Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Eur J Gastroenterol Hepatol. 2013 Mar;25(3):333-7. doi: 10.1097/MEG.0b013e32835a68a1.
Autoimmune liver disease (AILD) requires a constellation of clinical, serological, biochemical, and histological findings for diagnosis. Liver biopsy forms the cornerstone for the definite diagnosis of AILD, despite histological features not being pathognomonic. Liver biopsies of AILD and nonautoimmune chronic liver disease (NACLD) were reviewed blindly to assess the role of typical histological findings in differentiating AILD from NACLD in a pediatric population.
Twenty-five liver biopsies of AILD and 34 liver biopsies of NACLD were reviewed retrospectively without knowledge of the final diagnosis.
The typical histology comprising all four features, interface hepatitis, portal lymphoplasmacytic infiltrate, rosette formation, and emperipolesis, was observed in 56% of AILD. Rosette formation and emperipolesis were associated significantly with the diagnosis of AILD. Rosette formation alone or in combination with emperipolesis or lymphoplasmacytic infiltrate had high specificity (96.2% each) but low sensitivity (68, 60, and 60%, respectively) for AILD. The diagnostic accuracy of typical histology comprising of a combination of at least three of four features, rosette formation, emperipolesis, and lymphoplasmacytic infiltrate, was 76.9%, with a positive predictive value of 93.3% and a negative predictive value of 70.2%.
Characteristic patterns of liver injury comprising typical histological features on liver biopsy may strongly suggest the diagnosis of AILD irrespective of other laboratory parameters in children. Rosette formation was the only independent significant histological factor to predict AILD. High specificity and predictability of typical histological features may be helpful in diagnosing seronegative AILD among cases of cryptogenic liver disease in the absence of other supportive findings.
自身免疫性肝病（AILD）的诊断需要结合临床、血清学、生化和组织学表现。尽管组织学特征不是特异性的，但肝活检仍是 AILD 明确诊断的基石。本研究回顾性分析了自身免疫性肝病和非自身免疫性慢性肝病（NACLD）的肝活检，旨在评估典型组织学表现在儿童人群中区分 AILD 和 NACLD 的作用。
回顾性分析了 25 例 AILD 和 34 例 NACLD 的肝活检，在不了解最终诊断的情况下进行盲法评估。
在 56%的 AILD 中观察到包含所有四个特征的典型组织学表现，即界面肝炎、门脉浆细胞浸润、玫瑰花环形成和吞噬现象。玫瑰花环形成和吞噬现象与 AILD 的诊断显著相关。单独的玫瑰花环形成或与吞噬现象或浆细胞浸润联合具有高特异性（分别为 96.2%），但对 AILD 的敏感性较低（分别为 68%、60%和 60%）。包含至少三种典型组织学特征（玫瑰花环形成、吞噬现象和浆细胞浸润）的组合的诊断准确性为 76.9%，阳性预测值为 93.3%，阴性预测值为 70.2%。
肝活检上包含典型组织学特征的特征性肝损伤模式可能强烈提示 AILD 的诊断，无论其他实验室参数如何。玫瑰花环形成是唯一独立的预测 AILD 的重要组织学因素。典型组织学特征的高特异性和可预测性可能有助于在缺乏其他支持性发现的情况下，诊断隐匿性肝病中的血清阴性 AILD。
