Pulmonary Alveolar Proteinosis

Borie, R.; Pradère, Pauline; Danel, Claire; Debray, Marie‐Pierre; Crestani, Bruno

doi:10.1007/978-1-4471-2401-6_18

Cited by 21 publications

(57 citation statements)

References 83 publications

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“…La PAP est une maladie rare et sa prévalence varie en fonction des pays ; elle serait de l'ordre de 4 à 40 cas par million d'habitants et l'incidence de 0,2 par million d'habitants par an [3]. Il existe une prédominance masculine de la PAP auto-immune avec un sex-ratio > 2 [2][3][4][5][6][7][8][9][10].…”

Section: éPidémiologieunclassified

“…Il existe une prédominance masculine de la PAP auto-immune avec un sex-ratio > 2 [2][3][4][5][6][7][8][9][10]. L'âge moyen lors du diagnostic est de 49 ± 9 ans dans la série française [2], correspondant aux données de la littérature.…”

Section: éPidémiologieunclassified

“…Les lipides et les protéines du surfactant jouent un rôle dans la diminution de la tension de surface des alvéoles (évitent le collapsus télé-expiratoire) et interviennent dans les processus de défense innée au niveau pulmonaire, notamment par SP-A et SP-D qui appartiennent à la famille des collectines [12]. Les mutations des gènes des protéines du surfactant entraînent une anomalie de la production du surfactant responsable d'une accumulation toxique pour le pneumocyte de type II [3].…”

Section: Physiopathologie Le Surfactantunclassified

“…Les auteurs rapportent une amélioration de la survie dans les séries les plus récentes. Il semble que la survie à 5 ans soit actuellement de l'ordre de 95 % [3,6].…”

Section: éVolution Et Complicationsunclassified

“…Dans notre expérience, l'amélioration radiologique peut être retardée par rapport à l'amélioration des autres paramètres cités ci-dessus. Un seul GLPT (bilatéral) est suffisant chez la moitié des patients, ce qui suggère que la présence des anticorps anti-GM-CSF n'est pas suffisante pour maintenir la maladie [3]. Chez l'autre moitié des patients, il sera nécessaire de répéter les GLPT, avec en moyenne un lavage de plus (extrêmes : 1 à 22) [10].…”

Section: Complications Et Efficacité Des Lavages Thérapeu-tiquesunclassified

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La protéinose alvéolaire pulmonaire

Jouneau

Kerjouan

Briens

et al. 2014

Revue des Maladies Respiratoires

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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.

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Section: éPidémiologieunclassified

Section: Physiopathologie Le Surfactantunclassified

“…Les auteurs rapportent une amélioration de la survie dans les séries les plus récentes. Il semble que la survie à 5 ans soit actuellement de l'ordre de 95 % [3,6].…”

Section: éVolution Et Complicationsunclassified

Section: Complications Et Efficacité Des Lavages Thérapeu-tiquesunclassified

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La protéinose alvéolaire pulmonaire

Jouneau

Kerjouan

Briens

et al. 2014

Revue des Maladies Respiratoires

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Alveolar and lung interstitial macrophages: Definitions, functions, and roles in lung fibrosis

Shi

Denney

et al. 2020

Journal of Leukocyte Biology

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Mϕs are the main innate immune cells in the lung at homeostasis, with important roles in host defence and immune modulation. Alveolar Mϕs (AMs) and interstitial Mϕs (IMs) are the two lung Mϕ subsets, so called according to the sites they reside in. These subsets are also defined by their origins and immunological microenvironment, which endow these cells with distinct features and plasticity. This review summarizes the latest definitions and functions of lung Mϕs during homeostasis and provides exemplar of their divergent roles in lung fibrosis.

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IL‐1α induces CD11b^low alveolar macrophage proliferation and maturation during granuloma formation

Huaux

Giulia

et al. 2015

The Journal of Pathology

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Macrophages play a central role in immune and tissue responses of granulomatous lung diseases induced by pathogens and foreign bodies. Circulating monocytes are generally viewed as central precursors of these tissue effector macrophages. Here, we provide evidence that granulomas derive from alveolar macrophages serving as a local reservoir for the expansion of activated phagocytic macrophages. By exploring lung granulomatous responses to silica particles in IL-1-deficient mice, we found that the absence of IL-1α, but not IL-1β, was associated with reduced CD11b(high) phagocytic macrophage accumulation and fewer granulomas. This defect was associated with impaired alveolar clearance and resulted in the development of pulmonary alveolar proteinosis (PAP). Reconstitution of IL-1α(-/-) mice with recombinant IL-1α restored lung clearance functions and the pulmonary accumulation of CD11b(high) phagocytic macrophages. Mechanistically, IL-1α induced the proliferation of CD11b(low) alveolar macrophages and differentiated these cells into CD11b(high) macrophages which perform critical phagocytic functions and organize granuloma. We newly discovered here that IL-1α triggers lung responses requiring macrophage proliferation and maturation from tissue-resident macrophages.

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Pulmonary Alveolar Proteinosis

Cited by 21 publications

References 83 publications

La protéinose alvéolaire pulmonaire

La protéinose alvéolaire pulmonaire

Alveolar and lung interstitial macrophages: Definitions, functions, and roles in lung fibrosis

IL‐1α induces CD11b^low alveolar macrophage proliferation and maturation during granuloma formation

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Pulmonary Alveolar Proteinosis

Cited by 21 publications

References 83 publications

La protéinose alvéolaire pulmonaire

La protéinose alvéolaire pulmonaire

Alveolar and lung interstitial macrophages: Definitions, functions, and roles in lung fibrosis

IL‐1α induces CD11blow alveolar macrophage proliferation and maturation during granuloma formation

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Product

Resources

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IL‐1α induces CD11b^low alveolar macrophage proliferation and maturation during granuloma formation