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Joint or Bony Pain (including Paget's Ds)

    Paget's Disease
    • Epidemiology: 
      • Rare before 55 
      • Predominently European descent; Rare in AA and Asians and Indian Subcontinent
    • Symptoms:
      • Bone Pain (often worse with weight bearing) - thought to be due to increased metabolic activity 
        • DDx of Pain
          in patients with Paget's Disease are
          • Bone Pain (due to metabolic activity)
            • During rest and night
            • Worse on weight bearing (making differentiation from OA challenging) 
          • Nerve Compression Syndromes
          • Pseudofractures
            narrow radiolucent bands that traverse the cortex of long bones) 
          • Secondary OA
          • Other musculoskeletal conditions
      • Deafness
      • Symptoms of complications 
        • i.e Pathological fractures, Nerve Compression, Secondary OA
    • Signs: Warmth over the bone, bony deformity
    • Diagnosis: 
      • Normal ALP does not rule out the disease
      • Test for specific markers of bone turnover:  ALP, Type I NTP (Useful if co-existence of liver disease)
      • Radiographs, Radionuclide Bone Scan, 
    • Complications : Osteoarthritis, Spinal Stenosis, Pseudofractures, Osteosarcoma (< 0.5%), Obstuctive Hydrocephalus, High-output cardiac failure, Hypercalcemia of immobilization 
    • Treatment 
      • Indications:
        • Bone Pain (no benefit in symptomatic patients)
          • If OA is a differential and it is difficult to differentiate, therapeutic challenge can be done 
          • Treated with bisphosphonates
        • Psedofractures  
          • Asymptomatic: Observation
          • Symptomatic i.e Pain
            • Do NOT respond to Calcitonin
            • Can progress to pathological fracture on bisphosphonates
            • Surgical Stabilization is indicated if increasing pain in the affected site
      • Treatment Options
        • Bisphosphonates
          • MOA: Bisphosphonates are synthetic analogues of inorganic pyrophosphate that are not biodegradable, have skeletal half-lives measured in years, and adhere to mineralized surfaces. They are ingested selectively by osteoclasts and disrupt energy metabolism or specific enzymatic pathways. 
          • Amino-bisphosphonates or Nitrogen Containing Bisphosphonates (FIRST LINE)
            • Better than simple bisphosphonate in decreasing ALP, but improving symptoms
              • Alendronate (40 mg / day for 6 months)
                • Osteonecrosis of Jaw has been reported with oral supplements
              • Pamidronate
              • Risedronate
              • Zoledronic Acid
                • Side effects of IV 
                  • Acute phase response within 1-3 days; resolves completely in 7 days even without treatment; responds to acetaminophen
                  • Correct Vitamin D deficiency before treatment, and supplement calcium and Vitamin D for 1-2 weeks after infusion to avoid Hypocalcemia
          • Simple bisphosphonates are
            • Etidronate
            • Tiludronate
        • Calcitonin
          • 2nd line if bisphosphonates are CI
          • MOA: Decreases bony turnover and decreases pain 
        • Denosumab (not licensed, may be useful)
    • References

      with Normal ALP but increased Bone specific ALP
      Paget’s Disease of Bone NEJM 2016

      Joint Pain / Bony Pain case examples :

      Migrating Polyarthralgia
      • 1. 84 y/o Male Patient HTN, Aortic Stenosis, Heart block s/p Pacemaker placement, Anemia due to B12 deficiency is being seen for progressive increase in ALP (from 200 to >550) over a course of a year. GGT was slightly increased a year back. LFT were previously increased but is now normal. PT / INR is normal, and Bi is normal as well. He also has vertebral compression fracture of L1 and L2. Also, has pain in the shoulder region, and in hip. X ray confirms Pagets disease. How should you manage the patienr
        • a. Zoledronic acid 5 mg IV infusion
        • b. Alendronate 75 mg PO weekly
        • c. Alendronate 40 mg PO daily
        • d. Etidronate PO daily
        • e. Clinical observation and symptomatic Treatment
          • Answer cues: 
            • Symptomatic patients should be treated
            • ALP normalizes in most patient in about a year
            • Amino-bisphosphnates are preferred over simple bisphosphonates
            • PO preferred over IV without contraindication
            • Daily dose is given as against weekly dose