Radiological and Pathological Correlation in Anti-MDA5 Antibody-positive Interstitial Lung Disease: Rapidly Progressive Perilobular Opacities and Diffuse Alveolar Damage

Radiological and Pathological Correlation in Anti-MDA5 Antibody-positive Interstitial Lung Disease: Rapidly Progressive Perilobular Opacities and Diffuse Alveolar Damage
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DOI:
10.2169/internalmedicine.55.5774
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发表时间:
2016-01-01
期刊:
影响因子:
1.2
通讯作者:
Ogura, Takashi
Ogura, Takashi
中科院分区:
医学4区
文献类型:
--
作者:
Chino, Haruka;Sekine, Akimasa;Ogura, Takashi

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我们在此介绍第一例通过外科肺活检 (SLB) 评估的抗黑色素瘤分化相关蛋白 5 (MDA5) 抗体的快速进展性间质性肺疾病 (RP-ILD)。高分辨率 CT 扫描显示小叶周围混浊,迅速变厚并形成实变,导致肺容量显着减少。 SLB 标本显示膜组织存在肺泡闭塞、肺泡管扩张和肺泡囊塌陷,这是弥漫性肺泡损伤 (DAD) 的典型特征。快速进展的小叶周围混浊可能是具有抗 MDA5 抗体和 DAD 的 RP-ILD 的特征。
We herein present the first case of rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody evaluated by surgical lung biopsy (SLB). High-resolution CT scan revealed perilobular opacities, which rapidly became thicker and formed consolidation, resulting in remarkable loss of lung volume. Specimens taken from SLB revealed membranous organization with alveolar occlusion, dilation of alveolar ducts, and sacs with collapsed alveoli, which are typical features of diffuse alveolar damage (DAD). Rapidly progressive perilobular opacities may be characteristic of RP-ILD with anti-MDA5 antibody and DAD.