Secondary Sclerosing Cholangitis Research Articles

S1684 Secondary Sclerosing Cholangitis Complicating the Course of Severe COVID-19 Infection

Introduction: COVID-19 infection leads to complications in multiple organs, including the hepato-biliary system. Secondary sclerosing cholangitis (SCC) is diagnosed when a cause for progressive cholestatic liver injury is identified in patients with no prior history of liver disease. SSC seems to stem from inflammation that can be caused by critical illness, infection, ischemia, or autoimmune disease. We present a case of severe COVID-19 infection complicated by SSC requiring liver transplantation. Case Description/Methods: A 45-year-old male with history of type II diabetes presented to the emergency department in April 2020 with shortness of breath for 3 days and was found to be COVID-19 positive. His clinical course deteriorated, and he ultimately required ECMO for acute respiratory distress syndrome (ARDS). The patient was still recovering from his extended hospital stay, but in June he began noticing jaundice. His labs were notable for total bilirubin (11.9), direct bilirubin (8.9), AST (290), ALT (162), and alkaline phosphatase (2815). The patient underwent ERCP which revealed sludge and stones, with sphincterotomy performed and common bile duct stent placed. Beaded intrahepatic ducts with dilation consistent with sclerosing cholangitis were also noted on ERCP. Due to concern for acute cholangitis, he was started on broad spectrum antibiotics as well. The ERCP did not lead to clinical improvement, and the patient's hepatic lab abnormalities continued to worsen. An MRCP was obtained and showed diffuse dilatation and considerable beading and irregularity of the biliary tree, most compatible with an inflammatory cholangiopathy. Liver biopsy showed no evidence of chronic liver disease or primary sclerosing cholangitis but features consistent with biliary obstruction and multifocal acute cholangitis. He underwent orthotopic liver transplant in November 2020, and his MELD score was 31 at the time of surgery. Discussion: SSC in critically ill patients (SSC-CIP) subgroup is particularly rare and was first described in 2001. Persistent cholestasis despite clinical recovery of other organ systems differentiates SSC-CIP from other hepatobiliary diseases related to critically ill patients. The pathophysiology is theorized to be due to a combination of bile duct ischemia leading to cholangiocyte necrosis and bile duct obstruction via bile cast formation. We present a case of SSC in the setting of severe COVID-19 infection requiring ECMO, resulting in liver transplantation.Figure 1.: A: CT revealed an ill defined 5.5cm mesenteric mass (arrow) B: EUS showed hypoechoic mass encasing the superior mesenteric artery C: H&E stain, 10x field; FNB of the mass returned as fibrosis with chronic inflammation including lymphocytes, plasma cells, and eosinophils consistent with retroperitoneal fibrosis.

S2659 SARS-CoV-2-Associated Cholestatic Liver Injury: A Case Series in Critically Ill Patients

Introduction: Hepatocellular injury has been documented in 14% to 76% of patients suffering from Coronavirus Disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is associated with increased rates of intubation and mortality. SARS-CoV-2 enters target cells by binding to angiotensin-converting enzyme 2 present in the lungs, small intestine, cholangiocytes, and hepatocytes. While most patients experience mild acute liver injury, some critically ill patients develop a prolonged cholestatic injury pathologically similar to secondary sclerosing cholangitis. Histologic assessments have demonstrated microvesicular steatosis, diffuse intrahepatic vascular thrombosis, and chronic fibrous endothelial thickening. We present five cases of cholestatic liver injury in critically ill patients, including a novel case of severe hepatic arteriopathy during endoscopic retrograde cholangiopancreatography (ERCP). Case Description/Methods: We identified 5 patients without preexisting liver disease who developed prolonged cholestatic liver injury following severe COVID-19 infection requiring mechanical ventilation, antibiotics, and prolonged hospitalization (Table 1). Four patients developed shock requiring vasopressor support and two required extracorporeal membrane oxygenation. Additionally, Patient 1 underwent ERCP which revealed friable hepatic parenchyma and vascularture that appeared angiographically similar to secondary sclerosing cholangitis. While liver chemistries were normal or mildly elevated at the time of COVID-19 diagnosis, all patients experienced significant cholestasis with predominantly elevated alkaline phosphatase levels ranging from the 900s to 4000s. In patients who survived their initial hospitalization, liver chemistries remained elevated months beyond resolution of their pulmonary and renal injuries, and none had returned to baseline as of this writing. Discussion: We described several cases of prolonged cholestasis in patients with severe COVID-19 infection without preexisting liver disease. While a component of this acute liver injury could be attributed to drugs or ischemia, the timeframe and degree of injury suggest a different etiology, which we postulate may be a combination of secondary sclerosing cholangitis and direct hepatic injury from SARS-CoV-2. Additional research and longitudinal monitoring of these patients is needed to better characterize this type of injury and determine the risk of clinically relevant, long-term liver dysfunction.Table 1-A.: Patient Characteristics (1) COVID-19, Coronavirus Disease 2019 (2) ARDS, Acute Respiratory Distress Syndrome.Table 1-B.: Patient Characteristics (1) COVID-19, Coronavirus Disease 2019 (2) ARDS, Acute Respiratory Distress Syndrome.

