Physical Activity and Pulmonary Rehabilitation for Interstitial Lung Disease

(Actigraphy Trial)

Overseen By Elisabeth Ballans, BSN

Age: 18+

Sex: Any

Trial Phase: Academic

Sponsor: Debabrata Bandyopadhyay

Must be taking: Immunosuppressants, Antifibrotics

Must not be taking: Full-dose anticoagulants

Disqualifiers: Pulmonary hypertension, Metastatic malignancy, Severe COPD, others

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Compensation: Varies

Number of Visits: 6

Duration: 52 weeks

70 Participants Needed

Trial Summary

Will I have to stop taking my current medications?

The trial requires that participants be on a stable dose of immunosuppressants and/or antifibrotic therapy for at least 30 days before joining. If you're on these medications, you won't need to stop them, but other medications aren't specified in the protocol.

What data supports the effectiveness of the treatment for interstitial lung disease?

Research shows that pulmonary rehabilitation can improve exercise capacity and quality of life in lung disease patients, while antifibrotic drugs like nintedanib and pirfenidone help slow disease progression in idiopathic pulmonary fibrosis. Combining these treatments may help maintain these benefits over the long term. ^{1 2 3 4 5}

Is the treatment for interstitial lung disease safe?

The drugs nintedanib and pirfenidone, used for treating lung conditions like idiopathic pulmonary fibrosis, are generally safe but can cause side effects, mostly mild and manageable, such as stomach issues. About 20% of patients might stop using them due to side effects, but regular check-ups can help manage these issues. ^{4 6 7 8 9}

How is the treatment for interstitial lung disease with anti-fibrotic therapy, physical activity, and pulmonary rehabilitation different from other treatments?

This treatment is unique because it combines anti-fibrotic drugs like Nintedanib or Pirfenidone, which slow lung damage, with physical activity and pulmonary rehabilitation to improve exercise capacity and quality of life. The combination aims to maintain long-term benefits, unlike other treatments that may not address both lung function and physical fitness together. ^{3 5 10 11 12}

What is the purpose of this trial?

The planned study is a prospective cohort interventional study in IPF and PF-ILD patients after initiating anti-fibrotic therapy and pulmonary rehabilitation. The study aims to investigate if accelerometer measured PA parameters, such as total daily steps, moderate-vigorous PA demonstrate significant and sustained changes longitudinally from baseline in this cohort and can predict disease progression. The study also explores if the actigraphic PA indices correlate with patients' quality of life, change in six-minute walk distance (6MWD), GAP score, fatigue score, change in patients' dyspnea score/scale, radiographic extent of the disease, and pulmonary function test parameters. The study is exploratory in nature. It will provide vital information for clinical as well as research purposes. Clinically, accelerometer measured PA can be utilized for therapeutic target and prognostication, helping to develop patient centric care. The measured indices can also be useful to serve as meaningful endpoints to plan larger and definitive studies in IPF and PF-ILD patients.

Eligibility Criteria

This trial is for adults over 40 with certain types of lung scarring diseases who can walk more than 150 meters and have not been on anti-scarring lung medication for long. They must be able to do a pulmonary rehab program, have specific levels of lung function, and agree to use contraception if applicable.

Inclusion Criteria

Patients willing to provide consent and comply with study procedures

I haven't used antifibrotic drugs or have been on them for less than 3 months.

I can walk more than 150 meters without stopping.

Exclusion Criteria

My BMI is over 35.

I have been on antifibrotic therapy for my lung condition for over 6 months.

I have severe or very severe COPD.

Timeline

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Baseline Assessment

Participants undergo baseline assessments including actigraphy for seven continuous days

1 week

1 visit (in-person)

Treatment

Participants begin or continue nintedanib treatment and participate in pulmonary rehabilitation

52 weeks

5 visits (in-person) at weeks 0, 12, 24, 36, and 52

Follow-up

Participants are monitored for safety and effectiveness after treatment

4 weeks

Treatment Details

Interventions

• Anti-fibrotic Therapy
• Physical Activity
• Pulmonary Rehabilitation

Trial Overview The study tests how daily physical activity measured by an accelerometer affects the quality of life and disease progression in patients starting anti-fibrotic therapy and pulmonary rehabilitation. It will check if increased activity relates to better walking distance, fatigue, breathlessness scores, X-ray findings, and lung function tests.

Anti-fibrotic Therapy is already approved in European Union, United States for the following indications:

Approved in European Union as Nintedanib for:

• Idiopathic Pulmonary Fibrosis (IPF)
• Scleroderma-related lung fibrosis
• Progressive non-IPF fibrotic lung disease

Approved in European Union as Pirfenidone for:

• Idiopathic Pulmonary Fibrosis (IPF)
• Progressive unclassified fibrotic lung disease

Approved in United States as Nintedanib for:

• Idiopathic Pulmonary Fibrosis (IPF)
• Scleroderma-related lung fibrosis
• Progressive non-IPF fibrotic lung disease

Approved in United States as Pirfenidone for:

• Idiopathic Pulmonary Fibrosis (IPF)
• Progressive unclassified fibrotic lung disease

Who Is Running the Clinical Trial?

