The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis

The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis
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DOI:
10.1093/rheumatology/41.6.676
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发表时间:
2002-06-01
期刊:
影响因子:
5.5
通讯作者:
Venn, A
Venn, A
中科院分区:
医学1区
文献类型:
--
作者:
Hubbard, R;Venn, A

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目标。先前的报告表明,患有与结缔组织病相关的纤维化肺泡炎(FA-CTD)的患者比患有“孤立的”隐源性纤维化肺泡炎(LCFA)的患者有更好的预后。本研究旨在比较 FA-CTD 和 LCFA 患者的一般人群样本的生存率以及与一般人群的生存率。方法。使用来自全科医学研究数据库的 107 名 FA-CTD 患者、872 名 LCFA 患者和 5958 名年龄、性别和全科医学匹配的对照患者的数据进行生存分析。使用 Cox 回归分析数据,调整一些潜在的混杂因素,包括年龄、性别、吸烟习惯和口服皮质类固醇的使用。结果。中位随访期为 2.1 年,在此期间,54 名 (50%) FA-CFA 患者、386 名 (44%) LCFA 患者和 601 名 (10%) 对照死亡。 FA-CTD、LCFA 和对照患者的死亡率分别为每 1000 人年 284 人、270 人和 41 人。调整年龄、性别、吸烟习惯和口服皮质类固醇暴露后,FA-CTD 患者的生存率略低于 LCFA 患者(风险比 1.20,95% 置信区间 0.90-1.61)。与一般人群对照相比,LCFA 或 FA-CTD 患者的预后要差得多(风险比 5.56,95% 置信区间 4.77-6.49)。结论。纤维化肺泡炎患者的中位生存期不到 3 年。我们没有发现任何证据支持之前关于 FA-CTD 患者预后更好的报道。
Objectives. Previous reports have suggested that patients who have fibrosing alveolitis in association with a connective tissue disease (FA-CTD) have a better prognosis than patients with 'lone' cryptogenic fibrosing alveolitis (LCFA). The present study was designed to compare the survival of a general population-based sample of patients with FA-CTD and LCFA both with each other and with the general population.Methods. A survival analysis was performed using data for 107 patients with FA-CTD, 872 with LCFA and 5958 controls matched for age, sex and general practice, drawn from the General Practice Research Database. The data were analysed using Cox regression, adjusting for a number of potential confounders, including age, gender, smoking habit and use of oral corticosteroids.Results. The median follow-up period was 2.1 yr and during this time 54 (50%) patients with FA-CFA, 386 (44%) patients with LCFA and 601 (10%) controls died. The mortality rates for patients with FA-CTD, LCFA and the controls were 284, 270 and 41 per 1000 person-yr respectively. After adjusting for age, gender, smoking habit and exposure to oral corticosteroids, patients with FA-CTD had a marginally worse survival than patients with LCFA (hazard ratio 1.20, 95% confidence interval 0.90-1.61). Compared with the general population controls, patients with either LCFA or FA-CTD had a considerably worse prognosis (hazard ratio 5.56, 95% confidence interval 4.77-6.49).Conclusions. The median survival in patients with fibrosing alveolitis is less then 3 yr. We found no evidence to support previous reports of a better prognosis amongst patients with FA-CTD.