Proteinosis alveolar pulmonar

espanolla proteinosis alveolar pulmonar es una enfermedad rara que se produce por un acumulo anormal de lipoproteinas a nivel alveolar, siendo la broncoscopia una herramienta importante para su diagnostico y tratamiento. Se presenta el caso de una mujer de 44 anos, fumadora, con tos escasamente productiva blanquecina y disnea de tres meses de evolucion, tratada de forma previa con antibioterapia por sospecha de neumonia adquirida en la comunidad, con escasa mejoria. La tomografia toracica mostro un patron intersticial bilateral, compuesto por zonas en vidrio deslustrado, areas geograficas y crazy paving. A nivel analitico, se observaron niveles aumentados de LDH e IgE junto a hipoxemia moderada, siendo la serologia de VIH negativa. Las muestras de la broncoscopia fueron positivas para tinciones con acido peryodico de Schiff, hallazgo compatible con el diagnostico de proteinosis alveolar pulmonar. La paciente fue tratada con lavado broncoalveolar total, con buena respuesta y sin presentar recaidas hasta la fecha. EnglishPulmonary alveolar proteinosis is a rare disease caused by an abnormal accumulation of lipoproteins at the alveolar level in which bronchoscopy is an important tool for diagnosis and treatment. We present the case of a 44-year-old woman, smoker, with a slightly productive cough producing milky sputum and dyspnea progressing over three months, previously treated with antibiotics for suspected community-acquired pneumonia with little improvement. The thoracic CT showed a bilateral interstitial pattern composed of areas of ground glass opacity, geographic areas and crazy paving. At the analytical level, increased levels of LDH and IgE along with moderate hypoxemia were observed, with negative HIV serology. Bronchoscopy samples were positive for Periodic Acid-Schiff staining, a finding compatible with the diagnosis of pulmonary alveolar proteinosis. The patient was treated with a full bronchoalveolar lavage with a good response and she has not suffered a relapse to date.