Debabrata Bandyopadhyay

Lead Sponsor

Trials

1

Recruited

70+

University of South Florida

Lead Sponsor

Trials

433

Recruited

198,000+

Findings from Research

Pirfenidone and nintedanib have shown significant benefits in slowing disease progression in idiopathic pulmonary fibrosis (IPF), supported by multiple randomized studies.

While there is weak evidence from observational studies suggesting potential benefits of these antifibrotic agents in non-IPF interstitial lung diseases (ILDs), ongoing randomized controlled trials are being conducted to further evaluate their efficacy in these conditions.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Antifibrotic drugs as treatment of nonidiopathic pulmonary fibrosis interstitial pneumonias: the time is now (?). Kreuter, M., Wälscher, J., Behr, J.[2018]

In a study of 144 patients with idiopathic pulmonary fibrosis (IPF), both pirfenidone and nintedanib showed similar adherence rates, with slight improvements in adherence for pirfenidone over two years.

The study found that patients who responded well to treatment (measured by forced vital capacity, FVC) had adherence rates above 90%, indicating that effective treatment response is linked to better adherence to medication.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Adherence, Persistence, and Effectiveness in Real Life. Multicenter Long-Term Study on the Use of Pirfenidone and Nintedanib in the Treatment of Idiopathic Pulmonary Fibrosis. Santoleri, F., Auriemma, L., Spacone, A., et al.[2022]

A pilot study involving 21 patients with fibrotic interstitial lung disease showed that a 12-week home-based pulmonary rehabilitation program improved patient-reported outcomes related to dyspnea and quality of life, although the results were not statistically significant due to the small sample size.

Qualitative feedback indicated that participants, despite having varying expectations and disease severity, found the program beneficial for coping with their condition, highlighting the importance of psychological support alongside physical rehabilitation.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Home-Based Pulmonary Rehabilitation and Health Coaching in Fibrotic Interstitial Lung Disease: IMPLEMENTATION AND QUALITATIVE ASSESSMENT OF A PILOT TELEHEALTH PROGRAM. Duke, JD., Moua, T., Ridgeway, JL., et al.[2023]

In a study of 104 patients with idiopathic pulmonary fibrosis (IPF) receiving antifibrotic therapies, progression-free survival (PFS) at 6 months was significantly better (75.0%) compared to a control group (56.3%), indicating the effectiveness of these treatments in clinical practice.

The study also found that while the 12-month mortality rates were similar between the antifibrotic group (25.3%) and the control group (35.5%), nintedanib showed lower early treatment discontinuation rates compared to pirfenidone, suggesting it may be a preferable first-line therapy.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Real-world retrospective observational study exploring the effectiveness and safety of antifibrotics in idiopathic pulmonary fibrosis. Wright, WA., Crowley, LE., Parekh, D., et al.[2021]

The FITNESS study is a pioneering trial that will assess the long-term effects of combining pulmonary rehabilitation with nintedanib treatment in patients with idiopathic pulmonary fibrosis (IPF), involving 84 participants over a 12-month period.

The primary goal is to evaluate improvements in exercise capacity, specifically the 6-minute walk distance, to determine if nintedanib helps maintain the benefits of pulmonary rehabilitation in the long term.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Protocol for long-term effect of pulmonary rehabilitation under nintedanib in idiopathic pulmonary fibrosis. Nishiyama, O., Kataoka, K., Ando, M., et al.[2022]

Nintedanib and pirfenidone, the two approved anti-fibrotic drugs for idiopathic pulmonary fibrosis (IPF), have a comparable safety profile, with most side effects being mild and manageable based on data from clinical trials and post-marketing reports.

Despite their safety, about 20% of patients may stop treatment due to side effects, highlighting the importance of patient counseling and regular follow-ups to help manage these issues.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

The safety of new drug treatments for idiopathic pulmonary fibrosis. Fletcher, S., Jones, MG., Spinks, K., et al.[2022]

Nintedanib and pirfenidone, two antifibrotic agents approved for idiopathic pulmonary fibrosis (IPF), have shown acceptable tolerability in clinical trials, but they come with potential side effects that need careful evaluation for each patient.

Long-term data indicates that both nintedanib and pirfenidone have comparable tolerability profiles, emphasizing the importance of patient counseling and monitoring for adverse events during treatment to guide therapy decisions.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

An updated safety review of the drug treatments for idiopathic pulmonary fibrosis. Sgalla, G., Comes, A., Richeldi, L.[2022]

In a study of 176 patients with idiopathic pulmonary fibrosis treated with either pirfenidone or nintedanib, adverse drug reactions (ADRs) were reported in 78.3% of those on pirfenidone and 70.5% on nintedanib, indicating a high incidence of side effects for both medications.

