Jennifer L. Horsley-Silva

An update on cancer risk and surveillance in primary sclerosing cholangitis

Malignancy represents substantial morbidity and mortality in patients with primary sclerosing cholangitis (PSC). This subset of patients has been proven to be at increased risk for developing cholangiocarcinoma, gallbladder carcinoma and colorectal cancer in those with overlapping inflammatory bowel disease. Herein, we review the prevalence of these malignancies and recommend screening tools and current knowledge to reduce the disease burden in this population. Cholangiocarcinoma is the most dominant malignancy affecting PSC patients, with a lifetime risk ranging from 5% to 20%. We advocate for serial US or MRI/MRCP and CA 19‐9 to screen for cholangiocarcinoma. Gallbladder cancer has a lifetime risk around 2% in this population and we agree with annual imaging for lesions as recommended by national guidelines. Patients with PSC and concomitant IBD are at increased risk of colorectal carcinoma from time of diagnosis and therefore should likely undergo annual surveillance. The low rates of hepatocellular cancer and pancreatic cancer indicate surveillance for these malignancies is less advantageous.

Docetaxel Induced Sclerosing Cholangitis

A 78-year-old gentleman was referred for hepatology consultation. One year prior, he was diagnosed with metastatic high-grade prostate adenocarcinoma. Dual androgen deprivation therapy (ADT) with leuprolide and bicalutamide was started, and he underwent 43 fractions of palliative radiation to the pelvis. Due to evidence of improved survival outcomes with combination ADT and docetaxel in hormonal-sensitive metastatic prostate cancer, chemotherapy was initiated [1]. Prior to initiation of docetaxel, his liver injury tests (LITs) were normal, and computer tomography (CT) of the abdomen and pelvis demonstrated normal liver appearance. Docetaxel was initiated using every 3 week dosing (75 mg/m, total dose = 134 mg), and after administration, the patient developed elevated LITs for the first time: alkaline phosphatase 197 u/l, alanine aminotransferase (ALT) 67 u/l, aspartate aminotransferase (AST) 59 u/l, and total bilirubin 0.9 mg/dl. Patient was asymptomatic, no evidence of abdominal pain, jaundice, or pruritus. He was switched to weekly docetaxel due to side effects (30 mg/m weekly, total dose = 54 mg); however, LITs elevation continued: alkaline phosphatase 518 u/l, ALT 144 u/l, AST of 90 u/l, and total bilirubin of 0.8 mg/dl. Repeat CT demonstrated new diffuse intrahepatic biliary dilatation with periductal enhancement suggestive of a diffuse cholangitis picture (Fig. 1). Docetaxel was held, and patient was monitored. After 2 months without resolution of abnormal LITs, magnetic resonance imaging (MRI) was pursued, demonstrating continued multifocal narrowing and dilatation of intrahepatic bile ducts, including peripheral bile ducts, with the appearance of sclerosing cholangitis (Fig. 2). An IgG4 level was normal. Liver biopsy 2 months later was performed to determine whether further chemotherapy should be continued, and because of limited evidence that metastatic prostate cancer can simulate sclerosing cholangitis [2]. Biopsy revealed features of subacute bile duct obstruction and stricturing with moderate hepatocanalicular cholestasis, and reactive changes as evidenced by numerous eosinophils. There was biliary-type bridging fibrosis with no architectural distortion or regenerative nodules (Fig. 3). These findings are consistent with a druginduced inflammation and sclerosing cholangitis. Five months later, imaging portrayed continued evidence of secondary sclerosing cholangitis. Docetaxel (Taxotere ) is a potent semisynthetic derivative of paclitaxel, a member of the taxane class of chemotherapy that works by binding to microtubules and & Hugo E. Vargas vargas.hugo@mayo.edu

Segmental Arterial Mediolysis: A Case of Mistaken Hemorrhagic Pancreatitis and Review of the Literature

