18-5: Autoimmune Hepatitis and Cholangiopathies Flashcards
What are the HLA associations in autoimmune hepatitis
Caucasians DR3
Japanese DR4
South America DRB1
What are the four features on which the diagnosis of autoimmune hepatitis is based?
- exclusion of other etiologies
- Autoantibodies
- elevation of serum IgG
- supportive histologic findings on liver biopsy
which subtype of autoimmune hepatitis is associated with adults and children
Type 1 adults
Type 2 children
what is the predominant treatment for autoimmune hepatitis
prednisone w,w/o azathioprine
Liver transplantation may be necessary for cirrhotic pts
acute presentation of autoimmune hepatitis can be confused for what other pathologies
acute viral or drug induce hepatitis
more commonly sx are non-specific fatigue, anorexia, nausea, vomiting
between PBC and PSC which primarily affects men
primary sclerosing cholangitis
what is primary biliary cholangitis
autoimmune disease characterized by inflammatory destruction of small and medium sized intrahepatic bile ducts
What is the etiology of PBC
t lymphocyte mediated attack on on small interlobular bile ducts.
describe clinical features of PBC
- Asymptomatic - elevated serum alk phos
- Symptomatic - pruritus and fatigue
- splenomegaly and jaundice in advance disease
- hypercholesterolemia
- antimitochondrial antibodies
how do you establish dx of PBC
2 or more of the following
elevated alk phos; >6 mo
positive antimitochondrial antibodies
histologic findings
what kind of cancer is PBC associated with
HCC
describe PSC
inflammation and obliterative fibrosis of extrahepatic and large intrahepatic ducts and dilation of preserved segments
Male predominance
describe the clinical features of PSC
- 1/2 of pts are asymptomatic at presentation; found during screening UC; elevated alk phos
- fatigue, pruritus and jaundice
- ascending cholangitis can be initial presentation
what is the gold standard for PSC diagnosis
beading seen in large intrahepatic and extrahepatic biliary tree by endoscopic/magnetic retrograde cholangiopancreaticography