A 52-year-old man presented with painless jaundice and weight loss.
A computed tomography (CT) scan of the abdomen revealed an inflammatory mass in
the pancreatic head. Endoscopic retrograde cholangiopancreatography (ERCP)
demonstrated a double duct sign ([Fig. 1]), and a
biliary stent was placed. Brush cytology was negative and percutaneous biopsy
showed inflammatory changes. Laparotomy confirmed a large inflammatory mass in
the pancreatic head, and a cholecystojejunostomy was carried out. Several
months later, the patient developed recurrent jaundice and ERCP showed new
diffuse strictures involving the intra- and extrahepatic biliary tree ([Fig. 2]). CT abdomen revealed concentric thickening
of the common bile duct (CBD) with enlarged periportal nodes ([Fig. 3]), and endoscopic ultrasound with fine-needle
aspiration biopsy of the pancreatic head and lymph nodes was benign. A liver
biopsy showed bile duct inflammation and marked cholestasis, and ultrasound
revealed patent vasculature. Colonoscopy as well as autoimmune serology and
IgG4 levels were normal.
A diagnosis of secondary autoimmune pancreatitis associated
sclerosing cholangitis (AIP-SC) was considered. The patient was prescribed
steroids and methotrexate. Several months later ERCP showed marked improvement
of the biliary tree ([Fig. 4]). Over the next 5
years, the patient was maintained on azathioprine 100 mg daily, with
normal liver enzymes. On tapering the azathioprine to 50 mg daily, he
developed acute cholangitis. ERCP showed stricturing of the extrahepatic
biliary tree requiring balloon dilation and stenting ([Fig. 5]). The patient was restarted on a prednisone
taper and azathioprine 75 mg daily, with marked improvement seen on a
cholangiogram taken 3 months later ([Fig. 6]). The
patient is currently well with normal liver enzymes and is taking 75 mg
azathioprine daily.
Fig. 1 Endoscopic retrograde
cholangiogram demonstrating a short, tight stricture (arrow) in the distal
common bile duct with prestenotic dilatation.
Fig. 2 Endoscopic retrograde
cholangiogram demonstrating diffuse stricturing (arrowhead) and dilatation
(arrow) of intrahepatic radicles.
Fig. 3 CT scan of the abdomen
showing concentric thickening of the common hepatic duct (arrow) suggesting
marked inflammation.
Fig. 4 Endoscopic retrograde
cholangiogram demonstrating marked improvement in the diffuse stricturing and
dilatation of branches of intrahepatic biliary radicles (seen in
[Fig. 2]).
Fig. 5 Endoscopic retrograde
cholangiogram demonstrating balloon dilatation (arrow) of a distal bile duct
stricture.
Fig. 6 Endoscopic retrograde
cholangiogram demonstrating marked improvement in the distal bile duct
stricture (arrow) after dilation and several months of immunosuppression.
Clinicians should be aware that AIP-SC can mimic pancreatic cancer.
In addition, sclerosing cholangitis can complicate AIP, particularly after
biliary bypass surgery [1]
[2].
Until the etiology, pathogenesis, and natural history have been defined more
precisely, no definite recommendations with respect to medical therapy can be
made. However, treatment with corticosteroids and/or other immunosuppressive
therapy can be effective in patients presenting with this entity
[3].
Endoscopy_UCTN_Code_CCL_1AZ_2AZ
Endoscopy_UCTN_Code_CCL_1AZ_2AG