CONTEXT Segmental arterial mediolysis is an uncommon, non-atherosclerotic, non-inflammatory arteriopathy that involves areas of dissecting aneurysms and strictures that are caused by outer media lysis of the arterial wall from areas of medial necrosis of uncertain pathogenesis. It has a predilection for splanchnic arteries and often presents as abdominal pain or hemorrhage in late middle-aged and elderly patients. Diagnosis can be established by computed tomography angiography, magnetic resonance angiography, or angiogram by visualizing typical abnormalities, in addition to excluding other vasculitides. Histological confirmation is the gold standard but is not easily accessible and, as such, is not frequently performed. CASE REPORT Here we present an updated review of the literature and a case of segmental arterial mediolysis that presented with spontaneous intra-abdominal bleeding near the pancreas that was originally misdiagnosed as hemorrhagic pancreatitis. CONCLUSION Diagnosis is important because immunosuppressants for vasculitis can worsen the arteriopathy. Segmental arterial mediolysis can be self-limiting without treatment or may require urgent surgical or endovascular therapy for bleeding and carries a 50% mortality rate. Therefore, it should be included in the differential of causes of abdominal pain as well as in cases of unexplained abdominal hemorrhage.

Advances in primary sclerosing cholangitis

Primary sclerosing cholangitis is a chronic, progressive cholangiopathy that frequently affects men and is associated with inflammatory bowel disease. Although the cause of the disease is still debated, a genetic association and link to immune-mediated disease triggered by environmental factors are thought to contribute. The disease can present as isolated imaging abnormalities, biochemical changes, cholangiocarcinoma, or end-stage complications such as cirrhosis. Symptoms of primary sclerosing cholangitis include fatigue, jaundice, pruritus, or steatorrhoea. Differentiation of primary sclerosing cholangitis can be challenging because other chronic cholangiopathies can present similarly; however, the distinction is necessary to optimise disease surveillance. Management involves assessment for comorbid inflammatory bowel disease and exclusion of other associated cholangiopathic disorders. Patients with primary sclerosing cholangitis have a poor prognosis; progression to liver cirrhosis is common, and an increased risk of hepatobiliary and colorectal cancers is present in those with inflammatory bowel disease. Although much research involves locating an active therapy that can alter the disease course, the only available treatment is liver transplantation, and risk for disease recurrence remains. Use of ursodeoxycholic acid can improve alkaline phosphatase and bilirubin concentrations but does not alter the disease course. In this Review, we summarise aetiological theories, provide an update on hepatobiliary malignancies that require surveillance, and discuss exciting areas of investigation for potential treatment.

Endoscopic retrograde cholangiopancreatography in patients with surgically altered gastrointestinal anatomy

Endoscopic retrograde cholangiopancreatography (ERCP) in patients with surgically altered upper gastrointestinal anatomy, such as Roux-en-Y gastric bypass (RYGB), can be more challenging compared to those with a normal anatomy. Detailed assessment of cross-sectional imaging features by the radiologist, especially the pancreaticobiliary anatomy, strictures, and stones, is very helpful to the endoscopist in planning the procedure. In addition, any information on enteral anastomoses (for e.g., gastrojejunal strictures and afferent limb obstruction) is also very useful. The endoscopist should review the operative note to understand the exact anatomy prior to procedure. RYGB, which is performed for medically complicated obesity, is the most commonly encountered altered anatomy ERCP procedure. Other situations include patients who have had a pancreaticoduodenectomy or a hepaticojejunostomy. Balloon-assisted deep enteroscopy (single and double-balloon enteroscopy) or rotational endoscopy is often used to traverse the length of the intestine to reach the papilla. In addition, ERCP in these patients is further challenging due to the oblique orientation of the papilla relative to the forward viewing endoscope and the limited enteroscopy-length therapeutic accessories that are currently available. Overall, reported therapeutic success is approximately 70–75% with a complication rate of 3–4%. Alternative approaches include percutaneous transhepatic cholangiography, laparoscopy-assisted ERCP, or surgery. Given the complexity, ERCP in patients with surgically altered anatomy should be performed in close collaboration with body imagers, interventional radiology, and surgical services.

