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Review
. 2015 Jun;31(3):185-8.
doi: 10.1159/000431028. Epub 2015 Jun 9.

How to Diagnose Immunoglobulin G4-Associated Cholangitis: The Jack-of-All-Trades in the Biliary Tract

Lowiek M Hubers  1 , Ulrich Beuers  1
Affiliations
Review

How to Diagnose Immunoglobulin G4-Associated Cholangitis: The Jack-of-All-Trades in the Biliary Tract

Lowiek M Hubers et al. Viszeralmedizin. 2015 Jun.

Abstract

Background: Immunoglobulin (Ig) G4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract displaying characteristic features of IgG4-related disease (IgG4-RD): elevation of IgG4 serum levels, infiltration of IgG4+ plasma cells in the affected tissue, and good response to immunosuppressive treatment.

Methods and results: The clinical presentation of IAC is often misleading, mimicking other diseases of the biliary tract such as cholangiocarcinoma or primary and secondary sclerosing cholangitis. The HISORt criteria form the cornerstone in the diagnosis of IAC, combining histopathological (H), imaging (I), and serological (S) features including serum IgG4, other organ manifestations (O) of IgG4-RD and response to treatment (Rt). The accuracy of the HISORt criteria is limited. Novel diagnostic approaches are under evaluation.

Conclusion: More accurate biomarkers are needed to correctly diagnose IgG4-RD and prevent misdiagnoses and unnecessary therapeutic interventions.

Keywords: CCA; Cholangiocarcinoma; IgG4-related systemic disease; PSC; Primary sclerosing cholangitis.

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Figures

Fig. 1
Fig. 1
A 69-year-old painter presented with obstructive jaundice and weight loss. Dilated intrahepatic bile ducts were observed on CT scan. Endoscopic retrograde cholangiopancreatography showed a hilar stenosis suspect for a Klatskin tumor Bismuth type IIIa. Serum IgG4 was 1,440 mg/dl (normal <140 mg/dl). The stenosis dissolved completely after treatment with prednisolone (20 mg/day).
Fig. 2
Fig. 2
A 58-year-old warehouse man of a truck manufacturing company presented with weight loss, jaundice, and abdominal pain. Serum IgG4 was 166 mg/dl. A CT scan revealed a sausage-shaped pancreas, which normalized after prednisolone treatment (20 mg/day).
Fig. 3
Fig. 3
HISORt criteria for the diagnosis of AIP and IAC (histology, imaging, serology, other organ involvement, and response to therapy) (modified from Alderlieste et al. [20] and Hubers et al. [6]. FNA = Fine-needle aspiration; IgG4-RD = IgG4-related disease; CT = computed tomography; MRI = magnetic resonance imaging; MRCP = magnetic resonance cholangiopancreatography; ULN = upper limit of normal.
See this image and copyright information in PMC

References

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