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一名32岁男性,存在低氧血症,胸部影像学显示双侧上叶为主的磨玻璃样浸润影。

A 32-year-old man with hypoxemia and bilateral upper-lobe predominant ground-glass infiltrates on chest imaging.

作者信息

Helgeson Scott A, Heckman Alexander J, McCain Josiah D, Cowart Jennifer B, Maniaci Michael J, Garland Jeffrey L

机构信息

Department of Medicine, Mayo Clinic, Jacksonville, FL, USA.

出版信息

Oxf Med Case Reports. 2018 Oct 3;2018(10):omy072. doi: 10.1093/omcr/omy072. eCollection 2018 Oct.

DOI:10.1093/omcr/omy072
PMID:30302263
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6169198/
Abstract

Diffuse alveolar hemorrhage (DAH) is a rare, but potentially fatal, complication of antiphospholipid syndrome, and may present with acute and fulminant symptoms. We report a case of DAH presenting as sudden onset dyspnea in a gentleman with known antiphospholipid syndrome. Chest computed tomography angiography with pulmonary embolism protocol showed right lower lobe segmental filling defects, upper-lobe predominant diffuse ground-glass opacities, and centrilobular nodules bilaterally. The presence of DAH can be confirmed by bronchoalveolar lavage with serial aliquots, but this procedure typically does not elucidate the specific etiology for the hemorrhage. The treatment for patients with severe disease typically consists of a combination of immunosuppressive medications in the form of high-dose intravenous glucocorticoids plus rituximab, cyclophosphamide or mycophenolate; and/or plasma exchange. This case both provides an example of high-quality diagnostic imaging of diffuse alveolar hemorrhage as well as demonstrates the clinical and image-based improvement after treatment.

摘要

弥漫性肺泡出血(DAH)是抗磷脂综合征一种罕见但可能致命的并发症,可表现为急性和暴发性症状。我们报告一例已知患有抗磷脂综合征的男性患者,以突发呼吸困难为表现的DAH病例。采用肺栓塞方案的胸部计算机断层扫描血管造影显示右下叶节段性充盈缺损、上叶为主的弥漫性磨玻璃影以及双侧小叶中心结节。通过连续等分的支气管肺泡灌洗可确诊DAH,但该操作通常无法阐明出血的具体病因。重症患者的治疗通常包括大剂量静脉注射糖皮质激素加利妥昔单抗、环磷酰胺或霉酚酸酯形式的免疫抑制药物联合使用;和/或血浆置换。该病例既提供了弥漫性肺泡出血高质量诊断成像的示例,也展示了治疗后基于临床和影像的改善情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/7140bca57f08/omy072f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/c157df1bc7bf/omy072f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/ca8f06be9cfa/omy072f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/7140bca57f08/omy072f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/c157df1bc7bf/omy072f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/ca8f06be9cfa/omy072f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f8f/6169198/7140bca57f08/omy072f03.jpg

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本文引用的文献

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2
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Arthritis Care Res (Hoboken). 2014 Feb;66(2):301-10. doi: 10.1002/acr.22109.
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"Possible primary antiphospholipid syndrome" with concurrent diffuse alveolar hemorrhaging and Libman-Sacks endocarditis mimicking catastrophic antiphospholipid syndrome.
伴有并发弥漫性肺泡出血和类Libman-Sacks心内膜炎的“可能的原发性抗磷脂综合征”,酷似灾难性抗磷脂综合征。
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Lupus. 2012 Apr;21(4):438-40. doi: 10.1177/0961203311422713. Epub 2011 Oct 12.
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Pulmonary-renal syndrome, diffuse pulmonary hemorrhage and glomerulonephritis, associated with Wegener's granulomatosis effectively treated with early plasma exchange therapy.肺肾综合征,即弥漫性肺出血和肾小球肾炎,与韦格纳肉芽肿病相关,早期血浆置换疗法可有效治疗。
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Antiphospholipid antibodies as a cause of pulmonary capillaritis and diffuse alveolar hemorrhage: a case series and literature review.抗磷脂抗体作为肺毛细血管炎和弥漫性肺泡出血的病因:病例系列及文献综述
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Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations.胸部CT中孤立性弥漫性磨玻璃影:病因及临床表现
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