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Seminars in Liver Disease

Terumi Kamisawa, Sawako Kuruma, Kazuro Chiba
An accurate diagnosis should be made before treatment of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis. Once a diagnosis has been established, steroids are the standard therapy and symptoms are the major indications. Before steroid therapy, obstructive jaundice and hyperglycemia should be controlled. An initial dose of 0.6 mg/kg/d of oral prednisolone is administered for 2 to 4 weeks, and is gradually tapered over 2 to 3 months. After steroid therapy has begun, blood and imaging tests are performed periodically...
August 2016: Seminars in Liver Disease
Mitsuhiro Kawano, Kazunori Yamada
Immunoglobulin G4-related kidney disease (IgG4-RKD) is the collective name encompassing renal parenchymal and renal pelvic lesions. The hallmark of renal parenchymal lesions of IgG4-related disease is plasma cell-rich tubulointerstitial nephritis with numerous IgG4-positive plasma cells and characteristic fibrosis. In addition, glomerular lesions are sometimes present, with membranous glomerulonephritis being the most common. Although IgG4-RKD shows good responsiveness to corticosteroid therapy, follow-up imaging studies have revealed that partial cortical scars persist when the start of therapy is delayed...
August 2016: Seminars in Liver Disease
Motohisa Yamamoto, Hiroki Takahashi
Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity that was established in this century. It is a systemic and chronic inflammatory disorder. Autoimmune pancreatitis is a representative condition, but there is clearly involvement from other organs, including the kidneys, the retroperitoneal cavity, the lungs, and the thyroid, lacrimal, salivary, pituitary, and prostate glands. To aid in the resolution of the numerous issues related to IgG4-RD, the authors established a registry system of patients in multicenter institutes with IgG4-RD-the SMART registry...
August 2016: Seminars in Liver Disease
Shigeyuki Kawa
Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer...
August 2016: Seminars in Liver Disease
Yoh Zen
Immunoglobulin G4-related disease (IgG4-RD) in the pancreatobiliary system manifests as sclerosing cholangitis (SC), hepatic inflammatory pseudotumors, and type 1 autoimmune pancreatitis (AIP). The pathology of IgG4-RD involves an inflammatory process and fibrogenic pathway, the combination of which damages the affected organs. Fibroinflammatory injury is characterized by three microscopic findings: a diffuse lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis...
August 2016: Seminars in Liver Disease
Yasuni Nakanuma, Yoji Ishizu, Yoh Zen, Kenichi Harada, Takeji Umemura
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system. Two types of hepatic parenchymal involvement have been reported in IgG4-RD: IgG4-related autoimmune hepatitis (AIH) and IgG4-hepatopathy. Moreover, only three cases of IgG4-related AIH have been reported. Immunoglobulin G4-related AIH is clinicopathologically similar to AIH, except for an elevated serum IgG4 level and heavy infiltration of IgG4-positive plasma cells in the liver tissue...
August 2016: Seminars in Liver Disease
Takahiro Nakazawa, Shuya Shimizu, Itaru Naitoh
More men than women develop immunoglobulin G4-related sclerosing cholangitis (IgG4-SC). Age at clinical onset is significantly older in patients with IgG4-SC. Patients with IgG4-SC appear similar to those with cholangiocarcinoma and primary sclerosing cholangitis (PSC). The association between IgG4-SC and autoimmune pancreatitis (AIP) is useful for the diagnosis of IgG4-SC. However, some IgG4-SC cases are isolated from AIP and are difficult to diagnose. The authors focus on three distinct features of IgG4-SC...
August 2016: Seminars in Liver Disease
Laura C Lighaam, Theo Rispens
Human immunoglobulin G4 (IgG4) antibodies are in many ways unusual. In this review, an overview is given of the structural and functional aspects of IgG4 antibodies, the consequences of IgG4 antibody formation in various disease settings, and the factors involved in the regulation of IgG4 responses. Unlike most IgG antibodies, IgG4 antibodies exist in a dynamic equilibrium with other IgG4 antibodies, continuously exchanging half-molecules resulting in effectively monovalent antibodies that cannot cross-link...
August 2016: Seminars in Liver Disease
Kazushige Uchida, Toshihiro Tanaka, M Eric Gershwin, Kazuichi Okazaki
Immunoglobulin G4-related disease (IgG4-RD) is a recently described systemic inflammatory disease characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis. However, although IgG4-RD has become increasingly recognized, the number of patients with IgG4-RD remains unclear. Data from several studies indicate that patients who have a T-helper type 2 (Th2-) dominant immune response, which leads to the hyperproduction of Th2 cytokines, then progress to IgG4-RD...
August 2016: Seminars in Liver Disease
Carmen Sendra, Javier Ampuero, Álvaro Giráldez Gallego, José Manuel Sousa, María Serrano Jiménez, Manuel Romero-Gómez
Acute-on-chronic liver failure (ACLF) represents a reversible syndrome associated with high short-term mortality, characterized by acute decompensation in patients with chronic liver disease and extrahepatic organ failure. Diagnosis and prognosis assessment is based on a newly developed diagnostic score, the Chronic Liver Failure Consortium Organ Failure score. Susceptibility to infections and systemic inflammation are typical triggers. The authors report a case in which a patient with alcohol-related cirrhosis was admitted to the hospital with acute decompensation and developed ACLF during hospitalization...
