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Teriparatide
6%
5/83
Estrogen
0%
0/83
Zoledronate
71%
59/83
Dexamethasone
7%
6/83
Methotrexate
13%
11/83
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This patient is suffering from symptomatic Pagets bone disease. The first-line treatment for this condition is an aminobisphosphonate (Zoledronate). Pagets bone disease is an autosomal dominant disorder of abnormal bone remodeling that involves increased osteoblastic and osteoclastic activity. Bisphosponates act to inhibit osteoclasts and therefore slow resorption of bone. Diagnosis is often achieved through an incidental finding on a radiograph (shows thickened bone, bowing, and cotton wool spots on the skull). However, bone scan is the most sensitive diagnostic test. Less than one percent of patients will experience progression to Pagets sarcoma. Schneider et al. discuss the diagnosis and management of Pagets bone disease. The condition is frequently asymptomatic; however, when symptomatic, the presentation of the disease is dependent on the specific bones involved. Bone/joint pain as well as hearing, vision, cardiac (high output congestive heart failure), and oncologic (progression to secondary osteosarcoma) issues may arise in Pagets. Wat reviews the use of bisphosphonates in the management of Pagets bone disease. The use of bisphosphonates should be limited to symptomatic patients to ensure that the benefits outweigh the risks associated with this class of medications. Complications associated with bisphosphonate therapy can include atypical femoral fracture and/or osteonecrosis of the jaw. Calcium and vitamin D supplementation should precede and accompany bisphosphonate therapy to prevent the development of hypocalcemia. Figure A demonstrates a typical bone scan seen in Pagets disease with increased uptake. Illustration A shows the radiographic appearance of bone that may be seen in Pagets disease. Illustration B summarizes some of the sequela and complications associated with Pagets disease. Incorrect Answers: Answer 1: Teriparatide, recombinant parathyroid hormone, is used in the treatment of osteoporosis; it activates osteoblasts to stimulate bone formation. Answer 2: Estrogen hormone therapy may be used for the treatment of osteoporosis and other symptoms in menopausal women; it should not be used in pre-menopausal women or men. Answer 4: Glucocorticoid therapy does not have a role in the treatment of Pagets disease. Answer 5: Methotrexate is a third-line therapy for the treatment of Pagets disease; it should only be tried after bisphosphonates and calcitonin have been attempted.
4.5
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