原发性硬化性胆管炎13例临床分析

目的　探讨原发性硬化性胆管炎 (PSC)的临床表现。方法　回顾性分析 13例PSC患者的临床表现 ,并检测其血清自身抗体。结果　 13例PSC患者中 ,男性 8例 ,女性 5例。平均年龄5 5 .73岁。以重度黄疸、轻度乏力为主要表现 ,伴有瘙痒、上腹钝痛、白陶土样便。硷性磷酸酶(ALP) 2 74U/L ,谷氨酰氨基转肽酶 ( 2 98± 77.78)U/L。血清抗中性粒细胞胞浆抗体周围型 (pANCA)阳性 7例 ,抗核抗体 (ANA)阳性 2例 ,平滑肌抗体 (SMA)、抗线粒体抗体 (AMA)分别有 1例阳性 ,抗肝肾微粒体抗体 1(LKM 1)及抗可溶性肝抗原抗体 (SLA)均阴性。B超检查主要表现为胆管壁增厚、回声增强及胆管内径变细。内镜逆行胰胆管造影 (ERCP) 13例全部阳性 ,肝外型 9例 ,混合型 4例。纤维结肠镜检查提示溃疡性结肠炎 6例。结论　原发性硬化性胆管炎好发于中年男性 ,临床表现为梗阻性黄疸 ,硷性磷酸酶 (ALT)、γ 谷氨酰胺转移酶 (GGT)明显升高为其显著特点 ,pANCA阳性率较高 ,往往伴有溃疡性结肠炎。B超对本病诊断具有提示和辅助作用     

22例原发性硬化性胆管炎患者的临床、影像及病理学特征

目的 探讨原发性硬化性胆管炎(PSC)的临床、实验室、影像和病理学特点,以及熊去氧胆酸(UDCA)治疗对预后的影响. 方法 回顾性总结我院收治的22例PSC患者临床资料,对既往用药史、手术史、临床表现、实验室检查、影像学、肝组织学及UDCA治疗对预后影响等方面进行分析.数据比较用x2或校正x2检验.结果 22例患者中15例为男性,15例出现乏力、尿黄、体质量减轻,4例合并溃疡性结肠炎.22例患者的碱性磷酸酶(ALP)水平均明显升高,达(348±184)U/L;19例ALT水平升高至(94.0±67.0)U/L,AST水平升高至(98.0±67.0)U/L;15例总胆红素水平达(99.0±115.0)μmol/L,直接胆红素水平达(74.4±92.4)μmol/L.内镜逆行胰胆管造影检查可见肝内胆管节段性狭窄与扩张,胆管壁僵直,胆囊增大,胆总管及胰管显影不清;磁共振胆管造影可显示肝内胆管呈“串珠样”改变,胆管壁僵硬,胆总管扩张.组织病理学表现为胆管的纤维化和炎症及小胆管的增生,典型改变为呈“洋葱皮样”的纤维性闭塞性胆管炎.服用UDCA治疗组与未服用UDCA组比较,好转者分别为7例和5例,无效者均为5例,疗效差异无统计学意义(配对x2值及校正x2值分别为0.333和0.083,P值均＞0.05).结论 PSC患者男性居多,乏力、尿黄、体质量减轻为常见临床表现,血清生物化学指标升高明显,影像学检查有明显的特征性改变;UDCA治疗对PSC疗效不明显.     

Retrospective study of 404 cases of patients with ulcerative colitis in Zhongnan Hospital of Wuhan university

目的 调查近20年来我院溃疡性结肠炎(UC)住院患者的患病情况和临床病理变化,探讨溃疡性结肠炎的发病情况和临床特征.方法 收集近20年我院确诊为溃疡性结肠炎患者的临床资料,统计患病情况、临床特征、内镜及病理特点、治疗情况、病程与预后.结果 近20年我院溃疡性结肠炎患者明显增多,并呈逐渐上升趋势.404例患者中,男女比例1.23∶1,年龄6～84岁,平均年龄(42.7±14.8)岁,平均病程3.4年.溃疡性结肠炎主要临床表现为血便(80.2％)、腹泻(74.O％).肠外表现17例(4.2％),并发症19例(4.7％).病变以慢性复发型(62.1％)为主,轻、中度病变多见(85.9％),部位以左半结肠为主(68.3％).93.8％的溃疡性结肠炎患者经氨基水杨酸类药物和糖皮质激素治疗有效,手术患者14例(3.5％),死亡1例.结论 近20年来我院溃疡性结肠炎患者患病呈上升趋势,患者年龄偏大,病情较轻,病程较短,肠外表现和并发症少,手术病例少,死亡率低,内科治疗预后较好.     

