Poupon R, Chazouillères O, Balkau B, Poupon R E
Service d'Hépato-Gastroentérologie, Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris, France.
J Hepatol. 1999 Mar;30(3):408-12. doi: 10.1016/s0168-8278(99)80098-1.
BACKGROUND/AIMS: This study aimed to assess the relationships which may link elementary histological lesions with symptoms and biochemistries in primary biliary cirrhosis.
We studied 103 patients with primary biliary cirrhosis who participated in a double-blind, placebo-controlled trial of UDCA treatment and in whom liver biopsy specimens obtained at entry were reassessed. Relationships between histological features, fatigue, pruritus and biochemistries were calculated by using exact tests for 2 ordinal variables.
The degrees of severity of fatigue and pruritus were significantly and exclusively related to the presence of florid interlobular bile duct lesions (p<0.01 and p<0.02, respectively). The only laboratory parameter associated with the presence of interlobular bile duct florid lesion was IgM level. The most discriminant biochemical test for interlobular bile duct paucity was gamma glutamyltranspeptidase activity. The degree of severity of both lymphocytic hepatocellular piecemeal necrosis and lobular inflammation and necrosis was mainly associated with increased gammaglobulin and IgG levels and to a lesser extent with increased IgM and aspartate aminotransferase levels. The extent of fibrosis was mainly associated with gammaglobulin levels and to a lesser degree with serum albumin, bilirubin and IgG levels.
Symptoms and biochemistries classically used to assess primary biliary cirrhosis reflect in part the degree of severity of the main elementary histological lesions. We propose that the picture of primary biliary cirrhosis results from the clinical and biochemical expression of three distinct processes, e.g., bile duct inflammation and destruction, parenchymal inflammation and necrosis, and fibrosis. The various combinations of these processes may explain why the spectrum of primary biliary cirrhosis varies from typical primary biliary cirrhosis to mixed type of primary biliary cirrhosis and autoimmune hepatitis and suggests that the response to therapies may depend on the predominance of each process in a given patient.
背景/目的：本研究旨在评估原发性胆汁性肝硬化中可能将基本组织学病变与症状及生化指标联系起来的关系。
我们研究了103例原发性胆汁性肝硬化患者，这些患者参与了熊去氧胆酸治疗的双盲、安慰剂对照试验，并对入组时获取的肝活检标本进行了重新评估。通过对两个有序变量进行精确检验来计算组织学特征、疲劳、瘙痒与生化指标之间的关系。
疲劳和瘙痒的严重程度与小叶间胆管显著病变的存在显著且唯一相关（分别为p<0.01和p<0.02）。与小叶间胆管显著病变存在相关的唯一实验室参数是IgM水平。对小叶间胆管缺乏最具鉴别力的生化检测是γ-谷氨酰转肽酶活性。淋巴细胞性肝细胞桥接坏死以及小叶炎症和坏死的严重程度主要与γ球蛋白和IgG水平升高相关，在较小程度上与IgM和天冬氨酸转氨酶水平升高相关。纤维化程度主要与γ球蛋白水平相关，在较小程度上与血清白蛋白、胆红素和IgG水平相关。
传统上用于评估原发性胆汁性肝硬化的症状和生化指标部分反映了主要基本组织学病变的严重程度。我们提出原发性胆汁性肝硬化的情况是由三个不同过程的临床和生化表现引起的，即胆管炎症和破坏、实质炎症和坏死以及纤维化。这些过程的不同组合可以解释为什么原发性胆汁性肝硬化的范围从典型的原发性胆汁性肝硬化到原发性胆汁性肝硬化与自身免疫性肝炎的混合型，并且表明对治疗的反应可能取决于特定患者中每个过程的主导程度。