S2905 Prolonged Cholestasis After COVID-19

Introduction: The coronavirus SARS-CoV-2 (COVID-19) is known to cause both pulmonary and hepatic injury. Cholangiocytes have an abundance of angiotensin-converting enzyme 2 receptors, which are the main mode of entry for the virus. Whether the liver injury is caused directly by the virus or as a result of the cytokine storm remains unknown. We present a case of prolonged cholestatic liver injury secondary to COVID-19. Case Description/Methods: A 55-year-old woman presented to our institution with sudden onset of right leg pain and swelling. She had hypertension, diabetes, obesity, and hypothyroidism. She denied any history of liver disease, toxic habits and was not taking any hepatotoxic medications or supplements. Two months prior to presentation, she was admitted to another hospital with severe COVID-19 pneumonia requiring mechanical ventilation. She had received Remdesivir, Convalescent Plasma, Extra Corporeal Membrane Oxygenation (ECMO), and hemodialysis for acute renal failure, before being discharged. During this admission, she was found to have a right lower extremity thrombus and was started on Enoxaparin. Her liver function tests were significant for a markedly elevated alkaline phosphatase level. An ultrasound abdomen was significant only for a fatty liver. CT scan of the abdomen with contrast and an MRCP did not reveal any hepatobiliary abnormalities. Viral hepatitis serology and autoimmune workup were negative. She underwent a liver biopsy which demonstrated cholestasis, mild lobular necroinflammation, and macrovesicular steatosis. There was no PCR evidence of SARS-CoV-2. Her hospital course was complicated by MRSA and pseudomonas pneumonia. She suffered a cardiac arrest and ultimately succumbed to her infection 2 months later. Her Alkaline phosphatase remained elevated throughout her hospital stay. Discussion: Although our patient’s liver biopsy did not demonstrate PCR evidence of SARS-COV-2, it was consistent with histological features of COVID-19 related hepatic injury. Macrovesicular steatosis is the most common histological finding, followed by lobular necroinflammation, according to a case series of 40 patients who died of COVID-19. PCR for SARS-COV-2 in liver biopsy was positive in only 55% of patients. Recent reports have identified ECMO as a cause of secondary sclerosing cholangitis in critically ill patients, which remains a possibility in our case. However, a normal bilirubin level and MRCP did not support this diagnosis in our patient.Figure 1.: (A) 4 x magnification, (B) 10 x magnification, and (C) 40 x magnification of a liver biopsy specimen demonstrating hepatocellular cholestasis with feathery degeneration of hepatocytes, mild lobular neuroinflammation, and mild macrovesicular steatosis. There is sinusoidal dilatation and glycogenated nuclei. Portal tracts show mild to moderate mixed cellular infiltration, including eosinophils, endothelilitis, ductular reaction, and focal mild bile duct damage. There is no pericellular fibrosis to indicate non-alcoholic chronic steatohepatitis, although rare perivenular ballooned hepatocytes are noted on the H&E slide. CK7 immunostain shows moderate to marked ductular reaction, rare cholestatic hepatocytes, and damaged bile ducts. Findings are those of subacute cholestatic hepatitis.Table 1.: Laboratory values from the previous hospitalization, on admission day of this hospitalization, at 1-month of hospitalization, and prior to death.

Defining Primary Sclerosing Cholangitis: Results From an International Primary Sclerosing Cholangitis Study Group Consensus Process

Defining Primary Sclerosing Cholangitis: Results From an International Primary Sclerosing Cholangitis Study Group Consensus Process

Liver Transplantation for Langerhans Cell Histiocytosis: A US Population-Based Analysis and Systematic Review of the Literature.

Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. Liver involvement is seen in 10.1% to 19.8% of patients with LCH and can lead to secondary sclerosing cholangitis requiring liver transplantation (LT). We describe the characteristics and outcomes of patients undergoing LT for LCH. All patients undergoing a first LT for LCH in the United States were identified in the Scientific Registry of Transplant Recipients (SRTR) database (1987-2018). The Kaplan-Meier curve method and log-rank tests evaluated post-LT survival. A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. A total of 60 LCH LT recipients were identified in the SRTR, and 55 patients (91.7%) were children with median total bilirubin levels at LT of 5.8mg/dL (interquartile range [IQR], 2.7-12.9). A total of 49 patients (81.7%) underwent deceased donor LT (DDLT). The 1-year, 3-year, and 5-year patient survival rates were 86.6%, 82.4%, and 82.4%, respectively. The systematic review yielded 26 articles reporting on 50 patients. Of the patients, 41 were children (82.0%), 90.0% had multisystem LCH, and most patients underwent DDLT (91.9%; n=34/37). Pre-LT chemotherapy was administered in 74.0% and steroids in 71.7% (n=33/46) of the patients, and a recurrence of LCH to the liver was reported in 8.0% of the patients. Of the 50 patients, 11 (22.0%) died during a median follow-up of 25.2months (IQR, 9.0-51.6), and the 1-year patient survival rate was 79.4%. LT can be considered as a feasible life-saving option for the management of liver failure secondary to LCH in well-selected patients.

Secondary sclerosing cholangitis as cause of persistent jaundice in patients with severe COVID-19.

Background & AimsLittle is known about cholestasis, including its most severe variant secondary sclerosing cholangitis (SSC), in critically ill patients with coronavirus disease 19 (COVID‐19). In this study, we analysed the occurrence of cholestatic liver injury and SSC, including clinical, serological, radiological and histopathological findings.MethodsWe conducted a retrospective single‐centre analysis of all consecutive patients admitted to the intensive care unit (ICU) as a result of severe COVID‐19 at the University Hospital Zurich to describe cholestatic injury in these patients. The findings were compared to a retrospective cohort of patients with severe influenza A.ResultsA total of 34 patients with severe COVID‐19 admitted to the ICU were included. Of these, 14 patients (41%) had no cholestasis (group 0), 11 patients (32%, group 1) developed mild and 9 patients (27%, group 2) severe cholestasis. Patients in group 2 had a more complicated disease course indicated by significantly longer ICU stay (median 51 days, IQR 25‐86.5) than the other groups (group 0: median 9.5 days, IQR 3.8‐18.3, P = .001; and group 1: median 16 days, IQR 8‐30, P < .05 respectively). Four patients in group 2 developed SSC compared to none in the influenza A cohort. The available histopathological findings suggest an ischaemic damage to the perihilar bile ducts.ConclusionsThe development of SSC represents an important complication of critically ill COVID‐19 patients and needs to be considered in the diagnostic work up in prolonged cholestasis. The occurrence of SSC is of interest in the ongoing pandemic since it is associated with considerable morbidity and mortality.

Intraoperative digital single-operator cholangioscopy - extra-anatomical biliary access and insights into severe sclerosing cholangitis in a pediatric patient.

A 3-year-old boy with Downʼs syndrome, born prematurely with atrial septal defect type II and stenosis of the pulmonary artery, presented with hepatopathy and fibrosis. Magnetic resonance imaging (MRI) showed diffuse dilations of the biliary system, with large cystic changes suspicious for Caroli syndrome ([Fig. 1]). Endoscopic retrograde cholangiography (ERC) presented signs of secondary sclerosing cholangitis of the right liver lobe with missing contrast enhancement of the left biliary system ([Fig. 2]). Because there was no communication of the left liver lobe with the central biliary system, the patient underwent surgery to drain the left liver lobe by peripheral hepaticojejunostomy. Access to the biliary system was gained by the open biliary system of segment II and intraoperative cholangioscopy was performed using the newly developed, short digital system (SpyGlass Discover; Boston Scientific Corp., Marlborough, Massachusetts, USA) ([Video 1], [Fig. 3]). Intraoperative cholangioscopy revealed cystic dilated bile ducts, partly filled with biliary casts in accordance with the MRI imaging ([Fig. 4]). These findings further supported the diagnosis of systemic sclerosis due to severe postnatal respiratory insufficiency and circulatory problems.

