74例特发性非硬化性门静脉高压症患者的临床特征分析

Clinical features of idiopathic non-cirrhotic portal hypertension:An analysis of 74 patients

目的 特发性非硬化性门静脉高压(INCPH)是一种少见的引起门静脉高压的病因,对INCPH患者的临床特点进行分析以辅助诊断及鉴别诊断。方法 选取北京佑安医院2019年1月-2022年7月经肝穿刺病理明确诊断为INCPH的住院患者74例,并以同期住院的332例肝硬化患者作为对照组,记录其人口学指标、实验室指标、胃镜、肝弹性检查、病理检查及并发症等资料,进行组间差异比较,并根据受试者工作特征曲线(ROC曲线)评价LSM、APRI、FIB-4对INCPH的鉴别诊断能力,采用DeLong检验方法进行ROC曲线下面积(AUC)比较。符合正态分布的计量资料两组间比较采用成组t检验,非正态分布的计量资料两组间比较采用Mann-Whitney U检验。计数资料两组间比较采用χ^(2)检验。结果 INCPH患者中46.55%的患者起病时无明显症状,43.24%患者曾被误诊为肝硬化。INCPH患者合并消化道出血比例明显多于肝硬化患者(62.16%vs 41.27%,χ^(2)=10.67,P<0.01),但合并中-重度腹水患者比例明显少于肝硬化患者(16.21%vs 29.82%,χ^(2)=34.98,P<0.01),无肝性脑病发生。病理方面,89.19%(66/74)患者病理表现为典型的闭塞性门静脉病。INCPH患者肝功能指标、MELD评分、Child-Pugh评分等指标明显优于肝硬化患者,肝硬度值[9.05(7.18~12.33) vs 25.32(16.21~47.23),Z=-8.41,P<0.01]、APRI评分[0.70(0.41~1.28) vs 1.35 (0.80~2.39),Z=-6.21,P<0.01]、FIB-4指数[2.99(1.62~4.81) vs 6.68(4.06~10.42),Z=-8.39,P<0.01]均较肝硬化低。LSM、FIB-4、APRI对INCPH与肝硬化患者具有很好的鉴别诊断能力,尤其是LSM的AUC达到0.92(0.87~0.96),敏感度和特异度分别为92.68%和81.60%。结论 INCPH患者起病较隐匿,门静脉高压相关并发症发生率较高,而肝功能相对较好,尤其是LSM<14.5 kPa的患者,临床遇到此类患者需警惕INCPH可能。

Objective Idiopathic non-cirrhotic portal hypertension(INCPH)is a rare cause of portal hypertension,and this study aims to analyze the clinical features of patients with INCPH,and to assist in diagnosis and differential diagnosis.Methods A total of 74 patients who were hospitalized in Beijing YouAn Hospital from January 2019 to July 2022 and were diagnosed with INCPH were enrolled,and 332 patients with liver cirrhosis who were hospitalized during the same period of time were enrolled as control group.Demographic data,laboratory markers,gastroscopy,liver elasticity,pathological examination,and complications were recorded and compared between the two groups.The receiver operating characteristic(ROC)curve was used to investigate the ability of liver stiffness measurement(LSM),aspartate aminotransferase-to-platelet ratio index(APRI),and fibrosis-4(FIB-4)in the differential diagnosis of INCPH,and the DeLong test was used to compare the area under the ROC curve(AUC).The independent-samples t test was used for comparison of normally distributed continuous data between two groups,and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups;the chi-square test was used for comparison of categorical data between two groups.[WTHZ]Results Among the patients with INCPH,46.55%had no obvious symptoms at disease onset and 43.24%were misdiagnosed with liver cirrhosis.Compared with the patients with liver cirrhosis,the patients with INCPH had a significantly higher proportion of patients with gastrointestinal bleeding(62.16%vs 41.27%,χ^(2)=10.67,P<0.01)and a significantly lower proportion of patients with moderate-to-severe ascites(16.21%vs 29.82%,χ^(2)=34.98,P<0.01),and there were few patients with hepatic encephalopathy.As for pathology,89.19%(66/74)of the INCPH patients manifested as typical occlusive portal vein disease.The statistical analysis showed that compared with the patients with liver cirrhosis,the patients with INCPH had significantly better liver function parameters,MELD score,and Child-Pugh score and significantly lower LSM[9.05(7.18-12.33)vs 25.32(16.21-47.23),Z=-8.41,P<0.01],APRI score[0.70(0.41-1.28)vs 1.35(0.80-2.39),Z=-6.21,P<0.01],and FIB-4 index[2.99(1.62-4.81)vs 6.68(4.06-10.42),Z=-8.39,P<0.01].LSM,FIB-4,and APRI had a good ability in differentiating INCPH from liver cirrhosis,and in particular,LSM had an AUC of up to 0.92(95%[JP3]confidence interval:0.87-0.96),with a sensitivity of 92.68%and a specificity of 81.60%.[WTHZ]Conclusion INCPH patients tend to have an insidious onset,a relatively high incidence rate of portal hypertension-related complications,and relatively good liver function,especially the patients with LSM<14.5 kPa.The possibility of INCPH should be considered for such patients in clinical practice.