Hepatic Sarcoidosis: Current Concepts and Treatments

2020 ◽  
Vol 41 (05) ◽  
pp. 652-658
Author(s):  
Geoffrey Rossi ◽  
Marianne Ziol ◽  
Dominique Roulot ◽  
Dominique Valeyre ◽  
Matthieu Mahévas
Keyword(s):  
Portal Hypertension ◽  
Current Knowledge ◽  
Liver Involvement ◽  
Radiological Features ◽  
Hepatic Sarcoidosis ◽  
Common Manifestation

AbstractHepatic sarcoidosis is a relatively common manifestation of extrapulmonary sarcoidosis. It occurs in 20 to 30% of cases and is rarely severe. However, a cluster of patients may develop severe complications such as cirrhosis and portal hypertension. In this review, we describe the current knowledge of clinical, biological, pathological, and radiological features of liver involvement in sarcoidosis and discuss essential clues for management and treatment.

scholarly journals Sarcoidosis Complicated by Cirrhosis and Hepatopulmonary Syndrome

2008 ◽  
Vol 15 (3) ◽  
pp. 124-126 ◽  
Author(s):  
Samir Gupta ◽  
Marie E Faughnan ◽  
Gerald J Prud’homme ◽  
David M Hwang ◽  
David G Munoz ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Gas Exchange ◽  
Brain Abscess ◽  
Arteriovenous Malformations ◽  
Opportunistic Infections ◽  
Hepatopulmonary Syndrome ◽  
Liver Involvement ◽  
Pulmonary Arteriovenous Malformations ◽  
Lung Involvement ◽  
Multisystem Disorder

Sarcoidosis is a multisystem disorder commonly affecting the lungs, but also the liver, with cirrhosis and portal hypertension occurring in fewer than 1% of cases. Although hepatopulmonary syndrome (HPS) is seen in 15% to 20% of patients with cirrhosis of varying causes, it has rarely been associated with sarcoidosis. Also, although a brain abscess is not uncommon in patients with discrete pulmonary arteriovenous malformations, it is rarely seen in patients with the much smaller intrapulmonary vascular dilations that characterize HPS. A patient with an unusual series of uncommon sarcoidosis complications, including cirrhosis with HPS, brain abscess and finallyNocardiameningitis, is reported. The possibility of HPS should be considered in sarcoidosis patients with liver involvement, if gas-exchange abnormalities are out of proportion to the degree of lung involvement. These patients may also be susceptible to a cerebral abscess by paradoxical embolization, and to opportunistic infections due to cirrhosis.

scholarly journals Managing portal hypertension in patients with liver cirrhosis

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 533 ◽  
Author(s):  
Tilman Sauerbruch ◽  
Robert Schierwagen ◽  
Jonel Trebicka
Keyword(s):  
Portal Hypertension ◽  
Viral Infections ◽  
Current Knowledge ◽  
Beta Blockers ◽  
Recurrent Bleeding ◽  
The Past ◽  
Ascites Formation ◽  
Inflammatory Stimuli ◽  
Impact On Survival ◽  
Initiating Factors

Portal hypertension is one cause and a part of a dynamic process triggered by chronic liver disease, mostly induced by alcohol or incorrect nutrition and less often by viral infections and autoimmune or genetic disease. Adequate staging - continuously modified by current knowledge - should guide the prevention and treatment of portal hypertension with defined endpoints. The main goals are interruption of etiology and prevention of complications followed, if necessary, by treatment of these. For the past few decades, shunts, mostly as intrahepatic stent bypass between portal and hepatic vein branches, have played an important role in the prevention of recurrent bleeding and ascites formation, although their impact on survival remains ambiguous. Systemic drugs, such as non-selective beta-blockers, statins, or antibiotics, reduce portal hypertension by decreasing intrahepatic resistance or portal tributary blood flow or by blunting inflammatory stimuli inside and outside the liver. Here, the interactions among the gut, liver, and brain are increasingly examined for new therapeutic options. There is no general panacea. The interruption of initiating factors is key. If not possible or if not possible in a timely manner, combined approaches should receive more attention before considering liver transplantation.

