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Paget’s disease is most often characterized by a segmental remodeling of the bone structure. Patients have disorders in the process of bone tissue formation.
The x-rays of the bones show the foci of resorption and random trabecular neoplasm occurring next to each other. The intensification of the osteolytic process causes the bone to soften and then bend. On the other hand, the increased production process leads to bone hypertrophy and thickening, which becomes brittle and prone to fractures. The blood flow through the diseased bone fragments causes local skin heat, and with severe changes it even leads to hyperkinetic circulatory failure.
Who is at risk?
Paget’s disease develops in people over the age of 40. They are mainly men – they get sick twice as often as women.
Paget’s disease of the bone is particularly common in northern European countries. It is estimated that over 5% of people suffer from this disease. people over 55 years of age. The disease often runs in families. The risk of developing the disease is 3-7 times higher than for the rest of the population. There are no precise data on the occurrence of the disease in Poland. Scholars emphasize that this condition often occurs among people historically associated with England.
The disease was first described by Sir James Paget and the disease was named after him.
Paget’s Disease – Causes
They are not exactly known. It is often said about a viral infection of people genetically predisposed to the disease. Research conducted in recent years has expanded the understanding of the causes of Paget’s disease of the bone. It has been shown that patients, especially those with the multifocal form, have a significantly increased concentration of RANKL (Receptor Activator of Nuclear Factor-kB Ligand) and, to a lesser extent, osteoprotegerin (OPG) in the blood serum. Also, elevated levels of interleukin-6 in the blood serum were found in this group of patients. It was also found that in patients, bone stromal cells show the presence of RANKL and stimulate OC activity through the RANK receptor (Receptor Activator of Nuclear Factor-kB).
Osteoclasts of sick people show an excessive reaction to RANKL and to the active metabolite of vitamin D. Moreover, at least 4 gene mutations predisposing to the development of the disease have been identified. However, the new knowledge did not change the fact that the disease was incurable. In about 70 percent. cases is asymptomatic, which makes its correct diagnosis difficult.
Paget’s Disease – Symptoms
The disease develops slowly. The diagnosis is most often made years later, when pain and significant deformities appear with secondary degenerative changes in the joints formed by the affected bones.
The changes can be multiple, but they are not generalized. In the same bone, next to each other, there are lesions and areas of healthy tissue. Characteristic changes concern the pelvis, lumbar vertebrae, skull, femurs and tibia. The bones of the hands and feet are usually not affected by the disease. Depending on the location, the lesions may have different symptoms. If the bones of the skull are involved, its circumference is enlarged and the frontal tumors are emphasized. This is a symptom called the hat too tight symptom. In such a situation, headaches or other symptoms resulting from pressure on the cranial nerves may appear. In the case of the spine, it dorsally bends, which reduces its height and pushes the abdomen forward. The long bones of the limbs usually thicken. They are saber-like bent forward and sideways.
What does the research say?
The radiological image is characterized by diffuse thickening of the affected bones, excessive bending and a typical reconstruction of the bone structure characterized by the coexistence of adjacent zones of densification and thinning of the bone structure. X-ray of the skull resembles cotton balls.
Laboratory studies show two distinctive features. The first is a significant increase in serum alkaline phosphatase activity. The second is to increase the excretion of hydroxyproline in the urine.
Paget’s Disease – Treatment?
As we already know, the disease progresses for many years. The disability is the result of bone deformities. Often there are pathological breakdowns. There is also a restriction of movements in the hip and knee joints, which is a consequence of increasing degenerative changes. Hearing loss is also typical of Paget’s disease, and very rarely blindness. In 1-2% of cases, malignant bone changes occur.
Some forms of Paget’s disease do not require treatment. If the changes are advanced, distortions and fractures will occur – patients are given bisphosphonates and calcitonin.
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