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PAGET DISEASE (OSTEITIS DEFORMANS)

Features

  • 95% asymptomatic (M:F ratio = 2:1)—typically older men

  • It is a bone remodelling disorder leading to abnormal enlarged sections of bone

  • Symptoms may include joint pain and stiffness (e.g. hips, knees); bone pain (usually spine); deformity; headache; deafness (compression neuropathy)

  • Bone pain is typically deep and aching: may be worse at night

  • Signs may include deformity, enlarged skull—‘hats don’t fit anymore’, bowing of tibia, waddling gait

  • Bones most commonly affected (in order) are pelvis, femur, skull, tibia, vertebrae; increased risk of osteosarcoma

Diagnosis

  • Raised serum alkaline phosphatase (often very high >1000 U/L)

    Note: Calcium and phosphate normal

  • Plain X-ray: dense expanded bone best seen in skull and pelvis

    Note: Can mimic prostatic secondaries so every male Pagetic patient should have PR and PSA test

  • Bone isotopic scans: useful in locating specific areas

Treatment The two major goals are relief of pain and prevention of long-term complication (e.g. deafness, deformities).

Localised and asymptomatic disease requires no treatment.

Three groups of drugs currently available:

  • the bisphosphonates (i.e. etidronate, pamidronate, alendronate, tiludronate)—first line but serum Ca and vitamin D and eGFR must be OK

  • the calcitonins (salmon, porcine, human)—used if bisphosphonates contraindicated (e.g. adverse effects)

  • various antineoplastic agents (e.g. mithramycin)

Bisphosphonates are first-line agents (oral agents taken on an empty stomach)—one of:

  • alendronate 40 mg (o) daily for 6 mths (oesophagitis can be problematic)

  • pamidronate disodium 60 mg IV infused over 4 hrs (usually preferred option); repeated doses may rarely be required according to disease activity

  • risedronate 30 mg (o) daily for 2 mths

  • tiludronate 400 mg (o) daily for 3 mths

  • zoledronic acid 5 mg IV, over at least 15 mins once yearly

PAIN AND ITS MANAGEMENT

Pain is the capital symptom of humans—the great hallmark of disease—the signal par excellence to the patient and doctor that all is not well. In general terms, the origin of conscious pain can be subdivided into three broad types—nociceptive, neurogenic or nociplastic.

  1. Nociceptive pain is pain arising from stimulation of superficial or deep tissue pain receptors (nociceptors) from tissue injury or inflammation. It requires an intact nervous system. Nociception is stimulation of peripheral nociceptors (i.e. nerve endings sensitive to a noxious stimulus).

  2. Neuropathic pain is pain caused or initiated by a primary lesion or disease or dysfunction (i.e. damage) in the peripheral or central nervous system. It can be subdivided into central pain, when the primary lesion is in the central nervous system (e.g. CVA, MS) or peripheral pain, e.g. postherpetic neuralgia, peripheral neuropathy, trigeminal neuralgia.

  3. Nociplastic pain is pain arising from altered nociception despite no evidence of a nociceptive stimulus or lesion of the somatosensory system. It is a diagnosis of exclusion, and central sensitisation is the key contributor. Its experience depends on ...

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