Paget's disease

Paget’s disease (PD) (AKA osteitis deformans) named after Sir James Paget.

Paget's disease of bone (also termed ambiguously, just Paget's disease) is a chronic disorder that can result in enlarged and misshapen bones. Paget's is caused by the excessive breakdown and formation of bone, followed by disorganized bone remodeling.

see also Juvenile Paget's disease.

Paget's disease affects from 1.5 to 8.0 percent of the population, depending on age and country of residence much lower in Asia.

Paget's disease is rare in people less than 55 years of age.

Men are more commonly affected than women (3:2).

Family history of Paget’s disease is found in 15–30%of cases (accuracy is poor since most are asymptomatic).

Affinity for axial skeleton, long bones and skull. In approximate descending order of frequency: pelvis, thoracic and lumbar spine, skull, femur, tibia, fibula, and clavicle.

Its a disorder of osteoclasts (possibly virally induced) causing increased rate of bone resorption with reactive osteoblastic overproduction of new, weaker, woven bone, producing characteristic “mosaic pattern.” Initially there is a “hot” phase with elevated osteoclastic activity and increased intraosseous vas- cularity. Osteoblasts lay down a soft, nonlamellar bone. Later a “cool” phase occurs with disappearance of the vascular stroma and osteoblastic activity leaving sclerotic, radiodense, brittle bone ¹⁾ (“ivory bone”).

see Paget's disease clinical features.

see Paget's disease diagnosis.

see Paget's disease treatment.

About 1% (reported range: 1–14%) degenerate into sarcoma (osteogenic sarcoma, fibrous sarcoma, or chondrosarcoma), with the possibility of systemic (e.g. pulmonary) metastases. Malignant degeneration is much less common in the spine than in the skull or femur ²⁾.

Spinal osteosarcoma usually occurs in the lumbosacral region in males in their 40 s, sometimes arising from areas of osteoblastoma or Paget’s disease.

Basilar impression/invagination.

Spondylolisthesis.

Nocardiosis occurs primarily in patients with chronic debilitating illnesses including Paget's disease.

A rare case of Chiari malformation secondary to Paget's disease of skull was reported ³⁾.

Overall prognosis is better for Ewing sarcomas (EWS) compared to osteosarcomas (OSC). Chondroblastic OSC have the best overall survival, while OSC associated with Paget's disease of the bone has the poorest overall survival ⁴⁾.