系统性硬化症中皮肤和肺部受累之间的关系。

Relationship between cutaneous and pulmonary involvement in systemic sclerosis.

作者信息

Morelli S, Barbieri C, Sgreccia A, Ferrante L, Pittoni V, Conti F, Gualdi G, Polettini E, Carlesimo O A, Calvieri S

机构信息

Istituto di I Clinica Medica, Università La Sapienza, Rome, Italy.

出版信息

J Rheumatol. 1997 Jan;24(1):81-5.

PMID:9002015

Abstract

OBJECTIVE

Pulmonary disease may shorten survival in patients affected by systemic sclerosis (SSc). However, pulmonary involvement may commonly be silent, whereas skin fibrosis is usually the clinical feature drawing most attention. We investigated the relationship between cutaneous and pulmonary involvement during SSc.

METHODS

We studied 52 patients (mean age 50.2 +/- 13.7 years) affected by SSc (mean duration of disease 13.8 +/- 9.5 years). Twenty-eight had the diffuse form of the disease (dSSc) and 24 the limited form (lSSc). All patients underwent pulmonary function studies, high resolution computed tomography (HRCT) of the lungs, and complete echocardiographic examination. Pulmonary artery systolic pressure was measured by Doppler echocardiography. Pulmonary interstitial fibrosis and skin fibrosis were evaluated using a point system.

RESULTS

Mean percentages of predicted values of forced vital capacity and total lung capacity were significantly reduced in patients with dSSc compared to lSSc (80.0 +/- 18.9 vs 98.4 +/- 16.8%, p < 0.001; and 81.3 +/- 13.9 vs 92.1 +/- 14.2%, p < 0.01, respectively). The overall HRCT score was 6.1 +/- 4.9, with no significant differences between disease subgroups. However, a HRCT score of 10 or more was present in 10 patients with dSSc vs 2 patients with lSSc (p = 0.02). Pulmonary hypertension was present in 27 patients, 15 with lSSC and 12 with dSSc (p = NS). No significant correlation was observed between skin score and lung volumes, carbon monoxide diffusing capacity, HRCT score, or pulmonary artery systolic pressure for all patients and subgroups.

CONCLUSION

Extent and severity of cutaneous and pulmonary involvement in SSc are not directly correlated. Nevertheless, different patterns of pulmonary involvement between SSc subgroups were observed. Restrictive lung disease was more frequent in patients with dSSc, while a trend to higher prevalence of pulmonary hypertension was observed in patients with lSSc.

摘要

目的

肺部疾病可能会缩短系统性硬化症（SSc）患者的生存期。然而，肺部受累通常可能没有症状，而皮肤纤维化通常是最受关注的临床特征。我们研究了SSc患者皮肤和肺部受累之间的关系。

方法

我们研究了52例SSc患者（平均年龄50.2±13.7岁，疾病平均病程13.8±9.5年）。其中28例为弥漫型疾病（dSSc），24例为局限型疾病（lSSc）。所有患者均接受了肺功能研究、肺部高分辨率计算机断层扫描（HRCT）以及完整的超声心动图检查。通过多普勒超声心动图测量肺动脉收缩压。使用评分系统评估肺间质纤维化和皮肤纤维化。

结果

与lSSc患者相比，dSSc患者的用力肺活量和肺总量预测值的平均百分比显著降低（分别为80.0±18.9%对98.4±16.8%，p<0.001；以及81.3±13.9%对92.1±14.2%，p<0.01）。HRCT总评分为6.1±4.9，疾病亚组之间无显著差异。然而，10例dSSc患者的HRCT评分为10分或更高，而lSSc患者为2例（p = 0.02）。27例患者存在肺动脉高压，15例lSSC患者和12例dSSc患者（p = 无显著性差异）。在所有患者和亚组中，未观察到皮肤评分与肺容积、一氧化碳弥散能力、HRCT评分或肺动脉收缩压之间存在显著相关性。