原发性硬化性胆管炎合并炎症性肠病患者的临床特征分析

目的通过比较原发性硬化性胆管炎(PSC)合并炎症性肠病(IBD)患者和PSC未合并IBD患者临床特征的差异, 进一步探讨PSC合并IBD的疾病特点。方法横断面研究。纳入2000年1月至2021年1月北京协和医院收治的PSC患者42例, 其中合并IBD患者14例;对其人口学特征、临床表现、伴随疾病、辅助检查及治疗情况进行回顾性分析。结果 42例PSC患者的确诊年龄为11～74(43±18)岁, 其中14例合并IBD, 共患率33.3%, 确诊年龄为12～63(42±17)岁。PSC合并IBD患者与PSC未合并IBD患者相比较, 腹泻发生率更高, 黄疸、乏力发生率更低(均P<0.05)。PSC未合并IBD患者丙氨酸转氨酶、总胆红素、直接胆红素、总胆汁酸、糖类抗原19-9水平更高(均P<0.05), 而PSC合并IBD患者抗核抗体及粪便潜血的阳性率更高(均P<0.05)。PSC合并溃疡性结肠炎患者肠镜表现以广泛结肠受累为主。PSC合并IBD患者5-氨基水杨酸、糖皮质激素应用比例更高(P=0.025)。结论北京协和医院单中心PSC合并IBD的共患率低于西方国家。对于腹泻及粪便...     

转变模式:原发性硬化性胆管炎的真实发病率及临床病程

正原发性硬化性胆管炎(PSC)是一种复杂的胆汁淤积性肝病,主要由慢性胆管炎症反应及继发胆管破坏引起,最终导致胆管纤维化及肝硬化。已有报道北美、挪威及荷兰的PSC年发病率分别为每10万人0.9、1.3及0.5。PSC与炎症性肠病(IBD)存在相关性,据估计,约有50%~80%的PSC患者合并有IBD;反之亦然,在确诊溃疡性结肠炎(UC)患者中,PSC的发病率为0.8%~5.6%,而克罗恩病(CD)患者中,PSC的发病率为0.4%~6.4%。发病率的巨大范围提示对于这个问题发     

核周型抗中性粒细胞胞浆抗体在溃疡性结肠炎诊断中的意义

目的了解血清中核周型抗中性粒细胞胞浆抗体(pANCA)对溃疡性结肠炎诊断的敏感性及特异性。方法本文采用间接免疫荧光法对我院51例溃疡性结肠炎、15例克罗恩病、30例对照患者血清中核周型抗中性粒细胞胞浆抗体进行检测。结果 51例溃疡性结肠炎患者中32例核周型抗中性粒细胞胞浆抗体阳性,其敏感性及特异性分别为62.75%及95.56%。15例克罗恩病患者中有2例阳性核周型抗中性粒细胞胞浆抗体阳性,阳性率为13.33%,30例对照组未发现核周型抗中性粒细胞胞浆抗体阳性患者,组间差异具有统计学意义(P<0.05)。结论血清中抗中性粒细胞胞浆抗体对溃疡性结肠炎具有一定的敏感性及高度的特异性,对其检测有利于溃疡性结肠炎的诊断及鉴别诊断。     

