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Paget s Disease of Bone Singer FREndotext-/-ä 2000[]; ä (ä): äSir James Paget described a skeletal disorder affecting one or more areas of the skeleton in 1876. It is most common in England and in countries to which the English migrated. In recent years the prevalence in most countries has decreased. A common feature is skeletal deformity which evolves over many years and is most visible in the skull and lower extremities. Pathological fractures are most likely to occur in the femurs. Pain is a common feature in patients with Paget's disease and may be of skeletal, joint, neurologic, or muscle origin. The radiologic features begin with a localized area of osteolysis which advances very slowly in the absence of therapy. Over time the lesion becomes osteosclerotic and once an entire bone is affected the entire lesion is sclerotic with areas of osteolysis remaining. Bone scans utilizing technetium99m-labeled bisphosphonates exhibit markedly increased uptake in the untreated state. Histologic evaluation of early lesions reveals an increased number of osteoclasts advancing at the interface of normal bone. They are often larger than normal and contain many more nuclei than normal osteoclasts. Subsequently numerous osteoblasts are found to be producing a large amount of disorganized bone. Associated with the increase in osteoclasts and osteoblasts there is a highly vascular fibrocellular marrow replacing the hematopoietic marrow. The osteoclasts have an abnormal ultrastructure featuring nuclear inclusions, and sometimes, cytoplasmic inclusions resembling nucleocapsid-like structures of the Paramyxoviridae family. Measurement of serum or urine N- or C-telopeptides documents the degree of bone resorption and serum total alkaline phosphatase activity, serum bone specific alkaline phosphatase and serum procollagen type 1 amino-propeptide document bone formation. Serum total alkaline phosphatase activity is the least expensive and most widely used test. Patients may develop sarcomas or giant cell tumors in affected bone but this is rare. Metabolic complications include hypercalcemia associated with immobilization and hyperuricemia and gout in patients with more extensive disease. Increased cardiac output may occur in patients with extensive disease due to the vascularity of the lesions. The earliest effective treatment was calcitonin but with the increased efficacy of the more potent bisphosphonates calcitonin is seldom prescribed. The treatment of choice is presently an intravenous infusion of 5 mg zoledronate. This normalizes bone resorption and formation markers for up to six and a half years in most patients. Indications for treatment include bone pain, hypercalcemia, neurologic deficits with vertebral disease, congestive heart failure, preparation for orthopedic surgery, and prevention of complications such as hearing loss and deformity. Surgery most commonly is needed for lower extremity joint replacement and correction of deformities of the lower extremity. The etiology remains somewhat controversial with some studies indicating a role for measles virus. The observation that the prevalence of the disease has decreased could be explained by the introduction of measles vaccine in 1963. Clearly genetic factors also play a role. Mutations in the sequestosome 1 gene produce susceptibility to develop Paget's disease but not all family members with the mutation develop Paget's disease. Many other gene abnormalities may also increase disease susceptibility. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.ä |
