Glucocorticoids Versus Placebo for the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis

NCT ID: NCT05674994

Last Updated: 2025-08-19

Basic Information

• Recruitment Status: RECRUITING
• Clinical Phase: PHASE3
• Total Enrollment: 110 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2023-10-26
• Study Completion Date: 2026-12-31

Brief Summary

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is associated with a poor prognosis, with a 3-month mortality rate of over 50%. To date, no treatment has been proven to be effective in AI-FPI. The interest of glucocorticoids is controversial and needs to be confirmed. This confirmation is mandatory to validate the improvement of the prognosis of EA-IPF under this treatment but also to search for unsuspected deleterious effects as it has been shown with immunosuppressants in stable idiopathic pulmonary fibrosis.

Detailed Description

Idiopathic pulmonary fibrosis (IPF) is the most frequent idiopathic interstitial lung disease (ILD) in adults. Its prognosis is poor with a median survival time ranging from 2 to 3 years. Acute exacerbation of IPF (IPF-AE) is defined as acute, clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormalities. Recently, diagnosis criteria were defined now allowing standardized diagnosis of IPF-AE and thus its study. IPF-AE is a major event of the disease having a 5 to 10% annual incidence. In-hospital mortality after IPF-AE exceeds 50% and current evidence suggests that up to 46% of deaths in IPF patients are associated with IPF-AE.

For the time being, no treatment has been proved to be effective in IPF-AE. While the clinical practice guideline suggests treatment with steroids, this recommendation is based only on expert opinion (low level evidence). Retrospective studies suggested the efficacy of thrombomodulin, cyclophosphamide or of therapeutic strategy including plasma exchange, rituximab and intravenous immunoglobulins. A recent Japanese randomized trial failed to show the efficacy of thrombomodulin alfa. Investigators performed a randomized trial assessing the role of cyclophosphamide on top of pulse steroid (EXAFIP-NCT02460588) and showed that cyclophosphamide did not reduce the 3-month mortality. A study assessing the effect of therapeutic plasma exchange, rituximab and intravenous immunoglobulins for severe form of IPF-AE patients admitted to Intensive Care Unit (ICU) is still ongoing (NCT03286556). Presently, the clinical benefit of corticosteroids is questioned. Indeed, 2 retrospective series reported an absence of outcome improvement by corticosteroids among IPF-AE patients and even suggested a potential detrimental outcome.

It is therefore necessary to set-up a placebo-controlled randomized trial: investigator's goal is to test the hypothesis that a corticosteroid treatment is highly efficient in IPF-AE, compared to placebo.

This underlines that, as no good evidence is available to support the use of glucocorticoids in IPF-AE, randomized controlled trials are also needed to address their efficacy and safety in this indication.

The choices of glucocorticoids' dosage, primary objective (mortality) and primary assessment criteria (all cause mortality rate at Day 30) are driven by investigator's previous study, EXAFIP. In this study, glucocorticoids dosage was as follow: intravenous methylprednisolone, 10 mg/kg/d (without exceeding 1000 mg/d), 3 days in a row shift to prednisone at 1 mg/kg/d for 1 week, and 0.75 mg/kg/d for 1 week, then 0.5 mg/kg/d for 1 week, and 0.25 mg/kg/d for 1 week, and 10 mg/d if weight > 65 kg; 7.5 mg if weight ≤ 65 kg until M6. The 1-month mortality of patient under this high dose of glucocorticoids was 20%.

In view of the poor prognosis of IPF-AE, it seems also important to evaluate the effect of treatment on overall mortality at Day 90.

Conditions

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Study Design

• Allocation Method: RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: TREATMENT
• Blinding Strategy: DOUBLE

Study Groups

MethylPrednisone/Prednisone

• Day 1, 2 and 3: Intravenous Methylprednisolone 10 mg/kg/d (without exceeding 1000 mg/d) Vials of injectable solution of methylprednisolone® are diluted in 100 ml of NaCl 0.9% or G5%. Perfusion duration is between 20 to 30 minutes. The commercialized form for methylprednisolone injectable solution is not imposed and is taken from the stock of each pharmacy of the participating centers.
• From day 4 to Day 30: Oral Prednisone slow tappering

• 1 mg/kg/d for 7 days
• 0.5 mg/kg/d for 7 days
• 0.25 mg/kg/d for 7 days,
• 10 mg/d until Day 30. For 10mg/kg, 1 mg/kg, 0.5 mg/kg, 0.25 mg/kg; rounding to 5 decimal lower if decimal ≤ 7 and the top ten if decimal ≥ 8.

Group Type: EXPERIMENTAL

Methylprednisone/Prednisone

Intervention Type: DRUG

Patients will be enrolled during their hospitalization in pneumology department, as part of current practice, within 7 days of the screening visit. The investigator will perform randomization by connecting to the eCRF, randomization be stratified for the severity of IPF and the treatment with antifibrotic therapy (Nintedanib or Pirfenidone) (yes/no).

If patient is randomized in Glucocorticoids Group:

• Day 1, 2 and 3: Intravenous Methylprednisolone 10 mg/kg/d (without exceeding 1000 mg/d). Vials of injectable solution of methylprednisolone® are diluted in 100 ml of NaCl 0.9% or G5%. Perfusion duration is between 20 to 30 minutes.
• From day 4 to Day 30: Oral Prednisone slow tappering

• 1 mg/kg/d for 7 days
• 0.5 mg/kg/d for 7 days
• 0.25 mg/kg/d for 7 days,
• 10 mg/d until Day 30. For 10mg/kg, 1 mg/kg, 0.5 mg/kg, 0.25 mg/kg; rounding to 5 decimal lower if decimal ≤ 7 and the top ten if decimal ≥ 8.

Placebo

• Day 1, 2 and 3: Intravenous Methylprednisolone-Placebo
• From Day 4 to Day 30: Oral Prednisone-Placebo

Group Type: PLACEBO_COMPARATOR

Placebo

Intervention Type: OTHER

Patients will be enrolled during their hospitalization in pneumology department, as part of current practice, within 7 days of the screening visit. The investigator will perform randomization by connecting to the eCRF, randomization be stratified for the severity of IPF and the treatment with antifibrotic therapy (Nintedanib or Pirfenidone) (yes/no).

If patient is randomized in Placebo Group:

Day 1, 2 and 3: Intravenous Methylprednisolone-Placebo From Day 4 to Day 30: Oral Prednisone-Placebo The Methylprednisolone-Placebo corresponds to 100 ml of NaCl 0.9 % or G5%. Perfusion duration is between 20 to 30 minutes.

For the Prednisone-Placebo, the placebo was an oral solution formulated with a bittering agent (pharmaceutical excipient). Specifically, in place of prednisone, sucrose octaacetate (defined as a GRAS-'Generally Recognized as Safe' excipient by the EMA) was used at 5 mg/mL.

Interventions

Methylprednisone/Prednisone

Patients will be enrolled during their hospitalization in pneumology department, as part of current practice, within 7 days of the screening visit. The investigator will perform randomization by connecting to the eCRF, randomization be stratified for the severity of IPF and the treatment with antifibrotic therapy (Nintedanib or Pirfenidone) (yes/no).

If patient is randomized in Glucocorticoids Group:

• Day 1, 2 and 3: Intravenous Methylprednisolone 10 mg/kg/d (without exceeding 1000 mg/d). Vials of injectable solution of methylprednisolone® are diluted in 100 ml of NaCl 0.9% or G5%. Perfusion duration is between 20 to 30 minutes.
• From day 4 to Day 30: Oral Prednisone slow tappering

• 1 mg/kg/d for 7 days
• 0.5 mg/kg/d for 7 days
• 0.25 mg/kg/d for 7 days,
• 10 mg/d until Day 30. For 10mg/kg, 1 mg/kg, 0.5 mg/kg, 0.25 mg/kg; rounding to 5 decimal lower if decimal ≤ 7 and the top ten if decimal ≥ 8.