内镜下导管注药治疗重度溃疡性结肠炎

目的观察经内镜下注药对重度溃疡性结肠炎的治疗效果.方法随机对106例重度溃疡性结肠炎患者,应用内镜Bc-9L导管将中、西药物混合液直接注入病变部位进行治疗.选用药物锡类散、肿痛安、维生素、替硝唑、免疫抑制剂等.局部给药后,采取特殊体位,保留药液3h以上,1次/d,每4wk～6wk为一疗程.本组患者男72例,女34例,年龄20岁～73岁,其中30岁～59岁85例(80.2%),病程6mo～15a以上,临床表现多以腹痛、腹泻、粘液便或粘液血便为主.病变部位在直乙状结肠64例(60.37%),右半结肠24例(22.64%),全结肠18例(16.98%).结果经4wk～6wk治疗症状体征消失,镜检溃疡愈合86例(81.14%),症状体征基本消失,镜检溃疡范围缩小超过60%者10例(9.43%),症状体征有改善,镜检溃疡范围缩小未达50%者10例,视为无效,本组治疗有效率达90.6%.结论经内镜直接局部注药对重复溃疡性结肠炎有明显治疗作用.     

难治性远端溃疡性结肠炎的临床表现和治疗

目的:分析难治性远端溃疡性结肠炎的可能的发病原因及治疗方案.方法:收集我院2005-01/2011-12溃疡性结肠炎中145例DUC患者资料进行回顾性分析.根据对传统治疗的反应,分为有效组和难治组.比较两组患者的临床和实验室检查结果,分析难治DUC可能的原因及进一步治疗方案.结果:145例远端溃疡性结肠炎患者,其中117例符合条件纳入组,117例中有26例[22.2%(26/117)]患者难治.与有效组相比,难治组患者腹痛腹胀明显[42.3%(11/26)vs22.0%(20/91),P=0.038]、白细胞数明显增高[30.8%(8/26)vs12.1%(11/91),P=0.035],而血便、腹泻、肠外表现、C反应蛋白及血沉两组之间无明显差异(P>0.05).117例入组患者内镜下表现为直肠炎43例其中10例难治,直乙状结肠炎74例中16例难治,病变部位差异无统计学意义(P>0.05).26例难治患者仅1例行外科手术治疗,其余的通过激素静脉治疗、加用5-ASA新型剂型、适当的泻药等获得缓解.结论:远端溃疡性结肠炎患者腹泻和血便是临床最常见症状,难治组白细胞数较有效组明显增高,其有望成为评估DUC治疗转归的指标之一.难治性DUC患者可通过强化治疗、加用5-ASA的新型剂型、适当的泻药、手术等方法获得缓解.     

原发性硬化性胆管炎有/无合并炎症性肠疾患有何区别?

原发性硬化性胆管炎(PSC)往往与炎症性肠疾患(IBD),溃疡性结肠炎(UC)、结肠 Crohn 病等合并存在,有关 PSC 有/无合并 IBD 时的症状,体征,胆管造影表现,对原位肝移植的反应及胆管癌等的资料很少,且有争论。本文对66例 PSC 进行研究,合并 IBD47例(71.2%),UC39例(59.1%),中位数年龄为42.5±11.6岁,男:女为2.9:1。合并 Crohn 结肠炎8例(12.1%),中位数年龄40.5(?)6.5岁,男:女为1:1。     

原发性硬化性胆管炎的内镜治疗及预后分析

目的探讨原发性硬化性胆管炎(primary sclerosing cholangitis, PSC)患者行经内镜逆行胰胆管造影术(endoscopic retrograde cholangiopancreatography, ERCP)的诊疗效果及预后相关因素。方法纳入2009年5月—2020年5月西京医院接诊的PSC病例。回顾性收集人口学、临床症状、实验室和影像学检查、ERCP诊疗等数据, 探讨ERCP治疗人群特点及临床疗效, 随访观察疾病进展、无移植生存期和总体生存期等。结果共纳入74例PSC患者, 中位年龄53岁, 男性占54.1%(40/74), 合并胆管显性狭窄、炎症性肠病和其他自身免疫性肝病患者分别占32.4%(24/74)、18.9%(14/74)和17.6%(13/74), 接受ERCP患者占36.5%(27/74)。Logistic回归分析显示, 高总胆红素(OR=12.33, 95%CI:1.24～122.63, P=0.032)和合并胆管显性狭窄(OR=24.67, 95%CI:3.40～178.88, P=0.002)是ERCP诊疗的独立危险因素。ERCP操作和...     

