Acute Exacerbation and Proposed Criteria for Progressive Pulmonary Fibrosis in Patients with Fibrotic Hypersensitivity Pneumonitis and Idiopathic Pulmonary Fibrosis.
Respiration
; 102(9): 803-812, 2023.
Article
in En
| MEDLINE
| ID: mdl-37619546
ABSTRACT
BACKGROUND:
Acute exacerbation (AE) occasionally develops in the course of fibrotic hypersensitivity pneumonitis (HP).OBJECTIVE:
The aim of the study was to compare AE of fibrotic HP with that of idiopathic pulmonary fibrosis (IPF).METHODS:
Consecutive patients with pathologically confirmed fibrotic HP and IPF diagnosed based on a multidisciplinary discussion were included in the analysis. AE in patients with fibrotic HP and IPF was evaluated retrospectively.RESULTS:
This study included 309 and 160 patients with fibrotic HP and IPF, respectively. Their 50% survival times were 96.1 and 78.0 months, respectively (hazard ratio [HR] 0.54 [95% confidence interval, CI 0.36-0.77], log-rank test; p < 0.001). Notably, the cumulative AE rates of fibrotic HP were 3% at 1 year and 10% at 3 years. Moreover, the corresponding rates of IPF were 8% at 1 year and 20% at 3 years (HR 0.66 [95% CI 0.45-0.93], log-rank test; p = 0.034). The 90-day survival rates from the AE onset of fibrotic HP and IPF were 75% and 64%, respectively (HR 0.51 [95% CI 0.31-0.83], log-rank test; p = 0.006). The respiratory function test on the physiological criteria of progressive pulmonary fibrosis (PPF) was a predictor of AE in fibrotic HP. However, the high-resolution CT (HRCT) changes in the criteria of PPF were not. Nevertheless, both the physiological and radiological criteria of PPF were a predictor of AE of IPF.CONCLUSION:
AE of fibrotic HP has a lesser prognostic effect than that of IPF. HRCT criteria for PPF were not a risk factor for AE in patients with fibrotic HP.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Idiopathic Pulmonary Fibrosis
/
Alveolitis, Extrinsic Allergic
Type of study:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
Respiration
Year:
2023
Document type:
Article