Abstract
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and fatal disease. The primary aim of this national multicenter non-interventional study known as the INSPIRATION PLUS registry, is to describe the real world effectiveness and safety of pirfenidone and the clinical management practices in Canada.
Methods: The patients included had a confirmed diagnosis in accordance with the 2011 international guidelines. Data are collected approximately every 3 to 6 months for a minimum of 2 years. The primary objective is to describe and assess the disease course and outcomes in pirfenidone-treated IPF patients in routine clinical practice. The drug utilization patterns will also be described. A validity assessment of the ATAQ-IPF questionnaire will be done compared to three validated but non-specific to IPF Quality of life measures.
Results:
| Variable | n= 167 |
| Age (year) | 70.0 ± 8.6 |
| Sex | 27.5% female 72.5%male |
| BMI | BMI: 29.9 ± 7.1 |
| Ethnicity | Caucasian: 97.0% Native American: 1.2% Other: 1.8% |
| Smoking Status | Current: 4.8% Former: 69.5% |
| Exposure to particulates | 44 (26.3%) |
| Diagnostic by HRCT only | 119 /148 (80.4%) |
| Had a surgical lung biopsy | 28/145 (18.9%) |
| Oxygenotherapy | 19/87 (26.4%) |
| FVC >80% | 17 (22.1%) |
| 50-80% | 47 (61.0%) |
| <50% | 13 (16.9%) |
| mean FVC (%) | 68.1 ± 17.2 |
| mean DLCO (%) | 46.3 ± 16.1 |
| mean 6MWD (%) | 340.4 ± 115.4 |
| On corticosteroids | 36/87 (50%) |
Baseline demographics of the first 167 patients enrolled between 03/2013 and 12/2015
Conclusion: The characteristics of this IPF population at the initiation of pirfenidone indicate that pirfenidone is being used in a wide spectrum of IPF patients in Canada. Follow up data will be presented at a later time.
- Copyright ©the authors 2016
