A Study Using a Disease Registry to Observe the Long-term Effects of Nintedanib in People With Scleroderma-related Lung Fibrosis

NCT07080125 | Active Not Recruiting FDA Drug

• 2 other identifiers: 1199-0421EUPAS106792
• Study Type: observational
• Target: 2,000
• Locations: 1 country
• Sites: 1

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.

Conditions

Interstitial Lung Diseases; Systemic Sclerosis Associated Interstitial Lung Disease

Outcome Measures

Primary Outcomes (2)

• 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 Outcomes (19)

• 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

Study Arms (2)

Exposed group

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)

Drug: Ofev®

Unexposed group

Individuals that do not initiate Ofev® during the study period and can include the following: 1. Individuals on an IST regimen, described as 1a) those without an IST treatment escalation and 1b) those with an IST treatment escalation (i.e., increased dose, additional IST, switch in IST) to treat ILD- or non-ILD related SSc organ manifestations (e.g., mycophenolate, cyclophosphamide, methotrexate, tocilizumab, rituximab, etc.) 2. Individuals who receive no IST

Interventions

Ofev® DRUG

Ofev®

Also known as: Nintedanib

Exposed group

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

The study cohort will constitute patients with Systemic Sclerosis (SSc) and Interstitial Lung Disease (ILD) (based on radiological evidence) enrolled in the European Scleroderma Trials and Research (EUSTAR) registry.

You may qualify if:

• 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

You may not qualify if:

• 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)

Sponsors & Collaborators

• Boehringer Ingelheim: lead

Study Sites (1)

EUSTAR Registry

Basel, 4051, Switzerland

Location

Related Links

• Related Info

MeSH Terms

Conditions

Lung Diseases, Interstitial

Interventions

nintedanib

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 15, 2025
• First Posted: July 23, 2025
• Study Start: June 17, 2025
• Primary Completion (Estimated): December 31, 2033
• Study Completion (Estimated): December 31, 2033
• Last Updated: December 8, 2025

Record last verified: 2025-12

Data Sharing

• IPD Sharing: Will not share

Locations

CH (1)