Pathology of the GI Tract- Liver and Gallbladder (5) Flashcards
Autoimmune hepatitis has a strong association with HLA alleles. What are the 3?
DR3 in Caucasians; DR4 in Japanese; and DRB1 in South Americans
In patients with AIH, what is the lymphocytic infiltrate like?
it is composed predominantly of CD4+ helper T cells with with CD8+ cytotoxic T cells at the interface
the diagnosis of AIH is based on the combination of what 4 features?
- exclusion of other etiologies 2. autoantibodies 3. elevation of serum IgG 4. supportive histologic findings on liver biopsy
What are the histologic features of autoimmune hepatitis?
a focus of lobular hepatitis with prominent plasma cells
What are the different types of AIH?
type 1 (adults) and type 2 (children)
how is type 1 AIH characterized?
by the presence of either ANA or anti-smooth muscle actin (SMA) antibodies
how is type 2 AIH characterized?
anti-liver kidney microsome-1 (LKM-1) antibodies
what is the treatment of choice for AIH?
immunosuppression with prednisone with or without azathioprine
what is the common presentation of AIH?
nonspecific symptoms such as fatigue, anorexia, nausea, and abdominal pain
What are the 2 autoimmune cholangiopathies?
primary biliary cholangitis and primary sclerosing cholangitis
what is the median age of diagnosis for PBC?
50 years
what is the median age of diagnosis for PSC?
30 years
what gender is most likely to be affected by PBC?
females
what gender is most likely to be affects by PSC?
males
what are the conditions commonly associated with PBC?
other autoimmune diseases Sjogren syndrome, thyroid disease, and scleroderma
what are the conditions commonly associated with PSC?
IBD–> UC
what is the serology like in PBC?
95% AMA-positive; 40-50% ANA positive
what is the serology like in PSC?
65% ANCA positive; ANA variable; AMA typically negative
what are the radiologic findings in PBC?
normal findings
what are the radiologic findings in PSC?
strictures and beading of large bile ducts’ pruning of smaller ducts
what are the duct lesions like in PBC?
florid duct lesions; loss of small ducts
what are the duct lesions like in PSC?
there is inflammatory destruction of extrahepatic and large intrahepatic ducts
how is PBC characterized/ defined?
inflammatory destruction of small- and medium-sized intrahepatic bile ducts
what are the common clinical manifestations seen in PBC?
fatigue with pruritus, splenomegaly and jaundice, skin hyperpigmentation, xanthelasmas, steatorrhea, and vitamin D malabsorption (leads to osteomalacia or osteoporosis)
what is the most characteristic finding in PBC?
AMA directed against PDC-E2
how can you make the diagnosis of PBC?
the diagnosis can be established if two of the following are present: elevated alk phos for more than 6 months, positive test for AMA, and characteristic histologic findings
how do you treat PBC?
oral ursodeoxycholic acid, a naturally occurring bile acid, slows disease progression; liver transplantation
how is PSC characterized/defined?
by inflammation and obliterative fibrosis of extrahepatic and large intrahepatic ducts and dilation of preserved segments
what is the histologic features associated with PSC?
a degenerating bile duct is entrapped in a dense “onion skin” concentric scar
what are the most common presenting symptoms in PSC?
fatigue, pruritus, jaundice; ascending cholangitis
what is the gold standard for diagnosis of PSC?
the characteristic beading seen in the large intrahepatic and extrahepatic biliary tree by ERCP/MRCP
