Evidence map›Paper›PMID 40117382›Full record

ArticleClinical and experimental immunology2025

Pirfenidone alleviates interstitial lung disease in mice by inhibiting neutrophil extracellular trap formation and NLRP3 inflammasome activation.

Qiyan Su, Yingyue Feng, Jin Guo, Xi Cui, Jiarui Zhu, Jumei Yang, Sigong Zhang

Abstract read
In one paragraph

Article in Clinical and experimental immunology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors.

Qiyan SuThe Second Clinical Medical College, Lanzhou University, Lanzhou, Gansu, China.
Yingyue FengThe Second Clinical Medical College, Lanzhou University, Lanzhou, Gansu, China.ORCID 0009-0003-2714-4864
Jin GuoThe Second Clinical Medical College, Lanzhou University, Lanzhou, Gansu, China.
Xi CuiThe Second Clinical Medical College, Lanzhou University, Lanzhou, Gansu, China.
Jiarui ZhuDepartment of Cuiying Biomedical Research Center, Lanzhou University Second Hospital, Lanzhou, Gansu, China.
Jumei YangDepartment of Cuiying Biomedical Research Center, Lanzhou University Second Hospital, Lanzhou, Gansu, China.
Sigong ZhangThe Second Clinical Medical College, Lanzhou University, Lanzhou, Gansu, China.ORCID 0000-0003-1565-1431

Funding

Fundamental Research Funds for the Central Universities of Lanzhou University lzujbky-2024-oy03Gansu Province Traditional Chinese Medicine Research Project GZKZ-2024-29Medical Innovation and Development Project of Lanzhou University lzuyxcx-2022-168National Natural Science Foundation of China 82060302Natural Science Foundation of Gansu Province 24JRRA923
6 · The paper itself

Abstract

backgroundIdiopathic inflammatory myopathy (IIM) is a progressive autoimmune disease characterized by interstitial lung disease (ILD) with limited therapeutics available. Pirfenidone (PFD), a medication utilized for the treatment of idiopathic pulmonary fibrosis, exhibits notable antioxidant, anti-inflammatory, and inhibition of collagen synthesis. This study aims to clarify its efficacy and mechanism in treating IIM-ILD.

methodsA murine myositis-associated interstitial lung disease (MAILD) model was used to assess the therapeutic effect of PFD. The serum levels of interleukin (IL)-1β, IL-6, and tumor necrosis factor-α (TNF-α) were detected by enzyme-linked immunosorbent assay (ELISA). Pirfenidone was utilized to disrupt neutrophil extracellular traps (NETs) formation in vitro, and its inhibitory effect on NETs was assessed through immunohistochemistry of citrullinated histone H3 and myeloperoxidase in the lung tissue and the serum cfDNA level in mice. Immunohistochemical and western blot were utilized to examine alterations in epithelial-mesenchymal transition (EMT) and NOD-like receptor protein 3 (NLRP3) inflammasome markers.

resultsPirfenidone treatment inhibited pulmonary inflammation and fibrosis in the MAILD model. Pirfenidone intervention reduced NETs formation in vitro. Pirfenidone treatment significantly reduces NETs infiltration in the lung tissue and the level of cfDNA in the serum of mice. Additionally, PFD downregulated EMT and NLRP3-related proteins in vivo. Pirfenidone treatment also notably reduced serum levels of IL-1β, IL-6, and TNF-α. After NETs stimulation, A549 cells exhibited EMT and activation of NLRP3 inflammasome. Pirfenidone attenuated EMT in A549 cells and suppressed the activation of NLRP3 inflammasome.

conclusionPirfenidone alleviates ILD in a murine MAILD model by inhibiting NETs formation and NLRP3 inflammasome activation, suggesting that PFD might be a potential therapeutic agent for IIM-ILD.

Indexed as

Extracellular TrapsInflammasomesLung Diseases, InterstitialNeutrophilsNLR Family, Pyrin Domain-Containing 3 ProteinPyridonesAnimalsDisease Models, AnimalEpithelial-Mesenchymal TransitionHumansLungMaleMiceMice, Inbred C57BLInflammasomesNLR Family, Pyrin Domain-Containing 3 ProteinNlrp3 protein, mousepirfenidonePyridonesidiopathic inflammatory myopathyinterstitial lung diseaseneutrophil extracellular trappirfenidone

Identifiers

PMID40117382
PMCPMC12038160

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.