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a b s t r a c t Paget’s disease of bone is the paradigm of bone focal distortion with accelerated bone turnover. Over the years, a number of different drugs have been used to control its activity but, since biphosphonates were introduced for the treatment of the disease, they have become the preferred treatment. This review will update the therapeutic indications, available drugs and therapeutic response monitoring.

Objective Due to increasing improvement in the diagnosis, evaluation and management of osteoporosis and the development of new tools and drugs, the Spanish Society of Rheumatology (SER) has promoted the development of recommendations based on the best evidence available. These recommendations should be a reference to rheumatologists and other health professionals involved in the treatment of patients with osteoporosis. Methods Recommendations were developed following a nominal group methodology and based on a systematic review. The level of evidence and the degree of recommendation were classified according to the model proposed by the Center for Evidence Based Medicine at Oxford. The level of agreement was established through Delphi technique. Evidence from previous consensus and available clinical guidelines was used. Results We have produced recommendations on diagnosis, evaluation and management of osteoporosis. These recommendations include the glucocorticoid-induced osteoporosis, premenopausal and male osteoporosis. Conclusions We present the SER recommendations related to the biologic therapy risk management.

Resumen Objetivo Dado el creciente avance en el diagnostico como evaluacion y tratamiento de la osteoporosis, y la incorporacion de nuevas herramientas y medicamentos, desde la Sociedad Espanola de Reumatologia (SER) se ha impulsado el desarrollo de recomendaciones basadas en la mejor evidencia posible. Estas deben de servir de referencia para reumatologos y otros profesionales de la salud implicados en el tratamiento de pacientes con osteoporosis. Metodos Las recomendaciones se emitieron siguiendo la metodologia de grupos nominales. El nivel de evidencia y el grado de recomendacion se clasificaron segun el modelo del Center for Evidence Based Medicine de Oxford y el grado de acuerdo se extrajo por tecnica Delphi. Se utilizo toda la informacion de consensos previos y guias de practica clinica disponibles. Resultados Se realizan recomendaciones sobre el diagnostico, la evaluacion y el tratamiento en pacientes con osteoporosis. Estas recomendaciones incluyen la osteoporosis secundaria a glucocorticoides, la osteoporosis premenopausica y la del varon. Conclusiones Se presentan las recomendaciones SER sobre el diagnostico, la evaluacion y el manejo de pacientes con osteoporosis.

Resumen Objetivo Describir las caracteristicas clinicas y epidemiologicas de los sujetos incluidos en el Registro Nacional de Paget. Sujetos y metodo Registro de pacientes con enfermedad osea de Paget (EOP), confirmada radiologicamente, de 25 centros participantes. Se recogieron datos clinicoepidemiologicos (edad, sexo, fecha y manifestaciones al diagnostico y tratamientos), calidad de vida (CV) (cuestionario EuroQol de 5 dimensiones), estado de salud, factores ambientales (profesion, antecedentes de sarampion, contacto con animales, consumo de lacteos, condiciones de la vivienda, lugar de nacimiento y domicilio) y familiares (historia de EOP, procedencia de los ascendientes y numero de hijos). Se realizo una descripcion estadistica de los datos. Resultados El registro incluyo a 602 sujetos con edad media de 62 ± 11 anos con predominio de varones (55%). El 79% de los sujetos presentaba sintomas en el momento del diagnostico, fundamentalmente dolor (83%). El 82% de los sujetos habia recibido tratamiento, principalmente bisfosfonatos, con mas de un farmaco en el 47% de los casos. A pesar del tratamiento, una proporcion importante tenia limitacion de la CV, especialmente relacionada con dolor (64%), movilidad (47%) y ansiedad junto con malestar (33%). La mayor parte de los sujetos habian estado expuestos a situaciones que se consideran factores de riesgo. El 14% de los sujetos tenia historia familiar de EOP y el 1,5% de los sujetos tenia hijos con EOP. Los ascendientes de los casos familiares procedian con mas frecuencia de Avila, Salamanca, Malaga y La Coruna. Conclusiones El dolor y la limitacion de la movilidad disminuyen la CV del sujeto con EOP a pesar del tratamiento. Son frecuentes los antecedentes de exposicion a factores de riesgo.

a b s t r a c t Objectives: To describe the clinical and epidemiological characteristics of patients included in the National Register of Paget's disease. Methods: A Register of patients with Paget disease (PD), radiologically confirmed, and pertaining to 25 hospitals was analyzed. Clinical and epidemiological data were collected, including age, sex, date, and presentations at time of diagnosis, treatment, quality of life (QL) (EuroQol 5D) and perceived health, environmental factors (profession, history of measles, contact with animals, dairy consumption, housing conditions, place of birth and address) and family history (PD history, origin of the ancestors, number of children). We conducted a statistical description of the data. Results: The register included 602 patients with an average age of 62 (11) years and a predominance of male (55%). Of the patients included, 79% showed symptoms at the time of diagnosis, mainly pain (83%); 82% had received treatment, mainly bisphosphonates (47% more than one drug). Despite treatment, a significant proportion had limitations in their QL, especially related to pain (64%), mobility (47%), and anxiety/depression (33%). Most of the patients had been exposed to situations which were considered as risk factors. Of the patients included, 14% had family history of PD and 1.5% had children with PD. The ancestors of the familial cases came more frequently from Avila, Salamanca, La Coruna, and Malaga. Conclusions: The pain and the limitation of mobility decrease the QL of patients with PD despite treatment. Frequently, patients have a history of exposure to risk factors

Paget's disease of bone is the paradigm of bone focal distortion with accelerated bone turnover. Over the years, a number of different drugs have been used to control its activity but, since bisphosphonates were introduced for the treatment of the disease, they have become the preferred treatment. This review will update the therapeutic indications, available drugs and therapeutic response monitoring.

Background: Nail bed capillaroscopy represents a simple and innocuous diagnosis method, used in vasculitis or collagen diseases. In Fabry disease (FD), there is a vasculopathy due to the storage of globotriaosylceramide. This is the first series that studies capillaroscopy in FD. Objective: To describe and evaluate the capillary findings in Fabry patients. Materials and Methods: Eight Fabry patients were selected; five were under enzyme replacement therapy (ERT) with agalsidase-A. Results: In three patients (under ERT), the capillaroscopy showed no abnormalities; two patients had absence of some capillaries (one with ERT); in three (one with ERT) the capillaroscopy revealed ramification of capillaries. Patients with normal capillaroscopy had no symptom of acroparesthesia for more than a year. Conclusions: Capillaroscopy is a good method to evaluate small vessels in FD and seems to have a correlation with ERT benefits, clinical manifestations and capillary findings. We purpose to use the capillaroscopy in the study of Fabry patients.