PBC and PSC: Back to Basics
|
|
- Gillian Terry
- 6 years ago
- Views:
Transcription
1 Disclosure No financial interest or other relationship with the manufacturer(s) of the product(s) or provider(s) of the service(s) that will be discussed in this presentation. PBC and PSC: Back to Basics Outline In chronic cholestatic liver disease, liver biopsy plays a supporting role Case-based discussion Clinical information, including laboratory findings and radiographic features, must be integrated for diagnosis Case 1 clinical history 54 year old woman, asymptomatic, incidentally discovered to have elevated alkaline phosphatase (ALP) and positive antimitochondrial antibodies (AMA) Liver biopsy performed 1
2 What finding would you like to see on biopsy so you can diagnosis it as PBC? A. Portal and lobular inflammation B. Infiltrate with easily identified plasma cells C. Florid duct lesion D. Ductular reaction E. Ductopenia F. Periportal copper deposition G. CK7 positive periportal hepatocytes H. Nodular regenerative hyperplasia I. Biliary pattern of cirrhosis AASLD and EASL recommendations 2 of 3 required criteria for PBC diagnosis Presence of antimitochondrial antibodies (AMA) Alkaline phosphatase (ALP) at least 1.5 times upper limit of normal for >24 weeks Histologic evidence of PBC, specifically nonsuppurative cholangitis and duct injury Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev Apr-May;13(4-5): Lindor KD, et al. Primary biliary cirrhosis. Hepatology 2009;50: EASL. Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51: AASLD and EASL recommendations 2 of 3 required criteria for PBC diagnosis Presence of antimitochondrial antibodies (AMA) Alkaline phosphatase (ALP) at least 1.5 times upper limit of normal for >24 weeks Histologic evidence of PBC, specifically nonsuppurative cholangitis and duct injury Antimitochondrial antibodies (AMA) Major autoantigen target by PBC patient sera M2 antigen fraction E2 subunits of Pyruvate dehydrogenase complex (PDC-E2) Branched chain 2-oxo-acid dehydrogenase complex (BCOADC- E2) 2-oxo glutarate dehyrogenase complex (OGDC-E2) E1α and E3 binding protein of PDC Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev Apr-May;13(4-5): Lindor KD, et al. Primary biliary cirrhosis. Hepatology 2009;50: EASL. Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51: Muratori L, et al. Antimitochondrial Antibodies and Other Antibodies in Primary Biliary Cirrhosis: Diagnostic and Prognostic Value. Clin Liver Dis 2008 May;12(2):
3 AMA test methodology Indirect immunofluorescence assay (IFA) Positive titer is 1:40 Sensitivity 70-90% Enzyme-linked immunosorbent assay (EIA) AMA-M2 antibodies is most frequently used First generation utilized PDC-E2 10% of PBC pts only react to BCOADC-E2 and/or OGDC-E2 MIT3 or BPO expresses BCOADC-E2, PDC-E2 and OGDC- E2 epitopes Sensitivity 94% Venn diagram of serum reactivity of PBC patients Dähnrich C, et al. New ELISA for detecting primary biliary cirrhosis-specific antimitochondrial antibodies. Clin Chem May;55(5): Selmi C, Bowlus CL, Gershwin ME, Coppel RL. Primary biliary cirrhosis. Lancet May 7;377(9777): Dähnrich C, et al. New ELISA for detecting primary biliary cirrhosis-specific antimitochondrial antibodies. Clin Chem May;55(5): AMA positive also in <1% of healthy adults Possibly early preclinical stage Patients with other diseases 10-20% of AIH <10% of HCV <1% of steatohepatitis Rare reports associated with DILI AASLD and EASL recommendations 2 of 3 required criteria for PBC diagnosis Presence of antimitochondrial antibodies (AMA) Alkaline phosphatase (ALP) at least 1.5 times upper limit of normal for >24 weeks Histologic evidence of PBC, specifically nonsuppurative cholangitis and duct injury ~20% of pts have other autoimmune diseases Infection mycobacteria and E.coli (cross reactivity) O'Brien C, et al. Long-term follow-up of antimitochondrial antibody-positive autoimmune hepatitis. Hepatology Aug;48(2): Ravi S, et al. Autoimmune Markers Do Not Impact Clinical Presentation or Natural History of Steatohepatitis-Related Liver Disease. Dig Dis Sci Dec;60(12): Chantran Y, et al. Autoantibodies in primary biliary cirrhosis: antimitochondrial autoantibodies. Clin Res Hepatol Gastroenterol Sep;37(4): Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev Apr-May;13(4-5): Lindor KD, et al. Primary biliary cirrhosis. Hepatology 2009;50: EASL. Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51:
4 7829 new requests of autoantibody screen 29 pts had Incidental finding of +AMA by IFA, titer 1:40 Normal serum bilirubin, ALP, and transaminase Diagnostic liver histology of PBC at presentation in 40% of cases Liver histology was diagnostic of or compatible with PBC in 24 pts and normal in only 2 pts (7%) 10-year follow-up of cohort Conclusion Patients with AMA but no other signs or symptoms of PBC seem to have slow disease progression AASLD and EASL recommendations 2 of 3 required criteria for PBC diagnosis Presence of antimitochondrial antibodies (AMA) Alkaline phosphatase (ALP) at least 1.5 times upper limit of normal for >24 weeks Histologic evidence of PBC, specifically nonsuppurative cholangitis and duct injury Florid duct lesion aka chronic nonsuppurative cholangitis Lymphohistiocytic inflammation centered on an interlobular bile duct (<100µm) and is associated with bile duct epithelial damage and epithelial reactive changes. Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev Apr-May;13(4-5): Lindor KD, et al. Primary biliary cirrhosis. Hepatology 2009;50: EASL. Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51:
5 The name PBC First described Xanthomatous biliary cirrhosis Xanthoma seen in 15-50% of pts and first finding in <1% Primary biliary cirrhosis Expected survival no different from the general population in 2 of 3 patients diagnosed and treated with UDCA and only a minority will ever develop cirrhosis Acronym PBC Primary biliary cholangitis 2015 Name change approved by EASL, AASLD, AGA Addison T, Gull W. On a certain affection of the skin vitiligoidea a plana, b tuberosa. Guy s Hospital Reports 1851;7: Hanot V. Étude sur une forme de cirrhose hypertrophique du foie [cirrhose hypertrophique avec ictere chronique]. Paris: JBBaillère,1876. Macmahon HE, Thannhauser SJ. Xanthomatous biliary cirrhosis; a clinical syndrome. Ann Intern Med Jan;30(1): Corpechot C, et al. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology Feb;128(2): Sherlock S. Primary billiary cirrhosis (chronic intrahepatic obstructive jaundice). Gastroenterology Nov;37: Rubin E, Schaffner F, Popper H. Primary biliary cirrhosis. Chronic non-suppurative destructive cholangitis. Am J Pathol Mar;46: Beuers U, et al. Changing nomenclature for PBC: From 'cirrhosis' to 'cholangitis'. Hepatology Nov;62(5): Case 1 pathologic diagnosis Feature consistent with primary biliary cirrhosis/primary biliary cholangitis Meets all three criteria Case 1 treatment Ursodeoxycholic acid (UDCA) FDA approved drug for PBC since 1998 Well-tolerated Better prognosis when given at early stage 5
