摘要
肺朗格汉斯细胞肉芽肿病(肺LCG)的病程是可变的,难以预测,从自发性缓解到进行性呼吸功能不全和死亡不等。为了确定生存的决定因素,我们对45例肺LCG患者进行了生存分析。患者年龄28 +/- 10岁(平均+/- SD)(12-62岁),32名男性和13名女性,几乎完全是目前的吸烟者(96%),78%在诊断时有症状。25例(56%)通过肺活检确诊,20例(44%)通过支气管肺泡灌洗(BAL)分析确诊。患者在确诊后的中位随访时间为6年(范围1-29年)。在观察期间,33例(73%)患者存活(中位随访期= 5.8年;范围,1-29岁)和12例(27%)死亡或接受肺移植(中位随访期= 8.4年;范围1.4 - 16.1年)。中位生存率约为13年。单因素分析显示,生存率降低与以下因素显著相关:确诊时年龄较大(p = 0.0001); a lower forced expiratory volume in one second/forced vital capacity (FEV1/FVC) ratio at diagnosis (p = 0.005); a higher residual volume/total lung volume (RV/TLC) ratio at diagnosis (p = 0.02); and steroid therapy during follow-up (p = 0.03). Additional predictive information on mortality was: age > 26 yrs (sensitivity 83%, specificity 64%); FEV1/FVC ratio < 0.66 (sensitivity 75%, specificity 86%); and a RV/TLC ratio > 0.33 (sensitivity 75%, specificity 63%). In multivariate Cox analysis, the combination of factors which gave the best prognostic value was FEV1/FVC ratio and age (p < 0.01). The present findings suggest that adverse prognosis factors at diagnosis in pulmonary Langerhans' cell granulomatosis include older age, lower FEV1/FVC ratio and higher RV/TLC ratio, with additional predictive information on mortality if aged > 26 yrs, FEV1/FVC ratio < 0.66, and RV/TLC ratio > 0.33.