Comparison of clinical findings between autoimmune pancreatitis with bile duct involvement and primary sclerosing cholangitis]

BACKGROUND/AIMS: The involvement of bile ducts is frequently reported in autoimmune pancreatitis (AIP), which seem to have similar features to primary sclerosing cholangitis (PSC). Recent systematic comparative studies about these diseases are rare in Korea. METHODS: We retrospectively analyzed 26 patients with AIP with bile duct involvement and 30 patients with classic PSC who were diagnosed during the last decade. RESULTS: The mean age of patients was significantly higher in AIP than PSC at the time of diagnosis. There was a preponderance of men in both group, which was more prominent in AIP. The most common symptom in patients with AIP was jaundice, but PSC patients usually visited hospitals due to incidentally detected abnormal liver function tests. Most (26/31) of AIP had bile duct involvement. All of these patients showed narrowing of intrapancreatic common bile ducts and one patient exhibited hilar involvement as well. About 80% of PSC had both intra- and extrahepatic ducts involvement, and the characteristic features involve multifocal strictures. AIP patients showed improvement with steroid treatment, however, most PSC patients showed clinical deterioration. CONCLUSIONS: The clinical and cholangiographic findings of patients with AIP and PSC have many different characteristics. Therefore, further study of two diseases is required for the proper diagnosis and management.     

IgG4-related sclerosing cholangitis and autoimmune pancreatitis: histological assessment of biopsies from Vater's ampulla and the bile duct

Background and Aim: Autoimmune pancreatitis is commonly associated with immunoglobulin (Ig) G4‐related sclerosing cholangitis (IgG4‐SC). The discrimination between IgG4‐SC and pancreatobiliary malignancies or primary sclerosing cholangitis (PSC) is now an important issue. The present study was carried out to examine the usefulness of endoscopic biopsies from Vater's ampulla and the bile duct to diagnose IgG4‐SC. Methods: The present study included 29 IgG4‐SC patients (26 with both pancreatitis and cholangitis, and 3 with cholangitis only), 6 PSC patients, and 27 pancreatobiliary carcinoma patients. All patients underwent endoscopic biopsies from Vater's ampulla and the common bile duct. Biopsied specimens were histologically examined using immunostaining for IgG4. Results: For the ampullary and bile duct biopsies, the IgG4‐SC samples had a significantly greater number of IgG4‐positive plasma cells than the PSC or pancreatobiliary carcinoma specimens. In addition, bile duct biopsies from five patients (17%) with IgG4‐SC showed diffuse inflammatory cell infiltration with irregular fibrosis corresponding to the histological features of lymphoplasmacytic sclerosing pancreatocholangitis. Based on the threshold of 10 IgG4‐positive plasma cells per high power field, the diagnostic rates of the ampullar and bile duct biopsies were both 52% (15/29 cases). Twenty‐one patients (72%) had more than 10 IgG4‐positive plasma cells in at least one biopsy. The bile duct biopsy was significantly valuable for IgG4‐SC patients with swelling of the pancreatic head. Conclusion: The present study suggested that ampullar and bile duct biopsies are useful for diagnosing IgG4‐SC.     

The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis

OBJECTIVES: The epidemiology of primary sclerosing cholangitis (PSC) has been incompletely assessed by population-based studies. We therefore conducted a population-based study to determine: (a) incidence rates of large and small duct PSC in adults and children, (b) the risk of inflammatory bowel disease on developing PSC, and (c) patterns of clinical presentation with the advent of magnetic resonance cholangiopancreatography (MRCP). METHODS: All residents of the Calgary Health Region diagnosed with PSC between 2000 and 2005 were identified by medical records, endoscopic, diagnostic imaging, and pathology databases. Demographic and clinical information were obtained. Incidence rates were determined and risks associated with PSC were reported as rate ratios (RR) with 95% confidence intervals (CI). RESULTS: Forty-nine PSC patients were identified for an age- and gender-adjusted annual incidence rate of 0.92 cases per 100,000 person-years. The incidence of small duct PSC was 0.15/100,000. In children the incidence rate was 0.23/100,000 compared with 1.11/100,000 in adults. PSC risk was similar in Crohn's disease (CD; RR 220.0, 95% CI 132.4-343.7) and ulcerative colitis (UC; RR 212.4, 95% CI 116.1-356.5). Autoimmune hepatitis overlap was noted in 10% of cases. MRCP diagnosed large duct PSC in one-third of cases. Delay in diagnosis was common (median 8.4 months). A minority had complications at diagnosis: cholangitis (6.1%), pancreatitis (4.1%), and cirrhosis (4.1%). CONCLUSIONS: Pediatric cases and small duct PSC are less common than adult large duct PSC. Surprisingly, the risk of developing PSC in UC and CD was similar. Autoimmune hepatitis overlap was noted in a significant minority of cases.     

