A Case of Sclerosing Cholangitis without Pancreatic Involvement Thought to be Associated with Autoimmunity

Abstract

Sclerosing cholangitis (SC) is one of the lesions frequently seen in IgG4-related systemic diseases, causing biliary stricture and mimicking bile duct carcinoma and primary sclerosing cholangitis (PSC). Although it often accompanies autoimmune pancreatitis (AIP), autoimmune-related SC without a pancreatic lesion is very rare. A 79-year-old woman was referred to our institution with suspected diagnosis of bile duct carcinoma in the previous hospital. The patient was not icteric and fever free, but with an elevated level of serum biliary enzyme, which lead us to detect this disease. Clinical images including computed tomography (CT), endoscopic retrograde cholangiopancreatography (ERCP) and intraductal ultrasonography (IDUS) demonstrated multiple strictures at the intrahepatic bile duct and enhanced wall thickness at the upper common bile duct, however her pancreas was normal. Repeated endoscopic procedures with multiple biopsies from the biliary strictures demonstrated fibrous ductal tissues with lymph-plasma cell infiltration (>10 IgG4(+) cells/HPF). By positron emission tomography using 18 F-fluorodeoxyglucose (FDG-PET), the uptake of FDG was not remarkable in areas other than the biliary lesions. Additional laboratory tests showed elevated levels of serum IgG (2,571 mg/dL), and γ-globulin (29%), and positive autoantibodies, but normal IgG4 (53.2 mg/dL). Together with clinical images, laboratory and histological findings, we diagnosed this patient as sclerosing cholangitis which was thought to be associated with autoimmunity. After one year of follow-up without steroid therapy, idiopathic thrombocytopenic purpura (ITP) developed with an increased level of serological markers. We encountered a rare case of sclerosing cholangitis expected to be associated with autoimmunity, which showed biliary strictures mimicking bile duct carcinoma and needed careful diagnosis. Unlike the typical AIP, the current case demonstrated distinct serological findings and no other organ involvement. Further study is needed to clarify the characteristics of sclerosing cholangitis associated with autoimmunity with a large number of cases.

IgG4‐related sclerosing cholangitis without obvious pancreatic lesion: Difficulty in differential diagnosis

“…In contrast, PSC develops in middle-aged population (30-40 years), almost evenly in men and women (1.4:1), frequently showing eosinophilia, increasing titers of serum pANCA (about 80%) and an association with IBD (63-73%). 5,7,15,17,18 Nakazawa et al 5 analyzed clinicopathological findings in 62 cases of AIP-SC and 40 cases of PSC, and summarized three key parameters, including cholangiographic findings, liver biopsy and an association with IBD, to discriminate between these two entities. Band-like pruned-tree appearances are characteristic cholangiographic views of PSC.…”

“…These findings made us to question the use of the term IgG4-SC for this disease entity and to be concerned about the specificity of a low level cut-off of IgG4-positive cells in biopsy samples (>10 cells/HPF). 16 Autoimmune-related SC 18,25 is thought to be more appropriate for the overall entity and IgG4-SC should be used as a subtype characterized by the elevation of serum IgG4 or a marked aggregation of IgG4-positive cells in the biliary duct. Even in cases with normal serum IgG4, steroid response may be recognized, as in the present cases; therefore, steroid trials are worth pursuing when malignancy is refuted and an association with autoimmunity is suspected.…”

“…2b). 18 performed without histological examination. The resected specimens of the bile duct showed typical SC (Fig.…”

“…18 She had no history of autoimmune or allergic disease, but her father had had rheumatoid arthritis. Her serum CEA level was normal but the CA19-9 was slightly elevated (49 U/mL).…”

Aberrant Confluence of Intrahepatic Biliary Ducts - A Case Report

“…As to the absence of first ductules, it is important for clinical practice for two reasons: the first as a sign of fibro-obliterative deseases [18][19][20][21][22][23][24][25][26] and the second considering minimum detectable duct caliber in imaging radiologic researches [27][28][29]. From LDLT point of view it is important in evaluation of age and functional-size-mass related liver parenchyma changes for donors and recipients.…”