The incidence of serious ADRs was about 33 per 100 person-years for both drugs, while non-serious ADRs were more frequent, with 102 per 100 person-years for pirfenidone and 130 per 100 person-years for nintedanib, highlighting the need for careful monitoring of patients on these treatments.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Real-world safety profiles of pirfenidone and nintedanib in idiopathic pulmonary fibrosis patients. Fournier, D., Jouneau, S., Bouzillé, G., et al.[2022]

In a real-world study of patients with Idiopathic Pulmonary Fibrosis (IPF), both pirfenidone and nintedanib were found to have common side effects, primarily gastrointestinal, but these were manageable in most cases.

Despite treating older patients with more severe disease and additional health issues, the discontinuation rates due to side effects were similar to those observed in clinical trials, supporting the safety and efficacy of these treatments in broader patient populations.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Real World Experiences: Pirfenidone and Nintedanib are Effective and Well Tolerated Treatments for Idiopathic Pulmonary Fibrosis. Hughes, G., Toellner, H., Morris, H., et al.[2020]

Longer durations of pulmonary rehabilitation significantly improve functional capacity and quality of life for patients with interstitial lung diseases (ILD).

Tailored educational components alongside supervised exercise training enhance the effectiveness of pulmonary rehabilitation, while certain techniques like pursed-lip breathing may not be beneficial.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Pulmonary rehabilitation in interstitial lung diseases. Makhdami, N., Farooqi, M., Thom-Fernandes, C., et al.[2021]

Patients with fibrotic idiopathic interstitial pneumonia showed improvements in exercise tolerance, anxiety, depression, and quality of life after participating in a pulmonary rehabilitation program.

The rehabilitation program was effective without increasing the patients' daily physical activity levels, suggesting that structured rehabilitation can enhance well-being without requiring more physical exertion in their daily lives.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Effects of pulmonary rehabilitation on daily life physical activity of fibrotic idiopathic interstitial pneumonia patients. Wallaert, B., Masson, N., Le Rouzic, O., et al.[2022]

Pulmonary rehabilitation significantly improves exercise tolerance in patients with idiopathic pulmonary fibrosis, as evidenced by a 35.2-meter increase in the 6-minute walking distance compared to usual care, based on 11 trials with 549 participants.

The rehabilitation program also enhances quality of life, demonstrated by a reduction in the St George's Respiratory Questionnaire scores, although the overall quality of evidence is considered low to moderate, indicating a need for further large-scale studies.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Clinical evidence for improving exercise tolerance and quality of life with pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis: A systematic review and meta-analysis. Lei, S., Li, X., Xie, Y., et al.[2022]

References

1. United States pubmed.ncbi.nlm.nih.gov

Antifibrotic drugs as treatment of nonidiopathic pulmonary fibrosis interstitial pneumonias: the time is now (?). [2018]

2. United States pubmed.ncbi.nlm.nih.gov

Adherence, Persistence, and Effectiveness in Real Life. Multicenter Long-Term Study on the Use of Pirfenidone and Nintedanib in the Treatment of Idiopathic Pulmonary Fibrosis. [2022]

3. United States pubmed.ncbi.nlm.nih.gov

Home-Based Pulmonary Rehabilitation and Health Coaching in Fibrotic Interstitial Lung Disease: IMPLEMENTATION AND QUALITATIVE ASSESSMENT OF A PILOT TELEHEALTH PROGRAM. [2023]

4. England pubmed.ncbi.nlm.nih.gov

Real-world retrospective observational study exploring the effectiveness and safety of antifibrotics in idiopathic pulmonary fibrosis. [2021]

5. England pubmed.ncbi.nlm.nih.gov

Protocol for long-term effect of pulmonary rehabilitation under nintedanib in idiopathic pulmonary fibrosis. [2022]

6. England pubmed.ncbi.nlm.nih.gov

The safety of new drug treatments for idiopathic pulmonary fibrosis. [2022]

7. England pubmed.ncbi.nlm.nih.gov

An updated safety review of the drug treatments for idiopathic pulmonary fibrosis. [2022]

8. England pubmed.ncbi.nlm.nih.gov

Real-world safety profiles of pirfenidone and nintedanib in idiopathic pulmonary fibrosis patients. [2022]

9. Switzerland pubmed.ncbi.nlm.nih.gov

Real World Experiences: Pirfenidone and Nintedanib are Effective and Well Tolerated Treatments for Idiopathic Pulmonary Fibrosis. [2020]

10. United States pubmed.ncbi.nlm.nih.gov

Pulmonary rehabilitation in interstitial lung diseases. [2021]

11. England pubmed.ncbi.nlm.nih.gov

Effects of pulmonary rehabilitation on daily life physical activity of fibrotic idiopathic interstitial pneumonia patients. [2022]

12. England pubmed.ncbi.nlm.nih.gov

Clinical evidence for improving exercise tolerance and quality of life with pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis: A systematic review and meta-analysis. [2022]