A Prospective, Multicenter, Randomized, Open-Label Clinical Trial Evaluating the Efficacy of Intravenous Immunoglobulin in Patients Hospitalized for Acute Exacerbations of Idiopathic Pulmonary Fibrosis.
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.
• 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.
• The criteria of IPF-AE are as follows:
⁃ Previous or concurrent diagnosis of IPF
⁃ Acute worsening or development of dyspnoea 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
⁃ 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.
• A) 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.
• B) If no previous computed tomography is available, the qualifier new can be dropped from the third AE-IPF criterion.
• Patient able to understand and sign a written informed consent form. In case of incapacity of the patient, the written informed consent form will be signed by the patients' legally authorized representative.