It is estimated that 1 million patients in the United States suffer from cirrhosis, and > 10% suffer from chronic encephalopathy. Early results indicate safety and tolerability of GPB in patients with cirrhosis.9 Moreover, GPB contains no sodium, compared to almost 2.4 g of sodium in a standard adult dose of NaPBA. Thus, this remarkable clinical trial in ultraorphan UCDs may eventually expand treatment options for more frequently encountered patients with cirrhosis suffering
from chronic encephalopathy. “
“Background and Aim: This study aimed to determine the clinical characteristics of immunoglobulin G4 (IgG4)-associated sclerosing cholangitis (ISC) and provide clinical clues differentiating ISC from primary RG-7388 sclerosing cholangitis (PSC) or hilar cholangiocarcinoma (CCC). Methods:
Sixteen patients with ISC manifesting as hilar/intrahepatic strictures were analyzed for clinical characteristics and compared with patients with PSC and hilar CCC as disease controls for histology and serum IgG4 levels. Results: Distinguished biliary imaging findings of ISC included multifocal biliary tree involvement (n = 14), concentric bile duct thickening with preserved luminal patency (n = 13), and relatively mild proximal dilatation, despite prominent bile duct thickening VX-770 nmr (n = 11). Serum IgG4 levels were elevated in 12 patients (75%), but not in any of the 25 patients with hilar CCC. Ten patients (63%) had a past or concurrent history of autoimmune pancreatitis (AIP). The significant infiltration of IgG4-positive cells was observed with endobiliary or liver biopsy in 11 of 16 patients (69%) with ISC, but not in any patients with PSC or hilar CCC. Extrabiliary organ involvement, including sialadenitis, inflammatory pseudotumor of the liver and kidney, and retroperitoneal fibrosis, was present in seven patients. Marked improvement of biliary strictures and/or extrabiliary involvement was
observed in all ISC patients after steroid therapy. Conclusions: ISC should be considered in the differential diagnosis of hilar/intrahepatic biliary strictures. Past or concurrent AIP or extrabiliary organ involvement strongly check details suggests the possibility of ISC. Significant infiltration of IgG4-positive cells on endobiliary or liver biopsy specimens, and/or elevated serum IgG4 levels, highly support the diagnosis of ISC and provide the rationale for steroid therapy. Hilar or intrahepatic biliary strictures are caused by diverse etiologies from benign to malignant, including iatrogenic bile duct injury, primary sclerosing cholangitis (PSC), bile duct stone, other fibroinflammatory cholangitis, and malignancy. The most important issue is how to differentiate benign from malignant strictures.