Portal hypertension and ascites secondary to Erdheim Chester Disease without intrinsic liver involvement on liver biopsy

2013 ◽  
Vol 45 (11) ◽  
pp. 964-965 ◽  
Author(s):  
Donald N. Tsynman ◽  
Curtis Weaver ◽  
Sofia Taboada ◽  
Jennifer Findeis-Hosey ◽  
Benedict Maliakkal ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Liver Biopsy ◽  
Liver Involvement ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

Liver Involvement in Systemic Lupus Erythematosus: Case Review of 40 Patients

2008 ◽  
Vol 35 (11) ◽  
pp. 2159-2164 ◽  
Author(s):  
VAIDEHI R. CHOWDHARY ◽  
CYNTHIA S. CROWSON ◽  
JOHN J. POTERUCHA ◽  
KEVIN G. MODER
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Hepatic Encephalopathy ◽  
Viral Hepatitis ◽  
Lupus Erythematosus ◽  
Liver Involvement ◽  
Biliary Cirrhosis ◽  
Systemic Lupus ◽  
Cryptogenic Cirrhosis

ObjectiveSubclinical liver involvement is frequent in systemic lupus erythematosus (SLE). We sought to determine the presence of endstage liver disease in patients with SLE.MethodsWe carried out a retrospective chart review of our cohort of patients with SLE. Endstage liver disease was defined as presence or development of cirrhosis, portal hypertension, or hepatic encephalopathy.ResultsForty patients with liver enzyme abnormalities were identified. Major clinical diagnostic groups were drug-induced (n = 4), viral hepatitis (hepatitis B or C and cytomegalovirus; n = 8), non-alcoholic fatty liver disease (NAFLD; n = 8), autoimmune hepatitis (AIH; n = 6), primary biliary cirrhosis (PBC; n = 3), and miscellaneous [n = 11; liver involvement from infection (2), cryptogenic cirrhosis (2), lymphoma (1), and indeterminate (6)]. There were no differences in mean age, total and direct bilirubin, or aspartate aminotransferase and alkaline phosphatase levels. Alanine aminotransferase levels were higher in the miscellaneous group. Biopsies were performed in 20 patients and showed changes of NAFLD (n = 5), AIH (n = 4), PBC (n = 3), hepatitis C (n = 3), and cryptogenic cirrhosis (n = 2), and 1 each with phenytoin-induced liver injury, hepatic granulomas due to systemic candidiasis, and lymphomatous involvement of the liver. The median followup was 44 months (range 10–576). The estimated 5-year serious liver disease-free survival was 93% (95% confidence interval 84%–100%). Eight patients died. Mortality was not directly related to liver disease in any patient.ConclusionComplications of portal hypertension, cirrhosis, and hepatic encephalopathy are rare manifestations of SLE unless coexistent liver disease such as NAFLD, viral hepatitis, or AIH is present.

scholarly journals Targeting Gut–Liver Axis for Treatment of Liver Fibrosis and Portal Hypertension

Livers ◽  
2021 ◽  
Vol 1 (3) ◽  
pp. 147-179
Author(s):  
Eric Kalo ◽  
Scott Read ◽  
Golo Ahlenstiel
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Liver Fibrosis ◽  
Biomarker Discovery ◽  
Current Knowledge ◽  
Therapeutic Strategies ◽  
Decompensated Cirrhosis ◽  
Microbial Composition ◽  
Medicine Development ◽  
And Personalized Medicine

Antifibrotic therapies for the treatment of liver fibrosis represent an unconquered area of drug development. The significant involvement of the gut microbiota as a driving force in a multitude of liver disease, be it pathogenesis or fibrotic progression, suggest that targeting the gut–liver axis, relevant signaling pathways, and/or manipulation of the gut’s commensal microbial composition and its metabolites may offer opportunities for biomarker discovery, novel therapies and personalized medicine development. Here, we review potential links between bacterial translocation and deficits of host-microbiome compartmentalization and liver fibrosis that occur in settings of advanced chronic liver disease. We discuss established and emerging therapeutic strategies, translated from our current knowledge of the gut–liver axis, targeted at restoring intestinal eubiosis, ameliorating hepatic fibrosis and rising portal hypertension that characterize and define the course of decompensated cirrhosis.

scholarly journals Etiology and Management of Hemorrhagic Complications of Portal Hypertension in Children

