In the last 40 years, following the publication of the seminal paper by Laurell and Eriksson, 1
there have been significant advances in the understanding of genetic abnormalities related …

Since the first American Thoracic Society statement regarding the diagnosis and management
of severe alpha-1 antitrypsin (AAT) deficiency in 1989 (1) and the initial Canadian …

The potential for interleukin-8 (IL-8) and monocyte chemotactic protein-1 (MCP-1) to induce
neutrophil and mononuclear phagocyte accumulation in the lungs of patients with pulmonary …

Whole lung lavage (WLL) is still the gold-standard therapy for pulmonary alveolar proteinosis
(PAP). The few studies on the duration of the effect of WLL, belonging to a rather remote …

Rationale α1-Antitrypsin (AAT) deficiency is one of the commonest rare respiratory disorders
worldwide. Diagnosis, assessment of risk for developing chronic obstructive pulmonary …

Background: The predominant emphysema phenotype is associated with more severe
airflow limitation in patients with chronic obstructive pulmonary disease (COPD). A study was …

The binding of the receptor for advanced glycation end products (RAGE) with its ligands begins
a sustained period of cellular activation and inflammatory signal amplification in different …

Rationale: We identified a 6-year-old girl with pulmonary alveolar proteinosis (PAP), impaired
granulocyte-macrophage colony–stimulating factor (GM-CSF) receptor function, and …

High levels of granulocyte/macrophage–colony-stimulating factor (GM-CSF) autoantibodies
are thought to cause pulmonary alveolar proteinosis (PAP), a rare syndrome characterized …

Time to first investigator-reported acute exacerbation was a key secondary end-point in the
INPULSIS trials of nintedanib in patients with idiopathic pulmonary fibrosis (IPF). We used the …