Clinical, radiological and pathological features of anti-MDA5 antibody-associated interstitial lung disease.
Clinical, radiological and pathological features of anti-MDA5 antibody-associated interstitial lung disease.
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DOI：
10.1136/rmdopen-2023-003150
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发表时间：
2023-05
期刊：
影响因子：
6.2
通讯作者：
Wang, Guochun
中科院分区：
文献类型：
--
作者：
Chen, Xixia;Jiang, Wei;Jin, Qiwen;Peng, Qinglin;Zhang, Lu;Lin, Sang;Lu, Xin;Liu, Min;Wang, Yuli;Song, Aiping;Feng, Ruie;Wang, Guochun
关键词：
探讨抗黑色素瘤分化相关基因5抗体阳性皮肌炎（抗-MDA 5 +DM）患者间质性肺病（ILD）的临床、影像学和病理学特征。我们回顾性分析了抗MDA 5 +DM患者的病历，这些患者接受了放射学检查，其中17例进行了肺组织病理学检查。本研究检查了329例抗-MDA 5 +DM患者，其中308例（93.6%）诊断为ILD，177例（53.8%）表现为快速进展性ILD（RPILD）。最常见的影像学类型为机化性肺炎（OP）（43.2%）、非特异性间质性肺炎（NSIP）（26.4%）和NSIP+OP（18.5%）。组织学检查以NSIP（41.2%）和NSIP+OP（47.1%）为主。而17例经肺组织病理学检查的患者中，影像学诊断与组织病理学诊断的符合率仅为11.8%。与无RPILD的患者相比，RPILD患者在高分辨率CT上显示NSIP+OP的患病率较高（26.6% vs 10.7%，p=0.001），NSIP模式的患病率较低（21.5% vs 37.4%，p=0.002）。此外，影像学表现为NSIP+OP或弥漫性肺泡损伤（DAD）的患者预后不良的危险因素更多，12个月死亡率分别为45.9%和100%。RPILD常见于抗-MDA 5 +DM患者。OP被确定为主要的放射学模式，这对应于NSIP或NSIP+ OP的组织病理学模式。值得注意的是，表现出NSIP+OP或DAD的放射学模式的患者显示预后不良。
To investigate the clinical, radiographic and pathological features of interstitial lung disease (ILD) in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+DM). We retrospectively analysed the medical records of patients with anti-MDA5+DM who had undergone radiological examination, and lung histopathology was performed on 17 of them. This study examined 329 patients with anti-MDA5+DM, of whom 308 (93.6%) were diagnosed with ILD and 177 (53.8%) exhibited rapidly progressive ILD (RPILD). The most common radiographic patterns were organising pneumonia (OP) (43.2%), non-specific interstitial pneumonia (NSIP) (26.4%) and NSIP+OP (18.5%). Histological analysis showed NSIP (41.2%) and NSIP+OP (47.1%) to be the predominant patterns. However, in the 17 patients who underwent lung histopathology, the coincidence rate between radiological and histopathological diagnoses was only 11.8%. Compared with patients without RPILD, those with RPILD showed a higher prevalence of NSIP+OP (26.6% vs 10.7%, p=0.001) and a lower prevalence of NSIP pattern (21.5% vs 37.4%, p=0.002) on high-resolution CT. Furthermore, patients with radiographic patterns of NSIP+OP or diffuse alveolar damage (DAD) had more risk factors for poor prognosis, with 12-month mortality rates of 45.9% and 100%, respectively. RPILD was commonly observed in patients with anti-MDA5+DM. OP was identified as the predominant radiographic pattern, which corresponded to a histopathological pattern of NSIP or NSIP+OP. Notably, patients exhibiting radiographic patterns of NSIP+OP or DAD were shown to have a poor prognosis.
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影响因子：
1.2
作者：
Chino, Haruka;Sekine, Akimasa;Ogura, Takashi
通讯作者：
Ogura, Takashi
影响因子：
8.3
作者：
Pastre, Jean;Khandhar, Sandeep;Nathan, Steven D.
通讯作者：
Nathan, Steven D.
影响因子：
3.7
作者：
Yang, Q.;Lyu, K.;Li, T.
通讯作者：
Li, T.
影响因子：
7.3
作者：
Nombel A;Fabien N;Coutant F
通讯作者：
Coutant F
影响因子：
27.4
作者：
Lundberg IE;Tjärnlund A;Bottai M;Werth VP;Pilkington C;Visser M;Alfredsson L;Amato AA;Barohn RJ;Liang MH;Singh JA;Aggarwal R;Arnardottir S;Chinoy H;Cooper RG;Dankó K;Dimachkie MM;Feldman BM;Torre IG;Gordon P;Hayashi T;Katz JD;Kohsaka H;Lachenbruch PA;Lang BA;Li Y;Oddis CV;Olesinska M;Reed AM;Rutkowska-Sak L;Sanner H;Selva-O'Callaghan A;Song YW;Vencovsky J;Ytterberg SR;Miller FW;Rider LG;International Myositis Classification Criteria Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland)
通讯作者：
International Myositis Classification Criteria Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland)