寻常型间质性肺炎中纤维化的进展：单肺移植后对自体肺的系列评估

Progression of fibrosis in usual interstitial pneumonia: serial evaluation of the native lung after single lung transplantation.

作者信息

Grgic Aleksandar, Lausberg Henning, Heinrich Marc, Koenig Jochem, Uder Michael, Sybrecht Gerhard W, Wilkens Heinrike

机构信息

Klinik für Nuklearmedizin, Universitätsklinikum des Saarlandes, Homburg, Saar, Deutschland.

出版信息

Respiration. 2008;76(2):139-45. doi: 10.1159/000108440. Epub 2007 Sep 13.

DOI:10.1159/000108440

PMID:17851227

Abstract

BACKGROUND

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a poor prognosis. Usual interstitial pneumonia (UIP) is the histopathological pattern identifying patients with the clinical entity of IPF. Despite aggressive immunosuppressive therapy the clinical course is usually dismal. For selected patients only lung transplantation improves prognosis and quality of life. After lung transplantation patients often receive a potent cyclosporine-based immunosuppressive therapy. Some reports suggest that cyclosporine has the potential to prevent progression of fibrosis.

OBJECTIVE

In patients with single lung transplantation (sLTx) for UIP we evaluated the effect of cyclosporine-based immunosuppressive therapy on progression of fibrosis using a high-resolution computed tomography (HRCT) scoring system.

METHODS

This retrospective observational study included 13 patients (24-64 years old) with histologically confirmed UIP who had HRCT scans preceding and following sLTx and who survived at least 6 months after sLTx. All patients were initially treated with cyclosporin A, prednisone and azathioprine. Three radiologists analyzed HRCT scans by setting a score regarding fibrosis [fibrosis score (FS); range 0-5 for each lobe] and ground-glass opacity [ground-glass score (GGS); range 0-5 for each lobe]. A comparison of serial changes (interval: 12-96 months posttransplant, 2-4 HRCT examinations/patient) was performed with the sign test.

RESULTS

Mean pretransplant FS and GGS of the nontransplanted lung were 1.80 and 1.61, respectively. Comparing pre- and posttransplant HRCT scans, mean lung FS significantly increased (0.35 +/- 0.15/year; p = 0.00024), while GGS tended to decrease (0.06 +/- 0.26/year; p = 0.5).

CONCLUSION

A cyclosporin A based triple immunosuppressive regimen following sLTx does not seem to prevent progression of the fibrotic changes of the native lung in patients with IPF.

摘要

背景

特发性肺纤维化（IPF）是一种预后较差的进行性疾病。普通型间质性肺炎（UIP）是识别IPF临床实体患者的组织病理学模式。尽管采用了积极的免疫抑制治疗，但临床病程通常不佳。仅对于部分选定患者，肺移植可改善预后和生活质量。肺移植后患者常接受以环孢素为基础的强效免疫抑制治疗。一些报告表明，环孢素有预防纤维化进展的潜力。

目的

在因UIP接受单肺移植（sLTx）的患者中，我们使用高分辨率计算机断层扫描（HRCT）评分系统评估了以环孢素为基础的免疫抑制治疗对纤维化进展的影响。

方法

这项回顾性观察性研究纳入了13例组织学确诊为UIP的患者（年龄24 - 64岁），这些患者在sLTx前后均进行了HRCT扫描，且在sLTx后存活至少6个月。所有患者最初均接受环孢素A、泼尼松和硫唑嘌呤治疗。三名放射科医生通过设定纤维化评分[纤维化评分（FS）；每个肺叶范围为0 - 5]和磨玻璃影评分[磨玻璃影评分（GGS）；每个肺叶范围为0 - 5]来分析HRCT扫描。采用符号检验对系列变化（间隔：移植后12 - 96个月，每位患者进行2 - 4次HRCT检查）进行比较。