Hepato-biliary: Autoimmune Flashcards
Sequence of events in autoimmune heptatits
Sequence of events: • White cells attack the liver • Chronic inflammation • Fibrosis • Necrosis
3 types of autoimmune hepatitis (and their antibodies)
AIHI: ASMAs, ANAs
AIH2: LKM-1, anti LC1 (typically children)
AIH3: soluble liver proteins liver pancreas antigen
Presenation autoimmune hepatitis
• As chronic liver disease o Palmar erythema o Hepatomegaly o Splenomegaly o Myalgia
• As acute hepatitis o Jaundice o Abdominal pain o N+V o Loss of appetite
Investigations for autoimmune hepatitis
Blood Tests: • FBC- raised ESR, WCC • LFT’s (markers of liver damage) o Bilirubin o Aminotransferases (ALT + AST)
• Immunoglobulin’s
o Raised IgG
• Antibodies
o ASMA positive
o Antinuclear factor positive
Imaging:
• Liver Biopsy
Management for autoimmune hepatitis
Medical:
• Steroids e.g. Prednisolone
• Immunosuppressant’s e.g. Azathioprine
• Ursodeoxycholic Acid
Surgical:
• Liver transplant
What does PBC stand for?
Primary biliary cirrhosis
Who does PBC typically affect>
Women, typically manifests following pregnancy
Sequence of events in PBC
GRANULOMATOUS INFLAMMATION IN INTRAHEPATIC BILE DUCTS
Bile Duct Obstruction/Cholestasis
Cirrhosis
(T cells attack bile ducts, bile leaks out causing inflammation)
Presentation of PBC
(similar to cholestasis and obstructive jaundice)
PRURITIS: With or without jaundice
Jaundice - conjugated hyperbilirubinaemia
Fatigue
Arthralgia
Xanthelasma
Investigations for PBC
LFTs - bilirubin, alkp, GGT,
Immunoglobulins - raised IgM
Antibodies - AMA (Anti-Mitochondrial Antibodies) positive
Management PBC
Steroids eg prednisolone
Immunosuppressant’s e.g. Azathioprine
Ursodeoxycholic Acid - Life long
Cholestyramine - Pruritus
Surgical:
• Liver transplant
What is PBC associated with
Sjogren’s
RA
Prognosis PBC
Asymptomatic - normal life expectancy
Symptomatic - 5 year survival
What does PSC stand for?
Primary Sclerosing Cholangitis
What is PSC associated with?
IBD - mainly UC
Sequence of events in PSC
CHRONIC INFLAMMATION IN INTRAHEPATIC (+EXTRA) BILE DUCTS
Bile Duct
Obstruction/Cholestasis
Cells become inflamed, die and harden (tightening and dilating)
Presentation PSC
May be asymptomatic - detected in UC patients with abnormal liver biochemistry
- Pruritus
- Jaundice - Conjugated hyperbilirubinaemia
- Cholangitis- fever, rigors, jaundice, abdominal pain
- Fatigue
- Weight loss
V. close to portal veins so can cause PHT - hepatosplenomegaly, cirrhosis
Investigations for PSC
LFTs - bilirubin, alkp
Immunoglobulins - raised IgM
Antibodies - pANCA (myeloperoxidase antineutrophillic cytoplasmic antibodies) positive(ANA positive)
Imaging: ERCP/MRCP diagnosis
Management of PSC
Immunosuppressant’s e.g. Methotrexate
Ursodeoxycholic Acid - May slow disease progression
Cholestyramine - Pruritus
Endoscopic - ERCP
Sphincterectomy/stent insertion
Complications of PSC
Cholangiocarcinoma
Sclerosing cholangitis seen in AIDS
