Paget's disease

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I- Definition :

2th osteopathy after osteoporosis, characterized by a deformation : is a osteitis deformans – Localized may be diffuse, elevated bone resorption

Disruption of bone anabolism : anarchic bone formation and accelerated

The bone is abnormal as

II- Epidemiology :

Incidence 3-3.7%, adult > 40years, H=F, frq en anglo-saxon, rare in Asia and Scandinavia

III- Exam :

– Lucky find : long asymptomatic +++
– bone deformity DLR (pool, rachis, Mbre) : does not respond well to the constraints
– Reaching periarticular : when the bone segment is near the joint
– Reaching nerve compression : hearing, ocular, radicular
– Heart failure : cpc rare (diffuse deformation) (can reveal disease) Ice heart this is secondary to neovascularization has
– Transformation sarcomateuse : <1% exceeding regulatory mechanisms due to the acceleration of the multiplication (DLR permanent unresponsive to usual painkillers)

IV- Diagnostic :

1- radiological :

May be enough because the signs are specific :
– bone radiography : bone augmentation with bone deformity. Cracks and bone loss. uncontrolled disposal of trabecular. Enables tracking
– scintigraphy : for assessment of the lesions, and search for all the affected seats. Allows for the differential diagnosis with the degenerative diseases. Enables tracking (hyperfixation 4 affected area)
– TDM et IRM : Suspicion if cpc

2- Organic :

– Phosphatases alcalines : high
– VS : normal or slightly elevated. → If high suspicion of sarcomatous transformation

3- bone biopsy :

If doubtful diagnosis or sarcomatous transformation, but it is not required in current practice due to the specificity of radiological signs

V- Treatment :

Calm DLR and limit bone deformities :

– Bisphosphonate : TRT choice, perfusion de Pamidronate Adréa® 60mg IV/j (2-3h) two days to repeat as reactivation of the disease. Window if rémmission

This drug can slow the ostéoclastose → decreased runaway reaction ostéoblastose. It also has an analgesic effect

– analgesics, AINS, physiotherapy

Courses of Dr. Boudersa – Faculty of Constantine