Paget's disease

Important NOTE:
Paget's disease is used synonymously for two different diseases. On the one hand, Paget's disease is a disease in the field of gynecology and cancer.
Paget's gynecological disease is a malignant tumor (cancer) of the milk duct in the area of ​​the female nipple.

The following topic deals exclusively with orthopedic Paget's disease.

Synonyms in the broader sense

  • Osteitis deformans
  • Osteodystrophia deformans
  • Paget's disease

English: Paget's disease

definition

Paget's disease on the tibia

The Paget's disease is localized osteopathy (= bone disease).
In the context of this disease it comes to excessive bone remodeling. This transformation ultimately leads to an abnormal bone structure. These bone remodeling and abnormal bone structures make the affected bones vulnerable to fractures (eg femoral neck fracture) and deformities (bone deformation).

The clinical picture of Paget's disease can occur from the age of 40 years. The average age of those affected is 60 years. As the disease usually does not cause any particular or "typical" symptoms and in most cases is more likely to be diagnosed "accidentally".

At the beginning of the disease, an increased activity of the so-called osteoclasts (= cells that break down bone substances) can be detected.

One differentiates between an asymptomatic and a symptomatic course of the illness.
An asymptomatic course is understood to mean that the disease has been diagnosed as a so-called "incidental finding" and that no major manifestation site (which is a cooking that suffers particularly from Paget's disease) can be identified.
Patients with a symptomatic course have pain especially in the musculoskeletal system (especially: spinal complaints).

frequency

As mentioned above, diseases of Paget's disease usually occur from the age of 40 years. It is based on an average age, which is about 60 years.

The probability of disease is approximately 1: 30, 000, which means that on average 30, 000 people have a higher probability of Paget's disease.

causes

Currently, the exact cause of Paget's disease is still unclear.
It is discussed a so - called slow virus infection of the skeleton, which is now regarded as seemingly probable.

A slow virus infection is a viral infection which slowly progresses through months to years of incubation. The cause of Paget's disease is in particular a viral infection with so-called paramyxoviruses.

These paramoxy viruses promote the activity of osteoclasts (cells that degrade bone substances). This overactivity accelerates the bone breakdown, causing the osteoblasts (= cells that form bone) to compensate for this increased bone loss by repair attempts.
Due to these attempts at reparation, a hasty and uncoordinated bone cultivation occurs. Closer examination of these bone attachments shows that they have an under-mineralized bone structure, which can lead to deformities and very quickly and easily to bone fractures.

symptoms

As already described above, a distinction is made between an asymptomatic and a symptomatic course of the disease.
By an asymptomatic course is meant that the disease was diagnosed as a so-called "incidental finding" and no major manifestation site can be established.
Patients with a symptomatic course have pain especially in the musculoskeletal system (especially: spinal complaints).

Common to all two courses of Paget's disease is the increased activity of osteoclasts, which increasingly requires the removal of waste products from the body.
These "waste products" include, for example, amino acids (especially hydroxyproline) and can be detected in urine.

The osteoblasts on the other hand try to build up bone mass and to balance the process of osteoclasts.
This activity can be detected, for example, by a blood test / laboratory values. Due to the increased activity of osteoblasts, an increase in the enzyme "alkaline phosphatase" (= AP) occurs. The alkaline phosphatase occurs in many organs, such as the liver, so it is important to determine the "cook-specific AP" = ALP or Ostease called in the blood.

Which parts of the body are affected by Paget's disease can be different. Whether there is a main manifestation site (symptomatic form of Paget's disease) varies individually.

Possible symptoms of Paget's disease are listed below:

  • Deformations of the bones
  • Increased fracture probability (risk of fracture)
  • local pain
  • Cardiovascular stress
  • Muscle cramps due to bad stress
  • Overheating due to formation of new blood vessels
  • Varicose veins
  • Constriction of different nerve tracts (nerve compression)

Formation of malignant recurrences (= neoplasms) (rather rare: <1%), transition into an osteosarcoma.

diagnosis

Of top importance is the X-ray image, as there in the early stages of the disease osteolysis (bone dissolution) and later disease-typical coarse-striated structure of cancellous bone (= spongy scaffold fine bone beam) can be detected.

The increased bone remodeling can also be detected and demonstrated with bone scintigraphy. As a rule, these bone remodels are confirmed after the scintigraphy by means of an X-ray image.

Scintigraphy Paget's disease

One can very well recognize the strong accumulation in the right thighbone (femur) by the high activity of the bone metabolism

Increased activity of the osteoclasts, however, leads to increased degradation and consequently to the formation of waste products that must be excreted from the body.
These "waste products" include, for example, amino acids (hydroxyproline) and can be detected in urine.

As already described in the subchapter "Symptoms", the increased activity of osteoblasts can be demonstrated by the increase in the enzyme "alkaline phosphatase" (= AP), especially the "bone-specific alkaline phosphatase" ALP.
From a differential diagnosis, however, a disease of the liver must be excluded, as this can also be held responsible for the increase in AP.

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In cases where the diagnosis remains unclear after all examination methods, a bone biopsy (extraction of a tissue sample) can be performed.