Case report: Secondary sclerosing cholangitis induced by lapatinib and vinorelbine in a metastasis breast cancer patient

Secondary sclerosing cholangitis (SSC) is a rare cholestatic liver disease that may have a severe clinical course. A 61‐year‐old woman with a history of metastasis breast cancer was admitted to our hospital for the second cycle of chemotherapy with lapatinib and vinorelbine. The patient had no reports of elevated liver function tests (LFTs) in the previous multiple chemotherapies or history of liver disease. However, the admission laboratory results showed severe cholestatic liver injury with the possibility of SSC by magnetic resonance cholangiopancreatography. Although chemotherapy was discontinued and patient was treated with hepatoprotective drugs, the LFTs did not improve and liver biopsy indicated mild injury of intrahepatic bile duct epithelium and hepatocyte. We added ursodeoxycholic acid and prednisolone to protect the liver, and laboratory data showed a response. To prevent the progression, lapatinib and vinorelbine were reintroduced and transient increases in alanine aminotransferase and γ‐glutamyl transpeptidase were observed. With no evidence of viral or autoimmune liver disease, SSC induced by lapatinib and vinorelbine was diagnosed. This is the first case report of tyrosine kinase inhibitors and vinorelbine induced SSC and clinicians should be aware of the possibility of it. More case reports about this adverse drug reaction are needed to delineate optimal management.

The Gut-Liver Axis in Cholestatic Liver Diseases.

The gut-liver axis describes the physiological interplay between the gut and the liver and has important implications for the maintenance of health. Disruptions of this equilibrium are an important factor in the evolution and progression of many liver diseases. The composition of the gut microbiome, the gut barrier, bacterial translocation, and bile acid metabolism are the key features of this cycle. Chronic cholestatic liver diseases include primary sclerosing cholangitis, the generic term secondary sclerosing cholangitis implying the disease secondary sclerosing cholangitis in critically ill patients and primary biliary cirrhosis. Pathophysiology of these diseases is not fully understood but seems to be multifactorial. Knowledge about the alterations of the gut-liver axis influencing the pathogenesis and the outcome of these diseases has considerably increased. Therefore, this review aims to describe the function of the healthy gut-liver axis and to sum up the pathological changes in these cholestatic liver diseases. The review compromises the actual level of knowledge about the gut microbiome (including the mycobiome and the virome), the gut barrier and the consequences of increased gut permeability, the effects of bacterial translocation, and the influence of bile acid composition and pool size in chronic cholestatic liver diseases. Furthermore, therapeutic implications and future scientific objectives are outlined.

Post–Covid-19 Cholangiopathy—A New Indication for Liver Transplantation: A Case Report

Liver injury is one of the nonpulmonary manifestations described in coronavirus disease 2019 (COVID-19). Post–COVID-19 cholangiopathy is a special entity of liver injury that has been suggested as a variant of secondary sclerosing cholangitis in critically ill patients (SSC-CIP). In the general population, the outcome of SSC-CIP has been reported to be poor without orthotopic liver transplantation (OLT). However, the role of OLT for post–COVID-19 cholangiopathy is unknown. We present a case report of a 47-year-old man who recovered from acute respiratory distress syndrome from COVID-19 and subsequently developed end-stage liver disease from post–COVID-19 cholangiopathy. The patient underwent OLT and is doing well with normal liver tests for 7 months. To our knowledge, this is the first case report of a patient who underwent successful liver transplantation for post–COVID-19 cholangiopathy.

Meta-analysis and systematic review of liver transplantation as an ultimate treatment option for secondary sclerosing cholangitis.

