Idiopathic Pulmonary Fibrosis Is a Genetic Disease Involving Mucus and the Peripheral Airways
Idiopathic pulmonary fibrosis (IPF) is localized to the lung, is characterized by a pattern of heterogeneous, subpleural patches of fibrotic, remodeled lung, and is associated with a median survival of 3-5 years after diagnosis. A common gain-of-function MUC5B promoter variant, rs35705950, is the st...
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| Published in | Annals of the American Thoracic Society Vol. 15; no. Suppl 3; pp. S192 - S197 |
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| Main Author | |
| Format | Journal Article |
| Language | English |
| Published |
United States
American Thoracic Society
01.11.2018
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| Abstract | Idiopathic pulmonary fibrosis (IPF) is localized to the lung, is characterized by a pattern of heterogeneous, subpleural patches of fibrotic, remodeled lung, and is associated with a median survival of 3-5 years after diagnosis. A common gain-of-function MUC5B promoter variant, rs35705950, is the strongest risk factor (genetic and otherwise), accounting for at least 30% of the total risk of developing IPF. The MUC5B promoter variant can be used to identify individuals in the preclinical phase of this progressive disease, and, in the IPF lung, we have found that MUC5B is specifically overexpressed in bronchoalveolar epithelium. Thus, MUC5B represents a key molecule to understand the mechanisms that appear to initiate the fibroproliferative process in the bronchoalveolar epithelium. Moreover, focusing on MUC5B may provide a unique opportunity to define the early molecular events that lead to, and potentially prevent, the development of IPF. |
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| AbstractList | [...]we have recently found that lung tissue samples from approximately 40% of patients with IPF are highly enriched for transcripts of cilium genes, MUC5B, and MMP7 (27), and this molecular phenotype is associated with the expression of keratin 51 cells, supporting a role for MUC5B in abnormal repair and aberrant regeneration. [...]we postulate that: IPF is caused by recurrent injury/repair/regeneration at the bronchoalveolar junction secondary to overexpression of MUC5B, mucociliary dysfunction, retention of particles, ER stress, and disruption of normal reparative and regenerative mechanisms in the distal lung. The gain-of-function MUC5B promoter variant is the strongest risk factor (genetic and otherwise) for both PrePF (10, 37) and IPF (17, 28-36), and the radiographic features of PrePF and IPF are concordant (10). [...]we have recently found that, during a 5- to 6-year period of observation, approximately 75% of subjects with PrePF progressed radiographically, and that radiographic progression of PrePF is associated with a greater decline in forced vital capacity (P = 0.0001) and an increased risk of death (hazard ratio = 3.7 [95% confidence interval = 1.3-10.7]; P = 0.02) (38). [...]the emerging clinical phenotype of PrePF (>40 yr of age, asymptomatic ^ mild respiratory symptoms, and HRCT features of IPF) creates a window of opportunity to identify at-risk individuals with preclinical stages of pulmonary fibrosis before the injury/repair/regenerative process has permanently damaged substantial lung parenchyma (Figure 3). [...]the overall concept that we propose is that understanding the role of MUC5B in the early molecular stages of lung fibrosis and defining the predictive and prognostic biomarkers in preclinical stages of pulmonary fibrosis will, in aggregate, establish the scientific basis to ultimately prevent the progression of IPF. ? Idiopathic pulmonary fibrosis (IPF) is localized to the lung, is characterized by a pattern of heterogeneous, subpleural patches of fibrotic, remodeled lung, and is associated with a median survival of 3-5 years after diagnosis. A common gain-of-function MUC5B promoter variant, rs35705950, is the strongest risk factor (genetic and otherwise), accounting for at least 30% of the total risk of developing IPF. The MUC5B promoter variant can be used to identify individuals in the preclinical phase of this progressive disease, and, in the IPF lung, we have found that MUC5B is specifically overexpressed in bronchoalveolar epithelium. Thus, MUC5B represents a key molecule to understand the mechanisms that appear to initiate the fibroproliferative process in the bronchoalveolar epithelium. Moreover, focusing on MUC5B may provide a unique opportunity to define the early molecular events that lead to, and potentially prevent, the development of IPF. Idiopathic pulmonary fibrosis (IPF) is localized to the lung, is characterized by a pattern of heterogeneous, subpleural patches of fibrotic, remodeled lung, and is associated with a median survival of 3–5 years after diagnosis. A common gain-of-function MUC5B promoter variant, rs35705950, is the strongest risk factor (genetic and otherwise), accounting for at least 30% of the total risk of developing IPF. The MUC5B promoter variant can be used to identify individuals in the preclinical phase of this progressive disease, and, in the IPF lung, we have found that MUC5B is specifically overexpressed in bronchoalveolar epithelium. Thus, MUC5B represents a key molecule to understand the mechanisms that appear to initiate the fibroproliferative process in the bronchoalveolar epithelium. Moreover, focusing on MUC5B may provide a unique opportunity to define the early molecular events that lead to, and potentially prevent, the development of IPF. |
| Author | Schwartz, David A |
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| Snippet | Idiopathic pulmonary fibrosis (IPF) is localized to the lung, is characterized by a pattern of heterogeneous, subpleural patches of fibrotic, remodeled lung,... [...]we have recently found that lung tissue samples from approximately 40% of patients with IPF are highly enriched for transcripts of cilium genes, MUC5B,... |
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| SubjectTerms | Age Genetic disorders Genetic Predisposition to Disease Humans Idiopathic Pulmonary Fibrosis - genetics Idiopathic Pulmonary Fibrosis - metabolism Idiopathic Pulmonary Fibrosis - pathology Lung diseases Medical screening Mucin-5B - genetics Mucin-5B - metabolism Mucus - physiology Pathogenesis Pneumonia Population Promoter Regions, Genetic - genetics Pulmonary fibrosis Respiratory Mucosa - metabolism Smoking Transatlantic Airway Conference |
| Title | Idiopathic Pulmonary Fibrosis Is a Genetic Disease Involving Mucus and the Peripheral Airways |
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