Treatment for my patient

The Paget's Association supports all those involved in the care of patients with Paget’s disease and members of the Charity include a growing number of health professionals. We hope you find the information provided helpful. Please consider registering to access the resources in the Member's Area.

Download an information sheet to give your patient: Download pdf here

Paget's Association Centres of Excellence for care of patients and research: You can find the list of centres by following this link

Who needs treatment for Paget’s disease?

In many cases, Paget’s disease is found by chance, does not cause any symptoms and does not require any treatment. Treatment may be recommended if the affected bones are painful and/or if Paget's disease affects a site that might be expected to cause complications such as the skull or a weight bearing bone. Referral to a consultant is recommended in order to carry out a full assessment and decide management. Assessment usually includes an isotope bone scan and family history. Check with your local hospital as those with expertise in Paget's Disease are often but not always in the rheumatology or endocrinology department. Some areas have Metabolic Bone centres or clinics.

Treatment with Bisphosphonates

When treatment is required, Bisphosphonates are used and Zoledronate is often the drug of choice as it acts quickly and can be effective for many years.

Bisphosphonates are usually effective at reducing pain in active Paget's Disease, although the amount of pain relief will vary between individuals, particularly if there is significant osteoarthritis.

Alkaline Phosphatase (ALP) is an indicator of disease activity and should be measured prior to starting treatment and repeated 2-3 months later, when it should have decreased. If the level does not fall to within the normal range and symptoms, related to active disease, are still present, further treatment may be required. It should be noted that Paget's disease can be active and yet the ALP may remain within the normal range, particularly if the area involved is small.

Zoledronate

5 mg of Zoledronate intravenously over 15-30 minutes (out-patient)

• One dose may be effective for many years therefore often patients may not require further treatment

Outline of side-effects:

  • Flu like symptoms can occur 24 - 48 hours after infusion and treatment can be associated with bone and joint pains.
  • Rarely - inflammation of the eyes.
  • Zoledronate may cause Hypercalcemia. As a precaution vitamin D (25-hydroxy vitamin D) levels are measured and calcium and vitamin D supplements may be given prior to treatment.
  • Infusion with Zoledronate can occasionally be associated with atrial fibrillation but a causal link for this has not been established.
  • Osteonecrosis of the jaw has been rarely reported. As a precaution, it is wise to complete any extensive dental surgery/treatment prior to treatment.
  • Avoid in renal disease and pregnancy.

Reference: Ian R Reid, Kenneth Lyles, Guoqin Su, Jacques P Brown, John P Walsh, Javier del Pino-Montes, Paul D Miller, William D Fraser, Susan Cafoncelli, Christina Bucci-Rechtweg, David J Hosking (2012) A Single Infusion of Zoledronic Acid Produces Sustained Remissions in Paget Disease: Data to 6.5 Years. Journal of Bone Mineral Research. Sep; 26(9):2261-70.

Pamidronate

• Pamidronate intravenously over 2-4 hours

• Dosage (60 – 90 mg) and frequency will depend on severity of symptoms

  • Outline of side-effects:
  • Flu like symptoms can occur 24 - 48 hours after infusion and treatment can be associated with bone and joint pains.
    Rarely - inflammation of the eyes.
    Pamidronate may cause Hypercalcemia. As a precaution vitamin D (25-hydroxy vitamin D) levels are measured and calcium and vitamin D supplements may be given prior to treatment.
    Infusion with bisphosphonates can occasionally be associated with atrial fibrillation but a causal link for this has not been established.
    Osteonecrosis of the jaw has been rarely reported. As a precaution, it is wise to complete any extensive dental surgery/treatment prior to treatment.
    Avoid in renal disease and pregnancy.
Risedronate

One 30 mg tablet daily for 2 months

• If necessary a further course may be given after 6 months

  • • Taken on an empty stomach with a full glass of water, first thing in the morning. Wait at least 30 minutes before having food, drink and other medication and advise the patient not to lie down after taking the tablet.

    Side effects include heartburn and abdominal discomfort. Occasionally joint and bone pains. They can give rise to skin rashes and inflammation of the eyes, but these are rare occurrences. Osteonecrosis of the jaw has been reported with risedronate but again, very rarely. As a precaution, it is wise to complete any extensive dental surgery/treatment prior to treatment. Avoid in renal disease and pregnancy.

