Seely J M, Jones L T, Wallace C, Sherry D, Effmann E L
Department of Radiology, British Columbia's Children's Hospital, Vancouver, Canada.
AJR Am J Roentgenol. 1998 Mar;170(3):691-7. doi: 10.2214/ajr.170.3.9490955.
The purpose of this study was to determine the prevalence of interstitial lung disease and the severity of disease in children with systemic sclerosis using high-resolution CT (HRCT).
Eleven children (mean age, 11 years) with scleroderma underwent HRCT, chest radiography, and pulmonary function testing. Eight of these 11 patients also underwent follow-up HRCT. HRCT studies were assessed by two observers for ground-glass attenuation, honeycombing, and other abnormalities. Profusion scores for ground-glass attenuation and honeycombing were determined by multiplying severity of disease by the percentage of lung involvement.
Chest radiographs predicted interstitial lung disease in only two patients, whereas HRCT showed interstitial lung disease in eight patients (p = .05). On HRCT, ground-glass attenuation was found in eight patients (73%), honeycombing in five patients (45%), linear opacities in six patients (55%), and subpleural micronodules in seven patients (64%). By the end of the study, 10 patients (91%) had evidence of interstitial lung disease on HRCT. Overall, profusion scores for these 10 patients showed four patients with mild, one with moderate, and five with severe disease. Also, HRCT revealed worsening disease in three of eight patients. We found no correlation between duration of scleroderma and severity of interstitial lung disease (p > .02). Seven patients with evidence of lung disease on HRCT had abnormal results on pulmonary function tests; patients with the highest scores for ground-glass attenuation had the most abnormal results on pulmonary function tests (p < .01).
HRCT shows significant pulmonary disease in children with systemic sclerosis, revealing abnormalities in 91% of our patients. Pulmonary disease should be suspected in children with scleroderma, even if the chest radiograph has normal findings.
本研究旨在利用高分辨率计算机断层扫描（HRCT）确定系统性硬化症患儿间质性肺疾病的患病率及疾病严重程度。
11例硬皮病患儿（平均年龄11岁）接受了HRCT、胸部X线摄影及肺功能测试。这11例患者中的8例还接受了HRCT随访。两名观察者对HRCT研究结果进行评估，观察磨玻璃样影、蜂窝状改变及其他异常情况。磨玻璃样影和蜂窝状改变的病变程度评分通过疾病严重程度乘以肺部受累百分比来确定。
胸部X线摄影仅预测出2例间质性肺疾病，而HRCT显示8例患儿存在间质性肺疾病（p = 0.05）。HRCT检查发现，8例（73%）患儿有磨玻璃样影，5例（45%）有蜂窝状改变，6例（55%）有线状阴影，7例（64%）有胸膜下微小结节。到研究结束时，10例（91%）患儿的HRCT显示有间质性肺疾病证据。总体而言，这10例患者的病变程度评分显示，4例为轻度，1例为中度，5例为重度。此外，HRCT显示8例患者中有3例病情恶化。我们发现硬皮病病程与间质性肺疾病严重程度之间无相关性（p > 0.02）。HRCT显示有肺部疾病证据的7例患者肺功能测试结果异常；磨玻璃样影评分最高的患者肺功能测试结果异常最明显（p < 0.01）。
HRCT显示系统性硬化症患儿存在明显的肺部疾病，在我们91%的患者中发现了异常情况。硬皮病患儿即使胸部X线摄影结果正常，也应怀疑存在肺部疾病。
