Classification and characteristics of IgG4-associated cholangitis
Dr. med. Christian Dietrich / Philipp Herberg / Professor Dr. med. Ralf Jakobs
Medizinische Klinik C, Klinikum der Stadt Ludwigshafen
Classification and characteristics of IgG4-associated cholangitis and how to differentiate it from hepatobiliary malignancies — mimicry in hepatobiliary endoscopy
On the basis of a clinical case, typical endoscopic findings and diagnostic criteria for hepatobiliary IgG4 disease are described here, along with the classification of the condition. The guideline of the European specialist association (United European Gastroenterology, UEG) is also discussed.
A 73-year-old male patient with a suspected diagnosis of neoplasia in the pancreaticobiliary system was referred to us with jaundice, B symptoms, and upper abdominal symptoms, following magnetic resonance cholangiopancreatography (MRCP) at an outside center. The laboratory findings from his family physician showed raised liver values suggesting cholestasis (total bilirubin 11.8 mg/dL; ref. < 1.2 mg/dL) and a raised CA19-9 value (150 U/mL; ref. < 37 U/mL).
The UEG European guideline  proposes a classification into four types, which was originally first presented by Nakazawa et al. . Following extensive biopsy sampling from the biliary duct system, two endoprostheses were introduced here in suprahilar locations on the left and right.
Although the Nakazawa classification is of central importance for the differential diagnosis as a purely morphological description, it is only one of many different factors in the treatment and course of the condition.
|Bile duct configuration||Differential diagnosis|
|Type 1||Distal CCC, carcinoma of the pancreatic head, chronic pancreatitis|
|Types 2a and 2b||Primary or secondary sclerosing cholangitis|
|Type 3||Distal CCC, carcinoma of the pancreatic head with hilar metastases|
|Type 4||Klatskin tumor|
A diagnosis of gastrointestinal manifestations of IgG4 disease is established in the same way as with the other forms of this systemic disease, based on the HISORt criteria : histology, typical imaging findings, serology (which has a high diagnostic value if IgG4 > 4× upper limit of normal), manifestations in several organs, and the response to treatment with steroids.
In the present case, the IgG4 serum level was raised by more than tenfold, and treatment with prednisolone 40 mg was therefore initiated for IgG4 cholangitis in combination with autoimmune pancreatitis.
1. Löhr JM, Beuers U, Vujasinovic M. et al.; UEG guideline working group. European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations. United European Gastroenterol J. 2020 Jul;8(6):637-666. doi: 10.1177/2050640620934911. Epub 2020 Jun 18. PMID: 32552502; PMCID: PMC7437085.
2. Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol. 2013;19(43):7661-7670. doi:10.3748/wjg.v19.i43.7661