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Alejandro Costaguta ◽  
Fernando Alvarez
Keyword(s):  
Portal Hypertension ◽  
Treatment Outcomes ◽  
Pediatric Patients ◽  
Current Knowledge ◽  
Alcoholic Cirrhosis ◽  
Individual Characteristics ◽  
Small Children ◽  
Substantial Progress ◽  
Hypertension In Children ◽  
Management Challenge

Portal hypertension in children represents a particular diagnostic and management challenge for several reasons: (1) treatment outcomes should be evaluated in relationship with a long-life expectancy, (2) pediatric patients with portal hypertension constitute an heterogeneous population, both in terms of individual characteristics and diversity of liver diseases; making comparison between treatment outcomes very difficult, (3) application of techniques and procedures developed in adult patients (v.gr. TIPS) face size limitations in small children, and (4) absence of data from well-controlled trials in children forces pediatric specialists to adapt results obtained from adult cohorts suffering from diseases such as HCV and alcoholic cirrhosis. Despite those limitations, substantial progress in the treatment of children with portal hypertension has been achieved in recent years, with better outcomes and survival. Two main factors influence our therapeutic decision: age of the patient and etiology of the liver disease. Therefore, diagnosis and treatment of complications of portal hypertension in children need to be described taking such factors into consideration. This paper summarizes current knowledge and expert opinion.

scholarly journals Advances in therapeutic options for portal hypertension

2018 ◽  
Vol 11 ◽  
pp. 175628481881129 ◽  
Author(s):  
Marina Vilaseca ◽  
Sergi Guixé-Muntet ◽  
Anabel Fernández-Iglesias ◽  
Jordi Gracia-Sancho
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Clinical Research ◽  
Current Knowledge ◽  
Cellular Mechanisms ◽  
Deep Impact ◽  
Primary Mechanism ◽  
Clinical Consequences ◽  
Blood Inflow ◽  
Intrahepatic Vascular Resistance

Portal hypertension represents one of the major clinical consequences of chronic liver disease, having a deep impact on patients’ prognosis and survival. Its pathophysiology defines a pathological increase in the intrahepatic vascular resistance as the primary factor in its development, being subsequently aggravated by a paradoxical increase in portal blood inflow. Although extensive preclinical and clinical research in the field has been developed in recent decades, no effective treatment targeting its primary mechanism has been defined. The present review critically summarizes the current knowledge in portal hypertension therapeutics, focusing on those strategies driven by the disease pathophysiology and underlying cellular mechanisms.

Portal hypertension caused by acute cytomegalovirus infection with liver involvement in an immunocompetent patient

2007 ◽  
Vol 39 (2) ◽  
pp. 177-178 ◽  
Author(s):  
Konstantina Tzavella ◽  
Anestis Zantidis ◽  
Ippolyti Economou ◽  
Kalliopi Mandraveli ◽  
Stella Alexiou-Daniel ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Cytomegalovirus Infection ◽  
Immunocompetent Patient ◽  
Liver Involvement ◽  
Acute Cytomegalovirus

scholarly journals Oxidative Stress in Chronic Liver Disease and Portal Hypertension: Potential of DHA as Nutraceutical

Nutrients ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2627 ◽  
Author(s):  
Zoe Boyer-Diaz ◽  
Paloma Morata ◽  
Peio Aristu-Zabalza ◽  
Albert Gibert-Ramos ◽  
Jaime Bosch ◽  
...  
Keyword(s):  
Public Health ◽  
Oxidative Stress ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Docosahexaenoic Acid ◽  
Chronic Liver Disease ◽  
Current Knowledge ◽  
Chronic Liver ◽  
Public Health Issue ◽  
Clinical Scenarios

Chronic liver disease constitutes a growing public health issue worldwide, with no safe and effective enough treatment clinical scenarios. The present review provides an overview of the current knowledge regarding advanced chronic liver disease (ACLD), focusing on the major contributors of its pathophysiology: inflammation, oxidative stress, fibrosis and portal hypertension. We present the benefits of supplementation with docosahexaenoic acid triglycerides (TG-DHA) in other health areas as demonstrated experimentally, and explore its potential as a novel nutraceutical approach for the treatment of ACLD and portal hypertension based on published pre-clinical data.

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