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伴有临床-影像学分离及移植延迟的进行性系统性硬化症相关间质性肺疾病:一例报告

Progressive Systemic Sclerosis-Associated Interstitial Lung Disease With Clinical-Radiographic Dissociation and Delayed Transplant Access: A Case Report.

作者信息

Bakinde Nicolas, Johnson Antoinette, Ngo Bakinde Deborah, Pemu Priscilla, Flenaugh Eric

机构信息

Department of Medicine, Morehouse School of Medicine, Atlanta, USA.

General Internal Medicine, Grady Memorial Hospital, Atlanta, USA.

出版信息

Cureus. 2025 Jun 20;17(6):e86412. doi: 10.7759/cureus.86412. eCollection 2025 Jun.

DOI:10.7759/cureus.86412
PMID:40688948
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12276634/
Abstract

Systemic sclerosis-associated interstitial lung disease (ILD) is a major contributor to morbidity and mortality in patients with diffuse systemic sclerosis. We present the case of a 47-year-old man with fibrotic non-specific interstitial pneumonia who experienced worsening hypoxemia and dyspnea following COVID-19, despite ongoing treatment with mycophenolate mofetil and prednisone. His oxygen requirement increased from 6-8 L/minute to 15 L/minute at rest, even as imaging showed stable fibrosis with improvement in ground-glass opacities, highlighting a clinical-radiographic dissociation. Given concern for progressive fibrosing ILD, immunosuppression was escalated to intravenous cyclophosphamide. Referral for lung transplantation was delayed due to insurance non-acceptance at the region's transplant center, compounding the urgency of his decline. This case underscores the importance of timely recognition of progressive ILD, the limitations of radiographic assessment alone, and the impact of systemic barriers on access to life-saving interventions.

摘要

系统性硬化症相关间质性肺疾病(ILD)是弥漫性系统性硬化症患者发病和死亡的主要原因。我们报告了一例47岁患有纤维化非特异性间质性肺炎的男性病例,尽管正在接受霉酚酸酯和泼尼松治疗,但在感染新冠病毒后出现了低氧血症和呼吸困难加重的情况。他的静息氧需求从6 - 8升/分钟增加到15升/分钟,即便影像学显示纤维化稳定且磨玻璃影有所改善,这突出了临床与影像学表现的分离。鉴于对进行性纤维化ILD的担忧,免疫抑制治疗升级为静脉注射环磷酰胺。由于该地区移植中心保险不接受,肺移植转诊被推迟,这加剧了他病情恶化的紧迫性。该病例强调了及时识别进行性ILD的重要性、单纯影像学评估的局限性以及系统性障碍对获得挽救生命干预措施的影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/60d81d5b4c55/cureus-0017-00000086412-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/d307ca0fc8c4/cureus-0017-00000086412-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/2daa9da93b2d/cureus-0017-00000086412-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/1feab3c1a787/cureus-0017-00000086412-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/60d81d5b4c55/cureus-0017-00000086412-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/d307ca0fc8c4/cureus-0017-00000086412-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/2daa9da93b2d/cureus-0017-00000086412-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/1feab3c1a787/cureus-0017-00000086412-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eadf/12276634/60d81d5b4c55/cureus-0017-00000086412-i04.jpg

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本文引用的文献

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Multidisciplinary teams in the clinical care of fibrotic interstitial lung disease: current perspectives.纤维化间质性肺疾病临床护理中的多学科团队:当前观点
Eur Respir Rev. 2022 Sep 7;31(165). doi: 10.1183/16000617.0003-2022. Print 2022 Sep 30.
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Prognosis of patients with systemic sclerosis-related interstitial lung disease on the lung transplant waiting list: a retrospective study.
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Update on Morbidity and Mortality in Systemic Sclerosis-Related Interstitial Lung Disease.系统性硬化症相关间质性肺疾病的发病率和死亡率最新情况
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Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities.进行性纤维性间质性肺病:临床不确定性、共识建议和研究重点。
Lancet Respir Med. 2020 Sep;8(9):925-934. doi: 10.1016/S2213-2600(20)30355-6.
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Is there today a place for corticosteroids in the treatment of scleroderma?如今皮质类固醇在硬皮病的治疗中有一席之地吗?
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Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases.特发性肺纤维化等进行性纤维化性间质性肺疾病的治疗:间质性肺疾病治疗的里程碑。
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Update of EULAR recommendations for the treatment of systemic sclerosis.EULAR 系统性硬皮病治疗推荐更新。
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