6 Diagnostic criteria Emphasize Patchy distribution and range of histologic features Stains supportive of chronic biliary disease Case 2 not much clinical history 55 year old woman with elevated LFT Liver biopsy performed Case 2 pathologic diagnosis Portal and lobular inflammation or mild nonspecific changes. Do you then Go on to next case? 2. Send it to UCSF GI/Hepatobiliary Pathology Consult Service? 3. Inquire about laboratory results? 6
7 Diagnostic approach to cholestasis in adult patients We called for laboratory test results 2 Elevated ALP (264) Mildly elevated AST (62) and ALT (51) Positive AMA (M ) EASL. Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51: Case 2 clinical history and labs Scenario A ALP elevated and AMA positive Case 2 clinical history and labs Scenario B If ALP elevated and AMA negative Diagnosis: Portal and lobular inflammation; see comment. Comment: Describe features, state this can represent an early manifestation of PBC Other characteristic clinical features PBC-specific ANAs (Sp100 and gp210) Elevated serum IgM Hypercholesterolemia/Xanthomas Sicca syndrome Pruritus Fatigue Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev Apr-May;13(4-5):
8 Diagnostic approach to cholestasis in adult patients Case 2 clinical history and labs Scenario B If ALP elevated and AMA negative 4 3 Diagnosis: Portal and lobular inflammation; see comment. Comment: Describe features, consider repeat AMA test or use of a more sensitive assay; but could represent an early manifestation of AMAnegative PBC (autoimmune cholangitis), if DILI and primary and secondary sclerosing cholangitis has been excluded EASL. Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009;51: Bowlus CL, Gershwin ME. The diagnosis of primary biliary cirrhosis. Autoimmun Rev Apr-May;13(4-5): Range of histologic features in PBC Non-specific features including portal tracts with no alterations to mild portal and lobular inflammation Duct injury +/- ductular reaction Patchy ductocentric lymphocytic cholangitis to florid duct lesion Duct loss / ductopenia Accompanied by portal-based fibrosis to biliary cirrhosis 8
9 Portal tract alteration Duct injury Epithelial cells stratified, hyperplastic, attenuated, or ectatic Cytology with swollen, vacuolated, or eosinophilic cytoplasm and pyknotic nuclei Inflammation Lymphoid aggregate, ductocentric, intraepithelial or completely replace duct, and can have interface activity Composed of epithelioid or foamy histiocytes and lymphocytes, plasma cells, and/or eosinophils Florid duct lesion? 9
10 Florid duct lesion Other autoantibodies in PBC Antinuclear antibodies (ANA) Positive in ~50% of PBC pts irrespective of AMA status Positive in up to 85% of AMA-negative PBC pts Rim-like pattern with g210 and p62 Multiple nuclear dots with Sp100 Smooth muscle antibody (SMA) Positive in 2/3 of PBC pts 10
11 Supportive of chronic biliary disease Periportal hepatocytes Have cytoplasmic copper deposition on copper stain Immunoreactive for CK7 Case 3 clinical history 46 year old woman with elevated ALP (238) Normal AST (33), ALT (53), Tbili (0.3) 10 months later found to have positive AMA (titer 1:640) Liver biopsy performed Copper (rubeanic acid) stain 11
12 40x CK7 Case 3 pathologic diagnosis Liver, core needle biopsy: Features consistent with primary biliary cirrhosis/primary biliary cholangitis; see comment. Comment: Portal and lobular inflammation Duct injury and loss (confirmed on CK7) Cholestatic effect (focal periportal copper and CK7 positive hepatocytes) Early stage Staging systems Stage Ludwig Scheuer 1 2 Portal inflammation (duct damage can be present) Periportal inflammation Florid duct lesion Ductular reaction (periportal fibrosis often present) 3 Bridging fibrosis (ductopenia usually present) 4 Biliary cirrhosis Not useful in predicting prognosis due to Heterogeneity in the distribution of features Combined information about inflammation and fibrosis 12
13 Bridging fibrosis and ductular reaction 13
14 Case 4 clinical history 50 year old woman Negative HCV, HBsAb, and AMA Positive ANA Elevated IgM (622) and IgG (4051) Mildly elevated ALT (94), AST (93), total bili (13.4) Case 4 pathologic diagnosis Cirrhosis with ductopenia and cholate stasis suggestive of AMA-negative PBC; see comment. If negative imaging studies aka autoimmune cholangiopathy or autoimmune cholangitis Indistinguishable from PBC pts who are AMA-positive O'Brien C, et al. Long-term follow-up of antimitochondrial antibody-positive autoimmune hepatitis. Hepatology Aug;48(2):
15 Describe the features seen A. Portal and lobular inflammation B. Infiltrate with easily identified plasma cells C. Florid duct lesion D. Ductular reaction E. Ductopenia F. Periportal copper deposition G. CK7 positive periportal hepatocytes H. Nodular regenerative hyperplasia I. Biliary pattern of cirrhosis Hepatologist wants to know Can the described features be seen in PBC Or is it another disease process Autoimmune hepatitis Overlap syndrome Primary sclerosing cholangitis Drug reaction (DILI) Large duct obstruction Sarcoidosis Cholangiography is gold standard for diagnosis of PSC Magnetic resonance cholangiopathy (MRCP) Method of choice because it is non-invasive Sensitivity of 86% and specificity of 94% Endoscopic retrograde cholangiopancreatography (ERCP) Optimal for early changes when duct have shallow ulcers without stricture Distribution Frequent involves intra- and extra-hepatic bile ducts, 87% Intrahepatic ducts alone, 11-25% Extrahepatic ducts alone, 2% Case 5 clinical history 45 year old man with ulcerative colitis and primary sclerosing cholangitis who undergoes liver transplant 15
16 Cirrhotic with duct ectasia or loss Hilar periductal inflammation Hilar duct dialation or ulcerated with bile extravasation and inflammation Range of histologic features in PSC* Non-specific features including portal tracts with no alterations to mild portal inflammation Ductular reaction with duct injury Can have lobular inflammation Accompanied by portal-based fibrosis Periductal fibrosis or fibro-obliterative duct lesion Biliary cirrhosis *Histologic features overlaps PBC 16
