title: Role of granulocyte-macrophage colony-stimulating factor in pulmonary fibrosis following pulmonary alveolar proteinosis.
abstract: Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease characterized by accumulation of lipoproteinacious material in alveoli, with distinct features on high resolution computed tomography and biopsy. Its association with pulmonary fibrosis is infrequently encountered, and a clear understanding of the underlying pathogenesis is yet to be established. We report the case of a 48-year-old woman with known autoimmune PAP (aPAP) first diagnosed 20 years ago, who presented with worsening hypoxemia and radiological features consistent with pulmonary fibrosis, after many years of stable disease. We present a review of previously considered mechanisms of causation behind such changes, and in particular, postulate the role of granulocyte-macrophage colony-stimulating factor deficiency in pulmonary fibrosis seen in aPAP.
authors: Department of Thoracic Medicine Frankston Hospital Melbourne Australia.
authors: Joy
authors: J
authors: Sha
authors: Department of Thoracic Medicine Frankston Hospital Melbourne Australia; Southern Clinical School, Faculty of Medicine, Nursing and Health Sciences Monash University Melbourne Australia.
authors: David
authors: D
authors: Langton
keywords: Autoimmune disease; granulocyte‐macrophage colony stimulating factor; pulmonary alveolar proteinosis; pulmonary fibrosis; rare lung diseases