Pulmonology Department, Centro Hospitalar e Universitário de São João, Alameda Prof. Hernâni Monteiro, Porto 4200-319, Portugal.
Pulmonology Department, Hospital Pedro Hispano, Matosinhos, Portugal.
Ther Adv Respir Dis. 2022 Jan-Dec;16:17534666221135316. doi: 10.1177/17534666221135316.
Fibrotic hypersensitivity pneumonitis (fHP) is associated with significant morbidity and mortality. Interstitial lung disease-gender-age-physiology (ILD-GAP) performance in fHP outside the initial cohort was never performed.
To assess the ILD-GAP index's ability to predict mortality in a Portuguese cohort of patients with fHP and analyse whether other clinical variables add value.
Retrospective analysis of fHP cohort in two Portuguese ILD centres. The baseline ILD-GAP index was calculated. Survival was analysed in months; mortality was the primary outcome. Univariate and multivariate analyses to identify mortality risk factors were performed.
A total of 141 patients were included. Fifty-three patients (37.6%) died during the follow-up. The usual interstitial pneumonia (UIP) pattern was found in 49.6%, and their survival was inferior to non-UIP [32 months (interquartile range, IQR = 19, 60) versus 52 months (IQR = 28, 98), = 0.048]. Patients with an ILD-GAP index higher than three double their risk of mortality [hazard ratio (HR) = 6.48, 95% confidence interval (CI) = (3.03-13.96)] when compared with the patients with an index between 2 and 3 [HR = 3.04, 95% CI = (1.62-5.71)] adjusting for acute exacerbation history. Even though UIP patients had worse survival, it did not reach statistical significance when UIP pattern was added to this model. Acute exacerbation history was an independent risk factor for mortality; however, ILD-GAP still predicted mortality after adjusting for this factor. PaO and 6-minute walk test desaturation were not significant risk factors.
ILD-GAP index is a good predictor for mortality in fHP, even after adjusting for other mortality risk factors.
纤维化性过敏性肺炎（fHP）与较高的发病率和死亡率相关。ILD-GAP 评分在最初队列之外的 fHP 中的表现从未被评估过。
评估 ILD-GAP 指数在葡萄牙 fHP 患者队列中的预测死亡率的能力，并分析其他临床变量是否具有附加价值。
对葡萄牙两家间质性肺病中心的 fHP 队列进行回顾性分析。计算基线 ILD-GAP 指数。以月为单位分析生存情况；死亡率为主要终点。进行单变量和多变量分析以确定死亡风险因素。
共纳入 141 例患者，53 例（37.6%）在随访期间死亡。49.6%的患者存在寻常型间质性肺炎（UIP）模式，其生存率低于非 UIP 模式[32 个月（四分位距，IQR=19，60）与 52 个月（IQR=28，98），=0.048]。ILD-GAP 指数大于 3 的患者死亡风险是指数在 2 到 3 之间的患者的两倍[风险比（HR）=6.48，95%置信区间（CI）=（3.03-13.96）]，调整急性加重史后[HR=3.04，95%CI=（1.62-5.71）]。尽管 UIP 患者的生存率较差，但在将 UIP 模式添加到该模型后，其差异并未达到统计学意义。急性加重史是死亡的独立危险因素；然而，在调整了该因素后，ILD-GAP 仍然预测了死亡率。PaO 和 6 分钟步行试验的低氧饱和度不是显著的危险因素。
即使在调整了其他死亡风险因素后，ILD-GAP 指数也是 fHP 死亡率的良好预测指标。
