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Background Treatment failure occurs in 20% of autoimmune hepatitis patients on prednisolone and azathioprine (AZA). There is no established second line treatment. Aim To assess the efficacy of mycophenolate mofetil as second line treatment after AZAintolerance or AZA-nonresponse in autoimmune hepatitis and overlap syndromes. Methods Consecutive patients from the Dutch Autoimmune Hepatitis Group cohort, consisting of 661 patients, with autoimmune hepatitis or overlap syndromes, AZAintolerance or AZA-nonresponse and past or present use of mycophenolate mofetil were included. Primary endpoint of mycophenolate mofetil treatment was biochemical remission. Secondary endpoints were biochemical response (without remission), treatment failure and prevention of disease progression. Results Forty-five patients treated with mycophenolate mofetil were included. In autoimmune hepatitis remission or response was achieved in 13% and 27% in the AZA-nonresponse group compared to 67% and 0% in the AZAintolerance group (P = 0.008). In overlap-syndromes remission or response was reached in 57% and 14% in the AZA-nonresponse group and 63% and 25% of the AZA-intolerance group (N.S.); 33% had side effects and 13% discontinued mycophenolate mofetil. Overall 38% had treatment failure; this was 60% in the autoimmune hepatitis AZA-nonresponse group. Decompensated liver cirrhosis, liver transplantations and death were only seen in the autoimmune hepatitis AZA-nonresponse group (P < 0.001). Conclusions Mycophenolate mofetil induced response or remission in a majority of patients with autoimmune hepatitis and azathioprine-intolerance and with overlap syndromes, irrespective of intolerance or nonresponse for azathioprine. In autoimmune hepatitis with azathioprine nonresponse mycophenolate mofetil is less often effective. [ABSTRACT FROM AUTHOR]

Autoimmune pancreatitis (AIP) is associated with a marked elevation of serum total IgG4. Although there is evidence of autoimmunity in AIP, there are also signs of an allergic nature of its pathogenesis. Therefore, we determined both IgE and IgG4 in 13 patients with AIP, in 12 patients with pancreatic carcinoma and in 14 patients with atopic allergy and investigated the relationship between IgE and IgG4. Total IgG4 was determined by automated nephelometry and total IgE by automated enzyme fluoroimmunoassay. Both total IgE and total IgG4 levels in patients with AIP were significantly higher than those in patients with pancreatic carcinoma ( P = 0.0004 and P = 0.015, respectively). There was a significant correlation between the total IgE and total IgG4 levels in patients with AIP and patients with atopic allergy ( rs = 0.82, P = 0.0006 and rs = 0.88, P < 0.0001, respectively). The IgE/ IgG4 ratio in sera from patients with atopic allergy was significantly different ( P = 0.0012) from this ratio in sera from patients with AIP. These results suggest that analysis of total IgE in serum might be useful in the differentiation between autoimmune pancreatitis and pancreatic carcinoma. [ABSTRACT FROM AUTHOR]

Aliment Pharmacol Ther 2010; 32: 529–534 Background The clinical significance of ascites in patients with extrahepatic portal vein thrombosis (EPVT) has been poorly defined. Aims To assess the frequency, natural history and prognostic implication of ascites in patients with EPVT and to identify risk factors for this complication. Methods A single-centre retrospective study of consecutive patients diagnosed with noncirrhotic nonmalignant EPVT between 1985 and 2009. Results One hundred and three patients [35% males; median age 43 (range 16–83) years] were included and followed up for a median time of 5.2 (range 0.9–32.5) years. Twenty-nine (28%) had ascites at the time of diagnosis. Overall survival was 91% at 5 years vs. 80% at 10 years. Survival in patients presenting with and without ascites was 83% vs. 95% at 5 years and 42% vs. 87% at 10 years ( P = <0.01). There was no correlation between the presence of ascites and extension of the thrombus into the large splanchnic veins, duration of thrombosis or presence of gastrointestinal bleeding. Conclusions Ascites is present in a quarter of patients presenting with noncirrhotic nonmalignant extrahepatic portal vein thrombosis. Ascites is a significant and independent prognostic factor and it is associated with a decreased long-term survival. [ABSTRACT FROM AUTHOR]

Background Extrahepatic portal vein thrombosis is an important cause of non-cirrhotic portal hypertension. Aim To provide an update on recent advances in the aetiology and management of acute and chronic non-cirrhotic non-malignant extrahepatic portal vein thrombosis. Method A PubMed search was performed to identify relevant literature using search terms including ‘portal vein thrombosis’, ‘variceal bleeding’ and ‘portal biliopathy’. Results Myeloproliferative disease is the most common risk factor in patients with non-cirrhotic non-malignant extrahepatic portal vein thrombosis. Anticoagulation therapy for at least 3 months is indicated in patients with acute extrahepatic portal vein thrombosis. However, in patients with extrahepatic portal vein thrombosis due to a prothrombotic disorder, permanent anticoagulation therapy can be considered. The most important complication of extrahepatic portal vein thrombosis is oesophagogastric variceal bleeding. Endoscopic treatment is the first-line treatment for variceal bleeding. In several of the patients with extrahepatic portal vein thrombosis biliopathy changes on endoscopic retrograde cholangiography (ERCP) have been reported. Dependent on the persistence of the biliary obstruction, treatment can vary from ERCP to hepaticojejunostomy. Conclusion Prothrombotic disorders are the major causes of non-cirrhotic, non-malignant extrahepatic portal vein thrombosis and anticoagulation therapy is warranted in these patients. The prognosis of patients with non-cirrhotic, non-malignant extrahepatic portal vein thrombosis is good, and is not determined by portal hypertension complications but mainly by the underlying cause of thrombosis. [ABSTRACT FROM AUTHOR]

