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Received:November 11, 2019 Published Online:July 20, 2020
Received:November 11, 2019 Published Online:July 20, 2020
中文摘要: 目的 分析原发性硬化性胆管炎(PSC)患者的临床特点,提高对该病的诊治水平。
方法 回顾性分析2009年5月至2019年5月住院治疗的PSC患者25例的临床资料,总结其临床表现、实验室和影像学检查特点、并发疾病和治疗方法。
结果 25例患者均诊断为大胆管PSC(其中1例合并小胆管损害),男16例,女9例;年龄19~79岁(中位年龄57岁)。确诊为PSC时临床表现:黄疸10例、腹胀8例、腹痛7例、乏力7例等。25例患者中8例诊断为肝硬化(确诊7例,疑诊1例),4例诊断为溃疡性结肠炎(与PSC同时2例,在PSC之前和之后各1例),合并自身免疫性肝炎、强直性脊柱炎和银屑病性关节炎各1例,无患者并发胆管癌。实验室化验:肝功能化验中γ-谷氨酰转肽酶(γ-GT)、碱性磷酸酶(ALP)和总胆红素(T-bil)升高最明显,γ-GT升高22例(中位值215 U/L),ALP升高19例(中位值284 U/L),T-bil升高14例(中位值27.4 μmol/L);22例完成免疫球蛋白(Ig)定量化验,其中IgG升高10例(中位值14.5 g/L),IgM升高6例(中位值为1.205 g/L);10例完成抗中性粒细胞胞浆抗体(ANCA)化验,3例阳性(核周型ANCA、抗蛋白酶3抗体和两者均阳性各1例);15例完成抗核抗体(ANA)滴度化验,9例升高(中位值1[DK]∶100),17例完成IgG4化验,5例升高(中位值0.862 g/L)。25例均完成磁共振胰胆管成像(MRCP)检查,其中13例表现肝外+肝内胆管受累,7例表现为单独肝外胆管受累,5例表现为单独肝内胆管受累;3例进行肝穿刺活检,1例符合小胆管PSC特点,1例符合自身免疫性肝炎特点;1例进行胃镜超声检查,其表现支持大胆管PSC诊断。25例患者中20例应用熊去氧胆酸治疗,9例应用糖皮质激素治疗(其中7例同时用熊去氧胆酸),1例应用胆管引流等方法缓解胆道梗阻;另2例对症治疗。
结论 PSC好发于中年,男性略多于女性,肝功能、免疫学检验可提示诊断,确诊主要依靠MRCP检查,目前的主要治疗药物为口服熊去氧胆酸,但其疗效仍需进一步验证。
Abstract:Objective To analyze the clinical features of patients with primary sclerosing cholangitis (PSC) and refine the diagnostic accuracy and treatment.
Methods The clinical data of 25 PSC patients hospitalized from May 2009 to May 2019 were analyzed retrospectively,and the clinical manifestations,laboratory and imaging features,concurrent diseases and therapeutic options were summarized.
Results Out of 25 patients diagnosed with large-duct PSC (including 1 case complicated with small bile duct damage),there were 16 males and 9 females,aged 19-79 years with a median age of 57 years.The main clinical presentations were jaundice in 10 cases,abdominal distention in 8 cases,abdominal pain in 7 cases and fatigue in 7 cases.Liver cirrhosis was found in 8 cases,including 7 confirmed and 1 suspected;ulcerative colitis occurred in 4 cases,including 2 cases concurrently,1 case before PSC and 1 case after PSC diagnosis;autoimmune hepatitis,ankylosing spondylitis and psoriatic arthritis occurred in one case,respectively.There were no patients complicated with cholangiocarcinoma.
In liver function test, γ-GT, ALP and T-Bil increased most obviously.
Elevations of γ-GT were found in 22 cases (median value:215 U/L),ALP in 19 cases (median value:284 U/L),T-bil in 14 cases (median value:27.4 umol/L).Out of 22 cases who completed quantitative analysis of immunoglobulin Ig,IgG increased in 10 cases (median value:14.5 g/L),IgM increased in 6 cases (median value:1.205 g/L).In 10 cases who received ANCA tests,3 cases were positive(1 case of perinuclear ANCA,1 case of antiproteinase 3 antibody and 1 case of positive for both).For ANA tests in 15 cases,elevation was found in 9 cases (median value 1∶100).Out of 17 cases who received IgG4 tests,elevation was found in 5 cases (median value:0.862 g/L).All the patients underwent MRCP examination,and 13 cases showed involvement of both extrahepatic and intrahepatic bile duct,7 cases showed extrahepatic bile duct involvement alone,5 cases showed intrahepatic bile duct involvement alone.After biopsy was conducted in 3 patients,1 case presented with findings consistent with small-duct PSC,and 1 case was in line with the characteristics of autoimmune hepatitis.Endoscopic ultrasound was performed in one patient and supported the diagnosis of large-duct PSC.Out of 25 cases,20 cases were treated with ursodeoxycholic acid(UDCA)and 9 cases with glucocorticoid (7 of which were also with UDCA), and 1 case perfomed with bile duct drainage, so that to relieve biliary obstruction, and other 2 cases were treated with symptomatic treatment.
Conclusions PSC usually occurs in the middle-aged adults with a slight male predominance.Liver fumtion and immunological tests could be suggestive of the disease.MRCP is the first-line diagnostic modality for PSC.UDCA is widely used in the treatment of PSC,but its efficacy is still to be validated.
keywords: Primary sclerosing cholangitis Autoimmune liver disease Ursodeoxycholate acid Clinical features
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