Lung Diseases, Interstitial
Conditions
Brief summary
The primary objective for this trial is to investigate the incidence probability of progression to Progressive Fibrosing Interstitial Lung Diseases (PF-ILDs) in patients with fibrosing ILD other than Idiopathic Pulmonary Fibrosis (IPF) in real-world setting in Japan. The secondary objective is to investigate the characteristics of procedures for management and treatment in patients with fibrosing ILD other than IPF in real-world setting in Japan.
Interventions
None listed
Sponsors
Boehringer Ingelheim
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
1. Patients diagnosed with at least two fibrosing Interstitial Lung Disease (ILD) codes on different dates in the patient identification period 2. Patients aged 18 years and older on the index date 3. Patients for whom data for the 12 months prior to the index date can be extracted as baseline data
Exclusion criteria
1. Patients grouped into the underlying disease of Idiopathic Pulmonary Fibrosis (IPF) 2. Patients who have met PF-ILD progression criteria during the baseline period
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Incidence Probability of Progression to Pulmonary Fibrosing-Interstitial Lung Disease (PF-ILDs) | At 6, 12, 18 and 24 months after the index date, defined between 01-Jan-2013 and 6 months before 28-May-2020 | The cumulative incidence probability is the estimate of the risk a patient will experience by 6, 12, 18 and 24 months after the second diagnosis of fibrosing ILD (index date) of progression to PF-ILDs. It is the complement of the Kaplan-Meier estimates. The Greenwood's variance estimate was used to calculate the 95% confidence interval. Disease progression was defined as 3 or more pulmonary function tests within 365 days, 3 or more tomographies within 365 days, 1 or more claims for oxygen therapy during follow-up, 1 or more respiratory hospitalizations during follow-up, 1 or more claims for palliative care during follow-up, 1 or more lung transplant during follow-up, 1 or more claims for immunosuppressive drugs during follow-up, 1 or more claims for oral corticosteroid during follow-up, and 1 or more claims for Nintedanib during follow-up. Follow-up was between 1-Jan-2013 to 28-May-2020, the last encounter in Medical Data Vision database, or in-hospital death, whichever occurs first. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Number of Patients With Treatment of Interest During Follow-up Period | Up to 7.43 years, from 01-Jan-2013 to 28-May-2020 | Number of patients treated with immunosuppressive drugs (Rituximab, Tacrolimus, Mycophenolate, Cyclosporine, Cyclophosphamide, Azathioprine, Tocilizumab), oral corticosteroids, or nintedanib during the follow-up period. The follow-up period was between the second diagnosis of a fibrosing ILD (index date) and end of study (28-May-2020), the last encounter in Medical Data Vision database, or in-hospital death, whichever occurs first. |
| Number of Patients With Management of Interest During Follow-up Period | Up to 7.43 years, from 01-Jan-2013 to 28-May-2020 | Number of patients with oxygen therapy (HOT), lung transplant and palliative care (oxygen inhalation, opioid use) as disease management during the follow-up period. The follow-up period was between the second diagnosis of a fibrosing ILD (index date) and end of study (28-May-2020), the last encounter in Medical Data Vision database, or in-hospital death, whichever occurs first. |
Countries
United States
Participant flow
Recruitment details
Retrospective cohort study using the Medical Data Vision (MDV) database to study progression to Progressive Fibrosing Interstitial Lung Disease (PF-ILDs) in a real-world setting in Japan. Data was extracted for the period of 01-Jan-2012 to 28-May-2020 for patients presenting pre-specified ILDs that received a second diagnosis of a fibrosing ILD other than Idiopathic Pulmonary Fibrosis (IPF) between 01-Jan-2013 to 6 months before 28-May-2020.
Pre-assignment details
Patients that met all inclusion criteria and none of the exclusion criteria were followed from the day after of the second diagnosis of a fibrosing ILD other than IPF - index date - until the end of study period (28-May-2020), the last encounter in the MDV database or in-hospital death, whichever occurs first.
