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Observational Study
. 2025 Sep 4;61(9):1594.
doi: 10.3390/medicina61091594.

Intravenous Immunoglobulin in Acute Exacerbations of Fibrotic Interstitial Lung Diseases: A Retrospective, Real-World Study

Vasilina Sotiropoulou  1 , Eva Theochari  1 , Matthaios Katsaras  1 , Panagiota Tsiri  1 , Dimitrios Komninos  1 , Ioannis Christopoulos  1 , Georgios Tsirikos  1 , Christina Kalogeropoulou  2 , Dimitrios Daoussis  3 , Kyriakos Karkoulias  1 , Fotios Sampsonas  1 , Argyrios Tzouvelekis  1
Affiliations
Observational Study

Intravenous Immunoglobulin in Acute Exacerbations of Fibrotic Interstitial Lung Diseases: A Retrospective, Real-World Study

Vasilina Sotiropoulou et al. Medicina (Kaunas). .

Abstract

Background and Objectives: Despite the devastating impact of acute exacerbations of fibrotic interstitial lung diseases (AE-fILDs), established treatment strategies are majorly lacking. The therapeutic potential of intravenous immunoglobulin (IVIG) in AE-fILDs was explored considering its anti-inflammatory and immunomodulatory effects, as well as the immunocompromised status of fILD patients and the high frequency of infections that AE-fILDs triggers. Materials and Methods: This was an observational, retrospective study. We investigated the therapeutic potential of IVIG in patients hospitalized for AE-fILDs between May 2021 and November 2024. Results: We included 39 patients diagnosed with AE-fILDs. All patients received IVIG (total dose of 1 g/kg, divided into three daily doses), pulse corticosteroids for three days and broad-spectrum antibiotics. No adverse events were considered to be related to IVIG therapy during the study period. The in-hospital and the 90-day mortality were 10 (26%) and 13 (33%) patients, respectively. Twenty-nine patients (74%) were discharged and 18 of them (62%) were in need of long-term oxygen therapy. The mean PaO2/FiO2 ratio (P/F ratio) was 183 mmHg on admission and 294 mmHg on discharge (t-test, p < 0.0001). Conclusions: This study suggests a potential therapeutic signal, indicating that IVIG is a relatively harmless, well-tolerated, and a potentially effective add-on treatment to current therapeutic approaches. Further research is essential to clarify the role of IVIG, determine optimal treatment protocols, and assess its efficacy in different ILD subtypes.

Keywords: acute exacerbation; idiopathic pulmonary fibrosis; interstitial lung diseases; intravenous immunoglobulin.

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Conflict of interest statement

A.T. has received grants and honoraria from Boehringer Ingelheim, Hoffmann La Roche, Chiesi, Elpen, Astra Zeneca, GlaxoSmithKline, Menarini, Guidotti, Pfizer, Pliant, and Puretech outside the submitted work. V.S., E.T., M.K., P.T., D.K., I.C., G.T., F.T., C.K., D.D., K.K. declare no conflicts of interest.

Figures

Figure 1
Figure 1
Study flowchart. Abbreviations: AE-fILD: acute exacerbation of fibrotic interstitial lung disease, IVIG: intravenous immunoglobulin.
Figure 2
Figure 2
PaO2/FiO2 ratio change on admission and on discharge—Panel (A) In patients with AE-fILDs (n = 29)—Mean P/F ratio ± SD on admission 183 ± 63 mmHg VS on discharge 294 ± 43 mmHg, p < 0.0001 (Paired samples t-test). Panel (B) In patients with AE-IPF (n = 7)—Mean P/F ratio ± SD on admission 163 ± 57 mmHg VS on discharge 287 ± 51 mmHg, p = 0.0064 (Paired samples t-test). Panel (C) In patients with AE-CTD-ILDs (n = 8)—Mean P/F ratio ± SD on admission 190 ± 59 mmHg VS on discharge 306 ± 34 mmHg, p = 0.007 (Paired samples t-test). Abbreviations: AE-fILD: acute exacerbation of fibrotic interstitial lung disease, CTD-ILDs: connective tissue disease associated interstitial lung disease, IPF: Idiopathic pulmonary fibrosis, IVIG: intravenous immunoglobulin, P/F ratio: PaO2/FiO2 ratio, SD: standard deviation.
See this image and copyright information in PMC

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