Decreased Survival and Lung Function in Progressive Pulmonary Fibrosis
Abstract
:1. Introduction
2. Methods
2.1. Patients
2.2. Progressive Pulmonary Fibrosis
2.3. MUC5B and Telomere Length
2.4. Statistical Analysis
3. Results
3.1. Patients
3.2. PPF Criteria
3.3. Associations with PPF Phenotype
3.4. Survival in Non-IPF and IPF patients
4. Discussion
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
CI | Confidence interval |
CTD-ILD | Connective tissue disease-associated interstitial lung disease |
DLCOc | Diffusing capacity for carbon monoxide corrected for hemoglobin |
FHP | Fibrotic hypersensitivity pneumonitis |
FVC | Forced vital capacity |
HRCT | High-resolution computed tomography |
ILD | Interstitial lung disease |
IPAFs | Interstitial pneumonia with autoimmune features |
IPF | Idiopathic pulmonary fibrosis |
LB | Lung biopsy |
OR | Odds ratio |
PF-ILD | Progressive fibrosing interstitial lung disease |
PFT | Pulmonary function test |
PPF | Progressive pulmonary fibrosis |
RA-ILD | Rheumatoid arthritis-associated interstitial lung disease |
Ref | Reference |
SNP | Single nucleotide polymorphism |
SR-ILD | Smoking-related interstitial lung disease |
TL | Telomere length |
TPF | Transplant-free |
UIP | Usual interstitial pneumonia |
Yrs | Years |
References
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Non-IPF without PPF (n = 158) | Non-IPF with PPF (n = 146) | p-Value | Non-IPF with PPF (n = 146) | IPF (n = 379) | p-Value | |
---|---|---|---|---|---|---|
Male (%) | 95 (60) | 88 (60) | 0.979 | 88 (60) | 297 (78) | <0.001 |
Ever smokers (%) | 97 (64) | 104 (75) | 0.051 | 104 (75) | 284 (77) | 0.612 |
Age at diagnosis | 63.9 ± 10.1 | 63.8 ± 11.5 | 0.945 | 63.8 ± 11.5 | 68.0 ± 8.8 | <0.001 |
Diagnosis | ||||||
FHP (%) | 53 (34) | 60 (41) | 0.173 | 60 (41) | - | |
Unclassifiable ILD (%) | 51 (32) | 54 (37) | 0.388 | 54 (37) | - | |
CTD-ILD (%) | 45 (28) | 28 (19) | 0.058 | 28 (19) | - | |
SR-ILD (%) | 9 (6) | 4 (3) | 0.203 | 4 (3) | - | |
Baseline FVC (%) | 82.4 ± 22.4 | 75.1 ± 21.3 | 0.005 | 75.1 ± 21.3 | 76.2 ± 24.6 | 0.630 |
Baseline DLCOc (%) | 40.5 ± 22.3 | 33.0 ± 20.8 | 0.003 | 33.0 ± 20.8 | 35.8 ± 20.0 | 0.153 |
MUC5B (rs35705950) genotype (minor allele = T) | ||||||
GG (%) | 110 (70) | 94 (64) | 0.332 | 94 (64) | 165 (44) | <0.001 |
GT (%) | 40 (25) | 49 (34) | 0.115 | 49 (34) | 181 (48) | 0.003 |
TT (%) | 8 (5) | 3 (2) | 0.161 | 3 (2) | 33 (9) | 0.007 |
Minor allele frequency | 0.18 | 0.19 | 0.722 | 0.19 | 0.33 | <0.001 |
UIP on HRCT (%) | 18 (11) | 26 (18) | 0.112 | 26 (18) | 215 (57) | <0.001 |
Underwent lung transplantation (%) | 4 (3) | 15 (10) | 0.005 | 15 (10) | 21 (6) | 0.055 |
Deceased (%) | 44 (28) | 80 (55) | <0.001 | 80 (55) | 217 (57) | 0.610 |
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Platenburg, M.G.J.P.; van der Vis, J.J.; Grutters, J.C.; van Moorsel, C.H.M. Decreased Survival and Lung Function in Progressive Pulmonary Fibrosis. Medicina 2023, 59, 296. https://doi.org/10.3390/medicina59020296
Platenburg MGJP, van der Vis JJ, Grutters JC, van Moorsel CHM. Decreased Survival and Lung Function in Progressive Pulmonary Fibrosis. Medicina. 2023; 59(2):296. https://doi.org/10.3390/medicina59020296
Chicago/Turabian StylePlatenburg, Mark G. J. P., Joanne J. van der Vis, Jan C. Grutters, and Coline H. M. van Moorsel. 2023. "Decreased Survival and Lung Function in Progressive Pulmonary Fibrosis" Medicina 59, no. 2: 296. https://doi.org/10.3390/medicina59020296