Objective: To explore disease characteristics of primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) and compare the differences between PSC with and without IBD. Methods: Study design was cross sectional. Forty-two patients with PSC who were admitted from January 2000 to January 2021 were included. We analyzed their demographic characteristics, clinical manifestations, concomitant diseases, auxiliary examination, and treatment. Results: The 42 patients were 11-74(43±18) years of age at diagnosis. The concordance rate of PSC with IBD was 33.3%, and the age at PSC with IBD diagnosis was 12-63(42±17) years. PSC patients with IBD had higher incidences of diarrhea and lower incidences of jaundice and fatigue than in those without IBD (all P<0.05). Alanine aminotransferase, total bilirubin, direct bilirubin, total bile acid and carbohydrate antigen 19-9 levels were higher in PSC patients without IBD than in those with IBD (all P<0.05). The positive rates for antinuclear antibodies and fecal occult blood were higher in PSC patients with IBD than in those without IBD (all P<0.05). Patients with PSC complicated with ulcerative colitis mainly experienced extensive colonic involvement. The proportion of 5-aminosalicylic acid and glucocorticoid application in PSC patients with IBD was significantly increased compared with that of PSC patients without IBD (P=0.025). Conclusions: The concordance rate of PSC with IBD is lower at Peking Union Medical College Hospital than in Western countries. Colonoscopy screening may benefit PSC patients with diarrhea or fecal occult blood-positive for early detection and diagnosis of IBD.
Humans ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Cholangitis, Sclerosing/therapy* ; Cross-Sectional Studies ; Inflammatory Bowel Diseases/diagnosis* ; Colitis, Ulcerative/complications* ; Diarrhea

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Cholangitis/therapy* ; Humans ; Liver Cirrhosis, Biliary/therapy*

In 2015, the first Chinese consensus on the diagnosis and management of primary sclerosing cholangitis was issued. In the past years, more data have emerged from the literature. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 21 recommendations on PSC clinical practice. To facilitate the differentiation between PSC and IgG4-related sclerosing cholangitis, 10 recommendations on IgG4-SC are also attached. These guidelines aim to provide a working reference for the management of PSC and IgG4-SC.
Autoimmune Diseases/diagnosis* ; Cholangitis, Sclerosing/therapy* ; Diagnosis, Differential ; Humans ; Immunoglobulin G

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued a consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations.
Cholangitis/therapy* ; Consensus ; Gastroenterology ; Humans ; Liver Cirrhosis, Biliary/therapy*

The hepatobiliary system is one of the most common sites of extraintestinal manifestation in patients with inflammatory bowel disease (IBD). The progression of IBD can lead to a primary hepatobiliary manifestation and can occur secondary to multiple drugs or accompanying viral infections. Primary sclerosing cholangitis is the representative hepatobiliary manifestation of IBD, particularly in ulcerative colitis. Although most agents used in the treatment of IBD are potentially hepatotoxic, the risk of serious hepatitis or liver failure is low. The prevalence of HBV and HCV in IBD is similar to the general population, but the clinical concern is HBV reactivation associated with immunosuppressive therapy. Patients undergoing cytotoxic chemotherapy or immunosuppressive therapy with a moderate to high risk of HBV reactivation require prophylactic antiviral therapy. On the other hand, HCV has little risk of reactivation. Patients with IBD are more likely to have nonalcoholic fatty liver disease than the general population and tend to occur at younger ages. IBD and cholelithiasis are closely related, especially in Crohn's disease.
Cholangitis, Sclerosing ; Cholelithiasis ; Colitis, Ulcerative ; Crohn Disease ; Drug Therapy ; Drug-Induced Liver Injury ; Hand ; Hepatitis ; Hepatitis Viruses ; Humans ; Inflammatory Bowel Diseases ; Liver Failure ; Non-alcoholic Fatty Liver Disease ; Prevalence

