Harari Sergio, Caminati Antonella, Confalonieri Marco, Poletti Venerino, Vancheri Carlo, Pesci Alberto, Rogliani Paola, Luppi Fabrizio, Agostini Carlo, Rottoli Paola, Sanduzzi Zamparelli Alessandro, Sebastiani Alfredo, Della Porta Rossana, Salton Francesco, Messore Barbara, Tomassetti Sara, Rosso Roberta, Biffi Alice, Puxeddu Ermanno, Cerri Stefania, Cinetto Francesco, Refini Rosa Metella, Bocchino Marialuisa, Di Michele Loreta, Specchia Claudia, Albera Carlo
U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare. Ospedale San Giuseppe - MultiMedica, IRCCS, Milano, Italy.
Department of Pulmonology, University Hospital of Cattinara, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy.
Clin Respir J. 2019 Mar;13(3):166-173. doi: 10.1111/crj.12999.
Gender, age, physiology (GAP) system have proven to be an easy tool for predicting disease stages and survival in idiopathic pulmonary fibrosis (IPF) patients.
To validate mortality risk as determined by the GAP system in a real-life multicentre IPF population treated with pirfenidone.
The study included patients who received pirfenidone for at least 6 months. The GAP calculator and the GAP index were determined. The primary outcome was all-cause mortality. The prognostic accuracy of the GAP system was evaluated with respect to calibration and discrimination.
Sixty-eight IPF patients were enrolled in the study. The median follow-up was 2.4 years (range 0.1-7.4 years). A total of 22 deaths as first event (32%) and of 10 lung transplantation (15%) were recorded. The cumulative incidence of mortality at 1, 2 and 3 years was 10.4%, 22.4% and 38.4%, respectively. The differences between the predicted and observed mortality were not significant for the GAP index while the observed mortality become comparable to that predicted by the GAP calculator only in the third year of follow-up. The C-index for the GAP index was 0.74 (95% CI 0.57-0.93) while the C-statistic value for the GAP calculator was 0.77 (95% CI 0.59-0.95).
性别、年龄、生理（GAP）系统已被证明是预测特发性肺纤维化（IPF）患者疾病阶段和生存情况的简便工具。
验证GAP系统所确定的死亡风险在接受吡非尼酮治疗的真实多中心IPF人群中的情况。
该研究纳入了接受吡非尼酮治疗至少6个月的患者。确定了GAP计算器和GAP指数。主要结局是全因死亡率。从校准和区分度方面评估了GAP系统的预后准确性。
68例IPF患者纳入该研究。中位随访时间为2.4年（范围0.1 - 7.4年）。记录到22例作为首要事件的死亡（32%）和10例肺移植（15%）。1年、2年和3年的累积死亡率分别为10.4%、22.4%和38.4%。GAP指数预测死亡率与观察到的死亡率之间差异不显著，而仅在随访第三年观察到的死亡率与GAP计算器预测的死亡率相当。GAP指数的C指数为0.74（95%CI 0.57 - 0.93），而GAP计算器的C统计值为0.77（95%CI 0.59 - 0.95）。
