Dataset Information

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

ABSTRACT: Our patient with primary biliary cholangitis, previously termed as primary biliary cirrhosis, presented with an unexpected and unusual cause of hematemesis in the form of multiple esophageal ulcers in-between variceal columns. Given that upon endoscopic examination, the esophageal ulcers were found to bleeding instead of the varices; they should be considered in the differential in the etiology of hematemesis in primary biliary cholangitis and thoroughly searched for during an endoscopic procedure for early treatment and subsequent secondary prevention.

SUBMITTER: Kolli S

PROVIDER: S-EPMC6188036 | biostudies-other | 2018 Oct

REPOSITORIES: biostudies-other

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Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

Kolli Sindhura S Mori Amit A Moshenyat Isaac I

Gastroenterology research 20181001 5

Our patient with primary biliary cholangitis, previously termed as primary biliary cirrhosis, presented with an unexpected and unusual cause of hematemesis in the form of multiple esophageal ulcers in-between variceal columns. Given that upon endoscopic examination, the esophageal ulcers were found to bleeding instead of the varices; they should be considered in the differential in the etiology of hematemesis in primary biliary cholangitis and thoroughly searched for during an endoscopic procedu ...[more]

PMID: 30344813

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Project description:Esophageal variceal bleeding is a severe complication of portal hypertension with significant morbidity and mortality. Although traditional screening and grading of esophageal varices has been performed by endogastroduodenoscopy (EGD), wireless video capsule endoscopy provides a minimally invasive alternative that may improve screening and surveillance compliance.The aim of the study was to perform a systematic review and structured meta-analysis of all eligible studies to evaluate the efficacy of wireless capsule endoscopy for screening and diagnosis of esophageal varices among patients with portal hypertension.Searches of PubMed, EMBASE, Web of Science, and the Cochrane Library databases were performed through December 2015. Bivariate and hierarchical models were used to compute the pooled sensitivity and specificity, and to plot the summary receiver operating characteristics curve with summary point and corresponding 95% confidence region. Bias of included studies was assessed using the quality assessment of diagnostic accuracy studies-2.Seventeen studies from 2005 to 2015 were included in this meta-analysis (n=1328). The diagnostic accuracy of wireless capsule endoscopy in the diagnosis of esophageal varices was 90% [95% confidence interval (CI), 0.88-0.93]. The diagnostic pooled sensitivity and specificity were 83% (95% CI, 0.76-0.89) and 85% (95% CI, 0.75-0.91), respectively. The diagnostic accuracy of wireless capsule endoscopy for the grading of medium to large varices was 92% (95% CI, 0.90-0.94). The pooled sensitivity and specificity were 72% (95% CI, 0.54-0.85) and 91% (95% CI, 0.86-0.94), respectively, for the grading of medium to large varices. The use of capsule demonstrated only mild adverse events. A sensitivity analysis limited to only high quality studies revealed similar results.Wireless esophageal capsule endoscopy is well tolerated and safe in patients with liver cirrhosis and suspicion of portal hypertension. The sensitivity of capsule endoscopy is not currently sufficient to replace EGD as a first exploration in these patients, but given its high accuracy, it may have a role in cases of refusal or contraindication to EGD.

Project description:Purpose of reviewPrimary biliary cholangitis (PBC) is characterized by autoimmune damage of intrahepatic bile ducts associated with a loss of tolerance to mitochondrial antigens. PBC etiopathogenesis is intriguing because of different perplexing features, namely: a) although mitochondria are present in all cell types and tissues, the damage is mainly restricted to biliary epithelial cells (BECs); b) despite being an autoimmune disorder, it does not respond to immunosuppressive drugs but rather to ursodeoxycholic acid, a bile salt that induces HCO3- rich choleresis; c) the overwhelming female preponderance of the disease remains unexplained. Here we present an etiopathogenic view of PBC which sheds light on these puzzling facts of the disease.Recent findingsPBC develops in patients with genetic predisposition to autoimmunity in whom epigenetic mechanisms silence the Cl-/HCO3- exchanger AE2 in both cholangiocytes and lymphoid cells. Defective AE2 function can produce BECs damage as a result of decreased biliary HCO3- secretion with disruption of the protective alkaline umbrella that normally prevents the penetration of toxic apolar bile salts into cholangiocytes. AE2 dysfunction also causes increased intracellular pH (pHi) in cholangiocytes, leading to the activation of soluble adenylyl cyclase, which sensitizes BECs to bile salt-induced apoptosis. Recently, mitophagy was found to be inhibited by cytosolic alkalization and stimulated by acidification. Accordingly, we propose that AE2 deficiency may disturb mitophagy in BECs, thus, promoting the accumulation of defective mitochondria, oxidative stress and presentation of mitochondrial antigens to the immune cells. As women possess a more acidic endolysosomal milieu than men, mitophagy might be more affected in women in an AE2-defective background. Apart from affecting BECs function, AE2 downregulation in lymphocytes may also contribute to alter immunoregulation facilitating autoreactive T-cell responses.SummaryPBC can be considered as a disorder of Cl-/HCO3- exchange in individuals with genetic predisposition to autoimmunity.

Dataset Information

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

Publications

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices.

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