17 Age (years) Comparison between PSC vs PBC Clinical, immunologic and imaging PSC Wide age range, most between 25-40; can occur in children PBC Typically >50, does not occur in children Gender Male preponderance (2-3:1) Female preponderance (9-14-:1) Disease association Serum immunoglobulins Autoantibodies Cholangiography Idiopathic inflammatory bowel disease, especially ulcerative colitis No significant increase No specific antibodies; AMA rare Stricturing and beading of large ducts; normal in smallduct PSC Other autoimmune diseases such as Sjogren syndrome, and autoimmune thyroid disease Elevated IgM AMA, ANA-centrometric type Normal extrahepatic and large intrahepatic ducts Modified Table 26.4 Practical Hepatic Pathology: A Diagnostic Approach, 1 st Edition A Volume in the Pattern Recognition Series, Expert Consult 2011 by Saunders, an imprint of Elsevier Inc. Philadelphia, PA Small duct PSC Frequency of 11-17% Involves smaller caliber bile ducts Liver biopsy required for diagnosis Bile duct injury Comparison between PSC vs PBC Pathology PSC Affects large intra- and extra-hepatic ducts PBC Affects small interlobular bile ducts Florid duct lesion Rare Characteristic Portal inflammation Tends to be less Typically prominent Ductular reaction Typically prominent Typically less prominent Obliterative fibrosis of medium and large ducts Characteristics but often not sampled on biopsy Absent Periportal copper* Present Present *Nonspecific feature of chronic cholestasis Fibro-obliterative duct lesion Modified Table 26.4 Practical Hepatic Pathology: A Diagnostic Approach, 1 st Edition A Volume in the Pattern Recognition Series, Expert Consult 2011 by Saunders, an imprint of Elsevier Inc. Philadelphia, PA CK7 17
18 Septal/interlobular NOT A SPECIFIC FEATURE ducts OF with periductal fibrosis PRIMARY SCLEROSING CHOLANGITIS Case 6 clinical history 42 year old man with ulcerative colitis and elevated alkaline phosphatase Negative autoimmune panel and hyperglobulinemia (IgG 1827) Normal MRCP Liver biopsy was performed Variability in distribution Portal zones 18
19 CK 7 immunohistochemical stain Copper (rubeanic acid) stain Case 6 pathologic diagnosis Features consistent with chronic biliary tract disease; see comment. Comment: Describe features (portal information with duct loss/ductopenia and septal and focal bridging fibrosis; chronic biliary process supported by stains). Potential etiology small duct PSC vs AMA-negative PBC. Chronic biliary tract diseases Elevated ALP No visible cholestasis on biopsy Small duct PSC Early stage PSC and PBC 19
20 Case 7 clinical history 71 year old man with progressive jaundice Infectious complication following enolymphatic sac enhancement surgery for vertigo. Elevated ALP (245) and Tbili (12) Is this PSC? Case 7 pathologic diagnosis Cholestasis, portal inflammation and ductular reaction; see comment. Differential includes large duct obstruction or drug reaction LiverTox database DILI due to Augmentin toxicity 20
21 Secondary sclerosing cholangitis Secondary sclerosing cholangitis Schistosomiasis Infection (AIDS cholangiopathy) Vascular (ischemic cholangiopathy/ischemic stricturing) Toxic (intra-arterial chemotherapy such as floxuridine) Congenital (Caroli disease) Infiltrative (cholangiocarcinoma) Hepatolithiasis Trauma (surgical biliary trauma) Immunologic (IgG4-related sclerosing cholangitis) IgG4-related sclerosing cholangitis * Case 8 clinical history * Most have associated autoimmune pancreatitis type 1 Isolated cholangitis (10%) Two forms Mass-forming hilar lesion mimics cholangiocarcinoma PSC-like intra- and extra-hepatic biliary strictures mimics PSC Steroid-responsive strictures 55 year old man with obstructive jaundice Imaging: common bile duct stricture, suggestive of cholangiocarcinoma Cytology and biopsy inconclusive Resection specimen 21
22 Courtesy of Dr. Sanjay Kakar Courtesy of Dr. Sanjay Kakar IgG4 plasma cells >10 HPF Courtesy of Dr. Sanjay Kakar Courtesy of Dr. Sanjay Kakar 22
23 Requests for IgG4 stain IgG-related sclerosing cholangitis One of the criterion is >10 IgG4+ plasma cells/hpf Also observed in other diseases PSC 23% (23/98 explanted liver large bile duct) 22% of pts had elevated serum IgG4 AIH Rare reports (1/9 and 2/60 liver biopsies) Case 9 clinical history 62 year old man with autoimmune pancreatitis Elevated AST (47) and ALP (267) ANA and AMA negative Elevated IgG4 (226) Zhang L, et al. IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis. Am J Surg Pathol Jan;34(1): Koyabu M, et al. Analysis of regulatory T cells and IgG4-positive plasma cells among patients of IgG4-related sclerosing cholangitis and autoimmune liver diseases. J Gastroenterol Jul;45(7): Umemura T, et al. Clinical significance of immunoglobulin G4-associated autoimmune hepatitis. J Gastroenterol Jan;46 Suppl 1:
24 Case 9 pathologic diagnosis Features of biliary obstruction; see comment. Comment: No IgG4+ plasma cells, does not exclude IgG4-related disease. ERCP demonstrated pancreatic duct and intrahepatic biliary stricturing felt to be consistent with either PSC or IgG4 cholangiopathy Treated with steroids with normalization of liver enzymes and decreased IgG4 Conclusions Chronic biliary tract diseases: PBC and PSC Similarities Overlapping histologic features Patchy distribution that can be non-specific early in the disease process Distinctive Clinical, laboratory and radiographic findings is essential PBC progresses slowly to cirrhosis and UDCA is available Periductal fibrosis observed in primary or secondary sclerosing cholangitis Nakanuma staging system Score Fibrosis Bile duct loss Deposition of orceinpositive granules No portal fibrosis or No bile duct loss No deposition fibrosis limited to portal tracts Portal fibrosis with Loss in <1/3 of Deposition of granules in periportal fibrosis or portal tracts periportal hepatocytes in incomplete septal <1/3 of portal tracts fibrosis Bridging fibrosis with variable lobular disarray Cirrhosis with regenerative nodules and extensive fibrosis Loss in 1/3-2/3 of portal tracts Loss in >2/3 of portal tracts Deposition of granules in periportal hepatocytes in 1/3-2/3 of portal tracts Deposition of granules in periportal hepatocytes in >2/3 of portal tracts 24
25 Stage Scheuer Ludwig Nakanuma 1 Florid duct lesion Portal Sum of 0 points (no progression) 2 Ductular reaction Periportal Sum of 1-3 points (mild progression) 3 Bridging fibrosis Bridging fibrosis Sum of 4-6 points (moderate progression) 4 Biliary cirrhosis Biliary cirrhosis Sum of 7-9 points (advance progression) Stage 4 (total score 9/9) Fibrosis 9/3, bile duct loss 3/3, orcein (copper) deposition 3/3 Hepatitis activity 0/3, cholangitis activity 0/3 HA 0 (no activity) adf Nakanuma grading system Grade Hepatitis Cholangitis Grade No interface and no or No cholangitis +/- mild minimal lobular hepatitis duct epithelial damage HA 1 (mild activity) HA 2 (moderate activity) HA 3 (marked activity) Focal interface hepatitis and mild to moderate lobular hepatitis Between HA1 and HA3 Marked interface hepatitis, moderate to marked lobular hepatitis or zonal necrosis Issue with interobserver variability At least one focus of evident chronic cholangitis Two more foci of evident chronic cholangitis At least of focus of chronic non-suppurative destructive cholangitis (granulomatous cholangitis) CA 0 (no activity) CA 1 (mild activity) CA 2 (moderate activity) CA 3 (marked activity) Granuloma Diagnosis? Case Quick look at APeX revealed history of sarcoidosis Granuloma Case 25
