title: [Pulmonary alveolar proteinosis].
abstract: Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90% of all AP); secondary AP, mainly occurring as a consequence of haematological diseases, or following on from toxic inhalation or infections, and genetic AP, which affects almost exclusively children. AP diagnosis is suspected where chest CT-scan demonstrates interstitial lung disease with a crazy paving aspect; and confirmed by bronchoalveolar lavage, which has a milky appearance and contains periodic acid Schiff positive proteinaceous alveolar deposits. The use of surgical lung biopsy to confirm AP is less frequent nowadays. In this context, positive antibodies against GM-CSF indicates an auto-immune etiology of the AP. Concerning management, whole lung lavage is the gold standard therapy. In refractory AP, new treatments are available such as subcutaneous or inhaled GM-CSF supplementation, or rituximab infusions. The clinical course is unpredictable. Spontaneous improvement or even cure can occur, and the 5-year actuarial survival is 95%. The most frequent complications are infectious etiology.
authors.affiliation: Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université Rennes 1, 35043 Rennes, France. Electronic address: stephane.jouneau@chu-rennes.fr.
authors.forename: S
authors.initials: S
authors.lastname: Jouneau
authors.affiliation: Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; Université Rennes 1, 35043 Rennes, France.
authors.forename: M
authors.initials: M
authors.lastname: Kerjouan
authors.affiliation: Service de pneumologie, hôpital Yves-Le-Foll, 22000 Saint-Brieuc, France.
authors.forename: E
authors.initials: E
authors.lastname: Briens
authors.affiliation: Service de chirurgie thoracique, cardiaque et vasculaire, hôpital Pontchaillou, 35033 Rennes, France.
authors.forename: J-P
authors.initials: JP
authors.lastname: Lenormand
authors.affiliation: Service d'imagerie thoracique, hôpital Pontchaillou, 35033 Rennes, France.
authors.forename: C
authors.initials: C
authors.lastname: Meunier
authors.affiliation: Université Rennes 1, 35043 Rennes, France; Service de réanimation médicale, hôpital Pontchaillou, 35033 Rennes, France.
authors.forename: J
authors.initials: J
authors.lastname: Letheulle
authors.affiliation: Service d'anatomie et de cytologie pathologiques, hôpital Pontchaillou, 35033 Rennes, France.
authors.forename: D
authors.initials: D
authors.lastname: Chiforeanu
authors.affiliation: Service d'immunologie, de thérapie cellulaire et d'hématopoïèse, hôpital Pontchaillou, 35033 Rennes, France.
authors.forename: C
authors.initials: C
authors.lastname: Lainé-Caroff
authors.affiliation: Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; Université Rennes 1, 35043 Rennes, France.
authors.forename: B
authors.initials: B
authors.lastname: Desrues
authors.affiliation: Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université Rennes 1, 35043 Rennes, France.
authors.forename: P
authors.initials: P
authors.lastname: Delaval
mesh_terms: D000818:Animals; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001706:Biopsy; D018893:Bronchoalveolar Lavage; D018450:Disease Progression; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D016040:Lung Transplantation; D011649:Pulmonary Alveolar Proteinosis / Q000145:classification / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D013902:Radiography, Thoracic; D035583:Rare Diseases; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D016178:Granulocyte-Macrophage Colony-Stimulating Factor
keywords: Grand lavage pulmonaire; Granulocyte-macrophage colony-stimulating factor; Protéinose alvéolaire pulmonaire; Pulmonary alveolar proteinosis; Rituximab; Surfactant; Whole lung lavage