Better approach for autoimmune pulmonary alveolar proteinosis treatment: inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor: a meta-analyses

Sheng, Gaohong; Chen, Peng; Wei, Yuanhuan; Chu, Jiaojiao; Cao, Xiao-Lei; Zhang, Huilan

doi:10.1186/s12931-018-0862-4

Cited by 38 publications

(35 citation statements)

References 45 publications

(39 reference statements)

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“…Further pharmacokinetic studies could define optimal dosing and timing of neonatal rGM-CSF to modify AM development. A meta-analysis revealed a higher response rate and greater improvements in PaO 2 in subjects with autoimmune pulmonary alveolar proteinosis treated with inhaled GM-CSF than with subcutaneous GM-CSF ( 41 ). Inhaled GM-CSF has recently been used to successfully treat Mycobacterium abscessus infections in two patients with cystic fibrosis ( 42 ).…”

Section: Discussionmentioning

confidence: 99%

Inhaled GM-CSF in neonatal mice provides durable protection against bacterial pneumonia

et al. 2019

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Pneumonia poses profound health threats to preterm infants. Alveolar macrophages (AMs) eliminate inhaled pathogens while maintaining surfactant homeostasis. As AM development only occurs perinatally, therapies that accelerate AM maturation in preterms may improve outcomes. We tested therapeutic rescue of AM development in mice lacking the actin-bundling protein L-plastin (LPL), which exhibit impaired AM development and increased susceptibility to pneumococcal lung infection. Airway administration of recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) to LPL−/− neonates augmented AM production. Airway administration distinguishes the delivery route from prior human infant trials. Adult LPL−/− animals that received neonatal GM-CSF were protected from experimental pneumococcal challenge. No detrimental effects on surfactant metabolism or alveolarization were observed. Airway recombinant GM-CSF administration thus shows therapeutic promise to accelerate neonatal pulmonary immunity, protecting against bacterial pneumonia.

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Section: Discussionmentioning

confidence: 99%

Inhaled GM-CSF in neonatal mice provides durable protection against bacterial pneumonia

et al. 2019

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“…For those with more advanced disease or even respiratory distress, whole‐lung lavage still remains the standard therapy . Novel therapies have been proposed and tested specifically for auto‐immune PAP, including GM‐CSF supplement (particularly delivered via inhalation), rituximab, and plasmapheresis . Efficacy of these experimental therapies for secondary PAP is still not established.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar proteinosis with upper‐lobe predominance in a non‐smoking female

Huang

2019

Respirology Case Reports

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In this report, we describe an unusual manifestation of pulmonary alveolar proteinosis (PAP). The patient is a 43‐year‐old non‐smoking female without underlying hematologic or auto‐immune disorder. Her initial presentation included non‐specific respiratory symptoms (exertional dyspnoea and cough), an unremarkable physical examination, a mild elevation in her serum level of lactate dehydrogenase, a mild impairment in the diffusion capacity for carbon monoxide but a normal spirometry, and multiple ground‐glass opacities with a “crazy‐paving” pattern predominantly in upper lung zones on her chest radiographic images. PAP was diagnosed histologically. PAP commonly occurs in males with smoking history, and tends to affect the lung parenchyma diffusely or, as in auto‐immune PAP, lower lobes predominantly. Upper‐lobe predominant PAP, particularly in a non‐smoking female, is rare. This report would add PAP to the list of differential diagnosis for upper‐lung ground‐glass opacities. A review on the relevant literature is also included in the discussion.

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“…Furthermore, inhaled GM-CSF was not associated to the bone marrow effects reported with the subcutaneous administration. A recent meta-analysis [ 40 ] of the studies of inhaled and subcutaneous GM-CSF therapy in autoimmune PAP patients showed that the pooled response rate of GM-CSF therapy (81%) was not inferior to WLL therapy in terms of pulmonary function tests, disease severity score and 6-min walking test. Moreover, they showed the superiority of inhaled versus subcutaneous GM-CSF therapy in term of disease-free relapse (89% versus 71%, p=0.023), P aO 2 increase (21.02 versus 8.28 mmHg, p<0.001) and alveolar–arterial oxygen difference improvement (19.63 versus 9.15 mmHg, p<0.001).…”

Section: Different Pap Forms Different Therapiesmentioning

confidence: 99%

Pulmonary alveolar proteinosis: from classification to therapy

Salvaterra

Campo

2020

Breathe

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Pulmonary alveolar proteinosis (PAP) is a rare respiratory syndrome characterised by the accumulation of surfactant lipoproteins within the alveoli. According to various pathogenetic mechanisms and aetiologies, PAP is classified as primary, secondary or congenital. Primary PAP is led by a granulocyte–macrophage colony-stimulating factor (GM-CSF) signalling disruption; the autoimmune form is driven by the presence of anti GM-CSF autoantibodies and represents 90% of all the PAP cases; and the hereditary form is the result of mutations in genes encoding GM-CSF receptor. Secondary PAP is associated with various diseases causing a reduction in function and/or number of alveolar macrophages. Congenital PAP emerges as a consequence of corrupted surfactant production, due to mutations in surfactant proteins or lipid transporter, or mutations affecting lung development. The clinical manifestations are various, ranging from insidious onset to acute or progressive respiratory failure, including premature death within the first days of life in neonates with congenital surfactant production disorders. The diagnostic workup includes clinical and radiological assessment (respiratory function test, high-resolution chest computed tomography), laboratory tests (anti-GM-CSF autoantibodies dosage, GM-CSF serum level and GM-CSF signalling test), and genetic tests. Whole-lung lavage is the current gold standard of care of PAP; however, the therapeutic approach depends on the pathogenic form and disease severity, including GM-CSF augmentation strategies in autoimmune PAP and other promising new treatments.Educational aimsTo update knowledge about a rare respiratory syndrome, pulmonary alveolar proteinosis, in order to promote early diagnosis and correct management.To highlight recent treatment options based on pathogenesis and disease severity.

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Better approach for autoimmune pulmonary alveolar proteinosis treatment: inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor: a meta-analyses

Cited by 38 publications

References 45 publications

Inhaled GM-CSF in neonatal mice provides durable protection against bacterial pneumonia

Inhaled GM-CSF in neonatal mice provides durable protection against bacterial pneumonia

Pulmonary alveolar proteinosis with upper‐lobe predominance in a non‐smoking female

Pulmonary alveolar proteinosis: from classification to therapy

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