Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience.
Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience.
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DOI：
10.1016/j.rmed.2021.106432
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发表时间：
2022-01
影响因子：
4.3
通讯作者：
Dudenhofer RB
中科院分区：
文献类型：
--
作者：
Wilfong EM;Young-Glazer JJ;Sohn BK;Schroeder G;Annapureddy N;Gillaspie EA;Barnado A;Crofford LJ;Dudenhofer RB
识别抗 tRNA 合成酶 (ARS) 相关的间质性肺疾病 (ILD) 是确保患者及时获得免疫抑制治疗的关键。这项回顾性队列研究的目的是确定可能延迟识别 ARS-ILD 的因素。 2017年9月17日至2018年10月31日期间在范德比尔特大学医学中心就诊的患者被纳入该观察队列。通过图表抽象获得临床和实验室特征。 Kruskal-Wallis 方差分析、Mann-Whitney U 和 Fisher 精确 t 检验用于确定统计显着性。 ARS 患者中 51.9% 患有 ILD，与系统性硬化症中 ILD 的发生率 (59.5%) 相当。 ARS 中 FVC 降低的严重程度 (53.2%) 与弥漫性皮肤系统性硬化症 (56.8%, p=0.48) 相当，并且大于皮肌炎 (66.9%, p=0.005) 或局限性皮肤系统性硬化症 (71.8%, p=0.005)。 ARS 抗体可观察到 Frank 蜂窝状结构，但其他肌炎自身抗体则不会。 ARS 患者更有可能首先就诊于三级护理机构的肺部医疗服务提供者 (53.6%)，这可能是由于肺外表现较少。仅 33% 的 ARS-ILD 抗核抗体、类风湿因子或抗环瓜氨酸肽呈阳性。与其他肌炎 ILD 患者相比，ARS-ILD 患者的中位诊断时间延长两倍（11.0 个月，IQR 8.5 至 43 个月 vs. 5.0 个月，IQR 3.0 至 9.0 个月，p=0.003）。没有明显肺外表现的 ARS 患者在没有全面血清学检查的情况下，很可能不被识别为患有与结缔组织病相关的 ILD，并被错误分类为常见的间质性肺炎/特发性肺纤维化。
Recognition of Anti-tRNA synthetase (ARS) related interstitial lung disease (ILD) is key to ensuring patients have prompt access to immunosuppressive therapies. The purpose of this retrospective cohort study was to identify factors that may delay recognition of ARS-ILD. Patients seen at Vanderbilt University Medical Center between 9/17/2017–10/31/2018 were included in this observational cohort. Clinical and laboratory features were obtained via chart abstraction. Kruskal-Wallis ANOVA, Mann-Whitney U, and Fisher’s exact t tests were utilized to determine statistical significance. Patients with ARS were found to have ILD in 51.9% of cases, which was comparable to the frequency of ILD in systemic sclerosis (59.5%). The severity of FVC reduction in ARS (53.2%) was comparable to diffuse cutaneous systemic sclerosis (56.8%, p=0.48) and greater than dermatomyositis (66.9%, p=0.005) or limited cutaneous systemic sclerosis (71.8%, p=0.005). Frank honeycombing was seen with ARS antibodies but not other myositis autoantibodies. ARS patients were more likely to first present to a pulmonary provider in a tertiary care setting (53.6%), likely due to fewer extrapulmonary manifestations. Only 33% of ARS-ILD were anti-nuclear antibody, rheumatoid factor, or anti-cyclic citrullinated peptide positive. Patients with ARS-ILD had a two-fold longer median time to diagnosis compared to other myositis-ILD patients (11.0 months, IQR 8.5 to 43 months vs. 5.0 months, IQR 3.0 to 9.0 months, p=0.003). ARS patients without prominent extra-pulmonary manifestations are at high risk for not being recognized as having a connective tissue disease related ILD and miscategorized as usual interstitial pneumonia/idiopathic pulmonary fibrosis without comprehensive serologies.
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影响因子：
5
作者：
Fidler, Lee;Doubelt, Irena;Shapera, Shane
通讯作者：
Shapera, Shane
影响因子：
--
作者：
van den Hoogen, Frank;Khanna, Dinesh;Fransen, Jaap;Johnson, Sindhu R.;Baron, Murray;Tyndall, Alan;Matucci-Cerinic, Marco;Naden, Raymond P.;Medsger, Thomas A., Jr.;Carreira, Patricia E.;Riemekasten, Gabriela;Clements, Philip J.;Denton, Christopher P.;Distler, Oliver;Allanore, Yannick;Furst, Daniel E.;Gabrielli, Armando;Mayes, Maureen D.;van Laar, Jacob M.;Seibold, James R.;Czirjak, Laszlo;Steen, Virginia D.;Inanc, Murat;Kowal-Bielecka, Otylia;Mueller-Ladner, Ulf;Valentini, Gabriele;Veale, Douglas J.;Vonk, Madelon C.;Walker, Ulrich A.;Chung, Lorinda;Collier, David H.;Csuka, Mary Ellen;Fessler, Barri J.;Guiducci, Serena;Herrick, Ariane;Hsu, Vivien M.;Jimenez, Sergio;Kahaleh, Bashar;Merkel, Peter A.;Sierakowski, Stanislav;Silver, Richard M.;Simms, Robert W.;Varga, John;Pope, Janet E.
通讯作者：
Pope, Janet E.
影响因子：
9.6
作者：
Connors, Geoffrey R.;Christopher-Stine, Lisa;Danoff, Sonye K.
通讯作者：
Danoff, Sonye K.
影响因子：
5.5
作者：
Pinal-Fernandez, Iago;Casal-Dominguez, Maria;Danoff, Sonye K.
通讯作者：
Danoff, Sonye K.
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)