Intravenous Immunoglobulin for the Treatment of Acute Exacerbations of Idiopathic Pulmonary Fibrosis

ConditionsIdiopathic Pulmonary Fibrosis Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Trial statusRecruiting

Trial phasePhase 3

Trial typeInterventional

Biological sexAll

Age18+

SponsorArgyrios Tzouvelekis

About this trial

Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) are sudden and severe worsening episodes that can be life-threatening. Currently, no treatment has been proven to clearly improve outcomes during these events. Inflammation and immune system imbalance are thought to play an important role in causing AE-IPF. Early clinical experience suggests that intravenous immunoglobulin (IVIG) can be beneficial for patients suffering from AE-IPF. This clinical trial aims to determine whether adding IVIG to usual treatment can improve outcomes for patients hospitalized with AE-IPF.

Eligibility criteria

This trial does not accept healthy volunteers

Qualifiers

Patients ≥ 18 years of age

Patients with IPF diagnosis that fulfils ATS/ERS Consensus Criteria.

Patients hospitalised with a definite or suspected AE-IPF diagnosis, as defined by the international working group criteria and as ascertained by the responsible Primary Investigator.

Previous or concurrent diagnosis of IPF

Disqualifiers

Patients with acute worsening due to uncontrolled heart failure or pulmonary embolism.

Patients with known hypersensitivity to corticosteroids, IVIG or any component of the study treatment.

Patients with known IgA deficiency (IgA level <7 mg/dL)- to preclude IVIG reactions.

Patients without a definite diagnosis of IPF or AE-IPF based on clinical, radiological, laboratory evaluation, and multidisciplinary discussion.

Trial design

Design model

Parallel

Treatments tested in this trial

Intravenous immunoglobulin (IVIG)
Drug
Total dose of 1 g/kg, divided over three consecutive days. The infusion will start at a rate of 0.5 mg/kg/hour for the first 15 minutes and, if no adverse reaction occurs, the rate will then be gradually increased step-wise as tolerated. Premedication with acetaminophen and levocetirizine. Usual treatment will be co-administered, as described.
Usual treatment
Drug
1. Corticosteroids: A pulse regimen of IV methylprednisolone at 250 mg daily (days 1 to 3), with no additional corticosteroids thereafter. 2. Antibiotics: Empirical broad-spectrum antibiotics starting from the first 24 hours of hospitalization - respiratory quinolone and/or an antipseudomonal penicillin. Duration or escalation of antibiotics may be adjusted based on available antibiograms or treating physician's clinical judgment. 3. Anticoagulation: Prophylactic-dose anticoagulation - low molecular weight heparin or fondaparinux - throughout hospitalization. Patients with an established indication for therapeutic anticoagulation will be maintained on their therapeutic regimen. 4. Antifibrotic therapy: Antifibrotics (nintedanib, pirfenidone, or nerandomilast) will be continued during hospitalization if already prescribed and not contraindicated. No new antifibrotic treatment will be initiated during the study period.

Treatment groups

196 Participants

are divided into 2 treatment groups

Group A: Usual treatmentActive comparator 1 intervention

Group B: Intravenous immunoglobulin plus Usual treatmentExperimental treatment 1 intervention

Trial outcomes

Primary outcomes

All-cause in-hospital mortality or intubation

This outcome corresponds to the all-cause mortality or intubation rate throughout hospitalisation.

Time frame

From date of randomization until the outcome mesure - the date of death from any cause or the date of endotracheal intubation, whichever comes first - assessed up to 4 weeks during hospitalisation.

Secondary outcomes

All-cause 30-day mortality

This outcome corresponds to all-cause mortality rate at Day 30

Time frame

30 days from hospital admission

All-cause 90-day mortality

This outcome corresponds to all-cause mortality rate at Day 90

Time frame

90 days from hospital admission

Hospital readmissions

This outcome corresponds to all-cause hospital readmission rates by Day 180

Time frame

180 days from hospital admission

New acute exacerbation IPF

This outcome corresponds to occurence of new acute exacerbation of IPF by Day 180

Time frame

180 days from hospital admission

Other outcomes

Change in FVC

This outcome corresponds to the absolute and change in percent Forced Vital Capacity (FVC) before acute exacerbation (if available) and at Day 90.

Time frame

90 days from hospital admission

Change in DLCO

This outcome corresponds to the absolute change in percent diffusing capacity for carbon monoxide (DLCO) before acute exacerbation (if available) and at Day 90.

Time frame

90 days from hospital admission

Sponsors and contacts

Click on the lead sponsor to view all of their trials.

Argyrios Tzouvelekis

Lead sponsor

University General Hospital of Patras

Sponsor institution

Source ClinicalTrials.gov

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