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Purpose of Review: IgG4-related sclerosing cholangitis (ISC) is a frequent occurrence. However, its diagnosis is difficult. This review summarizes the clinical features, pathogenesis, differential diagnosis, and management of ISC. Recent Findings: A precise diagnosis of ISC is important. Diagnosis is based on the Japanese criteria which has recently been provided. The characteristic features of plasma cell infiltration and raised IgG4 levels along with classical histopathological and imaging findings help in diagnosis. Steroid therapy is very effective in ISC. Immunomodulatory drugs have also shown promising results. Summary: Diagnostic approach of ISC mainly involves imaging modalities. Japanese diagnostic criteria is important in its diagnosis. It is also extremely important to differentiate IgG4 sclerosing cholangitis from various other cholangiopathies. Steroid therapy stays the treatment of choice. The role of other immunomodulators needs to be researched and reciprocated in clinical setting before it replaces steroid therapy. [ABSTRACT FROM AUTHOR]

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.

Over the past 2 decades, endoscopic retrograde cholangiopancreatography with stricture dilation and stent placement has gradually become the first-line treatment modality for the vast majority of benign biliary strictures (BBSs). Stricture remediation with progressive placement of multiple plastic stents with 3 months interval stent exchange during a period of one year has excellent long-term results in patients with postoperative BBSs. Covered self-expandable metal stents (SEMS) are a reasonable alternative to multiple plastic stenting, especially in patients with chronic pancreatitis. The use of covered SEMS should be limited to carefully selected cases of postoperative BBSs. Uncovered SEMS are contraindicated for any type of BBSs. Understanding of the etiology and exclusion of malignancy is essential for optimal treatment in some types of biliary strictures, especially in the case of autoimmune cholangiopathy and primary sclerosing cholangitis. [ABSTRACT FROM AUTHOR]

Objective: To determine the diagnostic performance of imaging criteria for distinguishing Ig-G4-associated autoimmune cholangiopathy (IAC) from primary sclerosing cholangitis (PSC) and bile duct malignancy.Methods: A medical records search between January 2008 and October 2013 identified 10 patients (8 M, 2 F, mean age 61 years, range 34-82) with a clinical diagnosis of IAC. Fifteen cases of PSC (6 M, 9 F, mean age 50, range 22-65) and 15 cases of biliary malignancy (7 M, 8 F, mean age 65, range 48-84) were randomly selected for comparative analysis. Three abdominal radiologists independently reviewed MRI with MRCP (n = 32) or CT (n = 8) and ERCP (n = 8) for the following IAC imaging predictors: single-wall bile duct thickness >2.5 mm, continuous biliary involvement, gallbladder involvement, liver disease, peribiliary mass, or pancreatic and renal abnormalities. Each radiologist provided an imaging-based diagnosis (IAC, PSC, or cancer). Imaging predictor sensitivity, specificity, accuracy, and association with IAC using Fisher's exact test. Inter-reader agreement determined using Fleiss' kappa statistics.Results: For diagnosis of IAC, sensitivities and specificities were high (70-93%). Pancreatic abnormality was strongest predictor for distinguishing IAC from PSC and cancer, with high diagnostic performance (70-80% sensitivity, 87-97% specificity), significant association (p < 0.01), and moderate inter-reader agreement (κ = 0.59). Continuous biliary involvement was moderately predictive (50-100% sensitivity, 53-83% specificity) and trended toward significant association in distinguishing from PSC (p = 0.01-0.19), but less from cancer (p = 0.06-0.62).Conclusion: It remains difficult to distinguish IAC from PSC or bile duct malignancy based on imaging features alone. The presence of pancreatic abnormalities, including peripancreatic rind, atrophy, abnormal enhancement, or T2 signal intensity, strongly favors a diagnosis of IAC. [ABSTRACT FROM AUTHOR]

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection. [ABSTRACT FROM AUTHOR]

Several hepatobiliary abnormalities have been described in association with inflammatory bowel disease (IBD), including primary sclerosing cholangitis (PSC), small duct PSC, chronic hepatitis, cryptogenic cirrhosis, cholangiocarcinoma, and cholelithiasis. PSC is the most common biliary condition in patients with IBD, with an incidence ranging from 2.5% to 7.5%. PSC usually progresses insidiously and eventually leads to cirrhosis independent of inflammatory bowel disease activity. There is a very high incidence of cholangiocarcinoma and an elevated risk for developing colon cancer in patients with PSC. Medical therapy has not proven successful in slowing disease progression or prolonging survival. Treatment of symptoms due to cholestasis, such as pruritis and steatorrhea, is an important aspect of the medical care of patients with PSC. Our preferred treatment of pruritis due to cholestasis is with bile acid binding exchange resins, such as cholestyramine or colestipol. Endoscopic manipulation is recommended for treating complications of recurrent cholangitis or worsening jaundice in the setting of a dominant stricture, but endoscopic approaches have not been conclusively demonstrated to improve survival or decrease the need for liver transplantation. Liver transplantation remains the only effective treatment of advanced PSC, and should be considered in patients with complications of cirrhosis or intractable pruritis or fatigue. [ABSTRACT FROM AUTHOR]

Presents an index related to pharmacology published in the June 15, 2003 issue of the journal 'Alimentary Pharmacology & Therapeutics.'

We describe a 43-year-old woman with clinical features compatible with autoimmune cholangiopathy recently reported by Ben Ari et al. She was negative for anti-mitochondrial antibody, positive for high titer anti-nuclear antibody with homogeneous pattern, high levels of serum immunoglobulin G and nearly normal levels of serum immunoglobulin M for more than five years. In the early stages of the disease, the elevations of serum transaminase, alkaline phosphatase and γ-glutamyl transpeptidase were well controlled by the administration of ursodeoxycholic acid. After five years of follow-up, she showed the second exacerbation of liver function tests, which then rapidly improved by prednisone administration. To analyze the antigen diversity recognized by T-cells in the liver, T-cell receptor repertoire was examined by immunohistochemistry. The liver biopsy obtained in the early stage showed clonal accumulation of Vβ5.1-positive cells in portal areas, which was found in patients neither with primary biliary cirrhosis nor autoimmune hepatitis. In conclusion, these data suggest that T-cell response in autoimmune cholangiopathy is different from those two autoimmune liver diseases, which may imply a distinct entity of the disease. [ABSTRACT FROM AUTHOR]