狼疮性肝炎患者的临床和免疫病理学特征。

Clinical and immunopathological features of patients with lupus hepatitis.

机构信息

Department of Gastroenterology and Hepatology, First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong 510080, China.

出版信息

Chin Med J (Engl). 2013 Jan;126(2):260-6.

PMID:23324274

Abstract

BACKGROUND

Lupus hepatitis is yet to be characterized based on its clinical features and is often difficult to differentially diagnose from other liver diseases. We aimed to elucidate clinical, histopathological and immunopathological features of lupus hepatitis and to evaluate primarily the effectiveness of liver immunopathological manifestations on differential diagnosis of lupus hepatitis from other liver diseases.

METHODS

A retrospective study was performed to analyze clinical features of lupus hepatitis in 47 patients out of 504 inpatients with systemic lupus erythematosus (SLE) in First Affiliated Hospital of Sun Yat-sen University, China from May 2006 to July 2009, and to evaluate the association between lupus hepatitis and SLE activity. Additionally, liver histopathological changes by hematoxylin and eosin (HE) staining and immunopathological changes by direct immunofluorescence test in 10 lupus hepatitis cases were analyzed and compared to those in 16 patients with other liver diseases in a prospective study.

RESULTS

Of 504 SLE patients, 47 patients (9.3%) were diagnosed to have lupus hepatitis. The prevalence of lupus hepatitis in patients with active SLE was higher than that in those with inactive SLE (11.8% vs. 3.2%, P < 0.05). The incidence of hematological abnormalities in patients with lupus hepatitis was higher than that in those without lupus hepatitis (40.4% vs. 21.7%, P < 0.05), such as leucocytes count (2.92×10(9)/L vs. 5.48×10(9)/L), platelets count (151×10(9)/L vs. 190×10(9)/L), serum C3 and C4 (0.34 g/L vs. 0.53 g/L; 0.06 g/L vs. 0.09 g/L) (P < 0.05); 45 of 47 (95.7%) lupus hepatitis patients showed 1 upper limit of normal (ULN) < serum ALT level < 5 ULN. The liver histopathological features in patients with lupus hepatitis were miscellaneous and non-specific, similar to those in other liver diseases, but liver immunopathological features showed positive intense deposits of complement 1q in 7/10 patients with lupus hepatitis and negative complement 1q deposits in all patients with other liver diseases (Fisher's exact test, P = 0.011).

CONCLUSIONS

Lupus hepatitis was not infrequent in active SLE patients which would be one of the indices indicating SLE activity. Positive intense deposit of complement 1q in liver may be a characteristic immunopathological feature of lupus hepatitis, which provides a new way to differentially diagnose lupus hepatitis from other liver diseases.

摘要

背景

狼疮性肝炎的临床特征尚未明确，且常难以与其他肝病相鉴别。本研究旨在阐明狼疮性肝炎的临床、组织病理学和免疫病理学特征，并评估肝脏免疫病理学表现对狼疮性肝炎与其他肝病鉴别的有效性。

方法

对中山大学附属第一医院 2006 年 5 月至 2009 年 7 月收治的 504 例系统性红斑狼疮（SLE）住院患者中 47 例狼疮性肝炎患者的临床特征进行回顾性分析，并评估狼疮性肝炎与 SLE 活动的关系。此外，对 10 例狼疮性肝炎患者的肝组织苏木精-伊红（HE）染色和直接免疫荧光检查的组织病理学变化进行分析，并与 16 例其他肝病患者的变化进行前瞻性比较。

结果

在 504 例 SLE 患者中，47 例（9.3%）诊断为狼疮性肝炎。活动期 SLE 患者狼疮性肝炎的发生率高于非活动期 SLE 患者（11.8% vs. 3.2%，P < 0.05）。狼疮性肝炎患者血液学异常的发生率高于无狼疮性肝炎患者（40.4% vs. 21.7%，P < 0.05），如白细胞计数（2.92×10(9)/L vs. 5.48×10(9)/L）、血小板计数（151×10(9)/L vs. 190×10(9)/L）、血清 C3 和 C4（0.34 g/L vs. 0.53 g/L；0.06 g/L vs. 0.09 g/L）（P < 0.05）；47 例狼疮性肝炎患者中，45 例（95.7%）患者血清丙氨酸氨基转移酶（ALT）水平为 1 个正常值上限（ULN）<血清 ALT 水平<5 ULN。狼疮性肝炎患者的肝组织病理学特征为混杂且非特异性的，与其他肝病相似，但肝脏免疫病理学特征显示 10 例狼疮性肝炎患者中有 7 例存在补体 1q 的强阳性沉积，而所有其他肝病患者均无补体 1q 沉积（Fisher 确切概率检验，P = 0.011）。