Hunter Michael, Loughrey Maurice B, Gray Moyra, Ellis Peter, McDougall Neil, Callender Michael
Department of Infectious Diseases, 3rd Floor, East Wing, Royal Victoria Hospital, Grosvenor Road, BT12 6BA Belfast.
Ulster Med J. 2011 Jan;80(1):15-8.
Overlap syndromes constitute a significant proportion of autoimmune liver disease. Our aim was to describe our cohort and evaluate practical methods of correctly diagnosing autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome as early as possible clinically.
118 autoimmune hepatitis patients were screened for cholestatic liver function tests. 24 patients with cholestatic liver function tests were investigated for possible primary sclerosing cholangitis by clinicopathological review and magnetic resonance cholangiography. Retrospectively, potential predictors of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome were compared with a control group.
Overlap syndrome was diagnosed in twelve (50%) of 24 autoimmune hepatitis patients with recent cholestasis. The cholestatic group had a lower AST (p=0.012) and International Autoimmune Hepatitis Group (IAHG) score (p=0.102), and higher IgM (p=0.002) at disease presentation. More patients in the cholestatic group developed ulcerative colitis (p=0.138).
Identifying AIH / PSC overlap syndrome at diagnosis is often difficult. Certain clinical and biochemical features should alert the clinician. All patients with AIH, and biochemical cholestasis should be investigated with MRC.
重叠综合征在自身免疫性肝病中占相当大的比例。我们的目的是描述我们的队列，并评估在临床上尽早正确诊断自身免疫性肝炎/原发性硬化性胆管炎重叠综合征的实用方法。
对118例自身免疫性肝炎患者进行胆汁淤积肝功能检查筛查。对24例胆汁淤积肝功能检查患者通过临床病理检查和磁共振胆管造影术调查是否可能患有原发性硬化性胆管炎。回顾性地将自身免疫性肝炎/原发性硬化性胆管炎重叠综合征的潜在预测因素与对照组进行比较。
24例近期胆汁淤积的自身免疫性肝炎患者中有12例（50%）被诊断为重叠综合征。胆汁淤积组在疾病表现时AST较低（p=0.012），国际自身免疫性肝炎小组（IAHG）评分较低（p=0.102），IgM较高（p=0.002）。胆汁淤积组更多患者发生溃疡性结肠炎（p=0.138）。
在诊断时识别自身免疫性肝炎/原发性硬化性胆管炎重叠综合征往往很困难。某些临床和生化特征应提醒临床医生。所有自身免疫性肝炎和生化胆汁淤积患者均应进行磁共振胆管造影检查。