Educational Intervention in Primary Care Residents' Knowledge and Performance of Hepatitis B Vaccination in Patients with Diabetes Mellitus.

Objectives Although guidelines recommend hepatitis B virus (HBV) immunization for adults with diabetes mellitus (DM), vaccination rates remain low. Our aim was to evaluate knowledge and practice regarding HBV and to assess the effectiveness of a multifaceted educational program. Methods Primary care residents (n = 244) at three academic institutions were surveyed about various aspects of HBV. Residents at one training program were then randomly assigned to an educational intervention (E) (n = 20) and control group (C) (n = 19). The E group received a focused didactic lecture and periodic e-mail reminders with immediate feedback. We compared knowledge scores before and after the intervention. Chart audits were conducted to evaluate the residents’ behavior. Results A total of 103 (42%) residents responded to the survey. The survey indicated that residents lacked the necessary knowledge and risk assessment skills concerning HBV in patients with DM. In the controlled trial of the E intervention, both groups had similar baseline knowledge scores. The E group had a significant increase in the immediate postintervention knowledge scores from a mean of 29% at baseline to 70% (P < 0.001) that was sustained 6 months postintervention (65%; P < 0.001). In the C group, 6-month postintervention scores were not different from baseline (38% vs 29%). No significant differences were observed in documentation skills. Conclusions A combined educational program was effective in enhancing knowledge about HBV and vaccination in DM but had limited influence on physicians’ practice. Further study incorporating system changes along with educational initiatives is required to improve clinical practice.

Ibrutinib-induced acute liver failure

Waldenstrom’s macroglobulinemia (WM) is a lymphoplasmacytic lymphoma characterized by monocloncal IgM gammopathy and bone marrow infiltration [1]. First-line treatment has traditionally consisted o...

An Unusual Suspect: Lymphoepithelial Cyst of the Pancreas

Lymphoepithelial cysts (LECs) of the pancreas are benign, rare pancreatic cysts that are found predominantly in men. These cysts can present as a diagnostic conundrum given their rarity and difficulty of distinguishing these cysts from those with malignant potential. We present an incidental case of a LEC in a middle-aged man.

Gastrointestinal endoscopy in the cirrhotic patient

As advances in liver disease continue, including the increasing use of liver transplantation, the endoscopist needs to be familiar with the standards of care and potential complications in the management of the cirrhotic population. This includes both elective endoscopic procedures, such as screening colonoscopies and variceal banding, as well as the acutely bleeding cirrhotic patient. Peri-procedural management and standards of care for acute gastrointestinal hemorrhaging of cirrhotic patients will be emphasized. This article will focus on the plethora of data available to highlight the benefits of endoscopic intervention in the care of patients with liver disease and outline the areas of future emphasis.

Association of Total Fluid Intake and Output with Duration of Hospital Stay in Patients with Acute Pancreatitis

Background/Aims The aim of this study was to evaluate the association of fluid balance with outcomes in patients hospitalized with acute pancreatitis (AP). Methods This was a retrospective study of patients hospitalized between May 2008 and June 2016 with AP and a clinical order for strict recording of intake and output. Data collected included various types of fluid intake and output at 24 and 48 hours after admission. The primary outcome was length of stay (LOS). Analysis was performed using single-variable and multivariable negative binomial regression models. Results Of 1256 patients hospitalized for AP during the study period, only 71 patients (5.6%) had a clinical order for strict recording of intake and output. Increased urine output was associated with a decreased LOS at 24 and 48 hours in univariable analysis. An increasingly positive fluid balance (total intake minus urine output) at 24 hours was associated with a longer LOS in multivariable analysis. Conclusions Few patients hospitalized for AP had a documented order for strict monitoring of fluid intake and output, despite the importance of monitoring fluid balance in these patients. Our study suggests an association between urine output and fluid balance with LOS in AP.