21/8-2017 
 
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Bone tissue diseases

Paget's disease of bone (osteitis deformans, Morbus Paget)

Definition and causes of Paget's disease of bone

Paget's disease of bone also called Morbus Paget or osteitis deformity is a rare condition in which the processes of bone turnover run amok. This leads to enlarged bones in some parts of the body, which, despite the fact that they are large, have a changed structure and therefore more likely to fracture. The disease often has no symptoms, but may in some cases show a broad picture of symptoms, see below.

The bones in healthy individuals are constantly re-modeled, i.e. degraded and rebuilt in order to maintain the structure and hence the strength of the bone. In Paget's disease of bone, there is a discrepancy between the bone-forming and bone depleting processes that leads to a changed structure and thickening of the bone due to deposition of bone material in the outer bone layer. This process also increases the number of blood vessels to the bone. The involved bones are generally the skull, back, pelvis, legs and over arms.

The disease is believed to be hereditary and affects primarily men over 50 years. The incidence of the disease varies. People from Scandinavia are rarely affected by the disease whereas in neighboring England there is an increased incidence of the disease.
 

Symptoms of osteitis deformans

It is only a small portion of people with the disease who experience real symptoms. These are usually:
  • Pain, which usually worsens at night.
  • Impaired vision or hearing (caused by a changed cranial shape that may exert pressure on cranial nerves).
  • Paralysis (In varying degrees, if the bones of the spine are affected, and therefore exerts pressure on the intervertebral nerves).
  • Changed bone form as these may not only be enlarged but also become bent.
  • Increased skin temperature over the affected bone.
  • Symptoms of congestive heart failure (as a large part of the body's blood may be diverted to the diseased bone).

Precautions and diagnosis og Paget's disease of bone

If experiencing any of the above symptoms, especially if the disease is known in the family, you should consult your doctor to find out if you suffer from Paget's disease or another bone disease.

There are many different diseases that can provide pain in the bones, and these should be excluded.

The doctor will make a clinical examination and with additional blood tests, a urine specimen and an X-ray make the diagnosis. If these tests suggest the disease, it may be necessary to make a bone scintigrafy. By injection an active substance into a vein, which is absorbed into the bones and using special X-ray examination, one can determine if there is increased bone activity in a bone. In some cases one may also make a CT scan to confirm the diagnosis.
 

Treatment of osteitis deformity

In many cases, the disease is without symptoms, and requires no treatment.

If there is pain it may be treated with so-called non-steroidal anti-inflammatory preparations such as Ibuprufen, Ibumetin and the like.

In more severe cases the disease is treated with bisphosphonates or calcitonin, which slows down the bone processes, and thus relieves pain from bone. Medical care and treatment of severe complications is made by a specialist.
 

Prognosis and complications

The course of disease is very dependent on to what extend the bone processes are influenced. In some cases the disease may cure itself.

In more severe cases there may be complications. The disease leads to increased risk of fractures, and in many cases, the disease is only discovered by chance when a person seeks medical attention and has e.g. an X-ray taken due to an accident.

As mentioned above, a changed skull shape may affect your hearing. This is due to the extra bone mass can put a pressure on the nerve to the ear as it passes the skull. In the worst cases it may lead to deafness.

Rarely, the disease can cause cancer of the bone or heart failure due to increased blood flow in the diseased bone.

In many cases, the symptoms may be alleviated, and the bone process normalized by good medical care.

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