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Paget disease of bone — SCE Rheumatology MCQ

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EasyMetabolic bone diseasePaget disease of boneSCE Rheumatology

A 68-year-old man reports progressive enlargement of his hat size and left hip pain. Examination shows increased warmth over the left tibia, and audiometry confirms sensorineural hearing impairment. Serum alkaline phosphatase is elevated, with normal liver tests and adjusted calcium. A pelvic radiograph shows bony expansion, cortical thickening and coarse trabeculation, while radionuclide bone scintigraphy shows increased uptake in the skull, left hemipelvis and left tibia. What is the most likely diagnosis?

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Correct answer: A — Paget disease of bone

The correct answer is A, Paget disease of bone. The discriminating features are late-onset focal bone pain, skull enlargement causing increased hat size, hearing impairment from skull involvement, warmth over metabolically active bone, elevated ALP with normal liver tests and calcium, and characteristic bony expansion with cortical thickening and coarse trabeculation. Scintigraphy demonstrates the extent of active disease rather than providing a specific diagnosis alone. Bone metastases may elevate ALP and produce focal uptake but do not usually produce this characteristic expansile pagetic pattern. Primary hyperparathyroidism would more often cause hypercalcaemia and different skeletal changes. Osteomalacia is generally associated with vitamin D or phosphate abnormalities and diffuse skeletal symptoms. Fibrous dysplasia can be expansile but usually presents earlier and has different radiographic morphology.

Reference: Ralston SH et al. Diagnosis and Management of Paget's Disease of Bone in Adults: A Clinical Guideline. Journal of Bone and Mineral Research. 2019;34(4):579–604. https://pubmed.ncbi.nlm.nih.gov/30803025/