Intervention Type: DRUG

Placebo

Patients will be enrolled during their hospitalization in pneumology department, as part of current practice, within 7 days of the screening visit. The investigator will perform randomization by connecting to the eCRF, randomization be stratified for the severity of IPF and the treatment with antifibrotic therapy (Nintedanib or Pirfenidone) (yes/no).

If patient is randomized in Placebo Group:

Day 1, 2 and 3: Intravenous Methylprednisolone-Placebo From Day 4 to Day 30: Oral Prednisone-Placebo The Methylprednisolone-Placebo corresponds to 100 ml of NaCl 0.9 % or G5%. Perfusion duration is between 20 to 30 minutes.

For the Prednisone-Placebo, the placebo was an oral solution formulated with a bittering agent (pharmaceutical excipient). Specifically, in place of prednisone, sucrose octaacetate (defined as a GRAS-'Generally Recognized as Safe' excipient by the EMA) was used at 5 mg/mL.

Intervention Type: OTHER

Eligibility Criteria

Inclusion Criteria

1. Patient is ≥ 18 years of age

2. IPF or IPF (likely) diagnosis defined on 2018 international recommendations

3. Definite or suspected Acute Exacerbation defined by the international working group criteria after exclusion of alternative diagnoses of acute worsening

*The criteria of IPF-AE are as follows:

• Previous or concurrent diagnosis of IPF (a)
• Acute worsening or development of dyspnea typically < 1-month duration
• Computed tomography with new bilateral ground-glass opacity and/or consolidation superimposed on a background pattern consistent with usual interstitial pneumonia pattern (b)
• Deterioration not fully explained by cardiac failure or fluid overload Patients who fail to meet all 4 criteria due to missing computed tomography should be considered as having "suspected Acute Exacerbation".

1. If the diagnosis of IPF is not previously established, this criterion can be met by the presence of radiologic and/or histopathologic changes consistent with usual interstitial pneumonia pattern on the current evaluation.

2. If no previous computed tomography is available, the qualifier "new" can be dropped from the third criterion.

4. For women of childbearing age: efficient contraception for the duration of the study*

*Effective contraception is defined as any contraceptive method that is used consistently and appropriately and has a low failure rate (i.e., less than 1% per year)

5. Affiliation to the social security

6. Patient able to understand and sign a written informed consent form or in case of incapacity of the patient to a relative whom understand and sign a written informed consent form

Exclusion Criteria

1. Identified etiology for acute worsening (i.e.: infectious disease)

2. Known hypersensitivity to glucocorticoids or to any component of the study treatment

3. Patient requiring mechanical ventilation or already on mechanical ventilation

4. Active bacterial, viral, fungal or parasitic infection. On swab collected, only positive for SARS-CoV-2, Influenzae A, Influenzae B and Respiratory Syncytial Virus (RSV) result, are considered active viral infection. The others viruses (i.e. Rhinovirus, Adenovirus…) are not considered to be responsible of pneumonia.

5. Active cancer

6. Patient on a lung transplantation waiting list

7. Treatment with glucocorticoids > 1 mg/kg/d from more than 7 days in the last 15 days

8. Patient participating to another interventional clinical trial

9. Documented pregnancy or lactation

10. Patient under tutorship or curatorship

11. Patient deprived of liberty

12. Patient under court protection

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Fondation Hôpital Saint-Joseph

