Investigating the efficacy and tolerability of nintedanib therapy in idiopathic-inflammatory-myopathy-related interstitial lung disease

A real-world analysis of Nintedanib therapy in Idiopathic-inflammatory-myopathy-related Interstitial Lung Disease (NIILD): an efficacy and tolerability pilot study

Status

Active, not recruiting

Phases

Unknown

Study type

Interventional

Source

ISRCTN

Registry ID

ISRCTN10507540

Enrollment

100

Registered

2020-07-30

Start date

2018-01-01

Completion date

Unknown

Last updated

2021-06-07

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Interstitial lung disease in idiopathic inflammatory myopathy Respiratory Interstitial pulmonary disease in Inflammatory myopathy

Interventions

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 rec

Eligibility

Sex/Gender

All

Inclusion criteria

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

Exclusion criteria

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

Design outcomes

Primary

Measure	Time frame
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.	—

Measure

Time frame

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.

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Secondary

Measure	Time frame
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. 2. Complications of pulmonary infection, measured and recorded in the process of follow-up, at the end of follow-up. Pulmonary infection will be identified based on International Statistical Classification of Diseases, 10th revision (ICD-10)-coded discharge diagnosis of community-acquired pneumonia (CAP), hospital-acquired pneumonia (HAP), pulmonary fungal infection or pulmonary infection. Responsible pathogens were recognized based on repeated cultures/smears of bronchoalveolar lavage fluid (BALF) or sputum before related treatment. 3. Difference in immunosuppressive regimen such as dose of steroid, whether the potent and expensive intravenous immunoglobulin was used, etc. measured and recorded in the process of follow-up, at the end of follow-up 4. Tolerability of nintedanib will be measured as the incidence of adverse events, the incidence of dose reduction, or discontinuation due to adverse events, at the end of follow-up	—

Measure

Time frame

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. 2. Complications of pulmonary infection, measured and recorded in the process of follow-up, at the end of follow-up. Pulmonary infection will be identified based on International Statistical Classification of Diseases, 10th revision (ICD-10)-coded discharge diagnosis of community-acquired pneumonia (CAP), hospital-acquired pneumonia (HAP), pulmonary fungal infection or pulmonary infection. Responsible pathogens were recognized based on repeated cultures/smears of bronchoalveolar lavage fluid (BALF) or sputum before related treatment. 3. Difference in immunosuppressive regimen such as dose of steroid, whether the potent and expensive intravenous immunoglobulin was used, etc. measured and recorded in the process of follow-up, at the end of follow-up 4. Tolerability of nintedanib will be measured as the incidence of adverse events, the incidence of dose reduction, or discontinuation due to adverse events, at the end of follow-up

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Countries

China

Contacts

Public ContactJunyu Liang

collinliangzju@zju.edu.cn+86 15168302715

Outcome results

None listed

Source: ISRCTN (via WHO ICTRP) · Data processed: Feb 4, 2026