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Background: We attempted to determine the indications and limitations of steroid therapy as the first-line therapy in patients with autoimmune pancreatitis (AIP) with cyst formation (ACF)., Methods: This Japanese multicenter survey was conducted to examine the merits/demerits of steroid treatment as the initial therapy for ACF., Results: Data of a total of 115 patients with ACF were analyzed. Complete remission was achieved in 86% (86/100) of patients who had received steroid treatment, but only 33.3% (5/15) of patients who had not received steroids. Relapse after the remission (n = 86) occurred in 7.6% (6/86) of patients who had received steroid therapy, but 40% (2/5) of patients who had not received steroid therapy. Multivariate analysis identified adoption of the wait and watch approach without steroid treatment (odds ratio = 0.126, P < .001) as a significant and independent negative predictor of remission of ACF. As for predictors of relapse, the presence of varix (odds ratio = 5.83, P = .036) was identified as an independent risk factor., Conclusion: Steroid therapy plays an important role as first-line therapy in AIP patients with pancreatic cyst formation, however, varix formation, besides the diameter of the cyst(s), is a risk factor for refractoriness to steroid therapy., (© 2022 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Objective: The acceptable duration of steroid therapy for patients with IgG4-sclerosing cholangitis (SC) has been under debate. Our aim is to clarify the feasible duration of steroid treatment., Design: We retrospectively reviewed the data of patients with IgG4-SC and analyzed the following: biliary status during the steroid therapy, incidence of remission, relapse, relapse-free survival rate, and steroid-related complications (SRCs)., Results: Remission was achieved 99.5% (763/767) of patients who received steroid therapy, while remission rate dropped to 63.6% (78/129) of patients who didn't receive it. Relapse was noted in 19.7% (151/763) of the patients who received steroid. Besides, relapse rate went up 38.4% (30/78) of the counterpart. Normalization of the serum total bilirubin and serum alkaline phosphatase (ALP) levels were achieved at two weeks regardless of biliary drainage. Multivariate analysis identified younger onset, MST less than three years, immunosuppressant, and steroid cessation as independent risk factors for relapse. Steroid-free was achieved in the patients underwent MST only 3.4% over 54 months. SRCs were recorded in a total of 99 patients (12.9%) despite sufficient preemptive medications. Multivariate analysis identified history of malignancy and immunosuppressant as independent risk factors for SRCs., Conclusion: Steroid therapy should be continued for no less than three years to reduce the risk of relapse, with use of preemptive measures taken around five years. The biliary drainage might not be mandatory. Steroid as 1 st line therapy could serve as a bridge to further promising treatments., (This article is protected by copyright. All rights reserved.)

Several years have passed since the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012 were published. New findings and knowledge have accumulated since then. The Research Committees for IgG4-related Diseases and for Intractable Diseases of the Liver and Biliary Tract, in association with the Ministry of Health, Labor, and Welfare of Japan and the Japan Biliary Association, have established a working group consisting of researchers specializing in IgG4-SC and have drawn up new clinical diagnostic criteria for IgG4-SC 2020. The diagnosis of IgG4-SC is based on a combination of the following six criteria: (a) narrowing of the intra- or extrahepatic bile duct; (b) thickening of the bile duct wall; (c) serological findings; (d) pathological findings; (e) other organ involvement; and (f) effectiveness of steroid therapy. These new diagnostic criteria for IgG4-SC are useful in practice for general physicians and other non-specialists., (© 2021 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background/purpose: Epidemiological data on immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are scarce., Method: We randomly selected healthcare facilities from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with IgG4-SC in 2018. Among responding facilities, we further inquired about the clinical characteristics of the patients with IgG4-SC., Results: We selected 1180 departments from health centers across Japan. Of them, 532 (45.1%) responded to the questionnaires. The number of reported patients with IgG4-SC was 1045, and the estimated point prevalence was 2.18 (95% confidence interval, 2.13-2.23) per 100 000 population. In addition, we enrolled 1096 patients with IgG4-SC, combining data from previously and newly registered patients. Male patients comprised 79.5% of the population and patients aged 60-80 years had the highest risk for developing the disease. The follow-up period was 5.1 ± 4.0 years and the 5- and 10-year overall survival rates were 95.3% and 89.0%, respectively., Conclusions: The point prevalence of IgG4-SC was 2.18 per 100 000 population. The epidemiological features were similar to those observed in previous studies., (© 2020 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

