Background: Theoutcomeof Primary sclerosing cholangitis ((PSC)) after liver transplantation can be affected by recurrent (PSC) (r(PSC)) and subsequent graft failure. IgG4-related sclerosing disease is a recently described entity that has a similar morphological appearance to (PSC), making the distinction difficult. However, IgG4-related sclerosing cholangitis has an excellent prognosis since it is steroid sensitive, but the impact of IgG4 on r(PSC) after liver transplant is still unknown. Objectives: To determine the association between IgG4 positive immunochemical staining in liver explants and recurrence of Primary sclerosing cholangitis post-liver transplantation. Methods: Clinical information on all adult patients who underwent liver transplantation for (PSC) from 1990 to 2014 at our institution was obtained. IgG4 immunochemical staining was performed on the porta-hepatis region of these patient’ s explanted livers. Immunochemical staining was considered to be positive if the score was > 5 cells/high power field (HPF). Results: Eighty patients met inclusion criteria. IgG4 staining was positive in 21 subjects. Median time for follow-up in the IgG4 positive group was 99. 6 months compared to 152. 6 months in the IgG4 negative. There were more instances of r(PSC) in the IgG4 negative group compared to the IgG4 positive group (26% vs. 5%, P < 0. 009), and r(PSC) occurred earlier in the IgG4 negative group (P = 0. 016). The frequency of graft failure, however was not significantly different among the two groups. Conclusions: The presence of IgG4 positive cells in liver explants of patient’ s transplanted for (PSC) is associated with fewer episodes of and longer time to recurrent (PSC). In addition, IgG4 status of the liver explants does not affect graft survival. Presence of IgG4 positive cells may suggest protective effect against recurrence of (PSC).

Primary sclerosing cholangitis is a chronic cholestatic liver disease defined by strictures of the biliary tree which could ultimately lead to liver cirrhosis and cholangiocarcinoma. Although the exact underlying etiology of this disorder is not fully understood, the pathology is believed to be caused by immune mediated mechanisms. Growing body of evidence suggests several treatment modalities mainly focusing on the inflammation aspect of this disorder. However, there is still no consensus regarding the best treatment option for these patients. Thus, the present study aimed to review the current treatment options for patients with Primary sclerosing cholangitis.

Introduction and Aims: We noticed in our practice that the patients with ulcerative colitis (UC) who have developed Primary sclerosing cholangitis ((PSC)) experience a milder course of colonic disease. Our objective in this study was to define whether there is any difference between UC activity and its course in patients with and without (PSC). Materials and Methods: 19 patients with UC and (PSC) (8 male, mean age 25) were enrolled. To every patient with UC and (PSC), 3 patients with UC alone (totally 57 patients, 28 male, mean age 24) matched for age at onset, duration of the disease, and extension of colonic disease were selected as the control group. We used number of hospitalizations due to activity of UC and number of short corticosteroid administrations in various years of follow-up as variables indicating course and severity of the colonic disease in this period. For comparing trends of UC activity between two groups, we used repeated measures two-way analysis of variances. Results: Mean duration of follow up in case and control groups was 12.2 ± 5.7 and 11.4 ± 4.9 years, respectively. Two groups had no significant difference in use of sulfasalazine or aminosalicylates. Number of hospitalizations and courses of steroid therapy because of UC activity decreased significantly over time (p<.000) in both groups and it was significantly higher in controls than in cases (p=.045 and .032, respectively). Conclusions: Development of (PSC) in patients with UC might have a positive effect on the colonic disease. Further investigation to evaluate basis of this improvement are warranted

Background: Primary sclerosing cholangitis is a rare and progressive cholestatic disease characterized by narrowing of the biliary tract. Despite high levels of ulcerative colitis, early diagnosis of Primary sclerosing cholangitis before the diagnosis of inflammatory bowel disease is rarely reported. Case history: A 28-year-old woman with no history of medical disease visited our clinic because of a mild generalized pruritus. In initial blood laboratory tests with elevated liver enzymes was observed. In Magnetic resonance cholangiopancreatography (MRCP ) was introduced Primary sclerosing cholangitis to the patient. Therefore, the patient was under colonoscopy and extensive colitis with terminal ileum involvement was detected and the diagnosis of ulcerative colitis was confirmed in the pathology. Conclusion: Patients diagnosed with Primary sclerosing cholangitis should undergo biopsy colonoscopy to rule out inflammatory bowel disease, even if they have no digestive tract symptoms.

