Abstract / Summary
Despite their profound clinical impact and high mortality, acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) still lack any effective or standardized treatment strategies. This manuscript describes the rationale and design of a randomized, multicenter, open-label Phase III clinical trial evaluating the efficacy of intravenous immunoglobulin (IVIG) compared with usual care in hospitalized patients with AE-IPF. This trial will enroll 196 patients across eight different sites in Greece. Inclusion criteria are designed to identify patients with AE-IPF according to the American Thoracic Society definition, while excluding those with cardiac decompensation or pulmonary embolism. The primary endpoint is a composite of all-cause in-hospital mortality or the need for intubation. Secondary endpoints include all-cause mortality at 30 and 90 days, hospital readmission or a new AE-IPF episode within 90 days, and the change in the PaO2/FiO2 ratio from hospital admission to discharge. Based on the concept that patients with IPF frequently demonstrate impaired cellular and humoral immunity, and inflammation and/or immune dysregulation may contribute to AE-IPF pathogenesis, there is a strong rationale for evaluating the therapeutic usefulness of IVIG in this setting. Retrospective data indicate that IVIG could provide clinical benefit in AE-IPF, potentially through its anti-inflammatory and immunomodulatory effects. In this trial, IVIG will be administered as an adjunct to usual care, which includes pulse corticosteroids, broad-spectrum antibiotics, prophylactic anticoagulation, and oxygen therapy. This intervention has the potential to significantly influence current treatment strategies for AE-IPF. https://clinicaltrials.gov/, identifier NCT07299695.
Primary Source
Frontiers in immunology
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