Division of Gastroenterology, Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
European Reference Network on Hepatological Diseases (ERN RARE-LIVER), San Gerardo Hospital, Monza, Italy.
Hepatology. 2021 Sep;74(3):1496-1508. doi: 10.1002/hep.31810. Epub 2021 May 28.
Liver fibrosis holds a relevant prognostic meaning in primary biliary cholangitis (PBC). Noninvasive fibrosis evaluation using vibration-controlled transient elastography (VCTE) is routinely performed. However, there is limited evidence on its accuracy at diagnosis in PBC. We aimed to estimate the diagnostic accuracy of VCTE in assessing advanced fibrosis (AF) at disease presentation in PBC.
We collected data from 167 consecutive treatment-naïve PBC patients who underwent liver biopsy (LB) at diagnosis at six Italian centers. VCTE examinations were completed within 12 weeks of LB. Biopsies were scored by two blinded expert pathologists, according to the Ludwig system. Diagnostic accuracy was estimated using the area under the receiver operating characteristic curves (AUROCs) for AF (Ludwig stage ≥III). Effects of biochemical and clinical parameters on liver stiffness measurement (LSM) were appraised. The derivation cohort consisted of 126 patients with valid LSM and LB; VCTE identified patients with AF with an AUROC of 0.89. LSM cutoffs ≤6.5 and >11.0 kPa enabled to exclude and confirm, respectively, AF (negative predictive value [NPV] = 0.94; positive predictive value [PPV] = 0.89; error rate = 5.6%). These values were externally validated in an independent cohort of 91 PBC patients (NPV = 0.93; PPV = 0.89; error rate = 8.6%). Multivariable analysis found that the only parameter affecting LSM was fibrosis stage. No association was found with BMI and liver biochemistry.
In a multicenter study of treatment-naïve PBC patients, we identified two cutoffs (LSM ≤6.5 and >11.0 kPa) able to discriminate at diagnosis the absence or presence, respectively, of AF in PBC patients, with external validation. In patients with LSM between these two cutoffs, VCTE is not reliable and liver biopsy should be evaluated for accurate disease staging. BMI and liver biochemistry did not affect LSMs.
在原发性胆汁性胆管炎（PBC）中，肝纤维化具有重要的预后意义。常规使用振动控制瞬时弹性成像（VCTE）进行非侵入性纤维化评估。然而，关于其在 PBC 中的诊断准确性的证据有限。我们旨在评估 VCTE 在评估 PBC 患者初诊时晚期纤维化（AF）的诊断准确性。
我们从意大利六个中心的 167 例初治的 PBC 患者中收集了数据，这些患者在诊断时均接受了肝活检（LB）。VCTE 检查在 LB 后 12 周内完成。两名盲法专家病理学家根据 Ludwig 系统对活检进行评分。使用受试者工作特征曲线（AUROCs）评估 AF（Ludwig 分期≥III）的诊断准确性。评估了生化和临床参数对肝硬度测量（LSM）的影响。推导队列由 126 例具有有效 LSM 和 LB 的患者组成；VCTE 以 0.89 的 AUROC 识别出患有 AF 的患者。LSM 截断值≤6.5 和>11.0kPa 分别能够排除和确认 AF（阴性预测值[NPV]为 0.94；阳性预测值[PPV]为 0.89；误差率为 5.6%）。这些值在 91 例 PBC 患者的独立队列中得到了外部验证（NPV 为 0.93；PPV 为 0.89；误差率为 8.6%）。多变量分析发现，唯一影响 LSM 的参数是纤维化分期。BMI 和肝功能无相关性。
在一项针对初治 PBC 患者的多中心研究中，我们确定了两个截断值（LSM≤6.5 和>11.0kPa），它们能够在诊断时分别区分 PBC 患者中是否存在 AF，具有外部验证。在这两个截断值之间的 LSM 患者中，VCTE 不可靠，应评估肝活检以进行准确的疾病分期。BMI 和肝功能不影响 LSM。
