18-5 Flashcards
Autoimmune Hepatitis primarily affects?
Females
Which HLA’s are associated with Autoimmune Hepatitis?
DR3 - White
DR4 - Japanese
DRB1 - South Americans
Autoimmune Hepatitis involves an immune attack on hepatocytes. What cells make up the lymphocytic infiltrate in the liver?
CD4 and CD8 T cells
Which T cells activate B cells (autoantibodies) with Autoimmune Hepatitis?
CD4 T cells
A diagnosis of Autoimmune Hepatitis requires 4 things. What are they?
- Autoantibodies
- Increased serum IgG
- Exclusion of other etiologies
- Supportive Histo findings of the liver
A diagnosis of Autoimmune Hepatitis requires 4 things. What are they?
- Autoantibodies
- Increased serum IgG
- Exclusion of other etiologies
- Supportive Histo findings of the liver
There are 2 types of Autoimmune Hepatitis. Who is affected by each and what autoantibodies are common?
Type 1 - adults
= SMA and ANA
Type 2 - kids
= LKM-1 and ACL-1
There are 2 types of Autoimmune Hepatitis. Who is affected by each and what autoantibodies are common?
Type 1 - adults
= SMA and ANA
Type 2 - kids
= LKM-1 and ACL-1
What fluorescent staining pattern is seen with the ANA with Type 1 Autoimmune Hepatitis?
Homogenous or speckled
List the morphologic findings with Autoimmune Hepatitis
- Clusters of plasma cells
- Emperipolesis
- Rosettes
List the morphologic findings with Autoimmune Hepatitis
- Clusters of plasma cells
- Emperipolesis
- Rosettes
Emperipolesis
- Seen with Autoimmune Hepatitis
= Lymphocytes and plasma cells in the cytoplasm of hepatocytes
Rosettes
- Seen with Autoimmune Hepatitis
= Circular regenerative hepatocytes around a cannaliculus
Common progression of autoimmune liver diseases?
Cirrhosis
HCC
What treatment can cause remission for some Autoimmune Hepatitis patients?
Prednisone +/- Azathioprine
What are 2 Autoimmune Cholangiopathies?
Primary Biliary Cholangitis
Primary Sclerosing Cholangitis
Autoimmune Cholangiopathies are autoimmune disorders of?
Bile ducts
Primary Biliary Cholangitis
Destruction of small intrahepatic bile ducts
Destruction of small intrahepatic bile ducts
Primary Biliary Cholangitis
Who is often affected by Primary Biliary Cholangitis?
Females around 50 with other autoimmune disorders
What antibodies will be present with Primary Biliary Cholangitis?
Antimitochondrial (AMA) targeted against E2 of PDC-E2
AMA (+) targeted against E2 is seen with?
Primary Biliary Cholangitis
What morphologic finding will be seen with Primary Biliary Cholangitis?
Florid duct lesion
= lymphocytic/granulomatous bile duct destruction
Florid duct lesion
Lymphocyte/granulomatous bile duct destruction
– seen with Primary Biliary Cholangitis
Symptoms of Primary Biliary Cholangitis?
- Jaundice, pruritus, fatigue
- Xanthelasmas and hypercholesterolemia
- Splenomegaly
- Skin hyperpigmentation and steatorrhea
- Vitamin D Def. Osteoporosis
Symptoms of Primary Biliary Cholangitis?
- Jaundice, pruritus, fatigue
- Xanthelasmas and hypercholesterolemia
- Splenomegaly
- Skin hyperpigmentatioon and steatorrhea
- Vitamin D Def. Osteoporosis
Symptoms of Primary Biliary Cholangitis?
- Jaundice, pruritus, fatigue
- Xanthelasmas and hypercholesterolemia
- Splenomegaly
- Skin hyperpigmentation and steatorrhea
- Vitamin D Def. Osteoporosis
In patients with Primary Biliary Cholangitis, what can be elevated if they are asymptomatic?
Alkaline phosphatase
Primary Sclerosing Cholangitis
Inflammation and fibrosis of large intrahepatic ducts and extrahepatic ducts with dilation of other segments
Inflammation and fibrosis of large intrahepatic ducts and extrahepatic ducts with dilation of other segments
Primary Sclerosing Cholangitis
Who is commonly affected with Primary Sclerosing Cholangitis?
Males 30-50 with ULCERATIVE COLITIS
What disease do those with Primary Sclerosing Cholangitis usually have?
Ulcerative Colitis (IBD)
There are no specific antibodies associated with Primary Sclerosing Cholangitis, but what is usually (+)?
pANCA - ulcerative colitis
(+) pANCA may correlate with what autoimmune cholangiopathy?
Primary Sclerosing Cholangitis
What HLA is Primary Sclerosing Cholangitis associated with?
HLA-B8
What are the morphologic findings with Primary Sclerosing Cholangitis?
Onion skin fibrosis that turns into tombstone scar
Onion skin fibrosis that turns into tombstone scar
Primary Sclerosing Cholangitis
Symptoms of Primary Sclerosing Cholangitis?
Fatigue, jaundice, pruritus
Symptoms of Primary Sclerosing Cholangitis?
Fatigue, jaundice, pruritus
What other issues can Primary Sclerosing Cholangitis be associated with?
Ascending Cholangitis
Chronic pancreatitis/cholecystitis
What other issues can Primary Sclerosing Cholangitis be associated with?
Ascending Cholangitis
Chronic pancreatitis/cholecystitis
What will be seen on ERCP/MRCP with Primary Sclerosing Cholangitis?
Beading of biliary tree due to strictures and dilations
What will be seen on ERCP/MRCP with Primary Sclerosing Cholangitis?
Beading of biliary tree due to strictures and dilations
Beading of the biliary tree is seen with?
Primary Sclerosing Cholangitis
