Benign biliary disease

Question 1

What are 4 examples of benign causes of biliary obstruction?

Answer 1

1. Chronic pancreatitis
2. IgG4 disease
3. Primary sclerosing cholangitis
4. Surgical injury to bile duct due to lap chole

Question 2

What are 3 causes of biliary obstruction in primary sclerosing cholangitis?

Answer 2

1. Dominant biliary structures
2. Development of cholangiocarcinoma
3. Small biliary stones

Question 3

What is IgG4-related disease?

Answer 3

recently described chronic multisystemic fibroinflammatory disease characterised by unifying histological feature: tumefacien lesions with dense lymphocytic infiltration and storiform fibrosis

Question 4

How do patients typically present with IgG4 related disease?

Answer 4

sub-acutely with swelling of glandular tissue and allergic feature

Question 5

What are 5 commonly involved organs in IgG4 related disease?

Answer 5

1. salivary glands
2. lacrimal glands
3. ear
4. nose
5. sinuses

Question 6

What are 3 abdominal manifestations of IgG4 disease?

Answer 6

1. Autoimmune pancreatitis
2. Steroid-responsive sclerosing cholangitis
3. Retroperitoneal fibrosis

Question 7

What are 2 types of CNS manifestation of IgG4 related disease?

Answer 7

1. Pituitary dysfunction
2. Basal meningitis

Question 8

What are 4 biochemical findings typically seen in IgG4-related disease?

Answer 8

1. Raised IgG4
2. ESR elevated
3. Complement levels low
4. Peripheral blood eosinophilia

Question 9

What is the a management of IgG4-related disease?

Answer 9

where symptomatic management not sufficient, prednisolone given in short term

rituximab considered fo rmore long term management