A 36-year-old woman with no significant past medical history presented with fatigue
and a 5-kg weight loss. Blood work-up showed isolated elevated γ-glutamyltransferase
(3 × upper limit of normal). Magnetic resonance cholangiopancreatography (MRCP) demonstrated
a short stricture of the left main intrahepatic duct (IHD) with upstream dilatation
of the IHDs ([Fig. 1]). The biliary tract was otherwise unremarkable.
Fig. 1 Radiographic images showing: a on magnetic resonance cholangiography, a marked stenosis of the left main hepatic
duct (arrow) with dilatation of left intrahepatic ducts (the right intrahepatic ducts
and common bile duct are normal); b on gadolinium-enhanced T1-weighted magnetic resonance imaging, dilatation of left
intrahepatic ducts with contrast enhancement of the biliary ducts walls (arrows).
An endoscopic retrograde cholangiopancreatography (ERCP) was performed with retrograde
cholangioscopy. The endoscopic appearance of the stricture was worrisome, with irregular
pattern and anarchic vascularization ([Fig. 2]; [Video 1]). Different sampling techniques of the stricture, including brush cytology, bile
aspiration, and multiple targeted biopsies, were used. Pathology showed non-specific
signs of chronic inflammation. The patient’s serum IgG4 levels were non-significantly
elevated (1.3 × normal). The case was discussed at a multidisciplinary meeting and
it was decided to perform surgery, given the patient’s weight loss, asthenia, and
the suspicion of underlying neoplasia. A left hepatectomy with lymphadenectomy was
performed. Final pathology showed lesions of sclerosing cholangitis at the level of
the IHDs, with significant inflammation and IgG4 infiltration of the stenotic region,
but no tumor cells ([Fig. 3]). A positron emission tomography (PET) scan showed no signs of involvement of other
organs. The patient has had no further events during 1 year of follow-up.
Fig. 2 Cholangioscopic view of the left main intrahepatic duct stricture showing an irregular
vascularity and pattern.
Video 1 Cholangioscopy showing an isolated left main intrahepatic duct stricture with worrisome
features.
Fig. 3 Histopathological appearance of the resected tissue showing: a a bile duct with periductal fibrosis and plasma cell-rich inflammation on hematoxylin
and eosin (H&E) staining; b immunostaining with IgG4 highlighting numerous IgG4-positive plasma cells (> 50 per
high power field).
IgG4-related sclerosing cholangitis is an autoimmune biliary tract disease [1]
[2]. Amongst the IgG4-related diseases, bile duct and kidney involvement are usually
associated with manifestations in other organs, specifically the pancreas [3]. The lower bile duct is the most commonly involved [4]. Cholangioscopy has a high specificity and fair level of sensitivity for the diagnosis
of cholangiocarcinoma [5] and can therefore be helpful in differentiating malignant from benign strictures
[4].
This case illustrates how challenging the diagnosis of an indeterminate isolated IHD
stricture is. Endoscopic characterization of IgG4 sclerosing cholangitis lacks sensitivity.
Furthermore, detection of IgG4-positive plasma cells in cholangioscopy-targeted biopsies
remains difficult.
Endoscopy_UCTN_Code_CCL_1AZ_2AZ
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