Paget Disease of Bone

#med #disease #y2023 #endo

Presentation :

Most commonly diagnosed in asymptomatic older patients presenting with elevated alkaline phosphatase levels or incidental radiographic findings
Sx depend on site of involvement:
- Weight bearing bones: bone pain, deformity, fracture.
- Joints: Arthritis/degen joint disease
- Upper spine or skull base: spinal cord or cranial nerve compression

Diagnosis is based on radiographic findings of thickening of cortical bone, coarsened trabecular markings, and distortion and expansion of involved bone
Serum alkaline phosphatase, a marker of increased bone formation, is generally elevated but may be normal in long-term metabolically inactive disease
Patients with suspected Paget disease of bone require assessment of serum calcium, 25-hydroxyvitamin D, and a whole-body radionuclide bone scan. If the bone scan reveals other skeletal sites suspicious for this disease, radiography of those sites is required for further evaluation.

Indications for treatment include bone pain; risk for fracture and progressive deformity that may compromise bone, joint, or neurologic function; and possibly elevated alkaline phosphatase concentrations:
- A single dose of 5 mg of intravenous zoledronic acid often achieves the treatment goal of reducing pain and normalizing alkaline phosphatase for up to 5 years
- Retreatment is indicated if previously normalized levels of alkaline phosphatase exceed normal levels