Paget's Disease

Links

Services- Rheumatology

Definition

Bone disease in which there is enlargement, softening and deformityof bone. It is thought to be due to osteoclast dysfunction. Some data suggest a link with viral persistence including: measles, respiratory syncytial virus and canine distemper virus.

When to refer

1. Unsure of diagnosis: Disease is usually asymptomatic, identified by a raised alkaline phosphatase of bone origin or typical radiological appearance. Differential diagnosis: metastatic carcinoma.
2. Uncontrolled pain (see first line management).
3. Evaluation of deafness or other cranial nerve palsies (deafness ---> ENT).
4. Intolerance of Didronel.
5. Evaluation of Paget's/OA with respect to single joint pain.
   Rheumatology Referral
   

Investigations

• FBC
• ESR/PV
• Alkaline Phospatase (bone isoenzyme)
• Calcium & Phosphate
• Xray of target area
  

First Line Management

1. Pain Control: Simple analgesia
   NSAID for 2-3 weeks therapautic trial: try to obtain evidence of definite improvement on drug or deterioration when drug stopped.
2. If deformity or other complications e.g. deafness, fractures, worsening CCF consider Etidronate (Didronel) 5mg/Kg od for 6 months (NOT the Didronel PMO regime).
   

Special Points

1. Calcitonin may be used to treat resistant cases. Other bisphosphonates besides Etidronate, e.g. Pamidronate may also be helpful.
2. If patient requires joint replacement near to or involving Pagety bone then consider pretreatment with Etidronate (Didronel) 5mg/Kg od for 6 months.

Document Information
Expiry Date: 11/04/04
Author: Kennedy Tom Dr
Organisation: Arrowe Park Hospital