Matsumoto T, Kobayashi S, Shimizu H, Nakajima M, Watanabe S, Kitami N, Sato N, Abe H, Aoki Y, Hoshi T, Hashimoto H
First Department of Pathology, Juntendo University, School of Medicine, Japan.
Liver. 2000 Oct;20(5):366-73. doi: 10.1034/j.1600-0676.2000.020005366.x.
AIMS/BACKGROUND: Among patients with collagen diseases, liver enzyme abnormalities are a relatively common phenomenon. To establish the liver pathology in collagen diseases, detailed pathologic studies were performed on the hepatic diseases in many patients, including various kinds of collagen diseases.
The livers from 160 patients (120 autopsy and 40 liver biopsy patients) were examined pathologically: 73 with systemic lupus erythematosus (SLE), 32 with rheumatoid arthritis (RA), 18 with polymyositis and dermatomyositis (PM and DM), 15 with systemic sclerosis (SSc), 11 with mixed connective tissue disease (MCTD) and 11 with polyarteritis nodosa (PAN).
Liver diseases were divided into three groups: hepatic arteritis, liver diseases associated with collagen diseases (primary biliary cirrhosis, PBC; autoimmune hepatitis, AIH; nodular regenerative hyperplasia of the liver, NRH) and other liver diseases. Hepatic arteritis presenting the features of the PAN type of necrotizing arteritis was found in 27 autopsy patients. The incidence of arteritis in autopsy patients was 100% in PAN and 8.3-25% in other collagen diseases. Primary biliary cirrhosis was observed in 9 patients, 7 of whom (3 with SSc, 2 with RA, 1 with PM and DM, and 1 with MCTD) had antimitochondrial antibodies (AMA)-positive PBC, and 2 SLE patients had AMA-negative PBC. Three patients (2 with SLE and 1 with MCTD) were diagnosed clinicopathologically as having AIH. However, 3 patients (1 with SLE, 1 with MCTD and 1 with PM and DM) with clinical, biochemical and serologic data indicating probable AIH were excluded from the group with AIH association because of the liver histology (no characteristic features of AIH) and clinical course. These results indicated that data without histologic assessments of the liver are not adequate for diagnosing AIH in collagen diseases. Nodular regenerative hyperplasia of the liver was observed in 7 patients (5 with SLE, 1 with SSc and 1 with PAN).
The present study offers data that are useful for the diagnosis and treatment of patients with collagen diseases and liver abnormalities.
目的/背景：在胶原病患者中，肝酶异常是一种相对常见的现象。为了明确胶原病中的肝脏病理情况，我们对包括各种胶原病在内的许多患者的肝脏疾病进行了详细的病理研究。
对160例患者（120例尸检患者和40例肝活检患者）的肝脏进行病理检查：73例系统性红斑狼疮（SLE）患者，32例类风湿关节炎（RA）患者，18例多发性肌炎和皮肌炎（PM和DM）患者，15例系统性硬化症（SSc）患者，11例混合性结缔组织病（MCTD）患者和11例结节性多动脉炎（PAN）患者。
肝脏疾病分为三组：肝动脉炎、与胶原病相关的肝脏疾病（原发性胆汁性肝硬化，PBC；自身免疫性肝炎，AIH；肝脏结节性再生性增生，NRH）和其他肝脏疾病。在27例尸检患者中发现了表现为PAN型坏死性动脉炎特征的肝动脉炎。尸检患者中动脉炎的发生率在PAN中为100%，在其他胶原病中为8.3 - 25%。9例患者观察到原发性胆汁性肝硬化，其中7例（3例SSc患者，2例RA患者，1例PM和DM患者，1例MCTD患者）抗线粒体抗体（AMA）阳性PBC，2例SLE患者AMA阴性PBC。3例患者（2例SLE患者和1例MCTD患者）经临床病理诊断为自身免疫性肝炎。然而，3例临床、生化和血清学数据提示可能为AIH的患者（1例SLE患者，1例MCTD患者和1例PM和DM患者）因肝脏组织学（无AIH特征性表现）和临床病程而被排除在AIH相关组之外。这些结果表明，在胶原病中，没有肝脏组织学评估的数据不足以诊断AIH。7例患者观察到肝脏结节性再生性增生（5例SLE患者，1例SSc患者和1例PAN患者）。
本研究提供了对胶原病和肝脏异常患者的诊断和治疗有用的数据。
