Combined Pulmonary Fibrosis and Emphysema: Comparative Evidence on a Complex Condition
Abstract
:1. Introduction
2. Definition
3. Epidemiology
4. Etiology and Pathogenesis
5. Main Pathological Features
6. Clinical Features
7. Lung Function
8. Imaging
9. Comorbidities
9.1. Lung Cancer
9.2. Pulmonary Hypertension
10. Treatment
11. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Pulmonary Function Test Variable | CPFE | Fibrotic ILD | Emphysema |
---|---|---|---|
FVC | N/↓ | ↓ | N/↓ |
FEV1 | N/↓ | ↓ | N/↓ |
FEV1/FVC | N/↓/↑ | N/↑ | N/↓ |
TLC | N/↓/↑ | N/↓ | ↑ |
FRC | N/↓/↑ | N/↓ | ↑ |
RV | N/↓/↑ | N/↓ | ↑ |
DLCO | ↓ disproportionately | ↓ | ↓ |
KCO | ↓↓ | N/↓ | ↓ |
SaO2 following 6 min walk test | ↓↓ | ↓ | ↓ |
COPD | CPFE | IPF | |
---|---|---|---|
Prevalence | ↑↑↑ | ↓↓ | ↓ |
Smoking history | ↑↑↑ | ↑↑↑/− | ↑ |
Gender predilection | male predominance | male predominance | male predominance |
Age of clinical manifestations | 5th–6th decade | 6th–7th decade/earlier in CTD-ILD | 5th–6th decade |
Spectrum of environmental exposures | Tobacco, air pollution | Tobacco, noxious particles, or gases other than tobacco, asbestos, silica dust, and agrochemical chemicals, organic dust | Organic dust, metal and mineral dust, wood dust, asbestos, and ambient particulate matter |
Autoimmunity | − | −/+ | − |
Response to oxidative stress | cellular injury and death and ECM ↓ | cellular injury and death and ECM ↓ in the upper lobes, Activation of fibroblasts, ECM ↑ in the lower lobes | Activation of fibroblasts, ECM ↑ |
Role of epigenetic factors | |||
| ↓ | ↑ | ↑ |
| ↑ | ↑ | ↓ |
| ↓ | ↑ | ↑ |
| − | ↓ | ↓ |
Pathology | Small airways remodeling, fibrosis, and destruction of the lung parenchyma with an upper lobe predominance | Emphysema in the upper lobes and patchy fibrosis, fibroblast foci, and honeycombing—UIP/NSIP/DIP pattern—in the lower lobes. Presence of thick-walled cysts on a UIP background | Lung parenchyma and interstitium, with a lower lobe predilection |
Clinical features | |||
| Cough with sputum production, dyspnea later in the disease course | Dyspnea, dry cough later in the disease course | Dyspnea, dry cough later in the disease course |
| − | + | + |
| Not a typical sign | 50% | 70% |
Lung function | Obstruction +/− Low DLCO | Normal Low DLCO | Restriction Low DLCO |
Imaging | Centrilobular and/or bullous emphysema | Coexistence of paraseptal emphysema in the upper zones of the lungs and the UIP/NSIP/DIP in the lower zones, thick-walled cysts in the area of fibrosis | UIP pattern |
Comorbidities | |||
| ↑ | ↑↑ | ↑ |
| ↑ | ↑↑ | ↑ |
Treatment options | Bronchodilators +/− ICS +/− Oxygen therapy | Bronchodilators +/− ICS Antifibrotics (pirfenidone, nintedanib) Oxygen therapy | Antifibrotics (pirfenidone, nintedanib) Oxygen therapy |
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Calaras, D.; Mathioudakis, A.G.; Lazar, Z.; Corlateanu, A. Combined Pulmonary Fibrosis and Emphysema: Comparative Evidence on a Complex Condition. Biomedicines 2023, 11, 1636. https://doi.org/10.3390/biomedicines11061636
Calaras D, Mathioudakis AG, Lazar Z, Corlateanu A. Combined Pulmonary Fibrosis and Emphysema: Comparative Evidence on a Complex Condition. Biomedicines. 2023; 11(6):1636. https://doi.org/10.3390/biomedicines11061636
Chicago/Turabian StyleCalaras, Diana, Alexander G. Mathioudakis, Zsofia Lazar, and Alexandru Corlateanu. 2023. "Combined Pulmonary Fibrosis and Emphysema: Comparative Evidence on a Complex Condition" Biomedicines 11, no. 6: 1636. https://doi.org/10.3390/biomedicines11061636