26 Martin JA. Endoscopic Management of Primary Sclerosing Cholangitis: State of Art. Am J Gastroenterol. 2007;102,S Fibro-obliterative duct lesion Trichrome and copper stains CK7 26
27 Diagnosis? More information 66 year old man History of common bile duct stones and biliary strictures Liver showed focal portal inflammation, minimal ductular reaction, and rare periductal fibrosis Pathologic diagnosis: Features of biliary tract disease; see comment. Etiology includes early stage PSC, chronic obstruction (hepatolithiasis or recurrent pyogenic cholangitis). Case 8 clinical history 52 year old man with BMI of 30 and diabetes mellitus, and elevated alkaline phosphatase Negative ANA, ASMA, and AMA 27
28 What is the diagnosis/diagnoses? MRCP beaded Case 8 diagnosis Request for IgG4 stain Features supportive of early stage primary sclerosing cholangitis. Can cholestasis occur? Superimposed steatohepatits, possibly alcoholic steatohepatitis 28
29 PSC and IgG4 related sclerosing cholangitis IgG4 stain: 23% of PSC have >5 IgG4+ plasma cells per HPF Absent: Portal-based inflammatory nodules: lymphocytes, plasma cells, eosinophils, fibroblasts Obliterative phlebitis not seen Accentuation of inflammation around bile ducts uncommon Pseudotumor-like hilar mass 29
Autoimmune Liver Diseases
2nd Pannonia Congress of pathology Hepato-biliary pathology Autoimmune Liver Diseases Vera Ferlan Marolt Institute of pathology, Medical faculty, University of Ljubljana Slovenia Siofok, Hungary, May 2012
More informationBiliary tract diseases of the liver
Biliary tract diseases of the liver Digestive Diseases Course Bucharest 2016 Rob Goldin r.goldin@imperial.ac.uk How important are biliary tract diseases? Hepatology 2011 53(5):1608-17 Approximately 16%
More informationChronic Biliary Disease. Dr Susan Davies & Dr Bill Griffiths
Chronic Biliary Disease Dr Susan Davies & Dr Bill Griffiths Chronic Biliary Disease Terminology is confusing with pathologists and hepatologists using the same language BUT with different meanings. Chronic
More informationACCME/Disclosures. The Overlap Syndromes: Do They Exist? Key Points and Questions 4/6/2016. Hans Popper Hepatopathology Society
ACCME/Disclosures The USCAP requires that anyone in a position to influence or control the content of CME disclose any relevant financial relationship WITH COMMERCIAL INTERESTS which they or their spouse/partner
More informationACCME/Disclosures. PBC and PSC Revisited 4/6/2016. Primary Biliary Cirrhosis Cholangitis (PBC)
ACCME/Disclosures The USCAP requires that anyone in a position to influence or control the content of CME disclose any relevant financial relationship WITH COMMERCIAL INTERESTS which they or their spouse/partner
More informationPrimary Sclerosing Cholangitis and Cholestatic liver diseases. Ahsan M Bhatti MD, FACP Bhatti Gastroenterology Consultants
Primary Sclerosing Cholangitis and Cholestatic liver diseases Ahsan M Bhatti MD, FACP Bhatti Gastroenterology Consultants I have nothing to disclose Educational Objectives What is PSC? Understand the cholestatic
More informationUpdate on Autoimmune Liver Disease. Role of Liver Biopsy in Autoimmune Hepatitis, PBC and PSC
Update on Autoimmune Liver Disease Role of Liver Biopsy in Autoimmune Hepatitis, PBC and PSC Stefan Hübscher, School of Cancer Sciences, University of Birmingham Dept of Cellular Pathology, Queen Elizabeth
More informationSarah Landes October 23, 2014
Sarah Landes October 23, 2014 A T-cell mediated inflammatory destruction of intralobular bile ducts progressively leading to cholestasis and cirrhosis 9:1 F to M ratio Mostly diagnosed between 30-60 years
More informationAutoimmune Hepatobiliary Diseases PROF. DR. SABEHA ALBAYATI CABM,FRCP
Autoimmune Hepatobiliary Diseases PROF. DR. SABEHA ALBAYATI CABM,FRCP Autoimmune hepatobiliary diseases The liver is an important target for immunemediated injury. Three disease phenotypes are recognized:
More informationHépatopathies auto-immunes
16 ème Journée d'automne Lausanne, le 19 octobre 2017 Hépatopathies auto-immunes Nurullah Aslan et Darius Moradpour Service de Gastroentérologie et d'hépatologie Centre Hospitalier Universitaire Vaudois
More informationPBC/AIH variant/ overlap syndrome vs PBC with hepatitic features?
22 November 2018 BD-IAP UK-LPG Liver Update PBC/AIH variant/ overlap syndrome vs PBC with hepatitic features? in a UDCA non-responder Dina G. Tiniakos Institute of Cellular Medicine, Faculty of Medical
More informationChronic Cholestatic Liver Diseases
Chronic Cholestatic Liver Diseases - EASL Clinical Practice Guidelines - Rome, 8 October 2010 Ulrich Beuers Department of Gastroenterology and Hepatology Tytgat Institute of Liver and Intestinal Research
More informationLIVER SPECIALTY CONFERENCE USCAP Maha Guindi, M.D. Clinical Professor of Pathology Cedars-Sinai Medical Center Los Angeles, CA
LIVER SPECIALTY CONFERENCE USCAP 2016 Maha Guindi, M.D. Clinical Professor of Pathology Cedars-Sinai Medical Center Los Angeles, CA Nothing to disclose Case History 47-year-old male, long standing ileal
More information21/07/2017. Update on Autoimmune Biliary Disease. Changing Role of Liver Biopsy in PBC and PSC. Primary Biliary Cirrhosis Cholangitis
Primary Biliary Cirrhosis Cholangitis Update on Autoimmune Biliary Disease Changing Change in Nomenclature for PBC : From Cirrhosis to Cholangitis (EASL Panel, Beuers et al J Hepatol Nov 2015, Gut Nov
More informationNoncalculous Biliary Disease Dean Abramson, M.D. Gastroenterologists, P.C. Cedar Rapids. Cholestasis
Noncalculous Biliary Disease Dean Abramson, M.D. Gastroenterologists, P.C. Cedar Rapids Cholestasis Biochemical hallmark Impaired bile flow from liver to small intestine Alkaline phosphatase is primary
More informationLIVER TRANSPLANTATION FOR OVERLAP SYNDROMES OF AUTOIMMUNE LIVER DISEASES
LIVER TRANSPLANTATION FOR OVERLAP SYNDROMES OF AUTOIMMUNE LIVER DISEASES No conflict of interest Objectives Introduction Methods Results Conclusions Objectives Introduction Methods Results Conclusions
More informationSlide 7 demonstrates acute pericholangitisis with neutrophils around proliferating bile ducts.