IntroductionSecondary sclerosing cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation, and progressive destruction of the biliary tree, which leads to biliary cirrhosis. In recent years, the development of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) has increasingly been perceived as a separate disease entity.AimTo perform a systematic review and meta-analysis of secondary sclerosing cholangitis and ischaemic cholangiopathy in post organ transplant patients and intensive care unit (ICU).Material and methodsA comprehensive search strategy using the PubMed, Biosis, and EMBASE databases was designed to retrieve relevant clinical data from the published literature up to 2020. Demographic characteristics, laboratory, transplantation, mortality rate, and follow-up data undergoing liver transplantation were extracted from the inclusion studies. We used DerSimonian–Laird random-effects meta-analysis. Analysis was carried out using R statistical software version 4.02.ResultsA total of 862 patients with SSC-CIP were extracted from 16 studies. Eighteen studies were searched for the meta-analysis, out of which 16 studies were eligible for the meta-analysis and 2 were excluded. A proportion meta-analysis was performed on liver transplant patients with SSC-CIP and on mortality rate. Significant results were found (Prop = 0.30, 95% CI: 0.12–0.49, p < 0.01), with high heterogeneity among the studies (I2 = 98%, p < 0.01) and (Prop = 0.45; 95% CI: 0.35–0.56 with I2 = 80, p < 0.01), respectively. No indication of publication bias, as confirmed by the funnel plot and the risk of bias in the included studies, shows that the study reporting is adequate to judge that no major or minor sources of bias are likely to influence results.ConclusionsThe systematic review and meta-analysis show that liver transplantation is a valid option for patients with SSC-CIP, with excellent long-term outcome and improvement of quality of life.

Short-Term Efficacy of Bezafibrate for Cholestatic Liver Disease–Related Pruritus

Pruritus is a common complication of cholestatic liver disease such as primary and secondary sclerosing cholangitis (PSC and SSC) and primary biliary

S1385 First Case of Risperidone-Associated Secondary Sclerosing Cholangitis

INTRODUCTION: Secondary sclerosing cholangitis is a rare form of sclerosing cholangitis, with cholangiography and biopsy findings that are similar to primary sclerosing cholangitis. Early recognition of secondary causes is important to delay disease progression. There has not been any report that identified antipsychotics as a potential cause of secondary sclerosing cholangitis. Herein, we present a unique case of risperidone-associated secondary sclerosing cholangitis. CASE DESCRIPTION/METHODS: A 72-year-old Caucasian female, who had been treated with risperidone for catatonic schizophrenia for six months, presented to the hospital with altered mental status and fever. Pertinent laboratory findings upon arrival were leukocytosis (white blood cell count - 11.5 × 109/L), bacteriuria, pyuria, and transaminitis (alanine aminotransferase - 597 units/L, aspartate aminotransferase - 467 units/L, gamma-glutamyl transferase - 775 units/L, and alkaline phosphatase - 535 units/L). Ultrasound of the abdomen did not show ductal dilation. Risperidone was held. The patient was treated with antibiotics for a urinary tract infection, with resolution of fever and leukocytosis. As transaminitis improved, risperidone was restarted on day 4; however, transaminitis subsequently worsened. Further work-up was negative for viral hepatitis A/B/C and CMV/EBV infection. Liver core biopsy revealed periductal inflammation with onion skinning-like pattern (Figure 1–3), consistent with secondary sclerosing cholangitis. Transaminitis improved following discontinuation of risperidone. Subsequent endoscopic retrograde cholangiopancreatography revealed three stones in the lower common bile duct, with moderate, diffuse duct dilation. DISCUSSION: Risperidone and other antipsychotics have been reported to induce cholestatic hepatitis. Previous case reports have suggested an association of risperidone with drug-induced hepatitis, as evidenced by transaminitis and inflammatory liver parenchymal changes. Our case is the first to suggest a link between risperidone and secondary sclerosing cholangitis. The fibrotic changes of biliary duct, as well as the temporal relationship between risperidone intake and elevation of hepatic enzymes, are suggestive of this diagnosis. The findings of multiple ductal stones and ductal dilation likely represent the chronic inflammatory process.Figure 1.: Hematoxylin & eosin stain demonstrates the characteristic onion-skinning changes in periductal area.Figure 2.: Trichrome stain reveals high collagen content (blue) in the onion-skinning pattern, predominantly in the periductal area, consistent with active fibroblastic proliferation.Figure 3.: Inflammatory cells infiltrate hepatic parenchyma, consistent with non-specific immune response seen in hepatitis.

