Interstitial Lung Disease with Anti-melanoma Differentiation-associated Protein 5 Antibody: Rapidly Progressive Perilobular Opacity
Interstitial Lung Disease with Anti-melanoma Differentiation-associated Protein 5 Antibody: Rapidly Progressive Perilobular Opacity
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DOI：
10.2169/internalmedicine.2328-18
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发表时间：
2019-01-01
影响因子：
1.2
通讯作者：
Ogura, Takashi
中科院分区：
文献类型：
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作者：
Chino, Haruka;Sekine, Akimasa;Ogura, Takashi
目的抗黑色素瘤分化相关蛋白5（MDA 5）抗体阳性的快速进展性间质性肺疾病（RP ILD）可能具有致死性临床病程，需要早期强化治疗。最近，据报道，小叶周围混浊在病理学上对应于具有抗MDA 5抗体的RP-ILD中弥漫性肺泡损伤的急性期。我们的目的是调查是否小叶周围混浊是一个常见的放射学发现在RP-ILD患者与抗MDA 5 antibody.Methods我们进行了回顾性分析的病历8例连续RP- ILD与抗MDA 5 antibody。结果在8例抗MDA-5抗体阳性的RP-ILD患者中，6例表现为下叶小叶周围阴影，2例仅表现为高分辨率CT实变。所有6例患者的小叶周围阴影均增厚，并在短期内进展为实变伴肺体积损失。尽管进行了强化治疗，但仍有6例患者（75%）在首次访视后100天内死亡。值得注意的是，2例实变患者的临床病程非常快，均在13天内死亡。在两个幸存者，小叶周围的阴影和实变恢复改善的损失肺volume. Conclusionrapidprogressively小叶周围的阴影，增厚，并进行到实变是RP-ILD与抗MDA 5抗体的特点。胸科医生应立即检查抗MDA-5抗体的状态，以便对快速进展的小叶周围混浊的RP-ILD患者进行早期积极治疗。
Objective Rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation- associated protein 5 (MDA5) antibody potentially presents with a fatal clinical course and requires early intensive treatment. Recently, perilobular opacity was reported to pathologically correspond to the acute phase of diffuse alveolar damage in RP-ILD with anti-MDA5 antibody. We aimed to investigate whether or not perilobular opacity was a common radiological finding in RP-ILD patients with anti-MDA5 antibody.Methods We conducted a retrospective review of the medical records of eight consecutive patients with RP- ILD with anti-MDA5 antibody. The clinical features and radiological findings of follow-up computed tomography (CT) during the course of their disease were evaluated.Results Among eight RP-ILD patients with anti-MDA-5 antibody, six showed perilobular opacity in the lower lobes, and the remaining two had only consolidation on high-resolution CT, Of note, the perilobular opacity in all six patients thickened and progressed to consolidation with a loss of lung volume in a short period. Despite intensive treatment, 6 patients (75%) died within 100 days after the first visit. Notably, die two patients with consolidation presented with a very rapid clinical course and died in 13 days each. In the two survivors, the perilobular opacity and consolidation recovered with improvement in the loss of lung volume.Conclusion Rapidly progressive perilobular opacity that thickens and progresses to consolidation is characteristic of RP-ILD with anti-MDA5 antibody. Chest physicians should immediately check the status of anti- MDA-5 antibody in order to initiate early aggressive therapy in RP-ILD patients with rapidly progressive perilobular opacity.