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Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.

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Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.(AU)

El compromiso cardíaco en la esclerosis sistémica (ES) es raro, pero conlleva un mal pronóstico a corto plazo. La evidencia relativa al trasplante cardíaco (TC) es escasa y se basa en la experiencia con los casos aislados. Presentamos este caso con el objetivo de analizar las características de un paciente con esclerosis sistémica sometido a un trasplante exitoso.(AU)

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Cardiac involvement in systemic sclerosis (SSc) is rare but leads to poor short-term prognosis. Evidence regarding heart transplantation (HT) is scarce and is based on experience with isolated cases. We present this case with the aim of analysing the characteristics of a patient with SS who has undergone a successful transplant.

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Objetivo: Describir las características demográficas y trastornos de pacientes con diagnóstico de síndrome de activación macrofágica (SAM) en el periodo comprendido entre diciembre de 2008-enero de 2014. Métodos: Se revisaron las historias clínicas desde el diagnóstico de SAM y tras su alta hospitalaria hasta enero de 2014. Los pacientes se agruparon en 4 grupos: autoinmunes (AI), hemato-oncólogicas (HO), infecciosas (Inf) y oncológicas (Onc). Las variables fueron analizadas entre los 4 grupos y entre AI y HO. Resultados: Trece pacientes (7 hombres, con una mediana de 54 años [32-63]) se estudiaron. Las etiologías encontradas fueron: 5 AI, 5 HO, 2 Inf y una Onc. Se encontraron células hemofagocíticas en el líquido ascítico en uno de los pacientes. Se encontró un paciente con SAM secundario a enfermedad relacionada con la IgG4. Conclusiones: La mortalidad, el pronóstico y la evolución de la enfermedad puede verse influida por el retraso en el diagnóstico, el inicio del tratamiento y la etiología del SAM. Los pacientes con enfermedades HO presentaron peor pronóstico (AU)

Objective: Describe the demographic characteristics and disorders of patients with diagnosis of Macrophage Activation Syndrome (MAS) in the December 2008 - January 2014 period. Methods: Medical records were reviewed from diagnosis of MAS and after discharge until January 2014. Patients were divided into 4 groups according to the primary disease: Autoimmune (AI), Hemato - oncologic (HO), Infectious (Inf) and Oncologic (Onc). The variables were analyzed among the 4 groups and between AI and HO. Results: Thirteen patients [7 men, with a median of 54 years (32-63)] were studied. The etiologies were: 5 AI, 5 HO, 2 Inf. and 1 Onc. disease. Hemophagocitc cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. Conclusions: Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis (AU)

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OBJECTIVE: Describe the demographic characteristics and disorders of patients with diagnosis of Macrophage Activation Syndrome (MAS) in the December 2008 - January 2014 period. METHODS: Medical records were reviewed from diagnosis of MAS and after discharge until January 2014. Patients were divided into 4 groups according to the primary disease: Autoimmune (AI), Hemato - oncologic (HO), Infectious (Inf) and Oncologic (Onc). The variables were analyzed among the 4 groups and between AI and HO. RESULTS: Thirteen patients [7 men, with a median of 54 years (32-63)] were studied. The etiologies were: 5 AI, 5 HO, 2 Inf. and 1 Onc. disease. Hemophagocitc cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. CONCLUSIONS: Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis.

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Antecedentes. En los últimos años, las Unidades de Hospitalización de Día (UHdD) en Reumatología han experimentado un amplio desarrollo. Sin embargo, existe escasa documentación respecto a estándares de calidad, que mayoritariamente se limita a la estructura y no incluye aspectos específicos de la especialidad. Objetivo. Desarrollar estándares de calidad específicos para las UHdD en Reumatología. Métodos. Tras una revisión sistemática de la literatura y de documentos relacionados, un grupo de trabajo (GT) de 8 reumatólogos, bajo la supervisión de un metodólogo experto, elaboró una primera propuesta de estándares de calidad. Se realizó una segunda ronda para su revisión y sugerencias por todo el GT. Consensuado el contenido, se realizó un informe final. Resultados. Se definieron 17 estándares de estructura, 25 de proceso y 10 de resultados, con especial énfasis en aspectos específicos de una UHdD de Reumatología. La propuesta incluye: 1) estándares imprescindibles; 2) estándares de excelencia; 3) cartera de servicios de una UHdD reumatológica, y 4) criterios de funcionamiento. Conclusiones. Los estándares de calidad propuestos son la base para la elaboración de indicadores y de otras herramientas de gestión para las UHdD reumatológicas que garanticen una práctica homogénea, centrada en el paciente y basada en la evidencia y en la experiencia (AU)