S1394 A Rare Form of Secondary Sclerosing Cholangitis Seen in Critically Ill Patients

INTRODUCTION: Secondary sclerosing cholangitis (SSC) is caused by biliary obstruction and damage to the biliary tree often with progression to cirrhosis. There is a rare entity of SSC following resolution of critical illness without preexisting biliary obstruction called “secondary sclerosing cholangitis in critically ill patients” (SSC-CIP). We present a case of SSC-CIP in a patient with respiratory failure from influenza A. CASE DESCRIPTION/METHODS: A 44-year-old man with no preexisting hepatobiliary disease developed respiratory failure requiring intubation secondary to Influenza A. He required pronation as well as veno-venous extracorporeal membrane oxygenation (VV-ECMO). Two months after his respiratory failure resolved he was discharged. Shortly thereafter he returned with sepsis manifest as fever, leukocytosis and an elevated alkaline phosphatase to 1323 IU/L. MRCP demonstrated multifocal hepatic abscesses and irregular appearing right intrahepatic bile ducts with strong enhancement, suspicious for cholangitis (Figure 1). ERCP revealed irregularity and saccular dilation of the right intrahepatic ducts with viscous bile, brown stones, and debris. The extrahepatic common bile duct appeared normal. The endoscopic findings in this clinical context were consistent with SSC-CIP. DISCUSSION: While secondary sclerosing cholangitis most commonly presents with biliary obstruction, SSC-CIP has a unique presentation with hepatic abscesses in the absence of biliary obstruction following resolution of critical illness. Although rare, the majority of cases in the literature develop in patients recently recovered from ARDS secondary to influenza A. Although the pathophysiology underlying SSC-CIP has not been definitively elucidated, it has been postulated that biliary ischemia of the intrahepatic bile ducts supplied by the hepatosplanchnic plexus is the dominant cause of disease development. Given its novelty, it has been suggested that newer therapies in critical care medicine may contribute to SSC-CIP, specifically both lung protective mechanical ventilation for ARDS which includes high PEEP and low tidal volumes as well as prone positioning. These interventions increase intra-abdominal pressure and can lead to decreased hepatosplanchnic blood flow and subsequent biliary ischemia. With the advent of COVID19 we expect to see more patients affected by this entity, and patient care will be enhanced by physician awareness of SSC-CIP.Figure 1.: MRCP showing multifocal intrahepatic biliary ductal dilatation with diffuse irregularity.

S2531 A Rare Cause of Cholestasis: Secondary Sclerosing Cholangitis in a Critically Ill Patient

INTRODUCTION: Secondary sclerosing cholangitis in critically ill patient (SSC-CIP) is a newly diagnosed entity of severe biliary disease which can rapidly progress to liver cirrhosis. The mechanism leading to this form of cholangitis with stricture formation and complete obliteration of bile ducts is unknown (1). CASE DESCRIPTION/METHODS: 47 year old woman with history of roux en y gastric bypass was admitted for status epilepticus, pneumonia and shock requiring vasopressors and ventilator support for over a month in ICU. Patient’s LFTs at discharge were normal. Patient was readmitted few weeks later with a seizure and found to have abnormal LFTs (ALP 2500, GGT 4000, direct bilirubin 3.2, ALT 80, and AST 75, lipase 80) which persisted over the course of next ten months. The negative work up included HAV, HBV, HCV, HIV, ANA, AMA, ASMA, Immunoglobulins, Anti LKM, ANCA, Anti SP 100, Anti GP 210, ceruloplasmin, alpha 1 anti-trypsin, TTG IgG, TSH, AFP, CA19-9 and leukemia/lymphoma markers. Her ultrasound and CT scan was unremarkable. Patient was unable to get a MRCP due to a vagal nerve stimulator and ERCP was not done due to difficult anatomy. She underwent a IR guided cholangiogram; subtle stricturing and mild dilation of left intrahepatic ducts seen, followed by a liver biopsy. Biopsy showed large bile duct obstruction with secondary sclerosing cholangitis due to ischemic cholangiopathy with advanced biliary fibrosis. Patient was started on ursodiol with improvement of LFTs however she got readmitted few days later with hemorrhagic shock due to delayed bleed from the liver biopsy site with sepsis and multiorgan failure resulting in death. DISCUSSION: SSC-CIP can be caused by events such as prolonged hypotension and respiratory insufficiency that lead to severe irreversible injury of ducts (2). The trigger event of ischemic cholangoipathy in our patient was likely her initial critical illness (hypotension and respiratory failure) resulting in cholestasis. This variant SSC-CIP is a relatively new disease that is an under recognized cause of cholestasis and should be suspected as a differential in patients with new cholestasis after a critical illness.

