Investigating the efficacy and tolerability of nintedanib therapy in idiopathic-inflammatory-myopathy-related interstitial lung disease
| ISRCTN | ISRCTN10507540 |
|---|---|
| DOI | https://doi.org/10.1186/ISRCTN10507540 |
| ClinicalTrials.gov (NCT) | Nil known |
| Clinical Trials Information System (CTIS) | Nil known |
| Protocol serial number | Nil known |
| Sponsors | National Natural Science Foundation of China, Natural Science Foundation of Zhejiang Province |
| Funders | National Natural Science Foundation of China, Natural Science Foundation of Zhejiang Province |
- Submission date
- 24/07/2020
- Registration date
- 30/07/2020
- Last edited
- 24/05/2021
- Recruitment status
- No longer recruiting
- Overall study status
- Completed
- Condition category
- Respiratory
Plain English summary of protocol
Background and study aims
Interstitial lung disease (ILD) is a group of lung conditions that affects the network of tissue (interstitium) that supports the air sacs of the lungs. ILD can cause stiffness in the lungs and lead to shortness of breath and death.
Idiopathic inflammatory myopathy (IIM) is a group of autoimmune disorders that cause inflammation of the muscles used for movement and lead to progressive muscle weakness. IIM can also affect non-muscular areas and the lung interstitium is the ILD is the most common non-muscular area affected. It is reported that 78% of IIM patients will develop ILD.
Idiopathic-inflammatory-myopathy-related interstitial lung disease (IIM-ILD) is frequently aggressive and may not respond to conventional therapies including glucocorticoids and immunosuppressive drugs. Meanwhile, the rapid progression of interstitial lung disease (RP-ILD) is a major cause of death in IIM patients. It is therefore necessary to search for more successful treatment for IIM-ILD.
Nintedanib has been proven effective and relatively safe in idiopathic pulmonary fibrosis and systemic-sclerosis-associated interstitial lung disease, however, its efficacy and tolerability are not known in adult idiopathic-inflammatory-myopathy-related interstitial lung disease (IIM-ILD). This study aims to assess how effective and well-tolerated nintedanib is in patients with IIM-ILD.
Who can participate?
Adult patients who regularly attend the outpatient or inpatient department of the study center with a diagnosis of IIM-ILD.
What does the study involve?
Patients who agree to participate will receive nintedanib therapy (150 mg, twice daily by mouth) in addition to standard treatment. Those who do not agree will receive standard immunosuppressive medication only.
What are the possible benefits and risks of participating?
Patients who received the nintedanib therapy may benefit from a therapeutic effect of nintedanib of slowing the progress of interstitial lung disease, reducing the chance of rapid progression, and improved survival. However, participants might experience side effects such as diarrhea and hepatic (liver) insufficiency as a result of this medication.
Where is the study run from?
The First Affiliated Hospital, College of Medicine, Zhejiang University (China)
When is the study starting and how long is it expected to run for?
From December 2017 to April 2020
Who is funding the study?
The National Natural Science Foundation of China (81701602) and Natural Science Foundation of Zhejiang Province (LQ20H100003) (China)
Who is the main contact?
