Primary biliary cholangitis (cirrhosis) vs primary sclerosing cholangitis

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Primary Biliary Cirrhosis

VS

Primary Sclerosing CholangitisDone By Abdulwahab K Neyazi MBBS(Cholangitis)

Since cirrhosis occurs only in the late stage Changing the name to primary biliary cholangitis will better serve patients and the medical community worldwide 1

Content Introduction

Primary Biliary Cholangitis

Primary Sclerosing Cholangitis

Summary

References

IntroductionWhat are those ?!!

Different !! Or Same

Mixing Up

Primary Biliary Cholangitis

Cholestatic hepatic disorder of unknown etiology with autoimmune feature

T-lymphocyte-mediated attack on small intralobular bile ducts

Primary Biliary CholangitisAssociated disorders

Sicca syndrome (Sjogren) 40%Thyroid dysfunction 10 -15%CREST 5 to 15Rheumatoid arthritis 5 to 10

xerophthalmia (dry eyes), xerostomia (dry mouth), and parotid gland enlargement,(Calcinosis cutis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)Hashimoto's thyroiditis5

Epidemiology

Geographical variation Women > Men

Middle aged women (30-65 y)

Pathophysiology

Pathophysiology

Pathophysiology

Clinical Presentation

Asymptomatic

50-60 %

Clinical PresentationSymptomatic

This change is due to melanin deposition, not jaundice The cause is unknown, but similar findings occur in other types of chronic liver disease, such as hemochromatosis.12

Clinical PresentationExamination

Jaundice Itching marks

Hyperpigmentation

Clinical PresentationExamination

Xanthelasma

Xanthomata

Clinical PresentationExamination

Hepatosplenomegaly

Clinical Presentation

Diagnosis

Middle womenFatigueUnexplained itchingJaundice

Laboratory TestsLFTBilirubinALTAST Alkaline phosphatase GGT

Alka with GGT togather elevation more likely related to hepatic 18

Laboratory TestsLipid profilemild elevations (LDL and VLDL) and striking elevations of HDL

Serologic markersAntimitochondrial antibodies (AMA) 95 %

Antinuclear antibodies (ANA) 70 %

early presentationmild elevations (LDL and VLDL) and striking elevations of HDL

19

Criteria

No extrahepatic biliary obstruction

No comorbidity affecting the liver

20

CriteriaWith two of the following are present:

Alk-ph at least 1.5 times the upper limit of normal

AMA titre of 1:40 or higher

Histologic evidence of PBC

(nonsuppurative destructive cholangitis and destruction of interlobular bile ducts)While a liver biopsy is often not required to make the diagnosis of PBC, it may provide useful information with regard to staging and prognosis21

TreatmentUrsodeoxycholic acid(13 to 15mg/kgper day) (Grade 1A)

Treatment

Methotrexate

(0.25mg/kgbody weight per week orally)

Colchicine

BUT

TreatmentRandomized trials have not demonstrated a benefit of methotrexateor colchicine

Thus, we suggestAGAINST routine use of methotrexate or colchicine given alone or in combination with UDCA (Grade 2B).

TreatmentLiver Transplant

Serum bilirubin exceeding 6mg/dL(103micromol/L)

Decompensated cirrhosis with an unacceptable quality of life

TreatmentLiver Transplant

treatment-resistant ascites and spontaneous bacterial peritonitis, recurrent variceal bleeding, encephalopathy, or hepatocellular carcinoma

TreatmentLiver Transplant

Recurrence post transplantation is 30 percent after 10 years.

TreatmentEmerging drugs for thetreatmentofPrimaryBiliaryCholangitis.

Obeticholic Acid

2016 Feb 22.

Primary Sclerosing Cholangitis

Chronic liver disease characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts

Epidemiology6 0.77 per 100,000 person-years

Men > Women 2:1

Middle aged men around 40 years

Epidemiology

High association with Inflammatory Bowel Disease (70% Ulcerative colitis)

Cholangiocarcinoma 10-15%

ClassificationSmall Duct DiseaseTypical histologic feature with normal cholangiogram

Classic PSC Strictures of biliary tree can be detected by cholangiogram

Classification75 % Involve both intra and extrahepatic duct

15% intrahepatic duct only

10% Extrahepatic duct only

EtiologyImmune mediated

Infection

Genetic predispositionHLA-B8, DR3, DR2, and DR4

Environmental Factors

Pathophysiology

Figure 2.A, Intrahepatic and extrahepatic strictures in a patient with primary sclerosing cholangitis; B, corresponding ERCP cholangiogram.36

Pathophysiology

Clinical PresentationAsymptomatic 50%

Clinical PresentationSymptomatic

Weight loss

Fatigue

Acute cholangitis

DiagnosisLFT Abnormal function mainly Alk-ph

Hypergammaglobulinemia 30 %

IgM levels 40-50 %

DiagnosisSerology

p-ANCA 80%

ANA 50%

DiagnosisUltrasound

CT

MRI

DiagnosisMRCP

ERCP

Percutaneous transhepatic cholangiography (PTC)

ERCP

multiple segmental strictures typically short segmentintervening segments are of normal calibre or slightly dilated (beading) endoscopic retrograde cholangiopancreatography44

Percutaneous transhepatic cholangiography

DiagnosisLiver Biopsy

Histopathology

Staging

StagingStage 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

MRCP is preferred over (ERCP) to establish a diagnosis of PSC. (Strong recommendation, moderate quality of evidence)

American College of Gastroenterology guideline 2015

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

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

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 2015Antimitochondrial autoantibody testing can help exclude primary biliary cirrhosis.(Conditional recommendation, moderate quality of evidence)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

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

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 2015Liver 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 2015Patients 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 2015Annual 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 2015full 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

Some advocate repeating the exam every 35 years in those without prior evidence of colitis. (Weak recommendation, low quality of evidence)

American College of Gastroenterology guideline 2015

Consider screening for cholangiocarcinoma with regular cross-sectional imaging with ultrasound or MR and serial CA 19-9 every 612 months.(Conditional recommendation, very low quality of evidence)

American College of Gastroenterology guideline 2015

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

RefrencesLindor, Keith D., et al. "Ursodeoxycholic acid in the treatment of primary biliary cirrhosis."Gastroenterol