Background Ascites is the most common complication of cirrhosis, associated with an expected survival below 50% after 5 years. Prognosis is particularly poor for patients with refractory ascites and for those developing complications, including spontaneous bacterial peritonitis (SBP) and hepatorenal syndrome (HRS). Aim To provide an evidence-based overview of the pathophysiology, diagnosis and clinical management of ascites secondary to liver cirrhosis. Methods Review based on relevant medical literature. Results Portal hypertension, splanchnic vasodilatation and renal sodium retention are fundamental in the pathophysiology of ascites formation. The SAAG (serum-ascites albumin gradient) allows reliable assessment of the cause of ascites. The majority of cirrhotic patients with ascites can be managed with dietary sodium restriction in combination with diuretic agents. Large volume paracentesis with albumin suppletion and TIPS are therapeutic options in patients with refractory ascites. Prophylactic antibiotics for SBP should be given in certain patient populations. Conclusions Recent advances in the diagnosis and treatment of ascites and associated complications have improved the medical management and poor prognosis of patients with these manifestations of advanced liver disease. Early diagnosis, adequate treatment and focus on prevention of complications remain essential as well as timely referral for liver transplantation. [ABSTRACT FROM AUTHOR]

The article describes the case of a 60-year-old man who presented to the emergency department with transient loss of consciousness. The patient developed massive hematemesis with hemodynamic instability while being under observation 12 hours later and he underwent emergent upper gastrointestinal endoscopy. It was discovered that massive gastrointestinal bleeding is caused by gastric submucosal arterial collaterals due to occlusion of the splenic artery.

A response by L. Garcia-Buey and R. Moreno-Otero to a letter to the editor on their article "Mycophenolate mofetil for patients with autoimmune hepatitis and overlap syndromes" in the 2011 issue is presented.

Background: Pruritus is a common symptom associated with cholestatic liver diseases. To date only small single centre case series have suggested efficacy of nasobiliary drainage in relieving cholestatic pruritus., Aim: To perform a multicentre study to evaluate the safety and efficacy of nasobiliary drainage in cholestatic pruritus., Methods: This was a retrospective study of all patients treated with nasobiliary drainage for refractory cholestatic pruritus between 2006 and 2015 at five European centres. Pruritus was quantified using a visual analogue scale (VAS) and liver enzymes, serum bilirubin and total serum bile salts (TBS) were measured before (pre-NBD) and after nasobiliary drainage (post-NBD). We analysed the duration of treatment response and associated complications., Results: In total, 27 patients (59% females) underwent 29 nasobiliary drainage procedures. The median duration of NBD was 7 days. NBD decreased pruritus in 89.6% of cases (VAS from 10.0 to 0.3, P < 0.0001). The median percentage decline in pruritus VAS was 94% and 33% of patients were free of pruritus within 24 h of starting drainage. The duration of treatment response was independent of duration of drainage (P = 0.12) and bile output. Significant improvements were seen in the median levels of serum alkaline phosphatase (P = 0.001) and serum bilirubin (P = 0.03) but not in serum TBS (P = 0.07). Mild post-endoscopic retrograde cholangiopancreatography pancreatitis (31%) was the most frequent complication., Conclusions: Nasobiliary drainage is effective in relieving cholestatic pruritus in most patients and has favourable effect on biomarkers of cholestasis. Nasobiliary drainage may be associated with high risk of adverse events, especially pancreatitis. Prospective studies are needed to confirm our findings., (© 2015 John Wiley & Sons Ltd.)

Background: Ursodeoxycholic acid (UDCA) prolongs transplantation-free survival in primary biliary cirrhosis (PBC). However, the optimal therapeutic dose has not been established., Aim: To compare the effects of UDCA administered in daily doses of 10 vs. 20 mg/kg on symptoms, liver biochemistry and biliary UDCA enrichment., Methods: A 6-month multicentre randomized open controlled trial was conducted to assess the effects of an increase in the dose of UDCA to 20 mg/kg/day vs. continuation of 10 mg/kg/day for patients who had not achieved biochemical normalization during treatment for at least 6 months with the 10 mg/kg dose. Clinical and laboratory evaluations were performed at entry and at 3-month intervals. The percentage UDCA in duodenal bile was assessed at entry and at 6 months., Results: Sixty-one patients were enrolled. No side-effects of UDCA were observed. Within the 20 mg/kg/day group significant decreases were found for alkaline phosphatase (- 8%; P = 0.003), aspartate aminotransferase (- 11%; P = 0.01), alanine aminotransferase (- 17%; P < 0.001), gamma-glutamyl transferase (- 34%; P < 0.001), immunoglobulin M (- 11%; P = 0.002) and cholesterol (- 8.1%; P < 0.001). In the 10 mg/kg group none of these parameters differed significantly from baseline. No significant differences between dose groups for symptom scores or serum bilirubin were found. Biliary enrichment with UDCA increased from 37% to 46% in the 20 mg/kg group (P = 0.02) while remaining stable in the 10 mg/kg group., Conclusions: Liver biochemistry improved in PBC patients receiving UDCA 20 mg/kg/day compared to a dose of 10 mg/kg/day. Both doses were equally well tolerated. These results indicate that UDCA 10 mg/kg/ day is a suboptimal dose for treating PBC.

We present the first case of Budd-Chiari syndrome in association with portal and mesenteric vein thrombosis in a patient homozygous for the factor V Leiden mutation. She was treated by transjugular intrahepatic portosystemic stent (TIPS) placement followed by local thrombolytic therapy. Venous outflow from the liver was established and the thrombi in the portal and mesenteric veins were lysed completely. This therapeutic approach may be used for such patients with this severe thrombotic event, who generally have a poor prognosis.