Background. In recent years, the Rheumatology Day-Care Hospital Units (DHUs) have undergone extensive development. However, the quality standards are poorly documented and mainly limited to structure items rather than including broad and specific areas of this specialty. Objective. To develop specific quality standards for Rheumatology DHUs. Methods. After a systematic review of the literature and related documents, a working group (WG) involving 8 DHU-experienced rheumatologists developed an initial proposal of the quality standards, under the supervision of an expert methodologist. A second round was held by the WG group to review the initial proposal and to consider further suggestions. Once the content was agreed upon by consensus, a final report was prepared. Results. Seventeen structure standards, 25 process standards and 10 results standards were defined, with special emphasis on specific aspects of the Rheumatology DHU. The proposal includes: (1) essential standards to (2) excellent standards, (3) a Rheumatology DHU services portfolio and (4) performance criteria. Conclusions. The proposed quality standards are the basis for developing the indicators and other management tools for Rheumatology DHU, thereby ensuring a patient-oriented practice based on both the evidence and the experience (AU)

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Introducción. El síndrome hemofagocítico (SH) se produce en enfermedades autoinmunes y pertenece al grupo de enfermedades linfohistiocitosis hemofagocítica. El presente trabajo describe las características de 2 pacientes con lupus eritematoso sistemico (LES) que comenzaron con SH. Observaciones clínicas. Ambos pacientes presentaron fiebre prolongada no asociada a proceso infeccioso y que no respondía a antibióticos. Discusión. El diagnóstico de SH secundario a LES es complicado, ya que presentan características comunes; sin embargo, el SH presenta hiperferritinemia, hipofibrinogenemia, hipertrigliceridemia y descenso de la velocidad de sedimentación globular a diferencia del LES. El tratamiento no está bien establecido, pero los corticoides y/o inmunoglobulinas son efectivos en el tratamiento inicial, y en casos refractarios la ciclosporina o la ciclofosfamida se pueden asociar. Conclusiones. El SH puede ser la manifestación inicial del LES y debe sospecharse en pacientes con organomegalias, citopenias, trastornos en la coagulación, alteraciones hepáticas y fiebre prolongada que no responde a antibióticos. Anakinra puede ser una opción de tratamiento en el SH secundario al LES adulto (AU)

Introduction: Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs o the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation. Clinical observations: Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics. Discussion: The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated. Conclusions: HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE (AU)

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BACKGROUND: In recent years, the Rheumatology Day-Care Hospital Units (DHU have undergone extensive development. However, the quality standards are poorly documented and mainly limited to structure items rather than including broad and specific areas of this specialty. OBJECTIVE: To develop specific quality standards for Rheumatology DHU. METHODS: After a systematic review of the literature and related documents, a working group (WG) involving 8 DHU-experienced rheumatologists developed an initial proposal of the quality standards, under the supervision of an expert methodologist. A second round was held by the WG group to review the initial proposal and to consider further suggestions. Once the content was agreed upon by consensus, a final report was prepared. RESULTS: 17 structure standards, 25 process standards and 10 results standards were defined, with special emphasis on specific aspects of the Rheumatology DHU. The proposal includes: 1) essential standards to 2) excellent standards, 3) a Rheumatology DHU services portfolio and 4) performance criteria. CONCLUSIONS: The proposed quality standards are the basis for developing the indicators and other management tools for Rheumatology DHU, thereby ensuring a patient-oriented practice based on both the evidence and the experience.

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INTRODUCTION: Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs to the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation. CLINICAL OBSERVATIONS: Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics. DISCUSSION: The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated. CONCLUSIONS: HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE.

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