摘要目的 比较现行日本呼吸学会(Japanese respiratory society,JRS)评分系统和GAP(gender,age and physiologic variables)分期标准在评估特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)患者疾病严重程度上的一致性及临床应用价值.方法 回顾性收集2011年1月至2016年1月在上海市肺科医院住院期间确诊1PF的155例患者,其中男149例,女6例,年龄40～80岁,平均(63±7)岁.分别使用JRS评分系统及GAP分期标准对患者进行疾病严重度分期,JRS评分I期91例(91/155,58.7％),Ⅱ期29例(29/155,18.7％),Ⅲ期24例(24/155,15.5％),Ⅳ期11例(11/155,7.1％);GAP分期I期89例(89/155,57.4％),Ⅱ期52例(52/155,33.5％),Ⅲ期14例(14/155,9.0％).所有患者均进行为期2年的随访,分析不同严重程度患者的预后及生存情况.结果 按照GAP分期,各期患者的年龄、性别、体征、脉搏氧饱和度(SpO2)、超敏C反应蛋白、圣乔治评分(SGRQ)、二氧化碳分压(PaCO2)、6 min步行试验及6 min步行试验后SpO2差异无统计学意义(均P＞0.05);吸烟史(各期分别为68、37、9例)、存在呼吸道症状(各期分别为70、48、14例)、合并肺动脉高压(各期分别为22、20、8例)、ESR[各期分别为(24±17)、(30±21)、(41±22) mm/1 h]、PaO2[各期分别为(92±24)、(81±20)、(74±15) mmHg,1 mmHg=0.133 kPa]、FVC[各期分别为(2.9±0.6)、(2.2±0.5)、(1.6±0.3)L]及FEV1[各期分别为(2.4±0.5)、(1.8±0.5)、(1.4±0.3)L]差异有统计学意义(P＜0.05).按照JRS评分,各期患者的年龄、性别、吸烟史、呼吸道症状及体征、超敏CRP、ESR、圣乔治评分、6 min步行试验及FEV1差异无统计学意义(P＞0.05);PaO2[各期分别为(99±22)、(76±3)、(66±3)、(54±4) mmHg]、PaCO2[各期分别为(39±3)、(40±3)、(39±4)、(38±5)]、SpO2[各期分别为(97.2±1.2)％、(95.2±1.0)％、(93.2±1.3)％、(87.4±4.1)％]、6 min步行试验后SpO2[各期分别为(94.1±1.0)％、(93.2±1.2)％、(90.2±1.1)％、(87.4±4.1)％]、合并肺动脉高压(各期分别为21、13、11、5例)及FVC[各期分别为(2.6±0.7)、(2.6±0.7)、(2.2±0.6)、(2.2±0.7)L]差异有统计学意义(P＜0.05).两种评分方法结果存在一致性(P＜0.05),但一致性不强(Kappa值＜0.75).按照GAP分期标准的各期患者1年生存率差异无统计学意义(P＞0.05),2年生存率差异有统计学意义(P＜0.05).按照JRS评分系统的各期患者1年及2年生存率差异均无统计学意义(P＞0.05).结论 GAP分期标准和JRS评分系统在评估IPF患者疾病严重程度上准确性和一致性均不高,两种评分方法在评估生存率方面未能体现出价值.

abstractsObjective To compare the existing JRS(Japanese respiratory society) scoring system and GAP(gender,age,and physiologic variables)staging criterion regarding to the consistency and the clinical application value of evaluating the severity of idiopathic pulmonary fibrosis(IPF).Methods A total of 155 patients with IPF diagnosed by Shanghai Pulmonary Hospital of Tongji University between January,2011 and January,2016 were collected (male 149,female 6,age 40-80,average age 63±7).The patients were staged by the GAP staging criterion and JRS scoring system,respectively.According to the JRS scoringsystem,patients with stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were 91 (91/155,58.7％),29 (29/155,18.7％),24 (24/155,15.5％),and 11 (11/155,7.1％),respectively.According to the GAP staging criterion,patients with stage Ⅰ,Ⅱ,Ⅲ were 89(89/155,57.4％),52 (52/155,33.5％),and 14 (14/155,9.0％),respectively.Then,we evaluated the statistical significance of patients at each stage.All patients were followed up for 2 years in order to record the prognosis and survival.Finally,we compared the advantages and the disadvantages of these two systems and evaluated the clinical application value of the two systems.Results The distribution of age,sex,physical sign,SaO2％,high-sensitivity-C-reactive protein (hs-CRP),the St George's Respiratory Questionnaire(SGRQ),partial pressure of carbon dioxide (PaCO2),6-min walk test (6MWT) and the oxygen saturation on pulse oximetry (SpO2％) during a 6MWT by the GAP staging criterion in each stage was not statistically significant (P＞0.05).While smoking history (number of patients with stage Ⅰ,Ⅱ,Ⅲ were 68,37,9,respectively],respiratory symptoms (number of patients with stage Ⅰ,Ⅱ,Ⅲ were 70,48,14,respectively],pulmonary hypertension (number of patients with stage Ⅰ,Ⅱ,Ⅲ were 22,20,8,respectively],erythrocyte sedimentation rate [value of stage Ⅰ,Ⅱ,Ⅲ were (24±17),(30±21),(41±22)mm/h,respectively],PaO2 [value of stage Ⅰ,Ⅱ,Ⅲ were (92±24),(81±20),(74±15)mmHg,respectively],FVC [value of stage Ⅰ,Ⅱ,Ⅲ were (2.86±0.59),(2.20±0.5),(1.56±0.27)L,respectively] and FEV1 [value of stage Ⅰ,Ⅱ,Ⅲ were (2.35±0.46),(1.81±0.46),(1.35±0.27)L,respectively] had statistical significance in the GAP staging criterion in each stage (P＜0.05).The distribution of age,sex,smoking history,respiratory symptoms and sign,hs-CRP,blood sedimentation,the St George's Respiratory Questionnaire(SGRQ),6-min walk test (6MWT) and FEV1 by JRS scoring system in each stage was not statistically significant (P＞0.05).While PaO2 [value of stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were (99±22),(76±3),(66±3),(54±4) mmHg,respectively],PaCO2 [value of stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were (39±3),(40±3),(39±4),(38±5)mmHg,respectively],SaO2％ [value of stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were (97.2± 1.2)％,(95.2± 1.0)％,(93.2± 1.3)％,(87.4±4.1)％,respectively],SpO2％ [value of stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were (94.1 ± 1.0)％,(93.2± 1.2)％,(90.2± 1.1)％,(87.4±4.1)％,respectively] during a 6MWT,pulmonary hypertension (number of patients with stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were 21,13,11,5,respectively] and FVC [value of stage Ⅰ,Ⅱ,Ⅲ,Ⅳ were (2.63±0.68),(2.55±0.70),(2.19±0.59),(2.20±0.67)L,respectively] by JRS scoring system in each stage were statistically significant (P＜0.05).The results of the two scoring methods were consistent (P＜0.05),but the consistency was not strong (Kappa value＜0.75).There was no statistical significance in the one-year survival rate of the GAP staging criterion and the one-year and two-year survival rates of JRS scoring system.Conclusions The GAP staging criterion and JRS scoring system had poor accuracy and consistency in assessing the disease severity in IPF patients.The two scoring methods showed insufficient value in assessing the survival rates.