肺功能测试、高分辨率计算机断层扫描、α1-抗胰蛋白酶测量以及系统性硬化症患者肺部受累的早期检测。

Pulmonary function tests, high-resolution computerized tomography, alpha1-antitrypsin measurement, and early detection of pulmonary involvement in patients with systemic sclerosis.

作者信息

Shahin A A, Sabri Y Y, Mostafa H A, Sabry E Y, Hamid M A, Gamal H, Shahin H A

机构信息

Department of Rheumatology and Rehabilitation, Faculty of Medicine, Kasr Eleini Hospital, Cairo University, Egypt.

出版信息

Rheumatol Int. 2001 Apr;20(3):95-100. doi: 10.1007/s002960000089.

DOI:10.1007/s002960000089

PMID:11354564

Abstract

OBJECTIVE

Pulmonary disease represents a major complication of systemic sclerosis (SSc). However, pulmonary involvement is commonly silent. In this study, we investigated the relationship between serum alpha1-antitrypsin and other means of assessing pulmonary involvement.

METHODS

Twenty-two patients affected by SSc were studied (mean age 37.6+/-14.3 years, mean duration of disease 9.9+/-11.9 years). Fourteen had the diffuse form of disease (dSSc) and eight had the limited form (lSSc). All patients underwent pulmonary function tests, high-resolution computed tomography (HRCT) of the lungs, echocardiography, and serum assessment of alpha1-antitrypsin.

RESULTS

Mean percentage of predicted values of forced vital capacity was lower in patients with dSSc than with lSSc (72.3+/-17.8 vs 74.5+/-8, P=NS). Mean percentage of predicted values of forced expiratory volume in 1-s forced vital capacity (FEV1/FVC) was lower in patients with lSSc (79.8+/-7.5 for lSSc vs 84.4+/-7.8 for dSSc, P= NS). The overall HRCT score was 5.6+/-5.9 with no significant difference between disease subgroups. Pulmonary hypertension was detected in two cases, both with dSSc. Alpha1-antitrypsin was significantly higher in patients than in controls (P < 0.01), with no significant difference between disease subgroups, and correlated significantly with ground glass opacities in H RCT (P < 0.05) and the detection of diffusion defects (r= -0.61, P<0.01). No significant correlation was observed between skin score or degree of dyspnea with HRCT score, lung volume, or carbon monoxide diffusing capacity.

CONCLUSION

Restrictive lung disease was more pronounced in patients with dSSc. Alpha1-antitrypsin levels correlated significantly with ground glass opacities, an early finding of pulmonary involvement in SSc. Extent and severity of skin involvement and degree of dyspnea were not related to pulmonary involvement.

摘要

目的

肺部疾病是系统性硬化症（SSc）的主要并发症。然而，肺部受累通常没有症状。在本研究中，我们调查了血清α1-抗胰蛋白酶与评估肺部受累的其他方法之间的关系。

方法

对22例SSc患者进行了研究（平均年龄37.6±14.3岁，平均病程9.9±11.9年）。其中14例为弥漫型疾病（dSSc），8例为局限型（lSSc）。所有患者均接受了肺功能测试、肺部高分辨率计算机断层扫描（HRCT）、超声心动图以及α1-抗胰蛋白酶的血清评估。

结果

dSSc患者的用力肺活量预测值平均百分比低于lSSc患者（72.3±17.8对74.5±8，P=无显著性差异）。lSSc患者的1秒用力呼气量占用力肺活量的百分比（FEV1/FVC）较低（lSSc为79.8±7.5，dSSc为84.4±7.8，P=无显著性差异）。总体HRCT评分为5.6±5.9，疾病亚组之间无显著差异。在2例患者中检测到肺动脉高压，均为dSSc。患者的α1-抗胰蛋白酶水平显著高于对照组（P<0.01），疾病亚组之间无显著差异，且与HRCT中的磨玻璃影显著相关（P<0.05）以及与弥散功能障碍的检测显著相关（r=-0.61，P<0.01）。未观察到皮肤评分或呼吸困难程度与HRCT评分、肺容积或一氧化碳弥散量之间存在显著相关性。