reason are sensory disturbances of sound and rare conduction disturbances, which are due to bony joint bridging of the ear bones or a compression of the Hörnerven.

Paget's disease: malignant tumors

Malignant tumors occur in less than one percent of Paget's disease patients with symptoms. Bones affected by the disease develop an osteosarcoma - a malignant bone tumor commonly referred to as bone cancer. Pelvic, femoral and humeral bones are mainly affected. The affected patients notice that the symptoms suddenly worsen and the deformation of the bone increases. An increase in alkaline phosphatase (a liver enzyme) can be detected in the blood.

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Paget's disease: causes and risk factors

Bones are not static structures, but are constantly being rebuilt. Two different types of cells are involved in this: osteoclasts break down bone, while osteoblasts break it down. In a healthy person, this process is coordinated - bone formation and degradation are in balance.

Paget's disease: disturbed balance

In contrast to healthy bone, this process of bone formation and degradation in Paget's disease is not uniform and coordinated, but without any system and regional differences. This is due to pathologically altered osteoclasts. The altered bone areas contain so-called giant osteoclasts - they have up to a hundred cell nuclei, while a normal osteoclast has only three to 20 cell nuclei. These osteoclasts are significantly more active than the healthy variants and therefore break down more bone substance.

In response, the body of Paget's disease patient seeks to form new bone material. For example, zones with firmer bones lie next to those with a reduced content of bone calcium salt. The bone changes its shape, looks distended, thickened or deformed or breaks faster in places with reduced lime content. The remodeling processes particularly affect bones that are heavily stressed. These include pelvis (in about two thirds of Paget's sufferers), thighs, tibia, cranial bones (by chewing activity) and lumbar spine.

It is not known exactly why these giant osteoclasts form in Paget's disease. It is thought that a genetic predisposition plays a role in this. Experts also discuss whether infection with certain viruses (slow virus infection) is involved in the development of Paget's disease. With the electron microscope, inclusions are found in the nuclei of the giant osteoclasts, which are reminiscent of viruses. These inclusions are found only in Paget osteoclasts but not in any other bone cells.

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Paget's disease: examinations and diagnosis

People with Paget's disease are not always looking for a doctor because of their condition. Often, the disease is also discovered by accident, such as changes in blood levels or in the radiograph. The right contact person for suspected Paget's disease is the specialist in internal medicine and endocrinology. It can be visited but first of all the family doctor, who may cause further investigations if necessary.

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