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Jean-Marc NACCACHE

Role: STUDY_DIRECTOR

Fondation Hôpital Saint-Joseph

Locations

CHU ANgers

Angers, , France

Site Status: RECRUITING

CHU de Besancon

Besançon, , France

Site Status: RECRUITING

Hôpital Avicenne

Bobigny, , France

Site Status: NOT_YET_RECRUITING

CHU BOrdeaux

Bordeaux, , France

Site Status: RECRUITING

CHU Caen

Caen, , France

Site Status: RECRUITING

CHU Clermont-Ferrand

Clermont-Ferrand, , France

Site Status: NOT_YET_RECRUITING

CHIC

Créteil, , France

Site Status: RECRUITING

CHU de Dijon

Dijon, , France

Site Status: RECRUITING

CHU Grenoble

Grenoble, , France

Site Status: NOT_YET_RECRUITING

CHRU Lille

Lille, , France

Site Status: NOT_YET_RECRUITING

Hospices Civils de Lyon

Lyon, , France

Site Status: RECRUITING

Hôpital Nord

Marseille, , France

Site Status: RECRUITING

CHU de Montpellier

Montpellier, , France

Site Status: NOT_YET_RECRUITING

CHU Nancy

Nancy, , France

Site Status: RECRUITING

CHU de Nantes

Nantes, , France

Site Status: NOT_YET_RECRUITING

CHU Nice

Nice, , France

Site Status: NOT_YET_RECRUITING

Hôpital Paris Saint-Joseph

Paris, , France

Site Status: RECRUITING

Hôpital Bichat

Paris, , France

Site Status: RECRUITING

Hôpital Européen Georges Pompidou

Paris, , France

Site Status: RECRUITING

Hôpital FOCH

Paris, , France

Site Status: NOT_YET_RECRUITING

Hôpital Kremiln Bicetre

Paris, , France

Site Status: RECRUITING

Hôpital Saint-Louis

Paris, , France

Site Status: NOT_YET_RECRUITING

Hôpital Tenon

Paris, , France

Site Status: RECRUITING

CHU Reims

Reims, , France

Site Status: RECRUITING

CHU Rennes

Rennes, , France

Site Status: RECRUITING

CHU Rouen

Rouen, , France

Site Status: NOT_YET_RECRUITING

CHU Strasbourg

Strasbourg, , France

Site Status: NOT_YET_RECRUITING

CHU Toulouse

Toulouse, , France

Site Status: RECRUITING

CHU Tours

Tours, , France

Site Status: NOT_YET_RECRUITING

Countries

France

Central Contacts

Jean-Marc NACCACHE, MD

Role: CONTACT

jmnaccache@ghpsj.fr

144126747 ext. +33

Helene BEAUSSIER, PharmD, PhD

Role: CONTACT

crc@ghpsj.fr

144127883 ext. +33

Facility Contacts

Frederic GAGNADOUX

Role: primary

Mathilde DUPREZ, MD

Role: primary

Hilario NUNES, MD

Role: primary

Elodie BLANCHARD, MD

Role: primary

Emmanuel BERGOT, MD

Role: primary

Camille ROLLAND DEBORD, MD

Role: primary

Quentin GIBIOT, MD

Role: primary

Philippe BONNIAUD, MD

Role: primary

Sebastien QUETANT, MD

Role: primary

Victor VALENTIN, MD

Role: primary

Vincent COTTIN, MD

Role: primary

Martine REAYNAUD GAUBERT, MD

Role: primary

Arnaud BOURDIN, MD

Role: primary

Anne GUILLAUMOT, MD

Role: primary

Stéphanie DIROU, MD

Role: primary

Sylvie LEROY, MD

Role: primary

Jean-Marc NACCACHE, MD

Role: primary

Bruno CRESTANI, MD

Role: primary

Jean PASTRE, MD

Role: primary

Alexandre CHABROL, MD

Role: primary

David MONTANI, MD

Role: primary

Abdellatif TAZI, MD

Role: primary

Jacques CADRANEL, MD

Role: primary

Francois LEBARGY, MD

Role: primary

Stephane JOUNEAU, MD

Role: primary

Stéphane DOMINIQUE, MD

Role: primary

Sandrine HIRSCHI, MD

Role: primary

Gregoire PREVOT, MD

Role: primary

Thomas FLAMENT, MD

Role: primary

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Other Identifiers

EXAFIP2

Identifier Type: -

Identifier Source: org_study_id