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纤维化性超敏性肺炎:导致肺移植的诊断难题

Fibrotic Hypersensitivity Pneumonitis: A Diagnostic Challenge Leading to Lung Transplantation.

作者信息

Mot Maria-Daniela, Olar Dana Cristina, Vulciu Paula Alexandra, Barata Paula-Irina, Bouros-Tataru Ana-Liana, Butari Denis Bogdan, Șandor Florin Mihai, Bondar Laura Ioana

机构信息

Department of General Medicine, "Vasile Goldis" Western University of Arad, Blvd. Revoluției, No. 96, 310025 Arad, Romania.

Department of Biochemistry, "Vasile Goldis" Western University of Arad, Blvd. Revoluției, No. 96, 310025 Arad, Romania.

出版信息

Diagnostics (Basel). 2025 May 16;15(10):1267. doi: 10.3390/diagnostics15101267.

DOI:10.3390/diagnostics15101267
PMID:40428260
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12110211/
Abstract

: Hypersensitivity pneumonitis (HP), a subtype of interstitial lung disease (ILD), is often misdiagnosed as idiopathic pulmonary fibrosis (IPF), particularly when the causative antigen cannot be identified. Typically resulting from chronic exposure to inhaled organic particles smaller than 5 microns, HP presents a diagnostic challenge. This report outlines a case of fibrotic HP initially misclassified as asthma. No triggering antigen was identified despite extensive investigation. The disease progressed despite corticosteroid, immunosuppressive, and antifibrotic therapy, ultimately leading to an advanced fibrotic stage and requiring lung transplantation. This clinical course is rare and infrequently reported, particularly in cases requiring lung transplantation without an identifiable causative antigen. Such progression is uncommon and underreported, especially in patients initially misclassified as having asthma. : Medical records of 24 patients diagnosed with HP were reviewed. Only one case demonstrated progression to fibrotic HP; this case was selected for detailed analysis. : Clinical and functional deterioration occurred despite standard therapy. Given the advanced stage of fibrosis and treatment resistance, lung transplantation was deemed the next appropriate therapeutic option. : HP remains underdiagnosed due to difficulties in identifying the causative antigen and overlapping features with other ILDs. Early and accurate differentiation from IPF is essential, particularly in progressive fibrotic forms unresponsive to conventional therapies.

摘要

过敏性肺炎(HP)是间质性肺疾病(ILD)的一种亚型,常被误诊为特发性肺纤维化(IPF),尤其是在无法识别致病抗原时。HP通常由长期接触吸入的小于5微米的有机颗粒引起,这给诊断带来了挑战。本报告概述了一例最初被误诊为哮喘的纤维化HP病例。尽管进行了广泛调查,但未识别出触发抗原。尽管使用了皮质类固醇、免疫抑制和抗纤维化治疗,病情仍进展,最终导致晚期纤维化阶段并需要肺移植。这种临床病程罕见且报道较少,特别是在需要肺移植且无明确致病抗原的病例中。这种进展不常见且报道不足,尤其是在最初被误诊为哮喘的患者中。:回顾了24例诊断为HP的患者的病历。只有1例显示进展为纤维化HP;该病例被选作详细分析。:尽管进行了标准治疗,临床和功能仍恶化。鉴于纤维化的晚期阶段和治疗抵抗,肺移植被认为是下一个合适的治疗选择。:由于难以识别致病抗原以及与其他ILD的重叠特征,HP仍未得到充分诊断。早期和准确地与IPF区分至关重要,尤其是在对传统疗法无反应的进行性纤维化形式中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/686f478731e4/diagnostics-15-01267-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/874720c71d14/diagnostics-15-01267-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/17e0344414c5/diagnostics-15-01267-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/fdd9ef41e0f4/diagnostics-15-01267-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/686f478731e4/diagnostics-15-01267-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/874720c71d14/diagnostics-15-01267-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/17e0344414c5/diagnostics-15-01267-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/fdd9ef41e0f4/diagnostics-15-01267-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/563e/12110211/686f478731e4/diagnostics-15-01267-g004.jpg

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Insights on immune profile, pathogenesis and differential diagnosis of hypersensitivity pneumonitis and pulmonary sarcoidosis-A holistic review and bibliometric analysis.过敏性肺炎与肺结节病的免疫特征、发病机制及鉴别诊断见解——全面综述与文献计量分析
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Beyond the Graft: Recurrence of Interstitial Lung Diseases Post Transplant.
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J Clin Med. 2025 Feb 8;14(4):1093. doi: 10.3390/jcm14041093.
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Longitudinal Study of Patients with Connective Tissue Disease-Interstitial Lung Disease and Response to Mycophenolate Mofetil and Rituximab.结缔组织病相关性间质性肺病患者的纵向研究及对霉酚酸酯和利妥昔单抗的反应
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A Low Forced Vital Capacity (FVC)/Diffusing Capacity of the Lung for Carbon Monoxide (DLCO) Ratio Increases Clinical Suspicion for Fibrotic Hypersensitivity Pneumonitis (FHP) Over Idiopathic Pulmonary Fibrosis (IPF).低用力肺活量(FVC)/肺一氧化碳弥散量(DLCO)比值增加了临床对纤维化性过敏性肺炎(FHP)而非特发性肺纤维化(IPF)的怀疑。
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