Primary biliary cholangitis (cirrhosis) vs primary sclerosing cholangitis
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Transcript of Primary biliary cholangitis (cirrhosis) vs primary sclerosing cholangitis
Primary Biliary Cirrhosis
VS
Primary Sclerosing CholangitisDone By
Abdulwahab K Neyazi MBBS
(Cholangitis)
Content O Introduction
O Primary Biliary Cholangitis
O Primary Sclerosing Cholangitis
O Summary
O References
IntroductionO What are those ?!!
O Different !! Or Same
O Mixing Up
Primary Biliary Cholangitis
O Cholestatic hepatic disorder of unknown etiology with autoimmune feature
O T-lymphocyte-mediated attack on small intralobular bile ducts
Primary Biliary Cholangitis
O Associated disorders
O Sicca syndrome (Sjogren) 40%
O Thyroid dysfunction 10 -15%
O CREST 5 to 15
O Rheumatoid arthritis 5 to 10
Epidemiology O Geographical variation O Women > Men
O Middle aged women (30-65 y)
Pathophysiology
Pathophysiology
Pathophysiology
Clinical Presentation
O Asymptomatic
50-60 %
Clinical PresentationO Symptomatic
Clinical PresentationO Examination
Jaundice Itching marks
Hyperpigmentation
Clinical PresentationO Examination
Xanthelasma
Xanthomata
Clinical PresentationO Examination
Hepatosplenomegaly
Clinical Presentation
DiagnosisO Middle womenO FatigueO Unexplained itchingO Jaundice
Laboratory TestsO LFTBilirubinALTAST Alkaline phosphatase GGT
Laboratory TestsO Lipid profilemild elevations (LDL and VLDL) and striking elevations of HDL
O Serologic markersAntimitochondrial antibodies (AMA) 95 %
Antinuclear antibodies (ANA) 70 %
Criteria
O No extrahepatic biliary obstruction
O No comorbidity affecting the liver
CriteriaO With two of the following are
present:
O Alk-ph at least 1.5 times the upper limit of normal
O AMA titre of 1:40 or higher
O Histologic evidence of PBC
TreatmentO Ursodeoxycholic acid (13 to 15 mg/kg per day) (Grade 1A)
Treatment
O Methotrexate
(0.25 mg/kg body weight per week orally)
O ColchicineBUT
TreatmentO Randomized trials have not
demonstrated a benefit of methotrexate or colchicine
O Thus, we suggest AGAINST routine use of methotrexate or colchicine given alone or in combination with UDCA (Grade 2B).
TreatmentO Liver Transplant
Serum bilirubin exceeding 6 mg/dL (103 micromol/L)
Decompensated cirrhosis with an unacceptable quality of life
TreatmentO Liver Transplant
treatment-resistant ascites and spontaneous bacterial peritonitis, recurrent variceal bleeding, encephalopathy, or hepatocellular carcinoma
TreatmentO Liver Transplant
Recurrence post transplantation is 30 percent after 10 years.
TreatmentO Emerging drugs for
the treatment of Primary Biliary Cholangitis.
Obeticholic Acid2016 Feb 22.
Primary Sclerosing Cholangitis
O Chronic liver disease characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts
EpidemiologyO 6 0.77 per 100,000 person-years
O Men > Women 2:1
O Middle aged men around 40 years
Epidemiology
O High association with Inflammatory Bowel Disease (70% Ulcerative colitis)
O Cholangiocarcinoma 10-15%
ClassificationO Small Duct DiseaseTypical histologic feature with normal cholangiogram
O Classic PSC Strictures of biliary tree can be detected by cholangiogram
ClassificationO 75 % Involve both intra and
extrahepatic duct
O 15% intrahepatic duct only
O 10% Extrahepatic duct only
EtiologyO Immune mediated
O Infection
O Genetic predispositionHLA-B8, DR3, DR2, and DR4
O Environmental Factors
Pathophysiology
Pathophysiology
Clinical PresentationO Asymptomatic 50%
Clinical PresentationO Symptomatic
Weight loss
Fatigue
Acute cholangitis
DiagnosisO LFT Abnormal function mainly Alk-ph
O Hypergammaglobulinemia 30 %
O IgM levels 40-50 %
DiagnosisO Serology
p-ANCA 80%
ANA 50%
DiagnosisO Ultrasound
O CT
O MRI
DiagnosisO MRCP
O ERCP
O Percutaneous transhepatic cholangiography (PTC)
ERCP
Percutaneous transhepatic cholangiography
DiagnosisO Liver Biopsy
Histopathology
StagingStagingStage I – Enlargement, edema, and scarring of the portal triads, and mononuclear cell infiltration and damage to isolated bile ducts.
