Cholangitis - PSC, Autoimmune, IgG4, Mirizzi

Question 1

PSC - characteristics

Answer 1

inflammation, fibrosis, and stricturing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree

**stricture

Question 2

PSC - associated condition

Answer 2

associated with UC, less often with Crohns

Question 3

PSC - CF and Ix

Answer 3

Diagnosis of exclusion - other causes of stricture like Primary biliary Colangitis, IgG4 disease etc

often asymptomatic at diagnosis, may p/w fatigue, pruritis –>found to have abnormal LFTs

Usually ALP predominant, increased Ig levels (30%), p-ANCA +ve (30-80%), HLA DRw52a

Question 4

PSC - Imaging findings

Answer 4

-Beaded appearance of bile duct with strictures

Question 5

PSC - Biopsy

Answer 5

Fibrous obliteration of bile ducts can occur; the classical lesion is a concentric ‘onion skin’ fibrosis around an affected duct
Granulomas not typically seen

Question 6

Autoimmune cholangitis

Answer 6

AMA negative primary biliary cirrhosis

Histopathology of liver tissue reveals bile duct injury in the form of cholangitis and ductopenia with little or no portal inflammation.

Question 7

Mirizzi syndrome - features

Answer 7

• common hepatic duct obstruction caused by extrinsic compression from an impacted stone in the cystic duct or infundibulum of the gallbladder
• a/w gallbladder ca
• p/w recurrent cholangitis or painful jaundice
• Managed surgically

Question 8

IgG4 disease features

Answer 8

• Increasingly recognized syndrome of unknown etiology -Comprised of a collection of disorders, Most patients are men (60-80%) > 50 years old
• Characterized by lymphoplasmacytic infiltration and enlargement of multiple organs

Histo: Storiform (spokes on a wheel–appearing) fibrosis. abundant IgG4 plasma cells which are seen on biopsy

Question 9

IgG4 presentation

Treatment

Answer 9

Multiple, ex:

Inflammatory aortitis/vasculitis
Autoimmune pancreatitis**
Ormond disease (retroperitoneal fibrosis)
Tubulointerstitial nephropathy
Sclerosing cholangitis

Mx: immunosuppression - pred or steroid sparing agents