Características principales de pacientes con Enfermedad de Paget en un hospital de referencia

Introduction: Paget‘s disease of bone (PDB) mainly affects adults, with an irregular geographic distribution, with areas of high prevalence, mainly in the United Kingdom, France, and certain regions of Spain. Depending on the radiological findings, the cases are classified in stages: initial or osteolytic; intermediate or mixed and terminal, quiescent or osteosclerotic. The imaging techniques used for the diagnosis are scintigraphy and radiology. Biochemical markers of bone turnover indirectly reflect the activity of PDB and the therapeutic response. The objective of this study is to know the main clinical characteristics of patients with PDB in a reference hospital. Materials and Methods: A retrospective, descriptive, cross-sectional study of patients diag- nosed with PDB at the Vall d‘Hebron Hospital in Barcelona, from January 1985 to December 2009. Data from laboratories, clinics, images and treatment were evaluated. Paget‘s disease of bone was diagnosed by typical X-ray findings. Results: Thirty-five patients diagnosed with PDB were included, with an average age of 61.58 ± 20 years, with an F/M ratio of 1/3. The reason for consultation was pain in 15/35 (42.8%) patients. The most frequent location was hemipelvis, followed by vertebrae, tibia and femur. The involvement in 17/32 (49%) patients was monostotic and in 18/35 (51%) polyostotic. The alkaline phosphatase was elevated between 2 to 10 times the normal value in 30/35 (86%) of the patients at the time of diagnosis. The treatment of choice in all patients was a bisphosphonate, with a response of 57% at 3 months. The most frequent complication was the spinal stenosis. Conclusion: Paget‘s disease of bone affects elderly patients with predominance of the male gender, that may be of polyostotic or monostotic presentation. The most frequent reason for consultation was pain and the most common complication was spinal stenosis. Early diagnosis and treatment with bisphosphonates are very important strategies for these patients.