PAGET'S DISEASE - A BRIEF OUTLINE

Facts | Causes | Common Symptoms | Diagnosis | Treatment

FACTS

• A common, chronic bone disorder that may be found by chance and often causes no problems
  
• Occurs rarely in those under 50 but by the age of 80 about 8% of men and 5% of women will be affected. Over the past decade prevalence of the disease has decreased in the UK
  
• Either single or multiple bones may be affected with the commonest sites arising in the spine, skull, pelvis, hip and shin
  
• Symptoms include pain, possible deformity and fracture
  
• Treatment with drugs will control the disease
  

CAUSES

• Bone is constantly renewed by cells called osteoclasts and osteoblasts
  
• Osteoclasts break down bone at a faster rate than normal and osteoblasts make new bone very rapidly
  
• Size of bone increases but structure will be irregular and weakened
  
• Genetic factors play an important role in the development of the disease. Environmental factors, including exposure to certain viruses may also influence its development

COMMON SYMTOMS

• Pain in the affected bone is the most notable symptom. It is not relieved by rest or exercise and may be most severe at night
  
• A fracture after minor injury could be suggestive of Paget’s disease
  
• Deformity may develop, depending on the site and severity of the disease eg if the shin is involved this may become bowed and if the skull is affected this can become enlarged

DIAGNOSIS

• Identified by an x ray
  
• Isotope bone scan may be performed
  
• Simple blood test may show an increased alkaline phosphatase level that could indicate Paget’s disease
  

TREATMENT

In many cases Paget’s disease is found by chance, does not cause any symptoms and requires no treatment.

Treatment may be recommended if there is pain or bone deformity and may also be advised if the presence of the disease could cause future problems.

BISPHOSPHONATES

• A group of drugs that reduce abnormal bone turnover leading to a more normal bone structure.
  
• Control existing disease and help to reduce pain caused by active disease.
  
• Long lasting effect, therefore a course of treatment may last for months or years.
  
• Given as tablets or directly into the bloodstream (intravenous).

Oral Preparations

Risedronate (Actonel): one tablet daily for 2 months. Tiludronate (Skelid): two tablets daily for 3 months. Etidronate (Didronel): two tablets daily for 6 months.

Further treatment may be given after 6 m.

These drugs need to be taken whilst fasting and can cause side effects such as indigestion, stomach discomfort and rarely joint pains and skin irritation.

Prior to starting treatment it is essential to read instructions on how to take the tablets

Important to notify GP should side effects occur

Intravenous Preparations

Zoledronate (Aclasta): single dose into bloodstream, effective for at least 2 years. Pamidronate (Aredia): several doses into bloodstream, repeated when necessary.

These drugs may cause “flu like” symptoms 24-48 hours after treatment. Very rarely associated with osteonecrosis of the jaw (bone destruction of the jaw).

Treatment will be given as outpatient in hospital.

Important to notify GP or hospital should lasting side effects occur.

OTHER MEDICATION

• Calcitonin given by injection, now rarely used.
  
• Additional painkillers may also be required.

SURGERY

• As there is a relationship between Paget’s disease and osteoarthritis joint replacement surgery may be required
  
• Usually required to treat fractures should they occur
  
• Occasionally required to correct marked deformity

Facts | Causes | Common Symptoms | Diagnosis | Treatment