Therapy options in pulmonary alveolar proteinosis

Maurizio Luisetti, Zamir Kadija, Francesca Mariani, Giuseppe Rodi, Ilaria Campo, Bruce C. Trapnell

Research output: Contribution to journalArticle

Abstract

Pulmonary alveolar proteinosis is a rare condition characterized by the accumulation of lipoproteinaceous material within the airspaces, resulting in impaired gas transfer, and clinical manifestations ranging from asymptomatic to severe respiratory failure. To the best of the authorsg' knowledge, there are only a few conditions whose natural history has been so dramatically changed by the influence of advances in basic science, clinical medicine, and translational research in therapeutic approaches. Whole-lung lavage is the current standard of care and it plays a critical role as a modifier factor of the natural history of proteinosis. That notwithstanding, the identification of autoantibodies neutralizing granulocyte-macrophage colony-stimulating factor in serum and lung of patients affected by the form of proteinosis previously referred to as idiopathic, has opened the way to novel therapeutic options, such as supplementation of exogenous granulocyte-macrophage colony-stimulating factor, or strategies aimed at reducing the levels of the autoantibodies. The aim of this paper is to provide an updated review of the current therapeutic approach to proteinosis.

Original languageEnglish
Pages (from-to)239-248
Number of pages10
JournalTherapeutic Advances in Respiratory Disease
Volume4
Issue number4
DOIs
Publication statusPublished - Aug 2010

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Keywords

  • anti-GM-CSF
  • autoantibodies
  • granulocyte-macrophage colony-stimulating factor
  • plasmapheresis
  • pulmonary surfactant
  • whole-lung lavage

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Pharmacology (medical)

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