Burgart L J, Batts K P, Ludwig J, Nikias G A, Czaja A J
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
Am J Surg Pathol. 1995 Jun;19(6):699-708. doi: 10.1097/00000478-199506000-00010.
Whereas the histologic findings in clinically "chronic" autoimmune hepatitis have been well established, with piecemeal necrosis as a hallmark lesion, the histologic findings of clinically "acute" or recent-onset autoimmune hepatitis remain undefined. The goal of this study was to define more fully the liver histomorphology in patients with recent-onset autoimmune hepatitis. Twenty-six patients were identified at our institution who had well-characterized autoimmune hepatitis and had undergone a liver biopsy within 6 months of clinical presentation. A detailed histologic evaluation revealed evidence of chronic liver disease in 25 (of 26) patients despite the lack of correlating clinical chronicity. The histologic evidence of chronicity included, in addition to a portal lymphoplasmacytic infiltrate, bridging (septal) fibrosis (11 patients) and overt cirrhosis (four patients). Eighteen of these 25 cases with evidence of chronicity also showed zone 2 and 3 lobular hepatitis, including disarray and hepatocyte necrosis. A single case showed lobular hepatitis with confluent hepatocyte necrosis (submassive hepatocellular necrosis), but no evidence of chronic liver disease. Although autoimmune hepatitis remains in the differential diagnosis of lobular hepatitis, these data show that most patients with autoimmune hepatitis who undergo biopsy early in its clinical course will have histologic evidence of chronic liver disease. Most of these patients probably have a lobular "flare" in disease activity, which likely precipitated the clinical presentation. The findings herein reinforce the concept that autoimmune hepatitis is by definition a chronic disease and supports the proposal that the modifier "chronic" be eliminated from autoimmune hepatitis.
虽然临床上“慢性”自身免疫性肝炎的组织学表现已得到充分证实，以界面性坏死作为标志性病变，但临床上“急性”或新近发病的自身免疫性肝炎的组织学表现仍不明确。本研究的目的是更全面地明确新近发病的自身免疫性肝炎患者的肝脏组织形态学。我们机构确定了26例具有明确特征的自身免疫性肝炎患者，他们在临床表现后6个月内接受了肝活检。详细的组织学评估显示，26例患者中有25例存在慢性肝病的证据，尽管缺乏相关的临床慢性表现。慢性的组织学证据除了门管区淋巴细胞浆细胞浸润外，还包括桥接（间隔）纤维化（11例患者）和明显的肝硬化（4例患者）。这25例有慢性证据的病例中有18例还表现为2区和3区小叶性肝炎，包括肝细胞排列紊乱和坏死。1例表现为小叶性肝炎伴融合性肝细胞坏死（亚大块肝细胞坏死），但无慢性肝病证据。虽然自身免疫性肝炎仍在小叶性肝炎的鉴别诊断范围内，但这些数据表明，大多数在临床病程早期接受活检的自身免疫性肝炎患者会有慢性肝病的组织学证据。这些患者中的大多数可能在疾病活动期有小叶“炎症发作”，这可能促使了临床表现。本文的研究结果强化了自身免疫性肝炎本质上是一种慢性疾病的概念，并支持从自身免疫性肝炎中去除修饰词“慢性”的提议。