Transpapillary intraductal ultrasound in the evaluation of dominant bile duct stenoses in patients with primary sclerosing cholangitis

OBJECTIVE: Primary sclerosing cholangitis (PSC) is associated with the development of cholangiocarcinoma (CC) in approximately 9% of patients. Neither cholangiography nor endoscopic tissue sampling can reliably distinguish between CC and benign dominant bile duct stenosis. The aim of the present study was to assess the value of intraductal ultrasonography (IDUS) in distinguishing between benign and malignant dominant stenoses in PSC patients. MATERIAL AND METHODS: Forty PSC patients with dominant bile duct stenoses were studied prospectively. Transpapillary IDUS and endoscopic tissue sampling were performed in addition to endoscopic retrograde cholangiography (ERC). Cholangiography and IDUS findings were classified as malignant or benign by the investigators. Final diagnosis of malignant stenosis was based on positive histology and/or cytology, whereas a benign character was assumed in cases of negative tissue sampling and uneventful extended clinical follow-up. RESULTS: Eight PSC patients (20%) had dominant bile duct stenoses caused by CC, whereas 32 out of 40 patients (80%) had benign dominant bile duct stenoses. IDUS was significantly superior to ERC for detection of malignancy in terms of sensitivity (87.5% versus 62.5%, p=0.05), specificity (90.6% versus 53.1%, p<0.001), accuracy (90% versus 55%, p<0.001), positive predictive value (70% versus 25%, p<0.001), and negative predictive value (96.7% versus 85%, p=0.049). CONCLUSIONS: Transpapillary IDUS significantly increases the ability to distinguish malignant from benign dominant bile duct stenoses in patients with PSC.     

Forty-six patients with primary sclerosing cholangitis: radiological bile duct changes in relationship to clinical course and concomitant inflammatory bowel disease

The clinical features of primary sclerosing cholangitis were studied in 46 consecutive patients. Jaundice was the most common symptom (57%), followed by pruritus (28%), pain (24%), and fever (15%). Thirty-three per cent of the patients had no symptoms, merely laboratory changes. No significant relationship was observed between a numerical score of radiological bile duct changes at diagnosis and the clinical picture, or the clinical course during follow-up. If clinical deterioration occurred, this seemed to happen within the first eight years after the clinical presentation. Patients with only intra-hepatic bile duct changes (n = 10) did not differ clinically from those with extrahepatic changes as well. Forty-three out of 44 patients examined had inflammatory bowel disease, usually ulcerative colitis, with total colitis in 84%. Radiological bile duct changes had a significantly higher score in patients who had to be treated with a combination of sulfasalazine and steroids, suggesting a weak relationship between severity of bowel disease and bile duct disease.     

27例原发性胆汁性肝硬化的组织病理学特征

目的 总结原发性胆汁性肝硬化（PBC）患者的临床及病理组织学特点。方法 对27例原发性胆汁性肝硬化患者的临床资料进行分析，重点讨论其肝脏组织病理学特点。结果 本组男、女之比为1：8（3：24），年龄22－69岁。其主要临床症状为乏力（62．9％，17／27），其次为黄疸（59．2％，16／27）和皮肤瘙痒（29．6％，8／27）。患者的血清碱性磷酸酶（ALP）及γ－谷氨酰转肽酶（GGT）均明显升高，95．8％的患者（23／27）抗线粒体抗体或线粒体抗体M2亚型阳性。肝组织病理学特点为：小叶间胆管变性坏死、基底膜不完整，周围有淋巴细胞和浆细胞浸润（66％，18／27）；汇管区淋巴细胞聚集（100％，27／27）或淋巴滤泡形成（15％，4／27）；肉芽肿形成（26％，7／27）及小叶间胆管减少；细小胆管增生（55％，15／27），肝细胞羽毛状变性（59％，16／27）；肝细胞内胆色素沉积和（或）毛细胆管胆栓形成（52％，14／27）；纤维组织增生，小叶结构紊乱（26％，7／27），假小叶形成（11％，3／27）。结论 乏力、皮肤瘙痒及血清ALP、GGT升高及抗线粒体抗体阳性是PBC的主要临床特征；而小叶间胆管炎、胆管数目减少，汇管区淋巴细胞聚集、肉芽肿形成、细小胆管增生，以及肝细胞羽毛状变性是PBC的主要病理特点。     

Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

BACKGROUND: Patients with cholestatic liver function tests and histological features of primary sclerosing cholangitis (PSC) but a normal cholangiogram are considered to have small duct PSC. The natural history of this condition is unknown. METHODS: Thirty three patients with small duct PSC were identified among patients admitted for diagnostic workup of cholestatic liver function tests in one centre in the UK (Oxford) and one centre in Norway (Oslo). A total of 260 patients with large duct PSC were compared, and prognosis in terms of death, cholangiocarcinoma, biochemical features, histological features, and symptoms analysed. RESULTS: Mean age at diagnosis was 38 years and 39 years in small duct and large duct PSC, respectively. Mean follow up was 106 months in small duct and 105 months in large duct patients. Four patients originally considered to have small duct developed large duct PSC. Two of these underwent liver transplantation during follow up. Of the remainder who did not develop large duct PSC, two patients died during follow up: one of liver failure and the other of cardiac death unrelated to her liver disease. A total of 122 (47%) large duct patients either required liver transplantation (34 patients) or died (88 patients). Small duct patients had a significantly better survival compared with large duct patients. Among small duct patients, none developed cholangiocarcinoma compared with 28 of 260 (11%) large duct patients. CONCLUSIONS: Patients with small duct PSC seem to have a good prognosis in terms of survival and development of cholangiocarcinoma. Small duct PSC progresses to large duct PSC in a small proportion of patients.     

原发性硬化性胆管炎的诊断和治疗

原发性硬化性胆管炎（PSC）是一种以胆管的进行性炎症、纤维化和多发性狭窄为主要病理特征的慢性胆汁淤积性肝病,其发病可能与遗传及免疫机制有关,其病变范围可累及肝内和肝外胆管。部分患者具备典型的胆汁淤积表现和PSC的组织学特征,但胆管造影正常,称为小胆管PSC。PSC的病程多呈慢性进行性,最终演变为终末期肝病。60%～80%的PSC患者可并发炎症性肠病,约20%的患者还可并发胆管癌。目前针对PSC的治疗,除肝移植外尚无确切有效的治疗方法     

Variant forms of cholestatic diseases involving small bile ducts in adults

OBJECTIVE: Cholestasis may result from diverse etiologies. We review chronic cholestatic disorders involving small intrahepatic bile ducts in the adult ambulatory care setting. Specifically, we discuss variant forms of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) as well as other conditions that may present diagnostic and therapeutic difficulties. METHODS: We conducted a MEDLINE search of the literature (1981-1997) and reviewed the experiences at the Mayo Clinic. All articles were selected that discussed antimitochondrial antibody (AMA)-negative PBC, small-duct PSC (formerly pericholangitis), and idiopathic adulthood ductopenia. RESULTS: The most common chronic cholestatic liver diseases affecting adults are PBC and PSC. Patients without the hallmarks of either syndrome are diagnosed according to their clinical and histological characteristics. Autoimmune cholangitis is diagnosed if clinical and histological features are compatible with PBC but autoantibodies other than AMA are present. Isolated small duct PSC is diagnosed if patients have inflammatory bowel disease, biopsy features compatible with PSC, but a normal cholangiogram. If ductopenia (absence of interlobular bile ducts in small portal tracts) is found histologically in the absence of PSC, inflammatory bowel disease, and other specific cholestatic syndromes such as drug reaction or sarcoidosis, the most likely diagnosis is idiopathic adulthood ductopenia. CONCLUSIONS: Based on these definitions, an algorithm for diagnosis and therapy in patients with laboratory evidence of chronic cholestasis may be constructed, pending results of further investigations into the etiopathogenesis of these syndromes.