pathology: paget’s disease

yogabook / pathologie / Paget’s disease

Paget’s disease (ostitis deformans, osteodystrophia deformans, Paget’s syndrome)

Definition of

insidiously beginning, slowly progressing bone alteration disease of the skeletal system with one or more areas of increased and disorganized bone remodelling and subsequent severe thickening and curvature of the large tubular bones and the skull. Beginning with increased osteoclast activity, then increased formation, alternating phases of excessive osteoclast and osteoblast activity. The bone resorption is usually subcortical, whereas the subsequent bone formation takes place on the periosteum, resulting in a bone with a disturbed architecture and limited load-bearing capacity. Men are more frequently affected, onset usually after the age of 55, familial clustering, occurrence mainly in Europe, America, New Zealand, Australia among people of European origin; most common in England, very rare in Asia and Africa. In Western Europe, 5% of men and 8% of women are affected. Most patients are neither clinically conspicuous nor in need of treatment, mostly incidental findings. Only 30% are diagnosed during their lifetime. Only 1:25,000 people in the population need treatment for Paget’s disease. Immunological evidence of viral involvement is found in the altered bone areas. People from the Stone Age and dinosaurs (Dysalotosaurus) already had Paget’s disease. Probably the oldest indirect evidence of a viral infection!

ICD M88

Cause

  1. Unknown, genetic dispositions that also cause Paget-like syndromes; probably autoimmunological (autoaggressive) or a slow-virus disease (paramyxoviruses)

Diagnosis

  1. Laboratory: AP increased, hypercalcemia, hypercalcuria
  2. If necessary: X-ray, MRI, CT, scintigraphy
  3. Biopsy
  4. Giant osteoclases with up to 100 instead of 3 – 20 cell nuclei

Symptoms

  1. Asymptomatic in 1/3 of cases (incidental finding on X-ray). In the early stages: diffuse pain in the musculoskeletal system: burning, stinging with varying intensity and localization, preferably in the spine, os sacrum, tibia; occasionally with local overheating of the skin
  2. Early stage: diffuse pain in the musculoskeletal system: burning, stinging with varying intensity and localization, preferably in the spine, os sacrum, tibia; occasionally with local overheating
  3. Deformities of the bones and possibly joint problems:
  4. in the cranial region: enlargement of the cranial vault due to apositional growth (corticalis grows), hearing loss up to deafness, visual impairment up to blindness, more frequently: headache; often pain in the area of the spine due to the greater weight of the head
  5. in the lumbosacral spine: nerve root compression symptoms such as ischialgia, lumbalgia, as the intervertebral foramen become narrower(pressure on the spinal nerves)
  6. Pelvis: loses a large part of its cancellous bone structure, sintering (clumping) together.
  7. Highlight, radiographic: cardiac shape of the pelvis: the pelvis successively yields to the pressure of the spine from above or the femur from below, which leads to problems in the SI joint
  8. tibia: Highlight: remodeling processes, deformities -> vertebral sheath tibia(highlight)
  9. Fractures (spontaneous or stress fractures), arthrosis, joint degeneration, nerve root compression syndrome
  10. Varicosis, vascular neoplasms

Complications

  1. Secondary osteoarthritis
  2. Kidney stones due to released calcium, but no hypercalcemia
  3. Degeneration into osteosarcoma (1.5% of affected bones, mainly pelvis, femur, humerus)
  4. Heart failure due to increased cardiovascular stress

Therapy

  1. Bisphosphonates, calcitonin (prevent bone resorption and thus deformation when taken in good time), plus vitamin D and calcium
  2. If necessary, relief of affected bones and surgical stabilization
  3. KG
  4. For pain: NSAIDs
  5. If necessary: TEP