Disciplina MCP5777 Estudio Profundo del Tratamiento da Fibrosis Intersticial Pulmonar Idiopática Área de Concentración: 5150 Creación: 27/11/2014 Activación: 27/11/2014 Nro. de Créditos: 1 Carga Horaria: Teórica Práctica Estudios Duración Total (por semana) (por semana) (por semana) 3 5 7 1 semanas 15 horas Docentes Responsables: Ronaldo Adib Kairalla Carlos Roberto Ribeiro de Carvalho Bruno Guedes Baldi Objetivos: Discutir y analizar críticamente las opciones terapéuticas disponibles para la fibrosis pulmonar idiopática (FPI), teniendo en cuenta los aspectos fisiopatológicos y las nuevas opciones farmacológicas las que, basadas en estudios recientes, trajeron perspectivas más favorables a los pacientes. Justificativa: El mejor entendimiento de los mecanismos de lesión de la membrana alvéolo-capilar y de la respuesta reparadora en la fibrosis pulmonar idiopática (FPI) que se han adquirido en los últimos años, asociados al surgimiento de nuevas perspectivas de tratamiento con bloqueadores de la fibrosis, como la pirferidona y los inhibidores de la fibrosis tirosinquinasa, mejoran la perspectiva de los pacientes portadores de FPI. Esta disciplina tiene como objetivo la presentación y discusión de estos nuevos conceptos y de los resultados observados con los nuevos tratamientos de la FPI, además de discutir la monitorización óptima de la respuesta terapéutica. Contenido: Aulas Teóricas: 1. Estudio profundo de la fisiopatología da fibrosis pulmonar idiopática (FPI). 2. Correlación entre patología y función pulmonar en la FPI. Seminarios 1. Estudio profundo de la etiología y patogénesis de la fibrosis pulmonar
idiopática, incluyendo evaluación de modelos experimentales utilizados en la FPI pulmonar. 2. Evaluación de los factores prognósticos establecidos, incluyendo parámetros clínicos, tomográficos, funcionales e histopatológicos. 3. Estudio crítico del tratamiento farmacológico con corticoesteroides, inmunosupresores y n-acetil-cisteína. 4. Estudio crítico del tratamiento farmacológico con medicamentos que actúan sobre la fibrosis, como la pirferidona y los inhibidores de tirosinquinasa. 5. Tratamiento farmacológico: perspectivas futuras. 6. Evaluación de los parámetros utilizados para la evaluación de la respuesta terapéutica. 7. Análisis crítico del trasplante pulmonar como opción terapéutica Forma de Evaluación: 1. Presentación del seminario 2. Participación en las discusiones de los temas. Observación: Bibliografía: 1. American Thoracic Society. (2000) Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement; American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 161(2 pt 1),646-664. 2. Alton, EW, Johnson, M, Turner-Warwick, M. Advanced cryptogenic fibrosing alveolitis: preliminary report on treatment with cyclosporin A. Respir Med 1989; 83:277. 3. Baughman, RP, Lower, EE. Use of intermittent, intravenous cyclophosphamide for idiopathic pulmonary fibrosis. Chest 1992; 102:1090. 4. Carrington, CB, Gaensler, EA, Coutu, RE, et al. Natural history and treated course of usual and desquamative interstitial pneumonia. N Engl J Med 1978; 298:801. 5. Cegla, UH. Treatment of idiopathic fibrosing alveolitis. Therapeutic experiences with azathioprine-prednisolone and D-penicillamine-prednisolone combination therapy. Schw Med Woch 1977; 107:184. 6. Chapela, R, Zuniga, G, Selman, M. D-penicillamine in the therapy of fibrotic lung diseases. Int Jour Clin Pharm Ther Tox 1986; 24:16. 7. Costabel, U, Matthys, H. Different therapies and factors influencing response to therapy in idiopathic diffuse fibrosing alveolitis. Respiration 1981; 42:141. 8. Johnson, MA, Kwan, S, Snell, NJC, et al. Randomized controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis. Thorax 1989; 44:280. 9. Keogh, BA, Bernardo, J, Hunninghake, GW, et al. Effect of intermittent high dose parenteral corticosteroids on the alveolitis of idiopathic pulmonary fibrosis. Am Rev Respir Dis 1983; 127:18. 10. Panos, RJ, Mortenson, R, Niccoli, SA, King, TE Jr. Clinical deterioration in patients with idiopathic pulmonary fibrosis. Causes and assessment. Am J Med 1990; 88:396. 11. Peters, SG, McDougall, JC, Douglas, WW, et al. Colchicine in the treatment of pulmonary fibrosis. Chest 1993; 103:101. 12. Raghu, G, Depaso, WJ, Cain, K, et al. Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: A prospective, double-blind randomized, placebo-controlled clinical trial. Am Rev Respir Dis 1991; 144:291. 13. Schwartz, DA, Helmers, RA, Galvin, JR, et al. Determinants of survival in
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