Dr Junyu Liang
collinliangzju@zju.edu.cn
Contact information
Public
79 Qingchun Road
Hangzhou
310003
China
 ORCID ID |
0000-0003-1050-1274 |
|---|---|
| Phone | +86 15168302715 |
| collinliangzju@zju.edu.cn |
Study information
| Primary study design | Interventional |
|---|---|
| Study design | Single-center interventional non-randomized real-world analysis pilot study |
| Secondary study design | Non randomised study |
| Study type | Participant information sheet |
| Scientific title | A real-world analysis of Nintedanib therapy in Idiopathic-inflammatory-myopathy-related Interstitial Lung Disease (NIILD): an efficacy and tolerability pilot study |
| Study acronym | NIILD |
| Study objectives | Nintedanib is efficient and relatively safe in adult idiopathic-inflammatory-myopathy-related interstitial lung disease. |
| Ethics approval(s) | Approved 28/05/2020, the Research Ethics Committee of the First Affiliated Hospital of Zhejiang University (FAHZJU) (#79 Qingchun Road, Hangzhou, Zhejiang Province, P.R.China, 310003; +86 (0)571-87236629; zyiitlunli@163.com; kjkzyyy@163.com), ref: 2020-200, 2018-224 |
| Health condition(s) or problem(s) studied | Interstitial lung disease in idiopathic inflammatory myopathy |
| Intervention | Participants who agree to participate will receive nintedanib (150 mg, twice daily, orally) in addition to traditional immunosuppressive therapy. Patients who do not agree to participate will only receive traditional immunosuprresive medications. The duration of treatment and follow-up should be at least 6 months. |
| Intervention type | Drug |
| Phase | Not Specified |
| Drug / device / biological / vaccine name(s) | Nintedanib |
| Primary outcome measure(s) |
1. Occurrence of rapid progression of interstitial lung disease (RP-ILD), measured by the number of participants meeting the criteria of RP-ILD, assessed at the end of the follow-up. Patients with RP-ILD are defined as those presenting with progressive dyspnea and progressive hypoxemia, a worsening of interstitial change on the chest radiograph within 1 month after the initial visit or onset of respiratory symptoms. |
| Key secondary outcome measure(s) |
1. Time to death from any cause, measured through recording of survival or not and the length of follow-up, at the end of the follow-up. Cause of death will also be recorded in the follow-up such as exacerbation of interstitial lung disease, cardiopulmonary failure of unknown origin, pulmonary artery hypertension, etc. |
| Completion date | 30/04/2020 |
Eligibility
| Participant type(s) | Patient |
|---|---|
| Age group | Adult |
| Lower age limit | 18 Years |
| Sex | All |
| Target sample size at registration | 100 |
| Total final enrolment | 151 |
| Key inclusion criteria | 1. Aged ≥18 years 2. Diagnosis of dermatomyositis, polymyositis or amyopathic dermatomyositis that meets the 2017 ACR/EULAR classification criteria 3. Attending a regular outpatient visit or hospitalization in the First Affiliated Hospital, College of Medicine, Zhejiang University |
| Key exclusion criteria | 1. Overlap syndromes with other connective tissue diseases (CTDs) 2. Attending outpatient visit or hospitalization for reasons unrelated to myositis and its complications, such as fracture, pregnancy, acquired immunodeficiency syndrome, cataract, and etc. 3. Previous use of nintedanib, or previous/present use of pirfenidone 4. Loss to follow-up without death from any cause within 6 months after the initial outpatient visit or hospitalization |
| Date of first enrolment | 01/01/2018 |
| Date of final enrolment | 31/10/2019 |
Locations
Countries of recruitment
- China
Study participating centre
79 Qingchun Road
Hangzhou
310003
China
Results and Publications
| Individual participant data (IPD) Intention to share | Yes |
|---|---|
| IPD sharing plan summary | Other |
| IPD sharing plan | Data including therapy duration, doses, adverse events, age, and sex of patients receiving nintedanib therapy will be available as supplements of the future publication. Other parts of the data in this study will be provided upon request. |
Study outputs
| Output type | Details | Date created | Date added | Peer reviewed? | Patient-facing? |
|---|---|---|---|---|---|
| Results article | 03/02/2021 | 24/05/2021 | Yes | No | |
| Participant information sheet | Participant information sheet | 11/11/2025 | 11/11/2025 | No | Yes |
Editorial Notes
24/05/2021: The following changes have been made:
1. Publication reference added.
2. The final enrolment number has been added from the reference.
29/07/2020: Trial’s existence confirmed by the Research Ethics Committee of the First Affiliated Hospital of Zhejiang University.