Furthermore, the Paget's disease has differential diagnostic
still be distinguished from bone metastases and other bone diseases such as osteomalacia (= increased soft tissue and bending tendency of the bones due to deficient incorporation of minerals into the osteoid).

General

An involvement of the cranial bones is usually initially due to a deformation or increase in size of the skull, as it is visible at the head due to the lack of fat and connective tissue quite early. For example, patients report that hats or helmets no longer fit properly.

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roentgen

If it is suspected that the skull bones are affected by Paget's disease, an X-ray of the skull is usually made first.
In the early stage of the disease heartworm-shaped oval lucencies are recognizable, which indicate an onset of bone loss ( osteolysis ). Later, due to the "repair attempts" of the bone-building cells ( osteoblasts ) an excessive production of bone substance is added, which shows in the X-ray image by a widening of the skull bones with irregular bone structure ("cotton skull"). The changes usually begin in the area of ​​the frontal bone and the occipital bone and may later spread to the temporal bone ( Osteolysis circumscripta cranii ). Even fractures of the skull, which can occur as a result of bone degradation in the context of the disease, can be visible in the radiograph.

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scintigraphy

However, an X-ray of the skull should not be done by default in every patient with Paget's disease, but only if there are indications that the skull is affected.

This is also the case, for example, except in the case of symptoms when scintigraphy is performed during the initial diagnosis, during which an accumulation of the radiolabelled substance in the region of the head is noticeable. This can be an indication of increased metabolic activity in the cranial bone, which is typical of an infestation with Paget's disease.

CT and MRI

A CT or MRI scan can be performed to rule out other conditions such as osteoporosis or cancer metastases as conceivable causes. Also, to clarify possible complications, the sectional imaging by CT or MRI makes sense. In case of involvement of the skull, this is particularly recommended, since the deformation of the bones brain tissue or nerves can be compressed.

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Neurological examination

Therefore, in the case of Paget's disease of the skull, a neurological examination as well as a hearing examination should be carried out, as in 30 to 50 percent of the cases a hearing loss due to a narrowing of the auditory nerve or damage to the auditory ossicles occurs. Damage to the optic nerve or other cranial nerves is less common, but should be ruled out anyway.

bone biopsy

Relatively rarely, a tissue sample of the bone (bone biopsy) must be taken. This diagnostic method is only necessary if there is still a suspicion of a bone metastasis or a so-called Paget's sarcoma after CT and MRI examinations. The latter is a malignant bone tumor (osteosarcoma) that develops from degenerated osteoblasts in one percent of patients as a result of Paget's disease.

laboratory examination

As with all other forms of the disease, an increase of the enzyme alkaline phosphatase (AP) or the bone-specific alkaline phosphatase (ALP) in the blood and an increase of hydroxyproline in the urine can also be detected in the Paget's disease of the skull. These laboratory values ​​are an important part of the diagnostics and can be used for the follow-up of a therapy.

Therapy of Mobus Paget

The ultimate goal of the treatment of Paget's disease is the elimination of pain and, moreover, the arrest of the progressive deformities (bone deformation) and the inhibition of osteoclasts.

Not always a therapy of Paget's disease has to be carried out. A patient with an asymptomatic course of Paget's disease, in which no deformations could be detected, usually requires no therapy.

According to Ziegler, the indication for the treatment of Paget's disease is differentiated within three different stages

  • Absolute indication for therapy
    • Strong conversion activities with an AP> 600 IU / l
    • bone pain
    • Deformities (bone deformation)
    • High fracture risk (risk of fracture)
    • Failure of adjacent nerve structures
    • Infestation of the skull base
  • Relative indication for therapy
    • Mean disease activity
    • warm feeling
    • Infestation of the skullcap
    • Preparing for surgical therapies
    • Heart failure (heart failure)
  • no reliable indication for therapy
    • Patient is older than 75 years
    • no symptoms
    • lower conversion activities
    • only a few bones are affected

You can individually record the following types of therapy for Paget's disease:

  1. Pain relief by anti-inflammatory drugs and analgeitka
  2. Kalcitonin therapy (self-injection of hormone, nasal spray) to reduce osteoclast activity
    100 E for one month, then 300 E for another 6 months
  3. Bisphosphonates (eg Fosamax -> not approved for the treatment of Paget's disease) to inhibit increased bone loss
  4. Pain therapy and / or physiotherapy to support the drug therapy
  5. Surgical therapy (joint replacement surgery, conversion osteomies)

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Bisphosphonates for therapy

The following bisphosphonates are currently approved for the treatment of Paget's disease:

  • Etidronate 400 mg / day orally 6 months
  • Pamidronate 30 mg / week iv over 4 hours 6 weeks
  • Tiludronate 400 mg / day orally 3 months
  • Risedronate 30 mg / day orally 2 months
  • Zoledronic acid 5 mg short infusion 15 minutes once

The choice of the form of therapy and therefore in particular the medical therapy of Paget's disease must always be determined individually with regard to the substances administered, the dosage and the duration of the therapy. Combinations of the various therapeutic measures are also conceivable.

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