A135 HEPATOLITHIASIS AND CHOLANGIOPATHY IN PEDIATRIC CYSTIC FIBROSIS: A CASE REPORT

Abstract Background Liver disease has emerged as an important cause of cystic fibrosis (CF)-associated morbidity due to recent advancements in management and a resultant increase in life expectancy. Although focal biliary cirrhosis remains the pathognomonic finding in CF, other hepatic manifestations exist but are not as well described, particularly in the pediatric population. Aims Present two rare manifestations of pediatric CF-associated liver disease. Methods In this report, we present a pediatric CF patient with chronic abdominal pain who was found to have hepatolithiasis, cholangitis and secondary sclerosing cholangitis. Results A 12-year-old boy with CF was seen for abdominal pain with functional impairment. He had prior evidence of calculi in the common bile duct (CBD) and right intrahepatic (IH) duct, and had undergone endoscopic retrograde cholangiopancreatography (ERCP) twice with sphincterotomy once. Magnetic resonance cholangiopancreatography (MRCP) at presentation showed dilated CBD and bilateral IH ducts with calculi, evidence of acute cholecystitis and early cholangitis. He subsequently underwent a third ERCP with stone retrieval, stent placement and a laparoscopic cholecystectomy. Ursodiol dose was optimized and antibiotics were initiated. Follow-up MRCP showed improvement in CBD and IH duct dilation; the stent was removed. One month later, a fifth ERCP was performed for worsening symptoms associated with a distal filling defect in the left IH duct. Sludge was removed from the CBD and another stent was placed. A sixth ERCP with stent removal showed no stones. Interval MRCP showed resolution of IH calculi, a CBD filling defect suggestive of sludge, and thickening and enhancement of IH bile ducts with focal areas of alternating narrowing and dilation. He was maintained on antibiotics, and his symptoms persisted over the following months despite resolution of sludge, peri-portal edema and duct-wall enhancement. There was a background of increased IH ductal dilation with “beaded appearance”. Interval imaging has demonstrated persistence of these findings with recurrence of hepatolithiasis in both IH ducts, despite improved symptoms. Conclusions This case describes two rare manifestations of pediatric CF-associated liver disease: hepatolithiasis and secondary sclerosing cholangitis. While CFTR dysfunction alone predisposes to gallstone formation, the patient’s clinical course supports a theory for the development of hepatolithiasis resembling that of recurrent pyogenic cholangitis, whereby bacteriobilia from chronic or recurrent infection may promote lithogenesis in the presence of inflammatory bile duct damage. In this patient, secondary sclerosing cholangitis may have resulted from cycles of cholangitis and hepatolithiasis; alternatively, it may represent under-recognized cholangiopathy in the pediatric CF population. Funding Agencies None

Secondary sclerosing cholangitis from percutaneous transhepatic biliary drainage in a patient with gallbladder cancer after surgery: A case report

Secondary sclerosing cholangitis (SSC) is a chronic cholestatic liver disease characterized by fibrosis and stricture of the bile ducts. SSC in association with multiple factors such as spontaneous choledochoduodenal fistula and metastatic gallbladder cancer has rarely been reported. However, to the best of our knowledge, reports of SSC after percutaneous transhepatic biliary drainage (PTBD), especially in cases with diffuse calcification of the bile duct walls, have not been reported. We report a case of SSC from PTBD in a patient with gallbladder cancer after surgery. The patient underwent external percutaneous biliary drainage for malignant bile duct obstruction after cholecystectomy. Repeated exchanges were performed at the first and the sixth month after PTBD using an internal and external drainage catheter. Two months after the third catheter exchange, findings of laboratory and imaging examinations were suggestive of SSC. The liver function tests of the patient were suggestive of cholestasis. Multidetector computed tomography showed diffuse calcification of the bile duct walls. Cholangiography showed intrahepatic biliary stenosis or dilatation.

An Interprofessional Approach in Caring for a Patient on Maintenance Hemodialysis with COVID-19 in Toronto, Canada: An Educational Case Report.