Case Histories

Considerations in Diagnosis & Management of Paget’s Disease

Dr Clive Kelly is a Consultant Rheumatologist in Gateshead. Speaking at our Paget's Information Day in Middlesbrough, in 2014, Dr Kelly discussed three case histories of patients with Paget’s disease. None were straight forward and so they show the difficulties that can be encountered when diagnosing and treating Paget’s disease. Dr Kelly has kindly provided summaries of the individual cases below.

Experienced clinicians often look upon Paget’s disease as an easy diagnosis to make and a condition that is readily treated. Although treatment of the condition can be much easier these days, there are however, still some pitfalls in both the diagnosis and management of the condition. These are illustrated by the three patient summaries below which highlight some of these issues.

Case 1

A 76 year old gentleman had suffered with back pain for three months, associated with sleep disturbance and difficulty in micturition. Examination showed tenderness in the lower back on deep palpation, although the spinal architecture was normal. His blood tests showed an abnormal level of calcium, raised alkaline phosphatase (ALP) and a prostatic specific antigen (PSA) of 10 (normal 0-5). Plain x-rays of the lumbar spine demonstrated bony sclerosis in a vertebrae (L4) which was not present on his previous x-ray, taken five years before.
The trap here is to assume that the features represent Paget’s disease without exclusion of other possibilities. This patient may have prostatic cancer with bony metastasis as this may mimic Paget’s disease. The elevated PSA and micturition difficulty makes it important to refer him to a Urologist for further investigation. If a biopsy of the prostate or an MRI scan of the spine show evidence of cancer, then the treatment would be with hormonal manipulation therapy to treat the prostatic cancer. If however, investigations show monostotic Paget’s disease (Paget’s in just one bone), then therapy with a single infusion of 5 mg of intravenous Zoledronic acid may suffice to control his symptoms indefinitely.

Case 2

An 83 year old lady presented with increasing pain in her right hip causing limited mobility. She could hardly climb the stairs in her house and the pain was disturbing her sleep. Examination showed features of generalised osteoarthritis in her fingers,while her right hip demonstrated marked reduction in rotation and pain on attempted flexion. Blood tests showed a slight rise in alkaline phosphatase, while a plain x-ray of her hip confirmed severe osteoarthritis with narrowing of the joint space and sclerosis. There was however, also widening of the trabeculae around the neck and head of femur with some expansion of the femoral head and variation in bone density at the same site.
The mistake here would be to assume that all her symptoms were due to osteoarthritis of the hip and to anticipate that a total hip replacement would solve her problems. In this situation, co-existing Paget’s disease is important to recognise and treat, as surgery may be likely to fail otherwise as a consequence of bony softening and / or fracture. A single infusion of Zoledronic acid may suffice to relieve symptoms and improve bone quality and density in the region of the hip, permitting elective surgery three to six months later if pain persists. The intravenous bisphosphonate alone, however, may lead to significant symptomatic improvement.

Case 3

An 88 year old man presented to his GP with low back pain, radiating down the left leg. Examination showed a reduction in range of movement of the lumbar spine with little tenderness. Straight leg lift was reduced on the left and his ankle jerk and strength at the ankle were both reduced. Blood tests including alkaline phosphatase, calcium and PSA were all normal although a reduction in renal function was noted. A plain x-ray showed features of degenerative disc disease, most marked at the lumbosacral junction. There was also an area of abnormal bone in the right ilium, with sclerosis and coarsened trabeculae, strongly suggestive of Paget's disease, but no fracture was seen. These features were present on a previous x-ray 3 years earlier.
The patient appeared to have a disc prolapse at the lumbosacral junction with a resulting left radiculopathy causing symptoms. The radiographic features of his Paget's disease were unlikely to be associated with any symptoms, and the normal blood tests and stability of the radiographic changes made it most likely that his Paget's disease was neither active nor rapidly progressive. Treatment in this situation may actually carry more risk than benefit as there is no evidence that the treatment of asymptomatic lesions at this site alter outcome.

Summary

In summary, Pagets disease is common among the elderly and is easily treated in most cases with a single infusion of Zoledronic acid if renal function, calcium and vitamin d levels are adequate. Although further infusions can be given, in practice these are rarely needed at intervals of less than three years. Failure to correct vitamin D can precipitate hypocalcaemia and administration to patients with a glomerular filtration rate of less than 30 mls/minute, can also precipitate toxicity. Hence treatment of asymptomatic lesions in non-weight bearing bones may be avoided if the bone chemistry suggests low disease activity.