Search

IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. One out of three patients with IgG4 sclerosing cholangitis also presents autoimmune pancreatitis, although it can be associated with manifestations in other organs. One of the main features of IgG4-related disease is its good prognosis due to the great response to glucocorticoid therapy. However, relapse of the disease is not uncommon, especially when steroid therapy is decreased or stopped. Rituximab seems to be an effective treatment to achieve remission of the disease. We report the case of a 74 year-old man diagnosed with IgG4-related disease based on increase of serum IgG4 levels, imaging and histopathological findings, with systemic involvement including sclerosing cholangitis. Despite the absence of liver fibrosis at onset, the early use of glucocorticoids and rituximab therapy, the patient presented clinical and analytical deterioration, leading to secondary biliary cirrhosis. In conclusion, this clinical case highlights the importance of prompt diagnosis and therapeutics for sclerosing cholangitis secondary to IgG4-related disease in order to avoid progression of the disease and development of liver cirrhosis, as well as the refractory, aggressive nature of the disease in some cases as this one.

Sclerosing cholangitis (SC) is defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis and currently includes three categories: primary sclerosing cholangitis (PSC), secondary cholangitis, and IgG4-related sclerosing cholangitis (IgG4-SC). SC categories share similar clinical features, such as cholestasis. Patients with SC present with cholestatic symptoms, including jaundice and pruritus, and blood tests reveal elevation of cholestatic enzymes. Cholangiography, endoscopic or magnetic, is inevitably required for making a diagnosis. Although the presentation of IgG4-SC and PSC are similar, the comorbidities, treatment response, and outcomes differ significantly, and therefore, it is strongly advisable to be familiar with these two diseases to make a correct diagnosis. Differentiation of cholangiocarcinoma from IgG4-SC and PSC is also extremely important. In this review, the clinical characteristics, comorbidities, treatment and outcomes of IgG4-SC and PSC will be outlined based on experience mainly from Japan.

Sarcoidosis is a multisystem granulomatous disease. The liver is affected in up to 50–90% of cases. Sarcoidosis typically presents as non-necrotizing epithelioid granuloma. The occurrence of non-infective necrotizing sarcoid granuloma (NSG) is infrequent, and the finding of NSG in the liver is rare. We report a case of NSG of the liver and lymph nodes, granulomatous gastric ulcer, and secondary cholangitis coexisting in a patient. We discuss the clinical features of the case and briefly review NSG. There is only 1 previously reported case of NSG of the liver in literature.

International audience; Magnetic resonance cholangiography (MRC) has become the standard of reference for imaging of the biliary ducts. The use of three-dimensional (3D) sequences has resulted in improved spatial resolution with virtually isotropic voxel and improved signal/noise ratio. In addition to MRC images, 3D fat suppressed T1-weighted MR images should be systematically obtained to search for intrahepatic calculi. MRC plays a major role in the diagnosis of cholangiocarcinoma and assessment of its resectability. With modern MR systems the performance of MR is basically the same that of CT for evaluation of arterial and portal vein extent. MRC is a key imaging modality for the diagnosis of primary sclerosing cholangitis. Different imaging patterns may be observed including multifocal intra- and extrahepatic strictures alternating with slightly dilated ducts. Focal signal abnormality of the liver parenchyma and focal parenchymal atrophy represent the consequences of biliary duct obstruction on liver parenchyma. Diagnosis of biliary lithiasis is performed by combination of MRC and T1-weighted MR imaging. MRC can be performed for the diagnosis of secondary cholangitis including ascending cholangitis, ischemic cholangitis and IgG4-related sclerosing cholangitis. Hepatobiliary contrast agents could be used for demonstrating the site of biliary duct leakage after surgery and for functional imaging. MR imaging can also be used to determine the prognosis of PSC. The inherent limitations of MRC of bile ducts are still the suboptimal spatial resolution for evaluation of distal intrahepatic biliary ducts.

A 68-year-old woman was admitted because of septicaemia after cardiac valve replacement. Cause of the septicaemia was a proximal filiform common bile duct stenosis with recurrent gallstones and secondary cholangitis. A single pneumatic balloon dilatation widened the common bile duct. At the same time, specific antibiotic treatment cured the septicaemia. Nine months later no further dilatation has been necessary.