Pancreatic cancer is a major cause of cancer-related mortality and morbidity, and its incidence is increasing as the population is aging. On the other hand, significant improvement in the prognosis has not occurred. The absence of early diagnosis means that many patients are diagnosed only when they develop symptoms, such as jaundice, due to a biliary obstruction. The role of endoscopy in multidisciplinary care for patients with pancreatic cancer continues to evolve. Controversy remains regarding the best preoperative biliary drainage in patients with surgically resectable pancreatic head cancer. In general, patients undergoing a surgical resection usually do not require preoperative biliary drainage unless they have cholangitis or receive neoadjuvant chemotherapy. If biliary drainage is performed prior to surgery, the patient's condition and a multidisciplinary approach should be considered. With the increasing life expectancy of patients with pancreatic cancer, the need for more long-time biliary drainage or pre-operative biliary drainage is also increasing. Strong evidence of endoscopic retrograde cholangiopancreatography (ERCP) as a first-line and essential treatment for biliary decompression has been provided. On the other hand, the use of endoscopic ultrasound-guided biliary drainage as well as percutaneous biliary drainage has been also recommended. During ERCP, self-expandable metal stent could be recommended instead of a plastic stent for the purpose of long stent patency and minimizing stent-induced complications. In this review, several points of view regarding the relief of obstruction in patients with pancreatic cancer, and optimal techniques are being discussed.
Aging ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Decompression ; Drainage ; Drug Therapy ; Early Diagnosis ; Endoscopy ; Hand ; Head and Neck Neoplasms ; Humans ; Incidence ; Jaundice ; Life Expectancy ; Mortality ; Pancreatic Neoplasms ; Plastics ; Prognosis ; Stents

The hepatobiliary system is one of the most common sites of extraintestinal manifestation in patients with inflammatory bowel disease (IBD). The progression of IBD can lead to a primary hepatobiliary manifestation and can occur secondary to multiple drugs or accompanying viral infections. Primary sclerosing cholangitis is the representative hepatobiliary manifestation of IBD, particularly in ulcerative colitis. Although most agents used in the treatment of IBD are potentially hepatotoxic, the risk of serious hepatitis or liver failure is low. The prevalence of HBV and HCV in IBD is similar to the general population, but the clinical concern is HBV reactivation associated with immunosuppressive therapy. Patients undergoing cytotoxic chemotherapy or immunosuppressive therapy with a moderate to high risk of HBV reactivation require prophylactic antiviral therapy. On the other hand, HCV has little risk of reactivation. Patients with IBD are more likely to have nonalcoholic fatty liver disease than the general population and tend to occur at younger ages. IBD and cholelithiasis are closely related, especially in Crohn's disease.
Cholangitis, Sclerosing ; Cholelithiasis ; Colitis, Ulcerative ; Crohn Disease ; Drug Therapy ; Drug-Induced Liver Injury ; Hand ; Hepatitis ; Hepatitis Viruses ; Humans ; Inflammatory Bowel Diseases ; Liver Failure ; Non-alcoholic Fatty Liver Disease ; Prevalence

Pancreatic cancer is a major cause of cancer-related mortality and morbidity, and its incidence is increasing as the population is aging. On the other hand, significant improvement in the prognosis has not occurred. The absence of early diagnosis means that many patients are diagnosed only when they develop symptoms, such as jaundice, due to a biliary obstruction. The role of endoscopy in multidisciplinary care for patients with pancreatic cancer continues to evolve. Controversy remains regarding the best preoperative biliary drainage in patients with surgically resectable pancreatic head cancer. In general, patients undergoing a surgical resection usually do not require preoperative biliary drainage unless they have cholangitis or receive neoadjuvant chemotherapy. If biliary drainage is performed prior to surgery, the patient's condition and a multidisciplinary approach should be considered. With the increasing life expectancy of patients with pancreatic cancer, the need for more long-time biliary drainage or pre-operative biliary drainage is also increasing. Strong evidence of endoscopic retrograde cholangiopancreatography (ERCP) as a first-line and essential treatment for biliary decompression has been provided. On the other hand, the use of endoscopic ultrasound-guided biliary drainage as well as percutaneous biliary drainage has been also recommended. During ERCP, self-expandable metal stent could be recommended instead of a plastic stent for the purpose of long stent patency and minimizing stent-induced complications. In this review, several points of view regarding the relief of obstruction in patients with pancreatic cancer, and optimal techniques are being discussed.
Aging ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Decompression ; Drainage ; Drug Therapy ; Early Diagnosis ; Endoscopy ; Hand ; Head and Neck Neoplasms ; Humans ; Incidence ; Jaundice ; Life Expectancy ; Mortality ; Pancreatic Neoplasms ; Plastics ; Prognosis ; Stents

