Background: Even though IgG4-related disease has gained increased attention worldwide, the diagnosis remains challenging. IgG4-related sclerosing cholangitis (IgG4-SC) is not well described in the western hemisphere and may mimic cholangiocarcinoma (CC), especially when occurring without other symptoms such as, e.g. concurrent pancreatitis or retroperitoneal fibrosis. We present a case to add further information to the diagnosis and treatment of this challenging disease.
Case report: A 60-year-old male patient presented with painless jaundice. Prior medical history showed diabetes mellitus type I, high blood pressure, and deep vein thrombosis. Diagnostic investigations were strongly suspicious of a Klatskin tumor, although biopsies were inconclusive. The tumor marker Carbohydrate Antigen 19-9 (CA 19-9) was elevated. Prior to the recommended surgery, the patient had two second opinions in two different university hospitals, both arguing for surgery as well. The patient received hilar resection with right hemihepatectomy. During the postoperative course, some major complications occurred, i.e. recurrent pleural effusion, abscess in the liver resection area, sepsis, ileus, and restricted liver metabolism. Treatment with prednisolone did not show any improvement. Approximately 3 months after surgery, the patient died in consequence of acute respiratory failure. Histology showed no signs of CC, but IgG4-SC could be diagnosed.
Conclusion: In the case of preoperative signs of CC, differential diagnosis of IgG4-SC needs to be considered, in particular, in cases with missing histologic proof of malignant disease.
Keywords: IgG4; IgG4-related disease; autoimmune biliary disease; cholangiocarcinoma; sclerosing cholangitis.
©2018 Mittelstaedt A., et al., published by De Gruyter, Berlin/Boston.
IgG4-related Sclerosing Cholangitis Mimicking CholangiocarcinomaM Nguyen-tat et al. Z Gastroenterol 50 (9), 1008-12. PMID 22965631. - Case ReportsIgG4-related disease has gained increased attention worldwide. While the initial focus was on autoimmune pancreatitis which was first described in Asian populations and t …
IgG4-associated Sclerosing Cholangitis Masquerading as Hilar CholangiocarcinomaKS Yadav et al. Indian J Gastroenterol 35 (4), 315-8. PMID 27439915. - Case ReportsIgG4-sclerosing cholangitis (IgG4-SC) commonly presents with type 1 autoimmune pancreatitis. Isolated IgG4-SC is rare. Differentiating IgG4-SC from cholangiocarcinoma pre …
IgG4-Associated Cholangitis Can Mimic Hilar CholangiocarcinomaVM Zaydfudim et al. Gut Liver 9 (4), 556-60. PMID 26033685. - Case ReportsIgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated se …
Diagnosis of IgG4-related Sclerosing CholangitisT Nakazawa et al. World J Gastroenterol 19 (43), 7661-70. PMID 24282356. - ReviewIgG4-related sclerosing cholangitis (IgG4-SC) is often associated with autoimmune pancreatitis. However, the diffuse cholangiographic abnormalities observed in IgG4-SC ma …
Biliary and Hepatic Involvement in IgG4-related DiseaseD Joshi et al. Aliment Pharmacol Ther 40 (11-12), 1251-61. PMID 25312536. - ReviewIgG4-related sclerosing cholangitis is a common manifestation of IgG4-related disease which requires a multi-disciplinary approach to establish the diagnosis. Differentia …
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