拉丁美洲211例间质性肺病和肌炎相关抗体患者的多中心研究

F. Reyes, V. Wolff, L. Alberti, Ernesto Juárez, V. Leiva, L. Fassola, M. Mejía, I. Buendía, F. Caro, J. Serrano, F. Paulin, M. Florenzano
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引用次数: 0

摘要

简介:ILD是肌炎相关抗体(MRA)患者的常见表现。我们研究的目的是描述一组ILD和MRA患者的临床和放射学特征,以及它们与初始肺功能(PF)的关系。方法:对阿根廷、智利和墨西哥的3家ILD诊所2016-2018年间评估的多中心队列患者进行描述性研究。进行描述性统计、单因素和多因素分析。结果:184例患者首发ILD或同时伴有结缔组织病(CTD)。以女性居多,平均年龄57±12岁。抗合成酶(AS)抗体最为常见(Jo-1、PL-12、PL-7)。主要的CTD诊断为AS综合征和具有自身免疫特征的ILD (IPAF)。表1描述了主要的胸外症状、更频繁的HRCT模式和pft。结论:as抗体、NSIP和NSIP/OP模式是最常见的数据,在其他队列中也有报道。更严重的PF可能与没有胸外症状和CTD的“典型”抗体有关,从而导致ILD诊断的延迟。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Multicentric Latin American study of 211 patients with Interstitial lung disease and myositis related antibodies
Introduction: ILD is a common manifestation of patients with myositis related antibodies (MRA). The aim of our study is to describe the clinical and radiological features of a group of patients with ILD and MRA, and their association with the initial pulmonary function (PF). Methods: Descriptive study of a multicentric cohort of patients evaluated between 2016-2018 in 3 ILD clinics in Argentina, Chile and Mexico. Descriptive statistics, univariate and multivariate analysis were performed. Results: 184 patients presented initial ILD diagnosis or simultaneous with connective tissue disease (CTD). The majority were women, with a mean age of 57±12 years. Anti-Synthetase (AS) antibodies were the most frequent (Jo-1, PL-12, PL-7). Main CTD diagnoses were AS syndrome and ILD with autoimmune features (IPAF). Main extra-thoracic symptoms, more frequent HRCT patterns and PFTs are described in Table 1. Worse PF was defined as FVC Conclusions: AS antibodies, NSIP and NSIP/OP patterns were the most frequent data, as reported in other cohorts. Worse PF could be related to the absence of extra-thoracic symptoms and “classic” antibodies of CTD, causing a delay in ILD diagnosis.
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