Many of the histologic images and the tables are from MacSween s Pathology of the Liver (5 th Edition). Other images were used from an online source called PathPedia.com. A few images from other sources
More informationPITFALLS IN THE DIAGNOSIS OF MEDICAL LIVER DISEASE WITH TWO CONCURRENT ETIOLOGIES I HAVE NOTHING TO DISCLOSE CURRENT ISSUES IN ANATOMIC PATHOLOGY 2017
CURRENT ISSUES IN ANATOMIC PATHOLOGY 2017 I HAVE NOTHING TO DISCLOSE Linda Ferrell PITFALLS IN THE DIAGNOSIS OF MEDICAL LIVER DISEASE WITH TWO CONCURRENT ETIOLOGIES Linda Ferrell, MD, UCSF THE PROBLEM
More informationKey Points: Autoimmune Liver Disease: Update for Pathologists from the Hepatologist s Perspective. Jenny Heathcote, MD. University of Toronto
Autoimmune Liver Disease: Update for Pathologists from the Hepatologist s Perspective Jenny Heathcote, MD University of Toronto Key Points: AILD comprise autoimmune hepatitis, primary biliary cirrhosis
More informationPatologia sistematica V Gastroenterologia Prof. Stefano Fiorucci Autoimmune liver diseases
Patologia sistematica V Gastroenterologia Prof. Stefano Fiorucci Autoimmune liver diseases Harrison s Principles of Internal Medicine 18-19 Ed. 2012 e seguenti Chronic hepatitis classification by cause
More informationFat, ballooning, plasma cells and a +ANA. Yikes! USCAP 2016 Evening Specialty Conference Cynthia Guy
Fat, ballooning, plasma cells and a +ANA. Yikes! USCAP 2016 Evening Specialty Conference Cynthia Guy Goals Share an interesting case Important because it highlights a common problem that we re likely to
More informationBasic patterns of liver damage what information can a liver biopsy provide and what clinical information does the pathologist need?
Basic patterns of liver damage what information can a liver biopsy provide and what clinical information does the pathologist need? Rob Goldin r.goldin@imperial.ac.uk Fatty liver disease Is there fatty
More informationDiagnosis and Management of PBC
Diagnosis and Management of PBC Cynthia Levy, MD, FAASLD University of Miami Miller School of Medicine Miami, Florida 1 Primary Biliary Cholangitis (PBC) Chronic cholestatic liver disease Autoimmune in
More informationOverview of PSC Making the Diagnosis
Overview of PSC Making the Diagnosis Tamar Taddei, MD Assistant Professor of Medicine Yale University School of Medicine Overview Definition Epidemiology Diagnosis Modes of presentation Associated diseases
More informationBasic patterns of liver damage what information can a liver biopsy provide and what clinical information does the pathologist need?
Basic patterns of liver damage what information can a liver biopsy provide and what clinical information does the pathologist need? Rob Goldin r.goldin@imperial.ac.uk @robdgol FATTY LIVER DISEASE Brunt
More informationPrimary Biliary Cholangitis
Primary Biliary Cholangitis PBC Foundation (UK) Ltd 6 Hill Street Edinburgh EH2 3JZ Tel: +44 (0) 131 556 6811 info@pbcfoundation.org.uk www.pbcfoundation.org.uk PBC for Healthcare Practitioners Introduction
More informationTratamiento endoscópico de la CEP. En quien como y cuando?
Tratamiento endoscópico de la CEP. En quien como y cuando? Andrés Cárdenas, MD, MMSc, PhD, AGAF, FAASLD GI / Liver Unit, Hospital Clinic Institut de Malalties Digestives i Metaboliques University of Barcelona
More informationChronic Cholestatic Liver Disease
1 Cholestasis - Definition Biochemical Chronic Cholestatic Liver Disease Defect in bile secretory mechanisms leading to accumulation in blood of substances normally excreted in bile. In some conditions
More informationCASE 1 Plasma Cell Infiltrates: Significance in post liver transplantation and in chronic liver disease
CASE 1 Plasma Cell Infiltrates: Significance in post liver transplantation and in chronic liver disease Maria Isabel Fiel, M.D. The Mount Sinai Medical Center New York, New York Case A 57 yo man, 7 months
More informationValue of Autoantibody Analysis in the Differential Diagnosis of Chronic Cholestatic Liver Disease
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2009;7:1355 1360 Value of Autoantibody Analysis in the Differential Diagnosis of Chronic Cholestatic Liver Disease PIOTR MILKIEWICZ,*, HAYMAN BUWANESWARAN,* CATALINA
More informationHangzhou, 15 March Ulrich Beuers Department of Gastroenterology and Hepatology Academic Medical Center University of Amsterdam
Clinical Aspects of Primary Biliary Cirrhosis Hangzhou, 15 March 2008 Ulrich Beuers Department of Gastroenterology and Hepatology Academic Medical Center University of Amsterdam Epidemiology of Primary
More informationACG Clinical Guideline: Primary Sclerosing Cholangitis
ACG Clinical Guideline: Primary Sclerosing Cholangitis Keith D. Lindor, MD, FACG 1, Kris V. Kowdley, MD, FACG 2, and M. Edwyn Harrison, MD 3 1 College of Health Solutions, Arizona State University, Phoenix,
More informationMedical Policy An independent licensee of the Blue Cross Blue Shield Association
Ocaliva (obeticholic acid) Page 1 of 6 Medical Policy An independent licensee of the Blue Cross Blue Shield Association Title: Ocaliva (obeticholic acid) Prime Therapeutics will review Prior Authorization
More informationOverview of PSC Jayant A. Talwalkar, MD, MPH Associate Professor of Medicine Mayo Clinic Rochester, MN
Overview of PSC Jayant A. Talwalkar, MD, MPH Associate Professor of Medicine Mayo Clinic Rochester, MN 2012 Annual Conference PSC Partners Seeking a Cure May 5, 2012 Primary Sclerosing Cholangitis Multifocal
More informationdoi: /s
doi: 10.1007/s10620-013-2734-6 Harada - 1 - Clinicopathological significance of serum fractalkine in primary biliary cirrhosis Kenichi Harada 1), Yuko Kakuda 1), Minoru Nakamura 2), Shinji Shimoda 3),
More informationExtrahepatic Biliary Obstruction. Ductal Diseases: Stones Tumors. Acute Injury: Viral Hepatitis Toxin (APAP/Etoh) Reye s Shock.