May 2016: Seminars in Liver Disease
Laura Weichselbaum, Thierry Gustot
In this review to the authors provide evidence-based definitions of organ failure in patients with acute decompensation of cirrhosis, describe the prevalence and prognostic impact of organ failure in these patients, and highlight current knowledge of the mechanisms responsible for its development. Excessive inflammatory response and a reduction of the capacity of organs to respond adequately to stress may sensitize cirrhotic patients to inflammation-driven organ damage and failure. The dynamic evolution of organ failure during early intensive management may provide a useful tool for guiding subsequent management strategies, or in some cases, for defining the futility of care...
May 2016: Seminars in Liver Disease
Jonel Trebicka
Acute-on-chronic liver failure (ACLF) is a syndrome with high short-term mortality in patients with chronic liver disease. The definition of ACLF has been addressed recently in many publications, and despite regional differences the number and severity of organ failures are decisive for the presence and severity of ACLF. However, the predisposition of patients to develop ACLF has not been addressed in prospective studies. Several predisposing factors for ACLF have been mentioned, but still clear-cut analysis and the sequential processes have not been explored...
May 2016: Seminars in Liver Disease
Sakkarin Chirapongsathorn, Jayant A Talwalkar, Patrick S Kamath
After the Patient Protection and Affordable Care Act or "Obamacare" was signed into law in 2010, the problem of readmission has taken on a new sense of urgency. Hospitals with excess readmissions receive reduced reimbursement because readmission is considered to represent a poor quality measure in the healthcare delivery system. Cirrhosis places a major burden on the healthcare economy. Patients with cirrhosis frequently require hospitalization, and annual admission rates have doubled within 10 years. The costs of hospitalization associated with cirrhosis have also markedly increased...
May 2016: Seminars in Liver Disease
Han Li, Harvey Shi-Hsien Chen, Scott L Nyberg
Recognition of acute-on-chronic liver failure (ACLF) as a unique entity is slowly evolving, as are therapies to improve survival of affected patients. Further investigation into its disease process and proper treatments with critical timing are important for improving patient survival. At this time, liver transplant is the only treatment known to improve survival in liver-failure patients. However, liver transplantation has its own disadvantages, such as organ shortage and the need for lifelong immunotherapy...
May 2016: Seminars in Liver Disease
Francois Durand, Mitra K Nadim
Acute-on-chronic liver failure (ACLF) is defined by the occurrence of organ failure(s) other than the liver in patients with cirrhosis. Even though mortality rates are high, there should no longer be reluctance to admit patients with ACLF in the intensive care unit. The prevalence of multidrug-resistant bacteria is high and broad spectrum antibiotics should be initiated as soon as infection is suspected. In patients with circulatory failure, the assessment of circulatory status is challenging due to the hyperkinetic state and an imbalance between the splanchnic and systemic blood volume...
May 2016: Seminars in Liver Disease
Richard Moreau
Although systemic inflammation is a hallmark of acute-on-chronic liver failure (ACLF), its role in the development of this syndrome is poorly understood. Here the author first summarizes the general principles of the inflammatory response. Inflammation can be triggered by exogenous or endogenous inducers. Important exogenous inducers include bacterial products such as pathogen-associated molecular patterns (PAMPs) and virulence factors. Pathogen-associated molecular patterns elicit inflammation through structural feature recognition (using innate pattern-recognition receptors [PRRs]), whereas virulence factors generally trigger inflammation via functional feature recognition...
May 2016: Seminars in Liver Disease
Rajeshwar P Mookerjee
As formal definitions of acute-on-chronic liver failure (ACLF) have now been established, and given an increased recognition of the dynamic nature of this condition, there is a growing clinical need to assess prognosis and response to interventions. Conventional scoring systems such as Model for End-Stage Liver Disease (MELD) fail to capture the two key prognostic elements in ACLF-namely, extrahepatic organ failure and measures of systemic inflammation-and as such are limited in their prognostic accuracy. Even the best available scoring systems such as the recently described CLIF (Chronic Liver Failure) Consortium ACLF (CLIF-C ACLF) score, are at best 75% accurate and need to be applicable to all etiologies of liver disease...
May 2016: Seminars in Liver Disease
Alina M Allen, W Ray Kim
Chronic liver disease and cirrhosis, a common end result of viral hepatitis, alcohol abuse, and the emerging epidemic of nonalcoholic fatty liver disease are a significant source of morbidity and premature mortality globally. Acute clinical deterioration of chronic liver disease exemplifies the pinnacle of healthcare burden due to the intensive medical needs and high mortality risk. Although a uniformly accepted definition for epidemiological studies is lacking, acute-on-chronic liver failure (ACLF) is increasingly recognized as an important source of disease burden...
May 2016: Seminars in Liver Disease
Elsa Solà, Javier Fernandez, Pere Ginès
Acute-on-chronic liver failure (ACLF) is a complex syndrome that develops in patients with cirrhosis at any stage during the natural history of the disease. In most cases, the development of ACLF is associated with an identifiable precipitating factor. Overall, the most common precipitating events are bacterial infections. Although data suggest that any type of infection may trigger ACLF, spontaneous bacterial peritonitis, secondary peritonitis, and pneumonia appear to be the most common types of infection leading to ACLF...
May 2016: Seminars in Liver Disease
Vicente Arroyo, Rajiv Jalan
Acute-on-chronic liver failure (ACLF) is a recently recognized syndrome in cirrhosis characterized by acute decompensation (AD), organ failure(s), and high short-term mortality. Organ failure(s) is defined by the Chronic Liver Failure-Sequential Organ Failure (CLIF-SOFA) score or by its simplified version Chronic Liver Failure-Organ Failure Assessment (CLIF-OF) score. They include six types of organ failure: liver, renal, coagulation, cerebral, respiratory, and circulatory. One third of patients hospitalized with AD present with ACLF at admission or develop ACLF during hospitalization...
May 2016: Seminars in Liver Disease
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