A Study Using a Disease Registry to Observe the Long-term Effects of Nintedanib in People With Scleroderma-related Lung Fibrosis
- Conditions
- Interstitial Lung DiseasesSystemic Sclerosis Associated Interstitial Lung Disease
- Interventions
- Registration Number
- NCT07080125
- Lead Sponsor
- Boehringer Ingelheim
- Brief Summary
This post-approval registry study is planned to generate data to address remaining questions on long-term effectiveness and to better characterize longer term beneficial effects of Nintedanib in patients with systemic sclerosis associated interstitial lung disease (SSc-ILD) in terms of survival, quality of life, pattern of disease progression as well as effectiveness and safety in the subgroup of patients with pulmonary hypertension.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- ACTIVE_NOT_RECRUITING
- Sex
- All
- Target Recruitment
- 2000
- Enrolled in the EUSTAR registry
- Provided consent at their site to have their data included in the EUSTAR registry for a broad range of research studies, and agree to visit the site every 12 months
- Have a diagnosis of ILD defined by radiological findings on High Resolution Computed Tomography (HRCT) and/or X-ray that is documented in the electronic Case Report Form (eCRF) by the treating physician
- Women with Systemic Sclerosis Interstitial Lung Disease (SSc-ILD) who are pregnant, or breastfeeding will be excluded since Ofev® is contraindicated for women who are pregnant due to safety concerns
- SSc-ILD patients aged <18 years: only adults will be included in the study since Ofev® is approved for treatment of SSc-ILD in adults
- Patients with a previous Hematopoietic Stem Cell Transplantation (HSCT)
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description Exposed group Ofev® Individuals that initiate Ofev® including either: 1. with an ongoing or new immunosuppressive therapy (IST) regimen (i.e., Ofev® combination therapy) or 2. after discontinuing an IST regimen or individuals that have never been on IST (i.e. Ofev® monotherapy)
- Primary Outcome Measures
Name Time Method Time to absolute Forced Vital Capacity (FVC) decline (% predicted) ≥5%, lung transplantation (indicating end stage ILD), or death up to 10 years Time to absolute FVC decline (% predicted) ≥10%, lung transplantation (indicating end-stage ILD), or deat up to 10 years
- Secondary Outcome Measures
Name Time Method Time to absolute (% predicted) FVC decline ≥5% up to 10 years Time to absolute (% predicted) FVC decline ≥10% up to 10 years Time to relative (ml) FVC decline ≥5% up to 10 years Time to relative (ml) FVC decline ≥10% up to 10 years Time to lung transplantation (indicating end-stage ILD) up to 10 years Time to death up to 10 years Change from baseline in FVC (% predicted) at baseline, up to 10 years Change from baseline in FVC (ml) at baseline, up to 10 years Lung specific Quality of Life (QoL) measured as change from baseline of total, impact, and symptom scores using the Living with Pulmonary Fibrosis (L-PF) questionnaire, measured as change of baseline at years 1, 2, 3,….10 at baseline, up to 10 years The L-PF patient reported outcome measure (PROM) is a questionnaire designed for patients with fibrosing ILDs and developed with those patients to investigate their level of symptoms and QoL.
The L-PF consists of two modules, symptoms and impacts, and five scales: symptoms total, dyspnea, cough, fatigue, impacts total, and Total L-PF score.
Items in both modules have response options on a five-option numeric rating score with an anchor of 0 "Not at all" to 4 "Extremely". Overall scores range from 0 to 100, with higher numbers indicating a greater impairment.Time to worsening of Pulmonary Hypertension (PH) in the sub-population of patients with PH at baseline up to 10 years Worsening of PH is defined as a composite outcome of the following:
1. time to all-cause death;
2. time to non-planned PH-related hospitalization; or 3) time to PH-related deterioration identified by at least one of the following parameters: i. increase in World Health organization functional class, ii. deterioration in exercise testing; or iii. Signs or symptoms of right-sided heart failureIncidence rate of major bleeding (defined as those requiring intervention or hospitalization) in exposed vs unexposed treatment groups up to 10 years Final outcome for the major bleeding events (i.e., recovered or fatal) overall up to 10 years Proportion of patients with major bleeding that had a pre-existing risk for bleeding up to 10 years Incidence rate of gastrointestinal perforations in exposed vs unexposed treatment groups up to 10 years Final outcome (i.e., recovered or fatal) for gastrointestinal perforation events overall up to 10 years Proportion of patients with gastrointestinal perforations that had a pre-existing risk for gastrointestinal perforations up to 10 years Incidence rate of thromboembolism (arterial or venous) in exposed vs unexposed treatment groups up to 10 years Final outcome (i.e., recovered or fatal) for thromboembolic events overall up to 10 years Proportion of patients with thromboembolism that had a pre-existing risk for thromboembolism up to 10 years
Trial Locations
- Locations (1)
EUSTAR Registry
🇨🇭Basel, Switzerland
EUSTAR Registry🇨🇭Basel, Switzerland