Extrahepatic Biliary Obstruction Stones Tumors Ductal Diseases: Ductal Diseases: Primary Biliary Primary Biliary Cirrhosis Cirrhosis Sclerosing Cholangitis Sclerosing Cholangitis Acute Injury: Viral Hepatitis
More informationRisk stratification in PBC
Risk stratification in PBC Christophe Corpechot Reference Center for Inflammatory Biliary Diseases Saint-Antoine hospital, Paris, France What is currently known (background) PBC : chronic, progressive
More informationBritish Liver Transplant Group Pathology meeting September Leeds cases
British Liver Transplant Group Pathology meeting September 2014 Leeds cases Leeds Case 1 Male 61 years Liver transplant for HCV cirrhosis with HCC in January 2014. Now raised ALT and bilirubin,? acute
More informationHistometric and Serial Section Observations of the Intrahepatic Bile Ducts in Primary Biliary Cirrhosis
GASTROENTEROLOGY 761326-1332, 1979 Histometric and Serial Section Observations of the Intrahepatic Bile Ducts in Primary Biliary Cirrhosis YASUNI NAKANUMA and GOROKU OHTA Second Department of Pathology,
More informationCurrent Concepts in the Management and Treatment of PBC & PSC
Current Concepts in the Management and Treatment of PBC & PSC Michael A Heneghan, MD, MMedSc, FRCPI. Institute of Liver Studies, King s College Hospital, London A family affair? Central vein Hepatocytes
More informationDiagnosing Autoimmune Hepatitis in Children: Is the International Autoimmune Hepatitis Group Scoring System Useful?
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2004;2:935 940 Diagnosing Autoimmune Hepatitis in Children: Is the International Autoimmune Hepatitis Group Scoring System Useful? REGAN L. EBBESON* and RICHARD
More informationAASLD: Boston Rob Goldin
AASLD: Boston 2014 Rob Goldin r.goldin@imperial.ac.uk Autoimmune hepatitis 31 Does genuine acute autoimmune hepatitis have a better prognosis? 32 Patients with Autoimmune Hepatitis and Advanced Disease
More informationColangitis Esclerosante Primaria: Manejo Clínico y Endoscópico
Colangitis Esclerosante Primaria: Manejo Clínico y Endoscópico Andrés Cárdenas, MD, MMSc, PhD, AGAF, FAASLD GI / Liver Unit, Hospital Clinic Institut de Malalties Digestives i Metaboliques Associate Professor
More informationHwajeong Lee, MD, Robert T. Stapp, DO, Adrian H. Ormsby, MD, and Veena V. Shah, MD
Anatomic Pathology / Overlap Syndrome The Usefulness of IgG and IgM Immunostaining of Periportal Inflammatory Cells (Plasma Cells and Lymphocytes) for the Distinction of Autoimmune Hepatitis and Primary
More informationAutoimmune Hepatitis: Histopathology
REVIEW Autoimmune Hepatitis: Histopathology Stephen A. Geller M.D.*, Autoimmune hepatitis (AIH), a chronic hepatic necroinflammatory disorder, occurs mostly in women. AIH is characterized by prominent
More informationPediatric Primary Sclerosing Cholangitis and Potential Therapies
Pediatric Primary Sclerosing Cholangitis and Potential Therapies Philip Rosenthal, M.D. Professor of Pediatrics & Surgery University of California, San Francisco DISCLOSURE I have the following financial
More informationHow to Approach a Medical Liver Biopsy. 9 th Bryan Warren School of Pathology Pancreatic and Liver Pathology. Sarajevo, 6 th -7 th November 2015
1 A Brief Introduction to the Liver Sessions 9 th Bryan Warren School of Pathology Pancreatic and Liver Pathology Sarajevo, 6 th -7 th November 2015 Stefan Hübscher, Institute of Immunology & Immunotherapy,
More informationPediatric PSC A children s tale
Pediatric PSC A children s tale September 8 th PSC Partners seeking a cure Tamir Miloh Assistant Professor Pediatric Hepatology Mount Sinai Hospital, NY Incidence Primary Sclerosing Cholangitis (PSC) ;
More informationDILI PATHOLOGY. PHILIP KAYE November 2017 BSG Pathology Winter Meeting
DILI PATHOLOGY PHILIP KAYE November 2017 BSG Pathology Winter Meeting General Mechanisms Role of Liver Biopsy Outline Kleiner Categories Pathology! Differentials Severity Finally Drugs/Toxins may cause
More informationCASE 01 LA Path Slide Seminar 13 March, 08. Deepti Dhall, MD Department of Pathology and Laboratory Medicine Cedars-Sinai Medical Center
CASE 01 LA Path Slide Seminar 13 March, 08 Deepti Dhall, MD Department of Pathology and Laboratory Medicine Cedars-Sinai Medical Center Clinical History 60 year old male presented with obstructive jaundice
More informationAMR in Liver Transplantation: Incidence
AMR in Liver Transplantation: Incidence Primary AMR 1/3 to 1/2 of ABO-incompatible transplants Uncommon with ABO-compatible transplant Secondary AMR Unknown incidence: rarely tested Why is AMR uncommon
More informationPrognosis of untreated Primary Sclerosing Cholangitis (PSC) Erik Christensen Copenhagen, Denmark
Prognosis of untreated Primary Sclerosing Cholangitis (PSC) Erik Christensen Copenhagen, Denmark Study of Prognosis of PSC Difficulties: Disease is rare The duration of the course of disease may be very
More informationManagement of autoimmune hepatitis. Pierre-Emmanuel RAUTOU Inserm U970, Paris Service d hépatologie, Hôpital Beaujon, Clichy, France
Management of autoimmune hepatitis Pierre-Emmanuel RAUTOU Inserm U970, PARCC@HEGP, Paris Service d hépatologie, Hôpital Beaujon, Clichy, France Case 1 52 year-old woman, referred for liver blood tests
More informationA Review of Liver Function Tests. James Gray Gastroenterology Vancouver
A Review of Liver Function Tests James Gray Gastroenterology Vancouver Copyright 2017 by Sea Courses Inc. All rights reserved. No part of this document may be reproduced, copied, stored, or transmitted
More informationTwo Cases of Primary Sclerosing Cholangitis Overlapping with Autoimmune Hepatitis in Adults
CASE REPORT Two Cases of Primary Sclerosing Cholangitis Overlapping with Autoimmune Hepatitis in Adults Go Igarashi 1, Tetsu Endo 1, Kenichiro Mikami 1, Naoya Sawada 1,RyuSatake 1, Rie Ohta 1, Juichi Sakamoto
More informationAutoimmune hepatitis
Autoimmune hepatitis: Autoimmune hepatitis a spectrum within a spectrum Alastair Burt Professor of Pathology and Dean of Clinical Medicine Newcastle University Spectrum of autoimmune liver disease Autoimmune
More informationLiver Pathology Symposium - medical livers
Liver Pathology Symposium - medical livers London, April 13 th 2018 Dr Susan E Davies, Addenbrooke s Hospital, Case 1 SED 65/F Diabetic. Raised ALT, USS fatty. Fibroscan? cirrhosis Reticulin (untoned),
More informationMorning Report Presentation. Sarah Hughes, MD January 11, 2005
Morning Report Presentation Sarah Hughes, MD January 11, 2005 Primary Biliary Cirrhosis! PBC is a chronic, progressive, cholestatic liver disease of unknown cause that usually affects middle-aged women
More informationI have no disclosures relevant to this presentation LIVER TESTS: WHAT IS INCLUDED? LIVER TESTS: HOW TO UTILIZE THEM OBJECTIVES
LIVER TESTS: HOW TO UTILIZE THEM I have no disclosures relevant to this presentation José Franco, MD Professor of Medicine, Surgery and Pediatrics Medical College of Wisconsin OBJECTIVES Differentiate
More informationCholestatic Liver Diseases: Update on Diagnosis and Management. Cholestatic Liver Diseases: Location of Injury Determines Phenotype
Cholestatic Liver Diseases: Update on Diagnosis and Management R. Todd Stravitz, M.D. Hume-Lee Transplant Center Section of Hepatology Virginia Commonwealth University Cholestatic Liver Diseases: Location
More informationTHE BRITISH SOCIETY OF GASTROENTEROLOGY/UK-PBC PRIMARY BILIARY CHOLANGITIS TREATMENT AND MANAGEMENT GUIDELINES
THE BRITISH SOCIETY OF GASTROENTEROLOGY/UK-PBC PRIMARY BILIARY CHOLANGITIS TREATMENT AND MANAGEMENT GUIDELINES AUTHORS: HIRSCHFIELD GM, DYSON JK, ALEXANDER GJ, CHAPMAN M, COLLIER J, HUBSCHER S, PATANWALA
More informationIdiopathic adulthood ductopenia manifesting as jaundice in a young male
Idiopathic adulthood ductopenia manifesting as jaundice in a young male Deepak Jain*,1, H. K. Aggarwal 1, Avinash Rao 1, Shaveta Dahiya 1, Promil Jain 2 1 Department of Medicine, Pt. B.D. Sharma University
More informationProgram Disclosure. This activity is supported by an educational grant from Intercept Pharmaceuticals.