BackgroundPaclitaxel (PTX) could inhibit the growth of fibroblasts, which occurs in proliferative cholangitis and leads to biliary stricture. However, its use has been limited due to poor bioavailability and local administration for short time. This study designed and synthesized a new PTX-conjugated chitosan film (N-succinyl-hydroxyethyl chitosan containing PTX [PTX-SHEC]) and evaluated its safety and efficiency using in vivo and in vitro experiments.

Methods:The SHEC conjugated with PTX was confirmed by nuclear magnetic resonance (NMR) and Fourier-transform infrared spectroscopy (FT-IR) measurements. Drug releases in vitro and in vivo were determined using high-performance liquid chromatography. Cell viability in vitro was measured using 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide. Rabbit biliary stricture model was constructed. All rabbits randomly divided into five groups (n = 8 in each group): the sham-operated rabbits were used as control (Group A), Groups B received laparotomies and suture, Group C received laparotomies and covered SHEC suture without the PTX coating, Group D received laparotomies and covered PTX-SHEC suture, and Group E received laparotomies and 1000 μmol/L PTX administration. Liver function tests and residual dosage of PTX from each group were measured by enzyme-linked immunosorbent assay. Histological data and α-smooth muscle actin (SMA) immunohistochemical staining of common bile duct were examined.

Results:NMR and FT-IR indicated that PTX was successfully introduced, based on the appearance of signals at 7.41-7.99 ppm, 1.50 ppm, and 1.03 ppm, due to the presence of aromatic protons, methylene protons, and methyl protons of PTX, respectively. No bile leak was observed. The PTX-conjugated film could slowly release PTX for 4 weeks (8.89 ± 0.03 μg at day 30). The in vitro cell viability test revealed significantly different levels of toxicity between films with and without PTX (111.7 ± 4.0% vs. 68.1 ± 6.0%, P < 0.001), whereas no statistically significant difference was observed among the three sets of PTX-contained films (67.7 ± 5.4%, 67.2 ± 3.4%, and 59.1 ± 6.0%, P > 0.05). Histological examinations revealed that after 28 days of implantment, Groups D and E (but not Group C) had less granulation tissue and glandular hyperplasia in the site of biliary duct injury than Group B. The pattern was more obvious in Group D than Group E. Less α-SMA-positive cells were found in tissue from Groups D and E. Comparing with Group E, the liver function was improved significantly in Group D, including total bilirubin (2.69 ± 1.03 μmol/L vs. 0.81 ± 0.54 μmol/L, P = 0.014), alanine aminotransferase (87.13 ± 17.51 U/L vs. 42.12 ± 15.76 U/L, P = 0.012), and alkaline phosphatase (60.61 ± 12.31 U/L vs. 40.59 ± 8.78 U/L, P < 0.001).

ConclusionsPTX-SHEC film effectively inhibites the myofibroblast proliferation and extracellular matrix over-deposition during the healing process of biliary reconstruction. This original film might offer a new way for reducing the occurrence of the benign biliary stricture.

Animals ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Chitosan ; chemistry ; Cholangitis ; drug therapy ; Drug Carriers ; chemistry ; Humans ; Magnetic Resonance Spectroscopy ; Membranes, Artificial ; Paclitaxel ; chemistry ; pharmacology ; therapeutic use ; Rabbits ; Spectroscopy, Fourier Transform Infrared