Important note: Paget’s disease is used synonymously for two different diseases. On the one hand, Paget’s disease is a disease from the field of gynecology and cancer. Paget’s disease from the field of gynecology is a malignant tumor (cancer) of the mammary duct in the area of the female nipple.
Synonyms in a broader sense
- Osteitis deformans
- Osteodystrophia deformans
- Paget’s disease
Paget’s disease is a localized osteopathy (= bone disease). In the context of this disease, excessive bone remodeling occurs. This remodelling ultimately leads to an abnormal bone structure.
These bone remodeling and abnormal bone structures make the affected bones susceptible to fractures (e.g. femoral neck fracture) and deformations (deformation of the bones). The clinical picture of Paget’s disease can occur from the age of 40. The average age of those affected is 60 years.
As the disease does not usually cause any particular or “typical” symptoms and is usually diagnosed rather “by chance”. At the beginning of the disease, an increased activity of the so-called osteoclasts (= cells that break down bone substances) can be detected. A distinction is made between an asymptomatic and a symptomatic course of the disease. An asymptomatic course means that the disease was diagnosed as a so-called “random finding” and that no main site of manifestation (i.e. a boil that suffers particularly severely from Paget’s disease) can be determined. Patients with a symptomatic course have pain, especially in the musculoskeletal system (especially: spinal pain).
Frequency
As mentioned above, Paget’s disease usually occurs after the age of 40. The average age is assumed to be around 60 years. The probability of contracting the disease is approximately 1 in 30,000, which means that on average there is one patient with an increased probability of Paget’s disease in every 30,000 people.
Causes
Currently, the exact cause of Paget’s disease is still unclear. A so-called slow – virus – infection of the skeleton is being discussed, which is now also regarded as apparently probable. A slow virus infection is a viral infection that progresses slowly through months or years of incubation.
The cause of Paget’s disease is considered to be a viral infection with so-called paramyxoviruses. These paramyxoviruses promote the activity of osteoclasts (cells that break down bone substances). This overactivity accelerates bone resorption, and the osteoblasts (= cells that form bone) then cause this increased bone resorption to be compensated for by attempts at repair. These repair attempts result in a hasty and uncoordinated bone formation. On closer examination of these bone attachments, it is noticeable that they have an undermineralized bone structure, which is why deformations and very quickly and easily bone fractures can occur.
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