Program Disclosure This activity has been planned and implemented in accordance with the Essential Areas and Policies of the Accreditation Council for Continuing Medical Education through the joint providership
More informationA Case of Autoimmune Hepatitis with Antimitochondrial Antibody and No Detectable Antinuclear Antibody
FFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFF St. Marianna Med. J. Case Report Vol. 32, pp. 33 38, 2004 FFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFFF A Case
More informationSteatosis/Steatohepatitis
Prepared by Kurt Schaberg Introduction to Medical Liver Steatosis/Steatohepatitis Alcoholic Hepatitis Hepatocyte injury and inflammation resulting from chronic alcohol consumption AST/ALT ratio typically
More informationDomenico ALVARO, MD Sapienza, University of Rome, Italy. Congresso Nazionale della Società Italiana di GastroReumatologia, Roma, 25 Giugno 2015.
Epatite autoimmune e sindrome da "overlap Domenico ALVARO, MD Sapienza, University of Rome, Italy Congresso Nazionale della Società Italiana di GastroReumatologia, Roma, 25 Giugno 2015. AUTOIMMUNE HEPATITIS
More informationIgG4-Negative Autoimmune Pancreatitis with Sclerosing Cholangitis and Colitis: Possible Association with Primary Sclerosing Cholangitis?
CASE REPORT IgG4-Negative Autoimmune Pancreatitis with Sclerosing Cholangitis and Colitis: Possible Association with Primary Sclerosing Cholangitis? Keita Saeki 1, Shigenari Hozawa 1, Naoteru Miyata 1,
More informationCirculation T. Manchester July 5 th 2006
Circulation T Manchester July 5 th 2006 Case 242 8 year old female with polycystic disease Section from explanted liver. Case 242 Case 242 Case 242 Case 242 Case 242 Case 242 31 Congenital hepatic fibrosis
More informationApproach to the Patient with Liver Disease
Approach to the Patient with Liver Disease Diagnosis of liver disease Careful history taking Physical examination Laboratory tests Radiologic examination and imaging studies Liver biopsy Liver diseases
More informationNottingham Patterns of liver fibrosis and their clinical significance
Nottingham 2006 Patterns of liver fibrosis and their clinical significance Alastair D Burt Professor of Pathology and Dean of Clinical Medicine University of Newcastle upon Tyne Collapse of reticulin
More informationOcaliva (obeticholic acid tablets)
Ocaliva (obeticholic acid tablets) Policy Number: 5.01.619 Last Review: 11/2018 Origination: 11/2016 Next Review: 11/2019 Policy Blue Cross and Blue Shield of Kansas City (Blue KC) will provide coverage
More informationResident, PGY1 David Geffen School of Medicine at UCLA. Los Angeles Society of Pathology Resident and Fellow Symposium 2013
Resident, PGY1 David Geffen School of Medicine at UCLA Los Angeles Society of Pathology Resident and Fellow Symposium 2013 85 year old female with past medical history including paroxysmal atrial fibrillation,
More informationPrimary Biliary Cirrhosis at Hospital Kuala Lumpur: A Study of 11 Cases Seen Between
Primary Biliary Cirrhosis at Hospital Kuala Lumpur: A Study of 11 Cases Seen Between 1992 and 1999 R Kananathan, MRCPI, R L Suresh, MRCP, I Merican, FRCP, Department of Medicine, Hospital Kuala Lumpur,
More informationDrug-induced liver injury
Drug-induced liver injury Vincent Wong MBChB(Hons), MD, FRCP, FHKCP, FHKAM Professor, Department of Medicine and Therapeutics Director, Cheng Suen Man Shook Foundation Centre for Hepatitis Studies Deputy
More informationACP-BSG meeting The liver in systemic inflammatory disorders. Dr Adrian C Bateman Southampton University Hospitals NHS Trust
ACP-BSG meeting 10.12.09 The liver in systemic inflammatory disorders Dr Adrian C Bateman Southampton University Hospitals NHS Trust Wide range of diseases General inflammatory disorders Connective tissue
More informationA Retrospective Single-Center Review of Primary Sclerosing Cholangitis in Children
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2009;7:239 245 A Retrospective Single-Center Review of Primary Sclerosing Cholangitis in Children TAMIR MILOH,* RONEN ARNON,* BENJAMIN SHNEIDER, FREDERICK SUCHY,*
More informationDigestive and Liver Disease
Digestive and Liver Disease 42 (2010) 585 592 Contents lists available at ScienceDirect Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld Liver, Pancreas and Biliary Tract Intrahepatic
More informationPrimary Sclerosing Cholangitis. Bibleclass Felix Brunner
Primary Sclerosing Cholangitis Bibleclass 29.04.2015 Felix Brunner Overview Epidemiology Pathogenesis Clinical Features, Genetics, Immunology Diagnosing PSC Treatment Medications, Transplantation Cancer-Risk
More informationNavigating the Biliary Tract with CT & MR: An Imaging Approach to Bile Duct Obstruction
Navigating the Biliary Tract with CT & MR: An Imaging Approach to Bile Duct Obstruction Ann S. Fulcher, MD Medical College of Virginia Virginia Commonwealth University Richmond, Virginia Objectives To
More informationA Young Man with Non-alcoholic Steatohepatitis and Serum Anti-mitochondrial Antibody Positivity: A Case Report
doi: 10.2169/internalmedicine.0405-17 Intern Med Advance Publication http://internmed.jp CASE REPORT A Young Man with Non-alcoholic Steatohepatitis and Serum Anti-mitochondrial Antibody Positivity: A Case
More informationPrimary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study
LINDOR ET AL. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases Keith D. Lindor 1, Christopher L. Bowlus 2, James Boyer 3, Cynthia Levy 4,
More informationA Spectrum of Histopathologic Findings in Autoimmune Liver Disease
