Alveolar proteinosis

Proteinosis alveolar

Published 2017-08-01

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Vol. 29 No. 1 (2017)
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Presentación de casos
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Alveolar proteinosis. rev. colomb. neumol. [Internet]. 2017 Aug. 1 [cited 2024 Nov. 16];29(1):55-62. Available from: https://revistas.asoneumocito.org/index.php/rcneumologia/article/view/239
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https://doi.org/10.30789/rcneumologia.v29.n1.2017.239
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Carlos Bula G.
Hospital Fundación Universidad Metropolitano (FHUM). Barranquilla
    Ernesto Santiago H.
    Clínica General del Norte. Barranquilla
      Brenda Martínez O.
      Fundación Hospital Universidad Metropolitana de Barranquilla. Barranquilla
        Melisa Reyes
        Universidad Libre de Barranquilla.Barranquilla,
          Ricardo Pabón R.
          Fundación Universitaria Hospital Metropolitano, Barranquilla
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            Carlos Bula G.,

            Médico Neumólogo. Coordinador Departamento de Neumología y Docente de Postgrado Hospital Fundación Universidad Metropolitano (FHUM). Barranquilla,Colombia.

            Ernesto Santiago H.,

            Médico Internista Neumólogo: Clínica General del Norte. Barranquilla, Colombia.

            Brenda Martínez O.,

            Médico Internista: Fundación Hospital Universidad Metropolitana de Barranquilla. Barranquilla, Colombia

            Melisa Reyes,

            Residente de tercer año de Medicina Interna. Universidad Libre de Barranquilla.Barranquilla, Colombia.

            Ricardo Pabón R.,

            Residente de segundo año de Medicina Interna FHUM. Fundación Universitaria Hospital Metropolitano, Barranquilla, Colombia.

            Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of surfactant within the alveolar space, which leads to a deranged gas exchange. Its clinical course is variable, ranging from spontaneous resolution to respiratory failure and death. Three forms have been described: genetic, secondary, and primary or idiopathic. The latter is the most common form in the adult population. The pathophysiology is given by an autoimmune process against the granulocyte-macrophage colony-stimulating factor (GM-CSF); this process conditions a functional impairment of macrophages that leads to accumulation of surfactant within the alveoli, as well as pulmonary and extrapulmonary infectious manifestations, often due to unusual germs. The bulk of data yielded by image studies, clinical-radiological dissociation, and bronchoalveolar lavage material is usually enough for reaching diagnosis, although histopathology is sometimes required for confirmation. Recommended treatment is total pulmonary lavage. The mortality is low and is fundamentally related to infectious complications. We describe a case of pulmonary alveolar proteinosis that required confirmation by histopathologic study of thoracoscopic lung biopsy. We also offer a review of the literature.

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