原发性硬化性胆管炎17例临床分析

The analysis of characteristics inpatients with primary sclerosing cholangitis

目的探讨原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）的临床特征、诊断和治疗方法。方法回顾性研究经影像学或病理检查确诊的17例原发性硬化性胆管炎患者的临床特征、诊断、生化、免疫及药物治疗结果。结果本病多发于中、青年男性。主要的临床特点为乏力、黄疸、皮肤瘙痒和体重减轻，发生率分别为100％、95％、90％和81％，与同期进行观察的自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）的发生率比较，差异有显著性（P〈0．05、P〈0．01）。生化和免疫检查具有明显增高的GGT、ALP、TBIL、IgG和1．球蛋白。41．17％（7／17）的PSC患者伴发肝外自身免疫性疾病，35．29％（5／17）的患者伴发溃疡性结肠炎，患者均有较高的自身抗体发生率。本病诊断困难，从发病到确诊PBC平均为30个月，常常被误诊为多种其他的疾病。17例患者均见到胆管MRCP／ERCP异常，仅2例可见PSC典型的组织学变化。结论原发性硬化性胆管炎发病机制不明，临床表现复杂，误诊率高。肝功能异常以GGT、ALP和TBIL升高为主，内科药物治疗不能阻止疾病的发展。明确诊断需综合临床、生化、MRCP／ERCP和病理组织学等指标。

Objective To explore the features, diagnosis and treatment strategy of primary sclerosing cholangitis （PSC）. Methods The clinical manifestation,diagnostic laboratory test and the response to therapy of 17 cases of primary scterosing cholangitis were investigated retrospectively. Results The PSC affects mainly young men. The main symptom/sign were tiredness,jaundice, pruritus and losing weight,The incidence of those symptom/sign were 100% ,95% ,90% and 81% respectively,with significant difference from the patients of autoimmune hepatitis or primary biliary cirrhosis （ P 〈 0.05 or P 〈 0.01 ）. The levels of ALP,GGT,TBIL and γ-GI were significantly elevated in patients with PSC. The incidence of extrahepatic autoimmune diseases in PSC was 41.7% （7/17） and 35.29% （5/17） of the patients with ulcerative colitis. All of the patients had a high level of auto-antibody. With the diagnostic difficulty to PSC,it took the mean time of 30 months for making correct diagnosis. The disease was often misdiagnosed as many other diseases. All of the 17 cases appeared abnormity in MRCP or ERCP,Two cases of the patients showed typical morphology of PSC in histology. Conclusion PSC is characterized with no clear pathogenesis, complicated clinical findings and a high incidence of misdiagnosis. The liver function tests were markedly elevated in GGT,ALP and TBIL. The medical treatment cannot improve its prognosis satisfactorily. The diagnosis of PSC should be based on the clinical manifestation, biochemical test, ERCP/ MRCP screening and pathological changes.