Rationale:Hemodialysis patients are at significant risk from COVID-19 due to their frequent interaction with the health care system and medical comorbidities. We followed up the trajectory of the first COVID-19–positive maintenance hemodialysis patient at Sunnybrook Health Sciences Centre in Toronto. We present the lessons learned and changes in practices that occurred to prevent an outbreak in our center.Presenting concerns of the patient:The patient, a 66-year-old woman on in-center hemodialysis, initially presented with a 2-day history of a productive cough. She subsequently developed a fever, was placed on contact and droplet isolation, and admitted to hospital.Diagnoses:On March 13, 2020, the patient tested positive for COVID-19. Within the next 48 hours, she developed hypoxia and acute respiratory distress syndrome as a complication of her illness requiring an extended critical care stay. This extended critical care stay resulted in critical illness–associated secondary sclerosing cholangitis.Interventions:An interprofessional team was established, performing rapid Plan-Do-Study-Act quality improvement cycles to improve screening practices and promote the safety of patients and staff in the hemodialysis unit.Outcomes:We present here the lessons learned, the changes to our screening protocols, and the clinical course of our first in-center hemodialysis patient with SARS-CoV-2.Teaching points:Regular review of the infection screening processes is paramount in preventing outbreaks of COVID-19, particularly in hemodialysis units. Hospital admission should be arranged if a patient exhibits any clinical signs of hemodynamic compromise or hypoxia. Early education for health care practitioners caring for patients with COVID-19 and refresher information regarding personal protective equipment helped promote the safety of staff and prevent health care–associated outbreaks.

2337 Secondary Sclerosing Cholangitis and Nodular Regenerative Hyperplasia Caused by Hereditary Spherocytosis

INTRODUCTION: Hereditary spherocytosis is a hemolytic anemia that leads to premature cell destruction due to red cell membrane defect. It causes release of unconjugated bilirubin, and subsequent bilirubin gallstone formation occurs. Chronic recurrent choledocholithiasis can cause secondary sclerosing cholangitis (SSC). Hemolysis can lead to chronic vascular injury and portal vein thrombosis, resulting in Nodular Regenerative Hyperplasia (NRH). Here, we report a case of HS causing SSC with concurrent NRH. CASE DESCRIPTION/METHODS: A 37-year-old female with a history of hereditary spherocytosis with thrombocytosis presented to the office for abnormal liver function tests (LFTs). History of splenectomy and cholecystectomy in 2002. Patient was noted to have abnormal LFTs in 2009 (Figure 1). Her LFTs were routinely monitored until 2016 when she presented with epigastric pain, jaundice, and abnormal LFTs. Viral and autoimmune hepatitis serologies were negative. Iron studies were normal. Right upper quadrant (RUQ) ultrasound (US) showed mildly dilated intrahepatic ducts. Magnetic resonance cholangiopancreatography(MRCP) was performed which showed a questionable stricture in the proximal CBD and mild dilatation of the central intrahepatic duct. ERCP showed normal caliber CBD with multiple filling defects. A biliary sphincterotomy was performed and the stone was removed. ERCP performed in 2016 and 2018 revealed multiple stones in the biliary ductal system, which were removed and the CBD was dilated. Liver biopsy showed mild portal and lobular inflammation with mild portal fibrosis and changes suggestive of NRH. DISCUSSION: HS caused the patient's recurrent choledocholithiasis and contributed to SSC. Pathologically, SSC causes inflammation, obliterative fibrosis, stricture formation, and destruction of the biliary tree, which leads to biliary cirrhosis. Although often reversible, outcomes may not be as favorable if it is not recognized quickly, particularly in critical illness induced SSC. NRH is a liver disease characterized by many hyperplastic nodules without separation by fibrotic tissue. It is associated with any systemic disease that causes vascular injury, in this case our patient’s HS. Pathology in our case revealed only “mild” changes suggestive of NRH, so the patient hasn’t yet developed portal hypertension. There are no cases in literature that document HS as a factor contributing to either of these disease states. Early suspicion and liver biopsy are necessary for prompt diagnosis and improved outcomes.

Hepatic dysfunction in patients with cardiogenic shock

Cardiogenic shock is alife-threatening condition that is frequently associated with acute hepatic dysfunction. Due to low cardiac output resulting in end-organ hypoperfusion and hypoxia, different types of liver dysfunction can develop, such as hypoxic hepatitis or acute liver failure. Avery serious and late sequela is the secondary sclerosing cholangitis in critically patients. Clinical management of acute hepatic dysfunction involves the stabilization of cardiac output to improve hepatic perfusion and the optimization of liver oxygenation. However, despite maximum efforts in supportive treatment, the outcome of patients with cardiogenic shock and concomitant hepatic dysfunction remains poor.