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抗合成酶抗体阳性的间质性肺疾病患者队列的肺功能演变。

Evolution of Pulmonary Function in a Cohort of Patients with Interstitial Lung Disease and Positive for Antisynthetase Antibodies.

机构信息

From the Interstitial Lung Disease and Rheumatology Unit, and HLA Laboratory, and Research Direction, Instituto Nacional de Enfermedades Respiratorias; Programa de Maestría y Doctorado en Ciencias Médicas, Facultad de Medicina, Universidad Nacional Autónoma de México, México City, México.

M.I. González-Pérez, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias; J.G. Mejía-Hurtado, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias; D.I. Pérez-Román, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias; I. Buendía-Roldán, MD, PhD, Research Direction, Instituto Nacional de Enfermedades Respiratorias; M. Mejía, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias; R. Falfán-Valencia, PhD, HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias; H.N. Mateos-Toledo, MD, Professor, Programa de Maestría y Doctorado en Ciencias Médicas, Facultad de Medicina, Universidad Nacional Autónoma de México; J. Rojas-Serrano, MD, PhD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, and Professor, Programa de Maestría y Doctorado en Ciencias Médicas, Facultad de Medicina, Universidad Nacional Autónoma de México.

出版信息

J Rheumatol. 2020 Mar;47(3):415-423. doi: 10.3899/jrheum.181141. Epub 2019 Jun 15.

Abstract

OBJECTIVE

To describe the evolution of the pulmonary function in patients with interstitial lung disease (ILD) who are positive for at least 1 of the antisynthetase antibodies (ASAB) after medical treatment, and to compare whether the evolution of pulmonary function is associated with the type of ASAB.

METHODS

Patients with ILD and positive for at least 1 of the ASAB (anti-Jo1, anti-PL7, anti-PL12, anti-EJ, or anti-OJ) were included. The clinical evolution, time until death or censoring, and improvement of lung disease were registered.

RESULTS

The study included 118 patients. Most of the patients had a high extent of ground glass opacities in high-resolution computed tomography (HRCT) and low extent of fibrosis. In the final evaluation of pulmonary function (median 749.5 days of followup), 67% of the patients had lung disease improvement. The improvement occurred within the first 6 months after initiating medical treatment; thereafter, pulmonary function remained stable in most of the patients. A decrease of the extent of ground glass opacities was demonstrated in HRCT at followup in those patients with pulmonary improvement. No differences were observed in the percentage of patients who achieved improvement between the ASAB groups, or in survival.

CONCLUSION

Improvement of pulmonary function was observed in 67% of the patients. Improvement was observed in all ASAB groups and occurred within 6 months after initiating medical treatment.

摘要

目的

描述至少有一种抗合成酶抗体(ASAB)阳性的间质性肺疾病(ILD)患者在接受治疗后的肺功能演变,并比较肺功能的演变是否与 ASAB 的类型有关。

方法

纳入至少有一种 ASAB(抗-Jo1、抗-PL7、抗-PL12、抗-EJ 或抗-OJ)阳性的ILD 患者。记录临床演变、死亡或截止时间以及肺部疾病的改善情况。

结果

该研究纳入了 118 名患者。大多数患者的高分辨率计算机断层扫描(HRCT)有严重的磨玻璃影,纤维化程度较低。在肺功能的最终评估(中位随访 749.5 天)中,67%的患者肺部疾病得到改善。改善发生在开始治疗后的前 6 个月内;此后,大多数患者的肺功能保持稳定。在那些肺部功能改善的患者中,在 HRCT 随访时显示出磨玻璃影程度的降低。在改善的患者中,各组 ASAB 之间或生存率方面没有观察到改善的百分比存在差异。

结论

67%的患者肺功能得到改善。所有 ASAB 组均观察到改善,且发生在开始治疗后的 6 个月内。

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