Participants by arm
| Arm | Count |
|---|---|
| Patients With an ILD Other Than IPF Patients in Japan with data available in the Medical Data Vision (MDV) database, presenting an underlying pre-specified Interstitial Lung Disease (ILD) between 01-Jan-2012 to 28-May-2020 and received a second diagnosis of a fibrosing ILD other than Idiopathic Pulmonary Fibrosis (IPF) in the period of 01-Jan-2013 to 6 months before 28-May-2020. | 34,960 |
| Total | 34,960 |
Baseline characteristics
| Characteristic | Patients With an ILD Other Than IPF | — |
|---|---|---|
| Age, Continuous | 71.13 Years STANDARD_DEVIATION 12.16 | — |
| Race and Ethnicity Not Collected | — | — Participants |
| Sex: Female, Male Female | 16597 Participants | — |
| Sex: Female, Male Male | 18363 Participants | — |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | 0 / 0 |
| other Total, other adverse events | 0 / 0 |
| serious Total, serious adverse events | 0 / 0 |
Outcome results
Primary
Incidence Probability of Progression to Pulmonary Fibrosing-Interstitial Lung Disease (PF-ILDs)
The cumulative incidence probability is the estimate of the risk a patient will experience by 6, 12, 18 and 24 months after the second diagnosis of fibrosing ILD (index date) of progression to PF-ILDs. It is the complement of the Kaplan-Meier estimates. The Greenwood's variance estimate was used to calculate the 95% confidence interval. Disease progression was defined as 3 or more pulmonary function tests within 365 days, 3 or more tomographies within 365 days, 1 or more claims for oxygen therapy during follow-up, 1 or more respiratory hospitalizations during follow-up, 1 or more claims for palliative care during follow-up, 1 or more lung transplant during follow-up, 1 or more claims for immunosuppressive drugs during follow-up, 1 or more claims for oral corticosteroid during follow-up, and 1 or more claims for Nintedanib during follow-up. Follow-up was between 1-Jan-2013 to 28-May-2020, the last encounter in Medical Data Vision database, or in-hospital death, whichever occurs first.
Time frame: At 6, 12, 18 and 24 months after the index date, defined between 01-Jan-2013 and 6 months before 28-May-2020
Population: Full analysis set: Patients in Japan with data available in the Medical Data Vision (MDV) database, between 01-Jan-2012 to 28-May-2020, who met all inclusion criteria and none of the exclusion criteria. The number of patients analyzed displays the number of patients at risk of progression at the time of interest.
| Arm | Measure | Group | Value (NUMBER) |
|---|---|---|---|
| Patients With an ILD Other Than IPF | Incidence Probability of Progression to Pulmonary Fibrosing-Interstitial Lung Disease (PF-ILDs) | 6 months | 0.147 Cumulative incidence probability |
| Patients With an ILD Other Than IPF | Incidence Probability of Progression to Pulmonary Fibrosing-Interstitial Lung Disease (PF-ILDs) | 12 months | 0.249 Cumulative incidence probability |
| Patients With an ILD Other Than IPF | Incidence Probability of Progression to Pulmonary Fibrosing-Interstitial Lung Disease (PF-ILDs) | 24 months | 0.395 Cumulative incidence probability |
| Patients With an ILD Other Than IPF | Incidence Probability of Progression to Pulmonary Fibrosing-Interstitial Lung Disease (PF-ILDs) | 18 months | 0.334 Cumulative incidence probability |
Secondary
Number of Patients With Management of Interest During Follow-up Period
Number of patients with oxygen therapy (HOT), lung transplant and palliative care (oxygen inhalation, opioid use) as disease management during the follow-up period. The follow-up period was between the second diagnosis of a fibrosing ILD (index date) and end of study (28-May-2020), the last encounter in Medical Data Vision database, or in-hospital death, whichever occurs first.
Time frame: Up to 7.43 years, from 01-Jan-2013 to 28-May-2020
Population: Full analysis set: Patients in Japan with data available in the Medical Data Vision (MDV) database, between 01-Jan-2012 to 28-May-2020, who met all inclusion criteria and none of the exclusion criteria.
| Arm | Measure | Group | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Patients With an ILD Other Than IPF | Number of Patients With Management of Interest During Follow-up Period | Oxygen therapy (HOT) | 1641 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Management of Interest During Follow-up Period | Lung transplant | 0 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Management of Interest During Follow-up Period | Palliative care (oxygen inhalation, opioid use) | 9520 Participants |
Secondary
Number of Patients With Treatment of Interest During Follow-up Period
Number of patients treated with immunosuppressive drugs (Rituximab, Tacrolimus, Mycophenolate, Cyclosporine, Cyclophosphamide, Azathioprine, Tocilizumab), oral corticosteroids, or nintedanib during the follow-up period. The follow-up period was between the second diagnosis of a fibrosing ILD (index date) and end of study (28-May-2020), the last encounter in Medical Data Vision database, or in-hospital death, whichever occurs first.
Time frame: Up to 7.43 years, from 01-Jan-2013 to 28-May-2020
Population: Full analysis set: Patients in Japan with data available in the Medical Data Vision (MDV) database, between 01-Jan-2012 to 28-May-2020, who met all inclusion criteria and none of the exclusion criteria.
| Arm | Measure | Group | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Rituximab | 49 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Tacrolimus | 499 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Mycophenolate | 30 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Cyclosporine | 200 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Cyclophosphamide | 225 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Azathioprine | 201 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Oral corticosteroid | 2065 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Tocilizumab | 46 Participants |
| Patients With an ILD Other Than IPF | Number of Patients With Treatment of Interest During Follow-up Period | Nintedanib | 472 Participants |