Differential diagnosis of IgG4-SC^[1-3]

Favors IgG4-SC	Favors PSC	Favors secondary cholangitis	Favors malignancy
Extrahepatic stenosis	Intrahepatic stenosis	Diffuse or focal	Focal with cut off, liver invasion
Male sex	Male sex		No difference by sex, old age
Pancreatic involvement (89%), OOI (80%)*	IBD (80%, typically ulcerative colitis)	Bile duct stones, gallbladder stones, previous surgeries (eg, Roux-En-Y)	Chronic inflammation, viral hepatitis, liver flukes, choledochal cysts
Elevated IgG4			Somatic genetic factors
Jaundice common	Jaundice uncommon	Variable	Jaundice common
Abdominal pain	Pain uncommon	Fever, pain, WBC	Pruritus, weight loss, pale stools, dark urine
Responses to steroids, immunosuppression

IgG4-SC: immunoglobulin G4-related sclerosing cholangitis; PSC: primary sclerosing cholangitis; OOI: other organ involvement; IBD: inflammatory bowel disease; WBC: white blood cells.
* Refer to UpToDate topic on autoimmune pancreatitis.

References:

Banales JM, Cardinale V, Carpino G, et al. Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA). Nat Rev Gastroenterol Hepatol 2016; 13:261.
Okazaki K, Uchida K, Koyabu M, et al. IgG4 cholangiopathy: current concept, diagnosis, and pathogenesis. J Hepatol 2014; 61: 690.
Ali AH, Bi Y, Machicado JD, et al. The long-term outcomes of patients with immunoglobulin G4-related sclerosing cholangitis: the Mayo Clinic experience. J Gastroenterol 2020; 55:1087.

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Differential diagnosis of IgG4-SC[1-3]

Differential diagnosis of IgG4-SC^[1-3]