IgG4-related sclerosing cholangitis (IgG4-SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4-related disease. Although clinical diagnostic criteria of IgG4-SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4-SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions (CQs) with clinical statements were developed regarding diagnosis (14 CQs) and treatment (4 CQs). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4-SC., (© 2018 The Authors. Journal of Hepato-Biliary-Pancreatic Sciences published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background: Fluorescence in situ hybridization (FISH) of cytology specimens has been used to diagnose biliary strictures. However, the usefulness of FISH for differentiating between cholangiocarcinoma (CCA) and IgG4-related sclerosing cholangitis (IgG4-SC) has not been evaluated in forceps biopsy specimens., Methods: We retrospectively reviewed 74 specimens obtained by transpapillary forceps biopsy between 2008 and 2015 from 49 consecutive patients with CCA and 25 with IgG4-SC. Specimens were considered positive for malignancy by FISH with UroVysion ® if at least five cells exhibited polysomy (a gain of two or more in chromosomes 3, 7, or 17)., Results: A total of 27 (55.1%) patients with CCA, but none of the patients with IgG4-SC, were positive for malignancy by FISH. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of FISH for the diagnosis of CCA were 55.1%, 100%, 100%, 53.2%, and 70.3%, respectively. The complementary use of FISH increased the sensitivity of hematoxylin-and-eosin (H&E) staining from 69.4% to 77.6%; the specificity was not reduced when either H&E or FISH was positive., Conclusions: The use of FISH in the analysis of forceps biopsy specimens might be one option to differentiate CCA from IgG4-SC., (© 2018 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background: We aimed to clarify the clinical benefits of using 8-mm versus 10-mm diameter self-expandable metallic stent (SEMS) in bilateral endoscopic stent-in-stent (SIS) deployment for malignant hilar biliary obstruction (MHBO)., Methods: A total of 72 consecutive patients with MHBO treated by endoscopic bilateral SIS deployment were enrolled. The 8-mm and 10-mm diameter SEMSs were used in 37 patients (8-mm group) and 35 patients (10-mm group), respectively. We retrospectively compared technical success, functional success, complications, successful re-intervention, time to recurrent biliary obstruction (RBO) and survival time between two groups., Results: There were no significant inter-group differences in terms of technical success (8-mm vs. 10-mm group, both 100%), functional success (96% vs. 100%), early (5% vs. 9%) and late (19% vs. 14%) complications other than RBO, and successful endoscopic re-intervention (94% vs. 100%), respectively. Time to RBO (median: 198 days vs. 222 days; log-rank, P = 0.440) and survival time (median: 186 days vs. 235 days; log-rank, P = 0.883) did not differ significantly between the two groups. However, the success rate of endoscopic bilateral revisionary stent insertion for RBO was significantly higher in the 10-mm compared to the 8-mm group (68% vs. 31%; P = 0.044)., Conclusions: The 10-mm diameter SEMS is more suitable with regard to endoscopic re-intervention., (© 2015 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background: We aimed to clarify the clinical usefulness of one-step self-expandable metal stent (SEMS) placement for distal malignant biliary obstruction by comparing with two-step SEMS placement., Methods: We retrospectively compared early complications, bilirubin level decreasing rate, and period of hospitalization between 40 patients with one-step SEMS and 52 patients with two-step SEMS. We also evaluated the complications in patients with two-step SEMS by classifying them into first drainage (ERBD or ENBD) and second drainage (SEMS)., Results: The total incidence of early complications was significantly higher in patients with one-step SEMS than the second drainage of two-step SEMS (42.5% [17/40] vs. 13.5% [7/52]; P = 0.004). However, there was no significant difference in the early complications between one-step SEMS and two-step SEMS (total of first and second drainage) (42.5% [17/40] vs. 36.5% [19/52]; P = 0.561). The period required to reach the bilirubin level (<3 mg/dL) after initial drainage was significantly shorter in one-step SEMS than in two-step SEMS (5.7 vs. 10.1 days; P = 0.025). Similar results were observed in the period of hospitalization for patients without chemotherapy (22.2 vs. 37.3 days; P = 0.004)., Conclusions: One-step SEMS placement is the alternative option of drainage method for distal malignant biliary obstruction., (© 2013 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background/purpose: IgG4-related sclerosing cholangitis (IgG4-SC) is one of several diseases associated with autoimmune pancreatitis (AIP). However, diffuse cholangraphic abnormalities seen in association with AIP may resemble those seen in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma. IgG4-SC responds well to steroid therapy, whereas in contrast, liver transplantation is the only effective therapy for PSC, and surgical intervention is also needed for cholangiocarcinoma. The aim of this review was to establish the diagnostic procedures for IgG4-SC., Methods: A literature search was conducted, covering English-language articles dealing with IgG4-SC published between 1991 and March 2010. As clinical data on IgG4-SC are limited, the author also took into consideration his own clinical experience with the treatment of IgG4-SC over a period of more than 19 years., Results: When intrapancreatic stenosis is detected, pancreatic cancer should be ruled out. If multiple intrahepatic stenosis is evident, PSC should be discriminated on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining. An association with inflammatory bowel disease (IBD) is suggestive of PSC. If stenosis is demonstrated in the hepatic hilar region, cholangiocarcinoma should be discriminated by US, EUS, IDUS, and bile duct biopsy., Conclusion: For diagnosis of IgG4-SC, coexistence of AIP is the most useful finding. However, the most important consideration for clinicians is to be aware of IgG4-SC when encountering patients with obstructive jaundice.

Purpose: Only a few studies have documented the characteristics of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC). We aimed to clarify the clinical and histopathological characteristics of IBD associated with PSC (PSC-IBD)., Methods: Twenty-nine patients with PSC and 60 patients with ulcerative colitis (UC) but without complicating PSC were enrolled in this study. First, the age and sex distribution, affected area, clinical course, number of recurrent attacks, and severity of UC were investigated. Then, mucosal specimens obtained from the right side (cecum and ascending colon), transverse colon, and the left side (descending colon, sigmoid colon, and rectum) during colonoscopy were studied for inflammatory cell infiltration, the presence of crypt abscesses, the degree of goblet cell disappearance, and edema., Results: (1) The incidence of IBD in PSC patients was 68.9% (20/29). There were two peaks in the age distribution of PSC. Male PSC patients demonstrated a first peak and female patients a second peak. Male PSC-IBD patients were in their teens and 20s making the first peak. Female PSC-IBD patients were in their 50s and 60s making the second peak. The PSC-IBD patents were significantly younger than the patients without IBD (33.6 vs. 58.9 years, p < 0.001). (2) PSC-IBD showed a right-sided predominance colonoscopically. (3) None of the patients had a severe clinical course, and a half of them were asymptomatic. (4) Histopathological examination demonstrated severe inflammatory cell infiltration in the cecum and ascending colon, whereas the degree was mild in the rectum/descending colon., Conclusions: PSC-IBD shows characteristic clinical, colonoscopic, and histopathological findings.