Paget's

Question 1

Complications of Paget’s? (7)

Answer 1

Secondary OA

Fracture

High-output CCF

Renal stones

Gout (due to high bone turn over)

Osteosarcoma

Neurological - including spinal cord compression

Question 2

Paget’s disease - PRICMCP?

Answer 2

P:

• elevated ALP
• incidental finding on XR
• bone pain (constant, gnawing pain that persists through the night) or secondary OA
• change in height/hat size, gait abnormalities (change in length of long bone)
• hearing loss/neurological symptoms from e.g. spinal cord compression.

R: FH (15%)

I: how was the dx established - Bone scan/XR supported by ALP, urine hydroxyproline?

C: secondary OA, #, high-output CCF, renal stones, gout (due to high bone turn over), neurological complications. OsteoSarcoma.

M: observation (asymptomatic), Bisphosphonates, Vit D, Calcium supplements, pain relief

C: how is the patient affected? current bone pain/other sympoms

P: insight/prognosis

Question 3

Paget’s examination?

Answer 3

Limb deformity, Short stature, obvious bony swelling (Osteosarcoma)

Face: skull diameter (>55cm abnormal), ausculate skull (bruits), hearing (ossicle/VIII involvement), CN lesions (foramina overgrowth/basilar invagination)

Look at the neck for basilar invagunation (short neck, low hairline, head is held in extension, limited neck movement) - see pic

CVS - high output failure

Arm, Back, Legs - deformity (bowing of the tibia/femur), tenderness, warmth, swelling of the bones (suspicious of Sarcoma). Any ROM limitations in LL.

Nerological exam: CN, UL, LL (?SC compression, basilar invagination - there may even be quadriparesis)

Gait

Question 4

What is your approach in investigating suspected Paget’s?

Answer 4

T: Raised ALP, XR (skull, pelvis, femur, tibia) - look for bony enlargement and increased density, irregular/widened cortex. Bone scan more sensitive. CT - helpful to assess skull base involvement.

E: exclude other causes of high-bone turn over (sclerotic/lytic mets), e.g. malignancy. Do Calcium and PTH (also Vit D), consider CTCAP.

S: ALP and Urinary Hydroxyproline (markers of disease activity)

S: screen for complications - imaging of suspected #, OA, ECG/TTE (high-output failure), MRI spine (cord-compression), Brain (basilar invaginaion), CTKUB/USS - stones. Sarcma - CT/MRI.

Question 5

Indications for treatment of Paget’s disease? (4)

Answer 5

Bony pain

Progressive deformity

Complications (e.g. neural compression, CCF)

Prelude to orthopaedic surgery.

Question 6

What is your approach to management of Paget’s patient?

Answer 6

Goals: achieve remission (normalise ALP, filling in osteolytic lesions), control pain and prevent complicatins

Confirm dx: review XR/CT to look for bony enlargement, inc density, irregular cortex, Bone scan, raised ALP.

A: exclude secondary causes → osteomalacia (Vit D), consider cancer markers + CTCAP guided by symptoms suggestive of malignancy

Screen complications: audiometry (hearing impairment), imaging of the brain/spine if neurological complications, ECG/TTE (high-ouput CCF), USS kidney/CTKUB (stones), urate (gout). Consider CT/MRI if osteosarcoma suspected.

T: Non-pharm

• Education: complications, symptoms to look out for
• CV risk factor management (life style, statin, control BP)
• Shoe orthotics to assist with pain relief
• Joint replacement, Osteotomy.

T: Pharm

• If symptomatic disease or complications - Bisphosphonates are the 1st line (Pamidronate 1 or more 60mg IV doses, Alendronate 3-6m, Risedronate 2m) - check EUC, Vit D and Calcium, consider OPG, assess any planned dental procedure
• Correct Vitamin D: Ostelin and Calcium replacement - should be given together with Bisphosphonate (as it may impair bone mineralisation → osteomalacia)
• Calcitonin - impoves bone pain, useful for neurological complications (2nd line)
• Pain relief, NSAIDs

Ensure F/U and monitor

• 3 monthly ALP if started tx
• Tx can be ceased once ALP normalise
• Annual F/U with repeat ALP, repeat XR if osteolytic lesions

Question 7

If patient ha Pagets with spinal cord compression, hence rapid remission is required, which pharmacological agent would you use? 2 potential adverse effects?

Answer 7

Mithramycin can be very effective for rapid remission.

However,

1. Risk of fractures from increase in bone lysis
2. Bone marrow depression