18-5 Flashcards

1
Q

Autoimmune Hepatitis primarily affects?

A

Females

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2
Q

Which HLA’s are associated with Autoimmune Hepatitis?

A

DR3 - White
DR4 - Japanese
DRB1 - South Americans

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3
Q

Autoimmune Hepatitis involves an immune attack on hepatocytes. What cells make up the lymphocytic infiltrate in the liver?

A

CD4 and CD8 T cells

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4
Q

Which T cells activate B cells (autoantibodies) with Autoimmune Hepatitis?

A

CD4 T cells

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5
Q

A diagnosis of Autoimmune Hepatitis requires 4 things. What are they?

A
  1. Autoantibodies
  2. Increased serum IgG
  3. Exclusion of other etiologies
  4. Supportive Histo findings of the liver
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6
Q

A diagnosis of Autoimmune Hepatitis requires 4 things. What are they?

A
  1. Autoantibodies
  2. Increased serum IgG
  3. Exclusion of other etiologies
  4. Supportive Histo findings of the liver
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7
Q

There are 2 types of Autoimmune Hepatitis. Who is affected by each and what autoantibodies are common?

A

Type 1 - adults
= SMA and ANA
Type 2 - kids
= LKM-1 and ACL-1

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8
Q

There are 2 types of Autoimmune Hepatitis. Who is affected by each and what autoantibodies are common?

A

Type 1 - adults
= SMA and ANA
Type 2 - kids
= LKM-1 and ACL-1

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9
Q

What fluorescent staining pattern is seen with the ANA with Type 1 Autoimmune Hepatitis?

A

Homogenous or speckled

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10
Q

List the morphologic findings with Autoimmune Hepatitis

A
  • Clusters of plasma cells
  • Emperipolesis
  • Rosettes
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11
Q

List the morphologic findings with Autoimmune Hepatitis

A
  • Clusters of plasma cells
  • Emperipolesis
  • Rosettes
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12
Q

Emperipolesis

A
  • Seen with Autoimmune Hepatitis

= Lymphocytes and plasma cells in the cytoplasm of hepatocytes

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13
Q

Rosettes

A
  • Seen with Autoimmune Hepatitis

= Circular regenerative hepatocytes around a cannaliculus

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14
Q

Common progression of autoimmune liver diseases?

A

Cirrhosis

HCC

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15
Q

What treatment can cause remission for some Autoimmune Hepatitis patients?

A

Prednisone +/- Azathioprine

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16
Q

What are 2 Autoimmune Cholangiopathies?

A

Primary Biliary Cholangitis

Primary Sclerosing Cholangitis

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17
Q

Autoimmune Cholangiopathies are autoimmune disorders of?

A

Bile ducts

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18
Q

Primary Biliary Cholangitis

A

Destruction of small intrahepatic bile ducts

19
Q

Destruction of small intrahepatic bile ducts

A

Primary Biliary Cholangitis

20
Q

Who is often affected by Primary Biliary Cholangitis?

A

Females around 50 with other autoimmune disorders

21
Q

What antibodies will be present with Primary Biliary Cholangitis?

A

Antimitochondrial (AMA) targeted against E2 of PDC-E2

22
Q

AMA (+) targeted against E2 is seen with?

A

Primary Biliary Cholangitis

23
Q

What morphologic finding will be seen with Primary Biliary Cholangitis?

A

Florid duct lesion

= lymphocytic/granulomatous bile duct destruction

24
Q

Florid duct lesion

A

Lymphocyte/granulomatous bile duct destruction

– seen with Primary Biliary Cholangitis

25
Q

Symptoms of Primary Biliary Cholangitis?

A
  • Jaundice, pruritus, fatigue
  • Xanthelasmas and hypercholesterolemia
  • Splenomegaly
  • Skin hyperpigmentation and steatorrhea
  • Vitamin D Def. Osteoporosis
26
Q

Symptoms of Primary Biliary Cholangitis?

A
  • Jaundice, pruritus, fatigue
  • Xanthelasmas and hypercholesterolemia
  • Splenomegaly
  • Skin hyperpigmentatioon and steatorrhea
  • Vitamin D Def. Osteoporosis
27
Q

Symptoms of Primary Biliary Cholangitis?

A
  • Jaundice, pruritus, fatigue
  • Xanthelasmas and hypercholesterolemia
  • Splenomegaly
  • Skin hyperpigmentation and steatorrhea
  • Vitamin D Def. Osteoporosis
28
Q

In patients with Primary Biliary Cholangitis, what can be elevated if they are asymptomatic?

A

Alkaline phosphatase

29
Q

Primary Sclerosing Cholangitis

A

Inflammation and fibrosis of large intrahepatic ducts and extrahepatic ducts with dilation of other segments

30
Q

Inflammation and fibrosis of large intrahepatic ducts and extrahepatic ducts with dilation of other segments

A

Primary Sclerosing Cholangitis

31
Q

Who is commonly affected with Primary Sclerosing Cholangitis?

A

Males 30-50 with ULCERATIVE COLITIS

32
Q

What disease do those with Primary Sclerosing Cholangitis usually have?

A

Ulcerative Colitis (IBD)

33
Q

There are no specific antibodies associated with Primary Sclerosing Cholangitis, but what is usually (+)?

A

pANCA - ulcerative colitis

34
Q

(+) pANCA may correlate with what autoimmune cholangiopathy?

A

Primary Sclerosing Cholangitis

35
Q

What HLA is Primary Sclerosing Cholangitis associated with?

A

HLA-B8

36
Q

What are the morphologic findings with Primary Sclerosing Cholangitis?

A

Onion skin fibrosis that turns into tombstone scar

37
Q

Onion skin fibrosis that turns into tombstone scar

A

Primary Sclerosing Cholangitis

38
Q

Symptoms of Primary Sclerosing Cholangitis?

A

Fatigue, jaundice, pruritus

39
Q

Symptoms of Primary Sclerosing Cholangitis?

A

Fatigue, jaundice, pruritus

40
Q

What other issues can Primary Sclerosing Cholangitis be associated with?

A

Ascending Cholangitis

Chronic pancreatitis/cholecystitis

41
Q

What other issues can Primary Sclerosing Cholangitis be associated with?

A

Ascending Cholangitis

Chronic pancreatitis/cholecystitis

42
Q

What will be seen on ERCP/MRCP with Primary Sclerosing Cholangitis?

A

Beading of biliary tree due to strictures and dilations

43
Q

What will be seen on ERCP/MRCP with Primary Sclerosing Cholangitis?

A

Beading of biliary tree due to strictures and dilations

44
Q

Beading of the biliary tree is seen with?

A

Primary Sclerosing Cholangitis