Predictors of end stage lung disease in a cohort of patients with scleroderma
Predictors of end stage lung disease in a cohort of patients with scleroderma
复制标题
DOI：
10.1136/ard.62.2.146
复制
发表时间：
2003-02-01
影响因子：
27.4
通讯作者：
Silman, AJ
中科院分区：
文献类型：
--
作者：
Morgan, C;Knight, C;Silman, AJ
目的：估计硬皮病患者严重肺部疾病的发生率，并确定首次评估时出现的特征组合，这将有助于预测未来严重肺部疾病的风险。方法：对 1982 年 1 月 1 日或之后发病且首次评估前疾病持续时间少于五年的 561 名患者的数据进行了分析。在初次就诊时进行了详细的临床和实验室评估。终末期肺部疾病被定义为需要持续流动伊洛前列素的肺动脉高压，或需要持续吸氧的肺纤维化，或死于硬皮病相关肺部疾病。患者状况于 1997 年 12 月 3 日确定。通过 Cox 回归分析确定了最佳预测因子子集。结果：总共 24 名患者达到了终末期肺病。 5、7 和 14 岁时的累积发生率分别为 4%、6% 和 12%。正如预期的那样，基线时的肺功能测试，包括弥散性肺活量（风险比（HR）= 18.2，95％置信区间（CI）3.5至93.8）或用力肺活量（HR = 4.1，95％CI 1.1至15.2）的最低三分之一，是终末期肺病的高度显着的预测因子。有趣的是，除了蛋白尿的存在外，其他基线变量，包括皮肤病的程度和血清学标志物，都不能预测严重的肺部疾病。结论：终末期肺部疾病在这个大队列中并不常见，但累积发病率随着时间的推移显着增加。可以通过肺功能的基线评估来预测风险。特别是那些基线时肺功能正常的人风险非常低。
Objectives: To estimate the incidence of severe lung disease in patients with scleroderma and identify the combination(s) of features present at first assessment which would be useful to predict future risk of severe lung disease.Methods: Data were analysed on 561 patients with disease onset occurring on or after I January 1982 and disease duration of less than five years before the first assessment. Detailed clinical and laboratory assessments were undertaken at the initial visit. End stage lung disease was defined as pulmonary hypertension requiring continuous ambulatory iloprost, or pulmonary fibrosis requiring continuous oxygen, or death from a scleroderma related lung disease. Patient status was determined at 3 1 December 1997. The best subset of predictors was identified by Cox regression analysis.Results: In all, 24 patients reached end stage lung disease. The cumulative incidences were 4%, 6%, and 12% at five, seven, and 14 years respectively. As expected, the lung function tests at baseline, including being in the lowest third of either diffusing lung capacity (hazard ratio (HR) = 18.2, 95% confidence interval (CI) 3.5 to 93.8) or of forced vital capacity (HR=4.1, 95% CI 1.1 to 15.2), were highly significant predictors of end stage lung disease. Interestingly, apart from the presence of proteinuria, none of the other baseline variables, including the extent of skin disease and serological markers, were predictive of severe lung disease.Conclusion: End stage lung disease was infrequent in this large cohort, but the cumulative incidence increased importantly with time. The risk can be predicted from baseline assessment of pulmonary function. In particular, those with normal pulmonary function at baseline are at very low risk.