Service de Transplantation et Hépato-gastroentérologie, Hôpitaux Universitaires de Genève, Switzerland.
Service d'Hépato-Gastroentérologie, CHU de Tours, France.
Hepatology. 2021 Jul;74(1):364-378. doi: 10.1002/hep.31688. Epub 2021 Jun 11.
Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension.
Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n = 117), HCV infection (n = 110), or NAFLD (n = 46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 10 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6 kPa, respectively; P < 0.001). When compared with cirrhosis, a cutoff value of 10 kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20 kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD.
This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM < 10 kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20 kPa, PSVD is highly unlikely.
门-体静脉血管性肝病（PSVD）是一种罕见的门静脉高压症病因。PSVD 仍常被误诊为肝硬化，这强调了需要改进 PSVD 诊断策略。关于使用瞬态弹性成像（TE-LSM）测量 PSVD 中的肝硬度的数据有限。本研究的目的是评估 TE-LSM 区分 PSVD 与肝硬化的准确性，这些患者有门静脉高压的迹象。
这项回顾性多中心研究比较了根据血管性肝病兴趣小组标准诊断的 PSVD 患者与代偿性经活检证实的酒精性（n=117）、丙型肝炎病毒（HCV）感染（n=110）或非酒精性脂肪性肝病（NAFLD）（n=46）相关肝硬化患者的 TE-LSM。所有患者均有门脉高压的至少一个迹象，包括胃食管静脉曲张、脾肿大、门体侧支循环、腹水病史或血小板计数＜150×10 /L。纳入试验队列的 77 例 PSVD 患者的中位 TE-LSM（7.9kPa）低于酒精性、HCV 相关和 NAFLD 相关肝硬化患者（分别为 33.8、18.2 和 33.6kPa；P＜0.001）。与肝硬化相比，10kPa 的截断值对 PSVD 的诊断具有 97%的特异性和 85%的阳性预测值。20kPa 的截断值对排除 PSVD 的敏感性为 94%，阴性预测值为 97%。在这些患者中，94%得到了很好的分类。在包括 78 例 PSVD 患者的验证队列中，获得了更好的结果。
这项共纳入 155 例 PSVD 患者和 273 例肝硬化患者的研究表明，在有门静脉高压迹象的患者中，TE-LSM＜10kPa 强烈提示 PSVD。相反，当 TE-LSM＞20kPa 时，PSVD 极不可能。