Aim: The aim of this systematic review was to determine if the human colon, through the lower gut-liver axis, drives (PSC) activity by assessing the progression of the disease in patients with and without colectomy for colonic disease. Background: The gut-liver axis is involved in the pathogenesis of liver disease. Abnormal immune-mediated responses to intestinal microbiome are implicated in Primary sclerosing cholangitis ((PSC)) however the mechanisms remain poorly understood. Currently, no single animal model recapitulates all attributes of (PSC) in humans and this limits further studies of gut-liver interactions. Methods: A systematic search of PubMed, Medline, and Scopus was performed for articles that contained the terms “ colectomy” or “ bowel resection” AND “ Primary sclerosing cholangitis” up to 15th April 2018. Articles were reviewed by 2 reviewers and raw data collated. A Forest plot was used to illustrate the effect of colectomy on subsequent liver transplantation for (PSC). Linear regression was used to estimate mortality risk. Results: Colectomy appeared to have no effect on (PSC) progression, although high-quality studies were lacking. Rates of liver transplantation or transjugular intrahepatic portosystemic shunt for (PSC) were not affected by colectomy (OR 0. 59, 95% CI 0. 14-2. 53, p=0. 48). Mortality risk following colectomy in patients with (PSC) is 2. 11% per year (95% CI 0. 03%-4. 18%, p=0. 032, R2 = 0. 722). Conclusion: Current evidence is limited but suggests colectomy does not affect the progression of (PSC) in patients with colonic disease. Pathogenic micro-organisms or antigens that drive (PSC) may not be limited to the lower gut.

Background: Primary sclerosing cholangitis ((PSC)) is a chronic cholestatic disease that results in segmental stenosis of the bile ducts, cholestasis and fibrosis. During the previous two decades, patients with steroid responsive (PSC) have been presented in a few case reports and studies. This study aims to evaluate and compare IgG4 levels in patients with ulcerative colitis (UC), with and without (PSC).Materials and Methods: This cross-sectional study enrolled 116 patients with UC who referred to the Gastrointestinal Clinic of Imam Khomeini Hospital, Tehran, Iran. Patients with allergic diseases such as asthma and atopic dermatitis as well as those with autoimmune disorders such as vasculitis and systemic lupus erythmatosus were excluded. After signing an informed consent form, 5cc blood sample was taken for serum IgG4 evaluation. Patients with clinical or laboratory signs of (PSC) underwent Magnetic resonance cholangiopancreatography (MRCP) after which patients were divided into two groups (with and without (PSC)) according to the results of their MRCP findings. IgG levels more than 157 mg/dl were considered elevated. SPSS software package version 16 was used for data analysis. A significance level of 0.05 was considered statistically significant.Results: There were 57 (49.1%) male participants, of which 24 (20.7%) had evidence of (PSC). Among these, 7 (29.1%) had elevated IgG4 titers. Of participants, 92 did not have (PSC). From this group, 9 (9.7%) had elevated IgG4 levels. This difference was statistically significant (p=0.012). The odds ratio was 3.9 (CI: 1.28-12.1) and the area under the curve for IgG4 in predicting (PSC) in the ROC curve was 0.65. There was a significant relationship between IgG4 titer and colitis extension in both groups. However there was no significant relationship regarding the severity of colitis (p=0.247).Conclusion: It is necessary to evaluate IgG4 titers in all patients with (PSC) because of its treatable nature. Determining IgG4 levels in all UC patients can be a predicting factor for (PSC). Thus additional cohort studies with larger numbers of participants are recommended.

Objective: There are several studies reporting the therapeutic effects of Berberis vulgaris on liver diseases. This study was done with the purpose of examining the effect of B. vulgaris oxymel (BO) in patients with refractory Primary sclerosing cholangitis ((PSC)) and Primary biliary cholangitis (PBC), who did not respond to current treatment. Materials and Methods: Patients with (PSC) or PBC who were receiving ursodeoxycholic acid (UDCA, 13-15 mg/kg/day) for at least six months, but their serum levels of alkaline phosphatase (ALP) were still 1. 5 folds higher than the normal upper limit during the last six months, were asked to participate in this quasi-experimental study. Patients were asked to take 0. 5 ml/kg/day of BO two times a day for three months along with UDCA. At the end of the study, serum levels of ALP, aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT), total bilirubin (TB), direct bilirubin (DB), and creatinine as well as prothrombin time (PT), international normalized ratio (INR) and quality of life (QOL) based on PBC-40 questionnaire were assessed as outcomes. Results: Our results showed that BO notably attenuated the serum levels of ALP, AST, ALT, GGT, TB, and DB, as well as PT and INR and significantly improved QOL. Conclusion: For first time, we showed that additional therapy with BO has a promising effect in the treatment of refractory (PSC) and PBC.