Anatomic Pathology / HISTOPATHOLOGIC FINDINGS IN AUTOIMMUNE LIVER DISEASE A Spectrum of Histopathologic Findings in Autoimmune Liver Disease Luigi M. Terracciano, MD, 1 Roselyn A. Patzina, MD, 1 Frank
More informationDiagnostic utility of IgG and IgM immunohistochemistry in autoimmune liver disease
Online Submissions: http://www.wjgnet.com/1007-9327office wjg@wjgnet.com doi:10.3748/wjg.v16.i4.453 World J Gastroenterol 2010 January 28; 16(4): 453-457 ISSN 1007-9327 (print) 2010 Baishideng. All rights
More informationNational Liver EQA Scheme. Open meeting, Glasgow March 24th 2004
National Liver EQA Scheme Open meeting, Glasgow March 24th 2004 Participants meeting SOP8 Case discussion Are we quorate All please sign attendance sheet There were 20 EQA participants and 7 non-members
More informationPrimary sclerosing cholangitis: Evaluation with MR cholangiopancreatography (MRCP)
The Egyptian Journal of Radiology and Nuclear Medicine (2011) 42, 351 356 Egyptian Society of Radiology and Nuclear Medicine The Egyptian Journal of Radiology and Nuclear Medicine www.elsevier.com/locate/ejrnm
More informationCase n 1 ( B 92 / 4208 ) Case n 2 ( B 00 / 8249 ) Case n 3 ( B 98 / 8352 )
Slide Seminar Case n 1 ( B 92 / 4208 ) 16 month-old girl. HBV serology +. Clinic in favour of chronic hepatitis. 4 portal triads! classification limited Viral B chronic hepatitis Mild activity (Fig. 1
More informationIn 1993, the International Autoimmune Hepatitis Group
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2012;10:417 421 Validation and Modification of Simplified Diagnostic Criteria for Autoimmune Hepatitis in Children ELIZABETH MILETI,* PHILIP ROSENTHAL,*, and MARION
More informationRole of Liver Biopsy. Role of Liver Biopsy 9/3/2009. Liver Biopsies in Viral Hepatitis: Beyond Grading and Staging
Liver Biopsies in Viral Hepatitis: Beyond Grading and Staging for further reference: Liver biopsy assessment in chronic viral hepatitis: a personal, practical approach Neil Theise, MD. Depts of Pathology
More informationJaundice. Agnieszka Dobrowolska- Zachwieja, MD, PhD
Jaundice Agnieszka Dobrowolska- Zachwieja, MD, PhD Jaundice definition Jaundice, as in the French jaune, refers to the yellow discoloration of the skin. It arises from the abnormal accumulation of bilirubin
More informationAutoimmune Hepatitis in Clinical Practice
1 Autoimmune Hepatitis in Clinical Practice Atif Zaman, MD MPH Professor of Medicine Senior Associate Dean for Clinical and Faculty Affairs School of Medicine Oregon Health & Science University Disclosure
More informationPresentation and mortality of primary biliary cirrhosis in older patients
Age and Ageing 2000; 29: 305 309 Presentation and mortality of primary biliary cirrhosis in older patients JULIA L. NEWTON 1,DAVID E. JONES 2,JANE V. METCALF 2,JAY B. PARK 2,ALISTAIR D. BURT 2, MARGARET
More informationEvaluation of the revised versus the simplified scoring system in patients with autoimmune hepatitis
EXPERIMENTAL AND THERAPEUTIC MEDICINE 7: 131-136, 2014 Evaluation of the revised versus the simplified scoring system in patients with autoimmune hepatitis YI LI, MILIN PENG and GUOZHONG GONG Institute
More informationPersonal Profile. Name: 劉 XX Gender: Female Age: 53-y/o Past history. Hepatitis B carrier
Personal Profile Name: 劉 XX Gender: Female Age: 53-y/o Past history Hepatitis B carrier Chief complaint Fever on and off for 2 days Present illness 94.10.14 Sudden onset of epigastric pain 94.10.15 Fever
More informationPrimary Biliary Cirrhosis : NOT ANY MORE!! PRIMARY BILIARY CHOLANGITIS
Primary Biliary Cirrhosis : NOT ANY MORE!! PRIMARY BILIARY CHOLANGITIS Nikolaos T. Pyrsopoulos, MD, PhD, MBA, FACP, AGAF, FAASLD Associate Professor and Chief, Division of Gastroenterology and Hepatology
More informationTREATMENT OF PRIMARY BILIARY CIRRHOSIS (PBC)
TREATMENT OF PRIMARY BILIARY CIRRHOSIS (PBC) URSO not indicated Therapy for PBC Difficulties Etiology is uncertain Therapies are based on ideas regarding pathogenesis Present medical therapies have a limited
More informationPrototypes of autoimmune hepatitis and sclerosing cholangitis in childhood
RIUNIONE MONOTEMATICA AISF 2013 Personalizzazione della Cura in Epatologia 17-19 ottobre 2013, PISA Prototypes of autoimmune hepatitis and sclerosing cholangitis in childhood La sottoscritta dichiara di
More informationMagnetic resonance cholangiography compared with endoscopic retrograde cholangiography in the diagnosis of primary sclerosing cholangitis
Original Article Magnetic resonance cholangiography compared with endoscopic retrograde cholangiography in the diagnosis of primary sclerosing cholangitis Hossein Ahrar, Mohamad Saleh Jafarpishe, Ali Hekmatnia,
More informationSerologic Markers CONVENTIONAL ANTIBODIES ANTIBODIES UNCONVENTIONAL. AIH Type I
Autoimmune Hepatitis By Thomas Frazier Objective What we need to know about AIH Diagnosis Treatment Difficulties in both Liver transplantation concerns AASLD Guidelines: Hepatology. 2010 Jun;51(6):2193-213.
More informationS1727 Beyond Elevated Liver Enzymes: Recognizing Patterns of Liver Injury
S1727 Beyond Elevated Liver Enzymes: Recognizing Patterns of Liver Injury Safia N. Salaria, MD, FCAP Mary Kay Washington, MD, PhD, FCAP Objectives Identify patterns of liver injury caused by biliary disease,
More informationMikako Nakajima 1, Hidetake Shimizu 1, Akihisa Miyazaki, Sumio Watanabe 1, Noriyuki Kitami 2, and Nobuhiro Sato 1
J Gastroenterol 1999; 34:607 612 Detection of IgA, IgM, and IgG subclasses of anti-m2 antibody by immunoblotting in autoimmune cholangitis: Is autoimmune cholangitis an early stage of primary biliary cirrhosis?
More information