ArticleSarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG2022
Intravenous immunoglobulin for acute exacerbation of fibrotic idiopathic interstitial pneumonias.
Article in Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
5 citing papers in PubMed, 4 citations in OpenAlex.
- Design of the MERCURION-IPF trial - intravenous immunoglobulin for the treatment of acute exacerbations of idiopathic pulmonary fibrosis.Frontiers in immunology · 2026Trial
- Acute exacerbation in fibrotic interstitial lung disease: an international working group report.American journal of respiratory and critical care medicine · 2026Review
- Fatal interstitial lung disease associated with rituximab in diffuse large B-cell lymphoma: A case report and literature review.Medicine · 2025Review
- Intravenous Immunoglobulin in Acute Exacerbations of Fibrotic Interstitial Lung Diseases: A Retrospective, Real-World Study.Medicina (Kaunas, Lithuania) · 2025Observational
- Acute exacerbation of idiopathic pulmonary fibrosis a narrative review primary focus on treatments.Journal of thoracic disease · 2024Review
Corrections and comments
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Authors and funding
15 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
aimAcute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a fatal condition with no established treatment. Intravenous immunoglobulin (IVIG) is a unique therapy with both anti-inflammatory and anti-infective effects. Therefore, we hypothesized that IVIG may have a positive effect on AE of interstitial pneumonia. This study aimed to determine the effect of IVIG in patients with AE of fibrotic idiopathic interstitial pneumonias (IIPs), including IPF.
methodsWe retrospectively analyzed consecutive patients who were diagnosed with AE of fibrotic IIPs and treated with pulse corticosteroid therapy (methylprednisolone 500-1000 mg/day for 3 days) between April 2018 and May 2021 at Kagawa Rosai Hospital and KKR Takamatsu Hospital.
resultsThis study included 52 patients with AE of fibrotic IIPs (IPF,41; fibrotic IIPs other than IPF,11). Thirteen patients received IVIG (5 g/day for 3-5 days) concurrently with pulse corticosteroid therapy. The remaining 39 patients were assigned to the control group. The survival rate on day 90 was significantly higher in the IVIG group than that in the control group (76.9% vs. 38.5%, p = 0.02). IVIG administration (odds ratio [OR], 0.11; 95% confidence interval [CI], 0.02-0.69; p = 0.02) and C- reactive protein (OR, 1.19; 95% CI, 1.06-1.33, p < 0.01) were independently associated with 90-day mortality.
conclusionsThe results indicate that administration of IVIG may improve the survival of patients with AE of fibrotic IIPs. We are now conducting a prospective study to confirm the effect of IVIG on AE of IPF since May 2022 (jRCT1061220010).
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Registered trials
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