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

Prior treatment

• Patients with active malignancy or currently receiving cancer treatment, except for basal cell or squamous cell skin cancer or low-risk prostate cancer (T1 or T2a stage with PSA \<10 ng/dL). These criteria are aligned with current guidelines.
• Patients that have received treatment for \>14 days within the preceding month with \>20mg daily prednisone (or equivalent) or any treatment during the last month with immunosuppressants (e.g., cyclophosphamide, mycophenolate etc.) according to already published therapeutic protocols or \> 1 mg/kg/d from more than 7 days in the last 15 days.

Other illness

• 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.

Lab values

• 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.

Other

• Patients participating to another interventional clinical trial.
• Patients with documented pregnancy or lactation.
• Patients under tutorship or curatorship.
• Patients deprived of liberty or under court protection.
• Patients who refuse to participate or decline to provide written informed consent.

• 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".
  • 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.
  • 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.