Stage II – Expansion of portal triads with fibrosis extending into the surrounding parenchyma.
Stage III – Bridging fibrosis.
Stage IV – Cirrhosis.
American College of Gastroenterology guideline 2015
American College of Gastroenterology guideline 2015
O MRCP is preferred over (ERCP) to establish a diagnosis of PSC.
(Strong recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Liver biopsy is not necessary to make a diagnosis in patients with suspected PSC based on diagnostic cholangiographic findings.
(Conditional recommendation, low quality of evidence)
American College of Gastroenterology guideline 2015
O Liver biopsy is recommended to make a diagnosis in patients with suspected small duct PSC or to exclude other conditions such as suspected overlap with autoimmune hepatitis.
(Conditional recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Liver biopsy is recommended to make a diagnosis in patients with suspected small duct PSC or to exclude other conditions such as suspected overlap with autoimmune hepatitis.
(Conditional recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Antimitochondrial autoantibody testing can help exclude primary biliary cirrhosis.
(Conditional recommendation, moderate quality of evidence)O Patients with PSC should be tested at
least once for elevated serum immunogloblulin G4 (IgG4) levels.
(Conditional recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Ursodeoxycholic acid (UDCA) in doses >28 mg/kg/day should not be used for the management of patients with PSC.
(Strong recommendation and high quality of evidence)
American College of Gastroenterology guideline 2015
O ERCP with balloon dilatation is recommended for PSC patients with dominant stricture and pruritus, and/or cholangitis, to relieve symptoms.
(Strong recommendation, low quality of evidence)
American College of Gastroenterology guideline 2015
O Liver transplantation, when possible, is recommended over medical therapy or surgical drainage in PSC patients with decompensated cirrhosis, to prolong survival.
(Strong recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Patients should be referred for liver transplantation when their Model for End-Stage Liver Disease (MELD) score exceeds 14.
(Conditional recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Annual colon surveillance preferably with chromoendoscopy is recommended in PSC patients with colitis beginning at the time of PSC diagnosis.
(Conditional recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O full colonoscopy with biopsies is recommended in patients with PSC regardless of the presence of symptoms to assess for associated colitis at time of PSC diagnosis.
(Conditional recommendation, moderate quality of evidence)
American College of Gastroenterology guideline 2015
O Some advocate repeating the exam every 3–5 years in those without prior evidence of colitis.
(Weak recommendation, low quality of evidence)
American College of Gastroenterology guideline 2015
O Consider screening for cholangiocarcinoma with regular cross-sectional imaging with ultrasound or MR and serial CA 19-9 every 6–12 months.
(Conditional recommendation, very low quality of evidence)
American College of Gastroenterology guideline 2015
O Cholecystectomy should be performed for patients with PSC and gallbladder polyps >8 mm, to prevent the development of gallbladder adenocarcinoma.
(Conditional recommendation, very low quality of evidence)
Summary
RefrencesO Lindor, Keith D., et al. "Ursodeoxycholic acid
in the treatment of primary biliary cirrhosis." Gastroenterology 106.5 (1994): 1284-1290.
O Canadian liver foundationO American College of Gastroenterology
guideline 2015O Uptodate
Thanks
OGreetings people!Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC).. Because both have the word primary and
are associated with the hepatobiliary system, it's easy to mix em up.
Here's how I keep them straight -
The middle word is the key:
sclerOsing has an OO for onion
O looks like a bead
biliAry has an AA for autoimmune
A for antibodies
Periductal onion skin fibrosis on histology and beaded appearance (both strictures and dilation) on cholangiography is seen in primary sclerosing cholangitis.
Cholangitis and Colitis sound similar and that's how I make the Ulcerative colitis association with PSC!
Antimitochondrial antibodies are seen in primary biliary cirrhosis. It is autoimmune.
A also reminds me of the Attenuated bile duct epithelium due to destruction of interlobular bile ducts (those that course alongside the hepatic artery) by lymphocyte infiltration (Florid duct lesion).
Since autoimmune diseases are more commonly seen in females, PBC is also common in females.. So the other one, PSC, is seen in males!
Hope you all are doing awesome < 3
